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Sample records for lupus erythematosus prospective

  1. Cutaneous lupus erythematosus

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    Biazar, Cyrus; Sigges, Johanna; Patsinakidis, Nikolaos

    2013-01-01

    In this prospective, cross-sectional, multicenter study, we assessed clinical and laboratory characteristics from patients with cutaneous lupus erythematosus (CLE) using the Core Set Questionnaire of the European Society of Cutaneous Lupus Erythematosus (EUSCLE). 1002 (768 females, 234 males...... included gender, age at onset of disease, LE-specific and LE-nonspecific skin lesions, photosensitivity, laboratory features, and the criteria of the American College of Rheumatology (ACR) for the classification of systemic lupus erythematosus. The mean age at onset of disease was 43.0±15.7 years...

  2. Discoid Lupus Erythematosus

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    ... Name: Category: Share: Yes No, Keep Private Discoid Lupus Erythematosus Share | Discoid lupus erythematosus (DLE) is a chronic skin condition of ... occur. A small percentage of patients with discoid lupus can develop disease of the internal organs, which ...

  3. Prognostic implications of active discoid lupus erythematosus and malar rash at the time of diagnosis of systemic lupus erythematosus: Results from a prospective cohort study.

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    Drucker, A M; Su, J; Mussani, F; Siddha, S K; Gladman, D D; Urowitz, M B

    2016-04-01

    Cutaneous lupus erythematosus (CLE) may have prognostic implications for systemic lupus erythematosus (SLE). We aimed to determine the impact of discoid lupus erythematosus (DLE) and malar rash on SLE disease activity. Data were analyzed from the Toronto Lupus Clinic prospective cohort study. We compared SLE patients with active DLE or malar rash at SLE diagnosis to SLE patients who never developed CLE. Outcomes were assessed at one and five years, including Adjusted Mean Systemic Lupus Erythematosus Disease Activity Index 2000 (AMS). A total of 524 SLE patients (284 without CLE, 65 with DLE, and 175 with malar rash) were included. Mean AMS scores in patients without CLE at one and five years were 5.96 ± 5.06 and 4.00 ± 3.52, which did not differ significantly from scores at one (6.93 ± 5.31, p = 0.17) and five years (4.29 ± 2.62, p = 0.63) in the DLE group. In patients with malar rash, AMS scores at one (8.30 ± 6.80, p systemic disease over time, while DLE has no significant impact on general SLE disease activity. © The Author(s) 2015.

  4. Hypertrophic discoid lupus erythematosus.

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    Farley-Loftus, Rachel; Elmariah, Sarina B; Ralston, Jonathan; Kamino, Hideko; Franks, Andrew G

    2010-11-15

    Hypertrophic discoid lupus erythematosus is a distinct form of chronic cutaneous (discoid) lupus, which is characterized by hyperkeratotic plaques that typically are observed over the face, arms, and upper trunk. We present the case of a 43-year-old man with verrucous plaques that were distributed symmetrically over the face, who initially was treated with oral antibiotics and topical glucocorticoids for acne vulgaris. A biopsy specimen confirmed the diagnosis of hypertrophic discoid lupus erythematosus. The clinical and histopathologic features of this clinical variant are reviewed.

  5. The African Lupus Genetics Network (ALUGEN) registry: standardized, prospective follow-up studies in African patients with systemic lupus erythematosus.

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    Hodkinson, B; Mapiye, D; Jayne, D; Kalla, A; Tiffin, N; Okpechi, I

    2016-03-01

    The prevalence and severity of systemic lupus erythematosus (SLE) differs between ethnic groups and geographical regions. Although initially reported as rare, there is growing evidence that SLE is prevalent and runs a severe course in Africa. There is a paucity of prospective studies on African SLE patients. The African Lupus Genetics Network (ALUGEN) is a multicentred framework seeking to prospectively assess outcomes in SLE patients in Africa. Outcomes measured will be death, hospital admission, disease activity flares, and SLE-related damage. We will explore predictors for these outcomes including clinical, serological, socio-demographic, therapeutic and genetic factors. Further, we will investigate comorbidities and health-related quality of life amongst these patients. Data of patients recently (≤ 5 yrs) diagnosed with SLE will be collected at baseline and annual follow-up visits, and captured electronically. The ALUGEN project will facilitate standardized data capture for SLE cases in Africa, allowing participating centres to develop their own SLE registries, and enabling collaboration to enrich our understanding of inter-ethnic and regional variations in disease expression. Comprehensive, high-quality multi-ethnic data on African SLE patients will expand knowledge of the disease and inform clinical practice, in addition to augmenting research capacity and networking links and providing a platform for future biomarker and interventional studies. © The Author(s) 2015.

  6. Genetics Home Reference: systemic lupus erythematosus

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    ... Twitter Home Health Conditions systemic lupus erythematosus systemic lupus erythematosus Printable PDF Open All Close All Enable ... to view the expand/collapse boxes. Description Systemic lupus erythematosus (SLE) is a chronic disease that causes ...

  7. Kutan lupus erythematosus

    DEFF Research Database (Denmark)

    Sandreva, Tatjana; Voss, Anne; Bygum, Anette

    2016-01-01

    Cutaneous lupus erythematosus (LE) is an autoimmune disease. The most common clinical forms are acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE) and discoid LE (DLE). Cutaneous LE, mainly ACLE, can be the first sign of systemic LE (SLE). DLE and SCLE are less associated with development...

  8. Neuropsychiatric Systemic Lupus Erythematosus

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    Popescu, Alexandra; Kao, Amy H

    2011-01-01

    Neuropsychiatric systemic lupus erythematosus (NPSLE) is the least understood, yet perhaps the most prevalent manifestation of lupus. The pathogenesis of NPSLE is multifactorial and involves various inflammatory cytokines, autoantibodies, and immune complexes resulting in vasculopathic, cytotoxic and autoantibody-mediated neuronal injury. The management of NPSLE is multimodal and has not been subjected to rigorous study. Different treatment regimens include nonsteroidal anti-inflammatory drugs, anticoagulation, and immunosuppressives such as cyclophosphamide, azathioprine, mycophenolate mofetil, and methotrexate. For refractory NPSLE, intravenous immunoglobulin (IVIG), plasmapheresis, and rituximab have been used. Adjunctive symptomatic treatment complements these therapies by targeting mood disorders, psychosis, cognitive impairment, seizures or headaches. Several new biological agents are being tested including Belimumab, a human monoclonal antibody that targets B lymphocyte stimulator. This review focuses on the pathophysiology, treatment, and new potential therapies for neuropsychiatric manifestations of systemic lupus erythematosus. PMID:22379459

  9. Headache in Systemic Lupus Erythematosus

    DEFF Research Database (Denmark)

    Hanly, John G; Urowitz, Murray B; O'Keeffe, Aidan G

    2013-01-01

    To examine the frequency and characteristics of headaches and their association with global disease activity and health-related quality of life (HRQOL) in patients with systemic lupus erythematosus (SLE).......To examine the frequency and characteristics of headaches and their association with global disease activity and health-related quality of life (HRQOL) in patients with systemic lupus erythematosus (SLE)....

  10. Melanotic lupus erythematosus: A rare variant of discoid lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Seval Doğruk Kaçar

    2015-03-01

    Full Text Available Discoid lupus erythematosus (DLE is characterized by atrophic patches with peripheral hyperpigmentation on sun exposed skin areas. It rarely presents with diffuse hyperpigmented patches without erythema, adherent scale and atrophy on face and neck. This presentation is called as melanotic lupus erythematosus and it is rarely reported in literature. Other diseases that cause facial pigmentation as melasma, Riehl melanosis, lichen planus pigmentosus, drug related pigmentation should be considered in the differential diagnosis. We herein report a 66 year old male patient with diffuse blue-brown pigmentations on his face which was diagnosed as melanotic lupus erythematosus with the clinical, histopathological and immunofluoresence findings.

  11. Systemic lupus erythematosus serositis

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    Low, V.H.S.; Robins, P.D.; Sweeney, D.J. [Sir Charles Gairdner Hospital, Perth, WA (Australia). Dept. of Diagnostic Radiology

    1995-08-01

    The imaging appearances of a case of systemic lupus erythematosus, which manifested initially as a serositis, is described. Barium small bowel study showed segments of spiculation with tethering, angulation, and obstruction. Computed tomography scan of the abdomen confirmed ascites. It was also useful in demonstrating free fluid, bowel wall oedema, and serosal thickening . Follow up scanning to demonstrate resolution of changes may also be of value. The definitive diagnosis was made on the basis of marked elevation of antinuclear and anti-double stranded DNA antibodies. 10 refs., 2 figs.

  12. Systemic lupus erythematosus.

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    Lisnevskaia, Larissa; Murphy, Grainne; Isenberg, David

    2014-11-22

    Systemic lupus erythematosus is a remarkable and challenging disorder. Its diversity of clinical features is matched by the complexity of the factors (genetic, hormonal, and environmental) that cause it, and the array of autoantibodies with which it is associated. In this Seminar we reflect on changes in its classification criteria; consider aspects of its more serious clinical expression; and provide a brief review of its aetiopathogenesis, major complications, coping strategies, and conventional treatment. Increased understanding of the cells and molecules involved in the development of the diseases has encouraged the identification of new, better targeted biological approaches to its treatment. The precise role of these newer therapies remains to be established.

