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Sample records for vasculitis study group

  1. Brief Report: Childhood-Onset Systemic Necrotizing Vasculitides: Long-Term Data From the French Vasculitis Study Group Registry.

    Science.gov (United States)

    Iudici, Michele; Puéchal, Xavier; Pagnoux, Christian; Quartier, Pierre; Agard, Christian; Aouba, Achille; Büchler, Matthias; Cevallos, Ramiro; Cohen, Pascal; de Moreuil, Claire; Guilpain, Philippe; Le Quellec, Alain; Roblot, Pascal; Serratrice, Jacques; Bachmeyer, Claude; Daugas, Éric; Terrier, Benjamin; Mouthon, Luc; Guillevin, Loïc

    2015-07-01

    To describe the initial features and long-term outcomes of childhood-onset small vessel and medium vessel systemic necrotizing vasculitides (SNVs), including antineutrophil cytoplasmic antibody-associated vasculitides (AAVs) and polyarteritis nodosa (PAN). Data on patients with childhood-onset SNV registered in the French Vasculitis Study Group database were reviewed for demographic characteristics, clinical, laboratory, and histologic features, and outcomes. Disease activity was assessed with the Birmingham Vasculitis Activity Score and the Paediatric Vasculitis Activity Score, and damage was scored using the Vasculitis Damage Index. Relapse and survival rates and causes of death were analyzed. Fifty-six patients (35 with AAV and 21 with PAN) (median age at database enrollment 14 years [range 2-17]) were included in the study. The median duration of followup was 96 months (range 1-336); two-thirds of the patients were followed up beyond 18 years of age. Six patients (11%) died, mostly of SNV-related causes. Relapse rates ranged from 33% for microscopic polyangiitis to 50% for eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and 83% for granulomatosis with polyangiitis (Wegener's), with similar rates among AAV and PAN patients (76% and 75%, respectively); neither overall survival nor relapse-free survival differed significantly between the 2 disease groups. Rates of relapse increased after 18 years of age, both among patients with AAV and among patients with PAN. At the last followup evaluation, AAV patients had more major flares and more severe accrued damage compared with PAN patients. Despite similar relapse rates, patients with childhood-onset AAVs experienced more major flares with more cumulative damage than those with pediatric PAN. Treatments aimed at reducing the rates of mortality and relapse in this patient group need to be developed and assessed. © 2015, American College of Rheumatology.

  2. Vasculitis

    Science.gov (United States)

    ... nerves. It is crucial, of course, to prevent death and long-term disability from vasculitis. Yet, other issues often trouble patients. These include fatigue (feeling very tired), pain, arthritis, nose and sinus problems, ...

  3. A cross-sectional study of the Birmingham Vasculitis Activity Score version 3 in systemic vasculitis.

    LENUS (Irish Health Repository)

    Suppiah, Ravi

    2011-05-01

    Assessment of disease activity in vasculitis can be achieved using the BVAS, a clinical checklist of relevant symptoms, signs and features of active disease. The aim of this study was to revalidate the BVAS version 3 (BVAS v. 3) in a cohort of patients with systemic vasculitis.

  4. Urticarial vasculitis in northern Spain: clinical study of 21 cases

    National Research Council Canada - National Science Library

    Loricera, Javier; Calvo-Río, Vanesa; Mata, Cristina; Ortiz-Sanjuán, Francisco; González-López, Marcos A; Alvarez, Lino; González-Vela, M Carmen; Armesto, Susana; Fernández-Llaca, Héctor; Rueda-Gotor, Javier; González-Gay, Miguel A; Blanco, Ricardo

    2014-01-01

    Urticarial vasculitis (UV) is a subset of cutaneous vasculitis (CV), characterized clinically by urticarial skin lesions of more than 24 hours' duration and histologically by leukocytoclastic vasculitis...

  5. Urticarial Vasculitis

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    ... Store Home / Resources / Forms of Vasculitis / Urticarial Vasculitis Urticarial Vasculitis Urticarial vasculitis is a form of vasculitis that ... of complements; it’s called hypocomplementemic vasculitis. What causes Urticarial Vasculitis? The cause of most cases of urticarial vasculitis ...

  6. Urticarial vasculitis: a retrospective study of 15 cases.

    Science.gov (United States)

    Moreno-Suárez, F; Pulpillo-Ruiz, Á; Zulueta Dorado, T; Conejo-Mir Sánchez, J

    2013-09-01

    Urticarial vasculitis is a subtype of vasculitis characterized clinically by urticarial lesions and histologically by necrotizing vasculitis. To study the clinical and histologic features of urticarial vasculitis in patients seen in the dermatology department of Hospital Universitario Virgen de Rocío in Seville, Spain, and to examine the association between hypocomplementemia and systemic disease. We performed a chart review of histologically confirmed cases of urticarial vasculitis in the database of our department covering a period of 10 years. Fifteen patients (9 women and 6 men with a median age of 51 years) were included. In 14 patients (93%), the lesions persisted for more than 24hours, and in 9 cases (60%) the lesions resolved leaving residual purpura or hyperpigmentation. Seven patients (47%) had low complement levels in the blood, 12 (80%) had extracutaneous symptoms, and 8 (53%) had associated systemic disease, the most common of which was systemic lupus erythematosus. Urticarial vasculitis may be underdiagnosed. Response to treatment is variable, and hypocomplementemia and extracutaneous symptoms may indicate the presence of associated systemic disease. Copyright © 2010 Elsevier España, S.L. and AEDV. All rights reserved.

  7. Vasculitis integrated clinical assessment database: a data management system to support studies into systemic vasculitis.

    Science.gov (United States)

    Wilson, Andrew S; Bacon, Paul A; Young, Stephen P; Carruthers, David M

    2010-01-01

    The systemic vasculitides are characterized by immune inflammation affecting blood vessels, which can lead to organ and tissue damage. Treatment has improved but optimum long-term management still remains unsatisfactory, requiring ongoing therapeutic studies. These often base their measures of efficacy on the outcome of clinical assessments which include the Birmingham Vasculitis Activity Score and the Vasculitis Damage Index. Efficient management of assessment data is complex and often hampered by working with time-consuming paper-based systems. The Vasculitis Integrated Clinical Assessment Database (VICAD) was created to improve the process. VICAD was developed using Microsoft Access. Visual Basic for Applications and the Data Access Objects Application Programming Interface provide the functionality to assist with scoring, calculation of results, and storing of data. VICAD is an efficient system for managing data. Evaluation of its use showed an improvement in the completeness of patient assessments from 77% (paper based: n = 44) to 98% (computer based: n = 30). During development clinicians (n = 4) rated it at 8/10 for its layout and visual presentation and 8/10 for easy to use (intuitiveness and navigability). The development of an integrated and standardized system of data collection (VICAD) helps to support clinical decision making processes and report findings in a more timely manner. It is available free for use and modification under the GNU General Public License. The open source nature of VICAD could help to inform the design of other databases where management of complex information into important multisystem diseases is needed.

  8. Equine pastern vasculitis: a clinical and histopathological study.

    Science.gov (United States)

    Psalla, Dimitra; Rüfenacht, Silvia; Stoffel, Michael H; Chiers, Koen; Gaschen, Véronique; Doherr, Marcus G; Gerber, Vincent; Welle, Monika M

    2013-11-01

    Equine pastern vasculitis is clinically challenging and the underlying aetiopathogenesis is unclear. The aims of this retrospective study were to establish histopathological criteria for pastern vasculitis, to look for an underlying cause, to investigate whether the histopathological lesions are associated with a distinct clinical picture, to assess if and how the clinical picture varies, and to determine the treatment response. Skin biopsies and clinical data from 20 horses with a diagnosis of vasculitis of the distal extremities were investigated and histology was compared to biopsies from healthy horses. It was concluded that intramural inflammatory cells, leukocytoclasia with nuclear dust, thickening and oedema of the vessel walls, and microhaemorrhages are highly specific histological findings in equine pastern vasculitis. Based on the feedback from the clinicians, the lesions were mostly seen on the lateral and medial aspects of un-pigmented legs. Lesions in white skin were characterised by exudation and crusts, whereas those in pigmented skin were alopecic and characterised by scaling. The response to treatment was poor and the prognosis guarded. No association was found between any of the histopathological findings and a distinct clinical picture. An underlying cause of equine pastern vasculitis could not be identified. Considering the large number of confounding factors, the causative agents are difficult to identify, but may involve drugs or a hypersensitivity reactions to yet unknown antigens. Copyright © 2013 Elsevier Ltd. All rights reserved.

  9. Clinical and molecular studies in ANCA associated vasculitis

    OpenAIRE

    Wendt, Mårten

    2014-01-01

    ANCA associated vasculitis (AAV) is a heterogeneous group of diseases characterised by sterile pauci-immune systemic small vessel inflammation and closely associated with the presence of anti-neutrophil cytoplasmatic antibodies (ANCA). Although AAV can affect any organ, the kidney, skin, lungs and upper and lower airways are most commonly involved. In some patients there is granuloma formation and in some asthma and eosinophilia, and based on this patients can be further classi...

  10. Urticarial vasculitis in northern Spain: clinical study of 21 cases.

    Science.gov (United States)

    Loricera, Javier; Calvo-Río, Vanesa; Mata, Cristina; Ortiz-Sanjuán, Francisco; González-López, Marcos A; Alvarez, Lino; González-Vela, M Carmen; Armesto, Susana; Fernández-Llaca, Héctor; Rueda-Gotor, Javier; González-Gay, Miguel A; Blanco, Ricardo

    2014-01-01

    Urticarial vasculitis (UV) is a subset of cutaneous vasculitis (CV), characterized clinically by urticarial skin lesions of more than 24 hours' duration and histologically by leukocytoclastic vasculitis. We assessed the frequency, clinical features, treatment, and outcome of a series of patients with UV. We conducted a retrospective study of patients with UV included in a large series of unselected patients with CV from a university hospital. Of 766 patients with CV, UV was diagnosed in 21 (2.7%; 9 male and 12 female patients; median age, 35 yr; range, 1-78 yr; interquartile range, 5-54 yr). Eight of the 21 cases were aged younger than 20 years old. Potential precipitating factors were upper respiratory tract infections and drugs (penicillin) (n = 4; in all cases in patients aged urticarial lesions, other features such as palpable purpura (n = 7), arthralgia and/or arthritis (n = 13), abdominal pain (n = 2), nephropathy (n = 2), and peripheral neuropathy (n = 1) were observed. Hypocomplementemia (low C4) with low C1q was disclosed in 2 patients. Other abnormal laboratory findings were leukocytosis (n = 7), increased erythrocyte sedimentation rate (n = 6), anemia (n = 4), and antinuclear antibody positivity (n = 2). Treatment included corticosteroids (n = 12), antihistaminic drugs (n = 6), chloroquine (n = 4), nonsteroidal antiinflammatory drugs (n = 3), colchicine (n = 2), and azathioprine (n = 1). After a median follow-up of 10 months (interquartile range, 2-38 mo) recurrences were observed in 4 patients. Apart from 1 patient who died because of an underlying malignancy, the outcome was good with full recovery in the remaining patients. In conclusion, our results indicate that UV is rare but not exceptional. In children UV is often preceded by an upper respiratory tract infection. Urticarial lesions and joint manifestations are the most frequent clinical manifestation. Low complement serum levels are observed in a minority of cases. The prognosis is generally good

  11. Clinical study on single-organ cutaneous small vessels vasculitis (SoCSVV)

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    Pastuszczak, Maciej; Celińska-Löwenhoff, Magdalena; Sułowicz, Joanna; Wojas-Pelc, Anna; Musiał, Jacek

    2017-01-01

    Abstract Leukocytoclastic vasculitis (LCV) is a heterogenous group of disorders that may manifest as a mild disease isolated to the skin or be a part of life-threatening systemic vasculitis. According to the 2012 Chapel Hill Consensus Conference nomenclature, patients presenting symptoms of LCV confined only to the skin should be defined as suffering from a single-organ cutaneous small vessel vasculitis (SoCSVV). SoCSVV is a benign disease with a good clinical outcome but with a significant risk of relapse and skin ulcer formation. The aim of the current study was to characterize SoCSVV and to identify factors that may be associated with the risk of recurrence and skin ulcers. Medical records of patients with LCV hospitalized at the Department of Dermatology at University Hospital in Cracow in the years 2010 to 2015 were analyzed. A total of 24 patients fulfilled criteria of SoCSVV. Drugs and preceding infections were identified as precipitating factors in 40% and 20% of cases, respectively. Skin lesions other than palpable purpura (i.e., macules, urticarial vasculitis, or ulcers) were identified in almost half of the patients. Interestingly, the presence of macules independently increased the risk of skin ulcer formation (odds ratio = 16; 95% confidence interval: 1.5–176.6; P = 0.0075) in the multivariate logistic regression analysis. One-quarter of patients with SoCSVV experienced relapse during the 6-month follow-up. The greater number of affected skin areas was an independent risk factor of recurrence (odds ratio = 5; 95% confidence interval: 2–45; P = 0.02). SoCSVV was usually associated with drugs and preceding infections. The disease relapses in approximately one-quarter of the patients. The more severe the skin involvement in the course of SoCSVV, the higher is the risk of recurrence. PMID:28328827

  12. What does the future hold for clinical studies in vasculitis?

    Science.gov (United States)

    Hoffman, G S

    2011-05-01

    The era prior to 1990 was a time of careful observation of disease presentation, course, outcomes and meticulous pathology studies. These mainly single-centre studies introduced new life-saving therapies for drugs still used effectively today. In the 1970-1980s, cyclophosphamide (CyP) added to glucocorticosteroids (GCS) was shown to be life-saving. The trade-off was often severe adverse events. Some forms of vasculitis were found not as ominous as thought initially. Some could be treated with safer drugs [e.g. methotrexate (MTX)]. However, whether mild or severe, patients were not cured. From 1990 to the present large collaborative networks have provided studies were not possible heretofore. Randomized controlled trials captured and manipulated vast amounts of data, banked biological specimens and shared these resources and intellectual capital, moving the field forward at an extraordinary pace. We now know that even for severe forms of granulomatosis and polyangiitis [granulomatosis with polyangiitis (GPA), Wegener's granulomatosus (WG)], microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS), we do not need to use CyP for extended periods. We have learned recently that rituximab is as effective as CyP for severe WG and MPA. We should never again see the permanent toxicities born from years of chronic CyP use. However, short courses of CyP remain useful and can be life-saving. Step-down therapy from CyP is now a standard of care, perhaps to be replaced by rituximab in the future. If one accepts the premise that there are few cures at present for idiopathic large- and small-vessel vasculitis, we will serve our patients well if we can determine the most effective initial therapy that leads to a maintenance strategy for remission with least risk. Ultimately, we wish to identify causes of vasculitis so they can be used as a wedge to secure cures. Unmet needs and strategies are as follows: (1) to increase the numbers of vasculitis-trained physicians; (2) to

  13. Imaging large vessel vasculitis with fully integrated PET/MRI: a pilot study

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    Einspieler, Ingo; Pyka, Thomas; Eiber, Matthias [Technische Universitaet Muenchen, Klinikum rechts der Isar, Department of Nuclear Medicine, Munich (Germany); Thuermel, Klaus; Wolfram, Sabine; Moog, Philipp [Technische Universitaet Muenchen, Klinikum rechts der Isar, Department of Nephrology, Munich (Germany); Reeps, Christian [Technische Universitaet Muenchen, Department of Vascular Surgery, Klinikum rechts der Isar, Munich (Germany); Essler, Markus [Rheinische Friedrich-Wilhelms-Universitaet, Department of Nuclear Medicine, Universitaetsklinikum Bonn, Bonn (Germany)

    2015-04-16

    The aim of this study was to evaluate the feasibility of hybrid [{sup 18}F]fluorodeoxyglucose (FDG) positron emission tomography (PET)/MRI in patients with large vessel vasculitis (LVV) by comparing visual and quantitative parameters to that of PET/CT. Furthermore, the value of PET/MRI in disease activity and extent of LVV was assessed. A total of 16 [{sup 18}F]FDG PET/MRI and 12 [{sup 18}F]-FDG PET/CT examinations were performed in 12 patients with LVV. MRI of the vessel wall by T1-weighted and T2-weighted sequences was used for anatomical localization of FDG uptake and identification of morphological changes associated with LVV. In addition, contrast-enhanced (CE) magnetic resonance angiography (MRA) was performed. The vascular FDG uptake in the vasculitis group was compared to a reference group of 16 patients using a four-point visual score. Visual scores and quantitative parameters [maximum standardized uptake value (SUV{sub max}) and target to background ratio (TBR)] were compared between PET/MRI and PET/CT. Furthermore, correlations between C-reactive protein (CRP) and quantitative PET results, as well the extent of vasculitis in PET, MRI/CE-MRA and combined PET/MRI, were analysed. TBRs, SUV{sub max} values and visual scores correlated well between PET/MRI and PET/CT (r = 0.92, r = 0.91; r = 0.84, p < 0.05). There was no significant difference between both modalities concerning SUV{sub max} measurements and visual scores. In PET/MRI, PET alone revealed abnormal FDG uptake in 86 vascular regions. MRI/CE-MRA indicated 49 vessel segments with morphological changes related to vasculitis, leading to a total number of 95 vasculitis regions in combination with PET. Strong and significant correlations between CRP and disease extent in PET alone (r = 0.75, p = 0.0067) and PET/MRI (r = 0.92, p < 0.0001) in contrast to MRI/CE-MRA only were observed. Regarding disease activity, no significant correlations were seen between quantitative PET results and CRP, although there

  14. Decline in rheumatoid vasculitis prevalence among US veterans: a retrospective cross-sectional study.

    Science.gov (United States)

    Bartels, Christie; Bell, Carolyn; Rosenthal, Ann; Shinki, Kazuhiko; Bridges, Alan

    2009-09-01

    To examine trends in the prevalence of rheumatoid vasculitis in a national US population comprising both hospitalized and ambulatory patients with rheumatoid arthritis (RA). In this serial cross-sectional study, we analyzed data on hospitalized and ambulatory patients spanning 22 years (1985-2006) and 10 years (1997-2006), respectively, to determine the prevalence of rheumatoid vasculitis, as defined by the International Classification of Diseases, Ninth Revision. Our search encompassed data collected on a predominantly male study population during 10 million hospitalizations and outpatient visits, and included annual data on >37,000 RA patients. To test for a decrease in rheumatoid vasculitis prevalence, breakpoint analysis was performed using stepwise Chow and Durbin-Watson tests. There was a clear decline in the prevalence of rheumatoid vasculitis, and this decline remained evident even after accounting for a decreased number of hospitalizations among RA patients. Peak prevalence occurred among hospitalized patients in the 1980s, and prevalence gradually declined throughout the 1990s. Furthermore, simultaneous breakpoints representing a significant drop in rheumatoid vasculitis prevalence between the years 2000 and 2001 were demonstrated for both inpatients (P < 0.000) and outpatients (P < 0.003). The prevalence of vasculitis dropped 53% among inpatients and 31% among outpatients between 2000 and 2001. Our results demonstrate a significant decline in rheumatoid vasculitis prevalence after 2000 in this nationwide sample of hospitalized and ambulatory patients. The clear, consistent drop in prevalence provides an opportunity for the formulation of causal hypotheses, including consideration of the impact of biologic agents used to treat RA, on rheumatoid vasculitis.

  15. Patients with ANCA-associated vasculitis admitted to the intensive care unit with acute vasculitis manifestations: a retrospective and comparative multicentric study.

    Science.gov (United States)

    Demiselle, Julien; Auchabie, Johann; Beloncle, François; Gatault, Philippe; Grangé, Steven; Du Cheyron, Damien; Dellamonica, Jean; Boyer, Sonia; Beauport, Dimitri Titeca; Piquilloud, Lise; Letheulle, Julien; Guitton, Christophe; Chudeau, Nicolas; Geri, Guillaume; Fourrier, François; Robert, René; Guérot, Emmanuel; Boisramé-Helms, Julie; Galichon, Pierre; Dequin, Pierre-François; Lautrette, Alexandre; Bollaert, Pierre-Edouard; Meziani, Ferhat; Guillevin, Loïc; Lerolle, Nicolas; Augusto, Jean-François

    2017-12-01

    Data for ANCA-associated vasculitis (AAV) patients requiring intensive care are scarce. We included 97 consecutive patients with acute AAV manifestations (new onset or relapsing disease), admitted to 18 intensive care units (ICUs) over a 10-year period (2002-2012). A group of 95 consecutive AAV patients with new onset or relapsing disease, admitted to two nephrology departments with acute vasculitis manifestations, constituted the control group. In the ICU group, patients predominantly showed granulomatosis with polyangiitis and proteinase-3 ANCAs. Compared with the non-ICU group, the ICU group showed comparable Birmingham vasculitis activity score and a higher frequency of heart, central nervous system and lungs involvements. Respiratory assistance, renal replacement therapy and vasopressors were required in 68.0, 56.7 and 26.8% of ICU patients, respectively. All but one patient (99%) received glucocorticoids, 85.6% received cyclophosphamide, and 49.5% had plasma exchanges as remission induction regimens. Fifteen (15.5%) patients died during the ICU stay. The following were significantly associated with ICU mortality in the univariate analysis: the need for respiratory assistance, the use of vasopressors, the occurrence of at least one infection event in ICU, cyclophosphamide treatment, sequential organ failure assessment at admission and simplified acute physiology score II. After adjustment on sequential organ failure assessment or infection, cyclophosphamide was no longer a risk factor for mortality. Despite a higher initial mortality rate of ICU patients within the first hospital stay (p < 0.0001), the long-term mortality of hospital survivors did not differ between ICU and non-ICU groups (18.6 and 20.4%, respectively, p = 0.36). Moreover, we observed no renal survival difference between groups after a 1-year follow-up (82.1 and 80.5%, p = 0.94). This study supports the idea that experiencing an ICU challenge does not impact the long-term prognosis of

  16. Urinary levels of high mobility group box-1 are associated with disease activity in antineutrophil cytoplasmic autoantibody-associated vasculitis.

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    Tian-Tian Ma

    Full Text Available High mobility group box-1 (HMGB1, a kind of pro-inflammatory mediator, is associated with inflammatory conditions and tissue damage. Our previous study demonstrated that the circulating levels of HMGB1 correlated with disease activity of antineutrophil cytoplasmic antibody (ANCA-associated vasculitis (AAV. In the current study, we aimed to measure urinary levels of HMGB1 in AAV patients, correlated them to clinical activity index and analysed the immunohistochemical HMGB1 staining in kidney specimens.50 patients with AAV in active stage and 56 patients with AAV in remission were recruited. The urinary levels of HMGB1 were determined by enzyme-linked immunosorbent assay. Moreover, renal biopsy specimens from 27 patients with active AAV were randomly collected to evaluate the deposition of HMGB1.Urinary HMGB1 levels in AAV patients in active stage were significantly higher than those in AAV patients in remission and healthy controls (1.46 [0.56-3.43] versus 0.38 [0.10-1.35] mg/μmolCr, P=0.001; 1.46 [0.56-3.43] versus 0.48 [0.40-0.60] mg/μmolCr, P=0.000, respectively. Further analysis found that urinary levels of HMGB1 correlated with erythrocyte sedimentation rate (r=0.354, p=0.012, C-reactive protein (r=0.289, p=0.042, and Birmingham Vasculitis Activity Score (r=0.350, p=0.013. Renal tissue of active AAV patients showed HMGB1 was mainly expressed in the cytoplasm and the extracellular space. The percentage of HMGB1-negative nuclei in renal tissue of patients with active AAV was significantly higher than that in normal controls (60.6±20.2 % versus 2.7±0.6 %, p<0.01.Urinary levels of HMGB1 may be associated with the disease activity in AAV patients.

  17. Accompanying conditions in patients with chronic spontaneous urticaria and urticarial vasculitis: Results of a retrospective study

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    Sevgi Akarsu

    2015-03-01

    Full Text Available Background and Design: Chronic spontaneous urticaria (CSU, the most common form of chronic urticaria, is characterized by spontaneous wheals and/or angioedema lasting longer than six weeks. Urticarial vasculitis (UV is a small vessel vasculitis; but is also included in the various classification systems of chronic urticaria by some authors. The aim of our study was to evaluate the frequency of accompanying conditions, and to compare the demographic, clinical and laboratory features of patients with CSU and UV. Materials and Methods: In this study, the files of CSU (n=146 and UV (n=43 patients who had been hospitalized between January 2010-December 2013 were retrospectively reviewed in terms of demographic data, disease-specific history, accompanying diseases, medications and laboratory findings. Results: When comparing between the two groups for statistical differences, it was found that the frequencies of personal atopy and angioedema were significantly higher in patients with CSU; however, the patients with UV had significantly higher rates for female gender and the presence of systemic symptoms compared to those with CSU. It was determined that the mean values for erythrocyte sedimentation rate and C-reactive protein, as well as the rates of antinuclear antibody positivity and hypocomplementemia were significantly higher in the UV group, compared with those in the CSU group. Accompanying factors were determined as infections, medications, autoimmune diseases and malignities in order of frequency in both CSU and UV groups, but only the frequency of autoimmune diseases was significantly higher in patients with UV group. Conclusion: Although various authors have reported that a large number (up to 40-60% of patients with UV may present only with wheals (clinically indistinguishable from CSU; we assumed that it would be more appropriate to be considered UV as a priority in patients especially who had systemic symptom, autoimmune disease

  18. Clinicopathologic profile of normocomplementemic and hypocomplementemic urticarial vasculitis: a study from South India.

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    Dincy, C V P; George, R; Jacob, M; Mathai, E; Pulimood, S; Eapen, E P

    2008-07-01

    This study aims to study the clinical and histopathological characteristics of hypocomplementemic and normocomplementemic urticarial vasculitis (HUVS and NUV) among dermatology clinic attendees in a tertiary care hospital in South India. A prospective study was conducted in the dermatology department from February 2003 to May 2004. Seventy-five patients met the inclusion criteria for UV. Sixty-eight patients in whom complement levels were available were classified into either NUV or HUVS groups. Clinical features, laboratory parameters and histological features were compared, and the significance of differences was established using Pearson's Chi-squared test. There was a female preponderance among patients with HUVS. Wheals > 24 h were seen in 90% of patients, and in 54.4% of patients, the wheals were partially blanching or non-blanching. Angioedema was more prevalent in patients with NUV than HUVS (44.4% vs. 21.4%). Systemic involvement was seen in 64.3% of patients with HUVS and 44.4% of patients with NUV. Fever, ANA positivity and systemic lupus erythematosus (SLE) were significantly associated with HUVS. In most cases of UV, a provoking factor could not be identified. Neutrophilic small vessel vasculitis was seen in 42.9% of patients with HUVS and 16.6% patients with NUV. Direct immunofluorescence test showing immunoreactants at the dermo-epidermal junction were present in 60% of patients with HUVS and 33.3% patients with NUV. The clinical features of Indian patients with UV were similar to those reported from the West. Fever, ANA positivity and SLE were significantly associated with HUVS.

  19. The role of metals in autoimmune vasculitis: epidemiological and pathogenic study.

    Science.gov (United States)

    Stratta, P; Messuerotti, A; Canavese, C; Coen, M; Luccoli, L; Bussolati, B; Giorda, L; Malavenda, P; Cacciabue, M; Bugiani, M; Bo, M; Ventura, M; Camussi, G; Fubini, B

    2001-04-10

    A possible relationship between Silica (Si) exposure and antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis has been reported. Furthermore, tuberculosis (TBC) has been frequently described in patients with silicosis, and TBC infection shares with ANCA-associated vasculitis the formation of granulomas. Therefore, an intriguing network including Silica, Vasculitis, TBC and ANCA might be hypothesized. The aim of this work was to further investigate these correlations using both epidemiological and pathogenic approaches. Study I--epidemiological study. A case-control study to compare the occupational histories of 31 cases of biopsy proven vasculitis (18 pauci-immune crescentic glomerulonephritis, 9 microscopic polyangitis, 4 Wegener's granulomatosis) with those of 58 age, sex and residence-matched controls (affected by other kidney diseases), was performed. Occupational Health physicians designed an appropriate questionnaire in order to evaluate a wide spread of exposures and calculate their entity by the product of Intensity x Frequency x Duration. Study II--tuberculosis association. A case-control study to evaluate the frequency of a previous history of tuberculosis (TBC) in 45 patients with vasculitis and 45 controls were performed. Study III--ANCA positivity. A case-control study to evaluate the presence of ANCA was performed by testing blood samples of 64 people with previous professional exposure and 65 sex/age matched patients hospitalized in a General Medicine Unit. Furthermore, the same evaluation was made in a pilot study in 16 patients with ongoing or previous TBC. Study IV--experimental study. The oxygen free radicals (OFR) and IL-12 production (both involved in the pathogenesis of vasculitis) from human phagocytic cells stimulated with an amorphous (diatomaceous earth) and a crystalline (quartz) form of Si at the doses of 10 and 100 microg ml(-1) was evaluated. Study I--a positive history of exposure to Si resulted in significantly more

  20. Refractory vasculitis

    NARCIS (Netherlands)

    Rutgers, Bram; Kallenberg, Cees G. M.

    Refractory vasculitis occurs in 4-5% of patients with anti-neutrophil cytoplasmic antibody associated vasculitis (AAV). Differences between therapies used for refractory disease are mostly reflected in the percentages of complete and partial remissions, but also in the number of serious side

  1. HMGB1 in ANCA-associated vasculitis: A longitudinal study

    NARCIS (Netherlands)

    Silva De Souza, A.W.; Westra, J.; Bijzet, J.; Limburg, P.C.; Bijl, M.; Stegeman, C.A.; Kallenberg, C.G.M.

    2013-01-01

    Introduction.- Extra-cellular high mobility group box 1 (HMGB1) acts as an alarmin and has been shown to be a biomarker of disease activity in systemic lupus erythematosus (SLE). This study aims to assess anti- HMGB1 antibodies and HMGB1 levels as biomarkers for disease activity and predictors of

  2. A STUDY OF AETIOLOGICAL FACTORS IN THE OCCURRENCE OF CUTANEOUS VASCULITIS IN A TERTIARY CARE CENTRE IN NORTH KERALA

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    Praveen Malayath

    2017-03-01

    Full Text Available BACKGROUND Cutaneous vasculitis is a condition caused by various aetiologies. They can be primary or secondary. Cutaneous lesions maybe a pointer to systemic diseases. So, it is important to identify the various aetiological factors in the occurrence of the various types of cutaneous vasculitis. The patterns and the various aetiologies of cutaneous vasculitis in Kerala is not well documented in the existing literature. MATERIALS AND METHODS The present study is a cross-sectional descriptive study of patients with a clinical diagnosis of cutaneous small vessel vasculitis admitted in Medicine and Dermatology Wards of Calicut, Government Medical College during January 2013 to December 2013. A detailed history and clinical examination of patients along with histopathological examination of skin biopsy was done. RESULTS Out of the 70 cases of cutaneous vasculitis studied, idiopathic cutaneous small vessel vasculitis was the most common type followed by Henoch-Schonlein purpura. The most common aetiology identified was drugs followed by infections. No aetiological factor was identified in 42.8% of the cases. CONCLUSION An aetiological association could be found in 57.8% of cases. The causes identified include drugs, infections, malignancy, connective tissue disorder associated, chronic systemic diseases and Behcet’s disease in decreasing order of frequency.

  3. Vasculitis Pregnancy Registry

    Science.gov (United States)

    2017-04-27

    Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (GPA); Wegener's Granulomatosis; IgA Vasculitis; Henoch-Schoenlein Purpura (HSP); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Takayasu Arteritis (TAK); Urticarial Vasculitis; Systemic Vasculitis

  4. [Vasculitis and viral infection].

    Science.gov (United States)

    Martínez Aguilar, N E; Guido Bayardo, R; Vargas Camaño, M E; Compañ González, D; Miranda Feria, A J

    1997-01-01

    Viruses have been implicated in vasculitis. To determine activity of viral infection associated with vasculitis. 17 patients with vasculitis had been in immunological and antiviral antibodies evaluation. Twenty five healthy controls sex and age matched with hematic biometry (BH) and AA. All subjects were negative to HIV and HBV. Viral activity was demonstrated in eight patients; vascular purpura (5), Takayasu disease (1), polyarteritis nodosa (1), erythema nodosum (1). None subject of control group had IgM activity. Antibodies response of IgG in patients were of lesser intensity than in control group. 14 abnormalities in BH were found in patients and 4 in control group. Immune response in patients, measured by lymphocyte subpopulations and circulating immune complexes was abnormal. In conclusion 47% showed viral activity, but the dominant feature was abnormal immune response in 82%.

  5. Prospective study of radioimmunoassay for antibodies against neutrophil cytoplasm in diagnosis of systemic vasculitis

    Energy Technology Data Exchange (ETDEWEB)

    Savage, C.O.S.; Winearls, C.G.; Jones, S.; Marshall, P.D.; Lockwood, C.M.

    1987-06-20

    The diagnosis and management of Wegener's granulomatosis and microscopic polyarteritis are complicated by the lack of specific diagnostic tests. The diagnostic performance of a solid-phase radioimmunoassay, which detects the autoantibodies against neutrophil cytoplasm present in these disorders, was assessed in a prospective study of patients with suspected vasculitis and/or rapidly progressive nephritis. The assay had a sensitivity and specificity of 96% when carried out in combination with a specific inhibition stage and indirect immunofluorescence staining of alcohol-fixed normal neutrophils.

  6. Granulomatous vasculitis.

    Science.gov (United States)

    Marzano, A V; Balice, Y; Tavecchio, S; Desimine, C; Colombo, A; Berti, E

    2015-04-01

    Granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Wegener's granulomatosis and Churg-Strauss Syndrome respectively, are systemic granulomatous vasculitides affecting small- and medium-sized blood vessels. Both GPA and EGPA are included within the group of antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitides, on the basis of the detection of such autoantibodies in a significant proportion of affected patients. Two main settings of GPA, possibly overlapping each other, are recognized: a localized form, which is limited to the upper airways but is highly relapsing and refractory, and a diffuse form, which is initially more severe but then less commonly recurrent. In EGPA, a prodromic phase characterized by asthma and rhino-sinusitis is followed by an eosinophilic phase, marked by peripheral eosinophilia, and then by a vasculitic phase, in which skin lesions are a prominent feature together with peripheral neuropathy and renal involvement. Polymorphic cutaneous manifestations can occur during the course of both GPA and EGPA, and include palpable purpura, livedo reticularis, papules, nodules, vesiculo-bullae and necrotic-ulcerative lesions most commonly involving the lower extremities; pyoderma gangrenosum-like ulcers and lesions resembling erythema multiforme have been described in GPA and EGPA, respectively. Oral involvement is not uncommon in GPA and may manifest as nonspecific erosive lesions or as a hyperplastic gingivitis named strawberry gingivitis. Considering that skin involvement is common in ANCA-associated vasculitides and may also be their presenting sign, the role of dermatologist is crucial in the early diagnosis of these forms as well as of vasculitis in general.

  7. Thrombosis in vasculitis.

    Science.gov (United States)

    Springer, Jason; Villa-Forte, Alexandra

    2013-01-01

    To review the association of thrombosis and vasculitis and discuss some of the proposed causal mechanisms. It is becoming increasingly evident that various systemic inflammatory diseases such as vasculitis are associated with an increased risk of both venous and arterial thrombosis. Increasing evidence supports the use of immunosuppression in the management of venous thrombosis in Behçet's disease. An increased incidence of thromboembolic disease in antineutrophil cytoplasmic antibody-associated vasculitis has been recognized, especially during periods of active disease. In addition, a higher risk of ischemic heart disease in these patients has also been observed. As in giant cell arteritis, recent evidence supports the role of aspirin in the prevention of ischemic events in Takayasu's disease. Thromboembolic disease is an important complication of several forms of systemic vasculitis, and it may result in significant morbidity and mortality. Many questions such as the role for screening of asymptomatic patients, prevention of thrombosis, and duration of anticoagulation in patients with vasculitis remain unanswered. Future studies exploring the mechanisms of thrombosis and its link to inflammation may provide insights in predicting patients at a higher risk for thrombosis and improve outcomes.

  8. Pediatric Vasculitis Initiative

    Science.gov (United States)

    2017-05-01

    Wegeners Granulomatosis (Granulomatosis With Polyangiitis); Microscopic Polyangiitis; Churg Strauss Syndrome (Eosinophilic Granulomatosis With Polyangiitis); Polyarteritis Nodosa; Takayasu Arteritis; Primary CNS Vasculitis; Unclassified Vasculitis

  9. Impact of Vasculitis on Employment and Income

    Science.gov (United States)

    2016-01-26

    Vasculitis; Systemic Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis; Temporal Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa (PAN); Takayasu's Arteritis; Urticarial Vasculitis

  10. Urticarial vasculitis in an infant.

    Science.gov (United States)

    Koch, Phoebe E; Lazova, Rossitza; Rosen, James R; Antaya, Richard J

    2008-01-01

    Urticarial vasculitis (UV), a subtype of leukocytoclastic vasculitis, is a small vessel necrotizing vasculitis characterized by urticarial lesions and decreased serum complement. Primarily a disease of adult women, this immune complex disorder is seldom reported in children. Pathologic examination of skin lesions in patients with UV reveals leukocytoclastic vasculitis. UV is a frequent finding, particularly involving early components of the classical complement cascade C1q and C2 to C4. We report a 9-month-old male infant who presented with chronic urticaria of 7 months' duration that was unresponsive to conventional therapies for urticaria. His lesions appeared as erythematous wheals followed by the development of purpuric papules and resolving with ecchymoses and postinflammatory hyperpigmentation. Laboratory studies and skin biopsy results were consistent with UV.

  11. Epidemiology of ANCA associated vasculitis

    Directory of Open Access Journals (Sweden)

    Wenche Koldingsnes

    2009-10-01

    Full Text Available ANCA associated vasculitis (AAV comprises three syndromes with systemic vasculitis (Wegener’s granulomatosis (WG, Churg Strauss syndrome (CSS and icroscopic polyangiitis (MPA, which all involve small and medium sized vessels and are associated with antibodies against cytoplasmatic antibodies in neutrophils (ANCA. Polyarteritis nodosa (PAN is included in this review as it also affects medium sized vessels, and has many clinical findings in common with the AAV.Since the recognition of ANCA, increasing data have become available on the epidemiology of these vasculitidis. WG constitutes half of the AAV and its prevalence has increased from 30/million in the late 1980’s in the USA to 160/million in this century in northern Europe. The prevalence for the whole group of primary systemic vasculitides is now 300/million in Sweden. The annual incidence of WG increased from 6.0/million to 14/million during the 1990’s in Tromsø, but it is unknown if this is a true increase or the result of an increased awareness of the diagnosis. For the whole group of AAV, the annual incidence in most more recent studies is relatively constant over time and by geographical location, ranging from 13 to 21/million. Nonetheless there are interesting differences in the prevalence of specific vasculitis between different geographical areas, as well as for sub specificities of ANCA.There seems to be a South-North gradient for WG and PR3-ANCA with high figures reported from northern Europe and southern New Zealand. In European studies WG is 90% PR3-ANCA positive. MPA which is predominantly MPO-ANCA associated are more frequent in the Mediterranean countries and also has an increasing gradient towards east-Asia, as almost all AAV in China and Japan are diagnosed as MPA, predominantly MPO-ANCA positive.There are also some ethnic and gender differences. WG is most prevalent among Caucasians in the USA and in people with European ancestors in Paris and in New Zealand, less

  12. A reassessment of diagnostic criteria and treatment of idiopathic urticarial vasculitis: a retrospective study of 47 patients.

    Science.gov (United States)

    Tosoni, C; Lodi-Rizzini, F; Cinquini, M; Pasolini, G; Venturini, M; Sinico, R A; Calzavara-Pinton, P

    2009-03-01

    Urticarial vasculitis (UV) is an uncommon type of chronic urticaria (CU), which exhibits leucocytoclastic vasculitis. Painful and long-lasting (> 24 h) weals associated with purpura or bruising are considered indicative of UV. It is often responsive to oral corticosteroids and poorly to oral antihistamines. Hypocomplementaemia and systemic involvement are also commonly reported. To diagnose patients with UV histologically and then compare their clinical features and response to various treatment regimens. Biopsies were taken from 312 subjects with CU unresponsive to oral antihistamines; of these, 47 were histologically diagnosed as having UV. Biopsies were taken irrespective of the clinical features of weal eruption. Other diseases known to be associated with small-vessel vasculitis had previously been excluded. Results. Individual weals lasted < 24 h in 57.4% of patients, and pain or tenderness was reported only by 8.6%. Extracutaneous features were present in 81%, hypocomplementaemia in 11% and abnormalities of other laboratory parameters (i.e. raised erythrocyte sedimentation rate, microscopic haematuria) in 76.6%. Hydroxyzine was effective in only one patient. Both oral corticosteroids and cinnarizine were effective in a high percentage of the patients. This diagnostic approach allowed us to identify a large group (47 patients) with UV. Most did not present the clinical (prolonged duration of weals and bruising) and laboratory features that have previously been described as characteristic of UV. Cinnarizine was found to be a valuable treatment option.

  13. Clinical Transcriptomics in Systemic Vasculitis (CUTIS)

    Science.gov (United States)

    2018-01-18

    Cryoglobulinemic Vasculitis (CV); Drug-induced Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); IgA Vasculitis; Isolated Cutaneous Vasculitis; Granulomatosis With Polyangiitis (GPA); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Urticarial Vasculitis; Vasculitis

  14. Urticarial vasculitis in infancy

    Directory of Open Access Journals (Sweden)

    Kaur S

    2003-05-01

    Full Text Available Urticarial vasculitis is an uncommon manifestation of cutaneous vasculitis closely resembling chronic urticaria. It is an immune complex deposition disorder, which is not commonly observed in children. We report an 9-month-old infant with urticarial vasculitis and discuss its clinical course and differentiation from common urticaria.

  15. Clinical significance of immunoglobulin deposition in leukocytoclastic vasculitis: a 5-year retrospective study of 88 patients at cleveland clinic.

    Science.gov (United States)

    Alalwani, Mohamed; Billings, Steven D; Gota, Carmen E

    2014-09-01

    To study the diagnostic utility and clinical associations of immunoglobulin deposition, determined by direct immunofluorescence (DIF) in cutaneous leukocytoclastic vasculitis (LCV). We performed a retrospective study of all biopsy-proven LCV cases seen at Cleveland Clinic between 2007 and 2012. All LCV cases in which DIF was performed were included. Of the 218 LCV cases, 106 cases had DIF performed and data from 88 cases were available: median (SD) age 53.3 (19.4), 52% male, 64.1% white, duration of rash 5.5 (20.8) months; follow-up 14 (19.7) months. DIF results showed any immunoglobulin and/or complement and/or fibrinogen in 70.5%, immunoglobulin A (IgA) in 36.4%, immunoglobulin M (IgM) in 21.6%, immunoglobulin G (IgG) in 11.4%. Patients with IgA deposition by DIF, compared with those without IgA, were younger, 44 (19) versus 56 (17) (P = 0.006), more likely to be white (P = 0.025) and had more organs affected by vasculitis (P = 0.002), higher incidence of gastrointestinal tract involvement (P = 0.0001) and renal disease (P = 0.006). No differences between rates of infection or malignancy were seen between DIF IgA, IgM, or IgG-positive versus negative patients. In patients with cutaneous LCV, IgA is the most common immunoglobulin found by DIF. IgA deposition, but not IgM or IgG, is predictive of associated renal and gastrointestinal organ involvement by vasculitis. No association between the type of immunoglobulin and preexisting infection or malignancy was found. DIF results add information that is clinically relevant to the diagnosis and management of LCV.

  16. Telmisartan induced urticarial vasculitis.

    Science.gov (United States)

    Mahajan, Vikram K; Singh, Ravinder; Gupta, Mrinal; Raina, Rashmi

    2015-01-01

    A 53-year-old man developed urticarial vasculitis following ingestion of telmisartan and hydrochlorothiazide combination for hypertension. Treatment with prednisolone and cetirizine was curative, but his lesions recurred when he continued telmisartan and hydrochlorothiazide against medical advice. Re-challenge with the same doses of telmisartan precipitated similar lesions with telmisartan and not with hydrochlorothiazide. This uncommon cutaneous adverse reaction of angiotensin II receptor blockers has implication for the clinicians as more such cases may become apparent with their wider use than in premarketing studies.

  17. Current status of outcome measure development in vasculitis.

    Science.gov (United States)

    Merkel, Peter A; Aydin, Sibel Z; Boers, Maarten; Cornell, Christina; Direskeneli, Haner; Gebhart, Don; Hatemi, Gulen; Luqmani, Raashid; Matteson, Eric L; Milman, Nataliya; Robson, Joanna; Seo, Philip; Tomasson, Gunnar

    2014-03-01

    The conduct of randomized controlled trials for vasculitis, especially for the antineutrophil cytoplasmic antibody-associated vasculitides [AAV, granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis], has been greatly advanced by the development, use, and acceptance of validated outcome measures. Trials have subsequently provided the opportunity to validate and refine reliable, valid outcome measures for these multisystemic and relapsing rare diseases. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group was formed in 2004 to foster development of validated and widely accepted outcomes in vasculitis using data-driven analyses, a dedication to building consensus, and adherence to, and guidance by, the principles of the OMERACT approach. This work led to the endorsement by OMERACT of the core set of domains and associated outcome measures for AAV. Next steps for the study of existing outcome tools in AAV include better definition of response criteria through development of more data-driven weighting of the elements of activity and damage assessment. The Working Group is now also embarking on a series of linked projects to develop validated patient-reported outcomes for use in clinical research in vasculitis. Additionally, the Working Group is studying how current methods of disease assessment and plans for new outcomes can be informed by the conceptual framework of the International Classification of Function of the World Health Organization. The success of the Group's work in AAV has also led to a formal process for developing outcomes for the large vessel vasculitides (Takayasu arteritis and giant cell arteritis) and Behçet disease.

  18. Vasculitis in Systemic Sclerosis

    Directory of Open Access Journals (Sweden)

    Lily Kao

    2010-01-01

    Full Text Available Systemic sclerosis (SSc is a multiorgan connective tissue disease characterized by autoantibody production and fibroproliferative stenosis of the microvasculature. The vascoluopathy associated with SSc is considered to be noninflammatory, yet frank vasculitis can complicate SSc, posing diagnostic and therapeutic challenges. Here, we have reviewed the literature for reports of small-, medium-, and large-vessel vasculitis occurring in SSc. Amongst 88 reported cases of vasculitis in SSc, patients with ANCA-associated vasculitis appear to present a unique subclass in that they combined typical features of SSc with the renal manifestation of ANCA-associated glomerulonephritis. Other vasculitic syndromes, including large-vessel vasculitis, Behcet's disease, cryoglobulinemia, and polyarteritis nodosa, are rarely encountered in SSc patients. ANCA-associated vasculitis needs to be considered as a differential diagnosis in SSc patients presenting with renal insufficiency, as renal manifestations may result from distinct disease processes and require appropriate diagnostic testing and treatment.

  19. Omalizumab for Urticarial Vasculitis

    DEFF Research Database (Denmark)

    Ghazanfar, Misbah Nasheela; Thomsen, Simon Francis

    2015-01-01

    include oral antihistamines, oral corticosteroids, dapsone, colchicine or hydroxychloroquine. We describe a male patient with urticarial vasculitis who was treated with omalizumab (anti-IgE) with convincing results and provide a review of previous reports of patients with urticarial vasculitis treated...

  20. Demographic characteristics, aetiology, and assessment of treatment options in leukocytoclastic vasculitis

    Directory of Open Access Journals (Sweden)

    Alkim Unal Cakiter

    2017-04-01

    Full Text Available Introduction : Vasculitides are a heterogeneous group of diseases characterized by inflammation of the blood vessel walls. Etiological factors include infections, drugs, connective tissue diseases, and malignancies. Aim : To examine the demographic characteristics, etiological factors, and treatment options in 75 patients with leukocytoclastic vasculitis. Material and methods : The study included 75 patients diagnosed with leukocytoclastic vasculitis at our clinic. The patients’ medical records were reviewed to determine their age, sex, presence of systemic symptoms, possible etiological factors, laboratory results, types of cutaneous lesions, locations of the lesions, treatment options, and disease course. Results : There were 43 women and 32 men. Cutaneous lesions affected only the lower limbs in 60 of the 75 patients (80% and usually presented as palpable purpura (64%, n = 48. Arthralgia (26.7%, n = 20 was the most frequent extracutaneous symptom. Of the patients with secondary vasculitis, the most common causes were infections and drugs. The mean age of the patients with Henoch-Schönlein purpura was 26.8 years. There was no significant association between age and renal, gastrointestinal, or joint involvement. Conclusions : The most common form of vasculitis in our study was cutaneous leukocytoclastic vasculitis. In most of the patients it appeared to be idiopathic. Among drugs, antibiotics were the most common etiological factor. In 4 patients, the cutaneous leukocytoclastic vasculitis behaved like the paraneoplastic syndrome.

  1. Journey of Patients With Vasculitis From First Symptom to Diagnosis

    Science.gov (United States)

    2018-01-18

    Vasculitis; Systemic Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatous Vasculitis; Temporal Arteritis; Giant Cell Arteritis; Granulomatosis With Polyangiitis; Wegener Granulomatosis; Henoch Schonlein Purpura; IgA Vasculitis; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu Arteritis; Urticarial Vasculitis

  2. Recurrent optic disc and retinal vasculitis in a patient with drug-induced urticarial vasculitis.

    Science.gov (United States)

    Batioğlu, Figen; Taner, Pelin; Aydintuğ, Olcay Tiryaki; Heper, Aylin Okçu; Ozmert, Emin

    2006-01-01

    The purpose of this study was to report recurrent optic disc and retinal vasculitis in a patient with drug-induced urticarial vasculitis. Complete ophthalmological examination including fluorescein angiography and visual field examination were done. A 53-year-old woman with recurrent painful urticarial skin lesions following trimethoprim sulfamethoxazole usage had the clinical and histopathological diagnosis of urticarial vasculitis. Two years after cutaneous manifestations, she began to notice visual disturbances in both eyes that recurred at 1-year intervals. Her ophthalmological findings were consistent with recurrent vasculitis of the optic nerve and retina. Treatment with high-dose corticosteroids and hydroxychloroquine resulted in the resolution of cutaneous and ocular manifestations. This patient demonstrates that recurrent occlusive vasculitis of the optic nerve and retina can occur in this rare disease. These patients should be examined periodically by ophthalmologists.

  3. Treatment with intravenous gammaglobulin in pediatric patients with primary vasculitis

    Directory of Open Access Journals (Sweden)

    Camacho Meza Ignacio

    2014-07-01

    Full Text Available Nowadays intravenous immunoglobulins have been used in differ- ent vasculitis with various results for each condition, being more recommended for its use in Kawasaki disease and ANCA-associated vasculitis. However, there is still no solid evidence to support its use in the entire group of these diseases. We present a review of the existing literature related to the use of intravenous immunoglobulin for the treatment of primary vasculitis in childhood.

  4. Refractory rheumatoid vasculitis.

    Science.gov (United States)

    Kumar, Ashok; Goel, Anshul; Lapsiwala, Mehul; Singhal, Suman

    2016-11-01

    Systemic rheumatoid vasculitis (SRV) can develop in rheumatoid arthritis of long duration and high disease activity. It most commonly manifests as cutaneous vasculitis and mononeuritis multiplex. This can involve any organ of the body and carries very high mortality. We report a case of a young male who had rheumatoid arthritis for the past 15 years and became refractory to standard drugs and anti-TNF agents. He subsequently developed SRV, which started as mononeuritis multiplex. Disease progressed to result in gangrene of hands and feet despite receiving intravenous cyclophosphamide. Intravenous immunoglobulin and rituximab also could not provide any response. Prolonged ICU stay resulted in critical care neuromyopathy. Central nervous system vasculitis developed even after repeated infusions of intravenous immunoglobulins and at last he died of complications. In this case report, we have presented rare and chronic protracted presentation of rheumatoid vasculitis involving skin, nerves, brain and testis, which was refractory to the recommended therapies.

  5. Urticaria and urticarial vasculitis.

    Science.gov (United States)

    Guha, Bhuvana; Youngberg, George; Krishnaswamy, Guha

    2003-01-01

    Urticaria and urticarial vasculitis may be triggered by allergens, infection, autoimmunity and other immunological conditions. Careful evaluation, skin biopsy and specific laboratory tests can assist in diagnosis. The appropriate use of antihistamines, glucocorticoids, and other immunomodulators are discussed.

  6. How Is Vasculitis Diagnosed?

    Science.gov (United States)

    ... more likely to occur in people of Scandinavian origin, but it can affect people of any race. ... of polyarteritis nodosa seem to be linked to hepatitis B or C infections. Mostly Small Vessel Vasculitis ...

  7. Living with Vasculitis

    Science.gov (United States)

    ... more likely to occur in people of Scandinavian origin, but it can affect people of any race. ... of polyarteritis nodosa seem to be linked to hepatitis B or C infections. Mostly Small Vessel Vasculitis ...

  8. What is Vasculitis?

    Science.gov (United States)

    ... more likely to occur in people of Scandinavian origin, but it can affect people of any race. ... of polyarteritis nodosa seem to be linked to hepatitis B or C infections. Mostly Small Vessel Vasculitis ...

  9. How Is Vasculitis Treated?

    Science.gov (United States)

    ... more likely to occur in people of Scandinavian origin, but it can affect people of any race. ... of polyarteritis nodosa seem to be linked to hepatitis B or C infections. Mostly Small Vessel Vasculitis ...

  10. Types of Vasculitis

    Science.gov (United States)

    ... more likely to occur in people of Scandinavian origin, but it can affect people of any race. ... of polyarteritis nodosa seem to be linked to hepatitis B or C infections. Mostly Small Vessel Vasculitis ...

  11. Review article : The role of CD4(+) T cells in ANCA-associated systemic vasculitis

    NARCIS (Netherlands)

    Abdulahad, Wayel H.; Stegeman, Coen A.; Kallenberg, Cees G. M.

    Antineutrophil cytoplasmic autoantibody (ANCA)-associated systemic vasculitis (AASV) constitutes a group of primary vasculitides associated with antineutrophil cytoplasmic autoantibodies, which are either directed to proteinase-3 or myeloperoxidase. In contrast to other forms of vasculitis,

  12. Long- term outcome of paediatric patients with ANCA vasculitis

    Directory of Open Access Journals (Sweden)

    Pusey Charles D

    2011-06-01

    Full Text Available Abstract Background Primary systemic vasculitis presenting in childhood is an uncommon but serious condition. As these patients transfer to adult clinics for continuing care, defining long term outcomes with emphasis on disease and treatment- related morbidity and mortality is important. The aim of this study is to describe the long- term clinical course of paediatric patients with ANCA vasculitis. Methods The adult patients in our vasculitis clinics who had presented in childhood, with a follow up time of greater than 10 years were included. We also reviewed the literature for articles describing the clinical outcome of paediatric patients with ANCA vasculitis. Results We describe the clinical course of 8 adults who presented in childhood with ANCA vasculitis. 7 patients had Wegener's granulomatosis and 1 had microscopic polyangiitis. The median age at presentation was 11.5 years, and follow up time ranged form 11 to 30 years. Induction therapy for all patients was steroids and/or cyclophosphamide. Maintenance therapy was with azathioprine or mycophenolate mofetil. Biological agents were used in 3 patients for relapsed disease in adulthood only. Seven patients achieved complete remission. All patients experienced disease relapse, with a median of 4 episodes. Kidney function was generally well preserved, with median eGFR 76 ml/min. Only one patient developed end-stage renal failure and one patient died after 25 years of disease. Treatment-related morbidity rates were high; 7 suffered from infections, 4 were infertile, 2 had skeletal complications, and 1 developed malignancy. Conclusion Close long- term follow up of paediatric patients with ANCA vasculitis is imperative, as this patient cohort is likely to live long enough to develop significant treatment and disease- related morbidities. Prospective cohort studies with novel therapies including paediatric patients are crucial to help us determine the best approach to managing this complex group

  13. History of primary vasculitis in Latin America.

    Science.gov (United States)

    Iglesias Gammara, Antonio; Coral, Paola; Quintana, Gerardo; Toro, Carlos E; Flores, Luis Felipe; Matteson, Eric L; Restrepo, José Félix

    2010-03-01

    A literature review utilizing Fepafem, Bireme, LiLacs, Scielo Colombia, Scielo Internacional, former MedLine, Pubmed, and BVS Colombia as well as manual searches in the libraries of major Latin American universities was performed to study vasculitis in Latin America. Since 1945, a total of 752 articles have been published by Latin American authors. However, only a minority are devoted to primary vasculitides, and even fewer have been published in indexed journals. Approximately 126 are in OLD, Medline, Pubmed, Bireme, and Scielo. Most publications are from Mexico, followed by Brazil and Colombia. Systematic studies of the epidemiology of primary idiopathic vasculitis are available for a few countries, i.e. Brazil, Mexico, Colombia, Chile, and Peru. Takayasu arteritis and ANCA-associated vasculitis are the best studied forms of vasculitis in Latin America. Interest and expertise in vasculitis is growing in Latin America, as reflected in the increased number of published articles from this region of the world in the last decade. Racial and environmental factors are possibly responsible for the differential expression of various types of primary vasculitis observed in Latin America. With time, the unique features, epidemiology, and better treatment strategies for idiopathic vasculitides in Latin America will emerge.

  14. Cocaine-Induced Vasculitis

    Directory of Open Access Journals (Sweden)

    Mark Berman

    2016-10-01

    Full Text Available The use of cocaine continues to grow worldwide. One of the possible side-effects of cocaine is vasculitis. Two distinct vasculitic syndromes have been described due to cocaine. One is cocaine-induced midline destructive lesion, secondary to a direct vasoconstrictor effect of cocaine, inducing ischemic necrosis of the septal cartilage and perforation of the nasal septum, mimicking findings of granulomatosis with polyangiitis in the upper airways. The other is ANCA-associated vasculitis, attributed to the levamisole component that contaminates about 70% of the cocaine. This type of vasculitis may be myeloperoxidase (MPO and proteinase 3 (PR3 positive, and its main manifestations are typical cutaneous findings, arthralgia, otolaryngologic involvement, and agranulocytosis. A high degree of suspicion and awareness is needed in order properly to diagnose and treat these patients.

  15. The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients.

    Science.gov (United States)

    Jachiet, Marie; Flageul, Béatrice; Deroux, Alban; Le Quellec, Alain; Maurier, François; Cordoliani, Florence; Godmer, Pascal; Abasq, Claire; Astudillo, Leonardo; Belenotti, Pauline; Bessis, Didier; Bigot, Adrien; Doutre, Marie-Sylvie; Ebbo, Mikaël; Guichard, Isabelle; Hachulla, Eric; Héron, Emmanuel; Jeudy, Géraldine; Jourde-Chiche, Noémie; Jullien, Denis; Lavigne, Christian; Machet, Laurent; Macher, Marie-Alice; Martel, Clotilde; Melboucy-Belkhir, Sara; Morice, Cécile; Petit, Antoine; Simorre, Bernard; Zenone, Thierry; Bouillet, Laurence; Bagot, Martine; Frémeaux-Bacchi, Véronique; Guillevin, Loïc; Mouthon, Luc; Dupin, Nicolas; Aractingi, Selim; Terrier, Benjamin

    2015-02-01

    Hypocomplementemic urticarial vasculitis (HUV) is an uncommon vasculitis of unknown etiology that is rarely described in the literature. We undertook this study to analyze the clinical spectrum and the therapeutic management of patients with HUV. We conducted a French nationwide retrospective study that included 57 patients with chronic urticaria, histologic leukocytoclastic vasculitis, and hypocomplementemia. We assessed clinical and laboratory data and evaluated the patients' cutaneous and immunologic responses to therapy. We evaluated treatment efficacy by measuring the time to treatment failure. Urticarial lesions were typically more pruritic than painful and were associated with angioedema in 51% of patients, purpura in 35%, and livedo reticularis in 14%. Extracutaneous manifestations included constitutional symptoms (in 56% of patients) as well as musculoskeletal involvement (in 82%), ocular involvement (in 56%), pulmonary involvement (in 19%), gastrointestinal involvement (in 18%), and kidney involvement (in 14%). Patients with HUV typically presented with low C1q levels and normal C1 inhibitor levels, in association with anti-C1q antibodies in 55% of patients. Hydroxychloroquine or colchicine seemed to be as effective as corticosteroids as first-line therapy. In patients with relapsing and/or refractory disease, rates of cutaneous and immunologic response to therapy seemed to be higher with conventional immunosuppressive agents, in particular, azathioprine, mycophenolate mofetil, or cyclophosphamide, while a rituximab-based regimen tended to have higher efficacy. Finally, a cutaneous response to therapy was strongly associated with an immunologic response to therapy. HUV represents an uncommon systemic and relapsing vasculitis with various manifestations, mainly, musculoskeletal and ocular involvement associated with anti-C1q antibodies, which were found in approximately half of the patients. The best strategy for treating HUV has yet to be defined

  16. Urticarial vasculitis secondary to paroxetine.

    Science.gov (United States)

    Welsh, John Patrick; Cusack, Carrie Ann; Ko, Christine

    2006-01-01

    Paroxetine (Paxil) is a widely prescribed antidepressant of the selective serotonin reuptake inhibitor (SSRI) class for which multiple cutaneous adverse effects have been reported, including urticaria, ecchymoses, vasculitis, and pruritus. In contrast, fluoxetine (Prozac) is the only SSRI previously reported to cause urticarial vasculitis. Urticarial vasculitis is a chronic disorder marked by recurrent episodes of erythematous, indurated wheals that histologically manifest the features of leukocytoclastic vasculitis. This case represents the first reported incidence of paroxetine-induced urticarial vasculitis, and highlights the need to consider the entire SSRI class as s as a potential cause of the condition.

  17. Systemic vasculitis in the geriatric population

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    Ari Stiel Radu Halpern

    2008-03-01

    Full Text Available Systemic vasculitis is a group of diseases characterized byinfl ammation of blood vessels with fi brinoid necrosis and resultingischemic changes. In the geriatric population it has been frequentlydescribed with patient age playing an important role in both thediagnosis and treatment of these diseases. Vasculitis may mimicmany common clinical situations of the elderly. They can affectmultiple organs at the same time with devastating consequences andchallenging diagnosis. The most common form of systemic vasculitisin older adults is giant cell arteritis or temporal arteritis that occursexclusively in people older than 50 years. Wegener granulomatosisand microscopic polyangiitis are also frequent in the geriatricpopulation. Treatment of vasculitis has developed to achieve a lowmortality and morbidity rate. Nevertheless, traditional therapeuticregimes are still related with severe side effects. New therapeuticroutines are being developed to induce a rapid remission allowingchange to a less iatrogenic treatment for remission maintenance.

  18. 3D-black-blood 3T-MRI for the diagnosis of thoracic large vessel vasculitis: A feasibility study

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    Treitl, Karla Maria; Saam, Tobias [Institute for Clinical Radiology, LMU Munich, Munich (Germany); German Center for Cardiovascular Disease Research (DZHK e.V.), Munich (Germany); Maurus, Stefan; Sommer, Nora Narvina; Coppenrath, Eva; Treitl, Marcus [Institute for Clinical Radiology, LMU Munich, Munich (Germany); Kooijman-Kurfuerst, Hendrik [Philips Healthcare, Hamburg (Germany); Czihal, Michael; Hoffmann, Ulrich [LMU Munich, Division of Vascular Medicine, Medical Clinic and Policlinic IV, Munich (Germany); Dechant, Claudia; Schulze-Koops, Hendrik [LMU Munich, Division of Rheumatology and Clinical Immunology, Medical Clinic and Policlinic IV, Munich (Germany)

    2017-05-15

    To evaluate the feasibility of T1w-3D black-blood turbo spin echo (TSE) sequence with variable flip angles for the diagnosis of thoracic large vessel vasculitis (LVV). Thirty-five patients with LVV, diagnosed according to the current standard of reference, and 35 controls were imaged at 3.0T using 1.2 x 1.3 x 2.0 mm{sup 3} fat-suppressed, T1w-3D, modified Volumetric Isotropic TSE Acquisition (mVISTA) pre- and post-contrast. Applying a navigator and peripheral pulse unit triggering (PPU), the total scan time was 10-12 min. Thoracic aorta and subclavian and pulmonary arteries were evaluated for image quality (IQ), flow artefact intensity, diagnostic confidence, concentric wall thickening and contrast enhancement (CWT, CCE) using a 4-point scale. IQ was good in all examinations (3.25 ± 0.72) and good to excellent in 342 of 408 evaluated segments (83.8 %), while 84.1 % showed no or minor flow artefacts. The interobserver reproducibility for the identification of CCE and CWT was 0.969 and 0.971 (p < 0.001) with an average diagnostic confidence of 3.47 ± 0.64. CCE and CWT were strongly correlated (Cohen's k = 0.87; P < 0.001) and significantly more frequent in the LVV-group (52.8 % vs. 1.0 %; 59.8 % vs. 2.4 %; P < 0.001). Navigated fat-suppressed T1w-3D black-blood MRI with PPU-triggering allows diagnosis of thoracic LVV. (orig.)

  19. The “other” vasculitis syndromes and kidney involvement

    Science.gov (United States)

    2009-01-01

    There are a number of vasculitides that are not confined to a specific vessel size, do not have characteristic features, and/or are not secondary to another disease. Most of these vasculitides are rare in childhood. Behçet disease is representative of this group as it involves vessels of any size on both the arterial and venous side. In addition to renal vascular involvement, Behçet disease may involve the kidney through glomerulonephritis, secondary amyloidosis and, rarely, tubulointerstital involvement. Vasculitis secondary to infections, malignancy, and drugs are not common among children. However, vasculitis may be associated with a number of rheumatic diseases in childhood and the auto-inflammatory syndromes (periodic fever syndromes). Auto-inflammatory syndromes are diseases characterized by periodic attacks of clinical and laboratory inflammation. Studies carried out during the past decade have provided valuable information on the mechanism of inflammation and innate immunity in general. This group of vasculitides is associated with secondary amyloidosis of the kidney if not treated. Hypocomplementemic urticarial vasculitis is an interesting vasculitic disease with frequent kidney involvement. Here, we introduce the reader to the wide scope of these diseases; although rare, such diseases represent a challenge to the nephrologist. PMID:19898878

  20. Primary central nervous system vasculitis and its mimicking diseases - clinical features, outcome, comorbidities and diagnostic results - A case control study.

    Science.gov (United States)

    Becker, J; Horn, P A; Keyvani, K; Metz, I; Wegner, C; Brück, W; Heinemann, F M; Schwitalla, J C; Berlit, P; Kraemer, M

    2017-05-01

    To compare clinical features and outcome, imaging characteristics, biopsy results and laboratory findings in a cohort of 69 patients with suspected or diagnosed primary central nervous system vasculitis (PCNSV) in adults; to identify risk factors and predictive features for PCNSV. We performed a case-control-study including 69 patients referred with suspected PCNSV from whom 25 were confirmed by predetermined diagnostic criteria based on biopsy (72%) or angiography (28%). Forty-four patients turned out to have 15 distinct other diagnoses. Clinical and diagnostic data were compared between PCNSV and Non-PCNSV cohorts. Clinical presentation was not able to discriminate between PCNSV and its differential diagnoses. However, a worse clinical outcome was associated with PCNSV (p=0.005). Biopsy (p=0.004), contrast enhancement (p=0.000) or tumour-like mass lesion (p=0.008) in magnetic resonance imaging (MRI), intrathecal IgG increase (p=0.020), normal Duplex findings of cerebral arteries (p=0.022) and conventional angiography (p 0.010) were able to distinguish between the two cohorts. In a cohort of 69 patients with suspected PCNSV, a large number (64%) was misdiagnosed and partly received treatment, since mimicking diseases are very difficult to discriminate. Clinical presentation at manifestation does not help to differentiate PCNSV from its mimicking diseases. MRI and cerebrospinal fluid analysis are unlikely to be normal in PCNSV, though unspecific if pathological. Cerebral angiography and biopsy must complement other diagnostics when establishing the diagnosis in order to avoid misdiagnosis and mistreatment. German clinical trials register: http://drks-neu.uniklinik-freiburg.de/drks_web/, Unique identifier: DRKS00005347. Copyright © 2017 Elsevier B.V. All rights reserved.

  1. The European Vasculitis Society 2016 Meeting Report

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    Ingeborg M. Bajema

    2017-11-01

    Full Text Available The 2016 European Vasculitis Society (EUVAS meeting, held in Leiden, the Netherlands, was centered around phenotypic subtyping in antineutrophil cytoplasmic antibody (ANCA–associated vasculitis (AAV. There were parallel meetings of the EUVAS petals, which here report on disease assessment; database; and long-term follow-up, registries, genetics, histology, biomarker studies, and clinical trials. Studies currently conducted will improve our ability to discriminate between different forms of vasculitis. In a project that involves the 10-year follow-up of AAV patients, we are working on retrieving data on patient and renal survival, relapse rate, the cumulative incidence of malignancies, and comorbidities. Across Europe, several vasculitis registries were developed covering over 10,000 registered patients. In the near future, these registries will facilitate clinical research in AAV on a scale hitherto unknown. Current studies on the genetic background of AAV will explore the potential prognostic significance of genetic markers and further refine genetic associations with distinct disease subsets. The histopathological classification of ANCA-associated glomerulonephritis is currently evaluated in light of data coming out of a large international validation study. In our continuous search for biomarkers to predict clinical outcome, promising new markers are important subjects of current research. Over the last 2 decades, a host of clinical trials have provided evidence for refinement of therapeutic regimens. We give an overview of clinical trials currently under development, and consider refractory vasculitis in detail. The goal of EUVAS is to stimulate ongoing research in clinical, serological, and histological management and techniques for patients with systemic vasculitis, with an outlook on the applicability for clinical trials.

  2. Prolonged urticaria with purpura: the spectrum of clinical and histopathologic features in a prospective series of 22 patients exhibiting the clinical features of urticarial vasculitis.

    Science.gov (United States)

    Lee, Joyce Siong See; Loh, Teck Hiong; Seow, Swee Chong; Tan, Suat Hoon

    2007-06-01

    Biopsy specimens of lesions with clinical features of urticarial vasculitis often show a predominantly lymphocytic infiltrate with eosinophils and red blood cell extravasation. Only occasionally is a leukocytoclastic vasculitis encountered, confirming a diagnosis of urticarial vasculitis. The aim of this study was to assess the clinical presentation and histologic features of patients who meet the clinical criteria for urticarial vasculitis. Patients were recruited who had persistent urticarial lesions individually lasting longer than 24 hours, associated with at least 2 of 3 of the following: pain or tenderness; purpura or dusky changes; and resolution with hyperpigmentation. Patients were interviewed based on a standard questionnaire with regard to their symptoms. Blood tests and chest radiographs were performed to exclude systemic involvement and hypocomplementemia. Skin biopsy specimens were sent for histology and direct immunofluorescence. Of 22 patients recruited, 19 (86.4%) showed a predominantly lymphocytic infiltrate on histology. Three cases (13.6%) had a neutrophil-predominant infiltrate associated with a leukocytoclastic vasculitis. Twenty (90.9%) had a superficial perivascular infiltrate, and two (9.1%) had a superficial and deep perivascular infiltrate. In all, 21 biopsy specimens (95.5%) showed inflammatory cells within dermal blood vessel walls, obscuring the vessel outline in some. Endothelial cell swelling was seen in 20 biopsy specimens (90.9%), erythrocyte extravasation in 17 (77.3%), nuclear dust in 5 (22.7%), and fibrin extravasation in 2 (9.1%). Multivariate analysis revealed the following features to be independently associated with neutrophil predominance: fulfillment of all 3 minor criteria for urticarial vasculitis-like lesions (P = .007); presence of fibrin on histology (P urticarial vasculitis reveal that only a minority of patients has leukocytoclastic vasculitis. The majority has a lymphocyte-predominant histology, associated with

  3. Urticarial Vasculitis in Northern Spain

    Science.gov (United States)

    Loricera, Javier; Calvo-Río, Vanesa; Mata, Cristina; Ortiz-Sanjuán, Francisco; González-López, Marcos A.; Alvarez, Lino; González-Vela, M. Carmen; Armesto, Susana; Fernández-Llaca, Héctor; Rueda-Gotor, Javier; González-Gay, Miguel A.; Blanco, Ricardo

    2014-01-01

    Abstract Urticarial vasculitis (UV) is a subset of cutaneous vasculitis (CV), characterized clinically by urticarial skin lesions of more than 24 hours’ duration and histologically by leukocytoclastic vasculitis. We assessed the frequency, clinical features, treatment, and outcome of a series of patients with UV. We conducted a retrospective study of patients with UV included in a large series of unselected patients with CV from a university hospital. Of 766 patients with CV, UV was diagnosed in 21 (2.7%; 9 male and 12 female patients; median age, 35 yr; range, 1–78 yr; interquartile range, 5–54 yr). Eight of the 21 cases were aged younger than 20 years old. Potential precipitating factors were upper respiratory tract infections and drugs (penicillin) (n = 4; in all cases in patients aged urticarial lesions, other features such as palpable purpura (n = 7), arthralgia and/or arthritis (n = 13), abdominal pain (n = 2), nephropathy (n = 2), and peripheral neuropathy (n = 1) were observed. Hypocomplementemia (low C4) with low C1q was disclosed in 2 patients. Other abnormal laboratory findings were leukocytosis (n = 7), increased erythrocyte sedimentation rate (n = 6), anemia (n = 4), and antinuclear antibody positivity (n = 2). Treatment included corticosteroids (n = 12), antihistaminic drugs (n = 6), chloroquine (n = 4), nonsteroidal antiinflammatory drugs (n = 3), colchicine (n = 2), and azathioprine (n = 1). After a median follow-up of 10 months (interquartile range, 2–38 mo) recurrences were observed in 4 patients. Apart from 1 patient who died because of an underlying malignancy, the outcome was good with full recovery in the remaining patients. In conclusion, our results indicate that UV is rare but not exceptional. In children UV is often preceded by an upper respiratory tract infection. Urticarial lesions and joint manifestations are the most frequent clinical manifestation. Low complement serum levels are observed in a minority of cases. The prognosis

  4. Hypocomplementemic urticarial vasculitis syndrome.

    Science.gov (United States)

    Jara, Luis J; Navarro, Carmen; Medina, Gabriela; Vera-Lastra, Olga; Saavedra, Miguel A

    2009-12-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is an uncommon immune complex-mediated entity characterized by urticaria with persistent acquired hypocomplementemia. First described in 1973, HUVS is associated with several systemic findings including leukocytoclastic vasculitis, severe angioedema, laryngeal edema, pulmonary involvement, arthritis, arthralgia, glomerulonephritis, and uveitis. These manifestations should be present for at least 6 months. Laboratory findings include low complement levels of classical pathway, namely C1q, C2, C3, and C4. The disease marker is the serum presence of anti-C1q antibodies. Treatment, based on disease severity, involves corticosteroids and other immunosuppressive agents that have demonstrated some success. Patients may have significant morbidity and mortality, most commonly caused by chronic obstructive pulmonary disease and acute laryngeal edema.

  5. Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis

    Science.gov (United States)

    Grayson, Peter C.; Sloan, J. Mark; Niles, John L.; Monach, Paul A.; Merkel, Peter A.

    2011-01-01

    Objectives Reports of an association between antineutrophil cytoplasmic antibodies (ANCA) and autoimmune neutropenia have rarely included cases of proven vasculitis. A case of ANCA-associated vasculitis (AAV) with recurrent neutropenia is described and relevant literature on the association between ANCA, neutropenia, and vasculitis is reviewed. Methods Longitudinal clinical assessments and laboratory findings are described in a patient with AAV and recurrent episodes of profound neutropenia from December 2008 – October 2010. A PubMed database search of the medical literature was performed for papers published from 1960 through October 2010 to identify all reported cases of ANCA and neutropenia. Results A 49 year-old man developed recurrent neutropenia, periodic fevers, arthritis, biopsy-proven cutaneous vasculitis, sensorineural hearing loss, epididymitis, and positive tests for ANCA with specificity for antibodies to both proteinase 3 and myeloperoxidase. Antineutrophil membrane antibodies were detected during an acute neutropenic phase and were not detectable in a post-recovery sample, whereas ANCA titers did not seem to correlate with neutropenia. An association between ANCA and neutropenia has been reported in 74 cases from 24 studies in the context of drug/toxin exposure, underlying autoimmune disease, or chronic neutropenia without underlying autoimmune disease. In these cases, the presence of atypical ANCA patterns and other antibodies were common; however, vasculitis was uncommon and when it occurred was usually limited to the skin and in cases of underlying toxin exposure. Conclusions ANCA is associated with autoimmune neutropenia, but systemic vasculitis rarely occurs in association with ANCA and neutropenia. The interaction between neutrophils and ANCA may provide insight into understanding both autoimmune neutropenia and AAV. PMID:21507463

  6. THROMBOTIC AND THROMBOEMBOLIC COMPLICATIONS IN SYSTEMIC VASCULITIS

    Directory of Open Access Journals (Sweden)

    E. V. Arseniev

    2017-01-01

    Full Text Available Nowadays, there is no doubt about the relationship between immune inflammation and the development of thrombosis due to the similarity of many pathogenetic machanisms underlying both pathological processes. Systemic vasculitis, along with other immuno- mediated inflammatory diseases, represent one of the most clear examples of such interaction. Thrombotic complications remain one of the most serious and life-threatening conditions that occur in patients with autoimmune diseases, in particular systemic vasculitis. This serves as powerful stimulus for studing the problem of hypercoagulation, which often accompanies the course of systemic vasculitis. Of interest are thrombosis that occure both in the injury of large vessel and in the involvement of medium and small vessels in the pathological process. According to the literature, thrombotic and thromboembolic complications are most common in ANCA-associated vasculitis and Behçet’s disease. This review discusses current studies regarding features of clinical picture and mechanisms of thrombosis development in systemic vasculitis. Because of these studies it became clear that for some vasculitis a high frequency of both arterial and venous thrombosis is characteristic. At the same time, other vasculitis are accompanied by high risk of only venous thrombosis. Finally, thrombosis and thromboembolism are quite rare complications for some vasculitis. Among the presented mechanisms of thrombosis involvement, disruptions of the hemostasis are widely considered. For examples, the presents the results of researches, which is actively discussed the role of neutrophil extracellular traps and antiendothelial cell antibodies in the development on thrombotic complictions. Special attention is paid to the possible role of some disorders of hemostasis, such as polymorphism V coagulation factor G1691FA and prothrombin G20210A. To search for literature sources, the following queries were used: “vasculitis”,

  7. Autoimmunity in chronic urticaria and urticarial vasculitis.

    Science.gov (United States)

    Napoli, D C; Freeman, T M

    2001-07-01

    In contrast to acute urticaria, etiology cannot be identified in most cases of chronic urticaria. Recent evidence suggests that a subset of patients with chronic urticaria may have an autoimmune basis for their condition. The demonstration of antithyroid autoantibodies in some patients with chronic idiopathic urticaria (CIU) provides support for an association. However, the discovery of a positive skin test response to intradermal injection of autologous serum in as many as 60% of patients with CIU led to the identification of autoantibodies to IgE and the alpha-chain of the high-affinity IgE receptor, Fc epsilon RI alpha. Additional studies have demonstrated that some of these autoantibodies are capable of releasing histamine from donor basophils and mast cells. This article reviews the literature that addresses a possible autoimmune etiology in a subset of patients with CIU. Urticarial vasculitis is differentiated from chronic urticaria based on clinical features and biopsy findings of leukocytoclastic vasculitis. Most cases of urticarial vasculitis are secondary to an underlying systemic disease. The presence of autoantibodies has also been demonstrated in a subset of patients with primary urticarial vasculitis. This article briefly reviews some of this data.

  8. Clinically Apparent Arterial Thrombosis in Persons with Systemic Vasculitis

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    Alexander Tsoukas

    2017-01-01

    Full Text Available Objective. To estimate the incidence rate of clinically apparent arterial thrombotic events and associated comorbidities in patients with primary systemic vasculitis. Methods. Using large cohort administrative data from Quebec, Canada, we identified patients with vasculitis, including polyarteritis nodosa (PAN and granulomatosis with polyangiitis (GPA. Incident acute myocardial infarctions (AMIs and cerebrovascular accidents (CVAs after the diagnosis of vasculitis were ascertained in the PAN and GPA group via billing and hospitalization data. These were compared to rates of a general population comparator group. The incidences of comorbidities (type 2 diabetes mellitus, dyslipidemia, and hypertension were also collected. Results. Among the 626 patients identified with vasculitis, 19.7% had PAN, 2.9% had Kawasaki disease, 23.8% had GPA, 52.4% had GCA, and 1.3% had Takayasu arteritis. The AMI rate was substantially higher in males aged 18–44 with PAN, with rates up to 268.1 events per 10,000 patient years [95% CI 67.1–1070.2], approximately 30 times that in the age- and sex-matched control group. The CVA rate was also substantially higher, particularly in adults aged 45–65. Patients with vasculitis had elevated incidences of diabetes, dyslipidemia, and hypertension versus the general population. Conclusion. Atherothrombotic rates were elevated in patients identified as having primary systemic vasculitis. While incident rates of cardiovascular comorbidities were also increased, the substantial elevation in AMIs seen in young adults suggests a disease-specific component which requires further investigation.

  9. Clinically Apparent Arterial Thrombosis in Persons with Systemic Vasculitis.

    Science.gov (United States)

    Tsoukas, Alexander; Bernatsky, Sasha; Joseph, Lawrence; Buckeridge, David L; Bélisle, Patrick; Pineau, Christian A

    2017-01-01

    To estimate the incidence rate of clinically apparent arterial thrombotic events and associated comorbidities in patients with primary systemic vasculitis. Using large cohort administrative data from Quebec, Canada, we identified patients with vasculitis, including polyarteritis nodosa (PAN) and granulomatosis with polyangiitis (GPA). Incident acute myocardial infarctions (AMIs) and cerebrovascular accidents (CVAs) after the diagnosis of vasculitis were ascertained in the PAN and GPA group via billing and hospitalization data. These were compared to rates of a general population comparator group. The incidences of comorbidities (type 2 diabetes mellitus, dyslipidemia, and hypertension) were also collected. Among the 626 patients identified with vasculitis, 19.7% had PAN, 2.9% had Kawasaki disease, 23.8% had GPA, 52.4% had GCA, and 1.3% had Takayasu arteritis. The AMI rate was substantially higher in males aged 18-44 with PAN, with rates up to 268.1 events per 10,000 patient years [95% CI 67.1-1070.2], approximately 30 times that in the age- and sex-matched control group. The CVA rate was also substantially higher, particularly in adults aged 45-65. Patients with vasculitis had elevated incidences of diabetes, dyslipidemia, and hypertension versus the general population. Atherothrombotic rates were elevated in patients identified as having primary systemic vasculitis. While incident rates of cardiovascular comorbidities were also increased, the substantial elevation in AMIs seen in young adults suggests a disease-specific component which requires further investigation.

  10. Anti-TNF-Alpha Therapy and Systemic Vasculitis

    Directory of Open Access Journals (Sweden)

    Pierre-André Jarrot

    2014-01-01

    Full Text Available TNF-α is a pleiotropic cytokine, which plays a major role in the pathogenesis of numerous autoimmune and/or inflammatory systemic diseases. Systemic vasculitis constitutes a group of rare diseases, characterized by inflammation of the arterial or venous vessel wall, causing stenosis and thrombosis. Treatment of the different type of vasculitis mainly relies on steroids and immunosuppressive drugs. In case of refractory or relapsing diseases, however, a second line of treatment may be required. Anti-TNF-α drugs have been used in this setting during the last 15 years with inconsistent results. We reviewed herein the use of anti-TNF-α therapy in different kind of vasculitis and concluded that, except for Behcet’s disease, this therapeutic option has not demonstrated significant improvement in the treatment of vasculitis.

  11. Levamisole-Induced Vasculitis: A Characteristic Cutaneous Vasculitis Associated With Levamisole-Adulterated Cocaine.

    Science.gov (United States)

    Roberts, Jordan A; Chévez-Barrios, Patricia

    2015-08-01

    Levamisole-induced vasculitis is a characteristic cutaneous vasculitis syndrome associated with the use of levamisole-adulterated cocaine. Patients will typically present with a painful, purpuric rash in a retiform or stellate pattern with or without central necrosis involving the extremities, trunk, nasal tip, digits, cheeks, and/or ears. A history of cocaine abuse can be elicited. Histologic features include microvascular thrombi and/or leukocytoclastic vasculitis involving small vessels of the superficial and deep dermis. Epidermal involvement is variably seen. Laboratory findings include leukopenia, neutropenia (including agranulocytosis), elevated erythrocyte sedimentation rate, normal coagulation studies, and positive autoantibodies including perinuclear and cytoplasmic antineutrophil cytoplasmic antibodies, antinuclear antibody, and lupus anticoagulant. Differential diagnosis includes other microscopic vasculitides, and clinical and laboratory correlation with histologic findings is essential. Lesions typically resolve with the cessation of cocaine use. Because of the treatment implications and rising incidence of this entity, rapid and accurate diagnosis is essential.

  12. Hypocomplementemic urticarial vasculitis in systemic lupus erythematosus.

    Science.gov (United States)

    Her, Min Young; Song, Joo Yeon; Kim, Dong Yook

    2009-02-01

    Urticarial vasculitis is characterized clinically by urticarial skin lesions and histologically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis is associated with connective tissue diseases such as systemic lupus erythematosus (SLE). We report a case of urticarial vasculitis that preceded manifestations of SLE.

  13. Hypocomplementemic Urticarial Vasculitis in Systemic Lupus Erythematosus

    OpenAIRE

    Her, Min Young; Song, Joo Yeon; Kim, Dong Yook

    2009-01-01

    Urticarial vasculitis is characterized clinically by urticarial skin lesions and histologically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis is associated with connective tissue diseases such as systemic lupus erythematosus (SLE). We report a case of urticarial vasculitis that preceded manifestations of SLE.

  14. Onychomadesis Following Cutaneous Vasculitis.

    Science.gov (United States)

    Damevska, Katerina; Gocev, Gorgi; Pollozahani, Nora; Nikolovska, Suzana; Neloska, Lence

    2017-04-01

    Beau lines are transverse, band-like depressions extending from one lateral edge of the nail to the other and affecting all nails at corresponding levels (1). Onychomadesis is considered an extreme form of Beau line with subsequent separation of the proximal nail plate from the nail bed. Both fall along a spectrum of nail plate abnormalities that occur secondary to temporary nail matrix arrest (NMA). Various systemic and dermatologic conditions have been reported in association with onychomadesis (2-7) (Table 1). Nail changes can affect all or some of the nails and both the fingernails and toenails; however, fingernails are more frequently affected. The severity of the nail changes varies depending on the underlying cause, its duration, and environmental factors (8). We present a case of onychomadesis following cutaneous leukocytoclastic vasculitis (CLCV). A 61-year-old woman presented to the Dermatology Clinic complaining of a purpuric rash that began on her lower extremities and rapidly progressed to her abdomen and upper extremities over the previous five days. Her medical history was remarkable for hypertension and diet-controlled diabetes mellitus. Her medications included enalapril, which she had been taking for the past four years. On three consecutive days before the skin eruption, the patient took oral diclofenac sodium for hip pain. A clinical examination revealed non-blanching petechial rash on the legs, abdomen, and upper limbs up to the elbow (Figure 1, A) with leukocytoclastic vasculitis on biopsy (Figure 1, B). Direct immunofluorescence was negative. Laboratory investigations revealed a white blood cell count of 14.5 × 109/L with a normal differential count, and a platelet count of 380 × 109/L. Westergren erythrocyte sedimentation rate was 65 mm/1st h, and C reactive protein was at 8.5 mg/dL. Antinuclear antibodies, rheumatoid factor, immune complexes, and cryoglobulinemia were negative, as were B and C hepatitis virus serological tests. Her renal

  15. Urticarial vasculitis: a unique presentation.

    Science.gov (United States)

    Stigall, Landon E; Sigmon, Justin R; Leicht, Stuart S

    2009-05-01

    Urticarial vasculitis is a relatively rare diagnosis in a patient presenting with urticaria. The process is classically described as a generalized eruption, painful more so than pruritic, lasting longer than 24 hours. Two forms of urticarial vasculitis have been described: ahypocomplementemic form more commonly associated with systemic disease, and a normocomplementemic form that is generally limited to the skin. We report on a uniquely distributed vasculitic eruption restricted mainly to the anterior belt line area in a patient presenting with urticaria and intense pruritus. Urticarial vasculitis as a unique entity is reviewed along with its clinical and histopathologic presentation and the pharmacologic agents used for treatment.

  16. Urticarial vasculitis and Schnitzler syndrome.

    Science.gov (United States)

    Zuberbier, Torsten; Zuberbier, H C Torsten; Maurer, Marcus

    2014-02-01

    Both urticarial vasculitis and Schnitzler syndrome are serious diseases but a recently gained better understanding of their underlying pathomechanism allows better treatment options than only a decade ago. Copyright © 2014 Elsevier Inc. All rights reserved.

  17. Endothelial cells, fibroblasts and vasculitis

    OpenAIRE

    Buckley, Christopher D.; Rainger, G.Ed; Nash, Gerard B; Raza, Karim

    2005-01-01

    One of the most important questions in vasculitis research is not why inflammation of blood vessels occurs but why it persists, often in a site-specific manner. In this review we illustrate how stromal cells, such as fibroblasts and pericytes, might play an important role in regulating the site at which vasculitis occurs. Smooth muscle cells and fibroblasts directly influence the behaviour of overlying vascular cells, amplifying the response of the endothelium to proinflammatory agents such a...

  18. Vancomycin-Associated Leukocytoclastic Vasculitis

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    Makhawadee Pongruangporn

    2011-01-01

    Full Text Available Vancomycin is U.S. Food and Drug Administration (FDA approved for treatment of serious infections caused by methicillin-resistant Staphylococcus aureus (MRSA or in individuals who have failed, cannot tolerate, or are allergic to other antibiotics. Very few cases of vancomycin-associated leukocytoclastic vasculitis have been published. We report on a patient who developed pruritus and palpable purpura in both lower extremities after receiving six days of intravenous vancomycin. Skin biopsy revealed leukocytoclastic vasculitis.

  19. Study Groups in Denmark

    DEFF Research Database (Denmark)

    Hjorth, Poul G.

    2007-01-01

    Since 1998 European Study Groups have been held in Denmark, and Danish companies from LEGO and NOVO to very small high-tech firms have participated. I briefly describe the history, the organisation and the format of the Danish Study Groups, and highlight a few problem solutions.......Since 1998 European Study Groups have been held in Denmark, and Danish companies from LEGO and NOVO to very small high-tech firms have participated. I briefly describe the history, the organisation and the format of the Danish Study Groups, and highlight a few problem solutions....

  20. Hepatitis B virus related cryoglobulinemic vasculitis: A multicentre open label study from the Gruppo Italiano di Studio delle Crioglobulinemie - GISC.

    Science.gov (United States)

    Mazzaro, Cesare; Dal Maso, Luigino; Urraro, Teresa; Mauro, Endri; Castelnovo, Laura; Casarin, Pietro; Monti, Giuseppe; Gattei, Valter; Zignego, Anna Linda; Pozzato, Gabriele

    2016-07-01

    Cryoglobulinemic vasculitis (CV) related to Hepatitis-B Virus (HBV) is rare and its treatment is ill-defined. To describe clinical and treatment characteristics of HBV-related CV patients. In addition, the efficacy of treatment with antiviral agent nucleotide (NUC), including Entecavir, Adefovir, and Lamivudine, was explored. In four Italian centres, 17 HBV-positive CV patients (median age 56 years, range 45-70) were enrolled. The extrahepatic manifestations were: purpura (100%), arthralgias (71%), peripheral neuropathy (29%), chronic hepatitis (47%), liver cirrhosis (29%), and glomerulonephritis (18%). Mixed cryoglobulinemias were type II (88%) and type III (12%). The median cryocrit was 3% (range 1-14), rheumatoid factor was 200U/L (range 20-5850), C4 was 12mg/dl (range 2-31), ALT 71U/L (range 36-114). All patients were HBsAg-positive and 80% anti-HbeAg-positive. At enrollment, they were treated with steroids (eight), Entecavir (five), Alpha-IFN (two), Adefovir and Lamivudine (one each). After NUC treatment, no disease progression was observed and, in all patients, HBV-DNA became undetectable. Moreover, a regression of purpura and a reduction of cryocrit were observed. Four patients died during therapy, two of kidney failure and two of liver cirrhosis. NUC therapy appeared to be safe and effective in CV-related HBV. Copyright © 2016 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  1. Neonatal cerebral oxygenation is not linked to foetal vasculitis and predicts intraventricular haemorrhage in preterm infants

    DEFF Research Database (Denmark)

    Sorensen, Line C; Maroun, Lisa L; Borch, Klaus

    2008-01-01

    Aim: The aim of the study was to compare the cerebral tissue oxygenation index (c-TOI) measured by near infrared spectroscopy (NIRS) in infants with and without foetal vasculitis. Methods: Twenty-four infants with placental signs of a foetal inflammatory response (FIR), foetal vasculitis, were co.......002). Conclusion: Cerebral oxygenation was not affected in the first day of life in preterm infants born with foetal vasculitis, while cerebral oxygenation in infants that later developed intraventricular haemorrhage was impaired....

  2. The ANCA Vasculitis Questionnaire (AAV-PRO©)

    Science.gov (United States)

    2017-05-01

    Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (Wegener's) (GPA); Wegener Granulomatosis (WG); Microscopic Polyangiitis (MPA); ANCA-Associated Vasculitis (AAV); Vasculitis

  3. Urticarial Vasculitis-Associated Intestinal Ischemia

    National Research Council Canada - National Science Library

    Wong, Uni; Yfantis, Harris; Xie, Guofeng

    2016-01-01

    Urticarial vasculitis (UV) is a rare small vessel vasculitis. UV is often idiopathic but can also present in the context of autoimmune disorders such as systemic lupus erythematosus, drug reactions, infections, or a paraneoplastic syndrome...

  4. Group theories: relevance to group safety studies.

    Science.gov (United States)

    Benevento, A L

    1998-01-01

    Promoting safety in the workplace has been attempted in a variety of ways. Increasingly, industries are using groups such as safety teams and quality circles to promote worker safety. Group influences on individual behavior and attitudes have long been studied in the social psychology literature, but the theories have not been commonly found outside the psychology arena. This paper describes the group theories of group polarization, risky shift, social loafing, groupthink and team think and attempts to apply these theories to existing studies that examine work group influences on safety. Interesting parallels were found but only one study examined group influences as their primary focus of research. Since groups are increasingly used for safety promotion, future research on safety that studies group influences with respect to current group theories is recommended.

  5. Urticarial vasculitis reveals unsuspected thyroiditis.

    Science.gov (United States)

    Ferreira, Olga; Mota, Alberto; Baudrier, Teresa; Azevedo, Filomena

    2012-01-01

    A 38-year-old woman presented with erythematous, violaceous plaques with a serpiginous and unusual appearance located on the left shoulder, left thigh, and right buttock, evolving for 5 days, which eventually became generalized. A skin biopsy revealed leukocytoclastic vasculitis and a diagnosis of urticarial vasculitis was made. The complete blood count, biochemistry, complement levels, and other immunological test results were unremarkable. However, antithyroid antibody titers were increased. Despite having normal thyroid function tests and an absence of specific symptoms, the patient underwent a thyroid ultrasound, which revealed features of thyroiditis, and was subsequently referred to an endocrinologist. Several diseases can be associated with urticarial vasculitis, namely infections and autoimmune connective-tissue disorders such as systemic lupus erythematosus and Sjögren syndrome. Thyroiditis is an uncommon association.

  6. The Pharmacogenomic Association of Fc gamma Receptors and Cytochrome P450 Enzymes With Response to Rituximab or Cyclophosphamide Treatment in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    NARCIS (Netherlands)

    Cartin-Ceba, Rodrigo; Indrakanti, Divya; Specks, Ulrich; Stone, John H.; Hoffman, Gary S.; Kallenberg, Cees G. M.; Langford, Carol A.; Merkel, Peter A.; Spiera, Robert F.; Monach, Paul A.; St. Clair, E. William; Seo, Philip; Tchao, Nadia K.; Ytterberg, Steven R.; Brunetta, Paul G.; Song, Huijuan; Birmingham, Dan; Rovin, Brad H.; Grp, RAVE-Immune Tolerance Network Res

    Objective The Rituximab in ANCA-Associated Vasculitis (RAVE) trial compared rituximab to cyclophosphamide as induction therapy for the treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. We undertook the current study to determine whether known single-nucleotide

  7. Clinical and immunological features of retinal vasculitis in systemic diseases

    Directory of Open Access Journals (Sweden)

    Paović Jelena

    2009-01-01

    Full Text Available Background/Aim. Vasculitis is a clinical, pathologic process characterized by inflammation and necrosis of blood vessel occurring anywhere in the body. The aim of the study was to present some clinical and immunologic features of retinal vasculitis in systemic diseases: systemic lupus erythematosus, multiple sclerosis, sarcoidosis, Behcet's diseases, and others. Methods. A total of 1 254 patients with uveitis were included in the study. The immunochemical diagnostic methods were used to determine the pathogenesis of ocular manifestations. Ocular manifestations were examined using biomicroscope, direct or indirect ophtalmoscopy. Results. Primary retinal vasculitis was diagnosed in 85/1254 (6.8% of total uveitis. In more than half of the cases of vasculitis (58.8%, both arteries and veins were involved in inflammatory process. Periphlebitis was diagnosed far more commonly (36.5% than periarteritis (4.7%. Retinal vasculitis associated with systemic lupus erythematosus was characterized by microinfarctions and the consequent foci of inflammatory cells or diseases of large arteries manifesting in vasospasm and occlusions. Cotton wool spots occurred in 38.3% and retinal hemorrhages in 34% of the cases. In this study periphlebitis of the retina was one of the less frequent ocular manifestations of multiple sclerosis. Comparing with the other changes of the retinal blood vessels, venous sheating occurred in 25.1% and occlusion and vein trombosis in 43.75% of the cases. Retinal vasculitis associated with chronic sarcoidosis occured in 37.5% of patients with sarcoidosis. The most frequent manifestation of ocular sarcoidosis was intermediary uveitis (43.75%. Anterior granulomatous uveitis occured in 37.5% of patients with sarcoidoses. Immune complexes occurred in 13/20 (65% of the patients. Antiretinal anti-S antibody in the serum occurred in 73% of the patients with retinal detachment as a complication of primary disease and in 25% those with vasculitis

  8. Urticarial vasculitis and urticarial autoinflammatory syndromes.

    Science.gov (United States)

    Marzano, A V; Tavecchio, S; Venturini, M; Sala, R; Calzavara-Pinton, P; Gattorno, M

    2015-02-01

    Urticaria is a frequent disorder classified as acute and chronic forms, which presents with wheals that can be associated with angioedema. Several entities may manifest with urticarial skin lesions, encompassing a heterogeneous group of conditions that have to be differentiated from ordinary urticaria. This review is focused on two of these urticarial syndromes: urticarial vasculitis (UV), which represents the most important differential diagnosis with common urticaria, and autoinflammatory diseases such as cryopyrin-associated periodic syndromes (CAPS) and Schnitzler's Syndrome, both rare multisystem forms that may masquerade as common urticaria. UV is a small-vessel vasculitis with predominant skin involvement, characterized by wheals persisting for more than 24 hours, burning rather than itching and resolving with hyperpigmentation as well as by other cutaneous manifestations including purpura, papules, vesicles, bullae and necrotic-ulcerative lesions. Histology shows a classic pattern of leukocytoclastic vasculitis, with possible presence of upper dermal edema. CAPS are classified as three distinct entities: familial cold autoinflammatory syndrome, Muckle-Wells Syndrome and chronic infantile neurological cutaneous and articular syndrome, which represent a spectrum of disorders caused by different mutations in a single gene, NLRP3 (NOD-like receptor 3). This gene encodes for cryopyrin, an inflammasome protein that activates interleukin-1β, leading to an overproduction of this pivotal proinflammatory cytokine. Histologically, urticarial lesions are generally characterized by a perivascular neutrophilic infiltrate. Unlike urticaria, neither UV nor urticarial autoinflammatory syndromes do respond to antihistamines: thus, it is important not to misdiagnose such conditions in order to give the patients specific treatments, potentially preventing serious systemic complications.

  9. Imaging of systemic vasculitis in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Soliman, Magdy [The Hospital for Sick Children, Department of Medical Imaging, Toronto, ON (Canada); Cairo University, Department of Medical Imaging, Cairo (Egypt); Laxer, Ronald; Yeung, Rae [The Hospital for Sick Children, Department of Rheumatology, Toronto, ON (Canada); Manson, David; Doria, Andrea S. [The Hospital for Sick Children, Department of Medical Imaging, Toronto, ON (Canada)

    2015-08-15

    The term ''systemic vasculitis'' encompasses a diverse set of diseases linked by the presence of blood-vessel inflammation that are often associated with critical complications. These diseases are uncommon in childhood and are frequently subjected to a delayed diagnosis. Although the diagnosis and treatment may be similar for adult and childhood systemic vasculitides, the prevalence and classification vary according to the age group under investigation. For example, Kawasaki disease affects children while it is rarely encountered in adults. In 2006, the European League Against Rheumatism (EULAR) and the Pediatric Rheumatology European Society (PReS) proposed a classification system for childhood vasculitis adopting the system devised in the Chapel Hill Consensus Conference in 1993, which categorizes vasculitides according to the predominant size of the involved blood vessels into small, medium and large vessel diseases. Currently, medical imaging has a pivotal role in the diagnosis of vasculitis given recent developments in the imaging of blood vessels. For example, early diagnosis of coronary artery aneurysms, a serious complication of Kawasaki disease, is now possible by magnetic resonance imaging (MRI) of the heart and multidetector computed tomography (MDCT); positron emission tomography/CT (PET/CT) helps to assess active vascular inflammation in Takayasu arteritis. Our review offers a unique approach using the integration of the proposed classification criteria for common systemic childhood vasculitides with their most frequent imaging findings, along with differential diagnoses and an algorithm for diagnosis based on common findings. It should help radiologists and clinicians reach an early diagnosis, therefore facilitating the ultimate goal of proper management of affected children. (orig.)

  10. F-18-fluorodeoxyglucose positron emission tomography in diagnosis and follow-up of patients with different types of vasculitis.

    Science.gov (United States)

    Bleeker-Rovers, C P; Bredie, S J H; van der Meer, J W M; Corstens, F H M; Oyen, W J G

    2003-10-01

    F-18-fluorodeoxyglucose (FDG) accumulates in inflammatory cells due to an increased metabolic rate. Therefore, FDG positron emission tomography (PET) represents a promising imaging technique in patients with vasculitis. The aim of this study was to assess the value of FDG PET in the diagnosis of different types of vasculitis. The results of FDG PET performed because of suspected vasculitis or fever of unknown origin with results indicating vasculitis were reviewed. These results were compared with the final diagnosis, based on the American College of Rheumatology 1990 criteria. FDG PET was ordered because of suspected vasculitis in 20 patients, because of fever of unknown origin in two patients, and for follow-up of vasculitis in five patients. Fourteen patients were diagnosed with vasculitis (giant cell arteritis n = 5, polymyalgia rheumatica n = 2, polyarteritis nodosa n = 3, Takayasu n = 1, Churge-Strauss n = 1, Wegener's granulomatosis n = 1, vasculitis skin n = 1), two patients were diagnosed with fibromuscular dysplasia and one patient had media necrosis of the aorta. In five patients no diagnosis could be reached. FDG PET results were considered to be true-positive in ten patients, true-negative in 14 patients and false-negative in three patients resulting in a positive predictive value of 100% and a negative predictive value of 82%. FDG PET appears to be a promising new imaging technique in diagnosing and determining the extent of various forms of vasculitis. Furthermore, FDG PET may become a useful tool for evaluating the effect of treatment of vasculitis.

  11. Active necrotizing cerebral vasculitis in systemic lupus erythematosus.

    Science.gov (United States)

    Goel, Deepa; Reddy, S Rajashekhar; Sundaram, Challa; Prayaga, Aruna K; Rajasekhar, Liza; Narsimulu, Gumdal

    2007-12-01

    Systemic lupus erythematosus (SLE) is a multisystemic disease with varied clinical manifestations. Focal cortical brain infarcts and CNS infections are the most common neuropathological features reported in most studies. This report describes a 32-year-old woman who had repeated episodes of strokes over 5 years. In view of polyarthritis, oral ulcers, presence of high titres of serum antinuclear antibodies, high titres of double-stranded DNA and strokes, she was treated as SLE. Despite prolonged immunosuppressive therapy with azathioprine and pulse cyclophosphamide, she succumbed to a brainstem stroke. Complete body autopsy showed multiple cerebral cortical and brainstem infarcts with fibrinoid necrosis of the vessel wall. Renal infarction with healed vasculitis and systemic vasculitis involving small vessels was seen. Extensive thrombosis was remarkable by its absence. Active necrotizing vasculitis of cerebral and renal vessels is a rare complication of SLE, which contributed to a fatal outcome in this patient.

  12. A Case of Crescentic Glomerulonephritis Complicated with Hypocomplementemic Urticarial Vasculitis Syndrome and ANCA-Associated Vasculitis

    Directory of Open Access Journals (Sweden)

    Marenao Tanaka

    2017-11-01

    Full Text Available Systemic urticaria in a 64-year-old woman was diagnosed as leukocytoclastic vasculitis by a punch biopsy of the skin. Her physical findings improved after prescription of prednisolone at a dose of 20 mg/day, but the skin rash relapsed with renal dysfunction, proteinuria, and hematuria when the dose of prednisolone was reduced over a period of 9 months to 1 mg/day. She was admitted to our institute for further examination, when urinary protein and plasma creatinine levels were 0.8 g/day and 1.7 mg/dL, respectively. Complement analysis showed that levels of total hemolytic component, component C3 fraction, and component C4 fraction were 30∼60% of normal values and the titer of anti-neutrophil cytoplasmic antibody for myeloperoxidase (MPO-ANCA was 89 EU (normal range, <10 EU, though there were no immunologic disorders such as systemic lupus erythematosus. Cellular crescentic glomerulonephritis was observed by light microscopy, and immunofluorescent studies showed positive staining for IgG, IgM, C3, C4, and C1q. Electron microscopy showed mesangial and subendothelial deposits with circumferential mesangial interposition. She fulfilled the diagnostic criteria for hypocomplementemic urticarial vasculitis syndrome (HUV, and ANCA-associated vasculitis (AAV was also indicated by small vessel vasculitis and positive MPO-ANCA. Steroid pulse therapy with methylprednisolone followed by oral prednisolone improved her general condition and hypocomplementemia, and MPO-ANCA became negative. HUV and AAV are distinct clinical disorders, though both affect small blood vessels. Here we report a case of AAV-complicated HUV with crescentic glomerulonephritis.

  13. Occurrence of antineutrophil cytoplasmic antibodies and associated vasculitis in patients with hyperthyroidism treated with antithyroid drugs : A long-term followup study

    NARCIS (Netherlands)

    Slot, MC; Links, TP; Stegeman, CA; Tervaert, JWC

    2005-01-01

    Objective. To test whether antineutrophil cytoplasmic antibodies (ANCA) and ANCA-associated vasculitis (AAV) are not only induced during treatment with antithyroid drugs, but can also become evident when medication has been ceased, possibly after years. Methods. Patients who visited our hospital for

  14. Meningococal Septicemia with Cutaneous Vasculitis

    Directory of Open Access Journals (Sweden)

    Radha Mittal

    1989-01-01

    Full Text Available Two cases-had meningococcal septicemia with vasculitis of skin. Out 9 year old female developed generalised ,asymptomatic, purpuric atrophic plaques on the limbs, trunk and face on the fourth day of high fever. The second patient was 20 year old male who developed purplish plaques. Nodules, and bullae mainly on the extensor surface of the hands and feet on the tenth day, of high fever. Bullae had clear fluid and bluish-black peripheral rim

  15. Are the 1990 American College of Rheumatology vasculitis classification criteria still valid?

    Science.gov (United States)

    Seeliger, Benjamin; Sznajd, Jan; Robson, Joanna C; Judge, Andrew; Craven, Anthea; Grayson, Peter C; Suppiah, Ravi S; Watts, Richard A; Merkel, Peter A; Luqmani, Raashid A

    2017-07-01

    Advances in diagnostic techniques have led to better distinction between types of vasculitis, potentially affecting the utility of the 1990 ACR classification criteria for vasculitis. This study tested the performance of these criteria in a contemporary vasculitis cohort. The Diagnosis and Classification in Vasculitis Study provided detailed clinical, serological, pathological and radiological data from patients with primary systemic vasculitis and clinical context-specific comparator conditions. Fulfilment of six ACR criteria sets and their diagnostic performance was evaluated in patients with a given type of vasculitis and its comparator conditions. Data from 1095 patients with primary systemic vasculitis and 415 with comparator conditions were available. For classification, sensitivities and specificities for ACR classification criteria were, respectively, 81.1% and 94.9% for GCA; 73.6% and 98.3% for Takayasu's arteritis; 65.6% and 88.7% for granulomatosis with polyangiitis; 57.0% and 99.8% for eosinophilic granulomatosis with polyangiitis; 40.6% and 87.8% for polyarteritis nodosa; 28.9% and 88.5% for microscopic polyangiitis; and 72.7% and 96.3% for IgA-vasculitis. Overall sensitivity was 67.1%. Of cases identified by their respective criteria, 16.9% also met criteria for other vasculitides. Diagnostic specificity ranged from 64.2 to 98.9%; overall, 113/415 comparators (27.2%) fulfilled at least one of the ACR classification criteria sets. Since publication of the ACR criteria for vasculitis, the sensitivity for each type of vasculitis, except GCA, has diminished, although the specificities have remained high, highlighting the need for updated classification criteria.

  16. Endothelial cells, fibroblasts and vasculitis.

    Science.gov (United States)

    Buckley, Christopher D; Rainger, G Ed; Nash, Gerard B; Raza, Karim

    2005-07-01

    One of the most important questions in vasculitis research is not why inflammation of blood vessels occurs but why it persists, often in a site-specific manner. In this review we illustrate how stromal cells, such as fibroblasts and pericytes, might play an important role in regulating the site at which vasculitis occurs. Smooth muscle cells and fibroblasts directly influence the behaviour of overlying vascular cells, amplifying the response of the endothelium to proinflammatory agents such as TNF-alpha and allowing enhanced and inappropriate leucocyte recruitment. An abnormal local vascular stromal environment can therefore influence local endothelial function and drive the persistence of local vascular inflammation. However, such local vascular inflammation can have distant effects on the systemic vascular system, leading to widespread endothelial cell dysfunction. Vascular endothelial dysfunction is common in a range of immune-mediated inflammatory diseases, is seen in multiple vascular beds, and is reversible following the induction of disease remission. The mechanisms that drive such systemic vascular endothelial dysfunction are unclear but factors such as TNF-alpha and CRP may play a role. Persistence of such widespread endothelial dysfunction in systemic vasculitis appears to have long-term consequences, leading to the acceleration of atherosclerosis and premature ischaemic heart disease. It may also underlie the accelerated atherosclerosis seen in other immune-mediated rheumatic diseases, such as rheumatoid arthritis.

  17. Key advances in the clinical approach to ANCA-associated vasculitis

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    The updated nomenclature for vasculitis defines this varied group of disorders by aetiology, specific features of pathogenesis and clinical symptoms; diagnostic and classification criteria for clinical practice are in development. Here, I review some important advances in the management of

  18. Is serum HMGB1 a biomarker in ANCA-associated vasculitis?

    NARCIS (Netherlands)

    de Souza, Alexandre W.S.; Westra, Johanna; Bijzet, Johan; Limburg, Pieter C.; Stegeman, Coen A.; Bijl, Marc; Kallenberg, Cees G.M.

    2013-01-01

    Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are systemic inflammatory disorders that include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), Churg-Strauss syndrome and renal limited vasculitis (RLV). Extracellular high-mobility group

  19. Urticarial Vasculitis-Associated Intestinal Ischemia

    Directory of Open Access Journals (Sweden)

    Uni Wong

    2016-01-01

    Full Text Available Urticarial vasculitis (UV is a rare small vessel vasculitis. UV is often idiopathic but can also present in the context of autoimmune disorders such as systemic lupus erythematosus, drug reactions, infections, or a paraneoplastic syndrome. Extracutaneous complications include intestinal ischemic injuries, in UV patients with nonspecific gastrointestinal symptoms such as abdominal pain and nausea. Prompt recognition and treatment can minimize morbidity and mortality. This paper describes a case of urticarial vasculitis-associated intestinal ischemia.

  20. Urticarial Vasculitis-Associated Intestinal Ischemia

    Science.gov (United States)

    Wong, Uni; Yfantis, Harris; Xie, Guofeng

    2016-01-01

    Urticarial vasculitis (UV) is a rare small vessel vasculitis. UV is often idiopathic but can also present in the context of autoimmune disorders such as systemic lupus erythematosus, drug reactions, infections, or a paraneoplastic syndrome. Extracutaneous complications include intestinal ischemic injuries, in UV patients with nonspecific gastrointestinal symptoms such as abdominal pain and nausea. Prompt recognition and treatment can minimize morbidity and mortality. This paper describes a case of urticarial vasculitis-associated intestinal ischemia. PMID:27190661

  1. Cutaneous leukocytoclastic vasculitis associated with levofloxacin therapy

    Directory of Open Access Journals (Sweden)

    Dana Blyth

    2012-01-01

    Full Text Available Many cases of cutaneous vasculitis are druginduced with histology revealing leukocytoclastic vasculitis (LCV. We present a case of levofloxacin- associated LCV successfully treated with prednisone and cessation of the offending drug. Although case reports describe a link between LCV and older fluoroquinolones, such as ciprofloxacin and ofloxacin, recent reports have implicated the newer fluoroquinolone levofloxacin. Recognition of fluoroquinoloneinduced cutaneous vasculitis is important as continuation or re-exposure of the offending agent may have life-threatening consequences.

  2. [Primary childhood vasculitis new classification criteria

    DEFF Research Database (Denmark)

    Herlin, T.; Nielsen, Susan

    2008-01-01

    Primary vasculitis is seen in both adults and children, but some of the diseases like Kawasaki disease occur primarily in children. The Chapel Hill Classification Criteria for primary vasculitis refers to the size of vessels but has not been validated in children. Recently, new criteria for the c......Primary vasculitis is seen in both adults and children, but some of the diseases like Kawasaki disease occur primarily in children. The Chapel Hill Classification Criteria for primary vasculitis refers to the size of vessels but has not been validated in children. Recently, new criteria...

  3. Educational Needs of Patients With Systemic Vasculitis

    Science.gov (United States)

    2014-07-11

    Behcet's Disease; Churg-Strauss Syndrome; Vasculitis, Central Nervous System; Giant Cell Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu's Arteritis

  4. Goodpasture's syndrome associated with pulmonary eosinophilic vasculitis.

    Science.gov (United States)

    Komadina, K H; Houk, R W; Vicks, S L; Desrosier, K F; Ridley, D J; Boswell, R N

    1988-08-01

    Lung hemorrhage and antiglomerular basement membrane (anti-GBM) antibody mediated nephritis define Goodpasture's syndrome. We present the case of a 19-year-old Caucasian woman with unique clinical findings of Goodpasture's syndrome. Our patient initially presented with leukocytoclastic vasculitis of the skin followed by the development of nephritis and lung hemorrhage. An open lung biopsy done prior to diagnosing anti-GBM antibody disease demonstrated an intense eosinophilic vasculitis. Skin vasculitis has only been rarely reported, and to our knowledge this is the first reported case of pulmonary eosinophilic vasculitis associated with Goodpasture's syndrome.

  5. Temporal concurrence of vasculitis and cancer: a report of 12 cases.

    Science.gov (United States)

    Hutson, T E; Hoffman, G S

    2000-12-01

    Vasculitis has been associated with solid organ and hematologic cancer. The rarity of these associations, and in many reports the lack of temporal relationships, has led to skepticism about vasculitis being a paraneoplastic syndrome. The objective of the present study was to review cases of concurrent vasculitis and cancer at the Cleveland Clinic Foundation over an 18.5-year period and explore evidence that would support the notion of vasculitis being a type of paraneoplastic disease. Retrospective review of the records of all patients diagnosed with vasculitis and cancer within 12 months of each other was performed using an ICD-9 diagnostic data base at the Cleveland Clinic Foundation. Patients with known chronic autoimmune disease or serologic evidence of hepatitis B or C infection were excluded. A standardized data collection instrument was used to document information about presentation, treatment, and course of illness. During the 18.5 years of our study, more than 15 million inpatients and outpatients were seen at the Cleveland Clinic. Of these, 2,800 patients had vasculitis independent of cancer, more than 69,000 patients had cancer, and 69 patients had been identified who had both malignancies and systemic vasculitis. Only 12 patients were identified in whom both vasculitis and cancer occurred within the same 12 months. Mean age was 65 years (range 45-79). There was no gender preference (M = F). In 8 of the 12 cases, diagnoses were made within 3 months of each other. In 6 of the patients, the diagnoses of both processes were made within 1 month. Ten of the 12 patients had vasculitis 1 to 3 months prior to or concurrent with the diagnosis of cancer. Six of the 12 patients had solid organ tumors, 4 had lymphoma, 1 had leukemia, and 1 had multiple myeloma. The most common vasculitis was cutaneous leukocytoclastic vasculitis (LCV), which occurred in 7 cases. Four cases of LCV were associated with solid organ tumors. Other vasculitides included giant cell

  6. Animal models of antineutrophil cytoplasm antibody-associated vasculitis.

    LENUS (Irish Health Repository)

    Salama, Alan D

    2012-01-01

    To provide an update on the experimental models that have been developed recapitulating clinical antineutrophil cytoplasm antibody (ANCA) associated vasculitis. The application of the models in the study of pathogenesis, and the therapeutic implications of this, are covered in the article by van Timmeren and Heeringa in this issue.

  7. Imaging of CNS Vasculitis

    OpenAIRE

    Nahid Sedighi

    2010-01-01

    The vasculitides are the most interesting assorted group of diseases. Most have an imunologic basis resulting in vascular injury."nInflammatory changes within and surrounding the vessel wall result in narrowing and obliteration of the vascular lumen with subsequent thrombotic occlusion or necrosis and rupture of the vessel. The pathologic features in this lesion include spasm, edema, cellular infiltration, and proliferation. "nThese inflammatory changes result in the classic angiogr...

  8. Testicular Vasculitis: A Sonographic and Pathologic Diagnosis

    Directory of Open Access Journals (Sweden)

    Anuj Dixit

    2017-01-01

    Full Text Available Very little has been published about single-organ vasculitis of the testicle in the radiological literature. Consequently, it is a diagnosis that is unfamiliar to most radiologists. This case report describes the sonographic, pathologic, and laboratory findings of testicular vasculitis and reviews the available literature with regard to this subject.

  9. Surface microscopy for discriminating between common urticaria and urticarial vasculitis.

    Science.gov (United States)

    Vázquez-López, F; Maldonado-Seral, C; Soler-Sánchez, T; Perez-Oliva, N; Marghoob, A A

    2003-09-01

    Urticarial vasculitis is a clinicopathological entity that overlaps with common urticaria, and biopsy is required for differentiation between them. To determine, for the first time, if skin surface microscopy can aid in the clinical differentiation between common urticaria and urticarial vasculitis in daily practice. Lesions in 20 consecutive patients with a clinical diagnosis of urticaria were studied by biopsy and skin surface microscopy (10x dermoscope) after covering the lesions with olive oil. Lesions were photographed with Dermaphot equipment. A biopsy was taken from all patients. Statistical analysis included Fisher's exact test and Cohen kappa statistics (intra-observer reproducibility). Two dermoscopic patterns were observed: (i). a red-lined vascular pattern (17/20 patients); and (ii). a purpuric globular pattern (3/20 patients). Leucocytoclastic vasculitis was demonstrated histologically in all lesions presenting purpuric globules (3/3) but in none of the lesions presenting a dermoscopic red-lined pattern (P urticarial lesions, and that purpuric globules indicate underlying leucocytoclastic vasculitis.

  10. Neonatal cerebral oxygenation is not linked to foetal vasculitis and predicts intraventricular haemorrhage in preterm infants

    DEFF Research Database (Denmark)

    Sørensen, Line Carøe; Maroun, Lisa Leth; Borch, K.

    2008-01-01

    AIM: The aim of the study was to compare the cerebral tissue oxygenation index (c-TOI) measured by near infrared spectroscopy (NIRS) in infants with and without foetal vasculitis. METHODS: Twenty-four infants with placental signs of a foetal inflammatory response (FIR), foetal vasculitis, were co.......002). CONCLUSION: Cerebral oxygenation was not affected in the first day of life in preterm infants born with foetal vasculitis, while cerebral oxygenation in infants that later developed intraventricular haemorrhage was impaired Udgivelsesdato: 2008/11...

  11. ANCA-associated vasculitis in scleroderma: a case series of fourteen patients

    Directory of Open Access Journals (Sweden)

    Kimberly P. Liang

    2011-01-01

    Full Text Available Antimyeloperoxidase (MPO, perinuclear antineutrophil cytoplasmic antibodies (pANCA, and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. These diagnoses were confirmed by retrospective review of complete medical records. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD features, type of scleroderma (limited or diffuse; ANCA serology, vasculitic organ system manifestations; and death. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. The majority (71% were female, with mean age at vasculitis diagnosis 53 years. Seven patients (50% had overlap CTD features, and the majority (79% had limited variant of scleroderma. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50% had glomerulonephritis, 11 (79% pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. Six patients (43% died during followup to 2008. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. Further studies are needed to clarify the role and clinical impact of ANCA in scleroderma patients with and without vasculitis.

  12. Vasculitis: Molecular Imaging by Targeting the Inflammatory Enzyme Myeloperoxidase

    Science.gov (United States)

    Su, Henry S.; Nahrendorf, Matthias; Panizzi, Peter; Breckwoldt, Michael O.; Rodriguez, Elisenda; Iwamoto, Yoshiko; Aikawa, Elena; Weissleder, Ralph

    2012-01-01

    Purpose: To determine if a molecular imaging approach targeting the highly oxidative enzyme myeloperoxidase (MPO) can help noninvasively identify and confirm sites of vascular wall inflammation in a murine model of vasculitis. Materials and Methods: Animal experiments were approved by the institutional animal care committee. Twenty-six mice were studied, including eight MPO-deficient and six sham-operated mice as controls. Vasculitis was induced with intraperitoneal injection of Candida albicans water-soluble fraction (CAWS). Aortic root magnetic resonance imaging was performed after intravenous injection of the activatable MPO sensor (bis-5-hydroxytryptamide-diethylenetriaminepentatacetate gadolinium) (n = 23), referred to as MPO-Gd, or gadopentetate dimeglumine (n = 10). Seven mice were randomly assigned to receive either MPO-Gd or gadopentetate dimeglumine first. Aortic root specimens were collected for biochemical and histopathologic analyses to validate imaging findings. Statistical significance was calculated for contrast-to-noise ratios (CNRs) by using the paired t test. Results: In the aortic root, the mean MPO-Gd CNRs after agent injection (CNR = 28.1) were more than 2.5-fold higher than those of sham-operated mice imaged with MPO-Gd and vasculitis mice imaged with gadopentetate dimeglumine (CNR = 10.6) (P gadopentetate dimeglumine. Histopathologic and biochemical analyses for MPO and myeloid cells confirmed imaging findings. In MPO-deficient mice, injection of CAWS did not result in a vasculitis phenotype, implying a key role of the imaging target in disease cause. Conclusion: Molecular imaging targeting MPO can be a useful biomarker to noninvasively detect and confirm inflammation in vasculitis by using a murine model of Kawasaki disease. © RSNA, 2011 Supplemental material: http://radiology.rsna.org/lookup/suppl/doi:10.1148/radiol.11110040/-/DC1 PMID:22084204

  13. Pathogenesis of ANCA-associated vasculitis : recent insights from animal models

    NARCIS (Netherlands)

    van Timmeren, Mirjan M.; Heeringa, Peter

    Purpose of review To provide an update on animal models of antineutrophil cytoplasmic autoantibody (ANCA)-mediated vasculitis and highlight recent insights gained from studies in these models pertaining to immunopathogenesis. Recent findings Animal models support the pathogenic potential of

  14. Churg-Strauss syndrome and hemorragic vasculitis

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    Rui Moreira Marques

    2011-12-01

    Full Text Available Churg-Strauss syndrome (CSS is a rare syndrome characterized by sinusitis, asthma and peripheral eosinophilia. This vasculitic syndrome affects medium and small-sized vessels, the lung being the most commonly affected organ, followed by the skin. The authors report a case of a 59-year-old male with a past history of asthma and allergic rhinitis. He presented necrohemorragic lesions in the distal phalanx of the 2nd, 3rd and 4th fingers of the lefthand and petechial lesions in the plant of both feet, accompanied by asthenia, anorexia and weight loss. The analytical study revealed leukocytosis with eosinophilia, elevated inflammatory parameters and p-ANCA positive antibodies. The diagnosis of CSS was established based on clinical and histopathological data. Cutaneous manifestations of hemorragic vasculitis are rare in CSS syndrome but can be the first manifestation of the disease. The recognition of this presentation is important for the early diagnosis and treatment of this syndrome.

  15. ANCA-associated vasculitis and malignancy

    DEFF Research Database (Denmark)

    Mahr, Alfred; Heijl, Caroline; Le Guenno, Guillaume

    2013-01-01

    In this review, we summarise the current understanding of the potential link between cancer and anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), including granulomatosis with polyangiitis (Wegener's; GPA) and microscopic polyangiitis (MPA). As is true for many autoimmune...

  16. Hypocomplementemic urticarial vasculitis syndrome: an interdisciplinary challenge.

    Science.gov (United States)

    Grotz, Wolfgang; Baba, Hideo A; Becker, Jan U; Baumgärtel, Martin W

    2009-11-01

    Chronic urticaria often points the way to the diagnosis of a systemic disease, particularly when urticarial vasculitis can be demonstrated. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is considered to be an independent immunological disease. Selective literature review and consideration of the author's own clinical experience. The main manifestation of HUVS is chronic urticarial vasculitis with complement deficiency and the demonstration of C1q antibody in the serum. Multiple other organs are involved, sometimes severely. The diagnosis is confirmed by skin biopsy, which reveals leukocytoclastic vasculitis as a pathogenetic correlate of this systemic disease. Although HUVS is relatively rare, the medical specialists that might encounter it-ophthalmologists, rheumatologists, nephrologists, dermatologists, general practitioners, and pediatricians-should include it in their differential diagnoses whenever appropriate. Awareness of HUVS and rational diagnostic evaluation will lessen the chance of it being misdiagnosed as another type of systemic immunological disease and will reduce superfluous diagnostic testing in patients suffering from it.

  17. Urticarial Vasculitis-Associated Intestinal Ischemia

    OpenAIRE

    Uni Wong; Harris Yfantis; Guofeng Xie

    2016-01-01

    Urticarial vasculitis (UV) is a rare small vessel vasculitis. UV is often idiopathic but can also present in the context of autoimmune disorders such as systemic lupus erythematosus, drug reactions, infections, or a paraneoplastic syndrome. Extracutaneous complications include intestinal ischemic injuries, in UV patients with nonspecific gastrointestinal symptoms such as abdominal pain and nausea. Prompt recognition and treatment can minimize morbidity and mortality. This paper describes a ca...

  18. Association of Lupus Anticoagulant With Long-Term Damage Accrual in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

    Science.gov (United States)

    Jordan, Natasha; D'cruz, David P

    2016-05-01

    Antiphospholipid antibodies (aPL) are frequently present in antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients; however, the prognostic impact of aPL in such patients has not been elucidated. The objective of this study was to determine whether persistent aPL positivity in AAV patients was associated with increased long-term damage accrual. Clinical data were retrospectively collected on all AAV patients who attended the vasculitis clinic at our center over a 4-year period. Data collection included presence of lupus anticoagulant (LAC) and IgG and IgM anticardiolipin (aCL) antibody titers, along with concurrent diagnosis of antiphospholipid syndrome (APS). Accumulation of long-term damage was quantified using the Vasculitis Damage Index (VDI). Data from 116 AAV patients were analyzed. A total of 34% (n = 40) had persistently positive aCL or LAC or a concurrent diagnosis of APS and were classified as AAV/aPL. A total of 76 patients (66%) were classified as AAV alone. LAC was present in a statistically higher proportion of AAV/aPL patients than those in the AAV-alone group (P LAC, is present in a significant proportion of AAV patients and is associated with a higher VDI score. Clinicians should consider screening AAV patients for aPL. © 2016, American College of Rheumatology.

  19. Ongoing Cerebral Vasculitis During Treatment of Rocky Mountain Spotted Fever.

    Science.gov (United States)

    Sun, Lisa R; Huisman, Thierry A G M; Yeshokumar, Anusha K; Johnston, Michael V

    2015-11-01

    Rocky Mountain spotted fever is a tickborne infection that produces a systemic small-vessel vasculitis; its prognosis is excellent if appropriate treatment is initiated early. Because the advent of effective antirickettsial therapies predates the widespread use of brain magnetic resonance imaging, there are limited data on the effect of untreated Rocky Mountain spotted fever infection on neuroimaging studies. We describe a 7-year-old girl with delayed treatment of Rocky Mountain spotted fever who suffered severe neurological impairment. Serial brain magnetic resonance images revealed a progressive "starry sky appearance," which is proposed to result from the same small vessel vasculitis that causes the characteristic skin rash of this infection. Neurological injury can continue to occur despite specific antirickettsial therapy in Rocky Mountain spotted fever. This child's clinical features raise questions about the optimal management of this infection, particularly the utility of immune modulating therapies in cases of delayed treatment and neurological involvement. Copyright © 2015 Elsevier Inc. All rights reserved.

  20. Unihemispheric central nervous system vasculitis

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    Sikawat Thanaviratananich

    2017-06-01

    Full Text Available Patients with primary central nervous system vasculitis (PCNSV usually manifest with multiple enhancing bilateral hemispheric lesions. We presented an extremely rare clinical course and follow-up of a patient with PCNSV affecting only a single (right hemisphere. A 33-year-old previously healthy man presented with a left hand clonic seizure followed by a secondary generalized tonic-clonic seizure and dysarthria. MRI brain revealed multiple hyperintense lesions confined to only the right hemisphere with contrast enhancement, involving both white and grey matters. He was treated with a methylprednisolone for 5 days followed by prednisone for suspected acute disseminated encephalomyelitis without improvements. He was presented again with left-sided weakness, transient dysarthria and black objects in left visual field. MRI brain was unchanged. MR angiogram and conventional cerebral angiogram were normal. Autoimmune work-ups were all negative. A brain biopsy showed evidence of PCNSV. He was then successfully treated with intravenous cyclophosphamide followed by oral azathioprine. On a follow-up 3 years later, he remains asymptomatic on azathioprine and a repeat MRI showed all areas of enhancement were gone.

  1. Role of Antioxidants in Horse Serum-mediated Vasculitis in Swine: Potential Relevance to Early Treatment in Mitigation of Coronary Arteritis in Kawasaki Disease.

    Science.gov (United States)

    Philip, Saji; Lee, Wen-Chuan; Cherian, Kotturathu Mammen; Wu, Mei-Hwan; Lue, Hung-Chi

    2017-08-01

    Horse serum-induced immune complex coronary vasculitis in swine is the first experimental model to mimic most of the pictures of Kawasaki disease. Immune complex mechanism has been implicated as one of the possible mechanisms in the pathogenesis of vasculitis in Kawasaki disease. Antioxidants have a significant role in the reduction of cardiovascular diseases in both human and animal studies. We tried giving vitamins A, E, and C to treat immune complex vasculitis, in the hope of mitigating coronary vasculitis in Kawasaki disease. Our study group consisted of 30 pure bred male piglets of 2-3 months of age, and they were divided into test and control groups. The test (AEC) group (n = 20) received two doses of horse serum, 10 mL (0.65 g protein)/kg body weight at 5-day intervals, and oral vitamins A, E, and C once daily for 14 days. The control group (n = 10) was further divided into the saline group (n = 3) receiving two doses of normal saline and the horse serum group (n = 7) receiving two doses of horse serum at 5-day intervals. Piglets were observed for the rashes and coronary artery dimensions. Both the AEC and the control horse serum group developed rashes after horse serum infusions, but the AEC group developed significantly fewer rashes, and no rashes were seen in the saline group. The control horse serum group (mean ± standard deviation = 2.13 ± 0.72) showed significant coronary artery dilatation, whereas there was no significant dilatation in the AEC group (mean ± standard deviation = 0.81 ± 0.58) or the control saline group (p = 0.002). Serum sickness is a prototype of immune complex vasculitis, and the severity can be ameliorated with antioxidants. A trial of therapeutic dosages of vitamins A, E, and C in acute phase of Kawasaki disease, may be effective in mitigation of coronary artery lesion in addition to intravenous immunoglobulin and aspirin. Copyright © 2017. Published by Elsevier B.V.

  2. Evaluation of D-dimer serum levels among patients with chronic urticaria, psoriasis and urticarial vasculitis*

    Science.gov (United States)

    Criado, Paulo Ricardo; Antinori, Lidi Che Leon; Maruta, Celina Wakisaka; dos Reis, Vitor Manoel Silva

    2013-01-01

    BACKGROUND It has been demonstrated that neutrophils, eosinophils and monocytes, under appropriated stimulus, may express tissue factor and therefore, activate the extrinsic pathway of coagulation. We performed a transversal and case-control study of patients with chronic urticaria and patients with psoriasis, in our outpatient clinic to evaluate the production of D-dimer. OBJECTIVE To evaluate D-dimer serum levels in patients with chronic urticaria and its possible correlation with disease activity. PATIENTS AND METHODS The study was conducted from October 2010 until March 2011. We selected 37 consecutive patients from our Allergy Unit and Psoriasis Unit, and divided them into three groups for statistical analysis: (i) 12 patients with active chronic urticaria (CU); (ii) 10 patients with chronic urticaria under remission and (iii) 15 patients with psoriasis (a disease with skin inflammatory infiltrate constituted by neutrophils, lymphocytes and monocytes). Another five patients with urticarial vasculitis were allocated in our study, but not included in statistical analysis. The serum levels of D-dimer were measured by Enzyme Linked Fluorescent Assay (ELFA), and the result units were given in ng/ml FEU. RESULTS Patients with active chronic urticaria had the highest serum levels of D-dimer (purticarial vasculitis. PMID:23793207

  3. Evaluation of D-dimer serum levels among patients with chronic urticaria, psoriasis and urticarial vasculitis.

    Science.gov (United States)

    Criado, Paulo Ricardo; Antinori, Lidi Che Leon; Maruta, Celina Wakisaka; Reis, Vitor Manoel Silva dos

    2013-01-01

    It has been demonstrated that neutrophils, eosinophils and monocytes, under appropriated stimulus, may express tissue factor and therefore, activate the extrinsic pathway of coagulation. We performed a transversal and case-control study of patients with chronic urticaria and patients with psoriasis, in our outpatient clinic to evaluate the production of D-dimer. To evaluate D-dimer serum levels in patients with chronic urticaria and its possible correlation with disease activity. The study was conducted from October 2010 until March 2011. We selected 37 consecutive patients from our Allergy Unit and Psoriasis Unit, and divided them into three groups for statistical analysis: (i) 12 patients with active chronic urticaria (CU); (ii) 10 patients with chronic urticaria under remission and (iii) 15 patients with psoriasis (a disease with skin inflammatory infiltrate constituted by neutrophils, lymphocytes and monocytes). Another five patients with urticarial vasculitis were allocated in our study, but not included in statistical analysis. The serum levels of D-dimer were measured by Enzyme Linked Fluorescent Assay (ELFA), and the result units were given in ng/ml FEU. Patients with active chronic urticaria had the highest serum levels of D-dimer (purticarial vasculitis.