  13. Outcomes of neuropsychiatric events in systemic lupus erythematosus based on clinical phenotypes; prospective data from the Leiden NP SLE cohort.

    Science.gov (United States)

    Magro-Checa, C; Beaart-van de Voorde, L J J; Middelkoop, H A M; Dane, M L; van der Wee, N J; van Buchem, M A; Huizinga, T W J; Steup-Beekman, G M

    2017-04-01

    Objective The objective of this study was to assess whether clinical and patient's reported outcomes are associated with a different pathophysiological origin of neuropsychiatric events presenting in systemic lupus erythematosus. Methods A total of 232 neuropsychiatric events presenting in 131 systemic lupus erythematosus patients were included. Neuropsychiatric systemic lupus erythematosus diagnosis was established per event by multidisciplinary evaluation. All neuropsychiatric events were divided according to a suspected underlying pathophysiological process into one of the following: non-neuropsychiatric systemic lupus erythematosus related, inflammatory and ischaemic neuropsychiatric systemic lupus erythematosus. The clinical outcome of all neuropsychiatric events was determined by a physician-completed four-point Likert scale. Health-related quality of life was measured with the subscales of the patient-generated Short Form 36 (SF-36) health survey questionnaire. The change between scores at paired visits of all domain scores, mental component summary (SF-36 MCS) and physical component summary (SF-36 PCS) scores were retrospectively calculated and used as patient-reported outcome. The association among these outcomes and the different origin of neuropsychiatric events was obtained using multiple logistic regression analysis. Results The clinical status of 26.8% non-neuropsychiatric systemic lupus erythematosus events, 15.8% ischaemic neuropsychiatric systemic lupus erythematosus and 51.6% inflammatory neuropsychiatric systemic lupus erythematosus improved after re-assessment. Almost all SF-36 domains had a positive change at re-assessment in all groups independently of the origin of neuropsychiatric events. Neuropsychiatric systemic lupus erythematosus ( B = 0.502; p < 0.001) and especially inflammatory neuropsychiatric systemic lupus erythematosus ( B = 0.827; p < 0.001) had better clinical outcome, with change in disease activity being the

  14. [Systemic lupus erythematosus and weakness].

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    Vinagre, Filipe; Santos, Maria José; da Silva, José Canas

    2006-01-01

    We report a case of a 13-year old young girl, with Juvenile Systemic Lupus Erythematosus and recent onset of muscle weakness. Investigations lead to the diagnosis of Myasthenia Gravis. The most important causes of muscle weakness in lupus patients are discussed.

  15. Drug-induced lupus erythematosus

    Directory of Open Access Journals (Sweden)

    M. Carrabba

    2011-09-01

    Full Text Available Drug-induced lupus is a syndrome which share symptoms and laboratory characteristics with the idiopathic systemic lupus erythematosus (SLE. The list of medications implicated as etiologic agents in drug-induced lupus continues to grow. The terms used for this condition are lupus-like syndrome, drug-induced lupus erythematosus (DILE and drug related lupus. More than 80 drugs have been associated with DILE. The first case of DILE was reported in 1945 and associated with sulfadiazin. In 1953 it was reported that DILE was related to the use of hydralazine. Drugs responsible for the development of DILE can divided into three groups, but the list of these drugs is quite long because new drugs are included yearly in the list. The syndrome is characterised by arthralgia, myalgia, pleurisy, rash and fever in association with antinuclear antibodies in the serum. Recognition of DILE is important because it usually reverts within a few weeks after stopping the drug.

  16. Systemic lupus erythematosus and Klinefelter's syndrome.

    OpenAIRE

    French, M A; Hughes, P

    1983-01-01

    A case of Klinefelter's syndrome presenting with systemic lupus erythematosus while receiving androgen replacement therapy is described. The association of systemic lupus erythematosus with Klinefelter's syndrome is discussed, particularly in terms of the effect of sex hormones.

  17. SYSTEMIC LUPUS ERYTHEMATOSUS : CASE REPORT

    Directory of Open Access Journals (Sweden)

    Ida Ayu Tri Wedari

    2014-02-01

    Full Text Available Systemic lupus erythematosus (systemic lupus erythematosus, SLE, an autoimmune disease characterized by the production of antibodies against components of the cellnucleus that is associated with a broad clinical manifestations. Ninety percent of casesof systemic lupus erythematosus attacking a young woman with a peak incidence at 1540yearsofageduringthereproductiveperiodwiththeratioofwomenandmen5:1.Itsetiologyis unclear, allegedly associated with a specific immune response genes in themajor histocompatibility complex class II, HLA-DR2 and HLA DR3. Clinicalmanifestations which appear heterogeneous and involve almost all organ systems of thecondition of the joints and skin of patients with mild to severe disease that attacks thecentral nervous system, lungs, gastrointestinal tract, and kidneys. Treatment is mainlyaimed at controlling the symptoms of the acute attack and suppress symptoms andweight at a level that can be tolerated and prevent organ damage.

  18. "Bound" globulin in the skin of patients with chronic discoid lupus erythematosus and systemic lupus erythematosus

    NARCIS (Netherlands)

    Cormane, R.H.

    1964-01-01

    In what respect chronic discoid lupus erythematosus is related to systemic lupus erythematosus is still uncertain. In discoid lupus the lupus-erythematosus (L.E.) phenomenon is negative, and the history does not suggest vascular lesions or involvement of serous membranes. In both diseases the pathog

  19. "Bound" globulin in the skin of patients with chronic discoid lupus erythematosus and systemic lupus erythematosus

    NARCIS (Netherlands)

    Cormane, R.H.

    1964-01-01

    In what respect chronic discoid lupus erythematosus is related to systemic lupus erythematosus is still uncertain. In discoid lupus the lupus-erythematosus (L.E.) phenomenon is negative, and the history does not suggest vascular lesions or involvement of serous membranes. In both diseases the

  20. "Bound" globulin in the skin of patients with chronic discoid lupus erythematosus and systemic lupus erythematosus

    NARCIS (Netherlands)

    Cormane, R.H.

    1964-01-01

    In what respect chronic discoid lupus erythematosus is related to systemic lupus erythematosus is still uncertain. In discoid lupus the lupus-erythematosus (L.E.) phenomenon is negative, and the history does not suggest vascular lesions or involvement of serous membranes. In both diseases the pathog

  1. Biomarkers for systemic lupus erythematosus.

    Science.gov (United States)

    Ahearn, Joseph M; Liu, Chau-Ching; Kao, Amy H; Manzi, Susan

    2012-04-01

    The urgent need for lupus biomarkers was demonstrated in September 2011 during a Workshop sponsored by the Food and Drug Administration: Potential Biomarkers Predictive of Disease Flare. After 2 days of discussion and more than 2 dozen presentations from thought leaders in both industry and academia, it became apparent that highly sought biomarkers to predict lupus flare have not yet been identified. Even short of the elusive biomarker of flare, few biomarkers for systemic lupus erythematosus (SLE) diagnosis, monitoring, and stratification have been validated and employed for making clinical decisions. This lack of reliable, specific biomarkers for SLE hampers proper clinical management of patients with SLE and impedes development of new lupus therapeutics. As such, the intensity of investigation to identify lupus biomarkers is climbing a steep trajectory, lending cautious optimism that a validated panel of biomarkers for lupus diagnosis, monitoring, stratification, and prediction of flare may soon be in hand.

  2. Lupus Erythematosus Panniculitis in Pregnancy

    OpenAIRE

    Swati Gondane; Rajkumar Kothiwala; Sapna Dangi; Ashok Meherda

    2015-01-01

    A case of lupus erythematosus (LE) panniculitis in pregnancy without any lesions of discoid LE or systemic LE is being reported. There were no systemic symptoms. Her ANA, anti-dsDNA, anti-Ro/SSA, and anti-La/SSB antibodies were within normal limits. Diagnosis of lupus panniculitis was considered on clinical and histopathological grounds. The condition responded favorably to systemic steroid therapy.

  3. Lupus erythematosus panniculitis in pregnancy

    Directory of Open Access Journals (Sweden)

    Swati Gondane

    2015-01-01

    Full Text Available A case of lupus erythematosus (LE panniculitis in pregnancy without any lesions of discoid LE or systemic LE is being reported. There were no systemic symptoms. Her ANA, anti-dsDNA, anti-Ro/SSA, and anti-La/SSB antibodies were within normal limits. Diagnosis of lupus panniculitis was considered on clinical and histopathological grounds. The condition responded favorably to systemic steroid therapy.

  4. [Systemic lupus erythematosus and pregnancy].

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    Diniz-da-Costa, Teresa; Centeno, Mónica; Pinto, Luísa; Marques, Aurora; Mendes-Graça, Luís

    2012-01-01

    Systemic lupus erythematosus is a chronic inflammatory disease, resulting from an auto-immune dysfunction. The etiology of this disease is unknown. It frequently occurs in women of childbearing age. Pregnancy in patients with systemic lupus erythematosus may be associated with several complications (maternal, obstetrical and fetal). The prognosis for both mother and child is better when systemic lupus erythematosus has been quiescent for at least six months before pregnancy. Thus, preconceptional assessment and management is crucial for helping women to achieve a period of disease remission before pregnancy as well as for allowing an adjustment of therapy. Maternal health and fetal development should be closely monitored during pregnancy. These patients should be surveilled by a multidisciplinary team (obstetrician, rheumatologist or internist, nephrologist if necessary and a pediatrician), in a tertiary care hospital. Antiphospholipid syndrome, positivity for anti-SSA/Ro or anti-SSB/LA antibodies, hypertension or renal involvement are associated with an increase of adverse pregnancy outcomes. In this article the authors review the main aspects of Systemic lupus erythematosus (SLE) and pregnancy.