  4. Urticarial vasculitis: etiologies and clinical course.

    Science.gov (United States)

    Kulthanan, Kanokvalai; Cheepsomsong, Meethawee; Jiamton, Sukhum

    2009-01-01

    Out of 64 patients diagnosed with urticarial vasculitis (UV), 49 (76.6%) presented with their first attack of UV. The others experienced recurrent attacks with a mean number of 3.3 past recurrences. Fifteen patients had angioedema (23.4%) and 16 (25%) suffered systemic involvement. The most common abnormal laboratory finding was an increased erythrocyte sedimentation rate. Six of 62 patients (9.7%) had decreased C3 levels. A cause could be identified in 19 patients (29.7%). The most common identified cause was infection; other causes included drugs, malignancy and systemic lupus erythematosus (SLE). The prevalence of immunoreactant deposits in the skin lesions measured by DIF was 54.7% (35 of 64 patients). The median disease duration of each episode was 85 days. The probability that patients were free of symptoms within one year was 70%. Patients with an idiopathic cause had a statistically significant longer course duration of each episode than the group with upper respiratory tract infection. Compared to reports from Westem countries, our patients seemed to have less severe symptoms and a lower percentage of hypocomplementemic UV and SLE.

  5. Clinical features and outcomes of ANCA-associated renal vasculitis

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    Sidy Mohamed Seck

    2012-01-01

    Full Text Available To determine the patterns and outcomes of the pauci-immune vasculitis in the nephrology department at hospital La Conception in Marseille, we conducted a retrospective study including all patients with diagnosis of pauci-immune renal vasculitis between January 1, 2000 and December 31, 2007. Among 33 cases, 25 were diagnosed as Wegener granulomatosis (WG, seven as microscopic polyangitis (MPA and one as Churg-Strauss syndrome (SCS. The median age of the patients was 57.7 years and the sex-ratio (M/F was 1.6. The visceral mani-festations included kidneys (100% of patients, lungs (75%, ENT (52% of WG, and nervous system (57% of MPA. The mean serum creatinine at admission was 3.3 mg/dL. Renal biopsies revealed a pauci-immune crescentic gromerulonephritis in 96% of the cases. Two patients with WG received plasmapheresis and seven patients required emergency hemodialysis. Induction therapy comprised cyclophosphamide IV and corticosteroids, while maintenance therapy included azathioprine for the majority of patients. Eighty four percent of the patients experienced complete remission after induction therapy. During maintenance therapy relapses were more frequent among patients with MPA (28% compared to WG cases (12%. After 35 months of follow-up, eight patients ended on chronic hemodialysis, and five patients died. ANCA associated vasculitis are frequent in our patients. Long-term outcomes are relatively good despite a mortality rate of 15% and 25% of the patients entering dialysis after three years of follow-up.

  6. Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus

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    Ana Paula Sakamoto

    Full Text Available Abstract Objectives: To assess clinical digital vasculitis (DV as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE within a large population. Methods: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. Results: DV was observed in 25/852 (3% cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%, periungual infarction in 7 (28%, tip finger ulceration in 4 (16%, painful nodules in 1 (4% and gangrene in 1 (4%. A poor outcome, with digital resorption, occurred in 5 (20%. Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008, discoid rash (16% vs. 4%, p = 0.017, photosensitivity (76% vs. 45%, p = 0.002 and other cutaneous vasculitides (80% vs. 19%, p 0.05. SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28 vs. 14 (0-58, p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014 was significantly lower in the DV group. Conclusion: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.

  7. Reproductive Health in Men and Women With Vasculitis

    Science.gov (United States)

    2014-06-25

    Giant Cell Arteritis; Takayasu's Arteritis; Polyarteritis Nodosa; Wegener's Granulomatosis; Microscopic Polyangiitis; Churg-Strauss Syndrome; Behcet's Disease; Kawasaki Disease; Henoch-schoenlein Purpura; Vasculitis, Central Nervous System; Drug-induced Necrotizing Vasculitis

  8. Plasmapheresis in a Patient With "Refractory" Urticarial Vasculitis

    OpenAIRE

    Kartal, Ozgur; Gulec, Mustafa; Caliskaner, Zafer; Nevruz, Oral; Cetin, Turker; Sener, Osman

    2012-01-01

    Immune complexes are found in the circulation of 30%-75% of patients with urticarial vasculitis and much evidence supports the role of these immune complexes in the pathogenesis of urticarial vasculitis. Plasmapheresis is effective for removing these immune complexes; however, there are few reports on the use of plasmapheresis in the treatment of urticarial vasculitis. We describe a case of "refractory" urticarial vasculitis in which the symptoms improved after plasmapheresis treatment. We su...

  9. Therapeutic advances in the treatment of vasculitis.

    Science.gov (United States)

    Eleftheriou, Despina; Brogan, Paul A

    2016-04-26

    Considerable therapeutic advances for the treatment of vasculitis of the young have been made in the past 10 years, including the development of outcome measures that facilitate clinical trial design. Notably, these include: a recognition that some patients with Kawasaki Disease require corticosteroids as primary treatment combined with IVIG; implementation of rare disease trial design for polyarteritis nodosa to deliver the first randomised controlled trial for children; first clinical trials involving children for anti-neutrophil cytoplasmic antibody (ANCA) vasculitis; and identification of monogenic forms of vasculitis that provide an understanding of pathogenesis, thus facilitating more targeted treatment. Robust randomised controlled trials for Henoch Schönlein Purpura nephritis and Takayasu arteritis are needed; there is also an over-arching need for trials examining new agents that facilitate corticosteroid sparing, of particular importance in the paediatric population since glucocorticoid toxicity is a major concern.

  10. Crescentic membranoproliferative glomerulonephritis and hypocomplementemic urticarial vasculitis.

    Science.gov (United States)

    Enríquez, Ricardo; Sirvent, Ana Esther; Amorós, Francisco; Pérez, Miguel; Matarredona, Jaime; Reyes, Adolfo

    2005-01-01

    We describe the association of crescentic membranoproliferative glomerulonephritis and hypocomplementemic urticarial vasculitis syndrome. A 39-year-old woman presented edema and proteinuria and later a non-pruritic urticarial rash. Laboratory results showed nephrotic syndrome, hypocomplementemia and positive anti-C1q antibodies. Skin biopsy disclosed leukocytoclastic vasculitis. Acute renal failure developed. Renal biopsy revealed crescentic membranoproliferative glomerulonephritis. She was treated with corticosteroids and cyclosphosphamide with improvement of the renal function and partial remission of the nephrotic syndrome. Afterwards the nephrotic syndrome relapsed, mycophenolate mofetil in monotherapy was administered with reduction in proteinuria. As far as we know only 3 cases, 2 in children and one in an adult, of crescentic glomerulonephritis and hypocomplementemic urticarial vasculitis syndrome have been reported. In our patient renal manifestations preceded urticarial lesions. We provide information on the evolution during a 42-month follow-up.

  11. Severe tuberculous retinal vasculitis in healthy adults

    Directory of Open Access Journals (Sweden)

    Sin Yee Fang

    2015-09-01

    Full Text Available Ocular tuberculosis may be a presenting feature of tuberculous infection, especially with extensive occlusive retinal vasculitis among Asians. Being a curable disease, awareness of its presentation and high index of suspicion are paramount as prompt treatment can halt the disease progression and prevent visual loss. We presented three cases of ocular tuberculosis in young healthy adults who presented with progressive blurring of vision with florid retinal vasculitis seen on funduscopy. Two of them were with no bacillus Calmette-Guerin scar. Fundus fluorescein angiography confirmed the presence of occlusive vasculitis with extensive area of ischemia. All cases showed a raise in erythrocyte sedimentation rate and strongly positive Mantoux tests. Pan-retinal photocoagulation was given to all patients. Two cases responded well to anti-tubercular therapy followed by oral steroids and regained normal vision in both eyes. One patient was not started on anti-tubercular therapy as he requested to return to his native country for further treatment.

  12. Neuroimaging of cerebral vasculitis; Bildgebung zerebraler Vaskulitiden

    Energy Technology Data Exchange (ETDEWEB)

    Wengenroth, M. [Universitaetsklinikum Schleswig-Holstein, Campus Luebeck, Institut fuer Neuroradiologie, Luebeck (Germany); Klinikum der Ludwig-Maximilians-Universitaet, Institut fuer klinische Radiologie, Muenchen (Germany); Saam, T. [Klinikum der Ludwig-Maximilians-Universitaet, Institut fuer klinische Radiologie, Muenchen (Germany); Haehnel, S. [Universitaetsklinikum Heidelberg, Abteilung Neuroradiologie, Heidelberg (Germany)

    2016-01-15

    Cerebral vasculitis can have a variety of origins. Furthermore, there are no vasculitis-specific symptoms or imaging signs and vasculitis of the CNS can mimic many other neurological diseases, which require different treatment approaches. Thus, the clinical and radiological diagnosis of cerebral vasculitis is challenging. Magnetic resonance imaging (MRI) and MR angiography (MRA) should be the radiological imaging methods of choice to assess the degree of parenchymal damage and to detect vessel wall changes. If the results are unclear digital subtraction angiography (DSA) should be pursued in order to also detect changes in medium sized vessels. Vasculitis of small vessels cannot be detected by vascular imaging and requires brain or leptomeningeal biopsy. In this review we present the current diagnostic approach and a variety of imaging findings in cerebral vasculitis and discuss the main radiological differential diagnoses. (orig.) [German] Die zerebrale Vaskulitis kann viele Ursachen haben. Daher ist die klinische und radiologische Diagnose schwierig, auch weil es etliche vaskulitistypische Symptome und MR-Befunde bei anderen Erkrankungen gibt. Die ZNS-Vaskulitis kann zahlreiche andere neurologische Erkrankungen imitieren, die unterschiedlicher Therapie beduerfen. MRT und MR-Angiographie (MRA) sind die Verfahren der Wahl, mit denen die radiologische Diagnostik begonnen werden sollte, um den Parenchymschaden und den Gefaessbefall zu beurteilen. Bei unklaren Befunden kann eine digitale Subtraktionsangiographie (DSA) ergaenzt werden, um auch mittelgrosse Gefaesse beurteilen zu koennen. Der Befall kleiner Gefaesse kann nur bioptisch nachgewiesen werden. In der folgenden Uebersicht stellen wir das diagnostische Herangehen bei zerebraler Vaskulitis vor, praesentieren zahlreiche Befunde und diskutieren die wichtigsten radiologischen Differenzialdiagnosen. (orig.)

  13. Hypocomplementemic urticarial vasculitis syndrome in three siblings.

    Science.gov (United States)

    Ozçakar, Z Birsin; Yalçınkaya, Fatoş; Altugan, F Semsa; Kavaz, Aslı; Ensari, Arzu; Ekim, Mesiha

    2013-03-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is relatively uncommon and generally seen in the fourth decade of life. There are very few pediatric cases with the diagnosis of HUVS in the literature. In this report, we describe the first familial cases of HUVS in three siblings. The disease onset was during childhood period in all patients. One of them developed severe renal involvement and died. The other two had ongoing skin and eye manifestations and the elder one developed lupus. Presence of these three patients is a strong evidence for the role of genetic factors in the pathogenesis of this rare vasculitis.

  14. Profile of retinal vasculitis in a tertiary eye care center in Eastern India

    Directory of Open Access Journals (Sweden)

    Kumar Saurabh

    2011-01-01

    Full Text Available Aims: To provide a fact file on the etiology, clinical presentations and management of retinal vasculitis in Eastern India. Materials and Methods: Retrospective, record based analysis of retinal vasculitis cases in a tertiary care center in Eastern India from January 2007 to December 2009 . Results: One hundred and thirteen eyes of 70 patients of retinal vasculitis were included in this study. Sixty (85.7% patients were male (mean age 33± 11.1 years and 10 (14.3% were female (mean age 32.4 ± 13.6 years. Vasculitis was bilateral in 43 (61.4% and unilateral in 27 (38.6% patients. Commonest symptoms were dimness of vision (73; 64.6% and floaters (36; 31.9%. Vascular sheathing (82; 72.6% and vitritis (51; 45.1% were commonest signs. Mantoux test was positive in 21 (30% patients but tuberculosis was confirmed in only four (5.71% patients. Raised serum angiotensin-converting enzyme level and positive antinuclear antibody level were reported in four (5.71% patients each. Human leukocyte antigen B5 (HLA B5 marker was present in one (1.4% patient. However, none of the total 70 patients were found to have a conclusively proven systemic disease attributable as the cause of retinal vasculitis. Oral corticosteroid (60; 85.7% was the mainstay of treatment. Forty-eight (42.5% eyes maintained their initial visual acuity and 43 (38% gained one or more line at mean follow-up of 16.6± 6.3 months. Conclusion: Retinal vasculitis cases had similar clinical presentations and common treatment plan. There was no systemic disease association with vasculitis warranting a careful approach in prescribing investigations.

  15. Plasmapheresis in a patient with "refractory" urticarial vasculitis.

    Science.gov (United States)

    Kartal, Ozgur; Gulec, Mustafa; Caliskaner, Zafer; Nevruz, Oral; Cetin, Turker; Sener, Osman

    2012-07-01

    Immune complexes are found in the circulation of 30%-75% of patients with urticarial vasculitis and much evidence supports the role of these immune complexes in the pathogenesis of urticarial vasculitis. Plasmapheresis is effective for removing these immune complexes; however, there are few reports on the use of plasmapheresis in the treatment of urticarial vasculitis. We describe a case of "refractory" urticarial vasculitis in which the symptoms improved after plasmapheresis treatment. We suggest that plasmapheresis be considered as an option in patients with severe or treatment-resistant urticarial vasculitis.

  16. Large Vessel Vasculitis Occurring in Rheumatoid Arthritis Patient under Anti-TNF Therapy

    Directory of Open Access Journals (Sweden)

    Valentina Cestelli

    2014-01-01

    Full Text Available Vasculitis is a heterogeneous group of disorders characterized by the presence of necrotic inflammatory phenomena and destruction of blood vessels. Vasculitis is classified as primary (idiopathic or secondary to infections, connective tissue diseases and drugs but can also be considered as a paraneoplastic phenomenon. Evidence shows that the increasing use of biological agents results in a growing number of reports of autoimmune diseases induced by these therapies. An inflammatory articular chronic disease such as rheumatoid arthritis may be complicated by extra-articular manifestations, such as cutaneous or systemic vasculitis. Herewith, we describe the case of a great vessels arteritis in a patient affected by rheumatoid arthritis in therapy with an anti-TNF agent (etanercept.

  17. Propylthiouracil induced leukocytoclastic vasculitis: A rare manifestation

    Directory of Open Access Journals (Sweden)

    Semra Ayturk

    2013-01-01

    Full Text Available Propylthiouracil (PTU is a common drug used in patients with hyperthyroidism. It may cause perinuclearantineutrophil cytoplasmic antibodies (p-ANCA in few patients with Graves′ disease. This antibody has been associated with different forms of vasculitis. We report a patient who presented with cutaneous manifestations of leukocytoclasticvasculitis with simultaneous development of p-ANCAs during PTU therapy for Graves′ disease.

  18. Randomized trial of plasma exchange or high-dosage Methylprednisolone as adjunctive therapy for severe renal vasculitis

    NARCIS (Netherlands)

    Jayne, David R. W.; Gaskin, Gill; Rasmussen, Niels; Abramowicz, Daniel; Ferrario, Franco; Guillevin, Loic; Mirapeix, Eduardo; Savage, Caroline O. S.; Sinico, Renato A.; Stegeman, Coen A.; Westman, Kerstin W.; van der Woude, Fokko J.; van Wijngaarden, Robert A. F. de Lind; Pusey, Charles D.

    Systemic vasculitis associated with autoantibodies to neutrophil cytoplasmic antigens (ANCA) is the most frequent cause of rapidly progressive glomerulonephritis. Renal failure at presentation carries an increased risk for ESRD and death despite immunosuppressive therapy. This study investigated

  19. Vasculites dos médios e grandes vasos Medium and large vessel vasculitis

    Directory of Open Access Journals (Sweden)

    Hebert Roberto Clivati Brandt

    2009-02-01

    Full Text Available As vasculites são constituídas por um grande grupo de síndromes caracterizadas por inflamação e necrose da parede dos vasos sangüíneos, resultando em estreitamento ou oclusão do lúmen. A distribuição dos vasos envolvidos varia consideravelmente e serve de base para a classificação das síndromes vasculíticas: grandes vasos (arterite de Takayasu, arterite temporal; pequenos e médios vasos (poliarterite nodosa, síndrome de Churg-Strauss, granulomatose de Wegener, vasculites nas doenças reumáticas; e pequenos vasos (vasculite de hipersensibilidade, púrpura de Henoch-Schonlein, poliangeíte microscópica, crioglobulinemia. Este artigo revisa as vasculites dos médios e grandes vasos e opções terapêuticas atuais; além de oferecer orientação sobre abordagem diagnóstica e tratamento dos pacientes com suspeita de vasculite cutânea.Vasculitis comprises a broad group of syndromes characterized by inflammation and necrosis on the walls of blood vessels, resulting in narrowing or occlusion of the lumen. The distribution of involved blood vessel varies considerably and serves as the basis for one classification of vasculitic syndromes: large vessels (Takayasu arteritis, giant cell arteritis; medium and small muscular arteries (polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, vasculitis in rheumatic diseases; and small vessels ("hypersensitivity" vasculitis, Henoch-Schonlein purpura, microscopic polyangiitis, cryoglobulinemia. This article reviews medium and large vessel vasculitis and current treatment options; it also presents a comprehensive approach to diagnosing and treating patients with suspected cutaneous vasculitis.

  20. Annular lesions of cutaneous sarcoidosis with granulomatous vasculitis.

    Science.gov (United States)

    Mizuno, Kana; Nguyen, Chuyen Thi Hong; Ueda-Hayakawa, Ikuko; Okamoto, Hiroyuki

    2017-05-01

    Sarcoidosis is known to be involved in diseases with vasculitis as sarcoid vasculitis. However, vasculitis in cutaneous sarcoidal lesions is extremely rare. Here we describe a case of sarcoidosis with multiple annular skin lesions with granulomatous vasculitis. A 62-year-old female was diagnosed with sarcoidosis by chest-abdominal computed tomographic examination and laboratory tests. The skin lesions had appeared on her lower limbs 2 years before. Physical examination showed multiple infiltrated annular eruptions on the lower extremities. A skin biopsy of an area of erythema showed multiple non-caseating epithelioid cell granulomas in the dermis and subcutaneous fat and granulomatous vasculitis with fibrinoid degeneration in the subcutaneous fat. There are two types of vasculitis in sarcoidosis: leukocytoclastic and granulomatous vasculitis. Ulcers and livedo were more common in granulomatous vasculitis than in leukocytoclastic vasculitis. The present case had unique annular skin lesions of sarcoidosis with granulomatous vasculitis. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. Randomized Trial of C5a Receptor Inhibitor Avacopan in ANCA-Associated Vasculitis.

    Science.gov (United States)

    Jayne, David R W; Bruchfeld, Annette N; Harper, Lorraine; Schaier, Matthias; Venning, Michael C; Hamilton, Patrick; Burst, Volker; Grundmann, Franziska; Jadoul, Michel; Szombati, István; Tesař, Vladimír; Segelmark, Mårten; Potarca, Antonia; Schall, Thomas J; Bekker, Pirow

    2017-09-01

    Alternative C activation is involved in the pathogenesis of ANCA-associated vasculitis. However, glucocorticoids used as treatment contribute to the morbidity and mortality of vasculitis. We determined whether avacopan (CCX168), an orally administered, selective C5a receptor inhibitor, could replace oral glucocorticoids without compromising efficacy. In this randomized, placebo-controlled trial, adults with newly diagnosed or relapsing vasculitis received placebo plus prednisone starting at 60 mg daily (control group), avacopan (30 mg, twice daily) plus reduced-dose prednisone (20 mg daily), or avacopan (30 mg, twice daily) without prednisone. All patients received cyclophosphamide or rituximab. The primary efficacy measure was the proportion of patients achieving a ≥50% reduction in Birmingham Vasculitis Activity Score by week 12 and no worsening in any body system. We enrolled 67 patients, 23 in the control and 22 in each of the avacopan groups. Clinical response at week 12 was achieved in 14 of 20 (70.0%) control patients, 19 of 22 (86.4%) patients in the avacopan plus reduced-dose prednisone group (difference from control 16.4%; two-sided 90% confidence limit, -4.3% to 37.1%; P=0.002 for noninferiority), and 17 of 21 (81.0%) patients in the avacopan without prednisone group (difference from control 11.0%; two-sided 90% confidence limit, -11.0% to 32.9%; P=0.01 for noninferiority). Adverse events occurred in 21 of 23 (91%) control patients, 19 of 22 (86%) patients in the avacopan plus reduced-dose prednisone group, and 21 of 22 (96%) patients in the avacopan without prednisone group. In conclusion, C5a receptor inhibition with avacopan was effective in replacing high-dose glucocorticoids in treating vasculitis. Copyright © 2017 by the American Society of Nephrology.

  2. Ischemic retinal vasculitis in an 18-year-old man with chickenpox infection

    Directory of Open Access Journals (Sweden)

    Poonyathalang A,Sukavatcharin S

    2014-02-01

    Full Text Available Anuchit Poonyathalang, Somsiri Sukavatcharin, Tharikarn Sujirakul Department of Ophthalmology, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand Abstract: Ocular involvement after primary infection with varicella zoster virus is very rare. We report a case of a healthy 18-year-old man who presented with unilateral ischemic retinal vasculitis 10 days after the onset of chickenpox. He developed acute severe visual loss and a relative afferent pupillary defect in his right eye. Fundus imaging, optical coherence tomography, fundus fluorescence angiography, and electrophysiologic studies confirmed the diagnosis of retinal vasculitis, which led to generalized retinal ischemia. Although aggressive treatment with systemic steroids and antiviral drugs was administered, a poor visual outcome still resulted. Keywords: ischemic retinal vasculitis, chickenpox, varicella zoster virus

  3. Deferasirox-induced urticarial vasculitis in a patient with myelodysplastic syndrome.

    Science.gov (United States)

    Polat, Asude Kara; Belli, Asli Akin; Karakus, Volkan; Dere, Yelda

    2017-01-01

    Deferasirox is an iron chelator agent used in the treatment of diseases with iron overload, such as thalassemia and myelodysplastic syndrome. Although the majority of adverse reactions of deferasirox involve gastrointestinal symptoms and increase in serum creatinine and transaminases, skin rashes, such as maculopapular and urticarial eruptions, have also been reported. This study reports a case of myelodysplastic syndrome with urticarial vasculitis due to deferasirox therapy. Drug eruption was been confirmed by means of a challenge test, together with histopathological and clinical findings. To the best of our knowledge, we report the first case of deferasirox-induced urticarial vasculitis. Physicians should be aware of the possibility of urticarial vasculitis on deferasirox therapy and the fact that the discontinuation of the drug generally results in improvement.

  4. Long term azathioprine maintenance therapy in ANCA-associated vasculitis: combined results of long-term follow-up data.

    Science.gov (United States)

    de Joode, Anoek A E; Sanders, Jan Stephan F; Puéchal, Xavier; Guillevin, Loic P; Hiemstra, Thomas F; Flossmann, Oliver; Rasmussen, Nils; Westman, Kerstin; Jayne, David R; Stegeman, Coen A

    2017-11-01

    We studied whether in ANCA-associated vasculitis patients, duration of AZA maintenance influenced relapse rate during long-term follow-up. Three hundred and eighty newly diagnosed ANCA-associated vasculitis patients from six European multicentre studies treated with AZA maintenance were included; 58% were male, median age at diagnosis 59.4 years (interquartile range: 48.3-68.2 years); granulomatosis with polyangiitis, n = 236; microscopic polyangiitis, n = 132; or renal limited vasculitis, n = 12. Patients were grouped according to the duration of AZA maintenance after remission induction: ⩽18 months, ⩽24 months, ⩽36 months, ⩽48 months or > 48 months. Primary outcome was relapse-free survival at 60 months. During follow-up, 84 first relapses occurred during AZA-maintenance therapy (1 relapse per 117 patient months) and 71 after withdrawal of AZA (1 relapse/113 months). During the first 12 months after withdrawal, 20 relapses occurred (1 relapse/119 months) and 29 relapses >12 months after withdrawal (1 relapse/186 months). Relapse-free survival at 60 months was 65.3% for patients receiving AZA maintenance >18 months after diagnosis vs 55% for those who discontinued maintenance ⩽18 months (P = 0.11). Relapse-free survival was associated with induction therapy (i.v. vs oral) and ANCA specificity (PR3-ANCA vs MPO-ANCA/negative). Post hoc analysis of combined trial data suggest that stopping AZA maintenance therapy does not lead to a significant increase in relapse rate and AZA maintenance for more than 18 months after diagnosis does not significantly influence relapse-free survival. ANCA specificity has more effect on relapse-free survival than duration of maintenance therapy and should be used to tailor therapy individually.

  5. Idiopathic hypocomplementemic urticarial vasculitis-linked neuropathy.

    Science.gov (United States)

    Filosto, Massimiliano; Cavallaro, Tiziana; Pasolini, Giorgio; Broglio, Laura; Tentorio, Marta; Cotelli, Mariasofia; Ferrari, Sergio; Padovani, Alessandro

    2009-09-15

    Hypocomplementemic urticarial vasculitis (HUV) is a rare form of cutaneous small-vessel vasculitis characterized by recurrent episodes of urticaria and painful, tender, burning or itchy skin lesions, often associated with extracutaneous involvement but usually with no significant peripheral nerve damage. We describe a patient with an HUV of undetermined cause that developed a progressive multifocal sensory neuropathy whose symptoms were temporarily relieved by intravenous immunoglobulin treatment. Sural nerve biopsy showed asymmetrical multifocal nerve fiber loss and axon degeneration in nerve fascicles, a picture suggestive of ischemic damage as a likely result of a vasculitic process. We point out that an axonal neuropathy may complicate idiopathic HUV and suggest looking for peripheral nerve involvement in HUV patients.

  6. Therapeutic advances in the treatment of vasculitis

    OpenAIRE

    Eleftheriou, D.; P.A. Brogan

    2016-01-01

    Considerable therapeutic advances for the treatment of vasculitis of the young have been made in the past 10 years, including the development of outcome measures that facilitate clinical trial design. Notably, these include: a recognition that some patients with Kawasaki Disease require corticosteroids as primary treatment combined with IVIG; implementation of rare disease trial design for polyarteritis nodosa to deliver the first randomised controlled trial for children; first clinical trial...

  7. Warfarin-induced leukocytoclastic vasculitis and proteinuria

    Directory of Open Access Journals (Sweden)

    Khalid Jumean

    2016-01-01

    Full Text Available Warfarin is typically prescribed for patients with thromboembolic diseases and atrial fibrillation. In addition to the complications of bleeding, allergic skin reaction is one of its rare adverse effects. We herein report a case of a 79 year old male patient with leukocytoclastic vasculitis and proteinuria secondary to warfarin. The warfarin was discontinued and oral prednisone therapy was initiated. The cutaneous lesions and the proteinuria resolved thereafter.

  8. Retinal vasculitis revealing immunoglobulin G subclass deficiency.

    Science.gov (United States)

    Hassani, Rachid Tahiri Joutei; Rousseau, Antoine; de Monchy, Ivan; el Sanharawi, Mohamed; Gendron, Gael; Barreau, Emmanuel; Goujard, Cécile; Labetoulle, Marc

    2013-01-01

    Immunoglobulin G (IgG) subclass deficiency is a rare primary immunodeficiency syndrome characterized by recurrent infections and autoimmune disorders. However, there have been no reports of ocular involvement, either inflammatory or infectious, in association with IgG subclass deficiency. The authors report the first case of retinal vasculitis that led to the diagnosis of IgG subclass deficiency, in a patient with a history of inflammatory bowel disease and recurrent infections of previously unknown origin.

  9. [Spontaneous achilles tendon rupture in granulomatous vasculitis].

    Science.gov (United States)

    Benthien, Jan Philipp; Delling, G; Rüther, W

    2003-08-01

    A 66-year old patient sustained a non-traumatic rupture of her left achilles tendon. She suffered from Sjögren's syndrome which occurred in conjunction with a systemic vasculitis, and recurrent episcleritis. The combination of Sjögren's syndrome and systemic vasculitis is well known. Subsequently, she was treated with high-dose systemic steroids over a period of 2 years. In order to reduce the amount of steroids due to preexisting severe osteoporosis and thoracic vertebral fractures, her medication was changed to cyclophosphamide shortly before her injury. Intraoperatively, a granuloma was discovered at the site of the rupture. This granuloma had infiltrated most of the achilles tendon at this site and virtually replaced viable tendon tissue. Originally, the rupture was supposedly due to the high dose steroids. This theory had to be revised according to the intraoperative findings. Following excision of the granuloma and operative treatment of the achilles tendon rupture, the continuity of the tendon could be completely restored. A MRI scan 3 months after the procedure demonstrated a completely healed Achilles tendon. Spontaneous achilles tendon rupture due to a granuloma in patients with vasculitis seems to be a rare event. However, tendon ruptures in combination with systemic lupus erythematodes have been described. Mostly, these events are attributed to long term application of steroids. Spontaneous rupture in combination with high dose treatment of steroids seems to be an underestimated problem.

  10. A classic mimicker of systemic vasculitis.

    Science.gov (United States)

    Moreno-Ariño, Marc; Ortiz-Santamaria, Vera; Deudero Infante, Aída; Ayats Delgado, Montserrat; Novell Teixidó, Francesc

    2016-01-01

    Embolic and constitutional manifestations of intracavitary cardiac tumors are included within the classic mimickers of systemic vasculitis, especially in those in which there are no cardiac manifestations. We present a case report of atrial myxoma in which the patient only presented systemic symptoms and in whom an initial diagnostic approach of systemic vasculitis was made. We also performed a literature search of the cases described. A case report of atrial myxoma with atypical presentation manifested as a systemic disease with no concomitant cardiac symptoms is described. The case report is discussed and 11 cases of atrial myxoma pseudovasculitis described in the literature are reviewed, emphasizing their similarities and differences. Constitutional symptoms and cutaneous manifestations were the most common. Most of the cases showed partial response to glucococorticosteroid treatment, reinforcing the theory of the inflammatory role in its pathogenesis. Mean delayed time to diagnosis was 12.27 months. Atrial myxoma is a systemic vasculitis mimicker, this being difficult to diagnose in the absence of cardiac manifestations. This delay in diagnosis entails serious complications. Copyright © 2015 Elsevier España, S.L.U. y Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  11. Hypocomplementemic urticarial vasculitis in mixed connective tissue disease.

    Science.gov (United States)

    Calistru, Ana Maria; Lisboa, Carmen; Cruz, Maria João; Delgado, Luis; Poças, Licínio; Azevedo, Filomena

    2010-12-15

    Urticarial vasculitis is characterized clinically by urticaria-like skin lesions and histologically by leukocytoclastic vasculitis. It may be idiopathic or associated with various conditions such as infections, hematologic disorders, drugs, and connective tissue diseases, primarily systemic lupus erythematosus; an association with mixed connective tissue disease (MCTD) has rarely been reported. We present a case of hypocomplementemic urticarial vasculitis in a patient with MCTD that responded to hydroxychloroquine after a period of corticosteroid dependence.

  12. Hypocomplementemic urticarial vasculitis complicated with diffuse alveolar hemorrhage: a case report and review of literature

    Directory of Open Access Journals (Sweden)

    Paola Gnerre

    2014-03-01

    Full Text Available Urticarial vasculitis is a small-vessel vasculitis characterized clinically by urticaria and microscopically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis syndrome is a rare subtype of urticarial vasculitis with organ involvement and persistent, acquired hypocomplementemia. Pulmonary involvement, usually seen as diffuse alveolar damage, is an uncommon complication and a significant cause of morbidity and mortality. Here we present a rare case of hypocomplementemic urticarial vasculitis syndrome complicated by diffuse alveolar damage and describe the beneficial effect of cyclophosphamide-dexamethasone.

  13. DNASE1L3 mutations in hypocomplementemic urticarial vasculitis syndrome.

    Science.gov (United States)

    Ozçakar, Z Birsin; Foster, Joseph; Diaz-Horta, Oscar; Kasapcopur, Ozgur; Fan, Yao-Shan; Yalçınkaya, Fatoş; Tekin, Mustafa

    2013-08-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is characterized by recurrent urticaria along with dermal vasculitis, arthritis, and glomerulonephritis. Systemic lupus erythematosus (SLE) develops in >50% of patients with HUVS, although the pathogenesis is unknown. The aim of this study was to identify the causative DNA mutations in 2 families with autosomal-recessive HUVS, in order to reveal the pathogenesis and facilitate the laboratory diagnosis. Autozygosity mapping was combined with whole-exome sequencing. In a family with 3 affected children, we identified a homozygous frameshift mutation, c.289_290delAC, in DNASE1L3. We subsequently identified another homozygous DNASE1L3 mutation leading to exon skipping, c.320+4delAGTA, in an unrelated family. The detected mutations led to loss of function, via either nonsense-mediated messenger RNA decay or abolished endonuclease activity, as demonstrated by a plasmid nicking assay. These results show that HUVS is caused by mutations in DNASE1L3, encoding an endonuclease that previously has been associated with SLE. Copyright © 2013 by the American College of Rheumatology.

  14. Renal Survival in Proteinase 3 and Myeloperoxidase ANCA-Associated Systemic Vasculitis

    NARCIS (Netherlands)

    de Joode, Anoek A. E.; Sanders, Jan Stephan; Stegeman, Coen A.

    2013-01-01

    Background and objectivesThis study evaluated predictors for patient and renal survival in patients with ANCA-associated vasculitis (AAV) with and without renal involvement.Design, setting, participants, & measurementsThere were 273 consecutive AAV patients from January 1990 until December 2007 who

  15. The incidence of vasculitis is increased in female stroke-prone ...

    African Journals Online (AJOL)

    Background: Vascular changes in hypertension share common characteristics with inflammatory wall injury. Since it is known that chronic inflammatory diseases are frequently more prevalent in females, this study tested the hypothesis that vasculitis would be more evident in female stroke-prone spontaneously hypertensive ...

  16. Varicella Zoster Virus and Large Vessel Vasculitis, the Absence of an Association

    Directory of Open Access Journals (Sweden)

    Gary W. Procop

    2017-06-01

    Full Text Available Objective: It is controversial whether microorganisms play a role in the pathogenesis of large and medium vessel vasculitides (eg, giant cell arteritis [GCA], Takayasu arteritis [TAK] and focal idiopathic aortitis [FIA]. Recent studies have reported the presence of Varicella Zoster Virus (VZV within formalin-fixed, paraffin-embedded temporal arteries and aortas of about three-quarters or more of patients with these conditions, and in a minority of controls. In a prospective study, we sought to confirm these findings using DNA extracted from vessels that were harvested under surgically aseptic conditions and snap frozen. Methods and Results: DNA samples extracted from 11 surgically sterile temporal arteries and 31 surgically sterile thoracic aortas were used in an attempt to identify the vessel-associated VZV genome. Two different validated PCR methods were used. Thirty-one thoracic aorta aneurysm specimens included biopsies from 8 patients with GCA, 2 from patients with TAK, 6 from patients with FIA, and 15 from patients without vasculitis, who had non-inflammatory aneurysms. Eleven temporal artery biopsies were collected from 5 patients with GCA and 6 controls. The presence of VZV was not identified in either the specimens from patients with large vessel vasculitis or from the controls. Conclusions: Using surgically sterile snap-frozen specimens, we were unable to confirm recent reports of the presence of VZV in either aortas or temporal arteries from patients with large vessel vasculitis or controls. Keywords: Aorta and temporal artery biopsies, Varicella Zoster Virus, Large Vessel Vasculitis

  17. Pathogenesis of ANCA-Associated Vasculitis, an Update

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    2011-01-01

    Clinical observations, including a report of neonatal vasculitis occurring in a child born from a mother with anti-neutrophil cytoplasmic antibody directed to myeloperoxidase (MPO-ANCA)-associated vasculitis, suggest a pathogenic role for ANCA. Such a role is supported by in vitro experimental data

  18. Paraneoplastic vasculitis associated to pelvic chondrosarcoma: a case report

    Directory of Open Access Journals (Sweden)

    Arvinius Camilla

    2016-01-01

    Full Text Available Vasculopathic syndromes have been associated with hematological and solid organ malignancies. The pathogenesis of these syndromes remains largely unknown and there are no biologic markers identified. Whether it is or is not a paraneoplastic syndrome is under discussion, the close temporal relationship of cancer and vasculitis suggests that these vasculitides are a paraneoplastic condition. We report a case of a 45-year-old female patient with pelvic chondrosarcoma who underwent surgical treatment and started to present visual loss, systemic inflammatory response syndrome (SRIS, cardiac insufficiency, hepatosplenomegaly, cholestasis as well as pulmonary bleeding suggesting a sarcoma-associated vasculitis. All antibodies were negative as in secondary vasculitis. After corticoideal therapy the vasculitis resolved and at 3-year follow-up the patient had not showed any further medical complications or recurrences of the vasculitis. The parallel evolution of the vasculitis and the solid tumor combined with the resolution of the vasculitis after corticotherapy enhances the likelihood of a paraneoplastic vasculitis associated with a chondrosarcoma according to literature review.

  19. Cutaneous vasculitis as a presenting manifestation of acute myeloid leukemia.

    Science.gov (United States)

    Jayachandran, Nambiar Veettil; Thomas, Joe; Chandrasekhara, Pradeep Kumar Shenoy; Kanchinadham, Suresh; Kadel, Jugal Kishore; Narsimulu, Gumdal

    2009-04-01

    One of the rare causes of secondary vasculitides is malignancy. Hematological malignancies produce secondary vasculitis more frequently than solid malignancies. Here in we report a case of acute myeloid leukemia presenting with anti-neutrophil cytoplasmic antibody-positive vasculitis. This case highlights the importance of looking for underlying malignancies, especially leukemias in patients presenting with features of systemic vasculitides.

  20. Persistent cryoglobulinemic vasculitis following successful treatment of hepatitis C virus.

    Science.gov (United States)

    Levine, James W; Gota, Carmen; Fessler, Barri J; Calabrese, Leonard H; Cooper, Sheldon M

    2005-06-01

    There is a well established link between type II mixed cryoglobulinemia (MC) and hepatitis C virus (HCV) infection, and HCV is believed to be the cause of cryoprotein formation and tissue deposition. Successful treatment of HCV infection has resulted in resolution of cryoglobulinemia and vasculitis. We describe 4 patients who had persistent MC and vasculitis despite successful eradication of HCV with antiviral therapy.

  1. Relationship between pressure and digital vasculitis in rheumatoid disease.

    OpenAIRE

    Edwards, J C

    1980-01-01

    The sites of 92 rheumatoid nailfold lesions were compared with sites of pressure as indicated by tissue blanching during gripping. A close correspondence between sites of blanching and sites of vasculitis was found, and this is discussed in relation to the possible role of vascular compression and blood stasis in the development of rheumatoid vasculitis.

  2. Retinal Vasculitis in Anti-Synthetase Syndrome.

    Science.gov (United States)

    Donovan, Christopher P; Pecen, Paula E; Baynes, Kimberly; Ehlers, Justis P; Srivastava, Sunil K

    2016-09-01

    A 31-year-old woman with a history of anti-synthetase syndrome-related myositis and interstitial lung disease presented with acute-onset blurry vision and rash on her hands and feet. Visual acuity was hand motion in her right eye and 20/40 in her left eye. Dilated fundus exam showed extensive retinal vasculitis, diffuse intraretinal hemorrhages, and subretinal fluid. Optical coherence tomography revealed significant macular thickening, and fluorescein angiography revealed vascular leakage with peripheral nonperfusion. Aggressive systemic immunosuppression was initiated, with gradual resolution of her disease during 8 months of follow-up. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:874-879.]. Copyright 2016, SLACK Incorporated.

  3. Urticarial vasculitis appearing in the progression of systemic sclerosis.

    Science.gov (United States)

    Kato, Yoko; Aoki, Mikako; Kawana, Seiji

    2006-11-01

    We report a case of urticarial vasculitis that appeared during the course of limited cutaneous systemic sclerosis. The urticarial lesions responded to systemic administration of prednisolone. After the appearance of urticarial vasculitis, the progression of scleroderma in distal sites of her elbow and knee joint became apparent. We consider this case to be consistent with limited cutaneous systemic sclerosis. The patient started treatment with prednisolone and her edema as well as scleroderma softened gradually. We analyzed, by immunohistochemistry, the number of tryptase-positive mast cells of this case in the lesions of urticarial vasculitis as well as systemic sclerosis. The number of tryptase-positive mast cells in the lesions of urticarial vasculitis as well as systemic sclerosis was significantly increased compared to normal skin (P urticarial vasculitis and systemic sclerosis as a common factor.

  4. Cryoglobulinemic vasculitis in a patient with CREST syndrome.

    Science.gov (United States)

    Hurst, Rebecca L; Berianu, Florentina; Ginsburg, William W; Klein, Christopher J; Englestad, Janean K; Kennelly, Kathleen D

    2014-10-01

    Cryoglobulinemic vasculitis is a rare entity. Although it has been reported in diffuse systemic sclerosis, it has not been reported in calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia (CREST) syndrome. We report a patient with cryoglobulinemic vasculitis with CREST syndrome who did not have typical clinical features of vasculitis. This 58-year-old woman presented with mild generalized weakness and a diagnosis of CREST syndrome, which included Raynaud's syndrome, dysphagia and telangiectasias. She was positive for serum cryoglobulins, which led to a sural nerve biopsy. The biopsy results were consistent with cryoglobulinemic vasculitis. Cryoglobulinemic vasculitis has not been previously reported in CREST syndrome to our knowledge. Additionally, the patient also had limited clinical symptoms. Our patient displays the importance of checking for cryoglobulins and obtaining a nerve biopsy when the serum is positive. Both of these diagnostic tests were integral for directing appropriate treatment for this patient. Copyright © 2014 Elsevier Ltd. All rights reserved.

  5. Aerococcus viridans infection presenting as cutaneous vasculitis in an immunocompetent patient.

    Science.gov (United States)

    Parrey, Ashaq Hussain; Sofi, Fayaz; Ahmad, Mushtaq; Kuchay, Abid

    2016-01-01

    Aerococcus viridans organisms are Gram-positive cocci that are widely distributed in hospital environments and room air. These bacteria have infrequently been encountered as human pathogens causing bacteremia, endocarditis and urinary tract infections. The significance of these bacteria may be overlooked due to their fastidious growth, and they are often confused with other strains of streptococci or staphylococci. We present a case of Aerococcus viridans manifesting as cutaneous vasculitis in an immunocompetent patient. A 30-year-old female patient was admitted to hospital after two weeks history of fever, chills and papular rash over the limbs and trunk. The clinical diagnosis of vasculitis was made. Investigations revealed elevated leucocytosis (21.7 × 10(9)/l) with 81% of neutrophils, and an elevated erythrocyte sedimentation rate or 60 mm/h. Serum anti-neutrophil cytoplasmic antibodies (ANCAs) were not found. Blood culture showed growth of Aerococcus viridans. Histopathological assessment of skin biopsy revealed cutaneous vasculitis. To date, no clinical case report of this kind has been reported implicating Aerococcus viridans in cutaneous vasculitis. Increased awareness and more studies of this genus should lead to the identification of its potential role in human infections.

  6. [A rapidly progressive orbital tumor in a seven-year-old girl revealing a primitive systemic vasculitis].

    Science.gov (United States)

    Picard, Cécile; Borgnat, Florent; Chastang, Marie-Caroline; Fleury, Aude; Guibaud, Laurent; Dijoud, Frédérique

    2014-12-01

    Orbital tumor lesions in pediatric population encompass a wide range of pathological processes, which are very different in etiology and prognosis. They usually require an emergency in histological diagnosis because of their quick growth. Beside malignant and benign tumors, the inflammatory pseudo-tumors group included dysimmune orbital involvement secondary to systemic vasculitis such as granulomatosis with polyangiitis (ex-Wegener's granulomatosis). We report the case of a seven-year-old girl suffering from a severe primitive ANCA vasculitis, revealed by an orbital mass. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  7. International Study Group Progress Report

    Energy Technology Data Exchange (ETDEWEB)

    Raubenheimer, Tor O

    2000-07-18

    The focus of the ISG work was on advancing the accelerator design and supporting technologies. This is a complex process which involves a close interaction between theoretical analysis of the collider design and R and D progress on hardware components. The sequence of efforts took place roughly in the following order: (1) Optimization of the collider parameters and definition of system and subsystem requirements, (2) Identification of design strategies and options, and (3) Development of specific technologies to achieve these requirements. Development and testing of the required components, and R and D on manufacturing techniques have been important activities of the ISG. Experiments at the major test facilities such as the ATF at KEK and ASSET at SLAC have also played a significant role in the ISG studies.

  8. Circulating soluble adhesion molecules in ANCA-associated vasculitis.

    Science.gov (United States)

    Ara, J; Mirapeix, E; Arrizabalaga, P; Rodriguez, R; Ascaso, C; Abellana, R; Font, J; Darnell, A

    2001-02-01

    To evaluate whether changes in concentrations of soluble (s) E-selectin, sP-selectin, sL-selectin, intercellular adhesion molecule 1 (sICAM-1), and vascular cell adhesion molecule 1 (sVCAM-1) reflect disease activity in patients with ANCA-associated vasculitis and whether serum levels of these adhesion molecules are related to the degree of renal failure in patients with chronic renal failure (CRF). A sandwich ELISA was used to measure these soluble adhesion molecules in (i) sera from 20 patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (10 patients with Wegener's granulomatosis (WG) and 10 patients with microscopic polyangiitis (MPA)), obtained at the time of diagnosis and during the remission period; (ii) sera from 40 patients with CRF not undergoing haemodialysis. At the time of diagnosis, serum levels of sE-selectin, sICAM-1 and sVCAM-1 (88+/-42 ng/ml, 437+/-184 ng/ml, 1720+/-1174 ng/ml respectively) were significantly higher in patients with ANCA-associated vasculitis than in healthy controls (P<0.0001, P=0.002 and P=0.001 respectively). Serum sP-selectin values did not differ from those obtained in normal donors. In contrast, sL-selectin levels (940+/-349 ng/ml) were significantly lower in patients than those recorded in healthy controls (P<0.0001). A significant decrease in concentrations of sE-selectin, sP-selectin, sICAM-1, and sVCAM-1 was observed between active and remission phases (P<0.0001, P=0.002, P=0.001 and P=0.001 respectively). No significant differences were observed in sL-selectin levels between active and remission phases. sL-selectin concentrations (802+/-306 ng/ml) during the remission phase remained lower than those observed in healthy controls (P<0.0001). No correlation was observed between serum creatinine and sE-selectin, sP-selectin, sICAM-1 and sVCAM-1 in patients of the CRF group. A slight negative correlation was established between creatinine and sL-selectin concentration. Increased serum levels of s

  9. Anti-proteinase 3 anti-neutrophil cytoplasm autoantibodies recapitulate systemic vasculitis in mice with a humanized immune system.

    LENUS (Irish Health Repository)

    Little, Mark A

    2012-01-01

    Evidence is lacking for direct pathogenicity of human anti-proteinase-3 (PR3) antibodies in development of systemic vasculitis and granulomatosis with polyangiitis (GPA, Wegener\\'s granulomatosis). Progress in study of these antibodies in rodents has been hampered by lack of PR3 expression on murine neutrophils, and by different Fc-receptor affinities for IgG across species. Therefore, we tested whether human anti-PR3 antibodies can induce acute vasculitis in mice with a human immune system. Chimeric mice were generated by injecting human haematopoietic stem cells into irradiated NOD-scid-IL2Rγ⁻\\/⁻ mice. Matched chimera mice were treated with human IgG from patients with: anti-PR3 positive renal and lung vasculitis; patients with non-vasculitic renal disease; or healthy controls. Six-days later, 39% of anti-PR3 treated mice had haematuria, compared with none of controls. There was punctate bleeding on the surface of lungs of anti-PR3 treated animals, with histological evidence of vasculitis and haemorrhage. Anti-PR3 treated mice had mild pauci-immune proliferative glomerulonephritis, with infiltration of human and mouse leukocytes. In 3 mice (17%) more severe glomerular injury was present. There were no glomerular changes in controls. Human IgG from patients with anti-PR3 autoantibodies is therefore pathogenic. This model of anti-PR3 antibody-mediated vasculitis may be useful in dissecting mechanisms of microvascular injury.

  10. Anti-proteinase 3 anti-neutrophil cytoplasm autoantibodies recapitulate systemic vasculitis in mice with a humanized immune system.

    Directory of Open Access Journals (Sweden)

    Mark A Little

    Full Text Available Evidence is lacking for direct pathogenicity of human anti-proteinase-3 (PR3 antibodies in development of systemic vasculitis and granulomatosis with polyangiitis (GPA, Wegener's granulomatosis. Progress in study of these antibodies in rodents has been hampered by lack of PR3 expression on murine neutrophils, and by different Fc-receptor affinities for IgG across species. Therefore, we tested whether human anti-PR3 antibodies can induce acute vasculitis in mice with a human immune system. Chimeric mice were generated by injecting human haematopoietic stem cells into irradiated NOD-scid-IL2Rγ⁻/⁻ mice. Matched chimera mice were treated with human IgG from patients with: anti-PR3 positive renal and lung vasculitis; patients with non-vasculitic renal disease; or healthy controls. Six-days later, 39% of anti-PR3 treated mice had haematuria, compared with none of controls. There was punctate bleeding on the surface of lungs of anti-PR3 treated animals, with histological evidence of vasculitis and haemorrhage. Anti-PR3 treated mice had mild pauci-immune proliferative glomerulonephritis, with infiltration of human and mouse leukocytes. In 3 mice (17% more severe glomerular injury was present. There were no glomerular changes in controls. Human IgG from patients with anti-PR3 autoantibodies is therefore pathogenic. This model of anti-PR3 antibody-mediated vasculitis may be useful in dissecting mechanisms of microvascular injury.

  11. Retinal vasculitis in a patient with abdominal tuberculosis

    Science.gov (United States)

    Kannan, Balaji; Govindarajan, Kummararaj; Kummararaj, Sherin; Balaji, Vijayalakshmi; Natarajapillai, Venugopal

    2010-01-01

    Tuberculosis (TB) is one of the most common systemic diseases in India. Intraocular TB is however, rare. Retinal vasculitis is a relatively rare manifestion of intraocular TB. We report a case of bilateral retinal vasculitis in a 19-year-old girl with abdominal tuberculosis. The patient responded well to anti-TB treatment along with a short course of low dose oral steroids. Vision in her right eye however remained compromised due to residual maculopathy. This is the first report of bilateral retinal vasculitis due to colonic TB. PMID:21217901

  12. Lepra reaction with lucio phenomenon mimicking cutaneous vasculitis.

    Science.gov (United States)

    Misra, Durga Prasanna; Parida, Jyoti Ranjan; Chowdhury, Abhra Chandra; Pani, Krushna Chandra; Kumari, Niraj; Krishnani, Narendra; Agarwal, Vikas

    2014-01-01

    Leprosy is a disease typically found in the tropics. Patients with leprosy can have varying presentation with constitutional symptoms, joint pains, skin nodules, and rarely a vasculitis-like picture with skin ulcers and neuropathy. We present a young lady who presented with the rare manifestation of skin infarcts mimicking cutaneous vasculitis, diagnosed on histopathology to have Lucio phenomenon on a background of lepromatous leprosy. With increasing migration and widespread use of biologic response modifiers, clinicians all over the world need to be aware of various presentations of leprosy as well as needing to keep an open mind while considering the differential diagnoses of vasculitis.

  13. Lepra Reaction with Lucio Phenomenon Mimicking Cutaneous Vasculitis

    Directory of Open Access Journals (Sweden)

    Durga Prasanna Misra

    2014-01-01

    Full Text Available Leprosy is a disease typically found in the tropics. Patients with leprosy can have varying presentation with constitutional symptoms, joint pains, skin nodules, and rarely a vasculitis-like picture with skin ulcers and neuropathy. We present a young lady who presented with the rare manifestation of skin infarcts mimicking cutaneous vasculitis, diagnosed on histopathology to have Lucio phenomenon on a background of lepromatous leprosy. With increasing migration and widespread use of biologic response modifiers, clinicians all over the world need to be aware of various presentations of leprosy as well as needing to keep an open mind while considering the differential diagnoses of vasculitis.

  14. Biomarkers of IgA vasculitis nephritis in children

    Science.gov (United States)

    Pillebout, Evangeline; Jamin, Agnès; Ayari, Hamza; Housset, Pierre; Pierre, Melissa; Sauvaget, Virginia; Viglietti, Denis; Deschenes, Georges

    2017-01-01

    Henoch–Schönlein purpura is a systemic vasculitis characterized by IgA deposits, which target the skin, joints, and kidneys, among other organs. In children, prognosis is often good but little is known about biomarkers of pediatric nephritis. We hypothesized that biological markers, including cytokines, immunoglobulins, IgA-immune complexes, IgA glycosylation and neutrophil gelatinase-associated lipocalin (NGAL), may discriminate IgA vasculitis (IgAV) pediatric patients with renal involvement from those without renal involvement. Fifty children at the time of IgAV rash between 2010 and 2015 were prospectively enrolled and compared to 21 controls. All patients were assessed for clinical and biological parameters at the time of diagnosis, including the levels of cytokines, immunoglobulins, immune complexes, IgA glycosylation and NGAL in serum and urine. Among IgAV patients, 33 patients exhibited nephritis (IgAV-N) and 17 children were without nephritis (IgAV-woN). The serum level of galactose-deficient (Gd)-IgA1 (pmarkers, urinary IgA and IgM had the highest AUC (0.86 and 0.87 respectively, p<0.0001). This prospective cohort study furthers our understanding of the pathophysiology of IgAV. We identified biomarkers that are able to distinguish patients initially with or without nephritis. To conclude, serum Gd-IgA1 and urinary IgA, IgG, IgM, IL-6, IL-8, IL-10, and IgA-IgG and IgA-sCD89 complexes could identify IgAV pediatric patients with renal involvement at the time of diagnosis. PMID:29190714

  15. Vasculitis inducida por metimazol: Reporte de caso

    Directory of Open Access Journals (Sweden)

    Miguel Pinto

    2011-07-01

    Full Text Available Se reporta el caso de una paciente con enfermedad de Graves, que presentó vasculitis asociada al uso de metimazol. Mujer de 14 años, que acudió a consulta por presentar intolerancia al calor, tremor distal y palpitaciones. El examen físico mostró bocio difuso, y el perfil tiroideo, TSH suprimida y hormonas tiroideas elevadas. Los anticuerpos antiperoxidasa tiroidea fueron positivos. Se inició tratamiento con metimazol y beta bloqueadores. Después de 20 días, la paciente regresó por presentar malestar general, fiebre, poliartralgia, lesiones cutáneas maculopapulares y edema de miembros inferiores. Los anticuerpos antinucleares fueron negativos y los anticuerpos anticitoplasma de los neutrófilos (ANCA, positivos. Se suspendió el metimazol y se inició prednisona. Después de 10 días de tratamiento, las molestias desaparecieron y la paciente recibió I 131.Las vasculitis asociadas al uso de tionamidas son poco frecuentes, no dependen de la dosis y están asociadas a la presencia de anticuerpos tipo ANCA. Clásicamente, afectan a los vasos pequeños de la piel; sin embargo, también pueden afectar los riñones y pulmones. El cuadro clínico se caracteriza por artralgias y mialgias. En algunos casos puede ocurrir insuficiencia renal de grado variable. En la mayoría de casos, el cuadro remite con la suspensión de la droga; pero, en algunos se requiere el uso de glucocorticoides o inmunosupresores.(Rev Med Hered 2011;22:147-150.

  16. Refractory disease in antineutrophil cytoplasmic antibodies associated vasculitis

    NARCIS (Netherlands)

    Rutgers, Abraham; Kallenberg, Cornelis

    Purpose of review Induction treatment of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) is not always successful and nonresponding patients are considered refractory. Recent findings Refractory disease should be subdefined to the treatment that was received.

  17. A Unique Case of Systemic Lupus Erythematosus Pelvic Vasculitis

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    Pamela Traisak

    2016-01-01

    Full Text Available The clinical presentation of Systemic Lupus Erythematosus (SLE is diverse and vasculitis can be a potential manifestation. Cutaneous lesions involving small vessels are the most frequent presentation. However, medium and large vessel vasculitis may present with life-threatening visceral manifestations. We present a unique case of pelvic vasculitis mimicking a pelvic mass as an initial presentation of SLE. There are case reports of systemic vasculitis involving the female genital tract with giant cell arteritis (GCA, polyarteritis nodosa (PAN, and granulomatous with polyangiitis and microscopic polyangiitis (GPA/MPA, among others, but only a few cases attributed to SLE. Awareness of this condition and a prompt diagnosis are warranted as this is a severe and potentially life-threatening condition.

  18. Genetically distinct subsets within ANCA-associated vasculitis

    DEFF Research Database (Denmark)

    Lyons, Paul A; Rayner, Tim F; Trivedi, Sapna

    2012-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single...

  19. Long-term patient survival in ANCA-associated vasculitis

    NARCIS (Netherlands)

    Flossmann, Oliver; Berden, Annelies; de Groot, Kirsten; Hagen, Chris; Harper, Lorraine; Heijl, Caroline; Hoglund, Peter; Jayne, David; Luqmani, Raashid; Mahr, Alfred; Mukhtyar, Chetan; Pusey, Charles; Rasmussen, Niels; Stegeman, Coen; Walsh, Michael; Westman, Kerstin

    Background Wegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain. Objective

  20. Genetically Distinct Subsets within ANCA-Associated Vasculitis

    NARCIS (Netherlands)

    Lyons, Paul A.; Rayner, Tim F.; Trivedi, Sapna; Holle, Julia U.; Watts, Richard A.; Jayne, David R. W.; Baslund, Bo; Brenchley, Paul; Bruchfeld, Annette; Chaudhry, Afzal N.; Tervaert, Jan Willem Cohen; Deloukas, Panos; Feighery, Conleth; Gross, Wolfgang L.; Guillevin, Loic; Gunnarsson, Iva; Harper, Lorraine; Hruskova, Zdenka; Little, Mark A.; Martorana, Davide; Neumann, Thomas; Ohlsson, Sophie; Padmanabhan, Sandosh; Pusey, Charles D.; Salama, Alan D.; Sanders, Jan-Stephan F.; Savage, Caroline O.; Segelmark, Mrten; Stegeman, Coen A.; Tesar, Vladimir; Vaglio, Augusto; Wieczorek, Stefan; Wilde, Benjamin; Zwerina, Jochen; Rees, Andrew J.; Clayton, David G.; Smith, Kenneth G. C.

    2012-01-01

    BACKGROUND Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it

  1. The hairy-print for levamisole-induced vasculitis.

    Science.gov (United States)

    Lazareth, Hélène; Peytavin, Gilles; Polivka, Laura; Dupin, Nicolas

    2012-08-08

    Levamisole-induced vasculitis is a well-characterised antineutrophil cytoplasm antibodies (ANCA)-positive vasculitis in cocaine abuser patients. However, due to the short half-life of levamisole in serum and urine, the causal role of levamisole is not established. Here we report the detection of both levamisole and cocaine in hair samples of a patient who presented with an ANCA-positive vasculitis. The higher concentration of levamisole in proximal sample of the hair confirms that the patient abused of cocaine added with levamisole in the days preceding the development of skin lesions. Although a direct causative role has not been established, our report strongly suggests that levamisole may have triggered vasculitis in this case.

  2. Hypocomplementaemic urticarial vasculitis syndrome: a mimicker of systemic lupus erythematosus.

    Science.gov (United States)

    Roy, Krishnendu; Talukdar, Arunansu; Kumar, Bappaditya; Sarkar, Sumanta

    2013-05-22

    A middle aged female patient presented with generalised palpable purpura associated with intense pruritus along with subconjunctival haemorrhage and orbital inflammation. There was extensive dermographism. Other systemic examinations were within normal limits. Haematological profile was normal except raised D-dimer. Skin biopsy revealed the presence of leucocytoclastic vasculitis. Antinuclear antibody was positive in a titre of 1 : 160, but antidouble-stranded DNA was negative. Urine examination revealed haematuria and proteinuria. Complement C3, C4 and C1q levels were decreased with the presence of anti-C1q antibody. There was a diagnostic dilemma between systemic lupus erythematosus and hypocomplementaemic urticarial vasculitis syndrome. However, as the patient did not fulfil the American College of Rheumatology criteria for systemic lupus erythematosus, but fulfilled all the criteria for hypocomplementaemic urticarial vasculitis syndrome, the case was finally diagnosed as hypocomplementaemic urticarial vasculitis syndrome and treated accordingly with favourable outcome.

  3. Complement system activation in ANCA vasculitis : A translational success story?

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.; Heeringa, Peter

    2015-01-01

    The ANCA-associated vasculitides (AAV) are characterized by pauci-immune necrotizing small to medium size vessel vasculitis frequently including necrotizing crescentric glomerulonephritis. Neutrophil activation by ANCA appears a primary pathogenic event. More recently, the complement system has been

  4. A Case of Tuberculous Granulomatous Panniculitis without Vasculitis

    Directory of Open Access Journals (Sweden)

    Yassaman Alipour Tehrany

    2015-07-01

    Full Text Available We report a case of tuberculous granulomatous panniculitis without vasculitis in an 87-year-old female patient with B-cell chronic lymphocytic leukaemia. One month after starting chemotherapy with chlorambucil and prednisone she presented superficial erythematous plaques on the anterior side of the left leg. Three weeks later erythematous painless deep nodules appeared on the left popliteal fossa and on the left thigh. Cutaneous biopsy revealed granulomatous panniculitis without caseation necrosis or vasculitis. Polymerase chain reaction for Mycobacterium tuberculosis revealed positivity in the skin. The final diagnosis was reactivation of latent tuberculosis (TB induced by deep immunosuppression associated with chemotherapy and haematological disease. Tuberculous granulomatous panniculitis without vasculitis is a rare presentation of cutaneous TB and may be part of the heterogeneous histopathologic spectrum of erythema induratum of Bazin (nodular vasculitis. Our case shows that the diagnosis of cutaneous TB requires the correlation of clinical findings with histopathology and microbiological tests.

  5. What Are the Signs and Symptoms of Vasculitis?

    Science.gov (United States)

    ... more likely to occur in people of Scandinavian origin, but it can affect people of any race. ... of polyarteritis nodosa seem to be linked to hepatitis B or C infections. Mostly Small Vessel Vasculitis ...

  6. Long-term patient survival in ANCA-associated vasculitis

    DEFF Research Database (Denmark)

    Flossmann, Oliver; Berden, Annelies; de Groot, Kirsten

    2011-01-01

    Wegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain.......Wegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain....

  7. Severe food-induced vasculitis in two children.

    Science.gov (United States)

    Businco, Luisa; Falconieri, Paola; Bellioni-Businco, Barbara; Bahna, Sami L

    2002-02-01

    Food-induced vasculitis seems to be rare and is considered by some as controversial. The reported cases in the literature are few and mostly on adult patients. Described in this report are two children with severe vasculitis caused by specific foods. They were diagnosed at two separate allergy centers that have a special interest in food allergies. Case 1 was an 8-year-old girl with a 9-month history of cutaneous vasculitis with large joints involvement. Case 2 was a 23-month-old girl with an 8-month history of multiple hospitalizations for recurrent acute severe cutaneous and mucous membrane vasculitis with large joints involvement. In both patients, skin biopsy showed leucocytoclastic vasculitis. In neither of the patients could the symptoms be attributed to drug intake, infection, autoimmunity, or other systemic disease. Case 1 had a moderately elevated serum total immunoglobulin E (IgE) level and strongly positive skin test and radioallergosorbent test (RAST) to cow's milk and hen's egg, both of which were proven to be the cause by elimination-challenge tests. Case 2 had a slightly elevated serum total IgE level, but negative skin tests to foods, including chocolate that was suspected by the mother. Avoidance of chocolate resulted in remission, except following accidental ingestion of cocoa-containing products. These findings support the few previous reports on food-induced vasculitis, an entity that seems rare but may be more common than currently realized.

  8. [Hypocomplementaemic urticarial vasculitis with bullous lesions and pericardial involvement].

    Science.gov (United States)

    Kervarrec, T; Binois, R; Bléchet, C; Estève, É

    2015-10-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare disease involving urticarial cutaneous vasculitis, hypocomplementaemia and systemic manifestations. Pericardial involvement occurs in very rare cases. We report a case of HUVS associated with specific pericarditis and bullous lesions. A 63-year-old woman consulted for chronic urticaria that had appeared ten months earlier. Her skin lesions were associated with weight loss of 10 kg, deterioration of respiratory function and abdominal pain. Leukocytoclastic vasculitis was seen in the skin biopsy sample. Hypocomplementaemia and anti C1q antibodies were present and a diagnosis of HUVS was made. During hospitalisation, extensive compressive pericardial effusion was identified, and histological examination of the biopsy revealed specific pericardial lymphocytic vasculitis. During follow-up, four episodes of infectious pneumonitis were noted. Bullous skin lesions were also observed. HUVS is a disease caused by an antibody against C1q complement responsible for urticarial lesions and vasculitis antibodies. To our knowledge, there have been only five reports in the literature of pericardial injury associated with HUVS. In our case, histological examination of the pericardium demonstrated lymphocytic vasculitis. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  9. Hypocomplementemic urticarial vasculitis: a rare presentation of systemic lupus erythematosus.

    Science.gov (United States)

    Aydogan, Kenan; Karadogan, Serap Koran; Adim, Saduman Balaban; Tunali, Sukran

    2006-09-01

    Urticarial vasculitis is a small-vessel vasculitis, presenting clinically as persistent urticarial skin lesions and microscopically as leucocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a distinct type of urticarial vasculitis with multiorgan involvement, whose etiology and link with other diseases are still unknown. Some authors have suggested that HUVS can be accompanied by systemic lupus erythematosus (SLE), and others believe that it is a rare subtype of SLE. Urticarial vasculitis is seen in 7-8% of SLE, while 50% of HUVS patients are diagnosed with SLE. We report a case of HUVS associated with SLE with fatal outcome unresponsive to the combination of systemic corticosteroids and azathioprine. SLE and HUVS share both clinical and laboratory features and are probably not separate entities. It is mostly likely that HUVS and SLE fall into the same spectrum of autoimmune diseases. HUVS is probably a subset of SLE. As both diseases can fatally, it should be kept in mind that the overlap of SLE and HUVS may exhibit a relatively rapid progression and poor prognosis.

  10. Kidney Damage in Hemorrhagic Vasculitis Occurring in Childhood and Adulthood

    Directory of Open Access Journals (Sweden)

    O.V. Syniachenko

    2016-12-01

    Full Text Available Introduction. Nephropathy is diagnosed in 30–60 % of patients with hemorrhagic vasculitis (HV (Schönlein Henoch puprupa and occurred in each fourth of them in the onset of the disease and with the same incidence at first recurrence of the patho­logical process. In recent years, the relative and absolute number of patients with this form of glomerular disease significantly increased. According to the results of the kidney biopsy in children, Henoch glomerulonephritis (HGN is the most common variant of the secondary immunoglobulin (Ig A nephritis. The nature of the clinical course and morphological manifestations of the HGN in patients with HV, which began in childhood and adulthood, remains unexplored. This was the purpose and objectives of this study. Materials and methods. The study included 174 patients with HV (83 % of men and 47 % of women. In 92 cases, vasculitis debuted in children (on average in 12 years, and in 82 — in the adults (on average in 25 years. I, II and III degree of activity of pathological process are set at a ratio of 1 : 2 : 2. Seropositivity for high levels of IgA occurred in 40 % of cases, by the presence of rheumatoid factor — in 27 %. At the time of the survey, cutaneous syndrome was diagnosed in 68 % of patients in the form of urticarial, hemorrhagic, papule-nodular, papule-necro­tic, pustular-ulcerative, necrotic-ulcerative, nodose-ulcerative and polymorphic forms, and articular syndrome — in 48 %. In 24 cases, kidney biopsy was performed. Results. Renal disease was revealed in 71 % of patients with HV, while on the background of nephropathy the integral index of the severity of extrarenal patho­logy was significantly higher. According to the characteristics of the articular syndrome, patients with nephropathy and without it differed little among themselves. The severity of muscle syndrome has the impact on the development of the HV. In turn, renal pathology significantly influenced the development

  11. Statistical study of some Lee galaxy groups

    Science.gov (United States)

    Mohamed, Sabry A.; Fouad, Ahmed M.

    2017-12-01

    Compact groups of galaxies are systems of small number of galaxies close to each other. They are a good laboratory to study galaxy properties, such as structure, morphology and evolution which are affected by the environment and galaxy interactions. We applied the tree clustering technique (the Euclidean separation distance coefficients) to test the physical reality of groups and used certain criteria (Sabry et al., 2009) depending on the physical attributes of the galaxies. The sample of the data is the quintets groups of Lee compact groups of galaxies (Lee et al., 2004). It is based on a modified version of Hickson's criteria (Hickson, 1982). The results reveal the membership of each galaxy and how it is related to its group. The tables of groups and their members are included. Our results indicates that 12 Groups are real groups with real members while 18 Groups have one galaxy that has attribute discordant and should be discarded from its group.

  12. Key advances in the clinical approach to ANCA-associated vasculitis.

    Science.gov (United States)

    Kallenberg, Cees G M

    2014-08-01

    The updated nomenclature for vasculitis defines this varied group of disorders by aetiology, specific features of pathogenesis and clinical symptoms; diagnostic and classification criteria for clinical practice are in development. Here, I review some important advances in the management of vasculitis within the category of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), which encompasses microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA). The clinical approach to the management of the patient with AAV should include testing for ANCA specificity; proteinase 3 (PR3)-specific ANCAs are most often associated with GPA, whereas myeloperoxidase (MPO)-ANCAs are usually associated with MPA. Also important to the management of AAV is an assessment of the disease stage and severity, to enable tailored treatment based on an algorithm derived from controlled-trial data. Remaining questions pertain to the dosage and duration of corticosteroid treatment, the selection of patients for, and duration of, maintenance treatment after induction of remission, and the identification of safer and more effective therapies than are currently in use. Outcome measures should assess not only disease activity, but also damage and quality of life. Infections, cardiovascular events and malignancies also contribute to outcome, and their prevention should therefore be part of the clinical approach to managing patients with AAV.

  13. Literature Study Groups: Literacy Learning "with Legs"

    Science.gov (United States)

    Parsons, Sue Christian; Mokhtari, Kouider; Yellin, David; Orwig, Ryan

    2011-01-01

    Literature study groups help promote critical thinking and improve reading skills. These groups, in general, are characterized by: (1) a flexible grouping--usually determined by a reader's choice of a given book at a given time; (2) participant-centered dialogue, where the teacher takes on the role of facilitator and expert participant rather than…

  14. Higher Serum Angiotensinogen Is an Indicator of IgA Vasculitis with Nephritis Revealed by Comparative Proteomes Analysis.

    Directory of Open Access Journals (Sweden)

    Xuelian He

    Full Text Available IgA vasculitis (IgAV, previously named as Henoch-Schönlein purpura, is the most common systematic vasculitis with unknown etiology. Lack of appropriate study system and/or animal model limits the understanding of its molecular pathogenesis and hinders the identification of targets for rational therapy, especially for its long-term complication, IgAV nephritis (IgAVN. In this study, we applied comparative analysis of serum proteomes to obtain an insight about disease pathogenesis. This study has utilized high sensitivity nanoscale ultra performance liquid chromatography-mass spectrometry (nanoLC-MS/MS to investigate the alterations in serum proteomic profiles in patients with IgAV (n=6, IgAVN (n=6 and healthy subjects (n=7. The differentially expressed proteins were subjected to functional pathway analysis by PANTHER and DAVID software. We identified 107 differentially expressed proteins among three different groups, and functional analysis suggested that, in addition to earlier reported pathways, such as acute phase response, immune response, complement and blood coagulation pathways, hemostasis and Wnt signaling pathway were probably involved in pathogenesis of IgAV. A few differentially abundant proteins identified, such as C4a, serum amyloid A, angiotensinogen, and kininogen 1, were further validated by ELISA. More importantly, we found that angiotensinogen concentration is correlated with IgAVN and could be used as a potential marker for the progression of IgAV. This is the first report of analyzing the proteomic alterations in IgAV patients and the differentially proteins identified in this study may enhance understanding of the pathology of IgAV and a few of them may be used to monitor disease progression.

  15. Ultraviolet erythema as an indicator of nonspecific reactivity during hemorrhagic vasculitis in children

    Energy Technology Data Exchange (ETDEWEB)

    Kharitonova, A.V.

    1973-01-01

    From 1955 to 1967 studies were carried out on 407 children suffering from hemorrhagic vasculitis. For patients in the severe stage of hemorrhagic vasculitis it is characteristic that there are functional shifts in the central nervous system, which are manifested by a changed sensitivity of the skin to ultraviolet irradiation. The appearance of slowed, inert or paradoxal reactions indicates the predominance of processes in the brain that slow down and decrease the reactivity of the organism. In rheumatoid and abdominal-rheumatoid syndromes of the disease, disturbances in indices to skin light-sensitivity are the most manifest. During the recovery period, the indices of ultraviolet erythema, on approaching normal values, still remain low. This behavior requires extensive outpatient observation and treatment of sick children to avoid recidivism. 9 references. (SJR)

  16. Pure Motor Aphasia Developed After Cerebral Vasculitis Due To Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Murat Terzi

    2011-09-01

    Full Text Available Systemic lupus erythematosus (SLE is a complex multisystem disease that may involve the central and peripheral nervous systems. Common clinical findings include seizures, depression, psychosis, aseptic meningitis, headache, cranial and peripheral neuropathies. The incidence of stroke as a neurological involvement in patients with SLE is between 3-20%. Possible reasons for the development of stroke are suggested as hypercoagulability and thrombosis due to antiphospholipid antibodies, cerebral vasculitis, cerebral embolus due to Libman Sacks endocarditis and hypertension due to the disease itself or long term corticosteroid use. We present clinical, laboratory and radiological imaging findings that confirm cerebral vasculitis of a SLE case with the clinical presentation of acute stroke in this study.

  17. Digital vasculitis in systemic lupus erythematosus: a minor manifestation of disease activity?

    Science.gov (United States)

    Gomes, C; Carvalho, J F; Borba, E F; Borges, C T L; Vendramini, M B; Bueno, C; Costa, L P; Bonfá, E

    2009-10-01

    The objective of this study is to determine if digital vasculitis (DV), a clinical manifestation with a high systemic lupus erythematosus disease activity index (SLEDAI) score, is associated with lupus severity. DV and other clinical manifestations defined according to the SLEDAI were evaluated in 168 consecutive patients with systemic lupus erythematosus (SLE). Two groups were defined according to presence (DV+, n = 27) or absence of DV (DV-, n = 141) at the time of evaluation. The exclusion criterion was the presence of antiphospholipid syndrome (Sapporo's criteria). The two groups were comparable with regard to age (P = 0.09), gender (P = 1.00), white race (P = 0.81), and disease duration (P = 0.78). Compared to the DV- group, the DV+ group had a significantly higher frequency of mucocutaneous manifestations (66.7 vs. 39.0%, P = 0.01), haematological abnormalities (22.2 vs. 6.4%, P = 0.02) and constitutional symptoms (11.1 vs. 0.7%, P = 0.01). Renal and neurological involvements were similar in both groups (P = 0.57 and P = 1.00, respectively). The evaluation of each SLEDAI parameter confirmed that the DV+ group had higher frequencies of mild manifestations, such as new rash (P = 0.02), alopecia (P = 0.02), oral ulcers (P = 0.045), fever (P = 0.01) and leucopenia (P = 0.005). In contrast, both groups had similarly increased anti-dsDNA (P = 0.78) and decreased complement levels (P = 0.29). In conclusion, DV in patients with SLE identifies a subgroup of a mild disease. The high 'weighted' index attributed to this alteration in the SLEDAI score should therefore be revised.

  18. Small-vessel vasculitis surrounding an uninflamed temporal artery: a new diagnostic criterion for polymyalgia rheumatica?

    Science.gov (United States)

    Chatelain, Denis; Duhaut, Pierre; Loire, Robert; Bosshard, Sylvie; Pellet, Hélène; Piette, Jean-Charles; Sevestre, Henri; Ducroix, Jean-Pierre

    2008-08-01

    To assess the prevalence and clinical significance of small-vessel vasculitis (SVV) surrounding an uninflamed temporal artery (TA) in patients diagnosed as having giant cell (temporal) arteritis (GCA) and/or polymyalgia rheumatica (PMR). Patients with GCA and/or PMR (n = 490) were included in this multicenter prospective study. Slides of TA biopsy specimens were reviewed by 2 pathologists who were blinded with regard to clinical information. SVV was defined as aggregates of mononuclear inflammatory cells surrounding a capillary, distant from an uninflamed temporal artery. Clinical and biologic data of patients in the SVV group (n = 35) were compared with data of patients with biopsy-proven GCA (n = 280) and with negative TA biopsy findings (n = 175). SVV was diagnosed in 18 women and 17 men (mean +/- SD age 74.5 +/- 9.4 years). The group of patients with SVV had a higher proportion of men than in the entire GCA series, had systemic symptoms, headache, jaw claudication, and an abnormal temporal artery less frequently at clinical examination, but had symptoms of PMR more often than patients in the biopsy-proven GCA group (P = 2.6 x 10(-7), odds ratio 9.17 [95% confidence interval 3.44-24.4]). Levels of inflammation markers were significantly lower in the SVV group. Patients in the SVV group had fever less frequently than patients in the group with negative TA biopsy findings, but otherwise shared the same clinical (including PMR symptoms) and biologic features. Eighteen of the 94 patients with pure PMR (19%) had SVV. SVV is often neglected by pathologists, and appears to be strongly associated with PMR symptoms in patients with a clinical diagnosis of GCA and/or PMR. However, SVV as a new diagnostic criterion for PMR must be assessed in prospective studies.

  19. Anti-neutrophil cytoplasmic antibody pathogenesis in small-vessel vasculitis: an update.

    Science.gov (United States)

    Gómez-Puerta, José A; Bosch, Xavier

    2009-11-01

    Vasculitides associated with serum positivity for anti-neutrophil cytoplasmic antibodies (ANCAs) that affect small- to medium-sized vessels are commonly known as ANCA-associated vasculitis (AAV) and include Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. Evidence derived from both in vitro studies and recent animal models points to a pathogenic role of ANCAs in AAV. In 2002, the first in vivo breakthrough in the pathogenesis of ANCAs showed that mouse ANCAs against myeloperoxidase (MPO) led to intrinsic pauci-immune renal vasculitis in mice. In 2004, a report using both in vitro and in vivo studies proposed that proteinase 3 (PR3)-directed autoimmunity involved the complementary peptide of PR3 (cPR3), which is encoded by the antisense strand of the PR3 gene. The last breakthrough came in October 2008 with a previously undescribed molecular explanation for the origin and development of injury in pauci-immune renal vasculitis, with potential clinical implications. This report showed that infection by fimbriated bacteria may trigger cross-reactive autoimmunity to a previously characterized ANCA antigen, lysosomal membrane protein-2, which is contained in the same vesicles that harbor MPO and PR3. Infection by fimbriated bacteria resulted in the production of autoantibodies, which activated neutrophils and killed human microvascular endothelium in vitro and caused renal vasculitis in rats. Although the evidence for a pathogenic role of ANCAs, mainly MPO-ANCAs, is striking, various questions remain unanswered. Understanding the key pathogenic mechanisms of AAV may provide a safer, more rational therapeutic approach than the traditional (ie, corticosteroids and immunosuppressants) treatment strategy.

  20. CT findings at lupus mesenteric vasculitis

    Energy Technology Data Exchange (ETDEWEB)

    Ko, S.F. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan); Lee, T.Y. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan); Cheng, T.T. [Chang Gung Medical College and Memorial Hospital, Dept. of Rheumatology, Kaohsiung Hsien (Taiwan); Ng, S.H. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan); Lai, H.M. [Chang Gung Medical College and Memorial Hospital, Dept. of Rheumatology, Kaohsiung Hsien (Taiwan); Cheng, Y.F. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan); Tsai, C.C. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan)

    1997-01-01

    Purpose: To describe the spectrum of early CT findings of lupus mesenteric vasculitis (LMV) and to assess the utility of CT in the management of this uncommon entity. Methods: Abdominal CT was performed within 1-4 days (average 2.2 days) of the onset of severe abdominal pain and tenderness in 15 women with systemic lupus erythematosus. Prompt high-dose i.v. corticosteroid in 11 patients after the CT diagnosis of LMV was made. CT was performed after abdominal symptoms subsided. Results: Eleven cases revealed CT features suggestive of LMV including conspicuous prominence of mesentric vessels with palisade pattern or comb-like appearance (CT comb sign) supplying focal or diffuse dilated bowel loops (n=11), ascites with slightly increased peritoneal enhancement (n=11), small bowel wall thickening (n=10) with double halo or target sign (n=8). Follow-up CT before high-dose steroid therapy revealed complete or marked resolution of the abnormal CT findings. Conclusion: CT is helpful for confirming the diagnosis of LMV, especially the comb sign which may be an early sign. Bowel ischemia due to LMV is less ominous than previously expected, and the abnormal CT findings were reversible when early diagnosis and prompt i.v. steroid therapy could be achieved. (orig.).

  1. Thoracic vasculitis presenting as surgical problems.

    LENUS (Irish Health Repository)

    Jansen, Michael

    2010-01-01

    We present four patients with vasculitis manifesting with unusual clinical or pathological features, generating surgical problems. Two cases presented with pulmonary hypertension, with investigations and radiological evidence prompting clinical suspicion of pulmonary thrombo-embolic disease. First case, with an antecedant history of Wegener\\'s granulomatosis (WG), demonstrated following "embolectomy", WG involving the large pulmonary elastic arteries. The second case of inoperable "pulmonary thrombo-embolic disease" was subsequently found at limited post mortem to have giant cell arteritis, which affected widespread small peripheral pulmonary arterial vessels. The other two cases were of aortitis occurring in the background of immune-mediated disease, which had been treated with aggressive immunosuppression regimens. The first of these was a case of Cogan\\'s syndrome complicated by descending aortitis, a rarely reported phenomenon, with co-existent acute endocarditis of the aortic valve leaflets. Most cases of endocarditis in this context occur secondary to and in continuity with ascending aortitis. That this case, and a case of ascending aortitis occurring in the context of relapsing polychondritis occurred in the face of aggressive immunosuppression with an apparent clinical response, underscores the need to not accept a clinical picture at face value. This has implications for clinical management, particularly in the follow-up of surgical prosthetic devices such as grafts which may be used in these cases. All four cases emphasise the continued importance of histology and the post-mortem examination in elucidating previously undetected or unsuspected disease.

  2. Vestibular Involvement in Patients With Otitis Media With Antineutrophil Cytoplasmic Antibody-associated Vasculitis.

    Science.gov (United States)

    Morita, Yuka; Takahashi, Kuniyuki; Izumi, Shuji; Kubota, Yamato; Ohshima, Shinsuke; Horii, Arata

    2017-01-01

    Otitis media (OM) with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) is a novel concept of ear disease that is characterized by progressive mixed or sensorineural hearing loss with occasional systemic involvement. Considering the accumulating knowledge about the characteristics of and treatment for auditory dysfunction in OMAAV, the objective of this study was to investigate the vestibular function and symptoms of patients with OMAAV. Retrospective chart review. University hospital. Thirty-one OMAAV patients met criteria proposed by the OMAAV study group in Japan. Clinical characteristics and vestibular tests. Eleven of 31 OMAAV patients had vestibular symptoms; 3 patients had acute vertigo attack with sudden hearing loss and 8 patients had chronic dizziness. Episodic vertigo was not seen in any of the patients. Three patients who received a less intensive therapy without immunosuppressive agents developed intractable persistent dizziness. All symptomatic patients and six of the nine OMAAV patients without vestibular symptoms showed unilateral or bilateral caloric weakness; therefore, vestibular involvement was present in 84% of OMAAV patients. Gain of vestibulo-ocular reflex was reduced in symptomatic patients. The eye-tracking test and optokinetic nystagmus revealed no evidence of central dysfunction. Vestibular dysfunction was seen in 84% of OMAAV patients. One-third of OMAAV patients showed vestibular symptoms such as acute vertigo attack or chronic dizziness, which are of peripheral origin. One-third of the symptomatic patients developed intractable dizziness. Initial intensive treatment by combination therapy with steroid and immunosuppressive agents may be essential for preventing the development of intractable dizziness.

  3. Clinical features and etiology of retinal vasculitis in Northern Thailand

    Directory of Open Access Journals (Sweden)

    Supanut Apinyawasisuk

    2013-01-01

    Full Text Available Purpose: To report on the clinical features and etiology of patients with retinal vasculitis (RV. Materials and Methods: We reviewed medical records of 47 patients (75 affected eyes diagnosed with RV. Clinical presentations, ocular complications, associated systemic diseases, and treatment regimens were registered. Results: Etiology of RV included infectious causes in 10/47, (21% while an association with systemic and/or ocular non-infectious disorders was noted in 22/47 (47%. Eales′ disease and Behcet′s disease represented the most common clinical entities in non-infectious group while tuberculosis-associated RV was diagnosed in 6/10 (60% among those with infectious disorders. RV was bilateral in 28/47 (60% patients. Retinal veins were most commonly affected (72%, 34/47. Involvement of arteries was present in 12/47 (25% and was associated with viral infections and Behcet′s disease. Ocular complications developed in 60/75 (80% eyes. The most common complications were elevated intraocular pressure and/or glaucoma (33/75, 44%. Retinal detachment, vitreous hemorrhage, and cystoid macular edema developed in similar percentages (15%. Conclusions: RV in Thailand manifested mostly in male patients, was typically bilateral and involved mostly veins. Involvement of arteries was observed in patients with viral infections and Behcet′s disease. Tuberculosis was the most common infectious cause.

  4. Florid urticarial vasculitis heralding a flare up of ulcerative colitis.

    Science.gov (United States)

    Boules, Evon; Lyon, Calum

    2014-12-22

    A 75-year-old man with ulcerative colitis (UC) and diet controlled diabetes mellitus presented with a 3-week history of slightly itchy, red plaques on both lower limbs ascending gradually to cover the trunk and arms. One week later, he developed a flare up of his UC. Routine blood tests showed modest drop in haemoglobin (122 g/L) and C reactive protein (85 mg/L). Serology was remarkable for high antiproteinase 3 (c-ANCA). Serum electrophoresis showed a mildly positive paraprotein band (γ region). Stool culture was negative. Urine analysis showed proteinuria. Skin biopsy showed features of urticarial vasculitis (UV). He underwent a flexible sigmoidoscopy after the flare up showed mildly active UC. The patient was given hydrocortisone for 7 days and then prednisolone. Both rash and UC subsided. Electrophoresis was repeated 4 weeks later showing normal pattern. Prednisolone has been gradually reduced. Although rare, UV can be considered as one of the skin manifestations of UC. 2014 BMJ Publishing Group Ltd.

  5. [Seronegative leukocytoclastic vasculitis preceding the onset of disseminated systemic lupus erythematosus].

    Science.gov (United States)

    Chevalier, X; Belmatoug, N; Kahn, M F

    1990-11-01

    We encountered 4 patients (3 women, 1 man) with cutaneous vasculitis: three have a delayed pressure urticaria, two a vascular purpura with which a mixt cryoglobulinemia. Histology show a leukocytoclastic vasculitis. Initially all studies for lupus erythematosus were negative. However, after 3 to 10 years of follow-up, the 4 patients developed clinical, serological and histological features of systemic lupus erythematosus meeting four or more criteria of the American Rheumatism Association for the diagnosis of SLE. In one case there is a moderate renal disease. In three others cases there are a severe visceral injury: one with aseptic valvula's injury treated by surgery, another who died from a septicemic incident. The last who died from a neurological complication of systemic lupus. During the isolated cutaneous vasculitis phasis antinuclear antibodies and antibodies to double stranded DNA were all negative. At the time of SLE's diagnosis anti-DNA antibodies were present with or without ANA. In all four cases hypocomplementaemia was not initially seen which distinguish these cases from others previously reported in the literature. The syndrome recognized in these patients may constitute an "ante" serological and clinical phasis of SLE.

  6. Necrotizing peripheral vasculitis/vasculopathy following the use of cocaine laced with levamisole.

    Science.gov (United States)

    Farmer, Russell W; Malhotra, Paula S; Mays, Michael P; Egger, Michael E; Smith, Jason W; Jortani, Saeed A; Spiller, Henry; Bosse, George M; Callen, Jeffrey P; Franklin, Glen A

    2012-01-01

    The objective of this study was to describe a novel presentation of peripheral vasculitis associated with levamisole-adulterated cocaine. Cocaine abuse is widespread in the United States with 5.3 million people using cocaine in 2008. Over the past decade, drug enforcement officials have noticed the presence of levamisole in confiscated cocaine samples as an adulterant. Known side effects of cocaine-related levamisole ingestion have included agranulocytosis and a cutaneous acral purpura that is histopathologically characterized by a mixture of inflammation (vasculitis) and occlusion (vasculopathy). A 54-year-old man who nasally ingested cocaine laced with levamisole developed widespread necrotic/purpuric skin lesions on approximately 20% of his body with an acral accentuation. These lesions were complicated by multiple areas of sloughing and necrosis. He was initially treated with topical silver sulfadiazine dressing changes but progressed to require debridement and split-thickness skin grafting. Peripheral vasculitis/vasculopathy with severe necrosis resembling Coumadin necrosis is a relatively recently recognized sequelae from levamisole-adulterated cocaine use.

  7. Intravenous immunoglobulin therapy for hypocomplementemic urticarial vasculitis associated with systemic lupus erythematosus in a child.

    Science.gov (United States)

    Yamazaki-Nakashimada, Marco A; Duran-McKinster, Carola; Ramírez-Vargas, Nadia; Hernandez-Bautista, Victor

    2009-01-01

    Hypocomplementemic urticarial vasculitis is a type of urticarial vasculitis with multisystemic involvement and poor prognosis, sometimes associated with systemic lupus erythematosus. Several therapies have been attempted with no consensus on an effective therapeutic regimen. Intravenous immunoglobulin has been used in severe manifestations of systemic lupus erythematosus and recently in hypocomplementemic urticarial vasculitis. We present a 7-year-old girl with hypocomplementemic urticarial vasculitis associated with systemic lupus erythematosus and pneumonia who responded favorably to intravenous immunoglobulin.

  8. Systematic review of the role of rituximab in treatment of antineutrophil cytoplasmic autoantibody-associated vasculitis, hepatitis C virus-related cryoglobulinemic vasculitis, Henoch–Schönlein purpura, ankylosing spondylitis, and Raynaud's phenomenon

    Directory of Open Access Journals (Sweden)

    Taha R

    2017-12-01

    Full Text Available Rbab Taha,1 Hadeel El-Haddad,1 Abdulqader Almuallim,2 Fatma Alshaiki,3 Elaf Obaid,2 Hani Almoallim1,2,4 1Department of Medicine, Dr Soliman Fakeeh Hospital, Jeddah, 2Department of Medicine, Faculty of Medicine, Umm Al-Qura University, Mecca, 3Department of Medicine, East Jeddah Hospital, Jeddah, 4Rheumatic Diseases, Umm Al-Qura University, Mecca, Saudi Arabia Abstract: Rituximab (RTX is established for the treatment of rheumatoid arthritis. This systematic review of the literature since 2006 summarizes evidence for the use of RTX in the treatment of additional rheumatological diseases: antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV, hepatitis C virus-related cryoglobulinemic vasculitis, Henoch–Schönlein purpura, ankylosing spondylitis, and Raynaud’s phenomenon. Data from randomized controlled trials are available only for AAV, confirming efficacy for remission induction, including in disease resistant to conventional treatment, and maintenance of remission. Further studies are required to confirm optimal maintenance regimens in AAV, important questions needing to be addressed including protocol administration versus treatment in response to clinical relapse and the importance of maintaining B-cell depletion. Sufficient data are available in other diseases to suggest RTX to be useful and that randomized controlled trials should be conducted. Keywords: anti-CD20 monoclonal antibody, anti-neutrophil cytoplasmic antibody (ANCA-associated vasculitis, refractory ankylosing spondylitis, resistant cryoglobulinemic vasculitis, refractory rheumatological diseases 

  9. The role of first-pass perfusion deficit in the detection of cardiac subendocardial manifestation in patients with autoimmune vasculitis.

    Science.gov (United States)

    Pfeil, Alexander; Lehmann, Gabriele; Böttcher, Joachim; Wolf, Gunter; Hansch, Andreas

    2013-01-01

    This report describes the potential of cardiac magnetic resonance imaging (cMRI) based on myocardial first-pass perfusion imaging in the visualization of cardiac manifestations in autoimmune vasculitis, which in the heart are typically localized at the level of small subendocardial vessels. Two patients with primary or secondary autoimmune vasculitis were investigated in this study. Myocardial first-pass perfusion imaging was performed using an ECG-gated T1-weighted MRI sequence after the injection of intravenous bolus of gadolinium chelate. In both cases, the cMRI showed findings of subendocardial first-pass perfusion deficit (FPPD), a phenomenon so far described as microvascular obstruction (MVO) only in patients with acute cardiac infarction due to thromboembolic obstruction of small myocardial vessels. The two patients showed local subendocardial and myocardial hypoenhancement (characterized by a darker appearance than normal myocardial tissue), which is the typical morphological stigma of FPPD initially after injection of contrast media. The perfusion deficit, although morphologically very similar to the well-known phenomenon of MVO in acute cardiac infarction, was conceivably caused by different vasculitis-specific mechanisms such as occlusion of the microvasculature with erythrocytes, neutrophils and cellular debris. This study indicates that FPPD is useful for the non-invasive assessment of the microvasculature in patients with acute cardiac involvement in primary and secondary vasculitis.

  10. Urticarial Vasculitis in a Teenage Girl

    Science.gov (United States)

    Vaughan, Amy; Wolford, Juliet

    2013-01-01

    This case involves a 13-year-old female who presented to the pediatrician for a routine check-up with complaints of a long history of intermittent diarrhea followed by a severe rash lasting for up to a week afterwards. The mother had described her daughter’s condition to multiple physicians, several whom had seen her during flare-ups. The nonmigratory lesions resembled “hives” with a single lesion lasting for 48 to 72 hours and resolving into what her parent described as a bruise. They often diagnosed her daughter with urticaria and prescribed steroids, which did resolve the acute flare-ups. None of the physicians, however, focused on the disease’s evolution and chronicity in an effort toward diagnosis and prevention. The patient was referred by her pediatrician to a dermatologist who diagnosed the patient with urticarial vasculitis. She was initially started on dapsone 25 mg and was increased over a period of months to a maintenance dose of 100 mg daily. She has had no recurrences in her cutaneous or systemic symptoms on this dose. She is closely monitored by her dermatologist on a regular basis with twice yearly complete blood counts. Several attempts have been made to discontinue the dapsone, resulting in a flare of her gastrointestinal symptoms. This patient suffered with this condition for almost 10 years. This is a reminder that spending extra time to think through a patient’s problem early on may prevent years of suffering for patients and their families. PMID:26425571

  11. Urticarial Vasculitis in a Teenage Girl

    Directory of Open Access Journals (Sweden)

    Aaron McGuffin MD

    2013-04-01

    Full Text Available This case involves a 13-year-old female who presented to the pediatrician for a routine check-up with complaints of a long history of intermittent diarrhea followed by a severe rash lasting for up to a week afterwards. The mother had described her daughter’s condition to multiple physicians, several whom had seen her during flare-ups. The nonmigratory lesions resembled “hives” with a single lesion lasting for 48 to 72 hours and resolving into what her parent described as a bruise. They often diagnosed her daughter with urticaria and prescribed steroids, which did resolve the acute flare-ups. None of the physicians, however, focused on the disease’s evolution and chronicity in an effort toward diagnosis and prevention. The patient was referred by her pediatrician to a dermatologist who diagnosed the patient with urticarial vasculitis. She was initially started on dapsone 25 mg and was increased over a period of months to a maintenance dose of 100 mg daily. She has had no recurrences in her cutaneous or systemic symptoms on this dose. She is closely monitored by her dermatologist on a regular basis with twice yearly complete blood counts. Several attempts have been made to discontinue the dapsone, resulting in a flare of her gastrointestinal symptoms. This patient suffered with this condition for almost 10 years. This is a reminder that spending extra time to think through a patient’s problem early on may prevent years of suffering for patients and their families.

  12. Drug-induced leukocytoclastic vasculitis: tigecycline a rare cause

    Directory of Open Access Journals (Sweden)

    Kalpana Bhairavarasu

    2015-01-01

    Full Text Available Drug-induced leukocytoclastic vasculitis is an inflammation of blood vessels triggered by various drugs. It presents with a localized skin rash but may involve the internal organ systems, including the gastrointestinal tract, kidneys, lungs, central nervous system, and joints. The clinical recognition of drug-induced vasculitis is very important because continued use of the culprit drug can be organ or life threatening. The prognosis is excellent if the disease is limited to the skin and diagnosed promptly. The use of tigecycline has recently increased due to resistance patterns of bacteria, and it is important to recognize this potential adverse effect of this drug and to diagnose and treat the patient early to achieve a favorable outcome. To best of our knowledge, we report the first case of tigecycline-induced leukocytoclastic vasculitis.

  13. Hypertrophic cardiomyopathy secondary to hepatitis C virus-related vasculitis.

    Science.gov (United States)

    Cavalli, Giulio; Berti, Alvise; Fragasso, Gabriele; De Cobelli, Francesco

    2016-12-01

    : Almost invariably associated with chronic HCV infection, cryoglobulinemic vasculitis is a small-vessel vasculitis commonly affecting the skin, kidneys, and peripheral nervous system. Cardiac involvement, possibly due to cardiac microcirculation involvement, is an utterly rare and severe complication. We describe a case of hypertrophic cardiomyopathy secondary to cryoglobulinemic vasculitis. Evaluation with transthoracic cardiac ultrasound and cardiac MRI evidenced severe left ventricular hypertrophy and diffuse hypokinesia, a marked decrease in left ventricular ejection fraction, and a subtle late enhancement of inferior and lateral left ventricular walls. Upon clinical stabilization, the patient received treatment with anti-CD20 monoclonal antibody rituximab. Clinical and radiological follow-up with cardiac ultrasound and cardiac MRI documented a dramatic and sustained clinical improvement, with marked reduction of left ventricular hypertrophy, resolution of late enhancement, recovery of left ventricular contractility and function.

  14. Hypocomplementemic Urticarial Vasculitis Syndrome with Membranous Nephropathy: Case Report.

    Science.gov (United States)

    Jung, Su Woong; Choi, Yun Young; Choi, In Seung; Kim, Seulki; Jeong, Kyung Hwan; Song, Ran; Lee, Sang Hoon; Yang, Hyung In; Hong, Seung Jae; Lee, Yeon Ah

    2017-12-01

    Urticarial vasculitis is a rare disorder that principally manifests with recurrent urticarial, sometimes hemorrhagic, skin lesions and/or angioedema. Its clinical presentation is not always limited to cutaneous lesions and it can potentially affect other organs, such as the joints, lungs, kidneys, and eyes. Systemic involvement can either be present at the onset of disease or develop over time. In cases with systemic manifestations, urticarial vasculitis is more likely to be associated with a low complement level. We present the case of a teenage boy with hypocomplementemic urticarial vasculitis syndrome (HUVS) that occurred shortly following swine-origin influenza A virus infection in 2009. Afterwards, HUVS was systemically complicated with myositis and membranous nephropathy that developed several months and about 2 years after its onset, respectively. A combination of glucocorticoid and immunosuppressive agents has been used to effectively control disease activity. © 2017 The Korean Academy of Medical Sciences.

  15. Leukocytoclastic vasculitis: A window to systemic Churg Strauss syndrome

    Directory of Open Access Journals (Sweden)

    Sudhir V Medhekar

    2012-01-01

    Full Text Available A twenty year old male presented with purpuric lesions with chronic painful ulcers over the lower extremities and a recurrent pruritic rash on the trunk for 10 years. He was diagnosed as idiopathic leukocytoclastic vasculitis (LCV after investigations failed to reveal a systemic association. He was treated with immunosuppressants at each visit with partial remission. In 2004, he was diagnosed with bronchial asthma and allergic rhinitis. In his recent admission, he showed necrotic ulcers on legs and extensive shiny, truncal micropapules. Examination revealed maxillary sinus tenderness and loss of sensation on the medial aspect of the left lower limb. Biopsy of ulcer and the micropapules showed the presence of extravascular eosinophils, while hematological investigations showed peripheral eosinophilia of 18%, raised serum Immunoglobulin E (IgE, Anti nuclear antibody (ANA positivity and negative antineutrophil cytoplasmic antibody (ANCA. Radiography confirmed maxillary sinusitis, nerve conduction studies revealed mononeuritis of the anterior tibial nerve and pulmonary function tests (PFT were normal. Clinical examination and investigations pointed towards the diagnosis of Churg-Strauss syndrome (CSS. This report highlights the development of full-blown CSS over a period of 12 years in a patient initially diagnosed as idiopathic LCV, emphasizing the need for regular follow-up of resistant and recurrent cases of LCV.

  16. Refractory urticarial vasculitis as a complication of ulcerative colitis successfully treated with rituximab.

    Science.gov (United States)

    Swaminath, Arun; Magro, Cynthia M; Dwyer, Edward

    2011-08-01

    Ulcerative colitis can be complicated by the development of leukocytoclastic vasculitis, a cutaneous vasculitis with the potential for systemic involvement. We present a man with a history of ulcerative colitis complicated by end-stage liver disease secondary to sclerosing cholangitis requiring a liver transplant. The patient developed new-onset vasculitis and diarrhea refractory to therapy with standard immunosuppression. He was treated with anti-CD20 therapy with a positive response. The basis of the vasculitis was likely one related to an underlying monoclonal paraprotein with cryoprecitable properties. Treatment with anti-B-cell therapy may be a new treatment option for patients with gammopathy-associated leukocytoclastic vasculitis.

  17. OMERACT Endorsement of Patient-reported Outcome Instruments in Antineutrophil Cytoplasmic Antibody-associated Vasculitis.

    Science.gov (United States)

    Robson, Joanna C; Tomasson, Gunnar; Milman, Nataliya; Ashdown, Sue; Boonen, Annelies; Casey, George C; Cronholm, Peter F; Cuthbertson, David; Dawson, Jill; Direskeneli, Haner; Easley, Ebony; Kermani, Tanaz A; Farrar, John T; Gebhart, Don; Lanier, Georgia; Luqmani, Raashid A; Mahr, Alfred; McAlear, Carol A; Peck, Jacqueline; Shea, Beverley; Shea, Judy A; Sreih, Antoine G; Tugwell, Peter S; Merkel, Peter A

    2017-10-01

    The antineutrophil cytoplasmic antibody-associated vasculitides (AAV) are multiorgan diseases. Patients with AAV report impairment in their health-related quality of life (HRQOL) and have different priorities regarding disease assessment compared with physicians. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group previously received endorsement for a core set of domains in AAV. Two approaches to measure patient-reported outcomes (PRO) were presented at OMERACT 2016. A novel 5-step tool was used to facilitate assessment of the instruments by delegates: the OMERACT Filter 2.0 Instrument Selection Algorithm, with a red-amber-green checklist of questions, including (1) good match with domain (face and content validity), (2) feasibility, (3) do numeric scores make sense (construct validity)?, (4) overall ratings of discrimination, and (5) can individual thresholds of meaning be defined? Delegates gave an overall endorsement. Three generic Patient-Reported Outcomes Measurement Information System (PROMIS) instruments (fatigue, physical functioning, and pain interference) and a disease-specific PRO, the AAV-PRO (6 domains related to symptoms and HRQOL), were presented. OMERACT delegates endorsed the use of the PROMIS instruments for fatigue, physical functioning, and pain interference (87.6% overall endorsement) and the disease-specific AAV-PRO instrument (89.4% overall endorsement). The OMERACT Vasculitis Working Group gained endorsement by OMERACT for use of the PROMIS and the AAV-PRO in clinical trials of vasculitis. These instruments are complementary to each other. The PROMIS and the AAV-PRO need further work to assess their utility in longitudinal settings, including their ability to discriminate between treatments of varying efficacy in the setting of a randomized controlled trial.

  18. Report of the Public Cryptography Study Group.

    Science.gov (United States)

    American Council on Education, Washington, DC.

    Concerns of the National Security Agency (NSA) that information contained in some articles about cryptography in learned and professional journals and in monographs might be inimical to the national security are addressed. The Public Cryptography Study Group, with one dissenting opinion, recommends that a voluntary system of prior review of…

  19. Metacognition and Group Differences: A Comparative Study

    Science.gov (United States)

    Al-Hilawani, Yasser A.

    2014-01-01

    In this study, metacognition refers to performing visual analysis and discrimination of real life events and situations in naïve psychology, naïve physics, and naïve biology domains. It is used, along with measuring reaction time, to examine differences in the ability of four groups of students to select appropriate pictures that correspond with…

  20. A Case of Rheumatoid Vasculitis Involving Hepatic Artery in Early Rheumatoid Arthritis.

    Science.gov (United States)

    Lee, Ji Eun; Kim, In Je; Cho, Min Sun; Lee, Jisoo

    2017-07-01

    Rheumatoid vasculitis is a rare, but most serious extra-articular complications of long-standing, seropositive rheumatoid arthritis (RA). Vasculitis of hepatic artery is an extremely rare but severe manifestation of rheumatoid vasculitis. A 72-year-old woman who presented with polyarthralgia for 2 months was diagnosed with early RA. Since she had manifestations of livedo reticularis, and liver dysfunction which was atypical for RA patients, a percutaneous needle liver biopsy was performed revealing arteritis of a medium-sized hepatic artery. Extensive investigations did not reveal evidences of other systemic causes such as malignancy or systemic vasculitis. The patient was diagnosed with rheumatoid vasculitis involving hepatic arteries based on Bacon and Scott criteria for rheumatoid vasculitis. With high dose corticosteroid and cyclophosphamide induction and methotrexate and tacrolimus maintenance treatment, she was successfully recovered. Association of rheumatoid vasculitis at very early stages of the disease may represent an early aggressive form of RA. © 2017 The Korean Academy of Medical Sciences.

  1. Urinary Soluble CD163 in Active Renal Vasculitis

    NARCIS (Netherlands)

    O'Reilly, Vincent P.; Wong, Limy; Kennedy, Claire; Elliott, Louise A.; O'Meachair, Shane; Coughlan, Alice Marie; O'Brien, Eoin C.; Ryan, Michelle M.; Sandoval, Diego; Connolly, Emma; Dekkema, Gerjan J.; Lau, Jiaying; Abdulahad, Wayel H.; Heeringa, Peter; Buckley, Colm; O'Brien, Cathal; Finn, Stephen; Cohen, Clemens D.; Lindemeyer, Maja T.; Hickey, Fionnuala B.; O'Hara, Paul V.; Feighery, Conleth; Moran, Sarah M.; Mellotte, George; Clarkson, Michael R.; Dorman, Anthony J.; Murray, Patrick T.; Little, Mark A.; Sanders, Johannes

    A specific biomarker that can separate active renal vasculitis from other causes of renal dysfunction is lacking, with a kidney biopsy often being required. Soluble CD163 (sCD163), shed by monocytes and macrophages, has been reported as a potential biomarker in diseases associated with excessive

  2. Assessment of disease activity in large-vessel vasculitis

    DEFF Research Database (Denmark)

    Aydin, Sibel Z.; Direskeneli, Haner; Merkel, Peter A.

    2017-01-01

    Objective. To arrive at consensus for candidate outcomes for disease activity assessment in largevessel vasculitis (LVV) in clinical trials. Methods.A Delphi survey including 99 items was circulated among international experts for 3 rounds. Results. Fifty-seven items were accepted for both giant ...

  3. Prednisone and vardenafil hydrochloride for refractory levamisole-induced vasculitis.

    Science.gov (United States)

    Mandrell, Joshua; Kranc, Christina L

    2016-08-01

    Levamisole is an immunomodulatory drug that was previously used to treat various medical conditions, including parasitic infections, nephrotic syndrome, and colorectal cancer. Over the last few years, increasing amounts of levamisole have been used as an adulterant in cocaine. Levamisole-cut cocaine has become a concern because it is known to cause a necrotizing purpuric rash, autoantibody production, and life-threatening leukopenia. Mixed histologic findings of vasculitis and thrombosis are characteristic of levamisole-induced purpura. The recommended management of levamisole-induced vasculitis currently involves withdrawal of the culprit along with supportive treatment. We describe a patient with levamisole-induced vasculitis who continued to develop skin lesions despite self-reported cocaine cessation. Complete resolution of cutaneous disease occurred with the addition of oral prednisone and vardenafil hydrochloride, suggesting the possibility of a new treatment option in patients with refractory disease. In addition, we review the clinical presentation, disease course, diagnostic approach, laboratory findings, histology, and management of levamisole-induced vasculitis. The harmful effects of levamisole-cut cocaine are serious enough that public alerts have been issued to increase awareness. Clinicians should consider the possibility of levamisole exposure in cocaine users presenting with any combination of fever, neutropenia, and necrotic skin lesions, especially in acral areas including the ears.

  4. Genetically distinct subsets within ANCA-associated vasculitis.

    LENUS (Irish Health Repository)

    Lyons, Paul A

    2012-07-19

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener\\'s granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis.

  5. Acute type II cryoglobulinaemic vasculitis mimicking atherosclerotic peripheral vascular disease.

    LENUS (Irish Health Repository)

    Saeed, A

    2012-01-31

    Atherosclerotic peripheral vascular disease is a common presenting cause for digital ischaemia in life long smokers. Acute severe Type II Cryoglobulinaemic vasculitis is a rare yet important cause, which may present with similar clinical features and which if undiagnosed may be rapidly fatal. Following the instigation of therapy with intravenous methylprednisolone and cyclophosphamide this patient made an excellent recovery.

  6. Effectiveness of omalizumab in a case of urticarial vasculitis.

    Science.gov (United States)

    Fueyo-Casado, A; Campos-Muñoz, L; González-Guerra, E; Pedraz-Muñoz, J; Cortés-Toro, J A; López-Bran, E

    2017-03-01

    Urticarial vasculitis (UV) is a mainly leucocytoclastic vasculitis with urticarial plaques. Treating these patients is challenging as the available treatments have poor efficacy. Oral corticosteroids are considered the first-line treatment, but H1 antihistamines, dapsone, colchicine, antimalarials, ciclosporin and antileucotrienes have all been tried also. However, because of their adverse effects and/or lack of efficacy, new agents are still needed. Omalizumab, an anti-IgE antibody, shows efficacy in chronic spontaneous urticaria, and might also be a good treatment for angio-oedema and urticarial vasculitis. To our knowledge, there have been only seven relevant case reports published in the English literature. We add a new case of severe chronic recurrent urticarial vasculitis refractory to all of the drugs mentioned above. We started the patient on subcutaneous omalizumab 300 mg every 4 weeks, which produced clinical improvement within the first month and total remission in the fifth month. The patient has remained stable for 23 months, and follow-up is ongoing. © 2017 British Association of Dermatologists.

  7. Levamisole adulterated cocaine and pulmonary vasculitis: Presentation of two lethal cases and brief literature review.

    Science.gov (United States)

    Karch, Steven B; Busardò, Francesco Paolo; Vaiano, Fabio; Portelli, Francesca; Zaami, Simona; Bertol, Elisabetta

    2016-08-01

    The first case reports of levamisole-related disease in cocaine users were published in 2010, although levamisole adulteration of cocaine was first recognized several years earlier. Currently, more than 70% of street cocaine seizures, in the US and the EU, contain levamisole, which could potentially be converted to aminorex, though the reasons for this practice still remain obscure. Here we report two fatal cases of isolated pulmonary vasculitis in abusers of levamisole-adulterated cocaine, where a complete autopsy, full toxicological analysis by gas chromatography-mass spectrometry (GC-MS) using a previously published method of Karch et al. and histological examination were performed. A control group composed of 11 cases of cocaine related deaths, where the presence of levamisole was excluded in blood, urine and hair, was used. Recent literature on the human pharmacokinetics of levamisole and aminorex is also reviewed. The toxicological analysis revealed positive qualitative and quantitative results for cocaine, benzoylecgonine and levamisole in both cases. In case 1 levamisole was found at the concentration of 13.5 and 61.3mg/L in blood and urine respectively, whereas in case 2 at 17.9 and 70.2mg/L. The histological examination highlighted in case 1 in heart samples microscopic evidence of the typical remodeling changes associated with chronic stimulant abuse, whereas lungs showed numerous lymphocytes surrounding and infiltrating the wall of small pulmonary vessels and a perivascular fibrosis with transforming fibroblasts. In case 2, the myocardial samples showed wide fields of myocardial necrosis characterized by hypercontraction of the myocytes with thickened Z-lines and short sarcomeres, whereas lung samples showed a significant intimal thickening of arteriole walls and lymphocytic infiltration of the wall and edema. Moreover, there were also numerous perivascular lymphocytic infiltrates. Although the pathological cardiac findings have allowed us to establish

  8. Physical Fatigue, Fitness, and Muscle Function in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

    Science.gov (United States)

    McClean, Andrew; Morgan, Matthew D; Basu, Neil; Bosch, Jos A; Nightingale, Peter; Jones, David; Harper, Lorraine

    2016-09-01

    This study investigated differences in cardiorespiratory fitness, muscular function, perceived exertion, and anxiety/depression between patients and healthy controls (HCs) and assessed which of these variables may account for the fatigue experienced by patients. Fatigue was measured in 48 antineutrophil cytoplasmic antibody-associated vasculitis patients and 41 healthy controls using the Multidimensional Fatigue Inventory (MFI-20), focusing on the physical component. Quality of life, anxiety/depression, and sleep quality were assessed by validated questionnaires. Muscle mass was measured by dual-energy x-ray absorptiometry scan, strength as the maximal voluntary contraction (MVC) force, and endurance as sustained isometric contraction at 50% MVC of the quadriceps. Voluntary activation was assessed by superimposed electrical stimulation. Cardiorespiratory fitness ( ˙Vo2 max and oxygen pulse [O2 pulse]) and perceived exertion (Borg scale) were measured during progressive submaximal exercise. Patients reported elevated physical fatigue scores compared to HCs (patients MFI-20 physical 13 [interquartile range (IQR) 8-16], HCs MFI-20 physical 5.5 [IQR 4-8]; P Muscle mass was the same in both groups, but MVC and time to failure in the endurance test were lower due to reduced voluntary activation in patients. Estimated ˙Vo2 max and O2 pulse were the same in both groups. For the same relative workload, patients reported higher ratings of perceived exertion, which correlated with reports of MFI-20 physical fatigue (R(2)  = 0.2). Depression (R(2)  = 0.6), anxiety (R(2)  = 0.3), and sleep disturbance (R(2)  = 0.3) were all correlated with MFI-20 physical fatigue. These observations suggest that fatigue in patients is of a central rather than peripheral origin, supported by associations of fatigue with heightened perception of exertion, depression, anxiety, and sleep disturbance but normal muscle and cardiorespiratory function. © 2016, American College

  9. [Periostitis in systemic necrotizing vasculitides: Study of the 4 cases identified among the 1762 patients of the FVSG database and review of the literature].

    Science.gov (United States)

    Périchon, Sophie; Pagnoux, Christian; Seror, Raphaèle; Dassonville, Lise; Mangouka, Laurette; Cohen, Pascal; Letellier, Philippe; Guillevin, Loïc

    2010-01-01

    Periostitis (periosteal new bone formation) is a rare manifestation of systemic necrotizing vasculitis. We searched the French Vasculitis Study Group (FVSG) database, established in 1980 and containing the data on 1762 patients with a systemic necrotizing vasculitis, i.e., polyarteritis nodosa (PAN), microscopic polyangiitis, Churg-Strauss syndrome or Wegener's granulomatosis, for those with periostitis. Herein, we describe their characteristics and outcomes. Only 4 patients with periostitis were identified. All had limited and localized PAN-like vasculitis, without poor prognosis factors. Periostitis was painful and localized to the lower limbs in all of them and associated with local myositis in 2. Pain and inflammation resolved under corticosteroids in all, but relapses or corticosteroid-dependence ultimately required the adjunction of an immunosuppressant for 3. With a median follow-up of 10 [range: 4-11] years, only 1 developed peripheral neuropathy and none progressed to a more systemic form of vasculitis, i.e., with severe visceral involvement. Physicians should be aware that periostitis, usually localized, is a potential manifestation of systemic necrotizing vasculitis, especially polyarteritis nodosa. The overall long-term outcome of these patients is good, but relapses or corticosteroid-dependence is frequent. Copyright 2010 Elsevier Masson SAS. All rights reserved.

  10. Individualization of the ANCA associated vasculitis treatment

    Directory of Open Access Journals (Sweden)

    Zbigniew Zdrojewski

    2014-09-01

    Full Text Available Vasculitides associated with the presence of anti-neutrophil cytoplasmic antibodies (AAV are the group of systemic diseases characterized by necrotizing inflammation of small and medium size vessels, without immunologic deposits and presence of circulating specific antibodies. Recent genetics and cohorts study should improve comprehension of pathogenesis of AAV, stratification patients in homogenous group and should progress therapeutic implication. Immunosuppressive therapy of AAV includes two major periods: first induction of remission, second maintenance therapy. It should be dedicated individually not only depending on the stage and severity of the disease but also on genetic and some prognostic factors. Previous randomized trials and clinical observations show some possible limitations of treatment with cyclophosphamide and steroids. Rituximab seems to be a good alternative in those patients in induction therapy as well as in maintenance therapy.

  11. Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

    Science.gov (United States)

    Morishita, Kimberly A; Moorthy, Lakshmi N; Lubieniecka, Joanna M; Twilt, Marinka; Yeung, Rae S M; Toth, Mary B; Shenoi, Susan; Ristic, Goran; Nielsen, Susan M; Luqmani, Raashid A; Li, Suzanne C; Lee, Tzielan; Lawson, Erica F; Kostik, Mikhail M; Klein-Gitelman, Marisa; Huber, Adam M; Hersh, Aimee O; Foell, Dirk; Elder, Melissa E; Eberhard, Barbara A; Dancey, Paul; Charuvanij, Sirirat; Benseler, Susanne M; Cabral, David A

    2017-07-01

    To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and the 12-month outcomes in children with AAV. Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of <0.2 mg/kg/day. Secondary outcome measures included the rates of inactive disease (PVAS of 0, with any corticosteroid dosage) and rates of improvement at postinduction (4-6 months after diagnosis) and at 12 months, presence of damage at 12 months (measured by a modified Pediatric Vasculitis Damage Index [PVDI]; score 0 = no damage, score 1 = one damage item present), and relapse rates at 12 months. In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years (interquartile range 10.9-15.8 years). Among the study cohort, 42% of patients achieved remission at 12 months, 49% had inactive disease at postinduction (4-6 months), and 61% had inactive disease at 12 months. The majority of patients improved, even if they did not achieve inactive disease. An improvement in the PVAS score of at least 50% from time of diagnosis to postinduction was seen in 92% of patients. Minor relapses occurred in 12 (24%) of 51 patients after inactive disease had been achieved postinduction. The median PVDI damage score at 12 months was 1 (range 0-6), and 63% of patients had ≥1 PVDI damage item scored as present at 12 months. This is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not

  12. ANCA-associated vasculitis: a comparison of cases presenting to nephrology and rheumatology services.

    Science.gov (United States)

    McNicholas, B A; Griffin, T P; Donnellan, S; Ryan, L; Garrahy, A; Coughlan, R; Giblin, L; Lappin, D; Reddan, D; Carey, J J; Griffin, M D

    2016-12-01

    Anti-neutrophil cytoplasmic antibody (ANCA) -associated vasculitis (AAV) is a disease characterized by inflammation of small vessels and detectable ANCA in the circulation. Patients may develop a broad spectrum of clinical features ranging from indolent sino-nasal disease and rashes to fulminant renal failure or acute life-threatening pulmonary haemorrhage. Consequently, patients with AAV present to a variety of specialties including nephrology and rheumatology, whose training and approaches to management of such patients may differ. There is little literature comparing patients presenting to different specialties and their outcomes. We compared two cohorts of patients with ANCA-positive AAV presenting to either the rheumatology or nephrology department at Galway University Hospitals from June 2002 to July 2011. A standardized data collection form was used to collect information regarding baseline demographics, manifestations of AAV, initial management, relapses and complications. Forty-five patients were included in this study (15 rheumatology/30 nephrology). The nephrology cohort was older, had a higher C-reactive protein, Birmingham Vascular Activity Score and ANCA titer at presentation compared to the rheumatology group. Induction treatment varied between the cohorts with rheumatology patients most commonly receiving a combination of oral corticosteroids (73%) and methotrexate (60%) and nephrology patients receiving a combination of intravenous corticosteroids (93%) and cyclophosphamide (90%). Fifty-three percent of the rheumatology patients who completed induction therapy relapsed compared to 30% of the nephrology patients. This study presents two different cohorts of patients with the same disease that were managed by two different disciplines. It highlights the heterogeneity of AAV and the importance of interdisciplinary communication and cooperation when managing these patients. © The Author 2016. Published by Oxford University Press on behalf of the

  13. Cocaine-induced vasculitis: is this a new trend?

    Directory of Open Access Journals (Sweden)

    García Pérez MR

    2013-10-01

    Full Text Available Miraida Reneé García Pérez,1 Vanessa L Ortiz-González,1 Maria Betancourt,1 Rogelio Mercado21Department of Internal Medicine, San Juan City Hospital, 2Department of Dermatology, University of Puerto Rico School of Medicine, San Juan, Puerto RicoAbstract: Cocaine-induced vasculitis is a rare complication found in drug abusers. It occurs due to cocaine adulterated with levamisole. Levamisole was once used as a chemotherapy and immunomodulator for different conditions. One of the side effects of this medication is necrotizing vasculitis which has been reported in the US and Puerto Rico. Here we present another case of cocaine induced vasculitis in Puerto Rico. We describe a 43-year-old female with past medical history of bronchial asthma, migraine, and crack smoking who presented to the emergency room due to blood in her urine for 5 days. She also reported fever, chills, and fatigue. At the physical exam she had a right knee ulcer with swelling erythema, warmth, and pain. Also, she had retiform purpuric plaque lesions in her ears, bilaterally. Eroded plaques with elevated borders at left foot and finger dorsum were also present. Laboratory workup was positive for cocaine. The patient showed leucopenia and microcytic anemia with a normal absolute neutrophil count in her cell blood count. Blood cultures, urine cultures, and ulcer cultures were negative. Urinalysis was positive for proteinuria and hematuria. Also, the patient had positive perinuclear anti-neutrophil cytoplasmic antibody, cytoplasmic anti-neutrophil cytoplasmic antibody, and antinuclear antibody tests and elastase specificity. She showed negative anticardiolipin and lupus anticoagulant antibodies. Her complement levels were decreased. The punch biopsy of her ear showed superficial thrombosis of superficial vascular plexus with perivascular lymphocytic infiltrates and deeper sections showed epidermal necrosis and necrotizing vasculitis. She was started on a high dose of steroids, but

  14. Epitope analysis of anti-myeloperoxidase antibodies in patients with ANCA-associated vasculitis.

    Directory of Open Access Journals (Sweden)

    Shen-Ju Gou

    Full Text Available OBJECTIVE: Increasing evidences have suggested the pathogenic role of anti-neutrophil cytoplasmic antibodies (ANCA directing myeloperoxidase (MPO in ANCA-associated vasculitis (AAV. The current study aimed to analyze the association between the linear epitopes of MPO-ANCA and clinicopathological features of patients with AAV. METHODS: Six recombinant linear fragments, covering the whole length amino acid sequence of a single chain of MPO, were produced from E.coli. Sera from 77 patients with AAV were collected at presentation. 13 out of the 77 patients had co-existence of serum anti-GBM antibodies. Ten patients also had sequential sera during follow up. The epitope specificities were detected by enzyme-linked immunosorbent assay using the recombinant fragments as solid phase ligands. RESULTS: Sera from 45 of the 77 (58.4% patients with AAV showed a positive reaction to one or more linear fragments of the MPO chain. The Birmingham Vasculitis Activity Scores and the sera creatinine were significantly higher in patients with positive binding to the light chain fragment than that in patients without the binding. The epitopes recognized by MPO-ANCA from patients with co-existence of serum anti-GBM antibodies were mainly located in the N-terminus of the heavy chain. In 5 out of the 6 patients, whose sera in relapse recognize linear fragments, the reactivity to linear fragments in relapse was similar to that of initial onset. CONCLUSION: The epitope specificities of MPO-ANCA were associated with disease activity and some clinicopathological features in patients with ANCA-associated vasculitis.

  15. Environmental studies group. Annual report for 1978

    Energy Technology Data Exchange (ETDEWEB)

    Hunt, D. C.; Hurley, J. D. [eds.

    1980-08-21

    Group projects included radioecological studies of aquatic and terrestrial systems, land management activities, foodstuff monitoring, dust transport studies including fugitive dust measurements and modeling, and several support programs involving evaluation of the plant's ambient air samplers and airborne tritium monitoring techniques. Some salient results from the several project reports include determination of an appropriate model for mechanically generated fugitive dust dispersion, a radionuclide inventory of Smart Ditch Pond (Pond D-1), a coefficient of community determination for two terrestrial sample plots on the plant site buffer zone, a natality and mortality rate determination for fawns in the plant deer herd (including one positive coyote-kill determination), inlet loss and filter paper collection efficiencies for the plant ambient air samplers, and differential tritium sampling measurements of the vapor in Building 771 stack effluent.

  16. Infectious urticaria with purpura: a mild subtype of urticarial vasculitis?

    Science.gov (United States)

    Oi, Mieko; Satoh, Takahiro; Yokozeki, Hiroo; Nishioka, Kiyoshi

    2005-01-01

    Urticaria is characterized by transient wheals. We report here five cases with long-lasting urticarial lesions persisting for more than 24 hours. Each lesion left purpura after fading. There was no systemic involvement. C-reactive protein and serum levels of complement were elevated or normal. Histologically, marked infiltration by eosinophils and neutrophils with karyorrhexis in the perivascular and intercollagenous spaces was observed, but there was no evidence of vasculitis (venulitis). Skin symptoms were resistant to systemic corticosteroids. In contrast, treatment of underlying bacterial infections resulted in marked improvement of skin lesions. E-selectin, VCAM-1 and ICAM-1 were expressed on endothelial cells. Marked deposition of C3a, C5a, neutrophil elastase and major basic protein in the dermis was observed. These urticarial lesions provoked by bacterial infections seem to lie on the continuum between urticaria and urticarial vasculitis.

  17. [Severe pulmonary involvement in hypocomplementemic urticarial vasculitis (HUV)].

    Science.gov (United States)

    Raoufi, Mohammed; Laine, Mustapha; Amrani, Hicham Naji; Souhi, Hicham; Janah, Hicham; Elouazzani, Hanane; Rhorfi, Ismail Abderrahmane; Abid, Ahmed

    2016-01-01

    Pulmonary involvement in hypocomplementemic urticarial vasculitis (HUV) or Mac Duffie syndrome is extremely rare with a poor prognosis. We report the case of a 55-year-old female patient treated for HUV over a period of 20 years. The diagnosis was confirmed on the basis of urticarial lesions, ocular inflammation, positive C1q-p test by immunodiffusion, with low rate of C1q. The patient was treated with cycles of cyclophosphamide, corticoids and rituximab as she developed class III dyspnea (NYHA classification ). The clinico-radiological and functional assessment showed thoracic distension and severe obstructive pulmonary disease which found no significant improvement with systemic treatment Aerosol therapy was started and the patient had a marked clinical improvement. Pulmonary involvement in Mac Duffie hypocomplementemic urticarial vasculitis worsens the patient short-term vital prognosis. The knowledge of the different types of pulmonary involvement opens new therapeutic prospects.

  18. Evalution of Autoimmunity in Patients with Primary Cutaneous Vasculitis

    Directory of Open Access Journals (Sweden)

    Hatice Ozkol

    2014-06-01

    Full Text Available Aim: In order to determine the clinical and etiological characteristics of primer cutaneous vasculitis and to evaluate its relation with immunological mechanisms. Material and Method: Twenty-eight hospitalized patients with cutaneous vasculitis between 2009 -2011 in dermatology service were investigated retrospectively. Patients%u2019 age, gender, disease duration, associated symptoms, infection, and drug history were recorded. Results of immune-fluorescence biopsy and all laboratory tests including detailed antibodies which were investigated for the differential diagnosis of vasculitis were given in percent (% after recording to SPSS 13.0 statistical software. Results: Median age of the 28 patients (18 female, 10 male was 37,89±23,30 (5-84 age years. 39.3% patients had neutrophilia, 67.9% patients had eosinophilia. Antinuclear antibodies (ANA in 10 patients (35.7% were positive. Total IgE in 15 patients (53.6%, IgA in 9 patients (32.1%, IgG in 10 patients (35.7% was higher than normal range. Thyroglobulin in 3 patients (10.7%, antiTPO 4 patients (14.3%, antirubella IgG in 14 patients (50.0% antitoxoplazma IgG in 10 patients (35.7% were positive. Anticardiolipin antibodies, anti-ssa1 and Anti-ssb2, only one patient (3.6% was positive. Discussion: Immunoglobulins and ANA were determined positively in a high ratio in patients with cutaneous leucocytoclastic vasculitis however there was no systemic involvement. Autoimmunity may have an important role in pathogenesis. Moreover drugs were most frequently determined among etiological factors.

  19. Brucella peritonitis and leucocytoclastic vasculitis due to Brucella melitensis

    Directory of Open Access Journals (Sweden)

    Murat Dizbay

    Full Text Available Brucellosis is a multisystemic disease that rarely leads to a fatal outcome. While reticuloendothelial system organs are mostly affected, peritonitis and posthepatitic cirrhosis are also complications of brucellosis, though they are very rare. Brucella spp. can also trigger immunological reactions. We report a case of brucellosis with peritonitis, renal failure and leucocytoclastic vasculitis caused by Brucella melitensis, which led to a fatal outcome. Brucellosis should be considered in the differential diagnosis of vasculitic diseases, especially in endemic areas.

  20. DPHEP: From Study Group to Collaboration

    CERN Document Server

    Shiers, Jamie

    2014-01-01

    The international study group on data preservation in High Energy Physics, DPHEP, achieved a major milestone in 2012 with the publication of its eagerly anticipated large-scale report [1]. This document contains a description of data preservation activities from all major high energy physics collider-based experiments and laboratories. A central message of the report is that data preservation in HEP is not possible without long term investment in not only hardware but also human resources, and with this in mind DPHEP will evolve to a new collaboration structure in 2013. This paper describes the progress made since the publication of that report – shortly before CHEP 2012 – as well as the future working directions of the new collaboration.

  1. Exact renormalization group study of fermionic theories

    Science.gov (United States)

    Comellas, Jordi; Kubyshin, Yuri; Moreno, Enrique

    1997-02-01

    The exact renormalization group approach (ERG) is developed for the case of pure fermionic theories by deriving a Grassmann version of the ERG equation and applying it to the study of fixed point solutions and critical exponents of the two-dimensional chiral Gross-Neveu model. An approximation based on the derivative expansion and a further truncation in the number of fields is used. Two solutions are obtained analytically in the limit N → ∞, with N being the number of fermionic species. For finite N some fixed point solutions, with their anomalous dimensions and critical exponents, are computed numerically. The issue of separation of physical results from the numerous spurious ones is discussed. We argue that one of the solutions we find can be identified with that of Dashen and Frishman, whereas the others seem to be new ones.

  2. Report of JLC site study group

    CERN Document Server

    Hasegawa, T; Yamashita, S

    2003-01-01

    This study group selected some good sites for construction of JLC (Electron-Positron Linear Collider) on the basis of investigation of data and field survey. The aims, activity, use of underground of private land, conditions of site, selection of site at present and future, summary and proposal are reported. 9 sites (Hidaka, Kitakami, Murayama, Abukuma, Kitaibaraki, Aichi and Gifu, Takamatsu, Hiroshima and Seburi range) are selected for the construction on the basis of firm ground and 4 sites (Okinawa, Harima, Tsukuba and Mutsuogawara) for development and researches. 9 sites area consists of plutonic rock or old strata of Paleozoic era. Many problems in each site are reported. There are three following proposals; 1) the self-governing communities of the sites have to understand JLC and start to construct it by information, 2) a site evaluation committee consists of specialist of civil engineering, building, social and natural environment and disaster prevention and 3) the vibration test should be carried out ...

  3. Cutaneous Small Vessel Vasculitis Accompanied by Pustulosis Palmaris et Plantaris

    Directory of Open Access Journals (Sweden)

    Motoko Kosaka

    2012-03-01

    Full Text Available We present the case of a 64-year-old woman who has suffered from pustulosis palmaris et plantaris for 10 years. At the first examination, many erythematous lesions with purpura, blood crusts, and blisters were present in the lower legs and dorsum of the feet. Painful swelling in the sternal region and dorsal pain were also noted. Elevation of the CRP and myogenic enzyme levels, and liver and renal dysfunctions were noted on blood testing. Histopathologically, leukocytoclastic vasculitis was noted in small blood vessels in the whole dermal layers, and deposition of IgM and C3 in the vascular wall was detected by the direct immunofluorescence techniques. Based on these findings, cutaneous small vessel vasculitis was diagnosed. Because the patient complained of a toothache during the clinical course, an X-ray examination was performed. On pantomography, a radicular cyst and apical periodontitis were noted. The tooth symptoms changed with exacerbation and remission of the skin symptoms. These findings indicate that odontogenic infection is very likely to be a cause of cutaneous small vessel vasculitis in a manner similar to pustulosis palmaris et plantaris.

  4. Pulmonary hemosiderosis with normocomplementemic urticarial vasculitis in a child.

    Science.gov (United States)

    Yuksel, H; Yilmaz, O; Savas, R; Kirmaz, C; Sogut, A; Ozalp, S

    2007-03-01

    Pulmonary hemosiderosis is rarely associated with urticarial vaculitis especially if normocomplementemic. An eigth year old girl presented with relapsing and remitting chronic and persistent urticarial lesions, conjunctival injection, recurrent cough and hemoptysis. Respiratory findings started at seven years of age. Physical examination revealed diffuse skin lesions mainly settled on the extremities, non-purulent conjunctival injection, rare ronchi and fine crackles in bilateral lower zones of the lungs. Biopsy of the urticaria like skin lesions demonstrated leukocytoclastic vasculitis. Rheumatological markers were negative. Levels of complement fractions 3 and 4 were normal. Chest x-ray demonstrated diffuse alveolar infiltrative images. High Resolution Computed Tomography of the chest revealed diffuse ground-glass appearance, increased interstitial density. Diagnostic flexible fiberoptic bronchoscopy was performed and bronchoalveolar lavage fluid revealed hemosiderin laden alveolar macrophages. She was started on systemic corticosteroid treatment. During follow up, pulmonary symptoms disappeared, however skin lesions and conjunctival symptoms persisted and exacerbated four times in two years. CT of lungs after two years of treatment revealed rare patchy areas of ground glass appearance in bilateral lower lobes and right upper lobe as well as a few of millimetric pleural nodules. This patient is still followed up under low dose steroids and pulmonary findings regressed but low grade inflammation due to vasculitis is thought to continue as supported by the persistence of tomographic findings in the lungs despite the absence of any symptoms. This case demonstrates association of urticarial vasculitis and pulmonary hemosiderosis in the setting of normocomplementemia.