  5. Systemisk lupus erythematosus og graviditet

    DEFF Research Database (Denmark)

    Schreiber, Karen; Lykke, Jacob Alexander; Nielsen, Henriette Svarre

    2016-01-01

    Systemic lupus erythematosus (SLE) is a complex autoimmune disease which most often affects women of childbearing age. Pregnancy is therefore an important issue for the patient and the responsible physician. Pregnancy outcomes in women with SLE has improved significantly over the latest decades...

  6. Systemisk lupus erythematosus og graviditet

    DEFF Research Database (Denmark)

    Schreiber, Karen; Lykke, Jacob Alexander; Nielsen, Henriette Svarre

    2016-01-01

    Systemic lupus erythematosus (SLE) is a complex autoimmune disease which most often affects women of childbearing age. Pregnancy is therefore an important issue for the patient and the responsible physician. Pregnancy outcomes in women with SLE has improved significantly over the latest decades...

  7. Systemic lupus erythematosus in Denmark

    DEFF Research Database (Denmark)

    Voss, A; Green, A; Junker, P

    1998-01-01

    A population based cohort of patients with systemic lupus erythematosus (SLE) was recruited from a for epidemiological purposes representative Danish region. Patients were ascertained from 4 different sources with a high degree of completeness as estimated by using capture-recapture analysis...

  8. Mood Disorders in Systemic Lupus Erythematosus

    DEFF Research Database (Denmark)

    Hanly, John G; Su, Li; Urowitz, Murray B

    2015-01-01

    OBJECTIVE: To examine the frequency, characteristics, and outcome of mood disorders, as well as clinical and autoantibody associations, in a multiethnic/racial, prospective inception cohort of patients with systemic lupus erythematosus (SLE). METHODS: Patients were assessed annually for mood...... disorders (4 types, according to the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition) and 18 other neuropsychiatric events. Global disease activity scores (SLE Disease Activity Index 2000 [SLEDAI-2K]), damage scores (Systemic Lupus International Collaborating Clinics/American College...... time. The lack of association with global SLE disease activity, cumulative organ damage, and lupus autoantibodies emphasizes the multifactorial etiology of mood disorders and a role for non-lupus-specific therapies....

  9. Systemic Lupus Erythematosus

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    ... in the fields of medicine, nursing, dentistry, veterinary medicine, the health care system, and preclinical sciences. Hope for the Future With research advances and a better understanding of lupus, the prognosis for people with lupus today is ...

  10. Breastfeeding in mothers with systemic lupus erythematosus.

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    Noviani, M; Wasserman, S; Clowse, M E B

    2016-08-01

    Breastfeeding is known to improve the well-being of a mother and her infant, and about half of all new mothers breastfeed, but it is unknown how breastfeeding is pursued in systemic lupus erythematosus (SLE; lupus) patients. We sought to determine the rate of breastfeeding and the factors influencing this among women with lupus. In addition, we reassessed the current safety data in lactation of lupus medications. Data were collected from lupus patients enrolled in a prospective registry who fulfilled the 2012 SLICC criteria, had a live birth, and for whom postpartum breastfeeding status was known. Data included physician assessments of lupus activity and medications, breastfeeding intentions during pregnancy and practice following pregnancy. The safety of medications in breastfed infants was assessed through a comprehensive review of LactMed, a national database about medications in lactation. A total of 51 pregnancies in 84 women with lupus were included in the study. Half of the lupus patients (n = 25, 49%) chose to breastfeed. The rate of breastfeeding was not significantly affected by socioeconomic factors. In contrast, low postpartum lupus activity, term delivery, and a plan to breastfeed early in pregnancy were significantly associated with breastfeeding in lupus patients. In reviewing the most up-to-date data, the majority of lupus medications appear to have very minimal transfer into breast milk and are likely compatible with breastfeeding. Half of women with lupus breastfed and most desire to breastfeed. Hydroxychloroquine, azathioprine, methotrexate, and prednisone have very limited transfer into breast milk and may be continued while breastfeeding. © The Author(s) 2016.

  11. Prospective Validation of the Lupus Impact Tracker: A Patient-Completed Tool for Clinical Practice to Evaluate the Impact of Systemic Lupus Erythematosus.

    Science.gov (United States)

    Jolly, Meenakshi; Kosinski, Mark; Garris, Cindy P; Oglesby, Alan K

    2016-06-01

    To evaluate the reliability, validity, responsiveness, and utility of the Lupus Impact Tracker (LIT). This was a prospective longitudinal study with 20 North American sites participating. Consenting patients completed the LIT, Medical Outcomes Study Short Form 36 (version 2), Patient Health Questionnaire 9 (PHQ-9), LupusQoL, and patient LIT feedback questionnaire. Rheumatologists completed the Safety of Estrogens in Lupus Erythematosus National Assessment (SELENA) version of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, and physician LIT feedback questionnaire. The reliability, convergent validity, construct validity, and responsiveness of the LIT were evaluated. Of the 325 SLE patients enrolled, 90% were female, 53% were white, and 33% were African American. Their mean age was 42 years. The mean ± SD baseline physician's global assessment and total SELENA-SLEDAI scores were 1.04 ± 0.8 and 4.28 ± 3.8, respectively, while 3-month scores were 0.94 ± 0.73 and 4.09 ± 3.79, respectively. Internal consistency reliability was high (>0.9) at both visits. LIT scores correlated highly with other measures of patient-reported outcomes, and construct validity was established against clinical measures. The LIT was highly responsive to patient-reported changes in SLE health status; however, LIT scores were not as responsive to changes in the SELENA-SLEDAI score. The majority of patients and physicians found LIT to be acceptable and feasible to administer in a clinical setting. The LIT is a reliable and valid instrument for assessing the impact of SLE on patients and captures unique and important information not included in physician assessments of disease. It may be useful in clinical practice to facilitate communication between the physician and the patient and enable efficient incorporation of the patient's perspective in disease

  12. Cardiovascular manifestations in systemic lupus erythematosus. Prospective study of 100 patients.

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    Badui, E; Garcia-Rubi, D; Robles, E; Jimenez, J; Juan, L; Deleze, M; Diaz, A; Mintz, G

    1985-07-01

    One hundred consecutive female patients with active systemic lupus erythematosus (SLE) were studied from the cardiovascular point of view by means of non invasive methods. Seventy percent of the cases presented some type of cardiovascular anomaly. Seventy four percent of the resting electrocardiograms were abnormal as well as 72% of the M mode echocardiograms and 55% of the cardiac X ray series. The most frequent observed complications were: pericarditis and or pericardial effusion (39%), arterial hypertension (22%), ischemic heart disease (16%), myocarditis (14%), congestive heart failure (10%), pulmonary hypertension (9%), valvular heart disease (9%), pleural effusion (7%) and cerebro vascular accident (3%). We analyzed each one of these complications and found of special interest the high incidence of ischemic heart disease which is more frequent than has been hitherto reported. Ischemic heart disease was observed in two types of patients: a) Those with long term steroid therapy. In these, the mechanism seems to be an atherosclerotic disease probably induced by the chronic use of steroids. The management of these cases do not differ from other types of coronary heart disease due to atherosclerosis. b) Those with frank episodes of vasculitis in whom the basic mechanism is an inflammatory process of the coronary arteries and its treatment is fundamentally that of the vasculitis. We consider necessary to study routinely all patients with SLE through non invasive cardiological methods.

  13. Systemic Lupus Erythematosus and Pregnancy.

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    Lateef, Aisha; Petri, Michelle

    2017-05-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with a strong female predilection. Pregnancy remains a commonly encountered but high-risk situation in this setting. Both maternal and fetal mortality and morbidity are still significantly increased despite improvements in outcomes. Maternal morbidity includes higher risk of disease flares, preeclampsia and other pregnancy-related complications. Fetal issues include higher rates of preterm birth, intrauterine growth restriction, and neonatal lupus syndromes. Treatment options during pregnancy are also limited and maternal benefit has to be weighed against fetal risk. A coordinated approach, with close monitoring by a multidisciplinary team, is essential for optimal outcomes.

  14. Fatigue in systemic lupus erythematosus.

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    Ahn, Grace E; Ramsey-Goldman, Rosalind

    2012-04-01

    Systemic lupus erythematosus is a chronic inflammatory autoimmune disease often characterized by fatigue, with significant effects on physical functioning and wellbeing. The definition, prevalence and factors associated with fatigue, including physical activity, obesity, sleep, depression, anxiety, mood, cognitive dysfunction, vitamin D deficiency/insufficiency, pain, effects of medications and comorbidities, as well as potential therapeutic options of fatigue in the systemic lupus erythematosus population are reviewed. Due to variability in the reliability and validity of various fatigue measures used in clinical studies, clinical trial data have been challenging to interpret. Further investigation into the relationships between these risk factors and fatigue, and improved measures of fatigue, may lead to an improvement in the management of this chronic inflammatory disease.

  15. Current Status of Treatment for Systemic Lupus Erythematosus in China and Its Prospect

    Institute of Scientific and Technical Information of China (English)

    杨虎天; 魏强华

    2002-01-01

    @@ CURRENT OVERALL LEVEL IN CHINA OF SLE TREATMENTSystemic lupus erythematosus (SLE) is a kind of most complicated auto-immune disease and its etiology is still not clear so far. There are at least one million patients in China. SLE was taken as an “inc urable disease” in the fifties, but recently th e understanding of SLE got gradually elevated, and the efficacy of treatment won further progresses, therefore prognosis was obviously improved. According to the re sult of an 18-year follow-up of 50 SLE patients treated by Shanghai Renji Hospital in 1999, their 5-, 10-, 15- and 18-year survival rate reached 98%, 84%, 76% and 70% respectively, and the international progressive level has been reach ed (1) , it was also discovered that 13 cases (26%) has stopped medication for 12 years (2-17 years) and did not have manifestation of SLE activation. SLE usually occurred in child-bearing women, hence pregnancy complicated with SLE was also a problem fr e quently encountered. The most frequent thought then was to take measures to stop pregnancy to save the life of the women, while at present, if a woman took small dosage of prednisone without the activation of SLE over 12 months, pregnancy can be considered; for those already pregnant women, dosage of pre dnisone could be inc reased under the guidance of rheumatologic and gynecologic specialists; during labor corticosterone (CS) intravenous dripping and other measures were added. Eighty-one new born babies by 80 cases of pregnancy complicated with SLE, have been delivered with both mot her and baby surviving: an inspiring good outcome (2) .

  16. Pathophysiology of cutaneous lupus erythematosus

    OpenAIRE

    Achtman, Jordan C; Werth, Victoria P.

    2015-01-01

    The pathophysiology of cutaneous lupus erythematosus (CLE) encompasses the complex interactions between genetics, the environment, and cells and their products. Recent data have provided enhanced understanding of these interactions and the mechanism by which they cause disease. A number of candidate genes have been identified which increase the risk of developing CLE. Ultraviolet radiation, the predominant environmental exposure associated with CLE, appears to initiate CLE lesion formation by...

  17. Pain and systemic lupus erythematosus

    OpenAIRE

    2014-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by heterogeneous clinical manifestations involving virtually the entire body. The pain in SLE can have different causes. The SLE classification criteria include mainly the musculoskeletal manifestations of pain, which are commonly reported as initial symptoms of SLE, such as arthralgia, arthritis and/or myalgia. Chronic widespread pain, which is typical of fibromyalgia (FM), is frequently associated with SLE. The aim of...

  18. Immunogenetics of cutaneous lupus erythematosus.

    Science.gov (United States)

    Hersh, Aimee O; Arkin, Lisa M; Prahalad, Sampath

    2016-08-01

    Systemic lupus erythematosus (SLE) is the prototypic autoimmune condition, often affecting multiple organ systems, including the skin. Cutaneous lupus erythematosus (CLE) is distinct from SLE and may be skin limited or associated with systemic disease. Histopathologically, the hallmark of lupus-specific manifestations of SLE and CLE is an interface dermatitis. The cause of SLE and CLE is likely multifactorial and may include shared genetic factors. In this review, we will discuss the genetic findings related to the cutaneous manifestations of SLE and isolated CLE, with a particular focus on the lupus-specific CLE subtypes. Several major histocompatibility complex and nonmajor histocompatibility complex genetic polymorphisms have been identified which may contribute to the cutaneous manifestations of SLE and to CLE. Most of these genetic variants are associated with mechanisms attributed to the pathogenesis of SLE, including pathways involved in interferon and vitamin D regulation and ultraviolet light exposure. Although there is overlap between the genetic factors associated with SLE and CLE, there appear to be unique genetic factors specific for CLE. Improved understanding of the genetics of CLE may lead to the creation of targeted therapies, improving outcomes for patients with this challenging dermatologic condition.

  19. Breast Cancer in Systemic Lupus Erythematosus

    DEFF Research Database (Denmark)

    Tessier Cloutier, B; Clarke, A E; Ramsey-Goldman, R

    2013-01-01

    Evidence points to a decreased breast cancer risk in systemic lupus erythematosus (SLE). We analyzed data from a large multisite SLE cohort, linked to cancer registries.......Evidence points to a decreased breast cancer risk in systemic lupus erythematosus (SLE). We analyzed data from a large multisite SLE cohort, linked to cancer registries....

  20. Drug-induced subacute cutaneous lupus erythematosus.

    Science.gov (United States)

    Callen, J P

    2010-08-01

    Subacute cutaneous lupus erythematosus (SCLE) is a subset of cutaneous lupus erythematosus with unique immunologic and clinical features. The first description dates back to 1985 when a series of five patients were found to have hydrochlorothiazide-induced SCLE. Since that time, at least 40 other drugs have been implicated in the induction of SCLE.

  1. Systemic Lupus Erythematosus and Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Aleksandra Plavsic

    2014-09-01

    Full Text Available Antiphospholipid syndrome is an autoimmune disorder defined as association of vascular thrombosis and/or pregnancy complications with presence of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin and anti-β2 glycoprotein I. It is the most common cause of acquired thrombophilia, and can occur as an independent entity or in relation with other diseases, especially systemic lupus erythematosus. Presence of antiphospholipid syndrome in systemic lupus erythematosus is additional vaso occlusive factor in already present inflammation, bringing further risk for thrombotic events. Clinical and serological manifestations of antiphospholipid syndrome and systemic lupus erythematosus are very similar, so possible connection for these two autoimmune disorders is assumed.

  2. Malignancies in systemic lupus erythematosus

    Science.gov (United States)

    Kale, Mruganka; Ramsey-Goldman, Rosalind; Gordon, Caroline; Clarke, Ann E; Bernatsky, Sasha

    2013-01-01

    The purpose of this review is to underline important advancements in the understanding of cancer risks in systemic lupus erythematosus (SLE). In SLE, there is an increased risk of specific kinds of malignancy. For example, the risk of non-Hodgkin’s lymphoma is increased several-fold in SLE versus the general population. In addition, heightened risks for lung cancer, thyroid cancer and cervical dysplasia in SLE have been found. Some have postulated that immunosuppressive drugs play a role, as well as other important mediators, such as lupus disease activity itself. One new frontier being explored is the significant finding of a decreased risk of certain nonhematologic cancers (e.g., breast, ovarian, endometrial and prostate) in SLE. The reasons for this are currently under study. PMID:19643208

  3. Dyslipidemia in systemic lupus erythematosus.

    Science.gov (United States)

    Szabó, Melinda Zsuzsanna; Szodoray, Peter; Kiss, Emese

    2017-02-07

    Cardiovascular disease is one of the major causes of morbidity and mortality in patients with systemic lupus erythematosus (SLE). Accelerated atherosclerosis is related to traditional (age, hypertension, diabetes mellitus, dyslipidemia, obesity, smoking, and positive family history) and non-traditional, disease-related factors. Traditional risk factors are still more prominent in patients with lupus, as both hypertension and hypercholesterinemia were independently associated with premature atherosclerosis in several SLE cohorts. In this work, the authors summarize the epidemiology of dyslipidemia in lupus patients and review the latest results in the pathogenesis of lipid abnormalities. The prevalence of dyslipidemia, with elevations in total cholesterol (TC), low-density lipoprotein (LDL), triglyceride (TG), and apolipoprotein B (ApoB), and a reduction in low-density lipoprotein (LDL) levels are about 30% at the diagnosis of SLE rising to 60% after 3 years. Multiple pathogenetic mechanism is included, C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) can suppress HDL and increase TG, auto-antibodies can cause the injury of the endothelium, lipoprotein lipase (LPL) activity can be reduced by circulating inflammatory mediators and antibodies, and increased oxidative stress may trigger a wide range of pro-atherogenic lipid modifications. As a major risk factor, dyslipidemia should be treated aggressively to minimize the risk of atherosclerosis and cardiovascular events. Randomized controlled trials with statins are controversial in the detention of atherosclerosis progression, but can be favorable by inhibiting immune activation that is the arterial wall and by decreasing lupus activity.

  4. Occurrence of systemic lupus erythematosus in a Danish community

    DEFF Research Database (Denmark)

    Laustrup, H; Voss, A; Green, A

    2009-01-01

    Objectives: To determine the prevalence and annual incidence of definite systemic lupus erythematosus (D-SLE) and incomplete SLE (I-SLE) in a community-based lupus cohort of predominantly Nordic ancestry in an 8-year prospective study from 1995 to 2003, and also to calculate the annual transition......-years at risk [95% confidence interval (CI) 1.44-7.55]. Conclusions: Denmark is a low-incidence lupus area but lupus prevalence is increasing slowly. I-SLE is a disease variant that may eventually convert into D-SLE....

  5. Biomarkers in systemic lupus erythematosus: challenges and prospects for the future

    Science.gov (United States)

    Kao, Amy H.; Manzi, Susan; Ahearn, Joseph M.