  5. Severe leukocytoclastic vasculitis secondary to the use of a naproxen and requiring amputation: a case report

    Directory of Open Access Journals (Sweden)

    Martin Jeanine

    2010-07-01

    Full Text Available Abstract Introduction Leukocytoclastic vasculitis (also known as hypersensitivity vasculitis and cutaneous necrotizing vasculitis can present with various manifestations, which often delays the diagnosis and treatment. In order to show the importance of the early recognition of leukocytoclastic vasculitis, we present a case which occurred secondary to the use of a common pharmaceutical, naproxen. We were unable to find a case of leukocytoclastic vasculitis secondary to naproxen in the literature. Case presentation We present the case of a 33-year-old African American woman with below the knee and bilateral digital gangrene from hypersensitivity vasculitis secondary to the non-steroidal anti-inflammatory medication naproxen. Conclusion This is an original case report focusing on the rheumatologic management of leukocytoclastic vasculitis. However, other specialties, such as internal medicine, dermatology, infectious disease, general surgery and pathology, can gain valuable information by reviewing this case report. Reporting a case of leukocytoclastic vasculitis secondary to treatment with naproxen will advance our understanding of this disease etiology by adding yet another non-steroidal anti-inflammatory drug to the list of potential causes of leukocytoclastic vasculitis.

  6. Facebook groups as LMS: A case study

    National Research Council Canada - National Science Library

    Meishar-Tal, Hagit; Kurtz, Gila; Pieterse, Efrat

    2012-01-01

    ..., and giving grades online. In 2010, Facebook made it possible to create closed groups that allow asynchronous and synchronous interactions between members. This also allows sharing of information, such as links to Web sites, text documents, pictures, and so on. The Facebook group contains at least two of the three components of learning management...

  7. Group heterogeneity increases the risks of large group size: a longitudinal study of productivity in research groups.

    Science.gov (United States)

    Cummings, Jonathon N; Kiesler, Sara; Bosagh Zadeh, Reza; Balakrishnan, Aruna D

    2013-06-01

    Heterogeneous groups are valuable, but differences among members can weaken group identification. Weak group identification may be especially problematic in larger groups, which, in contrast with smaller groups, require more attention to motivating members and coordinating their tasks. We hypothesized that as groups increase in size, productivity would decrease with greater heterogeneity. We studied the longitudinal productivity of 549 research groups varying in disciplinary heterogeneity, institutional heterogeneity, and size. We examined their publication and citation productivity before their projects started and 5 to 9 years later. Larger groups were more productive than smaller groups, but their marginal productivity declined as their heterogeneity increased, either because their members belonged to more disciplines or to more institutions. These results provide evidence that group heterogeneity moderates the effects of group size, and they suggest that desirable diversity in groups may be better leveraged in smaller, more cohesive units.

  8. Case Studies Working Group Report Volume 2

    Science.gov (United States)

    2012-03-01

    groups) will augment the number of ter- rorist incidents featuring military- grade CB warfare agents.71 Moreover, if a terrorist group were to ac- quire...military- grade CBRN threats, the department has been given the heavy burden of be- ing prepared to respond with support to multiple (up to three...profuse rhetoric regarding terrorist WMD threats, but to take only timid , incremental ac- tion to address the identified shortfalls. This behavior

  9. HLA-DR4, DR13(6) and the ancestral haplotype A1B8DR3 are associated with ANCA-associated vasculitis and Wegeners granulomatosis

    NARCIS (Netherlands)

    Stassen, Patricia M.; Cohen-Tervaert, Jan W.; Lems, Simon P. M.; Hepkema, Bouke G.; Kallenberg, Cees G. M.; Stegeman, Coen A.

    Objectives. As the HLA system is involved in recognition of self and non-self, an association with the development of ANCA-associated vasculitis (AAV) seems probable. In this study, the relation between HLA antigens and AAV and its severity were investigated. Methods. Consecutive patients diagnosed

  10. Renal survival and prognostic factors in patients with PR3-ANCA associated vasculitis with renal involvement

    NARCIS (Netherlands)

    Slot, MC; Tervaert, JWC; Franssen, CFM; Stegeman, CA

    Background. Severe renal disease is a feature of anti-neutrophil cytoplasmic antibodies (ANCA)-associated small-vessel vasculitis. We evaluated patient and renal survival and prognostic factors in patients with PR3-ANCA associated vasculitis with renal involvement at diagnosis during long-term

  11. Necrotizing leptomeningeal vasculitis associated with a compressive meningioma in a cat: a rare paraneoplastic syndrome

    Directory of Open Access Journals (Sweden)

    Les J. Gabor

    2012-02-01

    Full Text Available A 17 year old cat with a compressive meningioma was found to have an intradural, severe necrotizing vasculitis, spatially un associated with the neoplasm. Paraneoplastic vasculitis has been reported in two cases in the human literature associated with meningiomas. This is the first report of such an association in a domestic species.

  12. Diffuse Large B Cell Lymphoma in a Patient with Hypocomplementemic Urticarial Vasculitis

    Directory of Open Access Journals (Sweden)

    Calvo-Romero J

    2003-01-01

    Full Text Available Hypocomplementemic urticarial vasculitis (HUV is known to be associated with malignancies. Urticarial vasculitis has been linked to lymphomas, but to our knowledge, the association of HUV and non-Hodgkin lymphoma has not been described so far. A patient with HUV who developed 10 years later a diffuse large B cell lymphoma is reported here.

  13. Hypertensive encephalopathy mimicking cerebral vasculitis with pontine oedema, cerebellar white matter lesions and multiple cerebral infarctions.

    Science.gov (United States)

    Ceccarelli, Daniela; Hargroves, David; Balogun, Ibrahim; Webb, Thomas

    2017-07-19

    A 47-year-old man with poorly controlled hypertension presented with headaches, right-sided weakness and dysarthria. CT and MRI scans of the brain showed widespread abnormalities including significant pontine oedema, basal ganglia and corona radiata infarctions and cerebellar white matter high signal. Imaging of the intracerebral vasculature also demonstrated wall irregularities. Initially a central nervous system inflammatory disorder was thought to be the most likely diagnosis, possibly acute demyelinating encephalomyelitis or cerebral vasculitis, and the patient was treated with high-dose intravenous steroids. The diagnosis of hypertensive encephalopathy was made because (1) the patient was hypertensive and (2) the patients MRI findings resolved with antihypertensive treatment.Blood pressure treatment was instigated from admission, and the patients symptoms improved with resolution of the radiological abnormalities. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  14. NETosis: At the crossroads of rheumatoid arthritis, lupus, and vasculitis.

    Science.gov (United States)

    Berthelot, Jean-Marie; Le Goff, Benoit; Neel, Antoine; Maugars, Yves; Hamidou, Mohamed

    2017-05-01

    Suicidal NETosis differs from other mechanisms of cell death by the release of a lattice, composed of DNA associated with proteins citrullinated by protein-arginine deiminase 4, from neutrophils. These 'NETs' are composed of granule-derived proteins with microbicidal activity. A similar type of release occurs during vital NETosis, in which anuclear neutrophils maintain their chemotactic ability and imprison live bacteria, even after NET extrusion. Mitochondrial NETosis is limited to the expulsion of oxidised mitochondrial DNA and cytoplasmic enzymes. NETs include the targets of most autoantibodies found in rheumatoid arthritis, lupus, and vasculitis. The clinical and biological overlaps sometimes observed between bronchectiasis and RA, RA and SLE, or SLE and vasculitis, implicate NETosis as a major triggering event common to these disorders. NETosis increases the possibility of association between autoantigens and infectious antigens in mucosal biofilms, impairing the clearance of pathogens and possibly triggering autoimmune reactions. NETosis aggravates these three conditions and increases endothelial damage and the risk of thrombosis. However, the pathogenesis of RA, SLE, and vasculitis is not confined to autoantibodies against NET components, and other mechanisms have been suggested to explain the breakdown of tolerance to NET autoantigens, such as hypercitrullination. The question of whether continuous presentation of autoantigens mixed with antigens from dormant intracellular pathogens (released following suicidal, vital, or mitochondrial NETosis) is required to induce and sustain autoimmunity must be addressed. Inhibiting NETois may not be sufficient to improve autoimmune disorders whereas such latent infections remain uncontrolled. Copyright © 2016 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  15. Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis.

    Science.gov (United States)

    Hall, A C; Leong, A C; Jiang, D; Fitzgerald-O'Connor, A

    2013-07-01

    Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle-Wells syndrome. Previous reports have described the hearing loss to be progressive in nature. To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle-Wells syndrome. The patient underwent a cochlear implantation with a modest outcome. Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

  16. Disseminated gonococcal infection presenting as vasculitis: a case report

    OpenAIRE

    Jain, Sangita; Win, Htet Nwe; Chalam, Venkat; Yee, Lian

    2007-01-01

    A 50‐year‐old man with alcoholic liver disease presented with fever, tenosynovitis, polyarthritis and a vasculitic rash on the hands and feet for 4 days. He had neutrophilia and raised inflammatory markers. He had no history of sore throat, urethral discharge or travel abroad. His initial blood cultures were negative, and he was treated for vasculitis with steroids. The rash and arthritis seemed to improve initially, but he had another episode of fever. Repeat blood cultures grew Neisseria go...

  17. The EULAR Study Group for Registers and Observational Drug Studies

    DEFF Research Database (Denmark)

    Kearsley-Fleet, Lianne; Závada, Jakub; Hetland, Merete Lund

    2015-01-01

    OBJECTIVE: Under the auspices of the European League Against Rheumatism (EULAR), a study group of investigators representing European biologic DMARD (bDMARD) registers was convened. The purpose of this initial assessment was to collect and compare a cross section of patient characteristics...

  18. Juvenile systemic lupus erythematosus and dermatomyositis associated with urticarial vasculitis syndrome: a unique presentation.

    Science.gov (United States)

    Macêdo, Patrícia A; Garcia, Carolina B; Schmitz, Monique K; Jales, Levi H; Pereira, Rosa M R; Carvalho, Jozélio F

    2012-11-01

    To report a case of triple association of juvenile systemic lupus erythematosus (SLE), juvenile dermatomyositis and urticarial vasculitis as well as a review of the relevant literature. A 12-year-old male patient diagnosed with overlap syndrome between SLE and juvenile dermatomyositis since 2004 evolved with erythematous plaques, which were compatible with an urticarial rash. Clinical, laboratory and histopathological findings indicated a diagnosis of urticarial vasculitis. The patient previously had a C1q deficiency. Using the established treatment with methylprednisolone (1 g/day for 3 days), increasing doses of deflazacort and introduction of a dapsone, as well as mycophenolate mofetil regimen, with the suspension of azathioprine resulted in complete resolution of skin lesions. Urticarial vasculitis can present in various diseases. In SLE, presentation of urticarial vasculitis in children is rarely found. The triple association of juvenile-onset SLE, juvenile dermatomyositis and urticarial vasculitis is unusual, and this is the first case described in literature.

  19. The use of muscle biopsy in the diagnosis of systemic vasculitis affecting small to medium-sized vessels: a prospective evaluation in Japan.

    Science.gov (United States)

    Nunokawa, T; Yokogawa, N; Shimada, K; Enatsu, K; Sugii, S

    2016-01-01

    The aim of this study was to assess the use of muscle biopsy for histopathological confirmation of small vessel vasculitis (SVV) or medium vessel vasculitis (MVV). Muscle biopsies were performed for all consecutive cases of suspected SVV or MVV seen at Tokyo Metropolitan Tama Medical Centre between February 2012 and May 2014 except those for which a skin or renal biopsy was indicated. Forty-nine patients underwent muscle biopsies. All patients were followed for a minimum of 6 months. Diagnosis of SVV or MVV was made in 35 patients. An unrelated condition was diagnosed in 11 patients and no diagnoses were made in three patients. Of the 35 patients in whom SVV or MVV was diagnosed, positive muscle biopsies were obtained in 20 patients [15 microscopic polyangiitis (MPA), three polyarteritis nodosa (PAN), and two eosinophilic granulomatosis with polyangiitis (EGPA)], while other findings led to the same diagnosis in 15 (seven MPA, four GPA, three PAN, and one rheumatoid vasculitis). The sensitivity of the muscle biopsy was 57% [20/35; 95% confidence interval (CI) 50-57]. Of 13 patients presenting with peripheral neuropathy, the muscle biopsy demonstrated vasculitis in nine patients, with 75% sensitivity (9/12; 95% CI 69-75). There were no complications in the procedure apart from delayed wound healing in one patient. Muscle biopsy is a safe method that offers a high diagnostic yield for SVV or MVV, especially in patients with vasculitic neuropathy.

  20. Brief Report: Endothelial-Specific X-Box Binding Protein 1 Deficiency Limits Tumor Necrosis Factor-Induced Leukocyte Recruitment and Vasculitis.

    Science.gov (United States)

    Ziogas, Athanasios; Muders, Michael H; Economopoulou, Matina; Sprott, David; Grossklaus, Sylvia; Siegert, Gabriele; Baretton, Gustavo B; Mitroulis, Ioannis; Chavakis, Triantafyllos

    2015-12-01

    Endothelial cell activation by tumor necrosis factor (TNF) and associated leukocyte infiltration are hallmarks of vasculitis. The aim of this study was to investigate the potential role of the cellular stress-associated endothelial X-box binding protein 1 (XBP-1) transcription factor in TNF-induced endothelial cell inflammation and vasculitis. Mice with an endothelial cell-specific XBP-1 deficiency were used in a modified local Shwartzman reaction (LSR) model of TNF-induced small vessel vasculitis. To address the contribution of XBP-1 to the TNF-mediated inflammatory response in endothelial cells, we examined the activation of XBP-1 expression by TNF as well as the effect of XBP-1 knockdown in endothelial cells on TNF-induced signaling, proinflammatory gene expression, and leukocyte-endothelial cell adhesion. The active spliced form of XBP-1 in endothelial cells was triggered by TNF. In addition, endothelial XBP-1 contributed to the sustained TNF-triggered NF-κB-dependent transcriptional activation of proinflammatory molecules, which was associated with leukocyte-endothelial cell adhesion. In the LSR model, endothelial cell-specific XBP-1-deficient mice displayed significantly less vascular damage, accompanied by reduced perivascular neutrophil infiltration, as compared with wild-type mice. Endothelial XBP-1 is activated by TNF and regulates leukocyte-endothelial cell adhesion in vitro as well as neutrophil infiltration and vascular damage in murine vasculitis. © 2015, American College of Rheumatology.

  1. Bilateral idiopathic retinal vasculitis following coxsackievirus A4 infection: a case report.

    Science.gov (United States)

    Mine, Izumi; Taguchi, Manzo; Sakurai, Yutaka; Takeuchi, Masaru

    2017-07-19

    Coxsackieviruses are members of a group of viruses called the enteroviruses, which may cause respiratory and gastrointestinal symptoms, erythema, meningoencephalitis, myocarditis, pericarditis, and myositis. Unilateral acute idiopathic maculopathy caused by coxsackievirus A16 has been associated with hand, foot, and mouth disease, but only a few reports describe retinitis associated with coxsackievirus serotype B3 or B4. We report a case of bilateral multifocal obstructive retinal vasculitis that developed after coxsackievirus A4 infection. A 60-year-old woman was referred to our department with bilateral visual disturbance that developed following flu-like symptoms. At the initial examination, best corrected visual acuity was 20/200 in the right eye and 20/50 in the left eye. The critical flicker frequency (CFF) was 23 Hz in the right eye and 27 Hz in the left eye. Fine white keratic precipitates with infiltrating cells were presented in the anterior chamber of both eyes, and multifocal retinal ischemic lesions were observed in the macula and posterior pole of both eyes. The retinal lesions corresponded with scotomas observed in Goldmann visual field test. On spectral domain-optical coherence tomography (SD-OCT), retinal lesions were depicted as hyper-reflective regions in the inner retina layers in both eyes, and disruption of ellipsoid line in the left eye., Fluorescein angiography exhibited findings indicative of multifocal obstructive retinal vasculitis. The patient had a history of current hypertension treated with oral therapy and glaucoma treated with latanoprost eye drops. Blood test for coxsackievirus antibody titers revealed that A4, A6, A9, B1, B2, B3, and B5 were positive (titers: 8-32). Abdominal skin biopsy of necrotic tissue suggested vascular damage caused by coxsackievirus. The general symptoms improved after 6 weeks, and the multifocal retinal ischemic lesions were partially resolved with residual slightly hard exudates. Only coxsackievirus A4

  2. [Scanning electron microscopy of peripheral blood erythrocytes in the diagnosis and treatment of hemorrhagic vasculitis in children].

    Science.gov (United States)

    Nagaeva, T A; Balasheva, I I; Saratikov, A S; Bocharova, M N; Mikhalenko, A N

    2001-01-01

    Twenty-four children aged 3-15 years were examined, 16 of these with cutaneous and articulo-cutaneous hemorrhagic vasculitis (HV) and 8 normal controls. The patients were divided into 2 groups: 10 patients treated by basic therapy and 6 children whose treatment protocols were supplemented by membranoprotector locheine. The children were repeatedly examined 1 month after discharge from hospital. Scanning electron microscopy of peripheral blood erythrocytes provides valuable diagnostic data on erythrocyte membrane morphology and function in children with HV and can serve as a method for monitoring the efficiency of new approaches to therapy of this disease.

  3. Leukocytoclastic Vasculitis in a Patient with Ankylosing Spondylitis

    Directory of Open Access Journals (Sweden)

    Senol Kobak

    2014-01-01

    Full Text Available A 26-year-old male patient presented to our rheumatology clinic with pain, swelling and limitation of movement in his right ankle, and also purpuric skin lesions in the lower extremity pretibial region. He was asked questions, and he said that he had been having chronic low back pain and morning stiffness for the last few years. His physical examination revealed that he had arthritis in his right ankle, purpuric skin lesions in pretibial regions of both legs, and bilateral FABERE/FADIR positivity. The sacroiliac joint imaging and MRI revealed bilateral sacroiliitis findings, and the lateral heel imaging revealed enthesitis. HLA-B27 was positive. Skin biopsy from lower skin lesions was reported to be consistent with leukocytoclastic vasculitis. Based on clinical, laboratory, radiological, and pathological examinations, the patient was diagnosed with ankylosing spondylitis and leukocytoclastic vasculitis. Administration of corticosteroid, salazopyrin, and nonsteroid anti-inflammatory medications was started. Notable clinical and laboratory regression was observed during his checks 3 months later.

  4. T cell-macrophage interactions and granuloma formation in vasculitis

    Directory of Open Access Journals (Sweden)

    Marc eHilhorst

    2014-09-01

    Full Text Available Granuloma formation, bringing into close proximity highly activated macrophages and T cells, is a typical event in inflammatory blood vessel diseases, and is noted in the name of several of the vasculitides. It is not known whether specific properties of the microenvironment in the blood vessel wall or the immediate surroundings of blood vessels contribute to granuloma formation and, in some cases, generation of multinucleated giant cells. Granulomas provide a specialized niche to optimize macrophage-T cell interactions, strongly activating both cell types. This is mirrored by the intensity of the systemic inflammation encountered in patients with vasculitis, often presenting with malaise, weight loss, fever and strongly upregulated acute phase responses. As a sophisticated and highly organized structure, granulomas can serve as an ideal site to induce differentiation and maturation of T cells. The granulomas possibly seed aberrant Th1 and Th17 cells into the circulation, which are known to be the main pathogenic cells in vasculitis. Through the induction of memory T cells, aberrant innate immune responses can imprint the host immune system for decades to come and promote chronicity of the disease process. Improved understanding of T cell-macrophage interactions will redefine pathogenic models in the vasculitides and provide new avenues for immunomodulatory therapy.

  5. [Urticarial vasculitis associated with essential thrombocythaemia progressing to myelofibrosis].

    Science.gov (United States)

    Koudoukpo, C; Jachiet, M; Zini, J-M; Andreoli, A; Pinquier, L; Rybojad, M; Bosset, D; de Masson, A; Bagot, M; Lebbé, C; Bouaziz, J-D

    2014-12-01

    Urticarial vasculitis (UV) is a rare form of leukocytoclastic vasculitis in which skin lesions resemble urticaria. UV comprises hypocomplementemic and normocomplementemic subtypes. To date, only 4 cases of UV associated with myeloproliferative disorders have been described, including 3 cases with essential thrombocythaemia (ET) and one case with polycythaemia vera. We describe the case of a 59-year-old male patient with JAK2-positive TE and secondary myelofibrosis and who developed multiple urticarial papules persisting for more than 24hours. Skin biopsy showed perivascular neutrophilic infiltrate with margination of neutrophils in the lumen of vessels and some leukocytoclastic patterns, and with red cell extravasation consistent with UV. Treatment with ruxolitinib (a JAK2 inhibitor) induced transient and partial control of the haematological symptoms but did not prevent UV flare. Prednisolone 20mg once daily was added, with good clinical response. To our knowledge, this is the fourth reported case of UV associated with ET and the first case associated with MF. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  6. [Chromium-induced vasculitis-like purpuric allergic contact dermatitis].

    Science.gov (United States)

    Grange, A; Roth, B; Tortel, M-C; Guillaume, J-C

    2005-12-01

    Purpuric allergic contact dermatitis is a rare and poorly understood condition. A 27-year-old male patient with a personal history of atopic dermatitis since childhood consulted for chronic papular-purpuric rash present for 7 years. Moderate pruritus was seen. Profuse lesions were observed on the palms and soles and on the upper and lower limbs, with sparing of the trunk. These lesions consisted of purpuric papules, in some cases with crusts, forming large plaques. The clinical picture was initially suggestive of vasculitis, but this diagnosis was ruled out by histological examination and laboratory tests. Skin patch tests were evocative of chromium-induced contact dermatitis. Retrospective directed history-taking confirmed the relevance of the latter test since it revealed regular wearing of leather clothing. Lasting cure was achieved following eradication of the allergen. Reports of contact purpuric dermatitis are rare. This condition has been described principally for allergens consisting of rubber or dyes used in clothing. Our case was notable on account of the severity of the lesions, mimicking vasculitis, as well as the novelty of the incriminated allergen, chromium, found in leather garments. It underlines the value of routine skin patch tests in the event of chronic non-specific dermatitis. To our knowledge, this is the first reported case of chromium-induced purpuric allergic contact dermatitis.

  7. Cognitive distance, absorptive capacity and group rationality: a simulation study.

    Directory of Open Access Journals (Sweden)

    Petru Lucian Curşeu

    Full Text Available We report the results of a simulation study in which we explore the joint effect of group absorptive capacity (as the average individual rationality of the group members and cognitive distance (as the distance between the most rational group member and the rest of the group on the emergence of collective rationality in groups. We start from empirical results reported in the literature on group rationality as collective group level competence and use data on real-life groups of four and five to validate a mathematical model. We then use this mathematical model to predict group level scores from a variety of possible group configurations (varying both in cognitive distance and average individual rationality. Our results show that both group competence and cognitive distance are necessary conditions for emergent group rationality. Group configurations, in which the groups become more rational than the most rational group member, are groups scoring low on cognitive distance and scoring high on absorptive capacity.

  8. Efficacy and Safety of Sofosbuvir Plus Daclatasvir for Treatment of HCV-Associated Cryoglobulinemia Vasculitis.

    Science.gov (United States)

    Saadoun, David; Pol, Stanislas; Ferfar, Yasmina; Alric, Laurent; Hezode, Christophe; Si Ahmed, Si Nafa; de Saint Martin, Luc; Comarmond, Cloé; Bouyer, Anne Sophie; Musset, Lucile; Poynard, Thierry; Resche Rigon, Matthieu; Cacoub, Patrice

    2017-07-01

    Circulating mixed cryoglobulins are detected in 40%-60% of patients with hepatitis C virus (HCV) infection, and overt cryoglobulinemia vasculitis (CryoVas) develops in approximately 15% of patients. Remission of vasculitis has been associated with viral clearance, but few studies have reported the effectiveness of direct-acting antiviral drugs in these patients. We performed an open-label, prospective, multicenter study of the effectiveness and tolerance of an all-oral, interferon- and ribavirin-free regimen of sofosbuvir plus daclatasvir in patients with HCV-associated CryoVas. Forty-one consecutive patients with active HCV-associated CryoVas (median age, 56 y; 53.6% women) were recruited from hospitals in Paris, France, from 2014 through 2016. They received sofosbuvir (400 mg/day) plus daclatasvir (60 mg/day) for 12 weeks (n = 32) or 24 weeks (n = 9), and were evaluated every 4 weeks until week 24 and at week 36. Blood samples were analyzed for complete blood count, serum chemistry profile, level of alanine aminotransferase, rheumatoid factor activity, C4 fraction of complement, and cryoglobulin; peripheral blood mononuclear cells were isolated for flow cytometry analysis. Thirty-seven patients (90.2%) had a complete clinical response (defined by improvement of all the affected organs involved at baseline and no clinical relapse) after a median time of 12 weeks of therapy; all had a sustained virologic response (no detectable serum HCV RNA 12 weeks after the end of antiviral therapy). Patients' mean cryoglobulin level decreased from 0.56 ± 0.18 at baseline to 0.21 ± 0.14 g/L at week 36, and no cryoglobulin was detected in 50% of patients at this time point. After antiviral therapy, patients had increased numbers of T-regulatory cells, IgM+CD21-/low-memory B cells, CD4+CXCR5+ interleukin 21+ cells, and T-helper 17 cells, compared with before therapy. After a median follow-up period of 26 months (interquartile range, 20-30 mo), no patients had a serious adverse

  9. Cluster Analysis in Minority Group Poverty Studies.

    Science.gov (United States)

    Ross, E. Lamar

    This paper, one of a series which arose out of data gathered on Choctaw Indians, Negroes, and whites in a low income area of Mississippi, expands upon one aspect of a recently completed analysis by the author. In the study, an attempt was made to distinguish between the characteristics associated with income levels and those related to ethnic…

  10. Facilitating peer learning in study groups

    DEFF Research Database (Denmark)

    Adriansen, Hanne Kirstine; Madsen, Lene Møller

    2009-01-01

    In 2008 University of Aarhus, Denmark, issued a report concerning student experience with the study environment. Among the university's eight faculties, the Danish School of Education (DPU) held the sad record of having the lowest student well-being. This led to an action research project 'Facili...... on the students' own resources, using peer-learning and facilitating these activities....

  11. In vasculitis of small muscular arteries, activation of vessel-infiltrating CD8 T cells seems to be antigen-independent.

    Science.gov (United States)

    Kobayashi, Mikiko; Ogawa, Eisaku; Okuyama, Ryuhei; Kanno, Hiroyuki

    2017-11-11

    The etiology of polyarteritis nodosa (PAN) and localized PAN is still unknown, although a T cell-mediated immune mechanism has been considered. CD8 T cells participate not only in the antigen-dependent adaptive immune system, but also in the antigen-independent innate immune system. Non-antigen-activated CD8 T cells express a unique phenotype: granzyme B (GrB) positive /CD25 negative /programmed death-1 (PD-1) negative. The aims of this study were to assess the participation of T cells, especially innate CD8 T cells, in the development of vasculitis. Twenty-eight consecutive cases of skin biopsy specimens with cutaneous vasculitis of small muscular arteries (CVSMA) were retrieved. The series comprises of 21 cases of cutaneous arteritis, three cases of PAN, and four cases of rheumatoid vasculitis. Cases of antineutrophil cytoplasmic antibody-associated vasculitis were excluded. The phenotypes of infiltrating lymphocytes in vasculitis lesions were evaluated by immunohistochemistry. In most cases of CVSMA, the number of CD8 T cells infiltrating the intima was higher than that of CD4 T cells, and significant numbers of GrB-positive cells, which represent activated CD8 T cells, were observed. However, GrB/CD25-double-positive cells, which correspond to antigen-activated T cells, were very few in a small number of cases. Cells positive for PD-1, which is also expressed on antigen-activated CD8 T cells, were not detected. We conclude that a T cell-mediated immune mechanism, involving cytotoxic CD8 T cells, may play a role in the development of CVSMA. Low expression of CD25 in activated CD8 T cells suggests that activation was antigen-independent.

  12. Watching MOOCs Together: Investigating Co-Located MOOC Study Groups

    Science.gov (United States)

    Li, Nan; Verma, Himanshu; Skevi, Afroditi; Zufferey, Guillaume; Blom, Jan; Dillenbourg, Pierre

    2014-01-01

    Research suggests that massive open online course (MOOC) students prefer to study in groups, and that social facilitation within the study groups may render the learning of difficult concepts a pleasing experience. We report on a longitudinal study that investigates how co-located study groups watch and study MOOC videos together. The study was…

  13. Study on school lunch of four groups

    OpenAIRE

    大迫, 康子; 小住, フミ子; Yasuko, OSAKO; Fumiko, OZUMI

    1984-01-01

    There are many small islands, villages and fishing ports in Kagoshima. This study was designed to investigate whether a local color in school lnuch exist or not. It was found that the school lunch served in small island had the best nutritional quantity and quality and menu contents. Contradictionus results, vitamin deficiency in village and protein deficiency in fishing ports, were also obtained. There is a correlation between lunch cost and menu contents. The shotage of potatos and beans ob...

  14. Determinants of Vessel Targeting in Vasculitis

    Directory of Open Access Journals (Sweden)

    Gary S. Hoffman

    2004-01-01

    Full Text Available Studies of autoimmune diseases have not yet elucidated why certain organs or vessels become the objects of injury while others are spared. This paper will explore the hypothesis that important differences exist in regions of the aorta that determine vulnerability to diseases, such as atherosclerosis, aortitis, giant cell arteritis and Takayasu's disease. The reader is invited to reassess; (1 whether the aorta is indeed a single homogeneous structure, and (2 whether the initial stage of aortitis (and indeed other diseases considered “autoimmune” may be primarily due to acquired alterations of substrate, that influence unique immune profiles, which by themselves may not be pathogenic. Disease susceptibility and patterns are influenced by many factors that are inborn and acquired. Examples include genetic background, gender, ethnicity, aging, prior and concomitant illnesses, habits, diet, toxin and environmental exposures. Studies of vascular diseases must assess how such variables may affect regional differences in endothelial cells, subendothelial matrix, vascular smooth muscle and the response of each to a variety of stimuli.

  15. Simultaneous automated screening and confirmatory testing for vasculitis-specific ANCA.

    Directory of Open Access Journals (Sweden)

    Mandy Sowa

    Full Text Available Anti-neutrophil cytoplasmic antibodies (ANCA are the serological hallmark of small vessel vasculitis, so called ANCA-associated vasculitis. The international consensus requires testing by indirect immunofluorescence (IIF on human ethanol-fixed neutrophils (ethN as screening followed by confirmation with enzyme-linked immunosorbent assays (ELISAs. This study evaluates the combination of cell- and microbead-based digital IIF analysis of ANCA in one reaction environment by the novel multiplexing CytoBead technology for simultaneous screening and confirmatory ANCA testing. Sera of 592 individuals including 118 patients with ANCA-associated vasculitis, 133 with rheumatoid arthritis, 49 with infectious diseases, 77 with inflammatory bowel syndrome, 20 with autoimmune liver diseases, 70 with primary sclerosing cholangitis and 125 blood donors were tested for cytoplasmic ANCA (C-ANCA and perinuclear ANCA (P-ANCA by classical IIF and ANCA to proteinase 3 (PR3 and myeloperoxidase (MPO by ELISA. These findings were compared to respective ANCA results determined by automated multiplex CytoBead technology using ethN and antigen-coated microbeads for microbead immunoassays. There was a good agreement for PR3- and MPO-ANCA and a very good one for P-ANCA and C-ANCA by classical and multiplex analysis (Cohen's kappa [κ] = 0.775, 0.720, 0.876, 0.820, respectively. The differences between classical testing and CytoBead analysis were not significant for PR3-ANCA, P-ANCA, and C-ANCA (p<0.05, respectively. The prevalence of confirmed positive ANCA findings by classical testing (IIF and ELISA compared with multiplex CytoBead analysis (IIF and microbead immunoassay positive resulted in a very good agreement (κ = 0.831 with no significant difference of both methods (p = 0.735. Automated endpoint-ANCA titer detection in one dilution demonstrated a very good agreement with classical analysis requiring dilution of samples (κ = 0.985. Multiplexing by Cyto

  16. Radiocontrast-Related Leukocytoclastic Vasculitis Misdiagnosed as Diabetic Foot Ulcer in a Type 2 Diabetic Patient: A Case Report

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    Barış Sarıakçalı

    2013-09-01

    Full Text Available The skin is the most affected tissue by many vasculitis syndromes. Leukocytoclastic vasculitis is the most common type of vasculitis syndrome and involves the small vessels. A long list of causative factors has been reported for leukocytoclastic vasculitis. Here, we present a type 2 diabetic patient who had purpuric skin lesions predominantly on the lower limbs and acute renal failure overriding to underlying chronic kidney disease due to leukocytoclastic vasculitis associated with radiocontrast administration. He was initially diagnosed as having diabetic foot ulcer at our outpatient clinic. After single dose betamethasone depot (9.6 mg i.m., skin eruptions paled and improved; renal function showed an improvement on the following days. There are few case reports on the relationship of radiocontrast agent with leucocytoclastic vasculitis. Our case had leukocytoclastic vasculitis due to radiocontrast agent exposure which is very rare in the literature. Turk Jem 2013; 17: 78-80

  17. Anti-neutrophil cytoplasmic antibody-associated vasculitis with renal involvement: Analysis of 89 cases.

    Science.gov (United States)

    Caravaca-Fontán, Fernando; Yerovi, Estefanía; Delgado-Yagu E, María; Galeano, Cristina; Pampa-Saico, Saúl; Tenorio, Maria Teresa; Liaño, Fernando

    2017-01-06

    The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis with renal involvement are associated with high morbi-mortality. In this study we analyse if the prognosis of these diseases have improved in recent years, and which factors influence the outcomes. Retrospective single-centre observational study, which included all patients diagnosed with microscopic polyangiitis and granulomatosis with polyangiitis with renal involvement in the last 25 years. Demographic, clinical and biochemical parameters of prognostic interest were recorded. The differences between four chronological periods were analysed, along with the determinants of a poor outcome (death or end-stage renal disease). Eighty-nine patients were included (mean age 64±15 years). Sixty-four patients (72%) had microscopic polyangiitis and 25 (28%) granulomatosis with polyangiitis. During the study period, 37 (42%) patients died. Through Cox regression analysis, the best determinants of mortality were the initial glomerular filtration rate (HR 0.911; P=.003), Charlson comorbidity index (HR 1.513; P<.0001) and tobacco smoking (HR 1.816; P=.003). 35% developed end-stage renal disease, and the best determinants (by competing-risk regression) were: initial glomerular filtration rate (sub-hazard ratio [SHR]: 0.791; P<.0001), proteinuria (SHR: 1.313; P<.0001), and smoking status (SHR: 1.848; P=.023). No differences were found in patients' mortality or renal survival between the different study periods. Prognosis of anti-neutrophil cytoplasm antibodies vasculitis with renal involvement treated with conventional immunosuppressive therapy remains unsatisfactory, and continues to have increased long-term complications and mortality. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  18. Dialogical Approach Applied in Group Counselling: Case Study

    Science.gov (United States)

    Koivuluhta, Merja; Puhakka, Helena

    2013-01-01

    This study utilizes structured group counselling and a dialogical approach to develop a group counselling intervention for students beginning a computer science education. The study assesses the outcomes of group counselling from the standpoint of the development of the students' self-observation. The research indicates that group counselling…

  19. Issues in trial design for ANCA-associated and large-vessel vasculitis.

    Science.gov (United States)

    Tarzi, Ruth M; Mason, Justin C; Pusey, Charles D

    2014-08-01

    Randomized clinical trials (RCTs) have informed the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, although challenges still exist. The evidence base for treating large-vessel vasculitis (LVV) is weaker, but initiatives to standardize diagnostic criteria and outcome measures, and to validate biomarkers in LVV, together with newly initiated RCTs should start to address this need. In this Perspectives, we discuss the prerequisites for RCTs in vasculitis, existing trial evidence, continuing unmet needs, potential therapeutic avenues to explore and considerations in the design of future trials.

  20. Caracterización clínica de las vasculitis cutáneas

    OpenAIRE

    Loricera García, Javier

    2015-01-01

    RESUMEN: El término de vasculitis cutánea (VC) comprende un conjunto de síndromes caracterizados por la inflamación de los vasos de la piel. Clínicamente se va a traducir fundamentalmente en una púrpura palpable e histológicamente en una vasculitis leucocitoclástica. La VC puede tratarse de un proceso primario o bien ser secundaria a una causa subyacente como vasculitis necrotizantes sistémicas, otras alteraciones del tejido conectivo, infecciones sistémicas o neoplasias. Una adecuada clasifi...

  1. Omalizumab for Urticarial Vasculitis: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Misbah Nasheela Ghazanfar

    2015-01-01

    Full Text Available Urticarial vasculitis is characterised by inflamed itching or burning red patches or wheals that resemble urticaria but persist for greater than 24 hours. It is often idiopathic but is sometimes associated with collagen-vascular disease, particularly systemic lupus erythematosus. Treatment options include oral antihistamines, oral corticosteroids, dapsone, colchicine or hydroxychloroquine. We describe a male patient with urticarial vasculitis who was treated with omalizumab (anti-IgE with convincing results and provide a review of previous reports of patients with urticarial vasculitis treated with omalizumab.

  2. Omalizumab for Urticarial Vasculitis: Case Report and Review of the Literature.

    Science.gov (United States)

    Ghazanfar, Misbah Nasheela; Thomsen, Simon Francis

    2015-01-01

    Urticarial vasculitis is characterised by inflamed itching or burning red patches or wheals that resemble urticaria but persist for greater than 24 hours. It is often idiopathic but is sometimes associated with collagen-vascular disease, particularly systemic lupus erythematosus. Treatment options include oral antihistamines, oral corticosteroids, dapsone, colchicine or hydroxychloroquine. We describe a male patient with urticarial vasculitis who was treated with omalizumab (anti-IgE) with convincing results and provide a review of previous reports of patients with urticarial vasculitis treated with omalizumab.

  3. Outras vasculites pulmonares Other forms of pulmonary vasculitis

    Directory of Open Access Journals (Sweden)

    Carmen Sílvia Valente Barbas

    2005-07-01

    Full Text Available A poliangeíte microscópica, a arterite de Takayasu, a síndrome de Behçet, a púrpura de Henoch-Schönlein e as vasculites associadas às doenças do colágeno incluem-se entre as vasculites pulmonares. Seu diagnóstico é realizado associando-se as manifestações clínicas aos achados radiológicos e anatomopatológicos.Among the various forms of pulmonary vasculitis are microscopic polyangiitis, Takayasu's arteritis, Behçet's syndrome and Henoch-Schönlein purpura, as well as those forms related to rheumatologic diseases. The diagnosis is made through analysis of clinical manifestations, together with radiological and pathological findings.

  4. Disseminated gonococcal infection presenting as vasculitis: a case report.

    Science.gov (United States)

    Jain, Sangita; Win, Htet Nwe; Chalam, Venkat; Yee, Lian

    2007-01-01

    A 50-year-old man with alcoholic liver disease presented with fever, tenosynovitis, polyarthritis and a vasculitic rash on the hands and feet for 4 days. He had neutrophilia and raised inflammatory markers. He had no history of sore throat, urethral discharge or travel abroad. His initial blood cultures were negative, and he was treated for vasculitis with steroids. The rash and arthritis seemed to improve initially, but he had another episode of fever. Repeat blood cultures grew Neisseria gonorrhoeae,and he received intravenous ceftriaxone followed by oral ciprofloxacin. He had marked improvement in rash, tenosynovitis and arthritis, and the fever dropped. He also had chlamydial urethritis and received azithromycin. The presentation of disseminated gonococcal infection after a presumptive episode of asymptomatic urethral gonorrhoea is highlighted.

  5. Kaposi′s sarcoma following immunosuppressive therapy for vasculitis

    Directory of Open Access Journals (Sweden)

    Tarik Bouattar

    2011-01-01

    Full Text Available Kaposi′s sarcoma (KS is widely reported to develop after renal transplantation and is induced by activation of a latent human herpes virus 8. We report the clinical features and outcome of a 50-year-old woman who presented with KS 18 weeks after starting immuno-suppressive therapy for vasculitis. She had positive-titer IgG antibody to human herpes virus 8. Cyclophosphamide pulses were interrupted, and prednisone was decreased gradually to 10 mg/day. Skin lesions showed important regression with stabilization of the general state and renal function. Eight months later, the patient presented with a diffuse cutaneous KS that required the discontinuation of steroids. Within 1 month, her general status and renal function deteriorated, and she died with a disseminated intravascular coagulation syndrome.

  6. Ethnic Helping and Group Identity: A Study among Majority Group Children

    NARCIS (Netherlands)

    Sierksma, J.; Thijs, J.T.; Verkuyten, M.J.A.M.

    2014-01-01

    Two vignette studies were conducted on children's evaluations of ethnic helping. In the first study, 272 native Dutch children (mean age = 10.7) evaluated a child who refused to help in an intra-group context (Dutch-Dutch or Turkish-Turkish) or inter-group context (Dutch-Turkish or Turkish-Dutch).

  7. Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis.

    Science.gov (United States)

    Jarrot, Pierre-Andre; Chiche, Laurent; Hervier, Baptiste; Daniel, Laurent; Vuiblet, Vincent; Bardin, Nathalie; Bertin, Daniel; Terrier, Benjamin; Amoura, Zahir; Andrés, Emmanuel; Rondeau, Eric; Hamidou, Mohamed; Pennaforte, Jean-Loup; Halfon, Philippe; Daugas, Eric; Dussol, Bertrand; Puéchal, Xavier; Kaplanski, Gilles; Jourde-Chiche, Noemie

    2016-05-01

    The aim of the study was to report the clinical, biological, and pathological characteristics of patients with glomerulonephritis (GN) secondary to systemic lupus erythematosus (SLE)/antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) overlap syndrome.A nationwide survey was conducted to identify cases of SLE/AAV overlap syndrome. Data were collected from SLE and AAV French research groups. Inclusion criteria were diagnosis of both SLE and AAV according to international classification criteria and biopsy-proven GN between 1995 and 2014. Additional cases were identified through a systematic literature review. A cohort of consecutive biopsy-proven GN was used to study the prevalence of overlapping antibodies and/or overlap syndrome.The national survey identified 8 cases of SLE/AAV overlap syndrome. All patients were female; median age was 40 years. AAV occurred before SLE (n = 3), after (n = 3), or concomitantly (n = 2). Six patients had rapidly progressive GN and 3/8 had alveolar hemorrhage. All patients had antinuclear antibodies (ANA); 7/8 had p-ANCA antimyeloperoxidase (MPO) antibodies. Renal biopsies showed lupus nephritis (LN) or pauci-immune GN. Remission was obtained in 4/8 patients. A literature review identified 31 additional cases with a similarly severe presentation. In the GN cohort, ANCA positivity was found in 30% of LN, ANA positivity in 52% of pauci-immune GN, with no correlation with pathological findings. The estimated prevalence for SLE/AAV overlap syndrome was 2/101 (2%).In patients with GN, SLE/AAV overlap syndrome may occur but with a low prevalence. Most patients have an aggressive renal presentation, with usually both ANA and anti-MPO antibodies. Further studies are needed to assess shared pathogenesis and therapeutic options.

  8. Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis

    Science.gov (United States)

    Jarrot, Pierre-Andre; Chiche, Laurent; Hervier, Baptiste; Daniel, Laurent; Vuiblet, Vincent; Bardin, Nathalie; Bertin, Daniel; Terrier, Benjamin; Amoura, Zahir; Andrés, Emmanuel; Rondeau, Eric; Hamidou, Mohamed; Pennaforte, Jean-Loup; Halfon, Philippe; Daugas, Eric; Dussol, Bertrand; Puéchal, Xavier; Kaplanski, Gilles; Jourde-Chiche, Noemie

    2016-01-01

    Abstract The aim of the study was to report the clinical, biological, and pathological characteristics of patients with glomerulonephritis (GN) secondary to systemic lupus erythematosus (SLE)/antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) overlap syndrome. A nationwide survey was conducted to identify cases of SLE/AAV overlap syndrome. Data were collected from SLE and AAV French research groups. Inclusion criteria were diagnosis of both SLE and AAV according to international classification criteria and biopsy-proven GN between 1995 and 2014. Additional cases were identified through a systematic literature review. A cohort of consecutive biopsy-proven GN was used to study the prevalence of overlapping antibodies and/or overlap syndrome. The national survey identified 8 cases of SLE/AAV overlap syndrome. All patients were female; median age was 40 years. AAV occurred before SLE (n = 3), after (n = 3), or concomitantly (n = 2). Six patients had rapidly progressive GN and 3/8 had alveolar hemorrhage. All patients had antinuclear antibodies (ANA); 7/8 had p-ANCA antimyeloperoxidase (MPO) antibodies. Renal biopsies showed lupus nephritis (LN) or pauci-immune GN. Remission was obtained in 4/8 patients. A literature review identified 31 additional cases with a similarly severe presentation. In the GN cohort, ANCA positivity was found in 30% of LN, ANA positivity in 52% of pauci-immune GN, with no correlation with pathological findings. The estimated prevalence for SLE/AAV overlap syndrome was 2/101 (2%). In patients with GN, SLE/AAV overlap syndrome may occur but with a low prevalence. Most patients have an aggressive renal presentation, with usually both ANA and anti-MPO antibodies. Further studies are needed to assess shared pathogenesis and therapeutic options. PMID:27258503

  9. Incidence of anti-neutrophil cytoplasmic antibody-associated vasculitis before and after the February 2011 Christchurch Earthquake.

    Science.gov (United States)

    Farquhar, H J; McGettigan, B; Chapman, P T; O'Donnell, J L; Frampton, C; Stamp, L K

    2017-01-01

    It has been suggested that environmental pollution from an earthquake might be associated with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). To determine the incidence of AAV during the 3-year period before (period 1), and the 3 years following (period 2), the earthquake that occurred on 22 February 2011 in Christchurch, New Zealand. All ANCA tests performed in the Canterbury region for 3 years before the earthquake (period 1, 2007-2010), and for 3 years after the earthquake (period 2, 2011-2014) were examined. AAV was defined according to The European Medicines Agency classification algorithm. Medical records were reviewed and cases were included if they were newly diagnosed within the study period. Incidence was calculated using population data from the 2013 New Zealand census. A total of 52 new cases of AAV was identified. The incidence in period 1 was 1.87/100 000/annum (95% C.I. 1.23-2.72), and for period 2 was 1.73/100 000/annum (95% C.I. 1.12-2.55). There was no statistically significant difference in incidence between the two study periods. There was no difference when analysing by myeloperoxidase (MPO) or proteinase-3 status, or restricting the analyses to those residing in an urban environment. The mean age at diagnosis for MPO AAV was significantly younger in period 2 than period 1 (61 years vs 71 years, P = 0.05). There were no other clinically important differences between the two groups. This study does not support the hypothesis that an environmental agent, caused by dust pollution related to earthquake damage, has a causative role in the pathogenesis of AAV. © 2016 Royal Australasian College of Physicians.

  10. The prognostic value of baseline {sup 18}F-FDG PET/CT in steroid-naive large-vessel vasculitis: introduction of volume-based parameters

    Energy Technology Data Exchange (ETDEWEB)

    Dellavedova, L. [Ospedale Civile di Legnano, PET/CT Center - Nuclear Medicine Department, Legnano (Italy); University of Milan, Department of Health Sciences, Milan (Italy); Carletto, M.; Maffioli, L.S. [Ospedale Civile di Legnano, PET/CT Center - Nuclear Medicine Department, Legnano (Italy); Faggioli, P.; Sciascera, A.; Mazzone, A. [Ospedale Civile di Legnano, Internal Medicine Department, Legnano (Italy); Del Sole, A. [University of Milan, Department of Health Sciences, Milan (Italy)

    2016-02-15

    The aim of this study was to analyse if the result of a baseline {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT scan, in large-vessel vasculitis (LVV) patients, is able to predict the course of the disease, not only in terms of presence/absence of final complications but also in terms of favourable/complicated progress (response to steroid therapy, time to steroid suspension, relapses, etc.). A total of 46 consecutive patients, who underwent {sup 18}F-FDG PET/CT between May 2010 and March 2013 for fever of unknown origin (FUO) or suspected vasculitis (before starting corticosteroid therapy), were enrolled. The diagnosis of LVV was confirmed in 17 patients. Considering follow-up results, positive LVV patients were divided into two groups, one characterized by favourable (nine) and the other by complicated progress (eight), on the basis of presence/absence of vascular complications, presence/absence of at least another positive PET/CT during follow-up and impossibility to comply with the tapering schedule of the steroid due to biochemical/symptomatic relapse. Vessel uptake in subjects of the two groups was compared in terms of intensity and extension. To evaluate the extent of active disease, we introduced two volume-based parameters: ''volume of increased uptake'' (VIU) and ''total lesion glycolysis'' (TLG). The threshold used to calculate VIU on vessel walls was obtained by the ''vessel to liver'' ratio by means of receiver-operating characteristic analysis and was set at 0.92 x liver maximum standardized uptake value in each patient. Measures of tracer uptake intensity were significantly higher in patients with complicated progress compared to those with a favourable one (p < 0.05). Measures of disease extension were even more significant and TLG emerged as the best parameter to separate the two groups of patients (p = 0.01). This pilot study shows that, in LVV patients, the

  11. Cognitive distance, absorptive capacity and group rationality : A simulation study

    NARCIS (Netherlands)

    Curseu, P.L.; Krehel, O.; Evers, J.H.M.; Muntean, A.

    2014-01-01

    We report the results of a simulation study in which we explore the joint effect of group absorptive capacity (as the average individual rationality of the group members) and cognitive distance (as the distance between the most rational group member and the rest of the group) on the emergence of

  12. Susceptibility quantitative trait loci for pathogenic leucocytosis in SCG/Kj mice, a spontaneously occurring crescentic glomerulonephritis and vasculitis model

    Science.gov (United States)

    Hamano, Y; Abe, M; Matsuoka, S; Zhang, D; Kondo, Y; Kagami, Y; Ishigami, A; Maruyama, N; Tsuruta, Y; Yumura, W; Suzuki, K

    2014-01-01

    The spontaneous crescentic glomerulonephritis-forming/Kinjoh (SCG/Kj) mouse, a model of human crescentic glomerulonephritis (CrGN) and systemic vasculitis, is characterized by the production of myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody (MPO-ANCA) and marked leucocytosis. This study was performed to identify the specific populations of leucocytes associated with CrGN and susceptibility loci for pathogenic leucocytosis. Four hundred and twenty female (C57BL/6 × SCG/Kj) F2 intercross mice were subjected to serial flow cytometry examination of the peripheral blood (PB). Kidney granulocytes and monocytes were examined histopathologically. Linkage analyses were performed with 109 polymorphic microsatellite markers. Correlation studies revealed that increase of the granulocytes, F4/80+ cells, CD3+CD4−CD8− T cells and dendritic cells (DCs) in peripheral blood (PB) were associated significantly with glomerulonephritis, crescent formation and vasculitis. In kidney sections, F4/80low cells were observed in crescent, while F4/80high cells were around the Bowman's capsules and in the interstitium. Numbers of F4/80+ cells in crescents correlated significantly with F4/80+ cell numbers in PB, but not with numbers of F4/80+ cells in the interstitium. Genome-wide quantitative trait locus (QTL) mapping revealed three SCG/Kj-derived non-Fas QTLs for leucocytosis, two on chromosome 1 and one on chromosome 17. QTLs on chromosome 1 affected DCs, granulocytes and F4/80+ cells, but QTL on chromosome 17 affected DCs and granulocytes. We found CrGN-associated leucocytes and susceptibility QTLs with their positional candidate genes. F4/80+ cells in crescents are considered as recruited inflammatory macrophages. The results provide information for leucocytes to be targeted and genetic elements in CrGN and vasculitis. PMID:24654803

  13. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis : 2-year results of a randomised trial

    NARCIS (Netherlands)

    Jones, Rachel B.; Furuta, Shunsuke; Tervaert, Jan Willem Cohen; Hauser, Thomas; Luqmani, Raashid; Morgan, Matthew D.; Peh, Chen Au; Savage, Caroline O.; Segelmark, Marten; Tesar, Vladimir; van Paassen, Pieter; Walsh, Michael; Westman, Kerstin; Jayne, David R. W.; Stegeman, C. A.

    Objectives The RITUXVAS trial reported similar remission induction rates and safety between rituximab and cyclophosphamide based regimens for antineutrophil cytoplasm antibody (ANCA)-associated vasculitis at 12months; however, immunosuppression maintenance requirements and longer-term outcomes after

  14. [Uptodate in the management and treatment of ANCA-associated vasculitis].

    Science.gov (United States)

    Belaconi, Ionela Nicoleta; Toma, Claudia Lucia; Bogdan, Miron Alexandru

    2014-01-01

    The antineutrophil cytoplasm antibody (ANCA)-associated vasculitis are heterogeneous, multisystem, autoimmune diseases characterized by necrotizing small and medium vessel vasculitis and the association with ANCA. The diagnosis and management of these patients may be challenging due to the variability of clinical features, the possibility of life-threatening events (acute renal failure or pulmonary hemorrhage) and the relative rarity of these syndromes. ANCA-associated vasculitis include granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. The treatment requires significant immunosuppression and there are frequent treatment related side effects. Although the standard protocol with cytotoxic agents and glucocorticoids has dramatically improved patient outcome, its toxic profile remains a major problem. Recent progress in evidence base and consensus in understanding the pathogenic mechanism and the quantification of disease activity further improved patient's life. Special attention was paid in refining immunosuppressive treatment to minimize his toxicity. This review will focus on evidence based treatment of ANCA-associated vasculitis.

  15. B cell epitope specificity in ANCA-associated vasculitis : does it matter?

    NARCIS (Netherlands)

    Van der Geld, YM; Stegeman, CA; Kallenberg, CGM

    Pauci-immune idiopathic small-vessel vasculitis is strongly associated with the presence of antineutrophil cytoplasm autoantibodies (ANCA). Antibodies to PR3 predominate in patients with Wegener's granulomatosis; antibodies to myeloperoxidase (MPO) are found more frequently in patients with

  16. Cutaneous lesions and finger clubbing uncovering hypocomplementemic urticarial vasculitis and hepatitis C with mixed cryoglobulinemia.

    Science.gov (United States)

    Pinto-Almeida, Teresa; Caetano, Mónica; Alves, Rosário; Selores, Manuela

    2013-01-01

    Urticarial vasculitis is a rare clinicopathologic entity characterized by urticarial lesions that persist for more than 24 hours and histologic features of leukocytoclastic vasculitis. Patients can be divided into normocomplementemic or hypocomplementemic. The authors report the case of a healthy 49-year-old woman with a 1-year history of highly pruritic generalized cutaneous lesions and finger clubbing. Laboratory tests together with histopathologic examination allowed the diagnosis of hypocomplementemic urticarial vasculitis, chronic hepatitis C and type II mixed cryoglobulinemia. The patient started symptomatic treatment and was referred to a gastroenterologist for management of the hepatitis C, with progressive improvement of the skin condition. The development of hypocomplementemic urticarial vasculitis in the context of chronic hepatitis C is exceedingly rare and possible pathogenic mechanisms are discussed.

  17. Cutaneous lesions and finger clubbing uncovering hypocomplementemic urticarial vasculitis and hepatitis C with mixed cryoglobulinemia*

    Science.gov (United States)

    Pinto-Almeida, Teresa; Caetano, Mónica; Alves, Rosário; Selores, Manuela

    2013-01-01

    Urticarial vasculitis is a rare clinicopathologic entity characterized by urticarial lesions that persist for more than 24 hours and histologic features of leukocytoclastic vasculitis. Patients can be divided into normocomplementemic or hypocomplementemic. The authors report the case of a healthy 49-year-old woman with a 1-year history of highly pruritic generalized cutaneous lesions and finger clubbing. Laboratory tests together with histopathologic examination allowed the diagnosis of hypocomplementemic urticarial vasculitis, chronic hepatitis C and type II mixed cryoglobulinemia. The patient started symptomatic treatment and was referred to a gastroenterologist for management of the hepatitis C, with progressive improvement of the skin condition. The development of hypocomplementemic urticarial vasculitis in the context of chronic hepatitis C is exceedingly rare and possible pathogenic mechanisms are discussed. PMID:24474109

  18. Vasculitis secundaria a infección por Fasciola hepática Secondary vasculitis to infection by Fasciola hepatica

    Directory of Open Access Journals (Sweden)

    Germán Málaga

    2012-09-01

    Full Text Available Se presenta el caso de un paciente varón de 38 años, procedente de una zona endémica para fascioliasis hepática en el Perú. Fue hospitalizado por presentar un cuadro de cuatro semanas de evolución; con fiebre, mialgias intensas, lesiones eritematosas y dolorosas en las regiones de extensión de las extremidades. La electromiografía y la velocidad de conducción nerviosa mostraron una miopatía inflamatoria global. La biopsia de piel evidenció una vasculitis de tipo poliarteritis nodosa. En el proceso de evaluación previa a la terapia inmunosupresora, se hallaron huevos de Fasciola hepática en el examen coproparasitológico. El diagnóstico de fascioliasis se confirmó con fas2-ELISA: 0,46 (VN There is a case of a 38 year-old male patient coming from an area where hepatic fascioliasis is endemic in Peru. He was hospitalized because he showed 4 weeks of symptoms like fever, intense myalgias, erythematous and painful injuries on limb extensions. The electromyography and nerve conduction velocity showed a global inflammatory myopathy. A skin biopsy showed polyarteritis nodosa-type vasculitis. During the evaluation process prior to the immunosuppressive therapy, hepatic Fasciola eggs were found in the parasitological examination of stools. The fascioliasis diagnosis was confirmed by fas2-ELISA: 0.46 (VN <0.20. Clinical symptoms started to subside after treatment with ticlabendazol. Contact with the patient was maintained for a year and there was no evidence of disease recurrence, and he was asymptomatic

  19. Characteristic purpura of the ears, vasculitis, and neutropenia--a potential public health epidemic associated with levamisole-adulterated cocaine.

    Science.gov (United States)

    Chung, Catherine; Tumeh, Paul C; Birnbaum, Ron; Tan, Belinda H; Sharp, Linda; McCoy, Erin; Mercurio, Mary Gail; Craft, Noah

    2011-10-01

    Dermatologists at the University of California, San Francisco recently reported two patients in the online Journal of the American Academy of Dermatology with purpura presumably induced by levamisole in contaminated cocaine. Levamisole-induced vasculitis and neutropenia has been reported elsewhere in the United States and Canada. Up to 70% of cocaine in the United States could be contaminated. We sought to describe similar cases of vasculitis associated with cocaine use. This is a retrospective case series. We report 6 remarkably similar patients seen over just the past few months with retiform purpura on the body and tender purpuric eruptions, necrosis, and eschars of the ears after cocaine use in New York and California. All of these patients had positive perinuclear antineutrophil cytoplasmic antibody values and 3 of the 6 also had an associated neutropenia. Direct immunofluorescence studies suggested an immune complex-mediated vasculitis. This case series is descriptive in nature and, because testing is not easily performed, we did not test for levamisole in the serum or blood to prove this is the causative agent. It appears the use of cocaine is associated with the peculiar clinical findings of ear purpura, retiform purpura of the trunk, and neutropenia. We believe this case series may represent the tip of the iceberg as a looming public health problem caused by levamisole. Although the direct causal relationship may be difficult to establish, the astute dermatologist or primary care physician should be able to recognize the characteristic skin lesions and should be wary of the potential development of agranulocytosis. Copyright © 2010 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  20. Characteristic purpura of the ears, vasculitis, and neutropeniae–a potential public health epidemic associated with levamisole-adulterated cocaine

    Science.gov (United States)

    Chung, Catherine; Tumeh, Paul C.; Birnbaum, Ron; Tan, Belinda H.; Sharp, Linda; McCoy, Erin; Mercurio, Mary Gail; Craft, Noah

    2013-01-01

    Background Dermatologists at the University of California, San Francisco recently reported two patients in the online Journal of the American Academy of Dermatology with purpura presumably induced by levamisole in contaminated cocaine. Levamisole-induced vasculitis and neutropenia has been reported elsewhere in the United States and Canada. Up to 70% of cocaine in the United States could be contaminated. Objective We sought to describe similar cases of vasculitis associated with cocaine use. Methods This is a retrospective case series. Results We report 6 remarkably similar patients seen over just the past few months with retiform purpura on the body and tender purpuric eruptions, necrosis, and eschars of the ears after cocaine use in New York and California. All of these patients had positive perinuclear antineutrophil cytoplasmic antibody values and 3 of the 6 also had an associated neutropenia. Direct immunofluorescence studies suggested an immune complex–mediated vasculitis. Limitations This case series is descriptive in nature and, because testing is not easily performed, we did not test for levamisole in the serum or blood to prove this is the causative agent. Conclusion It appears the use of cocaine is associated with the peculiar clinical findings of ear purpura, retiform purpura of the trunk, and neutropenia. We believe this case series may represent the tip of the iceberg as a looming public health problem caused by levamisole. Although the direct causal relationship may be difficult to establish, the astute dermatologist or primary care physician should be able to recognize the characteristic skin lesions and should be wary of the potential development of agranulocytosis. (J Am Acad Dermatol 2011;65:722-5.) PMID:21658797

  1. Group-analytic training groups for psychology students: A qualitative study

    DEFF Research Database (Denmark)

    Nathan, Vibeke Torpe; Poulsen, Stig

    2004-01-01

    This article presents results from an interview study of psychology students' experiences from group-analytic groups conducted at the University of Copenhagen. The primary foci are the significance of differences in themotivation participants'  personal aims of individual participantsfor...

  2. D-penicillamine-induced ANA (+) ANCA (+) vasculitis in pediatric patients with Wilson's disease.

    Science.gov (United States)

    Lee, Yeonhee; Lee, Sang Taek; Cho, Heeyeon

    2016-05-01

    Anti-neutrophil cytoplasmic antibodies (ANCA) are associated with systemic vasculitis. The pathophysiology of ANCA-associated vasculitis (AAV) has not been clearly proven, and drug-induced ANCA-associated vasculitis has been reported. Wilson's disease is an inborn error of copper metabolism caused by a mutation in the copper transporting gene ATP7B, and traditional treatment is based on copper chelation with agents such as D-penicillamine. There have been rare reports that prolonged D-penicillamine therapy might cause adverse renal events such as membranous nephropathy and minimal change disease, but it is questionable if D-penicillamine induces ANCA-associated vasculitis. We describe 2 patients with Wilson's disease treated with D-penicillamine who presented with ANCA (+) vasculitis and renal involvement. The 2 patients also showed positive results for antinuclear antibody (ANA). Their kidney biopsy findings were compatible with crescentic/necrotizing glomerulonephritis, pauci-immune type. After diagnosis of AAV, D-penicillamine was stopped. Patients were then treated with plasmapheresis and immunosuppressants, including methylprednisolone pulse therapy and intravenous cyclophosphamide. One patient progressed to end-stage renal disease and the other showed persistent proteinuria. These cases suggest that D-penicillamine may induce ANA (+) ANCA (+) vasculitis with severe renal involvement in pediatric patients, and plasmapheresis combined with immunosuppressant should be considered.

  3. Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case report.

    Science.gov (United States)

    Park, Chuiyoung; Choi, Seung Won; Kim, Misung; Park, Jongha; Lee, Jong Soo; Chung, Hyun Chul

    2014-10-22

    Hypocomplementemic urticarial vasculitis syndrome is a rare disorder characterized by chronic urticarial vasculitis, arthralgia, arthritis, and hypocomplementemia. Previously, only six patients with concomitant hypocomplementemic urticarial vasculitis syndrome, Jaccoud's arthropathy, and valvular heart disease have been reported. A 30-year-old Korean man presented with hypocomplementemic urticarial vasculitis syndrome. In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema. He also developed nephritis with azotemia. His renal biopsy results revealed membranoproliferative glomerulonephritis, type I. He showed a partial response to a combination therapy of steroid, cyclophosphamide, and mycophenolate mofetil. We describe, to the best of our knowledge, the first case of glomerulonephritis presenting a arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis syndrome. A combination of corticosteroids, cyclophosphamide, and mycophenolate mofetil appear to be a safe and effective treatment for nephropathy, however are less effective for cutaneous vasculitis, cardiac valvulopathy, and arthropathy.

  4. Minocycline-induced polyarteritis nodosa-like vasculitis presenting as brainstem stroke.

    Science.gov (United States)

    Klaas, James P; Matzke, Thomas; Makol, Ashima; Fulgham, Jimmy R

    2015-05-01

    Minocycline use has been associated with the development of autoimmune disorders, including drug-induced vasculitis. Previously published reports suggest that clinical manifestations are limited to cutaneous, constitutional, or musculoskeletal symptoms. To our knowledge there has been only one reported patient with ischemic stroke in the setting of minocycline-induced vasculitis. We describe a 26-year-old woman, with no vascular risk factors, who had an ischemic pontine stroke in the setting of biopsy-proven minocycline-induced polyarteritis nodosa-like vasculitis. Discontinuation of minocycline resulted in resolution of the vasculitis, and she has not had any recurrent ischemic events. This report shows that ischemic strokes may occur as a result of minocycline-induced vasculitis. While this is likely a rare association, recognition is important given the widespread use of minocycline and the potential for devastating consequences in a young population. Consequently, drug-induced vasculitis should be considered in patients with an ischemic stroke taking minocycline. Copyright © 2014 Elsevier Ltd. All rights reserved.

  5. Leukocytoclastic vasculitis in an adolescent with ulcerative colitis: Report of a case and review of the literature

    Directory of Open Access Journals (Sweden)

    G Tyler Butts

    2014-08-01

    Full Text Available An adolescent female with long-standing, difficult-to-control ulcerative colitis developed leukocytoclastic vasculitis, a rare cutaneous extra-intestinal manifestation of the inflammatory bowel disease. The authors provide a literature review on leukocytoclastic vasculitis complicating ulcerative colitis. Furthermore, the clinical features of leukocytoclastic vasculitis are compared and contrasted with the more common cutaneous extra-intestinal manifestations of inflammatory bowel disease, erythema nodosum, and pyoderma gangrenosum.

  6. Efficacy of TNF-α antagonist and other immunomodulators in the treatment of patients with ophthalmologic manifestations of Behcet's disease and HLA B51 positive vasculitis

    Directory of Open Access Journals (Sweden)

    Zlatanović Gordana

    2012-01-01

    Full Text Available Bacground/Aim. Behcet's disease is genetically conditioned, immune-mediated multisystem occlusive vasculitis of small blood vessels, espesially venules, of unknown etiology. The aim of this study was to analyze the clinical features, disease activity and therapy of the patients with ophthalmologic manifestation of Behcet's disease. Methods. In this study symptoms and signs of the disease were analyzed both prospectively and retrospectively during the active manifestation of the disease. The diagnosis was reached according to the International Criteria for Behcet's Disease (2006. The treatment effects were evaluated based on the presence of the best corrected visual acuity and the inflammation of the vitreous humour before and after the application of our therapeutic method. The applied therapeutic modality consisted of the primary application of corticosteroid therapy in the active stage of the disease complemented with the choice of drugs from the immunosuppressive group. In this study there drugs were cyclosporine or methotrexate. A treatment refractory patients with poor vision prognosis were treated with a third drug, the biological preparation infliximab, a tumor necrosis factor-alpha (TNF-α antagonist. Results. The mean age of 11 patients with ophthalmologic manifestation of Behcet's disease was 50.6 years. HLA B-5(51 was positive in 81% of the patients while 36% of the patients had positive pathergy test. Changes in affected eyes included vitritis (100%, posterior uveitis (45%, panuveitis (54%, retinal vasculitis (54%, cystoid macular edema (54%, and cystoid degeneration (18%. Increased intraocular pressure was observed in 27% of the patients. There was no statistically significant variation in disease activity parameters in any of the patients (p > 0.05. A statistically significant improvement in visual acuity (p < 0.05 and a high statistically significant decrease of inflammation of the worst affected eyes (p = 0.001 were detected

  7. Speaking up in groups: a cross-level study of group voice climate and voice.

    Science.gov (United States)

    Morrison, Elizabeth Wolfe; Wheeler-Smith, Sara L; Kamdar, Dishan

    2011-01-01

    Despite a growing body of research on employee voice—defined as the discretionary communication of ideas, suggestions, or opinions intended to improve organizational or unit functioning—the effects of shared or collective-level cognitions have received scant attention. There has also been relatively little research on voice within work groups. Our goal in this study was to address these important gaps by focusing on the effects of group-level beliefs about voice (i.e., group voice climate) on individual voice behavior within work groups. We conducted a cross-level investigation of voice behavior within 42 groups of engineers from a large chemical company. Consistent with our hypotheses, group voice climate was highly predictive of voice and explained variance beyond the effects of individual-level identification and satisfaction, and procedural justice climate. Also consistent with predictions, the effect of identification on voice was stronger in groups with favorable voice climates. These findings provide evidence that voice is shaped not just by individual attitudes and perceptions of the work context, as past research has shown, but also by group-level beliefs. The results also highlight the importance of broadening our conceptual models of voice to include shared cognitions and of conducting additional cross-level research on voice.