    2013-01-01

    The search for lupus biomarkers to diagnose, monitor, stratify, and predict individual response to therapy is currently more intense than ever before. This effort is essential for several reasons. First, epidemic overdiagnosis and underdiagnosis of lupus, even by certified rheumatologists, leads to errors in therapy with concomitant side effects which may be more serious than the disease itself. Second, identification of lupus flares remains as much an art as it is a science. Third, the capacity to stratify patients so as to predict those who will develop specific patterns of organ involvement is not currently possible but would potentially lead to preventive therapeutic strategies. Fourth, only one new drug for the treatment of lupus has been approved by the US Food and Drug Administration in over 50 years. A major obstacle in this pipeline is the dearth of biomarkers available to prove a patient has responded to an experimental therapeutic intervention. This review will summarize the challenges faced in the discovery and validation of lupus biomarkers, the most promising lupus biomarkers identified to date, and the promise of future directions. PMID:23904865

  6. Lupus erythematosus panniculitis: A case report

    Directory of Open Access Journals (Sweden)

    Besma Ben Dhaou

    2017-07-01

    Full Text Available Lupus erythematosus panniculitis (LEP, an uncommon variant in the clinicopathological spectrum of lupus erythematosus (LE, is rare. There are only a few reported series of patients with this condition; none in individuals of North African ancestry. LEP is characterized by inflammation of the deep dermis and subcutaneous tissue. It usually consists of nodules and hardened subcutaneous plaques on the forehead, cheeks, proximal extremities, and buttocks. Leg involvement is rare and can lead to misdiagnosis. A case of LEP, with unusual involvement of legs, is reported in a 40-year-old woman who had the diagnosis of systemic lupus erythematosus (SLE four years ago.

  7. Manifestasi Systematic Lupus Erythematosus pada Paru

    OpenAIRE

    Helmi, Luthfi

    2010-01-01

    Systemic lupus erythematosus (SLE) is a systematical chronic inflammatory disease occurred as the effect from the auto-immune process. The most frequent to the death is infection. Lupus may attack all ages and it is mostly on women. SLE also attack some parts of the body and it is most dangerous on the heart, joint, skin, lung, blood gland, liver, and nervous system. Lupus can be treated simtosisly and it is particularly with corticosteroids and immunosuppressant. The type of the lupus shows...

  8. [Management of systemic lupus erythematosus].

    Science.gov (United States)

    Aringer, M; Schneider, M

    2016-11-01

    In the last few decades a number of small, often largely unrecognized steps have fundamentally changed the management of systemic lupus erythematosus (SLE). The current goal is to stop all disease activity without long-term use of more than 5 mg prednisolone per day. Remission, i.e. absence of activity in the SLE activity score of choice, is the defined target in the treat to target approach. The essential basic measures include life-long hydroxychloroquine as well as protection from sunlight (UV) and vitamin D substitution. Patients suffering from SLE need more vaccinations than the healthy population and control of risk factors for atherosclerosis is critical for long-term survival. Methotrexate is on par with azathioprine. If disease activity cannot be controlled in this way, belimumab is an approved therapeutic option. Cyclophosphamide is still used but only in life-threatening situations, such as lupus nephritis or central nervous system (CNS) vasculitis and in drastically reduced doses. Alternatively, off-label mycophenolate mofetil (MMF) can be used particularly for lupus nephritis and off-label rituximab in refractory disease courses. Numerous novel approaches are being tested in controlled trials and it is hoped that new drugs will be available for SLE patients within a few years.

  9. Neonatal lupus erythematosus in a Nigerian infant

    African Journals Online (AJOL)

    2017-06-15

    Jun 15, 2017 ... mon condition in children and usually manifests as the cutaneous form in ... area with a significant hair loss on the affected areas. ... totosis with follicular plugging. ... ous manifestations of lupus erythematosus seen in adults.

  10. Systemic Lupus Erythematosus Presenting as Acute Adrenal ...

    African Journals Online (AJOL)

    hanumantp

    Systemic lupus erythematosus (SLE) is the prototypic autoimmune disease ... association with diverse clinical manifestations encompassing almost all organ .... acute adrenal insufficiency. The diagnosis of Addison's disease in our patient was.

  11. A unique case of systemic lupus erythematosus.

    Science.gov (United States)

    Rafiq, Muhammad Khizar

    2009-01-01

    An 18-year-old Asian girl was referred to the nephrology unit with rapidly progressive renal failure. At the age of 15 she was diagnosed as having systemic lupus erythematosus but had defaulted treatment. Her renal functions improved with cyclophosphamide pulse treatment but she continued to have central nervous system vasculitis, gastrointestinal vasculitis and opportunistic infections making her a unique and challenging case of systemic lupus erythematosus.

  12. Pain and systemic lupus erythematosus.

    Science.gov (United States)

    Di Franco, M; Guzzo, M P; Spinelli, F R; Atzeni, F; Sarzi-Puttini, P; Conti, F; Iannuccelli, C

    2014-06-06

    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by heterogeneous clinical manifestations involving virtually the entire body. The pain in SLE can have different causes. The SLE classification criteria include mainly the musculoskeletal manifestations of pain, which are commonly reported as initial symptoms of SLE, such as arthralgia, arthritis and/or myalgia. Chronic widespread pain, which is typical of fibromyalgia (FM), is frequently associated with SLE. The aim of this review is to describe widespread pain and fatigue in SLE, and the association of SLE and FM. Although secondary FM is not correlated with the disease activity, it may interfere with the daily activities of SLE patients. Therefore it is necessary to identify its symptoms and treat them promptly to improve the quality of life of patients. In conclusion, it is essential to identify the origin of pain in SLE in order to avoid dangerous over-treatment in patients with co-existing widespread pain and FM.

  13. Pain and systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    M. Di Franco

    2014-06-01

    Full Text Available Systemic lupus erythematosus (SLE is an autoimmune disease characterized by heterogeneous clinical manifestations involving virtually the entire body. The pain in SLE can have different causes. The SLE classification criteria include mainly the musculoskeletal manifestations of pain, which are commonly reported as initial symptoms of SLE, such as arthralgia, arthritis and/or myalgia. Chronic widespread pain, which is typical of fibromyalgia (FM, is frequently associated with SLE. The aim of this review is to describe widespread pain and fatigue in SLE, and the association of SLE and FM. Although secondary FM is not correlated with the disease activity, it may interfere with the daily activities of SLE patients. Therefore it is necessary to identify its symptoms and treat them promptly to improve the quality of life of patients. In conclusion, it is essential to identify the origin of pain in SLE in order to avoid dangerous over-treatment in patients with co-existing widespread pain and FM.

  14. OSTEOPOROSIS IN SYSTEMIC LUPUS ERYTHEMATOSUS

    Directory of Open Access Journals (Sweden)

    N V Seredavkina

    2009-01-01

    Full Text Available Patients with systemic lupus erythematosus (SLE form a high risk group osteoporosis (OP. Its main causes are autoimmune inflammation, concomitant pathology, and their treatment. When OP occurs in SLE, bone mass loss is shown to occur early and is associated with the use of glucocorticosteroids (GC. To prevent OP, all patients with SLE should modify their lifestyle. To verify bone changes, densitometry is performed in patients who have risk factors of OP and/or a menopause. Calcium preparations and vitamin D are used to prevent OP; bisphosphonates that significantly reduce the risk of fractures of the vertebral column and femoral neck are employed for therapy of OP. A SLE patient with gluco-corticoid-induced OP and a good effect of bisphophonate treatment is described.

  15. A 12-year retrospective review of bullous systemic lupus erythematosus in cutaneous and systemic lupus erythematosus patients.

    Science.gov (United States)

    Chanprapaph, K; Sawatwarakul, S; Vachiramon, V

    2017-01-01

    Objective The aim of this study was to investigate the clinical features, laboratory findings, systemic manifestations, treatment and outcome of patients with bullous systemic lupus erythematosus in a tertiary care center in Thailand. Methods We performed a retrospective review from 2002 to 2014 of all patients who fulfilled the diagnostic criteria for bullous systemic lupus erythematosus to evaluate for the clinical characteristics, extracutaneous involvement, histopathologic features, immunofluorescence pattern, serological abnormalities, internal organ involvement, treatments and outcome. Results Among 5149 patients with cutaneous lupus erythematosus and/or systemic lupus erythematosus, 15 developed vesiculobullous lesions. Ten patients had validation of the diagnosis of bullous systemic lupus erythematosus, accounting for 0.19%. Bullous systemic lupus erythematosus occurred after the diagnosis of systemic lupus erythematosus in six patients with a median onset of 2.5 months (0-89). Four out of 10 patients developed bullous systemic lupus erythematosus simultaneously with systemic lupus erythematosus. Hematologic abnormalities and renal involvement were found in 100% and 90%, respectively. Polyarthritis (40%) and serositis (40%) were less frequently seen. Systemic corticosteroids, immunosuppressants, antimalarials and dapsone offered resolution of cutaneous lesions. Conclusion Bullous systemic lupus erythematosus is an uncommon presentation of systemic lupus erythematosus. Blistering can occur following or simultaneously with established systemic lupus erythematosus. We propose that clinicians should carefully search for systemic involvement, especially hematologic and renal impairment, in patients presenting with bullous systemic lupus erythematosus.

  16. Proton pump inhibitor-induced subacute cutaneous lupus erythematosus

    DEFF Research Database (Denmark)

    Sandholdt, L H; Laurinaviciene, R; Bygum, Anette

    2014-01-01

    Drug-induced subacute cutaneous lupus erythematosus (SCLE) has been known in the literature since 1985 and is increasingly recognized.......Drug-induced subacute cutaneous lupus erythematosus (SCLE) has been known in the literature since 1985 and is increasingly recognized....

  17. ACUTE RESPIRATORY DISEASE AS THE DEBUT OF SYSTEMIC LUPUS ERYTHEMATOSUS

    Directory of Open Access Journals (Sweden)

    A. Yu. Ischenko

    2015-01-01

    Full Text Available Systemic lupus erythematosus — a chronic autoimmune disease that is often associated with infectious processes. The paper presents two clinical cases of systemic lupus erythematosus , debuted with acute respiratory infection.

  18. Incidence of systemic lupus erythematosus and lupus nephritis in Denmark

    DEFF Research Database (Denmark)

    Hermansen, Marie-Louise F.; Lindhardsen, Jesper; Torp-Pedersen, Christian

    2016-01-01

    Objective. To determine the incidence of systemic lupus erythematosus (SLE) and SLE with concomitant or subsequent lupus nephritis (LN) in Denmark during 1995.2011, using data from the Danish National Patient Registry (NPR).  Methods. To assess the incidence of SLE, we identified all persons aged...

  19. Childhood-onset bullous systemic lupus erythematosus.

    Science.gov (United States)

    Lourenço, D M R; Gomes, R Cunha; Aikawa, N E; Campos, L M A; Romiti, R; Silva, C A

    2014-11-01

    Bullous systemic lupus erythematosus has rarely been described in pediatric lupus population and the real prevalence of childhood-onset bullous systemic lupus erythematosus has not been reported. From January 1983 to November 2013, 303 childhood-onset SLE (c-SLE) patients were followed at the Pediatric Rheumatology Unit of the Childreńs Institute of Hospital das Clínicas da Faculdade de Medicina Universidade da Universidade de São Paulo, three of them (1%) diagnosed as childhood-onset bullous systemic lupus erythematosus. All three cases presented tense vesiculobullous lesions unassociated with lupus erythematosus lesions, with the median duration of 60 days (30-60). All patients fulfilled bullous systemic lupus erythematosus criteria. Two had nephritis and serositis and presented specific autoantibodies. The histological pattern demonstrated subepidermal blisters with neutrophils-predominant infiltrates within the upper dermis. Direct immunofluorescence (DIF) showed deposits of IgG and complement along the epidermal basement membrane, in the presence or absence of IgA and/or IgM. A positive indirect immunofluorescence on salt-split skin demonstrating dermal binding was observed in two cases. All of them had moderate/severe disease activity at diagnosis with median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) of 18 (14-24). Two patients received dapsone and one with severe nephritis received immunosuppressive drugs. In conclusion, in the last 30 years the prevalence of bullous lupus in childhood-onset lupus population was low (1%) in our tertiary University Hospital. A diagnosis of SLE should always be considered in children with recurrent tense vesiculobullous lesions with or without systemic manifestations. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  20. Periorbital mucinosis: a variant of cutaneous lupus erythematosus?

    Science.gov (United States)

    Morales-Burgos, Adisbeth; Sánchez, Jorge L; Gonzalez-Chávez, José; Vega, Janelle; Justiniano, Hildamari

    2010-04-01

    Lupus erythematosus has a wide spectrum of cutaneous manifestations, including periorbital mucinosis. We report 3 cases of periorbital mucinosis occurring in association with other cutaneous signs of lupus erythematosus. Based on a review of the literature, periorbital mucinosis is a rare and not widely recognized clinical manifestation of the disease. Although unusual, familiarity with periorbital mucinosis as a manifestation of lupus erythematosus broadens our understanding of these entities and expands the spectrum of cutaneous lupus erythematosus.

  1. Lenalidomide for refractory cutaneous manifestations of pediatric systemic lupus erythematosus.

    Science.gov (United States)

    Wu, E Y; Schanberg, L E; Wershba, E C; Rabinovich, C E

    2017-05-01

    Objective Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide's efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. Methods We performed a retrospective chart review of 10 adolescents who received lenalidomide for recalcitrant cutaneous lupus erythematosus. Information was gathered at drug initiation and 6-month follow-up. The Wilcoxon matched-pairs signed-rank test was used to assess change in quantitative parameters of disease activity. Results Nine subjects were girls and six were African-American. Indications for lenalidomide treatment included alopecia, nasal and oral ulcers, extensive malar rash, discoid lesions, bullous lesions, panniculitis, cutaneous vasculitis, and Raynaud's phenomenon with digital ulcerations. Within 6 months, all patients demonstrated complete or near resolution based on physician report. Prednisone dose decreased from a mean 23.5 mg (SD± 13.3) to 12.25 mg (SD± 9.2) ( P= 0.008). Sedimentation rate decreased from a mean 29 mm/hour (SD± 31.5) to 17 mm/hour (SD± 18.1) ( P= 0.004). Lenalidomide was well tolerated. Conclusion Lenalidomide is an effective and safe treatment for a spectrum of dermatological conditions in pediatric systemic lupus erythematosus. Its use may allow a reduction in prednisone dose and decreased disfigurement. Prospective study is needed to clarify lenalidomide's role in treating cutaneous manifestations of systemic lupus erythematosus.

  2. 75 FR 35492 - Guidance for Industry on Lupus Nephritis Caused By Systemic Lupus Erythematosus-Developing...

    Science.gov (United States)

    2010-06-22

    ... availability of a guidance for industry entitled ``Lupus Nephritis Caused By Systemic Lupus Erythematosus... nephritis (LN) caused by systemic lupus erythematosus (SLE). This guidance finalizes the parts of the draft guidance entitled ``Systemic Lupus Erythematosus--Developing Drugs for Treatment'' (the draft guidance...

  3. Clinical Profile of Patients with Systemic Lupus Erythematosus

    OpenAIRE

    Paudyal, B; M Gyawalee

    2012-01-01

    Introduction: Clinical features of Systemic lupus erythematosus vary between different parts of the World; however, this information on Nepalese Sytemic lupus erythematosus does not exist. Methods: Patients with Systemic lupus erythematosus fulfilling the American College of Rheumatology criteria treated in Patan Hospital were studied by means of retrospective review of their case records. The results were compared with various regional and international studies. Res...

  4. Clinical characteristics during diagnosis of a prospective cohort of patients with systemic lupus erythematosus treated in Spanish Departments of Internal Medicine: The RELES study.

    Science.gov (United States)

    Canora, J; García, M; Mitjavila, F; Espinosa, G; Suárez, S; González-León, R; Sopeña, B; Boldova, R; Castro, A; Ruiz-Irastorza, G

    Patient registries are useful tools for assessing rare diseases. Our objective is to present the Spanish registry of patients with systemic lupus erythematosus (Registro español de pacientes con lupus eritematoso sistémico, RELES). RELES was started in 2008 as an observational, prospective, multicentre cohort registry that included patients from the time they were diagnosed. The registry's objective is to analyse the incidence and noninflammatory complications of systemic lupus erythematosus (SLE). The departments of internal medicine of 38 Spanish hospitals participate in this registry. A total of 298 patients with a mean age of 40.8±15.7 years were included, 88.9% of whom were women and 85.6% of whom were white. In the first visit, there was a predominance of joint manifestations (74.5%). One hundred and seventy-seven patients (59.4%) were positive for anti-native DNA. In these patients, there was a higher rate of lupus nephritis (26.7% vs. 14%, p=.009; relative risk [RR], 1.33), haemolytic anaemia (13.6% vs. 4.1%, p=.07; RR, 1.46) and lymphopenia (55.4% vs. 43.8%, p=.05; RR, 1.21). The median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2K) score was 9.64 points (interquartile range, 4-13). The patients treated with antimalarial drugs before the diagnosis of SLE had a median SLEDAI score in the first visit of 5, compared with 8 for those who were not treated with these drugs (p=.02). RELES constitutes the first Spanish patient cohort with SLE recorded from the time of the diagnosis. The presence of anti-DNA has been related to severe manifestations such as nephritis and haemolytic anaemia. Treatment with antimalarial drugs before the diagnosis was associated with less active disease at the initial presentation. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.

  5. Humor in systemic lupus erythematosus.

    Science.gov (United States)

    Moura, Cristiano S; Li, Rui; Lawrie, Sarah; Bar-Or, Amit; Clarke, Ann E; Da Costa, Deborah; Banerjee, Devi; Bernatsky, Sasha; Lee, Jennifer L; Pineau, Christian A

    2015-03-01

    Humor has neurophysiological effects influencing the release of cortisol, which may have a direct impact on the immune system. Laughter is associated with a decreased production of inflammatory cytokines both in the general population and in rheumatoid arthritis (RA). Our objective was to explore the effects of humor on serum cytokines [particularly interleukin-6 (IL-6)] and cortisol levels in systemic lupus erythematosus (SLE), after a standard intervention (120 min of visual comedy). We enrolled 58 females with SLE from consecutive patients assessed in the Montreal General Hospital lupus clinic. The subjects who consented to participate were randomized in a 1:1 ratio to the intervention (watching 120 min of comedy) or control group (watching a 120 min documentary). Measurements of cytokine and serum cortisol levels as well as 24-h urine cortisol were taken before, during, and after the interventions. We compared serum cytokine levels and serum and 24-h urine cortisol levels in the humor and control groups and performed regression analyses of these outcomes, adjusting for demographics and the current use of prednisone. There were no significant differences between the control and humor groups in demographics or clinical variables. Baseline serum levels of IL-6, IL-10, tumor necrosis factor-alpha, and B-cell activating factor were also similar in both groups. There was no evidence of a humor effect in terms of decreasing cytokine levels, although there was some suggestion of lowered cortisol secretion in the humor group based the 24-h urinary cortisol levels in a subgroup. In contrast to what has been published for RA, we saw no clear effects of humor in altering cytokine levels in SLE, although interesting trends were seen for lower cortisol levels after humor intervention compared with the control group.