  8. Comparative Study on Liver Enzymes Activity and Blood Group ...

    African Journals Online (AJOL)

    The aim of this study is to determine the activities of some selected liver enzymes amongst apparently healthy subjects of different blood groups. The study involved 95 apparently healthy students of Ambrose Alli University, Ekpoma, Edo State, Nigeria, between the ages of 18-30, and distributed as follows; blood group O ...

  9. A case study of psychodynamic group psychotherapy for bipolar disorder.

    Science.gov (United States)

    Gonzalez, Jodi M; Prihoda, Thomas J

    2007-01-01

    This study examines the effectiveness of long-term outpatient psychodynamic group psychotherapy for individuals with bipolar disorder who may have psychiatric comorbidity. The Integrative Outpatient Model (IOM) includes psychoeducation regarding illness, illness management, and exploration of dynamic and interpersonal issues. At one-year follow up, group members had significantly less depressive symptomatology and were less likely to be in a mood episode, compared to controls. There were no between-group differences in manic symptoms or global assessments of functioning. For group-treated patients, the number of days well per week improved significantly; there were no significant improvements in the control group. This pilot study indicates that psychodynamic group psychotherapy is feasible as a component of treatment for bipolar disorder diagnoses. The results suggest benefits on depressive, but not manic symptoms. The 73% retention rate suggests that further study of this treatment is warranted, especially in the cases of patients for whom pharmacotherapy alone is not sufficient.

  10. Thalidomide in treatment of connective diseases and vasculities

    Directory of Open Access Journals (Sweden)

    D. Ribatti

    2011-09-01

    Full Text Available Thalidomide is an immunomodulatory, anti-inflammatory and anti-angiogenic drug. Thalidomide exerts its effects by decreasing circulating CD4 positive T-cells and stimulating CD8 positive T-cells, by increasing the number of Natural Killer cells and T-helper 2 cells. Thalidomide also inhibits proliferation of stimulated T-cells and leukocyte chemotaxis. It modifies a number of integrin receptors and other leukocytic surface receptors and down-modulates cell-adhesion molecules involved in leukocyte migration. It has been demonstrated that thalidomide inhibits TNFa, IL-5, IL-6, IL-8, IL-12 production and increases production of IL-2, IL-10 and INFg. Moreover thalidomide plays an important role in inhibition of VEGF and FGF-2 mediated angiogenesis. Although the exact mechanism of action is not fully understood and only limited treatment opinions exist, thalidomide plays a role also in connective diseases and vasculities. Thalidomide has been seen efficacious in the treatment of cutaneous disorders in patients with systemic lupus erythematosus and in mucocutaneous disease in Behçet’s disease with a not dose-dependent response, even if it should be restricted to selected patients because of its important side effects.

  11. Normocomplementaemic Urticarial Vasculitis in a 19-Month-Old Girl

    Science.gov (United States)

    2016-01-01

    Urticaria is common in children. Urticarial vasculitis (UV) is a potentially more serious, rare variant. The youngest reported case was 12 months of age. A systemically well, 19-month-old girl presented with her mother who was concerned about the development of a rash. On presentation, the child had normal vital signs, was alert, and was well and playing with toys. There was a widespread urticarial rash (raised, pruritic, and erythematous) that was most apparent on the trunk with minimal rash on the legs. Overlying this urticarial rash in a similar distribution was a blotchy, palpable purpuric rash and associated hyperpigmentation. Investigations revealed a normal level of haemoglobin, white cells, platelets, and electrolytes. Renal function, international normalised ratio, and activated partial thromboplastin time were all normal. There was no blood or protein in the urine. The erythrocyte sedimentation rate was mildly elevated at 19 mm/hour. Complement results (including C1q) obtained later were normal. This case is striking not only because of the rarity of UV in children but also due to the unique diagnostic and prognostic challenges that it raises. PMID:27818822

  12. Normocomplementaemic Urticarial Vasculitis in a 19-Month-Old Girl

    Directory of Open Access Journals (Sweden)

    Peter Williams

    2016-01-01

    Full Text Available Urticaria is common in children. Urticarial vasculitis (UV is a potentially more serious, rare variant. The youngest reported case was 12 months of age. A systemically well, 19-month-old girl presented with her mother who was concerned about the development of a rash. On presentation, the child had normal vital signs, was alert, and was well and playing with toys. There was a widespread urticarial rash (raised, pruritic, and erythematous that was most apparent on the trunk with minimal rash on the legs. Overlying this urticarial rash in a similar distribution was a blotchy, palpable purpuric rash and associated hyperpigmentation. Investigations revealed a normal level of haemoglobin, white cells, platelets, and electrolytes. Renal function, international normalised ratio, and activated partial thromboplastin time were all normal. There was no blood or protein in the urine. The erythrocyte sedimentation rate was mildly elevated at 19 mm/hour. Complement results (including C1q obtained later were normal. This case is striking not only because of the rarity of UV in children but also due to the unique diagnostic and prognostic challenges that it raises.

  13. Henoch-Schönlein purpura (IgA vasculitis developing after postoperative wound infection by methicillin-resistant Staphylococcus aureus

    Directory of Open Access Journals (Sweden)

    Masataka Satoh

    2016-04-01

    Full Text Available Henoch-Schönlein purpura (HSP is an acute small-vessel leukocytoclastic vasculitis, affecting the skin, joints, gastrointestinal tract and kidneys. Its prognosis depends on the severity of nephritis. A wide variety of pathogens, drugs, and other environmental exposures have been associated with HSP. Although group A β-haemolytic streptococcus has been the most studied, the majority of cases showed no direct link to streptococcal infection. Here we report a case of methicillin-resistant Staphylococcus aureus (MRSA infection-associated HSP. A 68-year-old woman underwent a coronary artery bypass surgery. After the surgery, a postoperative chest wound was infected by MRSA and sternal osteomyelitis developed. Palpable purpura then appeared on the extremities, followed by hematuria, proteinuria and increased serum creatine. Treatments with antibiotics and debridement of the infected wound and sequestrum resulted in rapid improvement of skin symptoms. Renal function partially recovered, however mild hematuria and proteinuria remained. Published work review and the present case suggest that Staphylococcal infection-associated HSP frequently involves kidney disease and its prognosis is likely to be poor compared to a common type of HSP. Further studies are needed to establish an appropriate treatment strategy for Staphylococcal infection-associated HSP.

  14. Fibrosis pulmonar asociada a vasculitis con anticuerpos anticitoplasmáticos positivos Pulmonary fibrosis associated with anti-neutrophil cytoplasmic antibody-positive vasculitis

    Directory of Open Access Journals (Sweden)

    Marcelo Fernández Casares

    2012-08-01

    Full Text Available Las complicaciones pulmonares más conocidas de las vasculitis con anticuerpos anticitoplasmáticos de los neutrófilos (ANCA positivos (VAA, son la hemorragia alveolar, los granulomas y la estenosis de la vía aérea. En los últimos años han aparecido algunos informes aislados que muestran la asociación con fibrosis pulmonar (FP, sugiriendo que ésta sería otra complicación de las VAA. En este trabajo informamos dos casos con dicha asociación describiendo sus características clínicas, tomográficas e inmunológicas. Dado que en la asociación de FP y VAA notificada en los últimos años, la FP puede ser su primera manifestación, podría ser necesaria la búsqueda de ANCA en pacientes con FP, como causa de la misma y por el posible desarrollo posterior de vasculitis.The most frequently observed pulmonary complications of vasculitis (AAV with anti-neutrophil cytoplasmic positive antibodies (ANCA are alveolar hemorrhage, granulomas and airway stenosis. In recent years, some reports have been published that show the association of vasculitis with pulmonary fibrosis (PF, suggesting that it may be another complication of AAV. We report and describe here two cases with such association, and their clinical, tomographic and immunological characteristics. Given that in the association between PF and AAV, as reported in the last years, PF could be the first manifestation of AAV, the search for ANCA in patients with PF may be necessary, as a cause of it and for the possible subsequent development of vasculitis.

  15. Measuring Peer Group Effects: A Study of Teenage Behavior.

    OpenAIRE

    Evans, William N.; Wallace E. Oates; Schwab, Robert M

    1992-01-01

    Individuals or households often have some scope for choice of peer groups, whether through the selection of neighborhood of residence, school, or friends. This study addresses the estimation of peer group effects in cases in which measures of peer group influence are potentially endogenous variables. Using a rich data set on individual behavior, the paper explores teenage pregnancy and school dropout behavior. For both cases, the estimation of a straightforward single-equation model yields st...

  16. Religious and national group identification in adolescence: a study among three religious groups in Mauritius.

    Science.gov (United States)

    Ng Tseung-Wong, Caroline; Verkuyten, Maykel

    2013-01-01

    Religious group identification is an important but understudied social identity. The present study investigates religious group identification among adolescents of different faiths (Hindu, Muslim, Christian) living in multicultural Mauritius. It further explores how religious and national group identities come together among religious majority and minority adolescents. For three age groups (11 to 19 years, N = 2152) we examined the strength of adolescents' religious and national group identification, the associations between these two identities, and the relationships to global self-esteem. Across age and religious group, participants reported stronger identification with their religious group than with the nation. Identification with both categories declined with age, with the exception of Muslims, whose strong religious identification was found across adolescence. The association between religious and national identification was positive, albeit stronger for the majority group of Hindus and for early adolescents. We examined the manner in which religious and national identities come together using a direct self-identification measure and by combining the separate continuous measures of identification. Four distinct clusters of identification (predominant religious identifiers, dual identifiers, neutrals, and separate individuals) that were differently associated with global self-esteem were found. Dual identifiers reported the highest level of global self-esteem. The clusters of identification did not fully correspond to the findings for the direct self-identification measure. The results are discussed in terms of the meaning of dual identity and the positive manner in which adolescents can manage their multiple identities while taking into account the ideological framework in which those identities are played out.

  17. Content-Related Interactions in Self-initiated Study Groups

    Science.gov (United States)

    Christian, Karen; Talanquer, Vicente

    2012-09-01

    The central goal of the present exploratory study was to investigate the nature of the content-related interactions in study groups independently organized by college organic chemistry students. We were particularly interested in the identification of the different factors that affected the emergence of opportunities for students to co-construct understanding and engage in higher levels of cognitive processing. Our results are based on the analysis of in situ observations of 34 self-initiated study sessions involving over a 100 students in three academic semesters. The investigation revealed three major types of social regulation processes, teaching, tutoring, and co-construction in the observed study sessions. However, the extent to which students engaged in each of them varied widely from one session to another. This variability was mostly determined by the specific composition of the study groups and the nature of the study tasks in which they were engaged. Decisions about how to organize the study session, the relative content knowledge and conceptual understanding expressed by the participants, as well as the cognitive level of the problems that guided group work had a strong impact on the nature of student interactions. Nevertheless, group talk in the observed study groups was mostly focused on low-level cognitive processes. The results of our work provide insights on how to better support students' productive engagement in study groups.

  18. STUDIES ON HUMAN FALLOPIAN TUBAL EPITHELIUM IN DIFFERENT AGE GROUPS

    Directory of Open Access Journals (Sweden)

    Jayasri

    2016-02-01

    Full Text Available BACKGROUND AND AIMS The “fallopian tubes” (oviducts or uterine tubes are long paired flexuous reproductive organ which transports ova, spermatozoa, zygotes, the pre-implantation morulae and blastocyst. It has major role during reproductive period, but it remains as if vestigial organ before puberty and after menopause. Due to increasing rate of tubal block and infertility, oviducts and their structures gaining importance and have become a subject of research in present days particularly epithelium. The aim of the study is to ascertain any histological difference of tubal epithelium in different age groups and the research work could be utilized for investigation and management of infertility. MATERIALS AND METHODS Seven samples of each group i.e., prereproductive, reproductive & postmenopausal were collected from fresh unembalmed human cadavers received in the department of Anatomy, FAA Medical College, Barpeta, Assam. The slides were prepared using the standard laboratory procedure. Under low and high power objectives the type of cells were observed and epithelial height was measured in the different segments. Stress was given for any significant difference of epithelial height between the different age groups. RESULTS Study revealed that among the groups within the same segment, epithelial height was recorded highest (33.57µm in reproductive group as against the lowest (22.91µm in post-menopausal group. Epithelial structures of the prereproductive and reproductive groups were significantly differed (p<0.01 from the postmenopausal group. CONCLUSIONS From the findings of the present study it can be concluded that: 1. In all the groups fallopian tubal epithelium is of simple columnar type and contains three types of cells. Cells are ciliated, secretory & peg (intercalary cells. 2. In all the groups same type of increasing trend of epithelial height from intramural segment to ampullary segment was recorded. 3. In intergroup comparison of

  19. A Case Of Primary Central Nervous System Vasculitis Who Presented With Status Epilepticus

    Directory of Open Access Journals (Sweden)

    Sırma Geyik

    2014-12-01

    Full Text Available Primary central nervous system vasculitis (PCNV is limited with central nervous system and rare vasculitis that mostly seen in middle-aged men. PCNV vasculitis is usually presented that headache, dementia, stroke and multifocal common neurological symptoms. PCNV especially involves small medium-sized leptomeningeal and cortical arteries. 43 years old male patient who have been progressive forgetfulness and headache for 3 years. He applied with recurrent that before starting right focal and than sprawling whole body which generalized tonic-clonic seizures to us. During management that he was transfered to the intensive care unit due to status epilepticus (SE. Later than we found right hemiparesis, motor aphasia and right babinski positivity in neurologic examination. Diffusion restriction was revealed in left MCA territory in diffusion magnetic resonance imaging(MRI. EEG showed two types abnormality that a slow background ritm and epileptiform activity. Biochemistry of blood, complete blood count, blood sedimentation rate, CRP and markers of vasculitis were found in the normal range. Cerebral anjiography revealed that irregularities in the distal vascular areas and fusiform aneurysm at the top of basilar artery. He was consulted with rheumatology and diagnosed central nervous system vasculitis with the existing findings. Biopsy couldn't be taken from the brain to verify the diagnosis. Finally, we applied treatment that pulse steroid and cyclophosphamide to patient. This case has been presented due to emphasize that PCNV rarely may play a role in the etiology of recurrent stroke and status epilepticus.

  20. Piezoelectricity in quasicrystals: A group-theoretical study

    Indian Academy of Sciences (India)

    and tabulated employing a compact notation. The results of this group-theoretical study are briefly discussed. Keywords. Quasicrystals; pentagonal and icosahedral point groups; piezoelectricity; non-vanishing and independent tensor coefficients; irreducible representations; compo- sition series. PACS Nos 62.40+i; 77.60+v.

  1. Value of biomarkers for predicting immunoglobulin A vasculitis nephritis outcome in an adult prospective cohort.

    Science.gov (United States)

    Berthelot, Laureline; Jamin, Agnès; Viglietti, Denis; Chemouny, Jonathan M; Ayari, Hamza; Pierre, Melissa; Housset, Pierre; Sauvaget, Virginia; Hurtado-Nedelec, Margarita; Vrtovsnik, François; Daugas, Eric; Monteiro, Renato C; Pillebout, Evangeline

    2017-11-03

    Henoch-Schönlein purpura, more recently renamed immunoglobulin A vasculitis (IgAV), is a systemic vasculitis characterized by IgA deposits. The current markers used to assess IgAV inaccurately evaluate the risk of nephritis occurrence and its long-term outcomes. The current study assessed biomarkers of nephritis outcomes. This French multicentre prospective study enrolled 85 adult patients at the time of disease onset. Patients were assessed for clinical and biological parameters and re-examined after 1 year. Immunoglobulins, cytokines, IgA glycosylation, IgA complexes and neutrophil gelatinase-associated lipocalin (NGAL) concentrations were assessed in blood and urine. We identified 60 patients with IgAV-related nephritis (IgAV-N) and 25 patients without nephritis (IgAV-woN). At the time of inclusion (Day 1), the serum levels of galactose-deficient IgA1 (Gd-IgA1) and urinary concentrations of IgA, IgG, IgM, NGAL, interleukin (IL)-1β, IL-6, IL-8, IL-10, IgA-IgG and IgA-sCD89 complexes were higher in the IgAV-N patients than in the IgAV-woN patients (P < 0.005 for all comparisons). After follow-up (1 year), 22 patients showed a poor outcome. Among the tested markers, urine IgA at disease onset adequately reclassified the risk of poor outcome over conventional clinical factors, including estimated glomerular filtration rate, proteinuria and age (continuous net reclassification improvement = 0.72, P = 0.001; integrated discrimination improvement = 0.13, P = 0.009) in IgAV patients. Taken together, these results showed that serum Gd-IgA1 and urinary IgA, IgG, IgM, NGAL, IL-1β, IL-6, IL-8, IL-10, IgA-IgG and IgA-sCD89 complexes were associated with nephritis in IgAV patients. Urinary IgA level may improve patient risk stratification for poor outcome.

  2. A Study of Decomposer and Related Functions on Groups

    Directory of Open Access Journals (Sweden)

    M. H. Hooshmand

    2015-09-01

    Full Text Available Decomposer functions in algebraic structures are studied in many recent papers. They have close relations to factorization by two subsets. Also, idempotent endomorphisms form a class of (strong decomposer functions in groups. Now, if the algebraic structure is a group, then by introducing a type of local homomorphisms we obtain several properties and equivalent conditions for many classes of decomposer functions and get a new result regarding to factorization of a group by its two subsets. Moreover, we prove existence of (two-sided decomposer type functions in non-simple groups

  3. Peer Study Groups as Catalyst for Vocational Exploration

    Science.gov (United States)

    Arendale, David R.; Hane, Amanda R.

    2016-01-01

    Postsecondary peer assisted learning programs often cite improving academic achievement for students. This qualitative study investigated the potential effect of serving as student facilitators of a peer study group on their future vocation. This was a replication of previous studies of personal and professional outcomes for study group…

  4. STUDIES ON HUMAN FALLOPIAN TUBAL EPITHELIUM IN DIFFERENT AGE GROUPS

    National Research Council Canada - National Science Library

    Jayasri; Tribeni

    2016-01-01

    .... The aim of the study is to ascertain any histological difference of tubal epithelium in different age groups and the research work could be utilized for investigation and management of infertility...

  5. Mapping Future Education and Training: Group Concept Mapping Study

    NARCIS (Netherlands)

    Stoyanov, Slavi; Hoogveld, Bert; Kirschner, Paul A.

    2010-01-01

    Stoyanov, S., Hoogveld, A. W. M., & Kirschner, P. A. (2010). Mapping Future Education and Training: Group Concept Mapping Study. Heerlen, The Netherlands: Open University of the Netherlands; EU Forlic project.

  6. Open mic: Introduction to the CERN Study Group

    CERN Multimedia

    CERN. Geneva

    2016-01-01

    Mozilla Study Groups are knowledge- and skill-sharing meet-ups for people to get help with their research or work on open-science projects. A CERN chapter was launched recently and you are invited to participate!

  7. Cognitive group therapy for depressive students: The case study

    OpenAIRE

    Tiuraniemi, Juhani; Korhola, Jarno

    2009-01-01

    The aims of this study were to assess whether a course of cognitive group therapy could help depressed students and to assess whether assimilation analysis offers a useful way of analysing students' progress through therapy. ?Johanna? was a patient in a group that was designed for depressive students who had difficulties with their studies. The assimilation of Johanna's problematic experience progressed as the meetings continued from level one (unpleasant thoughts) to level six (solving the p...

  8. Cognitive group therapy for depressive students: The case study

    OpenAIRE

    Tiuraniemi, Juhani; Korhola, Jarno

    2009-01-01

    The aims of this study were to assess whether a course of cognitive group therapy could help depressed students and to assess whether assimilation analysis offers a useful way of analysing students' progress through therapy. "Johanna" was a patient in a group that was designed for depressive students who had difficulties with their studies. The assimilation of Johanna's problematic experience progressed as the meetings continued from level one (unpleasant thoughts) to level six (solving the p...

  9. Association of Lupus Anticoagulant With Long‐Term Damage Accrual in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

    National Research Council Canada - National Science Library

    Jordan, Natasha; D'cruz, David P

    2016-01-01

    ... and other immunosuppressive therapies. Box Significance & Innovations Antiphospholipid antibodies, particularly lupus anticoagulant, are prevalent in antineutrophil cytoplasmic antibody–associated vasculitis (AAV) patients. The vasculitis damage index is significantly higher in AAV with persistently positive antiphospholipid antibodies or a concurrent diagnosis of t...

  10. Levamisole-induced occlusive necrotising vasculitis in cocaine abusers: An unusual cause of skin necrosis and neutropenia

    Science.gov (United States)

    Belfonte, Cassius Diego; Shanmugam, Victoria Kate; Kieffer, Nicole; Coker, Shodeinde; Boucree, Suelyn; Kerr, Gail

    2013-01-01

    We present three cases describing the various skin manifestations of presumed levamisole-contaminated cocaine use. Antibody-mediated vasculitis and neutropenia were consistent findings in these cases and repeat exposure resulted in distinct dermatologic complications. This phenomenon of levamisole-induced vasculitis and neutropenia is being increasingly described and has characteristic wound manifestations that must be recognised and treated early. PMID:22716045

  11. Levamisole-Contaminated Cocaine: An Emergent Cause of Vasculitis and Skin Necrosis

    Directory of Open Access Journals (Sweden)

    Osama Souied

    2014-01-01

    Full Text Available The prevalence of cocaine adulterated with levamisole-induced vasculitis is increasing and physicians should be aware of this unique entity. There have been many reports of cutaneous vasculitis syndrome caused by cocaine which is contaminated with levamisole. Levamisole was used as an antihelminth drug and later was rescinded from use in humans due to adverse effects. Through this paper, we will report a 39-year-old crack cocaine user who presented with purpuric rash and skin necrosis of his ear lobes. Levamisole-induced vasculitis syndrome was suspected. A urine toxicology screen was positive for cocaine, opiates, and marijuana. Blood work revealed positive titres of ANA and p-ANCA, as well as anti-cardiolipin antibody. Biopsy taken from the left ear showed focal acute inflammation, chronic inflammation with thrombus formation, and extravasated blood cells. Treatment was primarily supportive with wound care.

  12. Intravascular crystal deposition: an early clue to the diagnosis of type 1 cryoglobulinemic vasculitis.

    Science.gov (United States)

    Gammon, Bryan; Longmire, Michelle; DeClerck, Brittney

    2014-09-01

    Cutaneous small vessel vasculitis (CSVV) is a nonspecific finding with an extensive differential diagnosis. It is critically important to distinguish skin-limited presentations of CSVV from severe life-threatening systemic vasculitides presenting with CSVV as an initial manifestation. It can be challenging to determine which patients presenting with CSVV are at risk for systemic disease. Standard histopathologic evaluation, direct immunofluorescence, and serologic evaluation is typically required to exclude a systemic vasculitis. Type 1 cryoglobulinemia may rarely present with CSVV. Herein, we report a case of type 1 cryoglobulinemia in the setting of occult multiple myeloma. CSVV with prominent intravascular crystal formation was noted. The presence of intravascular crystals in the setting of CSVV may represent an important early clue to the diagnosis of type 1 cryoglobulinemic vasculitis.

  13. Delayed diagnosis of ocular syphilis that manifested as retinal vasculitis and acute posterior multifocal placoid epitheliopathy

    Directory of Open Access Journals (Sweden)

    Jong Hoon Park

    2013-01-01

    Full Text Available A 55-year-old female presented with bilateral progressive retinal vasculitis. She was on systemic and intravitreal steroids on the basis of uveitis work-up result (negative result including rapid plasma reagin, but her visual acuity continued to deteriorate to light perception only. Ocular examination showed retinal vasculitis, multiple yellow placoid lesions and severe macula edema in both eyes. Repeated work-up revealed positivity of fluorescent treponemal antibody-absorption in serum and subsequently in cerebrospinal fluid. Ocular syphilis was diagnosed. And intravenous penicillin G resulted in rapid resolution of vasculitis and macular edema. To avoid delay in the diagnosis of ocular syphilis, high index of suspicion and repeating serological tests (including both treponemal and non-treponemal tests are warranted.

  14. Cocaine-induced vasculitis with cutaneous manifestation: A recurrent episode after 2 years

    Directory of Open Access Journals (Sweden)

    Thein Swe

    2016-01-01

    Full Text Available Cocaine is a popular recreational drug in the United States, and up to 70% of the seized cocaine contains levamisole which is an antihelminthic that can cause cutaneous vasculitis with necrosis and positive antineutrophil cytoplasmic antibodies (ANCAs. Here, we report a unique case of recurrent cocaine-induced vasculitis in a patient who smokes cocaine for more than 20 years. A 38-year-old woman complained of painful erythematous rash in her right arm and right thigh which appeared some hours after smoking cocaine. Physical examination revealed tender, erythematous base, retiform purpura with necrosis and bullae. Serological test showed high atypical perinuclear ANCA titer of 1:320 and antimyeloperoxidase antibody level of 20.4 U/mL. Cocaine-induced vasculitis should be one of the differential diagnoses in cocaine abusers who present with painful rash and areas of necrosis. Early diagnosis is important since it is an emerging public health concern.

  15. Acute kidney injury with granulomatous interstitial nephritis and vasculitis revealing sarcoidosis

    Directory of Open Access Journals (Sweden)

    Amel Harzallah

    2017-01-01

    Full Text Available Sarcoidosis is an inflammatory disease that affects mostly the lungs and lymph glands. Renal involvement is rare and especially vasculitis. We report a case who presented an acute kidney failure and had sarcoidosis with vasculitis and nodular splenic involvement. A 35-year-old woman presenting a Lofgren syndrome was hospitalized for acute renal failure with cervical lymphadenopathy without other clinical findings. Laboratory data disclosed elevated angiotensin converting enzyme serum level. Abdominal ultrasound showed a multinodular spleen. Renal histology revealed granulomatous interstitial nephritis with necrotizing vasculitis. Outcome was favorable after the institution of high dose corticosteroids along with cyclophosphamide. Renal involvement is rare in sarcoidosis. However, the diagnostic delay should be avoided to improve the outcome.

  16. Down-Syndrome associated with MBL-deficiency, IgG-deficiency, vasculitis and mutated prothrombin.

    Science.gov (United States)

    Wolf, Hermann M; Stöllberger, Claudia; Finsterer, Josef

    2009-01-01

    The association of Down syndrome with mannose-binding lectin (MBL)-deficiency, recurrent infections and vasculitis has not been reported. We report a 30 year-old female with Down-syndrome associated with MBL-deficiency with the genotype LXA/HYD, IgG-deficiency, recurrent uro-genital infections, cutaneous vasculitis, G20.210A prothrombin mutation, deep venous thrombosis, and pulmonary embolism. MBL-deficiency in combination with IgG deficiency might have favored the development of recurrent uro-genital infections. Immunodeficiency might be also involved in the pathogenesis of cutaneous vasculitis. Deep venous thrombosis and pulmonary embolism were attributed to the genetically determined prothrombotic state and intake of oral contraceptives.

  17. Study of the global environment of small galaxy groups

    Science.gov (United States)

    Duplancic, F.; Dávila, F.; Coldwell, G.

    2017-07-01

    The present work presents a study of the global density environment of small galaxy groups. To this end we use a catalog of small galaxy systems constructed from the 10th Data Release of the Sloan Digital Sky Survey. To characterize the global environment of small galaxy groups we use different estimators, including the number of significant neighbors within a fixed aperture, the distance to the nearest neighbor and the number density profile of these systems. In order to perform a comparative study, we select different categories of systems considering galaxy pairs, triplets of galaxies and groups with at least four member galaxies. We found differences between the global environment of pairs compared to triplet of galaxies and groups. Galaxy pairs inhabit environments of lower global density than triplets and groups which are located in higher global density regions. This result is in agreement with different studies in the literature which propose that triplets of galaxies and compact groups have similarities in their fundamental properties and are different from galaxy pairs. Our findings suggest that the global density environment of small galaxy groups plays a fundamental role in the characterization of the main properties of these systems and their member galaxies.

  18. Brief group psychotherapy for the spousally bereaved: a controlled study.

    Science.gov (United States)

    Lieberman, M A; Yalom, I

    1992-01-01

    A consecutive sample of mid- and late-life bereaved spouses were randomly assigned to treatment and no-treatment groups. Two hypotheses were tested: (1) that brief group psychotherapy during the early stages of loss would facilitate adjustment assessed by measures of mental health, positive psychological states, social role, and mourning; and (2) that positive effects would be maximized for subjects who were more distressed psychologically. Although group participants, compared with untreated controls, did over 1 year show modest improvement on role functioning and positive psychological states, overall the study failed to find substantial support for the two major hypotheses. Both experimental and control groups showed improvement over the year, particularly on measures of mental health and mourning. Differential benefit was not observed for the high-risk group.

  19. Case stories in general practice: a focus group study.

    Science.gov (United States)

    Abildsnes, Eirik; Flottorp, Signe; Stensland, Per

    2012-01-01

    To explore the interactive process of sharing case stories in small-group activity in general practice. Qualitative focus group study. Peer-group meetings of doctors attending specialist training or continuous medical education in general practice. Twenty female and 30 male doctors working in general practice in Norway. The storyline of case presentations included detailed stories with emotional engagement, co-authored by other group members. The stories initiated discussions and reflections concerning patients' and doctors' perspectives, medical ethics as well as clinical problems. The safe atmosphere allowed testing out boundaries of socially shared knowledge. Sharing case stories in small groups in general practice initiated interaction that facilitated meaning-making, reflection and peer support.

  20. Valvular heart disease in patients with hypocomplementemic urticarial vasculitis syndrome associated with Jaccoud's arthropathy.

    Science.gov (United States)

    Houser, S L; Askenase, P W; Palazzo, E; Bloch, K J

    2002-01-01

    Since 1973, more than 75 patients with hypocomplementemic urticarial vasculitis syndrome (HUVS) were reported, but valvular heart disease does not seem to have been noted in these patients. Since 1993, however, five patients with HUVS accompanied by Jaccoud's arthropathy (JA) were found to have serious valvular heart disease. To characterize the cardiac valvulopathy of the third patient with HUVS/JA to have undergone valve replacement, this study included the use of routine and special tissue stains, as well as immunohistochemical staining. We compared gross and histologic findings of this patient's valve to those of two other patients with this complex syndrome who underwent valve replacement. Pathologic findings of these latter two patients were described in separate earlier reports. Histologic examination of the resected valves in all three patients showed an acute necrotizing endocarditis and fibrin deposition on the surface of valve leaflets. Beneath the surfaces of the leaflets, there was evidence of chronic inflammation, consisting of lymphocytes and histiocytes. A fibrocalcific degenerative change was also present in all three valves. Positive staining for IgG, IgA, IgM, and light-chain determinant-bearing proteins was detected primarily at the valve surface in special studies of the aortic valve of the patient described in the current report. Patients with HUVS and associated JA should be evaluated for the presence of valvular heart disease. The latter is probably a nonrheumatic, inflammatory, and degenerative process, mediated by immune complex, as well as cellular immune mechanisms.

  1. Clinical analysis of ANCA-associated renal vasculitis patients with chronic dialysis.

    Science.gov (United States)

    Chen, Yong-Xi; Zhang, Wen; Chen, Xiao-Nong; Ni, Li-Yan; Shen, Ping-Yan; Wang, Wei-Ming; Chen, Nan

    2014-01-01

    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitute a subgroup of life-threatening diseases which affects the kidney in more than half of the patients at diagnosis. Currently, little has been published focusing on AAV patients with dialysis. We analysed AAV patients with chronic dialysis to provide more detailed information. From 1997 to 2011, AAV patients complicated by renal involvement resulting in end-stage renal disease (ESRD) and had undergone haemodialysis (HD) or peritoneal dialysis (PD) for at least 3 months in Shanghai Ruijin hospital were retrospectively analysed in this study. Their data were also compared to those without dialysis at the same time. We enrolled 49 AAV patients with chronic dialysis. 41 required dialysis at initial presentation and rest 8 progressed to ESRD during follow-up. 19 HD patients died and 6 PD patients died during follow-up, and infection was the most common cause among the patients. There was no significant difference regarding survival between HD patients and PD patients (p>0.05). However anaemia and level of triglyceride was more significantly improved in HD patients at the end of observation (pdialysis dependency, dialysis patients presented higher percentage of hypertension (pdialysis patients (pdialysis dependence. Our study suggests that haemodialysis and peritoneal dialysis are two comparable dialysis modalities for AAV patients with ESRD. However, AAV patients with dialysis dependency had worse outcome in comparison with those without dialysis.

  2. Studying the HI content of the NGC 4930 group

    Science.gov (United States)

    Wolfinger, Kathrin; Kilborn, Virginia; Koribalski, Baerbel

    2011-10-01

    We propose to observe the neutral hydrogen (HI) content of the spiral-rich NGC 4930 group using the ATCA. This notable group lies 2.5° east of the Centaurus cluster core and is probably infalling for the first time. Our primary goal is to trace the evolutionary changes of spirals in different environments and to map the first signs of interaction and transformation. Our aims of the ATCA observations are (i) to study the HI properties of the group, (ii) to determine if there is an HI deficiency in the members, (iii) to look for any signs of ram pressure stripping that would indicate an interaction with a hot intra-group medium and (iv) to conduct a ‘blind’ survey for new group members, such as dwarf companions within the survey volume. We will further test the latest galaxy finding routines such as Duchamp, which are vital for the success of the upcoming ASKAP HI surveys. The NGC 4930 group is covered in the HI Parkes All Sky Survey but only two out of the nine group members are detected in HI. We propose to make mosaic observations and we expect to detect all of the known galaxies in this group.

  3. IRT studies of many groups: The alignment method

    Directory of Open Access Journals (Sweden)

    Bengt eMuthen

    2014-09-01

    Full Text Available Asparouhov and Muthen (forthcoming presented a new method for multiple-group confirmatory factor analysis (CFA, referred to as the alignment method. The alignment method can be used to estimate group-specific factor means and variances without requiring exact measurement invariance. A strength of the method is the ability to conveniently estimate models for many groups, such as with comparisons of countries. This paper focuses on IRT applications of the alignment method. An empirical investigation is made of binary knowledge items administered in two separate surveys of a set of countries. A Monte Carlo study is presented that shows how the quality of the alignment can be assessed.

  4. Febrile ulceronecrotic Mucha-Habermann disease with central nervous system vasculitis.

    Science.gov (United States)

    Rosman, Ilana S; Liang, Ling-Chih; Patil, Sushama; Bayliss, Susan J; White, Andrew J

    2013-01-01

    Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA). We report a case of FUMHD in an 11-year-old boy who subsequently developed neurologic symptoms and was found to have necrotizing vasculitis on brain biopsy. Over 5 years of follow-up, he had one biopsy-proven recurrence of his skin lesions and continued rehabilitation and treatment for residual neurologic complications. This case provides histological evidence of central nervous system vasculitis associated with FUMHD. Our patient's disease was eventually controlled with cyclophosphamide. © 2012 Wiley Periodicals, Inc.

  5. Connective tissue disorders associated with vasculitis and vaso-occlusive disease of the hand.

    Science.gov (United States)

    Michelotti, Brett; Rizzo, Marco; Moran, Steven L

    2015-02-01

    Hand ischemia caused by vasculitis is a secondary finding in many autoimmune processes. Many of these autoimmune diseases are managed primarily with medications that can prevent the development of occlusive disease, tissue ischemia, and tissue loss. Unfortunately several disease conditions can be recalcitrant to medical management and can result in ischemic changes within the hand, which may require operative intervention. This article briefly reviews the major connective tissue disorders associated with vasculitis and vaso-occlusive disease of the hand, including scleroderma, lupus, and Buerger disease, and their surgical treatment. Copyright © 2015 Elsevier Inc. All rights reserved.

  6. Churg-Strauss syndrome associated with antiphospholipid antibodies in a patient with retinal vasculitis.

    Science.gov (United States)

    Sánchez-Vicente, J L; Gálvez-Carvajal, S; Medina-Tapia, A; Rueda, T; González-García, L; Szewc, M; Muñoz-Morales, A

    2016-11-01

    We present the case of a 69-year-old woman with unilateral retinal vasculitis. Investigations showed asthma, rhinosinusitis, nasal polyposis, peripheral blood eosinophilia, increased sedimentation rate, proteinuria, and antiphospholipid antibodies. Anti-neutrophil cytoplasmic antibodies (ANCA) were negative. Although her anti-neutrophil cytoplasmatic antibody (ANCA) status was negative, taking into account the other clinical and laboratory features, retinal vasculitis was thought to be an ocular manifestation of Churg-Strauss syndrome. Treatment was started with high-dose corticosteroids and anticoagulant therapy. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  7. Inflammatory myositis complicating hypocomplementemic urticarial vasculitis despite on-going immunosuppression.

    Science.gov (United States)

    Chew, Gary Y J; Gatenby, Paul A

    2007-08-01

    We present a patient with previously diagnosed hypocomplementemic urticarial vasculitis syndrome, with skin, lung, and renal involvement, who presented with congestive cardiac failure. During the course of her hospitalization, she was also found to have profound proximal muscle weakness in both upper and lower limbs associated with raised creatinine kinase levels. A muscle biopsy was performed, which demonstrated evidence of an inflammatory myositis with vasculitis, which had returned despite on-going immunosuppression. This occurrence of a new autoimmune disease may well be an example of the "waste disposal" hypothesis.

  8. A case of hypocomplementaemic urticarial vasculitis with a high serum level of rheumatoid factor.

    Science.gov (United States)

    Ashida, Atsuko; Murata, Hiroshi; Ohashi, Atsuko; Ogawa, Eisaku; Uhara, Hisashi; Okuyama, Ryuhei

    2013-08-01

    We report a case of hypocomplementaemic urticarial vasculitis with an elevated serum rheumatoid factor level. Hypocomplementaemic urticarial vasculitis is an immune complex-mediated disease characterised by urticarial eruptions. High levels of rheumatoid factor may be associated with hypocomplementaemia due to the consumption of complement, because the rheumatoid factor can form immune complexes with immunoglobulin. It is necessary to pay attention to the amounts of complement in cases of urticarial eruptions with elevated rheumatoid factor level. The eruptions were relieved with a combination of prednisolone and colchicine. © 2012 The Authors. Australasian Journal of Dermatology © 2012 The Australasian College of Dermatologists.

  9. Unusual exanthema combined with cerebral vasculitis in pneumococcal meningitis: a case report

    Directory of Open Access Journals (Sweden)

    Raissaki Maria

    2011-08-01

    Full Text Available Abstract Introduction Bacterial meningitis is a complex, rapidly progressive disease in which neurological injury is caused in part by the causative organism and in part by the host's own inflammatory responses. Case presentation We present the case of a two-year-old Greek girl with pneumococcal meningitis and an atypical curvilinear-like skin eruption, chronologically associated with cerebral vasculitis. A diffusion-weighted MRI scan showed lesions with restricted diffusion, reflecting local areas of immunologically mediated necrotizing vasculitis. Conclusions Atypical presentations of bacterial meningitis may occur, and they can be accompanied by serious unexpected complications.

  10. Impact of individual study on tutorial group discussion

    NARCIS (Netherlands)

    Hurk, M.M. van den; Dolmans, D.H.J.M.; Wolfhagen, H.A.P.; Muijtjens, A.M.M.; Vleuten, C.P.M. van der

    1999-01-01

    Background: This research focuses on the relation between individual study and group discussion. In a problem-based curriculum, it is expected that the way students prepare themselves during individual study (i.e., search and prepare the literature) will influence the quality of the reporting phase.

  11. Difficulties in Balint groups: a qualitative study of leaders' experiences.

    Science.gov (United States)

    Kjeldmand, Dorte; Holmström, Inger

    2010-11-01

    Balint groups (BGs) are a means of enhancing competence in the physician-patient relationship and are also regarded as beneficial for GPs' mental health. However, voluntary BGs are still few, some members terminate their participation, and problems are reported in obligatory groups in residency programmes. This raises questions about possible negative aspects of BGs. To examine difficulties in BGs as experienced by BG leaders. Qualitative study using interviews. Eight BG leaders from five countries were interviewed. The interviews focused on the informants' experiences of difficulties in their groups and were analysed with a systematic text-condensation method. Three categories of difficulties emerged from the analysis: 1) the individual physician having needs, vulnerabilities, and defences; 2) the group (including the leader) having problems of hidden agendas, rivalries, and frames; and 3) the surrounding environment defining the conditions of the group. BGs were found to fit into modern theories of small groups as complex systems. They are submitted to group dynamics that are sometimes malicious, and are exposed to often tough environmental conditions. Professionally conducted BGs seem to be a gentle, efficient method to train physicians, but with limitations. Participation of a member demands psychological stability and an open mind. BGs need support from the leadership of healthcare organisations in order to exist.

  12. Evaluation of support group interventions for children in troubled families: study protocol for a quasi-experimental control group study.

    Science.gov (United States)

    Skerfving, Annemi; Johansson, Fredrik; Elgán, Tobias H

    2014-01-24

    Support groups for children in troubled families are available in a majority of Swedish municipalities. They are used as a preventive effort for children in families with different parental problems such as addiction to alcohol/other drugs, mental illness, domestic violence, divorce situations, or even imprisonment. Children from families with these problems are a well-known at-risk group for various mental health and social problems. Support groups aim at strengthening children's coping behaviour, to improve their mental health and to prevent a negative psycho-social development. To date, evaluations using a control-group study design are scarce. The aim of the current study is to evaluate the effects of support groups. This paper describes the design of an effectiveness study, initially intended as a randomized controlled trial, but instead is pursued as a quasi-experimental study using a non-randomized control group. The aim is to include 116 children, aged 7-13 years and one parent/another closely related adult, in the study. Participants are recruited via existing support groups in the Stockholm county district and are allocated either into an intervention group or a waiting list control group, representing care as usual. The assessment consists of questionnaires that are to be filled in at baseline and at four months following the baseline. Additionally, the intervention group completes a 12-month follow-up. The outcomes include the Strength and Difficulties Questionnaire (SDQ S11-16), the Kids Coping Scale, the "Ladder of life" which measures overall life satisfaction, and "Jag tycker jag är" (I think I am) which measures self-perception and self-esteem. The parents complete the SDQ P4-16 (parent-report version) and the Swedish scale "Familjeklimat" (Family Climate), which measures the emotional climate in the family. There is a need for evaluating the effects of support groups targeted to children from troubled families. This quasi-experimental study

  13. 32nd European Study Group with Industry, Final Report

    DEFF Research Database (Denmark)

    ESGI (European Study Group with Industry) is Europe's leading workshop for interaction between mathematicians and industry. These workshops have taken place in Great Britain for a number of years, going back to 1968 when Prof. Alan Tayler initiated the so-called Oxford Study Group with Industry...... expertise.Danfoss wanted a an analysis and optimization of a scroll compressor.DANISCO wanted a model for the heat and moisture transport in sugar silos.Danish Maritime Institute wanted to optimize a dynamical position system in order to keep a wessel stationary on the surface of the ocean.Grundfos wanted...

  14. Situational Factors in Focus Group Studies: A Systematic Review

    Directory of Open Access Journals (Sweden)

    Arne Orvik MPolSc

    2013-02-01

    Full Text Available The aim of this study was to see how contextual factors are expressed, used, and analyzed in data collected in focus group discussions (FGDs. The study includes an assessment of how the methodological reporting of contextual factors might influence and improve the trustworthiness of articles. Articles reporting workplace health, stress, and coping among health professionals were identified in a systematic review and used in the analysis. By using Vicsek's framework of situational factors for analysis of focus group results as a starting point, we found that contextual factors were most frequently described in the method sections and less frequently in the results and discussion sections. Vicsek's framework for the analysis of focus group results covers six contextual and methodological dimensions: interactional factors, personal characteristics of the participants, the moderator, the environment, time factors, and the content of FGDs. We found that the framework does not include a consideration of psychological safety, ethical issues, or organizational information. To deepen the analysis of focus group results, we argue that contextual factors should be analyzed as methodological dimensions and be considered as a sensitizing concept. Credibility, confirmability, dependability, and transferability can be strengthened by using, reporting, and discussing contextual factors in detail. The study contributes to elucidating how reporting of contextual data may enrich the analysis of focus group results and strengthen the trustworthiness. Future research should focus on clear reporting of contextual factors as well as further develop Vicsek's model to enhance reporting accuracy and transferability.

  15. Revitalising PBL groups: evaluating PBL with study teams.

    Science.gov (United States)

    Moust, Jos; Roebertsen, Herma; Savelberg, Hans; De Rijk, Angelique

    2005-03-01

    In problem-based learning (PBL), students are actively engaged with psychological learning principles as activation of prior knowledge, elaboration and organization of knowledge. In their tutorial groups, however, students do not always apply these principles when working with a procedure like the "Seven-Jump" method. To stimulate students to use these principles more often, they were offered another format within a PBL context: PBL with study teams. During the period of self-study, students work on a regular basis in so-called study teams, small groups of 3-4 persons. In these groups they explain to each other their learning outcomes, clarify for each other their problems while studying texts and organize their knowledge to present this to the members of other study teams in their tutorial group. Previous research showed that students spent more time on self-study in a PBL with study team condition than in a traditional PBL context. In this study the achievement as well as appreciation of students participating in a PBL with study teams' environment, is compared with students working in a traditional PBL environment. To determine whether PBL with study teams differs from the traditional PBL environment in students' appreciation and study time. We conducted an experiment in two blocks over two years. Questionnaires were administered to collect data on appreciation and time for self-study. Students' appreciation of the two formats did not differ much. The large standard deviations indicate considerable differences in appreciation between individual students. Appreciation was slightly higher in the second experiment when instructions about how to collaborate were less strict. Students devoted twice as many hours studying in the study group format compared with the traditional PBL format. The students indicated that they enjoyed the format but that the increased workload disturbed their customary study rhythm. Assessment scores and tutors'impressions suggest that

  16. A best practice position statement on pregnancy in chronic kidney disease: the Italian Study Group on Kidney and Pregnancy.

    Science.gov (United States)

    Cabiddu, Gianfranca; Castellino, Santina; Gernone, Giuseppe; Santoro, Domenico; Moroni, Gabriella; Giannattasio, Michele; Gregorini, Gina; Giacchino, Franca; Attini, Rossella; Loi, Valentina; Limardo, Monica; Gammaro, Linda; Todros, Tullia; Piccoli, Giorgina Barbara

    2016-06-01

    Pregnancy is increasingly undertaken in patients with chronic kidney disease (CKD) and, conversely, CKD is increasingly diagnosed in pregnancy: up to 3 % of pregnancies are estimated to be complicated by CKD. The heterogeneity of CKD (accounting for stage, hypertension and proteinuria) and the rarity of several kidney diseases make risk assessment difficult and therapeutic strategies are often based upon scattered experiences and small series. In this setting, the aim of this position statement of the Kidney and Pregnancy Study Group of the Italian Society of Nephrology is to review the literature, and discuss the experience in the clinical management of CKD in pregnancy. CKD is associated with an increased risk for adverse pregnancy-related outcomes since its early stage, also in the absence of hypertension and proteinuria, thus supporting the need for a multidisciplinary follow-up in all CKD patients. CKD stage, hypertension and proteinuria are interrelated, but they are also independent risk factors for adverse pregnancy-related outcomes. Among the different kidney diseases, patients with glomerulonephritis and immunologic diseases are at higher risk of developing or increasing proteinuria and hypertension, a picture often difficult to differentiate from preeclampsia. The risk is higher in active immunologic diseases, and in those cases that are detected or flare up during pregnancy. Referral to tertiary care centres for multidisciplinary follow-up and tailored approaches are warranted. The risk of maternal death is, almost exclusively, reported in systemic lupus erythematosus and vasculitis, which share with diabetic nephropathy an increased risk for perinatal death of the babies. Conversely, patients with kidney malformation, autosomal-dominant polycystic kidney disease, stone disease, and previous upper urinary tract infections are at higher risk for urinary tract infections, in turn associated with prematurity. No risk for malformations other than those

  17. Synthesis and structural study of platinum group metal complexes ...

    Indian Academy of Sciences (India)

    Home; Journals; Journal of Chemical Sciences; Volume 126; Issue 4. Synthesis and structural study of platinum group metal complexes containing pyrimidine bridged pyrazolyl-pyridine ligand and 5 and 6 - cyclic hydrocarbons. Thirumala Prasad Kota Mohan Rao Kollipara. Volume 126 Issue 4 July 2014 pp 1143-1151 ...

  18. A study on abundance, group size and composition of ...

    African Journals Online (AJOL)

    Population status, group composition and social organization of the Soemmerring's gazelle (Gazella soemmeringii) were studied at Awash National Park (ANP) and Alledeghi Wildlife Reserve (AWR) in 2000. Total count method was used to assess the population size of the gazelle. Thirteen routes, each route being two ...

  19. Group Involvement in Decision-Making: A Pilot Study.

    Science.gov (United States)

    Genrich, Sandra J.; Banks, J. Carolyn; Bufton, Karen; Savage, Mary Ellen; Owens, Marcella Upshaw

    2001-01-01

    Health care leaders (n=27) read case studies and identified leadership styles appropriate for the scenarios. Results suggest that participating in a class on the use of the Vroom-Yetton-Jago Leadership Model may help leaders gain the skill needed to delegate decision making to groups. (Contains 16 references.) (JOW)

  20. When Children Discuss: A Study of Learning in Small Groups

    Science.gov (United States)

    Wilcox, Mary Anastole

    1976-01-01

    Describes a study designed to increase and improve participation in class discussions among urban low-income fifth and sixth graders. Comparisons are made between small group discussions of moral issues led by teachers, by trained students, and by untrained students. (CW)

  1. Cognitive group therapy for depressive students: The case study

    Science.gov (United States)

    Tiuraniemi, Juhani; Korhola, Jarno

    2009-01-01

    The aims of this study were to assess whether a course of cognitive group therapy could help depressed students and to assess whether assimilation analysis offers a useful way of analysing students' progress through therapy. “Johanna” was a patient in a group that was designed for depressive students who had difficulties with their studies. The assimilation of Johanna's problematic experience progressed as the meetings continued from level one (unpleasant thoughts) to level six (solving the problem). Johanna's problematic experience manifested itself as severe and excessive criticism towards herself and her study performance. As the group meetings progressed, Johanna found a new kind of tolerance that increased her determination and assertiveness regarding the studies. The dialogical structure of Johanna's problematic experience changed: she found hope and she was more assertive after the process. The results indicated that this kind of psycho-educational group therapy was an effective method for treating depression. The assimilation analysis offered a useful way of analysing the therapy process. PMID:20523883

  2. Adolescent girls' views on cosmetic surgery: a focus group study

    OpenAIRE

    Ashikali, E.-M.; Dittmar, H.; Ayers, S

    2016-01-01

    This study examined adolescent girls’ views of cosmetic surgery. Seven focus groups were run with girls aged 15 to 18 (N = 27). Participants read case studies of women having cosmetic surgery, followed by discussion and exploration of their views. Thematic analysis identified four themes: (1)\\ud Dissatisfaction with appearance, (2) Acceptability of cosmetic surgery, (3) Feelings about undergoing cosmetic surgery, and (4) Cosmetic surgery in the media. Results suggest the acceptability of cosm...

  3. Hypernephroma Presenting with Cutaneous Leukocytoclastic Vasculitis and Lupus Anticoagulant: Resolution after Nephrectomy

    Science.gov (United States)

    Murray, Nigel P.; Ruíz, Amparo; Reyes, Eduardo

    2012-01-01

    Hypernephroma can present as a variety of paraneoplastic, nonmetastatic conditions, including vasculitis, and rarely a lupus-type anticoagulant. Nephrectomy leads to the resolution of the systemic complaints. Malignancy, in this case hypernephroma, can present as an immune-mediated paraneoplastic syndrome which resolves after removal of the underlying tumor. PMID:22919534

  4. Hypernephroma Presenting with Cutaneous Leukocytoclastic Vasculitis and Lupus Anticoagulant: Resolution after Nephrectomy

    Directory of Open Access Journals (Sweden)

    Nigel P. Murray

    2012-01-01

    Full Text Available Hypernephroma can present as a variety of paraneoplastic, nonmetastatic conditions, including vasculitis, and rarely a lupus-type anticoagulant. Nephrectomy leads to the resolution of the systemic complaints. Malignancy, in this case hypernephroma, can present as an immune-mediated paraneoplastic syndrome which resolves after removal of the underlying tumor.

  5. Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis

    Science.gov (United States)

    ABSTRACT BACKGROUND Anti-neutrophil cytoplasmic autoantibodies (ANCA) specific for myeloperoxidase (MPO) or proteinase 3 (PR3) are detectable in >90% of patients with ANCA-associated vasculitis (AAV). ANCA titers do not correlate well with disease activity. In vivo and in vi...

  6. Bilateral Testicular Infarction from IgA Vasculitis of the Spermatic Cords

    Directory of Open Access Journals (Sweden)

    Mazen Toushan

    2017-01-01

    Full Text Available A 51-year-old man with type 2 diabetes mellitus and chronic obstructive pulmonary disease presented to the emergency room with increasing bilateral leg pain, rash, and scrotal swelling with pain. Skin biopsy from his thigh revealed IgA-associated vasculitis. Due to hematuria, a renal biopsy was performed and showed an IgA glomerulonephritis with focal fibrinoid necrosis and neutrophil accumulation. Bilateral orchiectomies were performed in two separate procedures ten and thirteen days after the renal biopsy, as a result of uncontrolled abscess formation in testicles. Microscopically, both testicles revealed large abscess formation destroying almost the entire testicular parenchyma without tumor cells. Spermatic cord margins were further scrutinized microscopically to show bilateral vasculitis in many small size vessels, confirmed by positive endothelial staining for IgA. Some of the affected arteries revealed central organizing thrombi with recanalization features, highly suggestive of vasculitis-associated thrombi formation, resulting in testicular ischemic infarction and abscess formation. We conclude that this adult patient developed a severe form of Henoch-Schönlein purpura, with vasculitis affecting multiple organs, including the most serious and unusual complication of bilateral testicular infarction.

  7. Venous thromboembolism in ANCA-associated vasculitis - incidence and risk factors

    NARCIS (Netherlands)

    Stassen, P. M.; Derks, R. P. H.; Kallenberg, C. G. M.; Stegeman, C. A.

    Objectives. In patients with ANCA-associated vasculitis (AAV), an increased incidence of venous thromboembolism (VTE), mainly during active disease, has been described. In a large cohort of AAV patients, live assessed the incidence of VTE and its relation with disease activity and classic risk

  8. Performance of two strategies for urgent ANCA and anti-GBM analysis in vasculitis

    NARCIS (Netherlands)

    de Joode, Anoek A. E.; Roozendaal, Caroline; van der Leij, Marcel J.; Bungener, Laura B.; Sanders, Jan Stephan; Stegeman, Coen A.

    Background: In anti-neutrophil cytoplasmic antibodies (ANCA) associated small vessel vasculitis (AAV), rapid testing for ANCA and anti-glomerular basement membrane (GBM) antibodies may be beneficial for therapeutic purpose. Objective: We analysed the diagnostic performance of two rapid ANCA and

  9. [Acute pancreatitis as the presenting feature of an IgA vasculitis: An unusual presentation].

    Science.gov (United States)

    Fertitta, L; Noel, N; Ackermann, F; Lerolle, N; Benoist, S; Rocher, L; Lambotte, O

    2017-10-01

    IgA vasculitis is a systemic small vessel leukocytoclastic vasculitis characterized by skin purpura, arthritis, abdominal pain and nephritis. Most of the abdominal complications are due to edema and hemorrhage in the small bowel wall, but rarely to acute secondary pancreatitis. Here, we report a 53-year-old woman who presented with acute pancreatitis and, secondarily, developed skin purpura and arthritis at the seventh day of the clinical onset. Biological tests and computed tomographic scan allowed to rule out another cause of pancreatitis and IgA vasculitis was diagnosed as its etiology. The outcome was favorable without any relapse on glucocorticoids. Despite its rarity, pancreatitis is a potential life-threatening complication of IgA vasculitis in which the role of glucocorticoids and immunosuppressive drugs remains uncertain. A prompt elimination of other usual pancreatitis etiologies is mandatory to improve the management of the patients. Copyright © 2017 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  10. Levamisole-Induced Leukocytoclastic Vasculitis with Negative Serology in a Cocaine User.

    Science.gov (United States)

    Salehi, Mashal; Morgan, Michael P; Gabriel, Abigail

    2017-06-08

    BACKGROUND Levamisole is a common adulterant of cocaine. It can cause agranulocytosis and cutaneous vasculitis that can possibly lead to cutaneous necrosis. In all reported cases of levamisole-induced vasculitis, it has been described as a clinical syndrome characterized by a constellation of typical clinical features and a positive serum serology for ANCA levels, especially very high-titer p-ANCA levels, in the background of cocaine abuse. However, patients may have a negative serology and here, we present the first such case. CASE REPORT A 58-year-old African American man with a history of polysubstance abuse, 4 days after last cocaine use, presented with sudden onset of painful pruritic rash and polyarthralgias. He was found to have normal vital signs, with bilateral tender knees and erythematous-purplish maculopapular lesions involving the abdomen and the left thigh. Laboratory work-up was significant for elevated CRP, negative c-ANCA, p-ANCA ANA, and RA levels, and a positive urine toxicology for cocaine. Urine analysis by high-performance liquid chromatography was positive for levamisole. Ultimately, a final diagnosis was made by skin biopsy, which revealed findings suggestive of leukocytoclastic vasculitis. CONCLUSIONS Cutaneous leukocytoclastic vasculitis can be caused by levamisole, which is used as an adulterant in cocaine. Most cases are associated with positive ANCA levels; however, a negative serology is also a possibility.

  11. Candida albicans-associated necrotizing vasculitis producing life-threatening gastrointestinal hemorrhage.

    LENUS (Irish Health Repository)

    Sargent, Jeremy

    2012-02-01

    Patients undergoing treatment of acute lymphoblastic leukemia are at risk for fungal infections including disseminated candidiasis. We describe a case of systemic Candida albicans infection associated with life-threatening gastrointestinal hemorrhage due to unusual necrotizing vasculitis involving the gastrointestinal tract. We explore the association between Candida and such vasculopathy.

  12. Patients with systemic vasculitis have increased levels of autoantibodies against oxidized LDL

    NARCIS (Netherlands)

    Swets, BP; Brouwer, DAJ; Tervaert, JWC

    Oxidation of low density lipoprotein (LDL) is considered to play an important role in the development of atherosclerosis and increased levels of autoantibodies against oxidized LDL have been found in patients with various manifestations of atherosclerosis. Patients with vasculitis are prone to the

  13. Urticaria vasculitis in a child: a case report and literature review.

    Science.gov (United States)

    Imbernón-Moya, Adrián; Vargas-Laguna, Elena; Burgos, Fernando; Fernández-Cogolludo, Eva; Aguilar-Martínez, Antonio; Gallego-Valdés, Miguel Ángel

    2017-08-01

    Annular urticarial lesions in a child must establish a main differential diagnosis with urticaria multiforme, common urticaria, acute hemorrhagic edema of infancy, erythema marginatum, erythema annulare centrifugum, annular erythema in childhood, erythema multiforme, Sweet's syndrome, Schönlein-Henoch purpura, erythematosus lupus, several systemic vasculitis, and serum sickness.

  14. Pulmonary capillaritis and its relationship to development of emphysema in hypocomplementaemic urticarial vasculitis syndrome.

    Science.gov (United States)

    Hunt, David P J; Weil, Rimona; Nicholson, Andrew G; Burke, Margaret M; Du Bois, Ron M; Wells, Athol U

    2006-03-01

    Hypocomplementaemic urticarial vasculitis syndrome (HUVS) is a rare disorder characterised by complement activation and the presence of C1q precipitins together with a syndrome of urticarial vasculitis, angioedema, arthralgia, ocular inflammation, glomerulonephritis and obstructive lung disease. The pathophysiology of the obstructive airways disease is poorly understood. We report a 46 year-old woman with HUVS who developed progressive obstructive airways disease. Lung biopsy early in the course of her disease revealed pulmonary capillaritis. The disease progressed despite treatment with steroids and cyclosporin and the patient eventually underwent successful double lung transplantation. The explanted lung showed the coexistence of a patchy active vasculitis with severe panacinar emphysema. This is the first description of the histopathological process of HUVS in an explanted lung. Through analysis of serial histopathological specimens and clinical data we show the evolution of pulmonary capillaritis to emphysema, and demonstrate that active vasculitis can coexist with emphysema in patients with HUVS and obstructive airways disease. We suggest that there is a role for ongoing immunosuppressive therapy in these patients.

  15. Rapid Growth of Lung Nodules due to Combined Pulmonary Vasculitis, Silicoanthracosis, and Chondrocalcinosis

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    Wolfgang Jungraithmayr

    2016-01-01

    Full Text Available Background. Silicoanthracosis is a pneumoconiosis due to occupational inhalation of silica and carbon dusts. Clinically, it can be associated with vasculitis or rheumatoid arthritis. In association with these diseases, silicoanthracosis can present within the lung with multiple pulmonary nodules which, as a differential diagnosis, can mimic metastatic disease or multiple abscesses. Case Presentation. We present the case of a 62-year old former pit worker with pulmonary nodules, chondrocalcinosis due to calcium pyrophosphate deposition (CPPD, and a history of renal cancer. Within a short period of time, pulmonary nodules grew rapidly. Thoracoscopically, the resected lung specimen revealed silicoanthracosis associated with small-to-medium-size vasculitis in the presence of antineutrophil cytoplasmatic autoantibodies (c-ANCA. Conclusion. Pulmonary silicoanthracotic lesions on the base of ANCA-associated vasculitis and CPPD arthritis can rapidly grow. A mutual correlation between silicoanthracosis, ANCA-associated vasculitis, and CPPD seems possible. Apart from this, consideration of metastatic disease should be obligatory in patients with a history of cancer at the same time being immunosuppressed.

  16. A study of the current group evaporation/combustion theories

    Science.gov (United States)

    Shen, Hayley H.

    1990-01-01

    Liquid fuel combustion can be greatly enhanced by disintegrating the liquid fuel into droplets, an effect achieved by various configurations. A number of experiments carried out in the seventies showed that combustion of droplet arrays and sprays do not form individual flames. Moreover, the rate of burning in spray combustion greatly deviates from that of the single combustion rate. Such observations naturally challenge its applicability to spray combustion. A number of mathematical models were developed to evaluate 'group combustion' and the related 'group evaporation' phenomena. This study investigates the similarity and difference of these models and their applicability to spray combustion. Future work that should be carried out in this area is indicated.

  17. Conformational studies of blood group A and blood group B oligosaccharides using NMR residual dipolar couplings.

    Science.gov (United States)

    Azurmendi, Hugo F; Bush, C Allen

    2002-05-13

    The conformations of two synthetic trisaccharides of blood group A and B (alpha-L-Fucp-(1-->2)-[alpha-D-GalpNAc-(1-->3)]-alpha-D-Galp and alpha-L-Fucp-(1-->2)-[alpha-D-Galp-(1-->3)]-alpha-D-Galp, respectively) and of a type A tetrasaccharide alditol, Fucp-(1-->2)-[alpha-D-GalpNAc-(1-->3)]-beta-D-Galp-(1-->3)-GalNAc-ol, were studied by NMR measurements of one-bond C-H residual dipolar couplings in partially oriented liquid crystal solutions. The conformations of the three oligosaccharides were analyzed by generating thousands of structures using a Monte-Carlo method. Two different strategies were applied to calculate theoretical dipolar couplings for these structures. In the first method, the orientation of the molecule was calculated from the optimal fit of the molecular model to the experimental data, while in the second method the orientation tensor was calculated directly from the moment of inertia of the molecular model. Both methods of analysis give similar results but with slightly better agreement with experiment for the former one. The analysis of the results implies a single unique conformation for both blood group epitopes in solution in disagreement with theoretical models suggesting the existence of two conformers in solution.

  18. Unilateral neglect and perceptual parsing: a large-group study.

    Science.gov (United States)

    Neppi-Mòdona, Marco; Savazzi, Silvia; Ricci, Raffaella; Genero, Rosanna; Berruti, Giuseppina; Pepi, Riccardo

    2002-01-01

    Array-centred and subarray-centred neglect were disambiguated in a group of 116 patients with left neglect by means of a modified version of the Albert test in which the central column of segments was deleted so as to create two separate sets of targets grouped by proximity. The results indicated that neglect was more frequent in array- than subarray-centred coordinates and that, in a minority of cases, neglect co-occurred in both coordinate-systems. The two types of neglect were functionally but not anatomically dissociated. Presence of visual field defects was not prevalent in one type of neglect with respect to the other. These data contribute further evidence to previous single-case and small-group studies by showing that neglect can occur in single or multiple reference frames simultaneously, in agreement with current neuropsychological, neurophysiological and computational concepts of space representation.

  19. Analysis of verbal interactions in tutorial groups: a process study.

    Science.gov (United States)

    Visschers-Pleijers, Astrid J S F; Dolmans, Diana H J M; de Leng, Bas A; Wolfhagen, Ineke H A P; van der Vleuten, Cees P M

    2006-02-01

    Collaborative learning, including problem-based learning (PBL), is a powerful learning method. Group interaction plays a crucial role in stimulating student learning. However, few studies on learning processes in medical education have examined group interactions. Most studies on collaboration within PBL used self-reported data rather than observational data. We investigated the following types of interactions in PBL tutorial groups: learning-oriented interactions (exploratory questioning, cumulative reasoning and handling conflicts about knowledge); procedural interactions, and irrelevant/off-task interactions. The central question concerned how much time is spent on the different types of interaction during group sessions and how the types of interaction are distributed over the meeting. Four tutorial group sessions in Year 2 of the PBL undergraduate curriculum of Maastricht Medical School were videotaped and analysed. The sessions concerned the reporting phase of the PBL process. We analysed the interactions using a coding scheme distinguishing several verbal interaction types, such as questions, arguments and evaluations. Learning-orientated interactions accounted for 80% of the interactions, with cumulative reasoning, exploratory questioning and handling conflicts about knowledge accounting for about 63%, 10% and 7% of the interactions, respectively. Exploratory questioning often preceded cumulative reasoning. Both types occurred throughout the meeting. Handling conflicts mainly occurred after the first 20 minutes. Task involvement in the tutorial groups was high. All types of learning-orientated interactions were observed. Relatively little time was spent on exploratory questions and handling conflicts about knowledge. Problem-based learning training should pay special attention to stimulating discussion about contradictory information.

  20. Stress and Disease Onset in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    Directory of Open Access Journals (Sweden)

    Christina V. Golemati

    2017-12-01

    Full Text Available ObjectiveTo explore the potential contribution of stress as a trigger for disease onset in patients with antineutrophil cytoplasmic antibody (ANCA associated vasculitis (AAV.Methods53 AAV and 85 rheumatoid arthritis (RA patients as well as 53 healthy controls (HC were thoroughly asked for the number and impact of stressful life events, coping strategies, and available social support 12 months prior to disease onset. Anxiety, depression, personality dimensions, insomnia, and fatigue were also determined.ResultsAAV patients reported higher scoring of the impact of stressful life events compared to the RA and HC group prior to disease onset (2.8 ± 3.1 vs 1.8 ± 2.1 vs 1.7 ± 2.3, p-values: 0.047 and 0.053, respectively. While the number of reported stressful events was found to be significantly higher in AAV vs RA patients but not HC, certain coping strategies and social support features were more commonly implemented by AAV patients compared to HC, but not RA patients. As far as personality and other psychosocial characteristics, AAV patients displayed significantly higher psychoticism traits compared to RA, with no other differences being detected between AAV patients and both RA and HC. After adjusting for potential cofounders, scoring of the impact of stressful life events >3 was independently associated with AAV development compared to both RA and HC [ORs (95% CI: 4.6 (1.6–13.4 and 4.4 (1.0–19.0, respectively].ConclusionThe perceived impact of stressful life events prior to disease onset emerged as a contributing factor for AAV development.