  6. Drug-induced cutaneous lupus erythematosus

    DEFF Research Database (Denmark)

    Laurinaviciene, Rasa; Sandholdt, Linda Holm; Bygum, Anette

    2017-01-01

    BACKGROUND: An increasing number of drugs have been linked to drug-induced subacute cutaneous lupus erythematosus (DI-SCLE). The recognition and management of DI-SCLE can be challenging, as the condition may be triggered by different classes of drugs after variable lengths of time. OBJECTIVES......: To determine the proportion of patients with cutaneous lupus erythematosus (CLE) whose drugs are an inducing or aggravating factor. MATERIALS & METHODS: We conducted a retrospective chart review of patients diagnosed with CLE at a dermatological department over a 21-year period. We registered clinical......, serological, and histological data with a focus on drug intake. RESULTS: Of 775 consecutive patients with a diagnosis of lupus erythematosus (LE) or suspected LE, a diagnosis of CLE could be confirmed in 448 patients. A total of 130 patients had a drug intake that could suggest DI-SCLE. In 88 cases, a drug...

  7. Vasculitis in systemic lupus erythematosus.

    Science.gov (United States)

    Barile-Fabris, L; Hernández-Cabrera, M F; Barragan-Garfias, J A

    2014-01-01

    Systemic lupus erythematosus (SLE) is a complex heterogeneous autoimmune disease with a wide variety of clinical and serological manifestations that may affect any organ. Vasculitis prevalence in SLE is reported to be between 11% and 36%. A diverse clinical spectrum, due to inflammatory involvement of vessels of all sizes, is present. Even though cutaneous lesions, representing small vessel involvement, are the most frequent, medium and large vessel vasculitis may present with visceral affection, with life-threatening manifestations such as mesenteric vasculitis, pulmonary hemorrhage, or mononeuritis multiplex, with detrimental consequences. Early recognition and an appropriate treatment are crucial. Recent studies have shown that vasculitis in patients with SLE may present different clinical forms based on the organ involved and the size of the affected vessel. It is noteworthy that the episodes of vasculitis are not always accompanied by high disease activity. Recent articles on this topic have focused on new treatments for the control of vascular disease, such as biological therapies such as Rituximab and Belimumab, among others.

  8. Periodontitis and systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Manuela Rubim Camara Sete

    2016-04-01

    Full Text Available ABSTRACT A large number of studies have shown a potential association between periodontal and autoimmune diseases, such as rheumatoid arthritis and systemic lupus erythematosus (SLE. Similar mechanisms of tissue destruction concerning periodontitis and other autoimmune diseases have stimulated the study of a possible relationship between these conditions. This study aims to review the literature about this potential association and their different pathogenic mechanisms. Considering that periodontal disease is a disease characterized by inflammation influenced by infectious factors, such as SLE, it is plausible to suggest that SLE would influence periodontal disease and vice-versa. However, this issue is not yet fully elucidated and several mechanisms have been proposed to explain this association, as deregulation mainly in innate immune system, with action of phagocytic cells and proinflammatory cytokines such as IL-1β and IL-18 in both conditions’ pathogenesis, leading to tissue destruction. However, studies assessing the relationship between these diseases are scarce, and more studies focused on common immunological mechanisms should be conducted to further understanding.

  9. The epidemiology of systemic lupus erythematosus.

    Science.gov (United States)

    Jiménez, Sònia; Cervera, Ricard; Font, Josep; Ingelmo, Miguel

    2003-08-01

    Systemic lupus erythematosus (SLE) is the most diverse of the autoimmune diseases because it may affect any organ of the body and display a broad spectrum of clinical and immunological manifestations. Although previously considered a rare disease, SLE now appears to be relatively common in certain groups of the population. This is probably due to the development of several immunological tests that have allowed the description of many atypical or benign cases that otherwise might not be diagnosed. Furthermore, with the introduction since 1982 of a set of more sensitive criteria for SLE classification, more cases can nowadays be detected. In the present article, we review the most important data regarding the incidence and prevalence of this disease in the general population, the epidemiologic information on the patterns of disease expression in specific subsets and the studies on mortality in SLE. An important amount of information comes from the data obtained from the "Euro-Lupus Cohort," a series of 1000 patients with SLE from several European countries that have been followed prospectively since 1991.

  10. Therapeutic strategies evaluated by the European Society of Cutaneous Lupus Erythematosus (EUSCLE) Core Set Questionnaire in more than 1000 patients with cutaneous lupus erythematosus

    DEFF Research Database (Denmark)

    Sigges, Johanna; Biazar, Cyrus; Landmann, Aysche

    2013-01-01

    The aim of this prospective, cross-sectional, multicentre study performed by the European Society of Cutaneous Lupus Erythematosus (EUSCLE) was to investigate different therapeutic strategies and their efficacies in cutaneous lupus erythematosus (CLE) throughout Europe. Using the EUSCLE Core Set...... Questionnaire, topical and systemic treatment options were analysed in a total of 1002 patients (768 females and 234 males) with different CLE subtypes. The data were correlated with the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and the criteria of the American College...... of Rheumatology (ACR) for the classification of systemic lupus erythematosus. Sunscreens were applied by 84.0% of the study cohort and showed a high efficacy in preventing skin lesions in all disease subtypes, correlating with a lower CLASI activity score. Topical steroids were used in 81.5% of the patients...

  11. Relationship between health-related quality of life, disease activity and disease damage in a prospective international multicenter cohort of childhood onset systemic lupus erythematosus patients

    DEFF Research Database (Denmark)

    Moorthy, L N; Baldino, M E; Kurra, V;

    2017-01-01

    Previously, we described associations between health-related quality of life (HRQOL) and disease-related factors among childhood onset systemic lupus erythematosus (cSLE) patients. Here we determined the relationship between HRQOL, disease activity and damage in a large prospective international...... cohort of cSLE. We compared HRQOL, disease activity and disease damage across different continents and examined the relationship between children's and parents' assessments of HRQOL. Patients with cSLE and their parents completed HRQOL measures at enrollment and ≥4 follow-up visits. Physicians assessed...... disease activity and damage. The multinational cohort ( n = 467) had relatively low disease activity and damage. Patient and parent HRQOL scores were significantly correlated. Asian and European patients had the highest HRQOL, while South and North American patients had lower HRQOL scores. Renal, CNS...

  12. Osteonecrosis in systemic lupus erythematosus.

    Science.gov (United States)

    Gontero, Romina Patricia; Bedoya, María Eugenia; Benavente, Emilio; Roverano, Susana Graciela; Paira, Sergio Oscar

    2015-01-01

    To define the proportion of osteonecrosis (ON) in our patient population with lupus and to identify factors associated with the development of ON in systemic lupus erythematosus, as well as to carry out a descriptive analysis of ON cases. Observational retrospective study of 158 patients with SLE (ACR 1982 criteria). Demographic and laboratory data, clinical manifestations, SLICC, SLEDAI, cytotoxic and steroid treatments were compared. In patients with ON, we analyzed time of disease progression and age at ON diagnosis, form of presentation, joints involved, diagnostic methods, Ficat-Arlet classification, and treatment. To compare the means, t-test or Mann-Whitney's test were employed and the cHi-2 test or Fisher's exact test, as appropriate, were used to measure the equality of proportions. ON was present in 15 out 158 patients (9.5%), 13 women and 2 men, with a mean age of 30 (r: 16-66) at diagnosis and 35 months of evolution until diagnosis (r: 1-195). Among the 15 patients, 34 joints presented ON, 23 were symptomatic and 22 were diagnosed by magnetic resonance images. Twenty-six occurred in hips (24 bilateral), 4 in knees and 4 in shoulders. In 13 patients, ON involved 2 or more joints. At onset, 28 joints were in stage i-ii, one in stage iii and 5 had no data and; in the end, 14 were in stage iii-iv, 5 in stage i-ii and 15 had no data. Twenty-nine underwent conservative treatment with rest and 8 hips required joint replacement. ON progression was associated with Cushing's syndrome (P=0.014) OR 4.16 (95% CI 1.4-12.6) and 2nd year SLICC (P=0.042). No relation with clinical manifestations, lab results, cytotoxic treatment, steroid treatment (total accumulated dose, mean daily dose and duration) metilprednisolone pulses, nor activity was found. All patients with ON received antimalarials, in contrast to 77% of those without ON. The proportion of ON was 9.5%, mainly in women, 76% in hips (26) and 92% bilaterally. They were associated significantly with Cushing

  13. Hypogammaglobulinemia in pediatric systemic lupus erythematosus.

    Science.gov (United States)

    Lim, E; Tao, Y; White, A J; French, A R; Cooper, M A

    2013-11-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease typically associated with elevated serum immunoglobulin G (IgG). Hypogammaglobulinemia in SLE patients has been attributed to immunosuppressive treatment or a transient effect associated with nephrotic syndrome. We retrospectively reviewed pediatric SLE patients from a single institution to identify patients with hypogammaglobulinemia and risk factors for hypogammaglobulinemia. A total of 116 pediatric SLE cases from 1997 to 2011 were reviewed and patients with hypogammaglobulinemia (IgG lupus nephritis at SLE diagnosis, disease activity at diagnosis, initial IgG level, and drug treatment. Eighty-six patients were included in our study, with a median age of 15 years and a median follow-up of 39.5 months. Seven percent (six of 86) of patients had hypogammaglobulinemia with a median onset of 27 months (0-72 months) after SLE diagnosis. Significant associations were noted for white race (p value 0.029), male sex (p value 0.009), and the presence of lupus nephritis at SLE diagnosis (p value 0.004). Use of immunosuppressive treatment did not show a statistical association with hypogammaglobulinemia, although two of the patients with hypogammaglobulinemia did receive rituximab. Most patients with hypogammaglobulinemia received intravenous immunoglobulin (IVIG) replacement therapy because of infections and/or concern for infection. Measurement of immunoglobulin levels during treatment in SLE could help identify patients with hypogammaglobulinemia who might require more aggressive follow-up to monitor for increased risk of infection and need for IVIG treatment. A prospective study is needed to validate associated risk factors identified in this study.