  1. Polymyalgia rheumatica and giant cell arteritis—three challenges—consequences of the vasculitis process, osteoporosis, and malignancy

    Science.gov (United States)

    Emamifar, Amir; Hess, Søren; Gerke, Oke; Hermann, Anne Pernille; Laustrup, Helle; Hansen, Per Syrak; Thye-Rønn, Peter; Marcussen, Niels; Svendstrup, Frank; Gildberg-Mortensen, Rannveig; Bang, Jacob Christian; Farahani, Ziba Ahangarani; Chrysidis, Stavros; Toftegaard, Pia; Andreasen, Rikke Asmussen; le Greves, Sebastian; Andersen, Hanne Randi; Olsen, Rudolf Nezlo; Hansen, Inger Marie Jensen

    2017-01-01

    Abstract Introduction: Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are common inflammatory conditions. The diagnosis of PMR/GCA poses many challenges since there are no specific diagnostic tests. Recent literature emphasizes the ability of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) to assess global disease activity in inflammatory diseases. 18F-FDG PET/CT may lead to the diagnosis at an earlier stage than conventional imaging and may also assess response to therapy. With respect to the management of PMR/GCA, there are 3 significant areas of concern as follows: vasculitis process/vascular stiffness, malignancy, and osteoporosis. Methods and analysis: All patients with suspected PMR/GCR referred to the Rheumatology section of Medicine Department at Svendborg Hospital, Denmark. The 4 separate studies in the current protocol focus on: the association of clinical picture of PMR/GCA with PET findings; the validity of 18F-FDG PET/CT scan for diagnosis of PMR/GCA compared with temporal artery biopsy; the prevalence of newly diagnosed malignancies in patients with PMR/GCA, or PMR-like syndrome, with the focus on diagnostic accuracy of 18F-FDG PET/CT scan compared with conventional workup (ie, chest X-ray/abdominal ultrasound); and the impact of disease process, and also steroid treatment on bone mineral density, body composition, and vasculitis/vascular stiffness in PMR/GCA patients. Ethics and dissemination: The study has been approved by the Regional Ethics Committee of the Region of Southern Denmark (identification number: S-20160098) and Danish Data Protection Agency (J.nr 16/40522). Results of the study will be disseminated via publications in peer-reviewed journals, and presentation at national and international conferences. PMID:28658131

  2. The Thrombolysis in Myocardial Infarction (TIMI) Study Group experience.

    Science.gov (United States)

    Braunwald, Eugene; Sabatine, Marc S

    2012-10-01

    The Thrombolysis in Myocardial Infarction (TIMI) study group, an academic research organization, was formed in 1984 with initial support from the National Heart, Lung, and Blood Institute. Its initial goal was to compare the effects of the then-new thrombolytic agent, recombinant tissue plasminogen activator, with streptokinase. The TIMI study group has remained active since then and has completed 50 multicenter clinical trials. The TIMI network now collaborates with more than 1000 separate sites in 45 countries on 5 continents. In addition to thrombolytic agents, TIMI has studied antithrombotic, antiplatelet, anti-ischemic, lipid lowering, and anti-inflammatory drugs. TIMI has also established robust biomarker and pharmacogenetics programs, and has devised a panel of risk assessment scores that are widely used. TIMI is currently conducting 7 large trials worldwide on novel agents designed to reduce the morbidity and mortality of a variety of cardiovascular disorders. Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  3. Macrophage migration inhibitory factor (MIF) and thyroid hormone alterations in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

    Science.gov (United States)

    Wendt, Mårten; Börjesson, Ola; Avik, Aune; Bratt, Johan; Anderstam, Björn; Qureshi, Abdul R; Miller, Edmund J; Gunnarsson, Iva; Bruchfeld, Annette

    2013-05-20

    Macrophage migration inhibitory factor (MIF) is a proinflammatory cytokine known to be released from lymphocytes, macrophages and endothelial cells and also in animal models shown to be inducible with glucocorticoids (GC). In contrast, thyroxine seems to antagonize MIF activity. To investigate whether MIF is increased in active antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and possible correlations with GC dosing and thyroid hormone levels, 27 consecutive patients with active AAV were studied and followed prospectively. Disease activity was assessed using Birmingham Vasculitis Activity Score 2003 (BVAS) at baseline and at follow-up at 3 and 6 months, along with MIF, thyroid hormones free triiodothyronine (fT3) and free thyroxine (fT4), C-reactive protein (CRP) and creatinine. MIF was elevated significantly at baseline compared with follow-up at 3 and 6 months (8,618 pg/mL versus 5,696 and 6,212 respectively; P < 0.002) but did not correlate to CRP, GC dose, creatinine or organ involvement. fT3 was depressed significantly at baseline compared with follow-up (1.99 pg/mL versus 2.31 and 2.67 respectively; P = 0.01) and correlated inversely to the BVAS score at baseline. We found a significant correlation between the MIF/fT4 ratio at baseline versus MIF/fT4 ratio at 6 months (ρ = 0.52, P < 0.005) and a trend between the baseline MIF/fT3 ratio versus MIF/fT3 ratio at 6 months (ρ = 0.39, P = 0.05). These results suggest a possible role for MIF and thyroid status in AAV. Further studies could reveal whether the association between AAV and thyroid hormone levels in the context of elevated MIF may present a link as well as a target of treatment.

  4. Diagnostic Value of Procalcitonin in ANCA-Associated Vasculitis (AAV) to Differentiate Between Disease Activity, Infection and Drug Hypersensitivity.

    Science.gov (United States)

    Herrmann, K; Schinke, S; Csernok, E; Moosig, F; Holle, J U

    2015-01-01

    Procalcitonin (PCT) is considered to be a specific marker for severe bacterial infections and sepsis. Elevated PCT levels have been reported in active autoimmune diseases without infection. The aim of this study was to assess the diagnostic value of PCT serum levels in ANCA-associated vasculitis (AAV) patients with respect to infection, disease activity and drug fever using a high sensitive PCT detection method. In 53 AAV patients with elevated C-reactive protein (CRP) PCT was determined by the Thermo Scientific BRAHMS PCT sensitive KRYPTOR assay. Patients underwent standardized diagnostic procedures for evaluation of disease activity and infection. 53 patients with AAV and elevated CRP (7.7±6.9 mg/dl, PCT 0.34±1.02 ng/ml) were assessed, 10 had infection with elevated CRP levels of 11.2±10.2 mg/dl and PCT levels of 1.06±2.07 ng/dl. 43 patients had no evidence of infection, 36 of them were presented with AAV with normal or only slightly positive PCT levels in active disease (n=36) (PCT 0.06±0.06 ng/ml). 7 patients had increased PCT levels due to azathioprine hypersensitivity (0.76±1.01 ng/ml). For discrimination between infection and vasculitis activity PCT was more useful than CRP with the best cut-off at 0.1 ng/ml (sensitivity 60%, specificity 92%). In contrast to previous studies using semiquantitative PCT assays, the KRYPTOR performs better with respect to discrimination of infection from active AAV. In all patients assessed with active AAV (and without infection) PCT levels remained below the PCT reference limit (0.5 ng/ml) for infections. Drug hypersensitivity seems to be an important differential diagnosis in the setting of elevated CRP and PCT in patients who receive azathioprine.

  5. Caveolin-1 single nucleotide polymorphism in antineutrophil cytoplasmic antibody associated vasculitis.

    Directory of Open Access Journals (Sweden)

    Sourabh Chand

    Full Text Available Immunosuppression is cornerstone treatment of antineutrophil cytoplasmic antibody associated vasculitis (AAV but is later complicated by infection, cancer, cardiovascular and chronic kidney disease. Caveolin-1 is an essential structural protein for small cell membrane invaginations known as caveolae. Its functional role has been associated with these complications. For the first time, caveolin-1 (CAV1 gene variation is studied in AAV.CAV1 single nucleotide polymorphism rs4730751 was analysed in genomic DNA from 187 white patients with AAV from Birmingham, United Kingdom. The primary outcome measure was the composite endpoint of time to all-cause mortality or renal replacement therapy. Secondary endpoints included time to all-cause mortality, death from sepsis or vascular disease, cancer and renal replacement therapy. Validation of results was sought from 589 white AAV patients, from two European cohorts.The primary outcome occurred in 41.7% of Birmingham patients. In a multivariate model, non-CC genotype variation at the studied single nucleotide polymorphism was associated with increased risk from: the primary outcome measure [HR 1.86; 95% CI: 1.14-3.04; p=0.013], all-cause mortality [HR:1.83; 95% CI: 1.02-3.27; p=0.042], death from infection [HR:3.71; 95% CI: 1.28-10.77; p=0.016], death from vascular disease [HR:3.13; 95% CI: 1.07-9.10; p=0.037], and cancer [HR:5.55; 95% CI: 1.59-19.31; p=0.007]. In the validation cohort, the primary outcome rate was far lower (10.4%; no association between genotype and the studied endpoints was evident.The presence of a CC genotype in Birmingham is associated with protection from adverse outcomes of immunosuppression treated AAV. Lack of replication in the European cohort may have resulted from low clinical event rates. These findings are worthy of further study in larger cohorts.

  6. Innovation in Accounting Tasks: Empirical Study in Two Professional Groups

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    Célia Cristina da Silva Vicente

    2016-09-01

    Full Text Available The aim of this study is to contribute to the knowledge on innovation in accounting tasks, from the point of view of two professional groups. Its goals are: evaluating the importance given by the professionals to accounting tasks; identifying whether there is convergence between the two professional groups, regarding the importance of the tasks; examining whether there is an association between the professionals’ individual characteristics and the importance they attach to the tasks. Two professional groups were surveyed: 105 financial officers of the top 500 Portuguese companies; and 412 Chartered Accountants. The results obtained allowed us to conclude that the respondents attach more importance to the traditional tasks, linked to the concept of a monetary-oriented accountant, and less importance to the more innovative tasks, related to business strategy; there is no convergence between the two professional groups in terms of the importance of the accountants’ participation in the strategic tasks. Regarding the association between individual characteristics and the level of importance assigned to the accounting tasks, we found an influence of the following characteristics: gender; academic degree of the professionals; and the institution where that degree was obtained.

  7. Tumor necrosis factor-like weak inducer of apoptosis and its receptor fibroblast growth factor-inducible 14 are expressed in urticarial vasculitis.

    Science.gov (United States)

    Li, Mengmeng; Chen, Tao; Guo, Zaipei; Li, Jingyi; Cao, Na

    2013-11-01

    Tumor necrosis factor (TNF)-like weak inducer of apoptosis (TWEAK), a member of the TNF family, has been implicated as a pro-inflammatory cytokine in many types of autoimmune and infectious diseases. However, information about TWEAK in dermatological diseases is limited. To date, no studies have investigated the roles of TWEAK in patients with urticarial vasculitis (UV). This study aimed to assess serum TWEAK levels, together with TWEAK and fibroblast growth factor-inducible 14 (Fn14) expressions of skin lesions in patients with UV. Serum TWEAK levels in patients with UV, together with patients with cutaneous leukocytoclastic angiitis (CLA) and healthy controls were detected by enzyme-linked immunosorbent assay; TWEAK and Fn14 expressions of skin lesions were analyzed by immunohistochemistry. Results showed that TWEAK and Fn14 were abundantly expressed in the dermal vessel wall of lesional skin in patients with UV but not healthy controls. Serum TWEAK levels in the acute stage in patients with UV were significantly higher than those in the convalescent stage and healthy controls. Serum TWEAK levels were elevated significantly in patients with CLA compared with those in healthy controls. Our previous study indicated that TWEAK may be an important mediator for the development of vascular inflammation in skin. In addition, we also found that TWEAK blockade substantially reduced vascular damage and perivascular leukocyte infiltrates in lipopolysaccharide-induced cutaneous vasculitis. Our study shows that TWEAK may be associated with the pathogenesis of UV; it is therefore suggested that TWEAK may be a potential therapeutic target for UV and other types of cutaneous vasculitis. © 2013 Japanese Dermatological Association.

  8. Adolescent girls' views on cosmetic surgery: A focus group study.

    Science.gov (United States)

    Ashikali, Eleni-Marina; Dittmar, Helga; Ayers, Susan

    2016-01-01

    This study examined adolescent girls' views of cosmetic surgery. Seven focus groups were run with girls aged 15-18 years (N = 27). Participants read case studies of women having cosmetic surgery, followed by discussion and exploration of their views. Thematic analysis identified four themes: (1) dissatisfaction with appearance, (2) acceptability of cosmetic surgery, (3) feelings about undergoing cosmetic surgery and (4) cosmetic surgery in the media. Results suggest the acceptability of cosmetic surgery varies according to the reasons for having it and that the media play an important role by normalising surgery and under-representing the risks associated with it. © The Author(s) 2014.

  9. Concentrated ERP Delivered in a Group Setting: A Replication Study.

    Science.gov (United States)

    Havnen, Audun; Hansen, Bjarne; Öst, Lars-Göran; Kvale, Gerd

    2017-09-01

    In a previous effectiveness study (Havnen et al., 2014), 35 obsessive compulsive disorder (OCD) patients underwent Concentrated Exposure Treatment (cET), which is a newly developed group treatment format delivered over four consecutive days. The primary aims of the present study were to evaluate the treatment results for a new sample of OCD patients receiving the cET treatment approach and to replicate the effectiveness study described in Havnen et al. (2014). Forty-two OCD patients underwent cET treatment. Treatment was delivered by different therapists than in Havnen et al. (2014), except for two groups led by the developers of the treatment. Assessments of OCD symptom severity, treatment satisfaction, and occupational impairment were included. The results showed a significant reduction in Yale-Brown Obsessive Compulsive Scale scores from pre-treatment to post-treatment, which was maintained at 6-month follow-up. At post-treatment, 74% of the sample was remitted; at 6-month follow-up, 60% were recovered. The sample showed a very high degree of overall treatment satisfaction. The results from the present study were statistically compared with those obtained in the previous study. The analyses showed that the study samples had comparable demographic data and equal application of treatment. The outcome of the present and original study did not differ significantly on primary and secondary outcome measures. This study shows that cET was successfully replicated in a new patient sample treated by different therapists than the original study. The results indicate that cET is well accepted by the patients, and the potential for dissemination is discussed.

  10. Características generales de 29 pacientes con vasculitis de pequeños vasos General characteristics of 29 patients with small vessel vasculitis

    Directory of Open Access Journals (Sweden)

    Nicolás Di Benedetto

    2010-04-01

    Full Text Available El objetivo de esta serie fue describir las características generales y manifestaciones clínicas de pacientes con vasculitis de pequeños vasos que concurrieron al servicio de autoinmunidad de un hospital de la comunidad y compararlas con la literatura. Se revisaron de manera retrospectiva las historias clínicas archivadas bajo el rótulo de vasculitis en un período de 16 años. Se seleccionaron aquellos pacientes que cumplieran criterios diagnósticos de vasculitis de pequeños vasos. Se extrajeron los datos de interés y se constató la supervivencia mediante contacto telefónico. Posteriormente se realizó una búsqueda bibliográfica y se compararon los resultados. Se incluyeron 13 pacientes con granulomatosis de Wegener, 6 con síndrome de Churg Strauss y 10 con poliangeítis microscópica. El 55% (16 fueron menores de 55 años al diagnóstico y hubo predominio del sexo femenino (2.6:1. La demora diagnóstica fue mayor a un año en el 46% de los casos. Los sistemas más frecuentemente afectados fueron respiratorio y otorrinolaringológico. El 79% presentó anticuerpos anti-citoplasma de neutrófilos (ANCA positivos. La mortalidad fue del 24% (7/29. Al comparar las características de los pacientes incluidos en esta serie con lo descripto en la literatura, se hallaron diferencias en cuanto a la forma de presentación, sistemas comprometidos y porcentaje de pacientes ANCA positivos; además se observó que el resultado negativo de este marcador parece asociarse con mayor demora diagnóstica y en consecuencia peor pronóstico, lo que remarca su importancia como herramienta adicional no invasiva al momento del diagnóstico.The objective of this series was to describe the general characteristics and clinical manifestations of patients with small vessel vasculitis who were assisted in the autoimmunity department of a community public hospital and to compare the results with the literature. Clinical records under the label of vasculitis in a

  11. Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS)

    DEFF Research Database (Denmark)

    Walsh, Michael; Merkel, Peter A; Peh, Chen Au

    2013-01-01

    Granulomatosis with polyangiitis (GPA, Wegener's) and microscopic polyangiitis (MPA) are small vessel vasculitides collectively referred to as anti-neutrophil cytoplasm antibody-associated vasculitis (AAV). AAV is associated with high rates of morbidity and mortality due to uncontrolled disease...

  12. Contrasting genetic association of IL2RA with SLE and ANCA – associated vasculitis

    Directory of Open Access Journals (Sweden)

    Todd John A

    2009-03-01

    Full Text Available Abstract Background Autoimmune diseases are complex and have genetic and environmental susceptibility factors. The objective was to test the genetic association of systemic lupus erythematosus (SLE and anti-neutrophil cytoplasmic antibody (ANCA – associated systemic vasculitis (AAV with SNPs in the IL2RA region and to correlate genotype with serum levels of IL-2RA. Methods Using a cohort of over 700 AAV patients, two SLE case-control studies and an SLE trio collection (totalling over 1000 SLE patients, and a TaqMan genotyping approach, we tested 3 SNPs in the IL2RA locus, rs11594656, rs2104286 & rs41295061, each with a prior association with autoimmune disease; rs11594656 and rs41295061 with type 1 diabetes (T1D and rs2104286 with multiple sclerosis (MS and T1D. Results We show that SLE is associated with rs11594656 (P = 3.87 × 10-7 and there is some evidence of association of rs41295061 with AAV (P = 0.0122, which both have prior association with T1D. rs2104286, an MS and T1D – associated SNP in the IL2RA locus, is not associated with either SLE or AAV. Conclusion We have confirmed a previous suggestion that the IL2RA locus is associated with SLE and showed some evidence of association with AAV. Soluble IL-2RA concentrations correlate with rs11594656 genotype in quiescent disease in both AAV and SLE. Differential association of autoimmune diseases and SNPs within the IL2RA locus suggests that the IL2RA pathway may prove to play differing, as yet undefined, roles in each disease.

  13. Intravenous Cyclophosphamide and Plasmapheresis in Dialysis-Dependent ANCA-Associated Vasculitis

    Science.gov (United States)

    Pepper, Ruth J.; Chanouzas, Dimitrios; Tarzi, Ruth; Little, Mark A.; Casian, Alina; Walsh, Michael; Pusey, Charles D.; Harper, Lorraine

    2013-01-01

    Summary Background and objectives Induction therapy with oral cyclophosphamide (CYP) has been a mainstay of treatment in patients with severe renal failure secondary to ANCA-associated vasculitis (AAV). Recent evidence proposes using pulsed intravenous CYP in less severe disease to minimize adverse events. It is unclear if this can be translated to those with dialysis-dependent renal insufficiency. Design, setting, participants, & methods All AAV patients presenting between 2005 and 2010 requiring dialysis at presentation were retrospectively analyzed. Patients were treated with plasma exchange, corticosteroids, and intravenous CYP. Rate of dialysis independence at 3 and 12 months and adverse effects were assessed and compared with the outcome of the plasmapheresis, prednisolone, and oral CYP arm of the randomized MEPEX (methylprednisolone versus plasma exchange) trial. Results Forty-one patients were included. At 3 months, 3 (7.3%) patients had died on dialysis, 12 (29.3%) remained dialysis dependent, and 26 (63.4%) were dialysis independent (creatinine, 2.5 mg/dl; GFR, 26 ml/min per 1.73 m2). Four patients subsequently reached ESRD at a median time of 83 days. Thirty-seven (90%) patients reached 1 year follow-up, 13 (35%) remained dialysis dependent, and 24 (65%) had independent renal function. Eleven patients (27%) had episodes of leukopenia (white cell count dialysis-dependent cohort treated with plasmapheresis in the MEPEX study in which 51% were alive with independent renal function at 1 year. Conclusions Intravenous CYP used with corticosteroids and plasmapheresis may be an effective alternative to oral CYP in patients with dialysis-dependent AAV. PMID:23160261

  14. A Clinico - Aetiological Study Of Dermatoses In Paediatric Age Group

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    Ghosh Sadhan K

    1995-01-01

    Full Text Available Five hundred patients of the age group 0-12 years were studied for different types of dermatoses. Pyoderma (35.6%, scabies (22.4% and eczema (17.6% were the most common dermatological conditions, followed by molluscum contagiosum (4.6%, popular urticaria with insect bite (4%, vitiligo (3.4%, miliaria (2.8%, nevus (1.6%. Other dermatoses (8% were pityriasis rosea, wart, chicken pox, herpes zoster, acne vulgaris, leprosy, angular stomatitis, pruritus vulvae, psoriasis, candidiasis, condylomatalata, fixed drug relation, tinea capitis and corporis, phrynoderma, alopecia areata, phimosis, geographic tongue, trichotillomania, canitis, pediculosis, hypertrophic scar and pityriasis versicolor.

  15. A case of central nervous system vasculitis related to an episode of Guillain-Barrè syndrome

    OpenAIRE

    Sinardi, Daniele; Spada, Antonella; Marino, Antonella; Mondello, Epifanio

    2000-01-01

    The authors report their knowledge about an uncommon case of isolated vasculitis, restricted to the left sylvian artery during an auto-immune Guillain-Barrè syndrome (GBS), sustained by cytomegalovirus (CMV). An acute cardiopulmonary failure requiring a ventilator and vasopressor support manifested, notwithstanding plasma exchanging and immune-modulating therapy. An IgM-enriched formula administration coincided with a rapid amelioration of GBS and vasculitis to a complete recovery the next mo...

  16. Hypocomplementemic Urticarial Vasculitis Syndrome in an 8-year-old Boy: A Case Report and Review of Literature

    Science.gov (United States)

    Al Mosawi, Zakiya Saleh Adnan; Al Hermi, Badriya Ebrahim Ahmed

    2013-01-01

    Hypocomplementemic urticarial vasculitis syndrome is an immune complex-mediated disease of unknown etiology. The clinical course is characterized by urticaria, conjunctivitis, joint pain, and hypocomplementemia. We here report a case of a child with hypocomplementemic urticarial vasculitis syndrome that progressed to nephritis. Renal biopsy was consistent with diffuse proliferative glomerulonephritis with diffuse subendothelial immune deposits. He responded well to a combination of steroid and mofetil micofenolate. PMID:23904922

  17. Hypocomplementemic Urticarial Vasculitis Syndrome in an 8-year-old Boy: A Case Report and Review of Literature

    Directory of Open Access Journals (Sweden)

    Zakiya Saleh Adnan Al Mosawi

    2013-07-01

    Full Text Available Hypocomplementemic urticarial vasculitis syndrome is an immune complex-mediated disease of unknown etiology. The clinical course is characterized by urticaria, conjunctivitis, joint pain, and hypocomplementemia. We here report a case of a child with hypocomplementemic urticarial vasculitis syndrome that progressed to nephritis. Renal biopsy was consistent with diffuse proliferative glomerulonephritis with diffuse subendothelial immune deposits. He responded well to a combination of steroid and mofetil micofenolate.

  18. Group Norms as Moderator in the Effect of Cross Group Friendship on Outgroup Attitude: a Study on Interreligious Group in Indonesia

    OpenAIRE

    Yustisia, Whinda

    2016-01-01

    Past studies indicate that the effect of intergroup contact on outgroup attitude is not isolated to contextual factors. One of the contextual factors that has begun to be studied is group norm. However, group norm in these studies is still merely conceptualized as the perception of how ingroup members evaluate outgroup members. In fact, according to norm focus theory, in a given context, individuals are influenced, at least, by two types of group norms, namely injunctive norms (i.e., what mos...

  19. Actions to promote energy efficient electric motors. Motors study group

    Energy Technology Data Exchange (ETDEWEB)

    Almeida, A.T. de [Coimbra Univ. (PT). Inst. of Systems and Robotics (ISR)

    1996-10-01

    Motor electricity consumption is influenced by many factors including: motor efficiency, motor speed controls, power supply quality, harmonics, systems oversizing, distribution network, mechanical transmission system, maintenance practices, load management and cycling, and the efficiency of the end-use device (e.g. fan, pump, etc.). Due to their importance, an overview of these factors is presented in this report. This study also describes the electricity use in the industrial and tertiary sectors and the electricity consumption associated with the different types of electric motors systems in the Member States of the European Union, as well as estimated future evolution until 2010. The studies for individual countries were carried out by the different partners of the motors study group at a previous stage. The study has found that there is a lack of accurate information about the motor electricity consumption, installed motor capacity and the motor market in almost all the European Union countries and only some general statistical sources are available. There is little field data, which is mainly available in Denmark, France, Italy and the Netherlands. Due to this lack of primary information, some common assumptions were made, based on the experience of the members of the study group. This lack of end-use characterisation data shows the need for improvement from the point of view of current knowledge. It is therefore recommended that further research is undertaken to arrive at more accurate figures. These could be the basis for a better understanding for motor use in practice and - as a consequence - for a more precise appraisal of potentials and barriers to energy efficiency. (orig.)

  20. The Use of Nominal Group Technique: Case Study in Vietnam

    Science.gov (United States)

    Dang, Vi Hoang

    2015-01-01

    The Nominal Group Technique (NGT) is a structured process to gather information from a group. The technique was first described in early 1970s and has since become a widely-used standard to facilitate working groups. The NGT is effective for generating large numbers of creative new ideas and for group priority setting. This article reports on a…

  1. Group hypnotherapy versus group relaxation for smoking cessation: an RCT study protocol

    Science.gov (United States)

    2012-01-01

    Background A significant number of smokers would like to stop smoking. Despite the demonstrated efficacy of pharmacological smoking cessation treatments, many smokers are unwilling to use them; however, they are inclined to try alternative methods. Hypnosis has a long-standing reputation in smoking cessation therapy, but its efficacy has not been scientifically proven. We designed this randomised controlled trial to evaluate the effects of group hypnosis as a method for smoking cessation, and we will compare the results of group hypnosis with group relaxation. Methods/Design This is a randomised controlled trial (RCT) to compare the efficacy of a single session of hypnosis with that of relaxation performed in groups of 8-15 smokers. We intend to include at least 220 participants in our trial. The inclusion criteria include smoking at least 5 cigarettes per day, not using other cessation methods and being willing to quit smoking. The intervention is performed by a trained hypnotist/relaxation therapist. Both groups first receive 40 min of mental preparation that is based on motivational interviewing. Then, a state of deep relaxation is induced in the hypnosis condition, and superficial relaxation is induced in the control condition. Suggestions are made in the hypnosis condition that aim to switch the mental self-image of the participants from that of smokers to that of non-smokers. Each intervention lasts for 40 min. The participants also complete questionnaires that assess their smoking status and symptoms of depression and anxiety at baseline, 2 weeks and 6 months post-intervention. In addition, saliva samples are collected to assess cotinine levels at baseline and at 6 months post-intervention. We also assess nicotine withdrawal symptoms at 2 weeks post-intervention. Discussion To the best of our knowledge, this RCT is the first to test the efficacy of group hypnosis versus group relaxation. Issues requiring discussion in the outcome paper include the lack of

  2. Group hypnotherapy versus group relaxation for smoking cessation: an RCT study protocol

    Directory of Open Access Journals (Sweden)

    Dickson-Spillmann Maria

    2012-04-01

    Full Text Available Abstract Background A significant number of smokers would like to stop smoking. Despite the demonstrated efficacy of pharmacological smoking cessation treatments, many smokers are unwilling to use them; however, they are inclined to try alternative methods. Hypnosis has a long-standing reputation in smoking cessation therapy, but its efficacy has not been scientifically proven. We designed this randomised controlled trial to evaluate the effects of group hypnosis as a method for smoking cessation, and we will compare the results of group hypnosis with group relaxation. Methods/Design This is a randomised controlled trial (RCT to compare the efficacy of a single session of hypnosis with that of relaxation performed in groups of 8-15 smokers. We intend to include at least 220 participants in our trial. The inclusion criteria include smoking at least 5 cigarettes per day, not using other cessation methods and being willing to quit smoking. The intervention is performed by a trained hypnotist/relaxation therapist. Both groups first receive 40 min of mental preparation that is based on motivational interviewing. Then, a state of deep relaxation is induced in the hypnosis condition, and superficial relaxation is induced in the control condition. Suggestions are made in the hypnosis condition that aim to switch the mental self-image of the participants from that of smokers to that of non-smokers. Each intervention lasts for 40 min. The participants also complete questionnaires that assess their smoking status and symptoms of depression and anxiety at baseline, 2 weeks and 6 months post-intervention. In addition, saliva samples are collected to assess cotinine levels at baseline and at 6 months post-intervention. We also assess nicotine withdrawal symptoms at 2 weeks post-intervention. Discussion To the best of our knowledge, this RCT is the first to test the efficacy of group hypnosis versus group relaxation. Issues requiring discussion in the outcome

  3. Group hypnotherapy versus group relaxation for smoking cessation: an RCT study protocol.

    Science.gov (United States)

    Dickson-Spillmann, Maria; Kraemer, Thomas; Rust, Kristina; Schaub, Michael

    2012-04-04

    A significant number of smokers would like to stop smoking. Despite the demonstrated efficacy of pharmacological smoking cessation treatments, many smokers are unwilling to use them; however, they are inclined to try alternative methods. Hypnosis has a long-standing reputation in smoking cessation therapy, but its efficacy has not been scientifically proven. We designed this randomised controlled trial to evaluate the effects of group hypnosis as a method for smoking cessation, and we will compare the results of group hypnosis with group relaxation. This is a randomised controlled trial (RCT) to compare the efficacy of a single session of hypnosis with that of relaxation performed in groups of 8-15 smokers. We intend to include at least 220 participants in our trial. The inclusion criteria include smoking at least 5 cigarettes per day, not using other cessation methods and being willing to quit smoking. The intervention is performed by a trained hypnotist/relaxation therapist. Both groups first receive 40 min of mental preparation that is based on motivational interviewing. Then, a state of deep relaxation is induced in the hypnosis condition, and superficial relaxation is induced in the control condition. Suggestions are made in the hypnosis condition that aim to switch the mental self-image of the participants from that of smokers to that of non-smokers. Each intervention lasts for 40 min. The participants also complete questionnaires that assess their smoking status and symptoms of depression and anxiety at baseline, 2 weeks and 6 months post-intervention. In addition, saliva samples are collected to assess cotinine levels at baseline and at 6 months post-intervention. We also assess nicotine withdrawal symptoms at 2 weeks post-intervention. To the best of our knowledge, this RCT is the first to test the efficacy of group hypnosis versus group relaxation. Issues requiring discussion in the outcome paper include the lack of standardisation of hypnotic

  4. A case of urticarial vasculitis in a female patient with lupus: Mycoplasma pneumoniae infection or lupus reactivation?

    Science.gov (United States)

    Diplomatico, Mario; Gicchino, Maria Francesca; Ametrano, Orsola; Marzuillo, Pierluigi; Olivieri, Alma Nunzia

    2017-05-01

    A 17-year-old female patient affected by systemic lupus erythematosus (SLE) (who had been taking 300 mg/die of hydroxychloroquine for 3 years), Graves' disease (treated with 10 mg/die of tapazole), and celiac disease came to our attention for urticarial vasculitis. She had been taking prednisone (25 mg/die) for 3 days, and her blood tests showed high levels of Mycoplasma pneumoniae IgM and IgG antibodies. The association between urticaria and M. pneumoniae infections can be present in up to 7% of the cases and, to the best of our knowledge, only two reports of urticarial vasculitis and M. pneumoniae in adults are available in the literature. Urticarial vasculitis can also be a rare cutaneous manifestation of SLE (affecting 2% of the patients), and our case is the first in the literature describing the coexistence of M. pneumoniae infection, SLE, and urticarial vasculitis in a pediatric patient, a case that rises an important differential diagnosis issue about the origin of urticarial vasculitis: SLE reactivation or urticarial vasculitis due to M. pneumoniae infection?

  5. Diagnosis related group grouping study of senile cataract patients based on E-CHAID algorithm

    Directory of Open Access Journals (Sweden)

    Ai-Jing Luo

    2018-02-01

    Full Text Available AIM: To figure out the contributed factors of the hospitalization expenses of senile cataract patients (HECP and build up an area-specified senile cataract diagnosis related group (DRG of Shanghai thereby formulating the reference range of HECP and providing scientific basis for the fair use and supervision of the health care insurance fund. METHODS: The data was collected from the first page of the medical records of 22 097 hospitalized patients from tertiary hospitals in Shanghai from 2010 to 2012 whose major diagnosis were senile cataract. Firstly, we analyzed the influence factors of HECP using univariate and multivariate analysis. DRG grouping was conducted according to the exhaustive Chi-squared automatic interaction detector (E-CHAID model, using HECP as target variable. Finally we evaluated the grouping results using non-parametric test such as Kruskal-Wallis H test, RIV, CV, etc. RESULTS: The 6 DRGs were established as well as criterion of HECP, using age, sex, type of surgery and whether complications/comorbidities occurred as the key variables of classification node of senile cataract cases. CONCLUSION: The grouping of senile cataract cases based on E-CHAID algorithm is reasonable. And the criterion of HECP based on DRG can provide a feasible way of management in the fair use and supervision of medical insurance fund.

  6. Study of the Moraxella group. I. Genus Moraxella and the Neisseria catarrhalis group.

    Science.gov (United States)

    Baumann, P; Doudoroff, M; Stanier, R Y

    1968-01-01

    A number of strains of oxidase-positive moraxellas and of neisserias related to Neisseria catarrhalis were characterized with respect to a number of nutritional and physiological properties and could be assigned to several species or species groups on the basis of their phenotypic traits. This grouping was consistent with that established by Bövre on the basis of transformation frequencies for streptomycin resistance. It is proposed to reserve the generic name Moraxella for the oxidase-positive rodshaped organisms, and a redescription of the genus is offered. Following the recent taxonomic proposals of Bövre and Henriksen, the specific name Moraxella osloensis is applied to the nutritionally unexacting strains that accumulate poly-beta-hydroxybutyrate as carbon reserve. The nutritionally exacting strains are assigned to three distinct groups which can be regarded as separate species or as varieties of M. lacunata. The epithets applicable to these groups appear to be lacunata, nonliquefaciens, and bovis. The "false neisserias" could be assigned to at least three subgroups, one of which constitutes the clearly defined entity, N. catarrhalis, which could be distinguished from N. caviae and N. ovis.

  7. Study of the Moraxella Group I. Genus Moraxella and the Neisseria catarrhalis Group1

    Science.gov (United States)

    Baumann, P.; Doudoroff, M.; Stanier, R. Y.

    1968-01-01

    A number of strains of oxidase-positive moraxellas and of neisserias related to Neisseria catarrhalis were characterized with respect to a number of nutritional and physiological properties and could be assigned to several species or species groups on the basis of their phenotypic traits. This grouping was consistent with that established by Bövre on the basis of transformation frequencies for streptomycin resistance. It is proposed to reserve the generic name Moraxella for the oxidase-positive rodshaped organisms, and a redescription of the genus is offered. Following the recent taxonomic proposals of Bövre and Henriksen, the specific name Moraxella osloensis is applied to the nutritionally unexacting strains that accumulate poly-β-hydroxybutyrate as carbon reserve. The nutritionally exacting strains are assigned to three distinct groups which can be regarded as separate species or as varieties of M. lacunata. The epithets applicable to these groups appear to be lacunata, nonliquefaciens, and bovis. The “false neisserias” could be assigned to at least three subgroups, one of which constitutes the clearly defined entity, N. catarrhalis, which could be distinguished from N. caviae and N. ovis. Images PMID:4866103

  8. Clozapine-Induced Late Agranulocytosis and Severe Neutropenia Complicated with Streptococcus pneumonia, Venous Thromboembolism, and Allergic Vasculitis in Treatment-Resistant Female Psychosis

    Directory of Open Access Journals (Sweden)

    Christina Voulgari

    2015-01-01

    Full Text Available Clozapine is a second-generation antipsychotic agent from the benzodiazepine group indicated for treatment-resistant schizophrenia and other psychotic conditions. Using clozapine earlier on once a case appears to be refractory limits both social and personal morbidity of chronic psychosis. However treatment with second-generation antipsychotics is often complicated by adverse effects. We present a case of a 33-year-old Caucasian woman with a 25-year history of refractory psychotic mania after switching to a 2-year clozapine therapy. She presented clozapine-induced absolute neutropenia, agranulocytosis, which were complicated by Streptococcus pneumonia and sepsis. Clozapine-induced thromboembolism of the common femoral and right proximal iliac vein, as well as allergic vasculitis, was diagnosed. She achieved full remission on granulocyte-colony stimulating factor and specific antibiotic treatment. Early detection of severe clozapine-induced absolute neutropenia and agranulocytosis enabled the effective treatment of two among its most severe complications. Additional evidence to the previously reported possible causal relation between clozapine and venous thromboembolism is offered. Finally, clozapine-induced allergic vasculitis is confirmed as a late adverse effect of clozapine therapy.

  9. Antenatal screening for Group B Streptococcus: A diagnostic cohort study

    Directory of Open Access Journals (Sweden)

    Darbyshire Philip

    2005-07-01

    Full Text Available Abstract Background A range of strategies have been adopted to prevent early onset Group B Streptococcal (EOGBS sepsis, as a consequence of Group B Streptococcal (GBS vertically acquired infection. This study was designed to provide a scientific basis for optimum timing and method of GBS screening in an Australian setting, to determine whether screening for GBS infection at 35–37 weeks gestation has better predictive values for colonisation at birth than screening at 31–33 weeks, to examine the test characteristics of a risk factor strategy and to determine the test characteristics of low vaginal swabs alone compared with a combination of perianal plus low vaginal swabs per colonisation during labour. Methods Consented women received vaginal and perianal swabs at 31–33 weeks gestation, 35–38 weeks gestation and during labour. Swabs were cultured on layered horse blood agar and inoculated into selective broth prior to analysis. Test characteristics were calculated with exact confidence intervals for a high risk strategy and for antenatal screening at 31–33 and 35–37 weeks gestation for vaginal cultures alone, perianal cultures alone and combined low vaginal and perianal cultures. Results The high risk strategy was not informative in predicting GBS status during labour. There is an unequivocal benefit for the identification of women colonised with GBS during labour associated with delaying screening until 36 weeks however the results for method of screening were less definitive with no clear advantage in using a combined low vaginal and perianal swabbing regimen over the use of a low vaginal swab alone. Conclusion This study can contribute to the development of prevention strategies in that it provides clear evidence for optimal timing of swabs. The addition of a perianal swab does not confer clear benefit. The quantification of advantages and disadvantages provided in this study will facilitate communication with clinicians and

  10. Recommendations of the Brazilian Society of Rheumatology for the induction therapy of ANCA-associated vasculitis

    Directory of Open Access Journals (Sweden)

    Alexandre Wagner Silva de Souza

    Full Text Available Abstract The purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV patients with active disease. The recommendations proposed by the Vasculopathies Committee of the Brazilian Society Rheumatology for induction therapy of AAV, including granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited vasculitis, were based on systematic literature review and expert opinion. Literature review was performed using Medline (PubMed, EMBASE and Cochrane database to retrieve articles until October 2016. PRISMA guidelines were used for the systematic review and articles were assessed according to the Oxford levels of evidence. Sixteen recommendations were made regarding different aspects of induction therapy for AAV. The purpose of these recommendations is to serve as a guide for therapeutic decisions by health care professionals in the management of AAV patients presenting active disease.

  11. [Agranulocytosis and vasculitis in a cocaine addict: levamisole, the hidden culprit].

    Science.gov (United States)

    Lemaignen, A; Goulenok, T; Kalamarides, S; Plat, A; Pfau, G; Fantin, B

    2014-10-01

    Adulterants are compounds added to street drugs to increase profits for the seller. Levamisole, a veterinary antihelminthic agent, has become the most common adulterant of cocaine. The prevalence of levamisole in samples of cocaine is increasing. Levamisole can lead to neutropenia and to a dramatic vasculopathy and even vasculitis of small and medium-size blood vessels. We here reported the first French case of levamisole related toxicity, due to cocaine use in a 50-year-old man, revealed by fever and agranulocytosis, high titters of antineutrophil cytoplasmic antibodies (ANCA), anticoagulant and positive Coombs tests. Outcome was slowly favorable with exposition withdrawal. Clinicians should be aware that agranulocytosis or vasculitis or vasculopathy could be related to levamisole toxicity in individuals who use cocaine. Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  12. Levamisole-induced myopathy and leukocytoclastic vasculitis: a case report and literature review.

    Science.gov (United States)

    Tsai, Meng-Hsuan; Yang, Jen-Hung; Kung, Sheng-Ling; Hsiao, Yu-Ping

    2013-01-01

    Levamisole, an immunomodulator and anthelmintic medication, has been used in dermatology for years. Even though the adverse effects are usually mild and reversible, attention should be paid toward severe events such as vasculitis and neutropenia. To the best of our knowledge, this is the first case report on a patient presenting with myopathy caused by levamisole. Here, we report a 34-year-old woman with recalcitrant warts who received levamisole 100 mg daily for 5 days. Subsequently, bilateral lower limb weakness accompanied by multiple painful and non-blanchable purpura was noted. Levamisole-induced myopathy and leukocytoclastic vasculitis were diagnosed by skin histopathology, direct immunofluorescence, and electromyography. After discontinuing levamisole and giving a short course of systemic steroid, these symptoms demonstrated a resolving trend. © 2013 Wiley Periodicals, Inc.

  13. ANCA Associated Vasculitis Secondary to Levamisole-Adultered Cocaine with Associated Membranous Nephropathy: A Case Series.

    Science.gov (United States)

    Collister, David; Sathianathan, Christie; Ryz, Krista; Karpinski, Martin; Bernstein, Keevin; Gibson, Ian W

    2017-01-01

    Cocaine is a risk factor for acute kidney injury and chronic kidney disease with progression to end-stage renal disease. Levamisole is an adulterant that is added to cocaine to enhance its euphoric effects. Levamisole-adulterated cocaine (LAC) is associated with the distinct clinical syndromes of agranulocytosis, leukocytoclastic vasculitis, cocaine-induced midline destructive lesions (CIMDL), and ANCA-associated vasculitis (AAV) with pauci-immune necrotizing glomerulonephritis. We reviewed all cases of AAV secondary to LAC at our institution. We report 3 cases of AAV secondary to LAC and associated membranous nephropathy (MN). The first and second cases are concurrent AAV secondary to LAC and associated MN while the third case involves the development of MN after AAV secondary to LAC. Clinicians should be aware of this novel association of LAC with MN. © 2017 S. Karger AG, Basel.

  14. Membranous nephropathy with crescents associated with levamisole-induced MPO-ANCA vasculitis.

    Science.gov (United States)

    Moinuddin, Irfan; Madhrira, Machaiah; Bracamonte, Erika; Thajudeen, Bijin; Sussman, Amy

    2016-07-01

    ANCA-associated vasculitis (AAV) is the most common cause of crescentic rapidly progressive glomerulonephritis (GN). Levamisole used as an adulterant in cocaine is increasingly recognized as a cause of AAV. We report the case of a 50 year old woman with atypical anti-MPO AAV associated with cocaine use and exposure to levamisole. In addition to the clinical and pathologic findings of crescentic GN, the patient also had biopsy evidence of secondary membranous nephropathy (MN). Although AAV and MN have been reported previously in the same patient and both have been induced by drug exposures, this is the first report of MN in a patient with AAV likely induced by levamisole. We suggest that MPO can cause both pauci-immune vasculitis and secondary membranous nephropathy in some cases, as in cases of levamisole-adulterated cocaine use. Copyright © 2016 Elsevier GmbH. All rights reserved.

  15. Mycoplasma pneumoniae infection associated with urticarial vasculitis mimicking adult-onset Still's disease.

    Science.gov (United States)

    Dua, Janet; Nandagudi, Anupama; Sutcliffe, Nurhan

    2012-12-01

    Mycoplasma pneumoniae is well known to be a frequent cause of atypical pneumonia worldwide. However, it may also present with a wide variety of clinical features, including cutaneous symptoms, which are not widely recognised. Urticarial vasculitis occurring with M. pneumoniae has been described to occur in only one other case report. This amalgamation of non-specific clinical symptoms and signs can lead to a diagnostic dilemma. We describe a case of M. pneumoniae infection presenting with extrapulmonary manifestations and urticarial vasculitis, which was misdiagnosed as adult-onset Still's disease (AOSD). Had immunosuppressive therapy been commenced for AOSD in the presence of undiagnosed infection, this may have resulted in potentially serious consequences. This case highlights the need to remain vigilant about diagnosing M. pneumoniae as its serological diagnosis may take weeks and it has many extrapulmonary manifestations, which can masquerade as other conditions.

  16. p-ANCA-Associated Vasculitis Caused by Levamisole-Adulterated Cocaine: A Case Report

    Directory of Open Access Journals (Sweden)

    Michelle R. Carter

    2013-01-01

    Full Text Available A rare case of a patient with an unusual skin rash after using cocaine is presented. A clinical diagnosis of levamisole-induced vasculitis was made based on initial presentation of purpuric skin lesions involving the ears and positive cocaine on urine toxicology screening. The diagnosis was confirmed after laboratory findings of neutropenia, positive serum levamisole, and a histopathologic test of the skin lesions. The pathophysiology of this condition is discussed. Treatment with methylprednisolone and prednisone led to the resolution of the neutropenia and complete recovery of the skin lesions. With the growing use of levamisole-tainted cocaine, clinicians should be aware of the symptoms of vasculitis and neutropenia induced by this combination of drugs to avoid unnecessary tests and delayed diagnosis.

  17. Group hypnotherapy versus group relaxation for smoking cessation: an RCT study protocol

    OpenAIRE

    Dickson-Spillmann Maria; Kraemer Thomas; Rust Kristina; Schaub Michael

    2012-01-01

    Abstract Background A significant number of smokers would like to stop smoking. Despite the demonstrated efficacy of pharmacological smoking cessation treatments, many smokers are unwilling to use them; however, they are inclined to try alternative methods. Hypnosis has a long-standing reputation in smoking cessation therapy, but its efficacy has not been scientifically proven. We designed this randomised controlled trial to evaluate the effects of group hypnosis as a method for smoking cessa...

  18. Isolated periostitis as a manifestation of systemic vasculitis in a child: imaging features.

    Science.gov (United States)

    Cheon, Jung-Eun; Kim, In-One; Kim, Woo Sun; Yeon, Kyung Mo

    2010-12-01

    We report a case of isolated periostitis associated with Takayasu arteritis in a 10-year-old boy presenting with calf pain. Radiographs revealed a localized, thick periosteal reaction with irregular margin in the proximal left fibula. MRI revealed irregular thickening with enhancement of the periosteum in the proximal fibula and heterogeneous enhancement along vascular bundles in the calf. Isolated periostitis is a rare skeletal manifestation of systemic vasculitis that could be misdiagnosed as neoplastic or traumatic periosteal reaction.

  19. Isolated periostitis as a manifestation of systemic vasculitis in a child: imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Cheon, Jung-Eun; Kim, In-One; Kim, Woo Sun; Yeon, Kyung Mo [Seoul National University College of Medicine and the Institute of Radiation Medicine, Department of Radiology, 101 Daehang-ro Jongro-gu, Seoul (Korea, Republic of)

    2010-12-15

    We report a case of isolated periostitis associated with Takayasu arteritis in a 10-year-old boy presenting with calf pain. Radiographs revealed a localized, thick periosteal reaction with irregular margin in the proximal left fibula. MRI revealed irregular thickening with enhancement of the periosteum in the proximal fibula and heterogeneous enhancement along vascular bundles in the calf. Isolated periostitis is a rare skeletal manifestation of systemic vasculitis that could be misdiagnosed as neoplastic or traumatic periosteal reaction. (orig.)

  20. Plasma exchange in antineutrophil cytoplasmic antibody-associated vasculitis--a 25-year perspective

    DEFF Research Database (Denmark)

    Szpirt, Wladimir M

    2015-01-01

    Demonstration of a pathogenic role for antineutrophil cytoplasmic antibodies (ANCA) underlies the scientific rationale for plasma exchange (PLEX) in the treatment of ANCA-associated vasculitis (AAV). Most clinical evidence of efficacy concerns the use of PLEX for the recovery of renal function...... in severe nephritis, when used in conjunction with immunosuppressive drug therapy. The development of PLEX for this indication, the strength of the clinical trial evidence supporting its use, its roles in other AAV indications and ongoing research are discussed....

  1. Postoperative spinal infection mimicking systemic vasculitis with titanium-spinal implants

    Directory of Open Access Journals (Sweden)

    Stathopoulos Konstantinos

    2011-09-01

    Full Text Available Abstract Background Secondary systemic vasculitis after posterior spinal fusion surgery is rare. It is usually related to over-reaction of immune-system, to genetic factors, toxicity, infection or metal allergies. Case Description A 14 year-old girl with a history of extended posterior spinal fusion due to idiopathic scoliosis presented to our department with diffuse erythema and nephritis (macroscopic hemuresis and proteinuria 5 months post surgery. The surgical trauma had no signs of inflammation or infection. The blood markers ESR and CRP were increased. Skin tests were positive for nickel allergy, which is a content of titanium alloy. The patient received corticosteroids systematically (hydrocortisone 10 mg for 6 months, leading to total recess of skin and systemic reaction. However, a palpable mass close to the surgical wound raised the suspicion of a late infection. The patient had a second surgery consisting of surgical debridement and one stage revision of posterior spinal instrumentation. Intraoperative cultures were positive to Staphylococcus aureus. Intravenous antibiotics were administered. The patient is now free of symptoms 24 months post revision surgery without any signs of recurrence of either vasculitis or infection. Literature Review Systemic vasculitis after spinal surgery is exceptionally rare. Causative factors are broad and sometimes controversial. In general, it is associated with allergy to metal ions. This is usually addressed with metal on metal total hip bearings. In spinal surgery, titanium implants are considered to be inert and only few reports have presented cases with systemic vasculitides. Therefore, other etiologies of immune over-reaction should always be considered, such as drug toxicity, infection, or genetic predisposition. Purposes and Clinical Relevance Our purpose was to highlight the difficulties during the diagnostic work-up for systemic vasculitis and management in cases of posterior spinal surgery.

  2. Disseminated Burkholderia gladioli infection in a lung transplant recipient with underlying hypocomplementemic urticarial vasculitis.

    Science.gov (United States)

    Thompson, G R; Wickes, B L; Herrera, M L; Haman, T C; Lewis, J S; Jorgensen, J H

    2011-12-01

    Burkholderia gladioli is difficult to definitively identify within the laboratory using phenotypic testing alone. We describe a case of recurrent B. gladioli infection in a lung transplant recipient with underlying hypocomplementemic urticarial vasculitis syndrome, discuss the difficulties encountered with laboratory identification, provide a review of the methodology required for definitive identification, and discuss potential pathophysiologic mechanisms in this patient responsible for the difficulty in treatment. © 2011 John Wiley & Sons A/S.

  3. Unusual exanthema combined with cerebral vasculitis in pneumococcal meningitis: a case report

    OpenAIRE

    Raissaki Maria; Geromarkaki Elisabeth; Ilia Stavroula; Spanaki Anna-Maria; Tavladaki Theonimfi; Briassoulis George

    2011-01-01

    Abstract Introduction Bacterial meningitis is a complex, rapidly progressive disease in which neurological injury is caused in part by the causative organism and in part by the host's own inflammatory responses. Case presentation We present the case of a two-year-old Greek girl with pneumococcal meningitis and an atypical curvilinear-like skin eruption, chronologically associated with cerebral vasculitis. A diffusion-weighted MRI scan showed lesions with restricted diffusion, reflecting local...

  4. Report of the Study Group on Assessment and Evaluation

    CERN Document Server

    Crouch, R; Netter, K; Crouch, Richard; Gaizuaskas, Robert; Netter, Klaus

    1996-01-01

    This is an interim report discussing possible guidelines for the assessment and evaluation of projects developing speech and language systems. It was prepared at the request of the European Commission DG XIII by an ad hoc study group, and is now being made available in the form in which it was submitted to the Commission. However, the report is not an official European Commission document, and does not reflect European Commission policy, official or otherwise. After a discussion of terminology, the report focusses on combining user-centred and technology-centred assessment, and on how meaningful comparisons can be made of a variety of systems performing different tasks for different domains. The report outlines the kind of infra-structure that might be required to support comparative assessment and evaluation of heterogenous projects, and also the results of a questionnaire concerning different approaches to evaluation.

  5. Accountable Metadata-Hiding Escrow: A Group Signature Case Study

    Directory of Open Access Journals (Sweden)

    Kohlweiss Markulf

    2015-06-01

    Full Text Available A common approach to demands for lawful access to encrypted data is to allow a trusted third party (TTP to gain access to private data. However, there is no way to verify that this trust is well placed as the TTP may open all messages indiscriminately. Moreover, existing approaches do not scale well when, in addition to the content of the conversation, one wishes to hide one’s identity. Given the importance of metadata this is a major problem. We propose a new approach in which users can retroactively verify cryptographically whether they were wiretapped. As a case study, we propose a new signature scheme that can act as an accountable replacement for group signatures, accountable forward and backward tracing signatures.

  6. Condensation of phenolic groups during coal liquefaction model compound studies

    Energy Technology Data Exchange (ETDEWEB)

    Trewhella, M.J.; Grint, A.

    1988-08-01

    The pyrolysis of 1-naphthol in nitrogen and hydrogen, with and without a donor solvent, has been studied. The results show that in the absence of a source of donatable hydrogen, phenolic groups can condense at around 450 degrees C to form fused furan type structures. The presence of a hydrogen donor (e.g. tetralin) or, to a lesser extent, gaseous hydrogen, eliminates this reaction. In the condensation reaction of 1-naphthol to dibenzofuran, the inhibition by tetralin and the product distribution were, in all cases, consistent with a mechanism involving the generation of free radicals via a bimolecular hydrogen atom transfer reaction. This is distinctly different from other commonly accepted radical production mechanisms involved in coal liquefaction or pyrolysis. The implications for low-rank coal hydroliquefaction are discussed in the light of these findings. 3 refs., 3 figs., 2 tabs.

  7. Functional renormalization group study of the Anderson-Holstein model

    Science.gov (United States)

    Laakso, M. A.; Kennes, D. M.; Jakobs, S. G.; Meden, V.

    2014-02-01

    We present a comprehensive study of the spectral and transport properties in the Anderson-Holstein model both in and out of equilibrium using the functional renormalization group (fRG). We show how the previously established machinery of Matsubara and Keldysh fRG can be extended to include the local phonon mode. Based on the analysis of spectral properties in equilibrium we identify different regimes depending on the strength of the electron-phonon interaction and the frequency of the phonon mode. We supplement these considerations with analytical results from the Kondo model. We also calculate the nonlinear differential conductance through the Anderson-Holstein quantum dot and find clear signatures of the presence of the phonon mode.

  8. The narrow range of perceived predation: a 19 group study

    Directory of Open Access Journals (Sweden)

    Olivier Mesly

    2013-05-01

    Full Text Available This paper rests largely on the works of Mesly (1999 to 2012. It argues that the phenomenon of perceived predation as a functional behavioural phenomenon is subjected to certain limits, a finding based on studies performed on 19 different groups spread over a four-year span. It also finds a constant of k = 1.3 which reflects the invariant nature of perceived predation. These findings add to the theory of financial predation which stipulates that financial predators operate below the limits of detection pertaining to their customers (and market regulators. They are experts at minimizing the perception that clients could have that they are after their money, causing them financial harm, by surprise (perceived predation. Understanding the narrow range in which financial predators operate is setting the grounds to offer better protection to investors and to implementing better control and punitive measures.

  9. Teaching Astronomy in Extracurricular Study Groups of Armenia

    Science.gov (United States)

    Khachatryan, Mher; Grigoryan, Avetik

    2016-12-01

    The report presents the history of activity of Extracurricular Study Groups of Ar¬menia teaching astronomy and related subjects. It mainly refers to the Aerospace Club founded in 1988, which has long been acting as an officially unre¬gis¬tered, but efficiently performing non-governmental organization - Armenian Youth Ae¬ro¬space Society. The Club teaches, provides a truly scientific view of the world, advocates astronomy and other scientific and technical areas, provides interesting lectures and ar¬ticles to schools and mass media, arranges seminars and meetings with renowned experts, publishes scientific ar¬ticles, manuals, books, puts forward important scientific and techno-logical problems and offer students to work together on them, seek for solutions and develop possible appli¬ca¬tions. All this is aimed at maintaining and further development of leading positions of Armenia's scientific potential, particularly in astronomy.

  10. Group Counseling for African American Elementary Students: An Exploratory Study

    Science.gov (United States)

    Steen, Sam

    2009-01-01

    This article describes a group counseling intervention promoting academic achievement and ethnic identity development for twenty fifth grade African American elementary students. The Multigroup Ethnic Identity Measure (MEIM) scores of students participating in the treatment group improved significantly over those in the control group. Implications…

  11. Piezoelectricity in quasicrystals: A group-theoretical study

    Indian Academy of Sciences (India)

    By group representation theory, the maximum number of non-vanishing and independent second- order piezoelectric coefficients required by the seven pentagonal and two icosahedral point groups - that describe the quasicrystal symmetry groups in two and three dimensions - is determined. The schemes of non-vanishing ...

  12. Multicultural Influences on Group Learning: A Qualitative Higher Education Study

    Science.gov (United States)

    Sweeney, Arthur; Weaven, Scott; Herington, Carmel

    2008-01-01

    Although the literature examining the usefulness of group projects is extensive, the link between cooperative learning, group performance and skills transfer in multicultural contexts remains unclear. Focus groups were conducted with a sample of 107 international and domestic postgraduate and undergraduate marketing students to investigate this…

  13. Gender-based education during clerkships: a focus group study

    Directory of Open Access Journals (Sweden)

    van Leerdam L

    2014-02-01

    Full Text Available Lotte van Leerdam, Lianne Rietveld, Doreth Teunissen, Antoine Lagro-JanssenDepartment of Primary and Community Care, Gender and Women's Health, Radboud University Medical Center, Nijmegen, The NetherlandsObjectives: One of the goals of the medical master's degree is for a student to become a gender-sensitive doctor by applying knowledge of gender differences in practice. This study aims to investigate, from the students’ perspective, whether gender medicine has been taught in daily practice during clerkship.Methods: A focus group study was conducted among 29 medical students from Radboud University, Nijmegen, The Netherlands, who had just finished either their internal medicine or surgical clerkships. Data were analyzed in line with the principles of constant comparative analysis.Results: Four focus groups were conducted with 29 participating students. Clinical teachers barely discuss gender differences during students’ clerkships. The students mentioned three main explanatory themes: insufficient knowledge; unawareness; and minor impact. As a result, students feel that they have insufficient competencies to become gender-sensitive doctors.Conclusion: Medical students at our institution perceive that they have received limited exposure to gender-based education after completing two key clinical clerkships. All students feel that they have insufficient knowledge to become gender-sensitive doctors. They suppose that their clinical teachers have insufficient knowledge regarding gender sensitivity, are unaware of gender differences, and the students had the impression that gender is not regarded as an important issue. We suggest that the medical faculty should encourage clinical teachers to improve their knowledge and awareness of gender issues.Keywords: medical education, clerkship, gender, hidden curriculum, clinical teachers

  14. Music during after-death care: a focus group study.

    Science.gov (United States)

    Holm, Marianne S; Fålun, Nina; Gjengedal, Eva; Norekvål, Tone M

    2012-01-01

    The intensive care unit (ICU) is not only a place to recover from injuries incurred during accidents and from serious illness. For many patients, it is also a place where they might die. Nursing care does not stop when a patient dies; rather, it continues with the care of the deceased and with family support. The aims of this study were (1) to explore the experiences and attitudes of nurses towards the use of ambient music in the ICU during after-death care and (2) to describe the feedback nurses received from relatives when music was used during the viewing. A qualitative design employing focus group interviews was used. Three focus group interviews with 15 nurses were conducted. All the interviews were audiotaped, transcribed verbatim and analysed using qualitative content analysis. Six main categories of attitudes emerged from the analysis: (1) different attitudes among nurses towards the use of music; (2) music affects the atmosphere; (3) music affects emotions; (4) use of music was situational; (5) special choice of music and (6) positive feedback from the bereaved. This study demonstrates that music might be helpful for nurses during after-death care as well as for the care of the relatives. Including ambient music in an after-death care programme can help nurses show respect for the deceased as the body is being prepared. Music played during the viewing may be a way of helping relatives in their time of grieving. It may ease the situation by making that event special and memorable. However, standardizing this intervention does not seem appropriate. Rather, the individual nurse and the family must decide whether music is to be used in a particular situation. © 2012 The Authors. Nursing in Critical Care © 2012 British Association of Critical Care Nurses.

  15. Gout in immigrant groups: a cohort study in Sweden.

    Science.gov (United States)

    Wändell, Per; Carlsson, Axel C; Li, Xinjun; Gasevic, Danijela; Ärnlöv, Johan; Holzmann, Martin J; Sundquist, Jan; Sundquist, Kristina

    2017-05-01

    Our aim was to study the association between country of birth and incidence of gout in different immigrant groups in Sweden. The study population included the whole population of Sweden. Gout was defined as having at least one registered diagnosis in the National Patient Register. The association between incidence of gout and country of birth was assessed by Cox regression, with hazard ratios (HRs) and 95% confidence intervals (95% CI), using Swedish-born individuals as referents. All models were conducted in both men and women, and the full model was adjusted for age, place of residence in Sweden, educational level, marital status, neighbourhood socio-economic status and co-morbidities. The risk of gout varied by country of origin, with highest estimates, compared to Swedish born, in fully adjusted models among men from Iraq (HR 1.82, 95% CI 1.54-2.16), and Russia (HR 1.69, 95% CI 1.26-2.27), and also high among men from Austria, Poland, Africa and Asian countries outside the Middle East; and among women from Africa (HR 2.23, 95% CI 1.50-3.31), Hungary (HR 1.98, 95% CI 1.45-2.71), Iraq (HR 1.76, 95% CI 1.13-2.74) and Austria (HR 1.70, 95% CI 1.07-2.70), and also high among women from Poland. The risk of gout was lower among men from Greece, Spain, Nordic countries (except Finland) and Latin America and among women from Southern Europe, compared to their Swedish counterparts. The increased risk of gout among several immigrant groups is likely explained by a high cardio-metabolic risk factor pattern needing attention.

  16. Phospholipid Syndrome and Vasculitis as a presentation of Systemic Lupus Erythematosus. Case report.

    Directory of Open Access Journals (Sweden)

    Sila Castellón Mortera

    2013-09-01

    Full Text Available The systemic Lupus Erythematosus is presented, generally, as a poli articular syndrome, with a long period of fever nephritico or nephrotico; other clinical ways are: neuropsychiatry, vasculitis, etc. They appeared in a progressive manner; but in rare cases as a sickness debutant. It has not being reported in Sancti Spiritus Province patients in which matches the debut of the systemic Lupus Erythematosus with the manifestations of phospholipid syndrome. A Woman with 24 years of age is hospitalized having vasculitis, articular pains, thrombose in her right foot, detecting anticoagulante lupico and possitive Rematoideo factor with periferic pattern diffused in the Inmunoelectroforesis. 5 years later was hospitalized again with poliserositis. She had a positive evolution with a dose in a month of Intacglobin and anticoagulante treatment. Two years later she was hospitalized with articular pains proving she had livedo reticular on her left knee and Raynaud phenomenon on her foot. Beta Prebeta Index and high triglycerides. Lupico anticoagulant positive again. A treatment with Intacglobin and Prednisona was given to the patient with a better clinic without being hospitalized again. There is no evidence (at 17 years of age of a sickness debut of renal dissorder. It is about a Systemic Lupus Eritematoso which debut was a vasculitis and a Phospholipid Syndrome associated.

  17. Hypocomplementemic urticarial vasculitis arising in a patient with immunoglobulin G4-related disease.

    Science.gov (United States)

    Takao, Mariko; Hamada, Toshihisa; Kaji, Tatsuya; Ikeda-Mizuno, Kazuko; Takehara-Yasuhara, Chinatsu; Ichimura, Kouichi; Yanai, Hiroyuki; Yshino, Tadashi; Iwatsuki, Keiji

    2016-04-01

    Hypocomplementemic urticarial vasculitis (HUV) has been defined as a syndrome associated with urticarial lesions caused by leukocytoclastic vasculitis. It has been observed in patients with systemic lupus erythematosus and related conditions. Immunoglobulin G4 (IgG4)-related disease is a lymphoproliferative disorder characterized by sclerosing pancreatitis, retroperitoneal fibrosis, sclerotic cholangitis, acute interstitial nephritis, and Mikulicz's disease, and associated with elevated levels of IgG4 and hypocomplementemia. Various cutaneous lesions may occur in IgG4-related disease. We report a 74-year-old woman with HUV, followed by systemic organ involvement suggestive of IgG4-related disease. Symptoms included submandibular gland swelling, lymphadenopathy, interstitial nephritis, and a thyroid nodule. Laboratory examinations revealed an elevated serum IgG4 level of 637 mg/dl in a total IgG level of 2176 mg/dl, and the production of autoantibodies such as rheumatoid factor and antiphospholipid antibodies. In the swollen lymph node, neither clonal proliferation of T or B cells nor Epstein-Barr virus infection was detected, although the structure had been destroyed by the infiltration of lymphocytes and plasma cells. Among total IgG-producing cells, approximately 40% were positive for IgG4. All symptoms and abnormal laboratory findings improved in response to oral prednisolone. Hypocomplementemic urticarial vasculitis may represent a clinical symptom of IgG4-related disease. © 2015 The International Society of Dermatology.

  18. Management of ANCA-associated vasculitis: Current trends and future prospects

    Directory of Open Access Journals (Sweden)

    Sally Hamour

    2010-06-01

    Full Text Available Sally Hamour, Alan D Salama, Charles D PuseyImperial College Kidney and Transplant Institute, Imperial College, London, UKAbstract: The antineutrophil cytoplasm antibody (ANCA-associated vasculitides are a spectrum of heterogeneous autoimmune diseases characterized by necrotizing small vessel vasculitis and the presence of ANCA. These chronic multisystem disorders may be life-threatening if there is major organ involvement, such as acute renal failure or pulmonary hemorrhage, and require significant initial immunosuppression and long-term maintenance treatment. Long-established protocols using cyclophosphamide and prednisolone have resulted in dramatically improved outcomes for patients since the 1970s. Subsequently, international collaboration has contributed to a growing evidence base and consensus in the management of these rare disorders. Modifications to traditional treatment protocols by the use of azathioprine or methotrexate rather than cyclophosphamide, and the introduction of newer agents, such as rituximab, has maintained outcomes whilst decreasing toxicity. However, the treatment limitations of incomplete efficacy, infection, and cumulative toxicity persist. These issues have continued to drive the search for safer and more effective modulation of the immune system using targeted immunotherapy. This review will explore the current evidence base for management of ANCA-associated vasculitis and future treatment prospects.Keywords: ANCA, vasculitis, treatment

  19. National logistics working groups: A landscape analysis study.

    Science.gov (United States)

    Leab, Dorothy; Schreiber, Benjamin; Kasonde, Musonda; Bessat, Olivia; Bui, Son; Loisel, Carine

    2017-04-19

    Several countries have acknowledged the contributions made by national logistics working groups (NLWG) to ensure equitable access to the expanded program on immunization's (EPI) vaccines against preventable diseases. In order to provide key insights to the United Nations Children's Fund (UNICEF) and the World Health Organization (WHO) supply chain hub - as well as other players, including national EPI - a landscape analysis study was conducted from September 2015 to February 2016. This is a cross-sectional survey taken by 43 countries that combines qualitative and quantitative approaches. Data was collected through a desk review, consultation, interviews, and distance questioning. References and guidance were used to determine and specify the underlying mechanisms of NLWGs. The key findings are:This study has provided a general overview of the status of NLWGs for immunization in various countries. Based on the key insights of the study, technical assistance needs have been identified, and immunization partners will be required to help countries create and reinforce their NLWGs. Copyright © 2017. Published by Elsevier Ltd.

  20. Karyometry in atypical endometrial hyperplasia: A Gynecologic Oncology Group study

    Science.gov (United States)

    Bartels, Peter H; Garcia, Francisco AR; Trimble, Cornelia L; Kauderer, James; Curtin, John; Lim, Peter C; Hess, Lisa M; Silverberg, Steven; Zaino, Richard J; Yozwiak, Michael; Bartels, Hubert G; Alberts, David S

    2014-01-01

    Objectives Treatment for atypical endometrial hyperplasia (AEH) is based on pathologic diagnosis. About 40% of AEH is found to be carcinoma at surgery. This study's objective is to derive an objective characterization of nuclei from cases diagnosed as AEH or superficially invasive endometrial cancer (SIEC). Methods Cases from GOG study 167A were classified by a central pathology committee as AEH (n=39) or SIEC (n=39). High resolution digitized images of cell nuclei were recorded. Features of the nuclear chromatin pattern were computed. Classification rules were derived by discriminant analysis. Results Nuclei from cases of AEH and SIEC occupy the same range on a progression curve for endometrial lesions. Cases of AEH and SIEC both comprise nuclei of two phenotypes: hyperplastic characteristics and premalignant/neoplastic characteristics. The principal difference between AEH and SIEC is percentage of premalignant/neoplastic nuclei. When this percentage approaches 50-60% superficial invasion is likely. SIEC may develop already from lesions at the low end of the progression curve. Conclusions AEH comprises cases which may constitute a low risk group involving 40 % of nuclei of preneoplastic phenotype. Nuclei of the preneoplastic phenotype in AEH lesions are almost indistinguishable from nuclei in SIEC, where this percentage exceeds 60%. The percentage of nuclei of the preneoplastic phenotype in AEH lesions might serve as criterion for assessment of risk for the development of invasive disease. PMID:22155796

  1. Occupational therapists' perceptions of gender - a focus group study.

    Science.gov (United States)

    Liedberg, Gunilla M; Björk, Mathilda; Hensing, Gunnel

    2010-10-01

    Women and men are shaped over the courses of their lives by culture, society and human interaction according to the gender system. Cultural influences on individuals' social roles and environment are described in occupational therapy literature, but not specifically from a gender perspective. The purpose of this qualitative study was to explore how a sample of occupational therapists perceives the 'gender' concept. Four focus group interviews with 17 occupational therapists were conducted. The opening question was: 'How do you reflect on the encounter with a client depending on whether it is a man or a woman?' The transcribed interviews were analysed and two main themes emerged: 'the concept of gender is tacit in occupational therapy' and 'client encounters'. The occupational therapists expressed limited theoretical knowledge of 'gender'. Furthermore, the occupational therapists seemed to be 'doing gender' in their encounters with the clients. For example, in their assessment of the client, they focussed their questions on different spheres: with female clients, on the household and family; with male clients, on their paid work. This study demonstrated that occupational therapists were unaware of the possibility that they were 'doing gender' in their encounters with clients. There is a need to increase occupational therapists' awareness of their own behaviour of 'doing gender'. Furthermore, there is a need to investigate whether gendered perceptions will shorten or lengthen a rehabilitation period and affect the chosen interventions, and in the end, the outcome for the clients. © 2010 The Authors. Australian Occupational Therapy Journal © 2010 Australian Association of Occupational Therapists.

  2. Italian Adolescents and Emergency Contraception: A Focus Group Study.

    Science.gov (United States)

    Olivari, Maria Giulia; Cuccì, Gaia; Confalonieri, Emanuela

    2017-02-01

    Using a qualitative method, the purpose of this study was to: (1) obtain information directly from the adolescents on their attitudes and knowledge regarding emergency contraception; and (2) investigate the presence of differences between male and female participants' attitudes and knowledge. This study consisted of 24 single-sex focus groups with 160 adolescents (male = 46.3% (74 of 160); female = 53.7% (86 of 160)) aged 15-19 years conducted among high schools in 3 regions of Italy. Data were analyzed through thematic analysis taking into account gender differences and 2 main themes emerged. The first was labeled "Adolescents' attitudes toward emergency contraception" and it was divided into 3 subthemes: You should be aware; It's a life line; and Everything but a child. The second theme was labeled "Adolescents' knowledge toward emergency contraception" and it was divided into 3 subthemes: False myths; Baseline information; and Just take it. Italian adolescents believed it is important to prevent the risk of unprotected sex by using contraceptive methods and their motivation to use emergency contraception is related to critical attitudes toward the consequences of irresponsible/ineffective contraception. Although adolescents have an awareness of emergency contraception, more comprehensive knowledge is needed. These findings can inform specific interventions aimed at educating adolescents in need of emergency contraception. Copyright © 2016 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  3. Henoch-Schönlein purpura in adults: a case series from a multidisciplinary study group Púrpura de Henoch-Schönlein em adultos: uma série de casos de um grupo de estudo multidisciplinar

    Directory of Open Access Journals (Sweden)

    Boris A. Cruz

    2006-12-01

    Full Text Available BACKGROUND: Henoch-Schönlein purpura (HSP is a systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA. It has been extensively studied in children, but in adults, its natural history is much less known. OBJECTIVES: to report a series of patients with HSP presenting in their adulthood. PATIENTS AND METHODS: the Minas Gerais Vasculitis Study Group´s Members (MGVSG were invited to report patients with HSP who appeared in their adulthood. A standardized retrospective chart review was done. RESULTS: eleven patients, two male and nine female, age 39.4 +/- 20.1 yearsold were studied. Nine patients presented purpura, seven presented arthritis, four patients had gastrointestinal involvement and ten patients (91% presented glomerulonephritis (GN. Eight patients were subjected to renal biopsies. Six of them presented endocapillary proliferative GN and only two of them had minimal mesangial proliferation. In the other three patients, HSP was confirmed by skin biopsies. All patients received steroids, in five of them steroids were combined with other immunosuppressive agents. After a follow-up of 39.0 +/- 64.6 months, four patients (36% presented impairment of renal function, but only one (9% developed end stage renal disease and was successfully appeared to renal transplantation. At the end of follow-up, seven patients (64% are in complete remission and four in partial remission. CONCLUSION: in adulthood, HSP represents a distinct clinical syndrome with a higher frequency of renal involvement and more severe systemic vasculitis. Nevertheless, the final outcome in this series was as good as reported in children, maybe due to aggressive immunosuppressive therapy.INTRODUÇÃO: púrpura de Henoch-Schönlein (PHS é uma vasculite sistêmica que acomete vasos de pequeno calibre com depósitos de imunocomplexos contendo IgA. Esta vasculite já foi extensamente estudada em crianças, mas sua história natural em adultos

  4. A novel immunopathological association of IgG4-RD and vasculitis with Hashimoto's thyroiditis.

    Science.gov (United States)

    Minamino, Hiroto; Inaba, Hidefumi; Ariyasu, Hiroyuki; Furuta, Hiroto; Nishi, Masahiro; Yoshimasu, Takashi; Nishikawa, Akinori; Nakanishi, Masanori; Tsuchihashi, Shigeki; Kojima, Fumiyoshi; Murata, Shin-Ichi; Inoue, Gen; Akamizu, Takashi

    2016-01-01

    A 73-year-old man with Hashimoto's thyroiditis (HT) suffered from purpura on the lower legs. He was diagnosed with IgG4-related disease (IgG4-RD) with serum IgG4 elevation and dacryo-sialadenitis confirmed histologically. Serum Th2 and Treg cytokines, interleukin 7 (IL7), IL8 and Th2 chemokine levels were elevated, while skewed Th1 balance was seen in fluorescence-activated cell sorting (FACS). Therefore, preferential Th1 balance in HT appeared to be followed by IgG4-RD characterized with Th2 and Treg polarization. The commencement of steroid therapy dramatically exacerbated clinical manifestations including IgG4-RD-associated HT. The measurement of cytokine and chemokine levels as well as FACS analysis in the development of IgG4-RD seemed to be beneficial. In conclusion, an innovative association of HT, IgG4-RD and vasculitis was observed. This report also offers novel diagnostic and therapeutic approaches for IgG4-RD. Recently, a subtype of HT has been considered to be a thyroid manifestation of IgG4-RD, although the etiology of IgG4-RD is not established yet.Immunologically a close association between HT and vasculitis was reported.Leukocytoclastic vasculitis is a rare skin presentation of IgG4-RD.In the current case, during the course of HT, IgG4-RD and leukocytoclastic vasculitis occurred; thus, innate immunity and acquired immunity seem to be involved in the development of IgG4-RD.The measurement of cytokine and chemokines appeared to be beneficial in the development of IgG4-RD.Remarkably, effectiveness of steroid therapy for HT suggested presence of IgG4-RD-associated HT. Therefore, this report highlights the pathogenesis of IgG4-RD and proposes novel therapeutic mechanisms. Clinicians should pay attention to the development of IgG4-RD and vasculitis during long course of HT.

  5. Collective Talent : A Study of Improvisational Group Performance in Music

    NARCIS (Netherlands)

    Jong, de Jacqueline B.

    2006-01-01

    Improvised music performance offers remarkable and dramatic examples of the talented ways in which group members can interact and inspire each other. Such musical sessions can serve as examples of improvised performance of groups in general. This thesis reports on ways of initiating and supporting

  6. A case study of a Postgraduate student's group expe

    African Journals Online (AJOL)

    students have to go through, often to collaborate for a presentation, a project or an assignment. The teaching and learning that happens within groups usually depends on the group dynamics that is .... attitudes, personality, hobbies, living conditions, and economic and family status. In the case of Shahid's undergraduate ...

  7. Technology User Groups and Early Childhood Education: A Preliminary Study

    Science.gov (United States)

    Parette, Howard P.; Hourcade, Jack J.; Blum, Craig; Watts, Emily H.; Stoner, Julia B.; Wojcik, Brian W.; Chrismore, Shannon B.

    2013-01-01

    This article presents a preliminary examination of the potential of Technology User Groups as a professional development venue for early childhood education professionals in developing operational and functional competence in using hardware and software components of a Technology toolkit. Technology user groups are composed of varying numbers of…

  8. Older Inmates’ Pursuit of Good Health: A Focus Group Study

    Science.gov (United States)

    Steffensmeier, Darrell

    2012-01-01

    A multitude of intersecting factors including the graying of the broader society, a paradigm shift away from rehabilitation, fewer opportunities for parole, and retrospective prosecutions contribute to an exponential increase in number of geriatric inmates. Elderly prisoners are likely to live in small tight quarters with other inmates, have two or more chronic health conditions, and encounter multiple barriers impeding health promotion while incarcerated. The purpose of this study was to identify perceived challenges to the health of older male inmates and to explore their self-care strategies. Focus group methodology was used. Data were collected from 42 male inmates age 50 and over who were aging in place and living with comorbidity. Cost issues, prison personnel and policies, food concerns, fellow inmates, and personal barriers all challenged older inmates’ abilities to maintain their health in prison. However, these older inmates engaged in a variety of self-care strategies, including: accessing resources and support; staying positive; managing diet and weight; engaging in physical activity; and protecting self. A key motivator for pursuing good health was to be respected and perceived as healthy and strong by fellow inmates. Looking to the future, development and testing of programs to enhance inmates' self-management of chronic conditions and to facilitate health promotion are in order. PMID:20795581

  9. Interpersonal processes in psychodynamic-interpersonal and cognitive behavioral group therapy: a systematic case study of two groups.

    Science.gov (United States)

    Tasca, Giorgio A; Foot, Meredith; Leite, Catherine; Maxwell, Hilary; Balfour, Louise; Bissada, Hany

    2011-09-01

    This mixed method systematic case study applied an interpersonal stage model of the therapeutic process to examine interpersonal processes among a highly adherent Group Psychodynamic-Interpersonal Psychotherapy (GPIP) therapist and a highly adherent Group Cognitive Behavioral Therapy (GCBT) therapist and their groups of binge eating disordered (BED) patients. This is the first case study to apply the interpersonal stage model of psychotherapy to compare GCBT and GPIP methods and the first to apply the model to group therapy. Early-, middle-, and late-stage transcribed video recordings of sequential interactions among therapists and patients in each of these two time-limited group therapies were analyzed with the Structural Analysis of Social Behavior (SASB). We also provide qualitative presentations of the transcripts from each stage as context for the quantitative analyses. BED patients in both groups achieved positive outcomes for binge eating and depression. Consistent with their treatment model, the GPIP therapist was more autonomy-giving, whereas the GCBT therapist was more controlling/directive. The GPIP therapist and her group had high levels of interpersonal complementary interaction sequences in the early stage followed by lower complementarity in the middle stage. The GCBT therapist and her group showed a high-low-high pattern of complementarity across the three stage of therapy. However, overall the GPIP group had higher levels complementarity than the GCBT group. This mixed method case study of group processes based on an interpersonal stage model of psychotherapy suggested specific therapist behaviors in each modality to maximize positive therapeutic interactions at each stage of group therapy. (c) 2011 APA, all rights reserved.

  10. Profiles in Successful Group Piano for Children: A Collective Case Study of Children's Group-Piano Lessons

    Science.gov (United States)

    Pike, Pamela D.

    2013-01-01

    The purpose of this collective case study was to explore the best practices in beginning group-piano instruction. Four beginning and intermediate groups of piano students (N =20) were observed. Data were triangulated through in-class observation of students and teachers, teacher interviews and student questionnaires. The master teachers…

  11. Situational Factors in Focus Group Studies: A Systematic Review

    National Research Council Canada - National Science Library

    Orvik, Arne; Larun, Lillebeth; Berland, Astrid; Ringsberg, Karin C

    2013-01-01

    .... By using Vicsek's framework of situational factors for analysis of focus group results as a starting point, we found that contextual factors were most frequently described in the method sections...

  12. Systemic vasculitis is associated with a higher risk of lower extremity amputation in patients with severe peripheral arterial occlusive disease: a secondary analysis of a nationwide, population-based health claims database.

    Science.gov (United States)

    Lu, Ming-Chi; Hsu, Honda; Lin, Ching-Hsing; Koo, Malcolm

    2017-11-01

    Previous research has shown that diabetes mellitus increases the risk of lower extremity amputation in patients with peripheral arterial occlusive disease. However, to our knowledge, no studies have investigated whether systemic autoimmune disease, in particular systemic vasculitis is associated with a higher risk of lower extremity amputation in these patients. To investigate the association between systemic autoimmune disease and lower extremity amputation in patients with severe peripheral arterial occlusive disease based on a secondary analysis of a nationwide, population-based health claims database. Using the inpatient datafile of the Taiwan's National Health Insurance Research Database (NHIRD), we identified 432 patients with severe peripheral arterial occlusive disease that required hospitalization between 2000 and 2012. We also identified patients who had undergone lower extremity amputation and their comorbidities using the same datafile. The risk of lower extremity amputation was assessed using multiple logistic regression analysis, adjusting for age, sex, insured amount, the urbanization level of residence, and the presence of comorbidities. Among patients with severe peripheral arterial occlusive disease, those with systemic vasculitis exhibited a significant higher risk of lower extremity amputation (adjusted odds ratio [aOR] = 6.82, p obstructive pulmonary disease (aOR = 2.87, p = 0.007) were also significantly associated with a higher risk of lower extremity amputation. Among patients with severe peripheral arterial occlusive disease, a significantly higher risk of lower extremity amputation was observed in those with systemic vasculitis.

  13. The Lung Cancer Surgical Study Group of the Japan Clinical Oncology Group: past activities, current status and future direction.

    Science.gov (United States)

    Nakagawa, Kazuo; Watanabe, Shun-Ichi; Kunitoh, Hideo; Asamura, Hisao

    2017-03-01

    The Lung Cancer Surgical Study Group of the Japan Clinical Oncology Group was organized in 1986. Initially, 26 collaborative institutions participated. In the early period, the Lung Cancer Surgical Study Group focused on combined modality therapies and conducted nine trials, including JCOG9101: adjuvant chemotherapy for resected small-cell lung cancer, and JCOG9806: induction chemoradiotherapy followed by surgery for superior sulcus tumor, which greatly impacted the treatment strategies for some special kinds of lung cancer. Since the 2000s, the Lung Cancer Surgical Study Group has defined radiologically noninvasive adenocarcinoma: JCOG0201 and investigated adequate modes of surgical resection for small-sized non-small cell lung cancer: JCOG0802, JCOG0804 and JCOG1211. The accrual of these trials is now complete and we are waiting for the maturation of follow-up data. In addition, two adjuvant trials have been conducted: JCOG0707; a Phase III study of adjuvant chemotherapy for resected pathological stage I (T1 > 2 cm) non-small cell lung cancer, and JCOG1205; a Phase III study of adjuvant chemotherapy for completely resected pulmonary high-grade neuroendocrine tumor. The accrual of JCOG0707 is complete and we are waiting for the maturation of follow-up data. At present, 44 institutions are active members of the Lung Cancer Surgical Study Group. In addition to thoracic surgeons, medical oncologists, pathologists and radiotherapists are participating in the Lung Cancer Surgical Study Group. The Lung Cancer Surgical Study Group continues to conduct various clinical trials in an effort to improve survival in patients with lung cancer. In this review, we provide an overview of the past 30 years, as well as the present status and future direction of the Lung Cancer Surgical Study Group. © The Author 2016. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  14. Multistate case-control study of maternal risk factors for neonatal group B streptococcal disease. The Active Surveillance Study Group.

    Science.gov (United States)

    Schuchat, A; Deaver-Robinson, K; Plikaytis, B D; Zangwill, K M; Mohle-Boetani, J; Wenger, J D

    1994-07-01

    Risk factors for early onset disease (EOD) caused by Group B streptococci (GBS) that are the foundation of prevention guidelines were identified in studies conducted in a few hospital centers. We investigated cases of EOD identified through laboratory-based active surveillance during 1991 and 1992 in a multistate population of 17 million. Ninety-nine cases were compared with 253 controls matched for hospital, date of birth and birth weight. Prematurity (cases; 53% of case mothers had rupture of membranes > 12 hours; and 48% reported intrapartum fever. The incidence of EOD in each surveillance area was higher among blacks. By multivariate analysis, case mothers were more likely than controls to have rupture of membranes before labor onset (adjusted odds ratio 8.7, P case mothers had intrapartum fever, < 37 weeks of gestation and/or prolonged rupture of membranes, indicators previously used to select high risk women for intrapartum chemoprophylaxis. Our findings extend data from single hospitals and suggest prenatal screening and selective intrapartum chemoprophylaxis of high-risk mothers could potentially prevent the majority of EOD in the United States.

  15. A comparative study of prelinguistic vocalizations in two groups of cleft toddlers and a non-cleft group

    DEFF Research Database (Denmark)

    Willadsen, Elisabeth; Enemark, Hans

    2000-01-01

    children in the comparison group. Both groups of subjects with clefts had significantly fewer plosives in their contoid inventory than the noncleft group, and there was no difference regarding place of articulation between the group that received delayed closure of the hard palate and the noncleft group.......Objective: This study examined the prelinguistic contoid (consonant-like) inventories of 14 children with unilateral cleft lip and palate (C-UCLP) at 13 months of age. The children had received primary veloplasty at 7 months of age and closure of the hard palate was performed at 3–5 years....... The results of this investigation were compared to results previously reported for 19 children with cleft palate and 19 noncleft children at the age of 13 months. The children with clefts in that study received a two-stage palatal surgery. This surgical procedure was formerly used at our center and included...

  16. Employee perceptions of diabetes education needs: a focus group study.

    Science.gov (United States)

    Wood, Felecia; Jacobson, Sharol

    2005-10-01

    The purpose of this descriptive non-experimental research was to assess employee perceptions of desired diabetes education through focus groups. Thirteen employees of a southern university in three focus groups identified many standard and some emerging educational topics of interest including healthier food choices when eating out, increasing activity, deciding whether highly advertised "special" products for diabetes were necessary, and recognizing the importance of obesity among children. The employees were willing to attend group meetings related to diabetes, but not to pay for them. The information will be used to plan an intervention to promote diabetes prevention and self-management in a worksite environment where diabetes costs are rising rapidly, and to support the need for a university wellness program. Occupational health nurses can address both employees' needs and employers' costs through such a program.

  17. What about N? A methodological study of sample-size reporting in focus group studies

    Science.gov (United States)

    2011-01-01

    Background Focus group studies are increasingly published in health related journals, but we know little about how researchers use this method, particularly how they determine the number of focus groups to conduct. The methodological literature commonly advises researchers to follow principles of data saturation, although practical advise on how to do this is lacking. Our objectives were firstly, to describe the current status of sample size in focus group studies reported in health journals. Secondly, to assess whether and how researchers explain the number of focus groups they carry out. Methods We searched PubMed for studies that had used focus groups and that had been published in open access journals during 2008, and extracted data on the number of focus groups and on any explanation authors gave for this number. We also did a qualitative assessment of the papers with regard to how number of groups was explained and discussed. Results We identified 220 papers published in 117 journals. In these papers insufficient reporting of sample sizes was common. The number of focus groups conducted varied greatly (mean 8.4, median 5, range 1 to 96). Thirty seven (17%) studies attempted to explain the number of groups. Six studies referred to rules of thumb in the literature, three stated that they were unable to organize more groups for practical reasons, while 28 studies stated that they had reached a point of saturation. Among those stating that they had reached a point of saturation, several appeared not to have followed principles from grounded theory where data collection and analysis is an iterative process until saturation is reached. Studies with high numbers of focus groups did not offer explanations for number of groups. Too much data as a study weakness was not an issue discussed in any of the reviewed papers. Conclusions Based on these findings we suggest that journals adopt more stringent requirements for focus group method reporting. The often poor and

  18. What about N? A methodological study of sample-size reporting in focus group studies.

    Science.gov (United States)

    Carlsen, Benedicte; Glenton, Claire

    2011-03-11

    Focus group studies are increasingly published in health related journals, but we know little about how researchers use this method, particularly how they determine the number of focus groups to conduct. The methodological literature commonly advises researchers to follow principles of data saturation, although practical advise on how to do this is lacking. Our objectives were firstly, to describe the current status of sample size in focus group studies reported in health journals. Secondly, to assess whether and how researchers explain the number of focus groups they carry out. We searched PubMed for studies that had used focus groups and that had been published in open access journals during 2008, and extracted data on the number of focus groups and on any explanation authors gave for this number. We also did a qualitative assessment of the papers with regard to how number of groups was explained and discussed. We identified 220 papers published in 117 journals. In these papers insufficient reporting of sample sizes was common. The number of focus groups conducted varied greatly (mean 8.4, median 5, range 1 to 96). Thirty seven (17%) studies attempted to explain the number of groups. Six studies referred to rules of thumb in the literature, three stated that they were unable to organize more groups for practical reasons, while 28 studies stated that they had reached a point of saturation. Among those stating that they had reached a point of saturation, several appeared not to have followed principles from grounded theory where data collection and analysis is an iterative process until saturation is reached. Studies with high numbers of focus groups did not offer explanations for number of groups. Too much data as a study weakness was not an issue discussed in any of the reviewed papers. Based on these findings we suggest that journals adopt more stringent requirements for focus group method reporting. The often poor and inconsistent reporting seen in these

  19. What about N? A methodological study of sample-size reporting in focus group studies

    Directory of Open Access Journals (Sweden)

    Glenton Claire

    2011-03-01

    Full Text Available Abstract Background Focus group studies are increasingly published in health related journals, but we know little about how researchers use this method, particularly how they determine the number of focus groups to conduct. The methodological literature commonly advises researchers to follow principles of data saturation, although practical advise on how to do this is lacking. Our objectives were firstly, to describe the current status of sample size in focus group studies reported in health journals. Secondly, to assess whether and how researchers explain the number of focus groups they carry out. Methods We searched PubMed for studies that had used focus groups and that had been published in open access journals during 2008, and extracted data on the number of focus groups and on any explanation authors gave for this number. We also did a qualitative assessment of the papers with regard to how number of groups was explained and discussed. Results We identified 220 papers published in 117 journals. In these papers insufficient reporting of sample sizes was common. The number of focus groups conducted varied greatly (mean 8.4, median 5, range 1 to 96. Thirty seven (17% studies attempted to explain the number of groups. Six studies referred to rules of thumb in the literature, three stated that they were unable to organize more groups for practical reasons, while 28 studies stated that they had reached a point of saturation. Among those stating that they had reached a point of saturation, several appeared not to have followed principles from grounded theory where data collection and analysis is an iterative process until saturation is reached. Studies with high numbers of focus groups did not offer explanations for number of groups. Too much data as a study weakness was not an issue discussed in any of the reviewed papers. Conclusions Based on these findings we suggest that journals adopt more stringent requirements for focus group method

  20. Functional renormalization group study of fluctuation effects in fermionic superfluids

    Energy Technology Data Exchange (ETDEWEB)

    Eberlein, Andreas

    2013-03-22

    This thesis is concerned with ground state properties of two-dimensional fermionic superfluids. In such systems, fluctuation effects are particularly strong and lead for example to a renormalization of the order parameter and to infrared singularities. In the first part of this thesis, the fermionic two-particle vertex is analysed and the fermionic renormalization group is used to derive flow equations for a decomposition of the vertex in charge, magnetic and pairing channels. In the second part, the channel-decomposition scheme is applied to various model systems. In the superfluid state, the fermionic two-particle vertex develops rich and singular dependences on momentum and frequency. After simplifying its structure by exploiting symmetries, a parametrization of the vertex in terms of boson-exchange interactions in the particle-hole and particle-particle channels is formulated, which provides an efficient description of the singular momentum and frequency dependences. Based on this decomposition of the vertex, flow equations for the effective interactions are derived on one- and two-loop level, extending existing channel-decomposition schemes to (i) the description of symmetry breaking in the Cooper channel and (ii) the inclusion of those two-loop renormalization contributions to the vertex that are neglected in the Katanin scheme. In the second part, the superfluid ground state of various model systems is studied using the channel-decomposition scheme for the vertex and the flow equations. A reduced model with interactions in the pairing and forward scattering channels is solved exactly, yielding insights into the singularity structure of the vertex. For the attractive Hubbard model at weak coupling, the momentum and frequency dependence of the two-particle vertex and the frequency dependence of the self-energy are determined on one- and two-loop level. Results for the suppression of the superfluid gap by fluctuations are in good agreement with the literature

  1. Multilevel Mediation Modeling in Group-Based Intervention Studies.

    Science.gov (United States)

    Krull, Jennifer L.; MacKinnon, David P.

    1999-01-01

    Proposes and evaluates a method to test for mediation in multilevel data sets formed when an intervention administered to groups is designed to produce change in individual mediator and outcome variables. Applies the method to the ATLAS intervention designed to decrease steroid use among high school football players. (SLD)

  2. Enhancing Student Engagement: A Group Case Study Approach

    Science.gov (United States)

    Taneja, Aakash

    2014-01-01

    Computing professionals work in groups and collaborate with individuals having diverse backgrounds and behaviors. The Accreditation Board for Engineering and Technology (ABET) characterizes that a computing program must enable students to attain the ability to analyze a problem, design and evaluate a solution, and work effectively on teams to…

  3. Experimentally-induced anti-myeloperoxidase vasculitis does not require properdin, MASP-2 or bone marrow-derived C5.

    Science.gov (United States)

    Freeley, Simon J; Popat, Reena J; Parmar, Kiran; Kolev, Martin; Hunt, Beverley J; Stover, Cordula M; Schwaeble, Willhelm; Kemper, Claudia; Robson, Michael G

    2016-09-01

    Anti-neutrophil cytoplasmic antibody vasculitis is a systemic autoimmune disease with glomerulonephritis and pulmonary haemorrhage as major clinical manifestations. The name reflects the presence of autoantibodies to myeloperoxidase and proteinase-3, which bind to both neutrophils and monocytes. Evidence of the pathogenicity of these autoantibodies is provided by the observation that injection of anti-myeloperoxidase antibodies into mice causes a pauci-immune focal segmental necrotizing glomerulonephritis which is histologically similar to the changes seen on renal biopsy in patients. Previous studies in this model have implicated the alternative pathway of complement activation and the anaphylatoxin C5a. Despite this progress, the factors that initiate complement activation have not been defined. In addition, the relative importance of bone marrow-derived and circulating C5 is not known. This is of interest given the recently identified roles for complement within leukocytes. We induced anti-myeloperoxidase vasculitis in mice and confirmed a role for complement activation by demonstrating protection in C3-deficient mice. We showed that neither MASP-2- nor properdin-deficient mice were protected, suggesting that alternative pathway activation does not require properdin or the lectin pathway. We induced disease in bone marrow chimaeric mice and found that circulating and not bone marrow-derived C5 was required for disease. We have therefore excluded properdin and the lectin pathway as initiators of complement activation and this means that future work should be directed at other potential factors within diseased tissue. In addition, in view of our finding that circulating and not bone marrow-derived C5 mediates disease, therapies that decrease hepatic C5 secretion may be considered as an alternative to those that target C5 and C5a. © 2016 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and

  4. Group Schema Therapy for Eating Disorders: A Pilot Study

    OpenAIRE

    Simpson, Susan G.; Emma Morrow; Michiel v Vreeswijk; Caroline Reid

    2010-01-01

    This paper describes the use of Group Schema Therapy for Eating Disorders (ST-E-g) in a case-series of eight participants with chronic eating disorders and high levels of co-morbidity. Treatment was comprised of 20 sessions which included cognitive, experiential and interpersonal strategies, with an emphasis on behavioural change. Specific schema-based strategies focused on bodily felt-sense and body-image, as well as emotional regulation skills. Six attended until end of treatment, two dro...

  5. Group Schema Therapy for Eating Disorders: A Pilot Study

    OpenAIRE

    Simpson, Susan G.; Morrow, Emma; van Vreeswijk, Michiel; Reid, Caroline

    2010-01-01

    This paper describes the use of Group Schema Therapy for Eating Disorders (ST-E-g) in a case series of eight participants with chronic eating disorders and high levels of co-morbidity. Treatment was comprised of 20 sessions which included cognitive, experiential, and interpersonal strategies, with an emphasis on behavioral change. Specific schema-based strategies focused on bodily felt-sense and body-image, as well as emotional regulation skills. Six attended until end of treatment, two dropp...

  6. Group Schema Therapy for Eating Disorders: A Pilot Study

    Directory of Open Access Journals (Sweden)

    Susan G Simpson

    2010-11-01

    Full Text Available This paper describes the use of Group Schema Therapy for Eating Disorders (ST-E-g in a case-series of eight participants with chronic eating disorders and high levels of co-morbidity. Treatment was comprised of 20 sessions which included cognitive, experiential and interpersonal strategies, with an emphasis on behavioural change. Specific schema-based strategies focused on bodily felt-sense and body-image, as well as emotional regulation skills. Six attended until end of treatment, two dropped-out at mid-treatment. Eating disorder severity, global schema severity, shame and anxiety levels were reduced between pre- and post therapy, with a large effect size at follow-up. Clinically significant improvement in eating severity was found in four out of six completers. Group completers showed a mean reduction in schema severity of 43% at post-treatment, and 59% at follow-up. By follow-up, all completers had achieved over 60% improvement in schema severity. Self-report feedback suggests that group factors may catalyze the change process in schema therapy by increasing perceptions of support and encouragement to take risks and try out new behaviours, whilst providing a de-stigmatising and de-shaming therapeutic experience.

  7. Group schema therapy for eating disorders: a pilot study.

    Science.gov (United States)

    Simpson, Susan G; Morrow, Emma; van Vreeswijk, Michiel; Reid, Caroline

    2010-01-01

    This paper describes the use of Group Schema Therapy for Eating Disorders (ST-E-g) in a case series of eight participants with chronic eating disorders and high levels of co-morbidity. Treatment was comprised of 20 sessions which included cognitive, experiential, and interpersonal strategies, with an emphasis on behavioral change. Specific schema-based strategies focused on bodily felt-sense and body-image, as well as emotional regulation skills. Six attended until end of treatment, two dropped-out at mid-treatment. Eating disorder severity, global schema severity, shame, and anxiety levels were reduced between pre- and post-therapy, with a large effect size at follow-up. Clinically significant improvement in eating severity was found in four out of six completers. Group completers showed a mean reduction in schema severity of 43% at post-treatment, and 59% at follow-up. By follow-up, all completers had achieved over 60% improvement in schema severity. Self-report feedback suggests that group factors may catalyze the change process in schema therapy by increasing perceptions of support and encouragement to take risks and try out new behaviors, whilst providing a de-stigmatizing and de-shaming therapeutic experience.

  8. Vancomycin-Induced Leukocytoclastic Vasculitis and Acute Renal Failure Due to Tubulointerstitial Nephritis.

    Science.gov (United States)

    Pingili, Chandra Shekar; Okon, Emmanuel E

    2017-09-25

    BACKGROUND Methicillin-resistant Staphylococcus aureus (MRSA) bacteremia and sepsis are commonly treated with intravenous vancomycin. However, vancomycin treatment is associated adverse reactions, including skin rashes and nephrotoxicity. We present a case of acute renal failure due to acute tubulointerstitial nephritis associated with a diffuse leukocytoclastic vasculitic skin eruption following intravenous vancomycin treatment. CASE REPORT A 79-year-old Caucasian male patient was treated with intravenous vancomycin for MRSA bacteremia. Prior to treatment, his creatinine was normal at 0.6 mg/dl. He presented one week later with shortness of breath, lower limb edema, and acute renal failure. He had a diffuse maculopapular rash involving the trunk and both upper and lower extremities. A renal biopsy and left arm skin biopsy were examined histologically. The skin biopsy showed leukocytoclastic vasculitis. Renal biopsy showed some sclerosed glomeruli, some with mesangial proliferation, and tubulointerstitial inflammation with eosinophils and plasma cells and mild interstitial fibrosis. Although there was some renal arteriolosclerosis, no vasculitic changes were seen, and no vascular thrombosis was present. A diagnosis of leukocytoclastic vasculitis and acute tubulointerstitial nephritis secondary to intravenous vancomycin therapy was made. CONCLUSIONS Although skin reactions associated with drug therapy are common, vancomycin-associated dermal vasculitis is rare. Tubulointerstitial nephritis is also a rare association with vancomycin treatment. This case report has highlighted that patients being treated with intravenous vancomycin should be carefully observed for acute skin rashes and deterioration in renal function, which can be managed by ceasing treatment with vancomycin, steroid challenge, and preventing future exposure to similar antimicrobial agents.

  9. Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis.

    Science.gov (United States)

    Veronese, F V; Dode, R S O; Friderichs, M; Thomé, G G; da Silva, D R; Schaefer, P G; Sebben, V C; Nicolella, A R; Barros, E J G

    2016-01-01

    Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs) and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs. His laboratory tests on admission showed serum creatinine of 4.56 mg/dL, white blood count 3,800/μL, hemoglobin 7.3 g/dL, urinalysis with 51 white blood cells/μL and 960 red blood cells/μL, and urine protein-to-creatinine ratio 1.20. Serum ANCA testing was positive (>1:320), as well as serum anti-myeloperoxidase and anti-proteinase 3 antibodies. Urine toxicology screen was positive for cocaine and levamisole, with 62.8% of cocaine, 32.2% of levamisole, and 5% of an unidentified substance. Skin and renal biopsies were diagnostic for leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis, respectively. The patient showed a good clinical response to cocaine abstinence, and use of corticosteroids and intravenous cyclophosphamide. Last serum creatinine was 1.97 mg/dL, white blood cell count 7,420/μL, and hemoglobin level 10.8 g/dL. In levamisole-induced systemic vasculitis, the early institution of cocaine abstinence, concomitant with the use of immunosuppressive drugs in severe cases, may prevent permanent end organ damage and associate with better clinical outcomes.

  10. Traumatic endophthalmitis presenting as isolated retinal vasculitis and white-centered hemorrhages: Case report and review of literature

    Directory of Open Access Journals (Sweden)

    Nidhi Relhan

    2012-01-01

    Full Text Available The article reports a case and review of the literature of endophthalmitis presenting as isolated retinal vasculitis. A 26-year-old male was observed to have white-centered retinal hemorrhages and retinal vasculitis following an occult scleral perforation. At presentation, the visual acuity was 20/60. With clinical suspicion of early endophthalmitis, he underwent wound exploration, scleral tear repair, vitreous biopsy and administration of intravitreal antibiotics. Microbiology evaluation revealed significant presence of methicillin-resistant coagulase-negative Staphylococcus epidermidis. Final visual acuity improved to 20/20 at 6 weeks postoperatively. Literature search revealed eight similar cases, all of them due to Staphylococcus species. Retinal vasculitis and white-centered retinal hemorrhages can be a presenting sign of early endophthalmitis, especially with non-fulminant pathogens like S. epidermidis.

  11. Hypocomplementemic urticarial vasculitis with Jaccoud's arthropathy and valvular heart disease: case report and review of the literature.

    Science.gov (United States)

    Amano, H; Furuhata, N; Tamura, N; Tokano, Y; Takasaki, Y

    2008-09-01

    We describe a female Japanese patient with concomitant hypocomplementemic urticarial vasculitis, Jaccoud's arthropathy and valvular heart disease. In 1996, she developed arthritis with swelling of both proximal interphalangeal joints and urticarial vasculitis on both arms that was resolved by administration of glucocorticoid (prednisolone 30 mg/day). Tests for antineutrophil cytoplasmic antibodies, antinuclear antibody and rheumatoid factor gave negative results. The findings of a skin biopsy examination were consistent with 'leukocytoclastic vasculitis'. During 10 years of observation, the patient manifested polyarthritis leading to progressive deformity of the joints of the hands and feet (without loss of cartilage or erosion of bone), persistent urticaria exacerbated by cold and accompanied by hypocomplementemia and progressive cardiac valvular disease with mitral valve regurgitation. There are only three reports described previously documenting five patients with this rare combination of manifestations.

  12. Mesenteric vasculitis in adults with Henoch-Schonlein purpura: a not-so-benign condition.

    LENUS (Irish Health Repository)

    Sibartie, V

    2009-02-07

    INTRODUCTION: The gastrointestinal manifestations of Henoch-Schonlein purpura (HSP) are well characterised, but their recognition can be difficult when they occur in isolation. Furthermore, HSP can run a more serious course in adults, compared to children, in whom the disease usually occurs. MATERIALS AND METHODS: We describe two cases that illustrate the challenges of HSP with mesenteric vasculitis and the outcome in adults. CONCLUSION: Although self-limiting in most patients, the outcome of HSP in adults can be far from benign and even fatal.

  13. Spontaneous abdominal hemorrhage with AA-amyloidosis and vasculitis in a patient with rheumatoid arthritis.

    Science.gov (United States)

    Jayawardene, S A; Sheerin, N; Pattison, J M; Hartley, B; Goldsmith, D J

    2001-04-01

    Both rheumatoid vasculitis and amyloidosis in rheumatoid arthritis (RA) are uncommon. We describe a patient in whom they occurred together and were associated with fatal intra-abdominal hemorrhage. A 56-year-old Caucasian woman was referred because of increasing lethargy, edema, and proteinuria. She had suffered from seropositive, erosive, nodular RA for 14 years. Previously, she had undergone numerous joint replacements, a thyroidectomy for amyloid-associated (AA) amyloidosis of the thyroid that caused a large goiter and a renal biopsy that showed renal AA-amyloidosis in the context of nephrotic syndrome. As her condition deteriorated, this patient became increasingly reluctant to go to the hospital and to take drugs beyond analgesics. Thus, her RA was chronically under treated. While in the hospital for evaluation, this patient suddenly developed hypotension, tachycardia, and a severe colicky left-sided abdominal pain radiating from the left upper quadrant/epigastric region to the left iliac fossa. Computed tomography (CT) showed a large amount of echogenic free fluid within the abdomen and marked thickening of the omentum. At laparotomy, 2 liters of free blood was found adjacent to a hematoma of the greater omentum, and it was evacuated without identification of a discrete bleeding point. All solid and hollow organs were normal. The omentum was noted to be very friable. She developed a more disseminated bleeding diathesis and persistent peritoneal hemorrhage via her abdominal drains. She succumbed shortly afterward. Histology revealed extensive omental hemorrhage and one large vessel within the area of hemorrhage showed a severe necrotizing vasculitis. Extensive amyloid deposition was also found within the walls of the smaller omental arterioles. Vasculitis in the context of RA is relatively rare and is associated with under treated, seropositive disease. Skin and nerve involvement are most common, but bowel involvement has been reported, with a highly

  14. Upper Respiratory Tract Symptoms, Renal Involvement and Vasculitis: A Case Report and Review of Wegener Granulomatosis

    Science.gov (United States)

    Said, Mohd Shahrir Mohamed

    2010-01-01

    Wegener’s Granulomatosis is a condition associated with systemic vasculitis which can present with upper respiratory tract symptoms initially. On September 2001, a 15-year-old girl presented with symptoms of nasal block for 3 weeks. She later developed joint pains and worsening renal status requiring dialysis. A renal biopsy was performed which showed pauci-immune cresentric glomerulonephritis. Her cANCA levels were positive. She was treated with oral cyclophosphamide and steroids and later responded. Keywords Wegener granulomatosis; Young girl; Cyclophophamide; cANCA PMID:21629538

  15. Recurrence of ANCA-associated vasculitis in a patient with kidney trasplant

    Directory of Open Access Journals (Sweden)

    Pedro García Cosmes

    2016-03-01

    Full Text Available Renal disease secondary to vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA can lead to chronic renal disease requiring renal replacement therapy. In these patients, kidney transplantation offers excellent long-term rates of allograft and patient survival; consequently, they can be trasplanted when the clinical disease activity has remitted. However, the risk of disease relapses in the renal allograft remains, although at lower rates due to modern immunosuppressive regimes. We describe the case of a male patient with extracapillary glomerulonephritis type III C-ANCA (+ who developed a recurrence in the renal allograft 8 years after transplantation. Intensive immunosupression with plasmapheresis controlled the disease.

  16. EULAR points to consider in the development of classification and diagnostic criteria in systemic vasculitis

    DEFF Research Database (Denmark)

    Basu, Neil; Watts, Richard; Bajema, Ingeborg

    2010-01-01

    The systemic vasculitides are multiorgan diseases where early diagnosis and treatment can significantly improve outcomes. Robust nomenclature reduces diagnostic delay. However, key aspects of current nomenclature are widely perceived to be out of date, these include disease definitions, classific......, classification and diagnostic criteria. Therefore, the aim of the present work was to identify deficiencies and provide contemporary points to consider for the development of future definitions and criteria in systemic vasculitis.......The systemic vasculitides are multiorgan diseases where early diagnosis and treatment can significantly improve outcomes. Robust nomenclature reduces diagnostic delay. However, key aspects of current nomenclature are widely perceived to be out of date, these include disease definitions...

  17. Hypocomplementemic urticarial vasculitis syndrome: a rare cause of basilar panacinar emphysema.

    Science.gov (United States)

    Pujara, Akshat C; Mohammed, Tan-Lucien H

    2012-05-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare condition characterized by an immune complex-mediated disruption of multiple organ systems. Pulmonary involvement is a major cause of morbidity and mortality in patients with HUVS. HUVS has been described by clinical, histological, and laboratory findings. However, the role of radiography in the diagnosis and management of HUVS has not been established. We examine computed tomography findings in a patient with HUVS and explore the potential of thoracic computed tomography imaging to augment the management of HUVS by serving as a diagnostic tool and marker of disease severity.

  18. Necrotizing Polyarteritis Nodosa-like Vasculitis in a Child with Systemic Lupus Erythematosus.

    Science.gov (United States)

    Nada, Ritambhra; Matthews, Joseph L; Bhattad, Sagar; Gupta, Anju; Singh, Surjit

    2017-02-15

    A 10-year-old child presented with prolonged fever, lymphadenopathy, weight loss, oral ulcers, alopecia and parotitis. She later developed arterial thrombosis, poly-serositis, nephritis, myocarditis, sacro-ilitis, autoimmune hemolytic anemia and refractory thrombocytopenia. Though anti-dsDNA was negative, she was diagnosed to have systemic lupus erythematosus (SLE). Terminally, she had pulmonary symptoms and succumbed to her illness. The autopsy showed lupus nephritis-Class II, polyserositis, myocarditis, inflammatory myositis, immune mediated vasculitis involving renal, coronary, pancreatic, adrenal, dermal and intramuscular arteries, and pulmonary hemorrhages and edema.

  19. Settings and artefacts relevant for Doppler ultrasound in large vessel vasculitis

    DEFF Research Database (Denmark)

    Terslev, L; Diamantopoulos, A P; Døhn, U Møller

    2017-01-01

    Ultrasound is used increasingly for diagnosing large vessel vasculitis (LVV). The application of Doppler in LVV is very different from in arthritic conditions. This paper aims to explain the most important Doppler parameters, including spectral Doppler, and how the settings differ from those used...... in arthritic conditions and provide recommendations for optimal adjustments. This is addressed through relevant Doppler physics, focusing, for example, on the Doppler shift equation and how angle correction ensures correctly displayed blood velocity. Recommendations for optimal settings are given, focusing...

  20. Leg Pain From Periostitis as the First Clinical Manifestation of Large-Vessel Vasculitis: A Case Report.

    Science.gov (United States)

    Bogaert, Bert; Brys, Peter; Peers, Koen

    2017-07-03

    We report a case of a 47-year-old woman with isolated periostitis of the lower leg as a first manifestation of large-vessel vasculitis. The diagnosis was first suspected when a second magnetic resonance imaging of the lower leg approximately 4 months after onset of shin pain showed edema near intramuscular vessels. Isolated periostitis is a very rare skeletal manifestation of systemic vasculitis and easily could be misdiagnosed. This case demonstrates that maintaining a broad differential diagnosis for a common presenting complaint such as localized leg pain is imperative. To be determined. Copyright © 2017 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.

  1. Leukocytoclastic Vasculitis as a Complication of Recombinant Granulocyte Colony-Stimulating Factor Therapy in a Heart Transplant Patient

    Directory of Open Access Journals (Sweden)

    Giovanbattista Ippoliti

    2014-01-01

    Full Text Available Recombinant granulocyte colony-stimulating factor (rG-CSF is a myeloid growth factor that is widely used in haematology to recover neutropenia secondary to myelosuppressive chemotherapy. Leukocytoclastic vasculitis is an acknowledged side effect of the above therapy. Its pathogenesis involves many mechanisms that collectively induce an increase in neutrophil function and a subsequent release of cytokines. Here, we report a case of leukocytoclastic vasculitis proven by skin biopsy, following the use of rG-CSF in a heart transplant patient with leukopenia secondary to immunosuppressive therapy.

  2. A case of precocious emphysema and lung cancer in a woman with a history of hypocomplementemic urticarial vasculitis.

    Science.gov (United States)

    Jamison, Suzanna C; Brierre, Stephen; Sweet, Jon; de Boisblanc, Ben

    2008-03-01

    Severe emphysema developed in a white woman with a 26-pack-year history of tobacco use. Serum alpha(1)-antitrypsin levels were normal. A history of autoimmune hemolytic anemia, angioedema, low complement, and recurrent urticaria prompted an immunologic workup that ultimately led to a diagnosis of hypocomplementemic urticarial vasculitis syndrome. Treatment with oral prednisone and inhaled bronchodilators improved symptoms, but 4 months after diagnosis non-small cell lung cancer was discovered and she ultimately died. Hypocomplementemic urticarial vasculitis is an uncommon cause of precocious emphysema and has not previously been reported in a patient with bronchogenic carcinoma.

  3. Intermediate-risk grouping of cervical cancer patients treated with radical hysterectomy: a Korean Gynecologic Oncology Group study.

    Science.gov (United States)

    Ryu, S Y; Kim, M H; Nam, B H; Lee, T S; Song, E S; Park, C Y; Kim, J W; Kim, Y B; Ryu, H S; Park, S Y; Kim, K T; Cho, C H; Lee, C; Kim, S M; Kim, B G; Bae, D S; Kim, Y T; Nam, J-H

    2014-01-21

    In this study, we sought to identify a criterion for the intermediate-risk grouping of patients with cervical cancer who exhibit any intermediate-risk factor after radical hysterectomy. In total, 2158 patients with pathologically proven stage IB-IIA cervical cancer with any intermediate-risk factor after radical hysterectomy were randomly assigned to two groups, a development group and a validation group, at a ratio of 3 : 1 (1620 patients:538 patients). To predict recurrence, multivariate models were developed using the development group. The ability of the models to discriminate between groups was validated using the log-rank test and receiver operating characteristic (ROC) analysis. Four factors (histology, tumour size, deep stromal invasion (DSI), and lymphovascular space involvement (LVSI)) were significantly associated with disease recurrence and included in the models. Among the nine possible combinations of the four variables, models consisting of any two of the four intermediate-risk factors (tumour size ≥3 cm, DSI of the outer third of the cervix, LVSI, and adenocarcinoma or adenosquamous carcinoma histology) demonstrated the best performance for predicting recurrence. This study identified a 'four-factor model' in which the presence of any two factors may be useful for predicting recurrence in patients with cervical cancer treated with radical hysterectomy.

  4. Alcoholics in interactional group therapy: an outcome study.

    Science.gov (United States)

    Yalom, I D; Bloch, S; Bond, G; Zimmerman, E; Qualls, B

    1978-04-01

    Three interactional therapy groups of alcoholic patients (N = 20) were formed, and treatment outcome after eight months and again after 12 months of therapy was compared with the outcome of 17 neurotic patients in comparable therapy. Outcome assessment was obtained from three sources: patient, therapist, and independent judge, using both nomothetic and ideographic measures. The results indicated that although more alcoholic than neurotic patients terminated therapy within the first six sessions, a higher percentage of alcoholic patients remained in therapy for 12 months. At the end of 12 months, both samples had improved along a wide variety of variables, and there were no significant differences between the alcoholic and neurotic population in degree of improvement.

  5. High Precision Renormalization Group Study of the Roughening Transition

    CERN Document Server

    Hasenbusch, M; Pinn, K

    1994-01-01

    We confirm the Kosterlitz-Thouless scenario of the roughening transition for three different Solid-On-Solid models: the Discrete Gaussian model, the Absolute-Value-Solid-On-Solid model and the dual transform of the XY model with standard (cosine) action. The method is based on a matching of the renormalization group flow of the candidate models with the flow of a bona fide KT model, the exactly solvable BCSOS model. The Monte Carlo simulations are performed using efficient cluster algorithms. We obtain high precision estimates for the critical couplings and other non-universal quantities. For the XY model with cosine action our critical coupling estimate is $\\beta_R^{XY}=1.1197(5)$. For the roughening coupling of the Discrete Gaussian and the Absolute-Value-Solid-On-Solid model we find $K_R^{DG}=0.6645(6)$ and $K_R^{ASOS}=0.8061(3)$, respectively.

  6. The View-Master Health Study Focus Groups.

    Science.gov (United States)

    Krall, Kathleen; Douglas, Jae; Perrin, Nancy A; Austin, Donald; Lambert, William E; Heumann, Michael

    2008-01-01

    Trichloroethylene (TCE), a common groundwater contaminant, was found at high levels at an Oregon work site in 1998. According to a recent report released by the National Research Council, "the evidence on carcinogenic risk and other health hazards from exposure to trichloroethylene has strengthened since 2001." A convenience sample of 13 former workers from the Oregon work site was recruited for a series of focus groups. Information obtained on plant processes, safety procedures, attitudes regarding medical record access, and opinions about proxy accuracy was subjected to qualitative content analysis. Workers recalled few safety policies and no training or support for control of safety. Most thought co-workers and family members would be the best source of proxy exposure information and favored granting access to medical records. Job-role mobility confirmed the importance of using a job or task exposure matrix. Information obtained will be used in development of an exposure assessment interview tool.

  7. Medication errors in home care: a qualitative focus group study.

    Science.gov (United States)

    Berland, Astrid; Bentsen, Signe Berit

    2017-11-01

    To explore registered nurses' experiences of medication errors and patient safety in home care. The focus of care for older patients has shifted from institutional care towards a model of home care. Medication errors are common in this situation and can result in patient morbidity and mortality. An exploratory qualitative design with focus group interviews was used. Four focus group interviews were conducted with 20 registered nurses in home care. The data were analysed using content analysis. Five categories were identified as follows: lack of information, lack of competence, reporting medication errors, trade name products vs. generic name products, and improving routines. Medication errors occur frequently in home care and can threaten the safety of patients. Insufficient exchange of information and poor communication between the specialist and home-care health services, and between general practitioners and healthcare workers can lead to medication errors. A lack of competence in healthcare workers can also lead to medication errors. To prevent these, it is important that there should be up-to-date information and communication between healthcare workers during the transfer of patients from specialist to home care. Ensuring competence among healthcare workers with regard to medication is also important. In addition, there should be openness and accurate reporting of medication errors, as well as in setting routines for the preparation, alteration and administration of medicines. To prevent medication errors in home care, up-to-date information and communication between healthcare workers is important when patients are transferred from specialist to home care. It is also important to ensure adequate competence with regard to medication, and that there should be openness when medication errors occur, as well as in setting routines for the preparation, alteration and administration of medications. © 2017 John Wiley & Sons Ltd.

  8. Treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis with high-dose intravenous immunoglobulin.

    Science.gov (United States)

    Richter, C; Schnabel, A; Csernok, E; De Groot, K; Reinhold-Keller, E; Gross, W L

    1995-07-01

    In this uncontrolled study 15 patients with ANCA-associated systemic vasculitis, who were poor responders to conventional therapy, were treated with single or multiple courses of intravenous immunoglobulin (IVIG), 30 g/day over 5 days. Clinical and serological evaluation was performed before and 4 weeks after IVIG. Six of the 15 patients experienced clinically significant benefit from IVIG. Improvement was confined to single organ manifestations (skin, ENT findings), no improvement was seen with conjunctivitis and scleritis, pericarditis or nephritis. No patient experienced complete remission after IVIG. Repeated courses of IVIG at 4-week intervals were no more effective than single courses. In six anti-proteinase 3 (PR3)-positive patients pretreatment sera were incubated with F(ab')2 fragments of the IVIG preparation in vitro to measure the inhibitory effect of IVIG on anti-PR3 activity. An inhibition of anti-PR3 activity by 25-70% was observed; this did not correlate with clinical effects. Approximately 40% of patients benefited from IVIG treatment, though complete remission of disease activity did not occur. Neither clinical characteristics nor the inhibitory effect of the IVIG preparation on serum anti-PR3 activity in vitro predicted clinical response to this treatment modality.

  9. Vasculite cerebral e uso de cocaína e crack Cerebral vasculitis and cocaine and crack abuse

    Directory of Open Access Journals (Sweden)

    Fernando Madalena Volpe

    1999-09-01

    Full Text Available O abuso de cocaína e crack está associado com importante parcela dos acidentes vasculares cerebrais, especialmente em pacientes jovens. O presente estudo relata o caso de um usuário de cocaína e crack que desenvolveu vasculite do sistema nervoso central, resultando em infartos cerebrais e edema extensos, levando à demência com alterações comportamentais e convulsões. Ressalta-se a importância de suspeitar do uso de drogas em jovens que se apresentam com acidente vascular cerebral, assim como avaliar possíveis lesões cerebrais em usuários de drogas com deterioração cognitiva.Cocaine and crack abuse is strongly related to stroke, particularly in young patients. The present study reports the case of a cocaine and crack abuser who developed central nervous system vasculitis, resulting in extensive cerebral infarctions, leading to dementia, behavioural disturbances and seizures. The relevance of detecting drug abuse in young stroke patients is stressed. Assessing possible brain lesions in drug abusers with cognitive impairment is also important.

  10. NASBE Study Group Surveys State Leadership Development Policy

    Science.gov (United States)

    Newman, Bobbi; Hull, Robert

    2015-01-01

    State board members, working in partnership with the Consortium for Policy Research in Education (CPRE) at the University of Pennsylvania, conducted an in-depth study of states' school leadership development policies and practices. Data from this study are being analyzed to determine ways that states can create systems and structures for…

  11. In-Depth Cultural Studies in Multicultural Group

    Science.gov (United States)

    Silina-Jasjukevica, Gunta; Briška, Ilze

    2016-01-01

    There is much research and educational practices at all levels of education on how to deal with promoting acceptance and understanding between different cultures. A cultural study forms an important part of shaping intercultural understanding. "The aim" of the research is to analyze an innovative way of incorporating cultural studies in…

  12. Medical Student Perspectives of Active Learning: A Focus Group Study.

    Science.gov (United States)

    Walling, Anne; Istas, Kathryn; Bonaminio, Giulia A; Paolo, Anthony M; Fontes, Joseph D; Davis, Nancy; Berardo, Benito A

    2017-01-01

    Phenomenon: Medical student perspectives were sought about active learning, including concerns, challenges, perceived advantages and disadvantages, and appropriate role in the educational process. Focus groups were conducted with students from all years and campuses of a large U.S. state medical school. Students had considerable experience with active learning prior to medical school and conveyed accurate understanding of the concept and its major strategies. They appreciated the potential of active learning to deepen and broaden learning and its value for long-term professional development but had significant concerns about the efficiency of the process, the clarity of expectations provided, and the importance of receiving preparatory materials. Most significantly, active learning experiences were perceived as disconnected from grading and even as impeding preparation for school and national examinations. Insights: Medical students understand the concepts of active learning and have considerable experience in several formats prior to medical school. They are generally supportive of active learning concepts but frustrated by perceived inefficiencies and lack of contribution to the urgencies of achieving optimal grades and passing United States Medical Licensing Examinations, especially Step 1.

  13. In-depth Cultural Studies in Multicultural Group

    Directory of Open Access Journals (Sweden)

    Siliņa-Jasjukeviča Gunta

    2016-12-01

    Full Text Available There is much research and educational practices at all levels of education on how to deal with promoting acceptance and understanding between different cultures. A cultural study forms an important part of shaping intercultural understanding. The aim of the research is to analyze an innovative way of incorporating cultural studies in teacher education program from the perspective of encouraging multinational students to reveal common values within diverse manifestations of different cultures. The present article describes a qualitative study of multinational students’ experiences in international project related to the learning about Nordic and Baltic cultural traditions. In the conclusion of the article, the efficiency of the structure of content and the process of in-depth cultural studies are analyzed. The discussion contains problems for further research of this topic.

  14. Análisis Histórico de la Vasculitis. Clasificación y propuestas para un entendimiento.

    Directory of Open Access Journals (Sweden)

    Antonio Iglesias Gamarra

    2002-04-01

    Full Text Available

    Apartir de 1978, siendo residente de Reumatología iniciamos el estudio de las vasculitis en el Hospital San Juan de Dios de Bogotá. Se escribió el primer texto de vasculitis necrotizante que ganó el premio Salvat en 1980 y se publicó en 1982: Este libro tenía como objeto informar el tema a la comunidad médica.

    Durante mi estadía en Barranquilla, se realizó una descripción de 9 pacientes con los doctores Eduardo Egea, Gloria Garavito y Silvio Severini sobre vasculitis linfomonocítica. Previamente se conocían algunos informes en la literatura médica como el realizado por Mc’Duffie fundador del Laboratorio de la Clínica Mayo en Rochester y Jorge Maldonado quienes describieron una vasculitis asociada a urticaria y se documentó una precipitina C1q, pero que no reunía los criterios de lupus eritematoso sistémico.

    Otra publicación era la de Soter quien describió dos tipos de vasculitis, la leucocitoclastica y la linfomonocitica. Con estos estudios previos, Iglesias y col estudiamos 9 pacientes que consultaron por urticaria, lesiones eritematosas, papulas, etc. Las lesiones de urticaria tenían una característica importante y era que permanecían por más de 24 horas en el sitio de la piel comprometida, eran de tipo urente y al tomar una biopsia de esta lesión se observaba un infiltrado linfoóplasmocitario; posiblemente su patogénesis está relacionada con un mecanismo de tipo celular y participan una serie de proteínas (como integrinas, selectinas y moléculas de adhesión.

    Se documenta que las vasculitis linfomonocíticas se asocian a algunos medicamentos, procesos infecciosos, o son secundarias a algunas enfermedades del tejido conjuntivo como el lupus y el síndrome de Sjögren primario.

    Desde hace más o menos cinco años estamos estudiando en las unidades de reumatología, dermatología y patología del Hospital San Juan de Dios, a los pacientes con lesiones nodulares, paniculitis y eritema

  15. Fibrosis pulmonar asociada a vasculitis con anticuerpos anticitoplasmáticos positivos

    Directory of Open Access Journals (Sweden)

    Marcelo Fernández Casares

    2012-08-01

    Full Text Available Las complicaciones pulmonares más conocidas de las vasculitis con anticuerpos anticitoplasmáticos de los neutrófilos (ANCA positivos (VAA, son la hemorragia alveolar, los granulomas y la estenosis de la vía aérea. En los últimos años han aparecido algunos informes aislados que muestran la asociación con fibrosis pulmonar (FP, sugiriendo que ésta sería otra complicación de las VAA. En este trabajo informamos dos casos con dicha asociación describiendo sus características clínicas, tomográficas e inmunológicas. Dado que en la asociación de FP y VAA notificada en los últimos años, la FP puede ser su primera manifestación, podría ser necesaria la búsqueda de ANCA en pacientes con FP, como causa de la misma y por el posible desarrollo posterior de vasculitis.

  16. A Case of Polyarteritis Nodosa Associated with Vertebral Artery Vasculitis Treated Successfully with Tocilizumab and Cyclophosphamide

    Directory of Open Access Journals (Sweden)

    Kae Watanabe

    2016-01-01

    Full Text Available Pediatric polyarteritis nodosa is rare systemic necrotizing arteritis involving small- and medium-sized muscular arteries characterized by aneurysmal dilatations involving the vessel wall. Aneurysms associated with polyarteritis nodosa are common in visceral arteries; however intracranial aneurysms have also been reported and can be associated with central nervous system symptoms, significant morbidity, and mortality. To our knowledge extracranial involvement of the vertebral arteries has not been reported but has the potential to be deleterious due to fact that they supply the central nervous system vasculature. We present a case of a 3-year-old Haitian boy with polyarteritis nodosa that presented with extracranial vessel involvement of his vertebral arteries. After thorough diagnostic imaging, including a bone scan, ultrasound, Magnetic Resonance Imaging/Angiography, and Computed Tomography Angiography, he was noted to have vertebral artery vasculitis, periostitis, subacute epididymoorchitis, arthritis, and myositis. He met diagnostic criteria for polyarteritis nodosa and was treated with cyclophosphamide, methylprednisolone, and tocilizumab, which resulted in improvement of his inflammatory markers, radiographic findings, and physical symptoms after treatment. To the authors’ knowledge, this is the first report of vertebral artery vasculitis in polyarteritis nodosa as well as successful treatment of the condition using the combination cyclophosphamide and tocilizumab for this condition.

  17. A Case of Polyarteritis Nodosa Associated with Vertebral Artery Vasculitis Treated Successfully with Tocilizumab and Cyclophosphamide

    Science.gov (United States)

    Watanabe, Kae; Rajderkar, Dhanashree A.; Modica, Renee F.

    2016-01-01

    Pediatric polyarteritis nodosa is rare systemic necrotizing arteritis involving small- and medium-sized muscular arteries characterized by aneurysmal dilatations involving the vessel wall. Aneurysms associated with polyarteritis nodosa are common in visceral arteries; however intracranial aneurysms have also been reported and can be associated with central nervous system symptoms, significant morbidity, and mortality. To our knowledge extracranial involvement of the vertebral arteries has not been reported but has the potential to be deleterious due to fact that they supply the central nervous system vasculature. We present a case of a 3-year-old Haitian boy with polyarteritis nodosa that presented with extracranial vessel involvement of his vertebral arteries. After thorough diagnostic imaging, including a bone scan, ultrasound, Magnetic Resonance Imaging/Angiography, and Computed Tomography Angiography, he was noted to have vertebral artery vasculitis, periostitis, subacute epididymoorchitis, arthritis, and myositis. He met diagnostic criteria for polyarteritis nodosa and was treated with cyclophosphamide, methylprednisolone, and tocilizumab, which resulted in improvement of his inflammatory markers, radiographic findings, and physical symptoms after treatment. To the authors' knowledge, this is the first report of vertebral artery vasculitis in polyarteritis nodosa as well as successful treatment of the condition using the combination cyclophosphamide and tocilizumab for this condition. PMID:27018080

  18. Necrotizing mycotic vasculitis with cerebral infarction caused by Aspergillus niger in a horse with acute typholocolitis.

    Science.gov (United States)

    Tunev, S S; Ehrhart, E J; Jensen, H E; Foreman, J H; Richter, R A; Messick, J B

    1999-07-01

    An 18-year-old Morgan mare was presented to the Veterinary Medical Teaching Hospital, University of Illinois, with a 10-day history of watery diarrhea, depression, and dysphagia. On admission, the animal was severely dehydrated, depressed, and unable to swallow and had no clinical signs of diarrhea. The respiratory and heart rate and body temperature were within normal limits. Following fluid therapy, the mare developed severe watery diarrhea and continued to be depressed, incoordinated, and dysphagic. The animal died on the fourth day after admission and was sent to the Laboratories of Veterinary Diagnostic Medicine for necropsy. Gross postmortem findings were consistent with an acute cerebral infarction in the right cerebral hemisphere, an acute necrotizing typhlocolitis, multifocal petechial and ecchymotic hemorrhages, enlarged and congested pars intermedia of the pituitary gland, and marked bilateral adrenocortical hyperplasia with multifocal areas of necrosis and hemorrhage. Histologic evaluation of the affected brain demonstrated an area of coagulative necrosis of the gray matter, with hemorrhage, vasculitis, and thrombosis. There were many fungal hyphae 3.5-6.0 microm, pale basophilic, septate, and occasionally branching at 45 degrees present in the arterial walls and throughout the necrotic tissue. Immunohistochemical analysis revealed Aspergillus niger as the etiologic agent responsible for the mycotic vasculitis and infarction in the brain. Bacteria culture and immunohistochemical staining of the colon and cecum failed to demonstrate specific pathogens.

  19. De Novo PTEN Mutation in a Young Boy with Cutaneous Vasculitis

    Directory of Open Access Journals (Sweden)

    Angela Mauro

    2017-01-01

    Full Text Available Phosphatase and tensin homolog (PTEN is the protein encoded by the PTEN gene (10q23.3. PTEN mutations are related to a variety of rare diseases referred to collectively as PTEN hamartoma tumor syndromes (PHTS, which include Cowden Syndrome, Bannayan-Riley-Ruvalcaba syndrome, Proteus Syndrome, and Proteus-like syndrome. These diseases are associated with an increased risk of malignancy and for this reason an accurate and early diagnosis is essential in order to institute cancer surveillance. PTEN is a regulator of growth and homeostasis in immune system cells, although there are limited data describing immune dysregulation caused by PTEN mutations. We describe a case of PHTS syndrome caused by a de novo mutation in PTEN detected using a targeted next generation sequencing (NGS gene panel which was instigated for workup of cutaneous vasculitis. We highlight the diagnostic utility of this approach and that mutations in PTEN may be associated with immune-dysregulatory features such as vasculitis in young children.

  20. [Severe occlusive bilateral retinal vasculitis within the scope of seronegative systemic lupus erythematosus].

    Science.gov (United States)

    Koch, J W; al Nawaiseh, I; Koch, F H

    1992-11-01

    Retinal vascular manifestation is the most common form of ophthalmic involvement in patients with systemic lupus erythematosus (SLE). Most frequently these consist of cotton-wool spots with or without intraretinal hemorrhages. Although rare, a more severe retinal vaso-occlusive disease, termed retinal vasculitis, has been described. We report on a 37-year-old white female with a 13-year follow-up of chronic discoid lupus erythematosus, which suffered massive bilateral visual loss coincident with the systemic exacerbation of her disease (proteinuria, pneumonia, serositis, leucopenia). The diagnosis of SLE was established with reference to the revised ARA-criteria (American Rheumatism Association). Ophthalmoscopy and fluorescein angiography revealed the typical aspect of a SLE-associated vaso-occlusive retinopathy on both eyes with marked ischemia of the macula. Immediate maximal immuno-suppressive therapy, early performed panretinal photocoagulation and subsequent cryoretinopexy did not stop the progression of the disease. Six months after the initial event vascularisations of the disc and rubeosis iridis occurred, but no secondary glaucoma up to date. In this patient, the almost complete absence of characteristic autoantibodies and immunological markers was striking. The correlation with other lupus manifestations, different therapeutic concepts and prognostic factors in SLE-associated retinal vasculitis are discussed.