  14. Systemic lupus erythematosus. Unusual cutaneous manifestations

    NARCIS (Netherlands)

    Stockinger, T.; Richter, L.; Kanzler, M.; Melichart-Kotik, M.; Pas, H.; Derfler, K.; Schmidt, E.; Rappersberger, K.

    2016-01-01

    Various different mucocutaneous symptoms may affect up to 80 % of systemic lupus erythematosus (SLE) patients. To investigate, various unspecific, but otherwise typical clinical symptoms of skin and mucous membranes that arise in SLE patients other than those defined as SLE criteria such as butterfl

  15. Systemic lupus erythematosus : a behavioural medicine perspective

    NARCIS (Netherlands)

    Daleboudt, Gabriëlle Mathilde Nicoline

    2014-01-01

    Systemic lupus erythematosus (SLE) and its immunosuppressive treatment have a great impact on the patient’s life. Previous studies on SLE have focused on the optimisation of diagnosis and treatment. Less attention has been given to the impact of diagnosis and treatment on patients’ well-being. Accor

  16. Autoimmune hepatitis and juvenile systemic lupus erythematosus

    NARCIS (Netherlands)

    Deen, M. E. J.; Porta, G.; Fiorot, F. J.; Campos, L. M. A.; Sallum, A. M. E.; Silva, C. A. A.

    2009-01-01

    Juvenile systemic lupus erythematosus (JSLE) and autoimmune hepatitis (AIH) are both autoimmune disorders that are rare in children and have a widespread clinical manifestation. A few case reports have shown a JSLE-AIH associated disorder. To our knowledge, this is the first study that simultaneousl

  17. 77 FR 38305 - Guidance for Industry on Lupus Nephritis Caused by Systemic Lupus Erythematosus-Developing...

    Science.gov (United States)

    2012-06-27

    ... availability of a guidance entitled ``Lupus Nephritis Caused By Systemic Lupus Erythematosus--Developing... HUMAN SERVICES Food and Drug Administration Guidance for Industry on Lupus Nephritis Caused by Systemic Lupus Erythematosus--Developing Medical Products for Treatment; Withdrawal of Guidance AGENCY: Food and...

  18. Psychosis in Patients with Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Raghavendra B Nayak

    2012-01-01

    Full Text Available Neuropsychiatric manifestations in systemic lupus erythematosus (SLE are common; however, psychosis per se is bit uncommon. They may be cognitive deficit, lupus headache, psychoses, seizures, peripheral neuropathy, and cerebrovascular events. Psychiatric symptoms in SLE can be functionally independent psychiatric disorders. It can be due to drugs (steroids used for SLE or secondary to SLE because of its brain involvement, which is termed as neuropsychiatric systemic lupus erythematosus (NPSLE. No single clinical, laboratory, neuropsychological, and imaging test can be used to differentiate NPSLE from non-NPSLE patients with similar neuropsychiatric manifestations. Presently we are discussing about three cases of SLE with psychosis and which had different clinical presentation. The present reports also depict the approach to case differential diagnosis and management of the same.

  19. Recurrent podocytopathy in a patient with systemic lupus erythematosus

    Science.gov (United States)

    Paramalingam, Shereen; Wong, Daniel D; Dogra, Gursharan K; Nossent, Johannes C

    2017-01-01

    Podocytopathy in systemic lupus erythematosus is characterised by diffuse foot process effacement without significant peripheral capillary wall immune deposits as seen on electron microscopy. Lupus podocytopathy falls outside the scope of the current International Society of Nephrology and the Renal Pathology Society classification of lupus nephritis. We present a case of relapsing podocytopathy with nephrotic syndrome occurring simultaneously with two extra-renal and serological disease flares, which makes it likely that podocytopathy was related to systemic lupus erythematosus activity. This case adds to the growing body of evidence that lupus podocytopathy must be considered in the differential diagnosis of systemic lupus erythematosus patients presenting with nephrotic syndrome.

  20. Recurrent podocytopathy in a patient with systemic lupus erythematosus.

    Science.gov (United States)

    Paramalingam, Shereen; Wong, Daniel D; Dogra, Gursharan K; Nossent, Johannes C

    2017-01-01

    Podocytopathy in systemic lupus erythematosus is characterised by diffuse foot process effacement without significant peripheral capillary wall immune deposits as seen on electron microscopy. Lupus podocytopathy falls outside the scope of the current International Society of Nephrology and the Renal Pathology Society classification of lupus nephritis. We present a case of relapsing podocytopathy with nephrotic syndrome occurring simultaneously with two extra-renal and serological disease flares, which makes it likely that podocytopathy was related to systemic lupus erythematosus activity. This case adds to the growing body of evidence that lupus podocytopathy must be considered in the differential diagnosis of systemic lupus erythematosus patients presenting with nephrotic syndrome.

  1. Autologous Fat Transfer in a Patient with Lupus Erythematosus Profundus

    Directory of Open Access Journals (Sweden)

    Jimi Yoon

    2012-10-01

    Full Text Available Lupus erythematosus profundus, a form of chronic cutaneous lupus erythematosus, is a rare inflammatory disease involving in the lower dermis and subcutaneous tissues. It primarily affects the head, proximal upper arms, trunk, thighs, and presents as firm nodules, 1 to 3 cm in diameter. The overlying skin often becomes attached to the subcutaneous nodules and is drawn inward to produce deep, saucerized depressions. We present a rare case of lupus erythematosus profundus treated with autologous fat transfer.

  2. Lupus erythematosus associated with erythema multiforme: does Rowell's syndrome exist?

    Science.gov (United States)

    Shteyngarts, A R; Warner, M R; Camisa, C

    1999-05-01

    We describe a patient with lupus erythematosus who experienced an unusual erythema multiforme-like eruption suggestive of Rowell's syndrome. We compare our case and 9 other reports of lupus erythematosus associated with erythema multiforme to the 4 cases reported by Rowell. Our findings indicate that Rowell's original criteria are not well preserved. The coexistence of lupus erythematosus with erythema multiforme does not impart any unusual characteristics to either disease, and the immunologic disturbances in such patients are probably coincidental.

  3. Lupus Enteritis as an Initial Presentation of Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Sisira Sran

    2014-01-01

    Full Text Available Systemic lupus erythematosus (SLE is an autoimmune disorder which can affect multiple organs and clinical presentation is often a myriad of symptoms; therefore, the index of suspicion should rise when evaluating patients with multiorgan symptomatology. Lupus enteritis is a distinct subset of SLE, defined as either vasculitis or inflammation of the small bowel, with supportive image and/or biopsy findings. The clinical picture of lupus enteritis is often nonspecific, with mild to severe abdominal pain, diarrhea, and vomiting being the cardinal manifestations. Although considered a form of visceral or serosal vasculitis, lupus enteritis is seldom confirmed on histology, making computerized tomography (CT the gold standard for diagnosis. Lupus enteritis is generally steroid-responsive, and the route of administration is based on clinical status and organ involvement, with preference for intravenous (IV route in flares with significant tissue edema. The following case describes a young woman presenting with lupus enteritis and lupus panniculitis as an initial manifestation of SLE, the utilization of abdominal CT in diagnosis, and current treatment protocols used for lupus enteritis.

  4. [Lupus erythematosus panniculitis presenting as palpebral edema and parotiditis].

    Science.gov (United States)

    Pérez-Pastor, G; Valcuende, F; Tomás, G; Moreno, M

    2007-10-01

    Lupus erythematosus panniculitis or lupus erythematosus profundus is characterized by inflammation of the deep dermis and subcutaneous tissue. It can occur in isolation or associated with chronic systemic or discoid lupus erythematosus. It usually consists of nodules and hardened subcutaneous plaques on the forehead, cheeks, proximal extremities, and buttocks. Periorbital and parotid involvement are rare and can lead to misdiagnosis. We present the case of a patient with lupus erythematosus panniculitis who presented with palpebral edema and involvement of the periocular fat and parotid gland.

  5. Spontaneous ureteral rupture in a patient with systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Benson, C.H.; Pennebaker, J.B.; Harisdangkul, V.; Songcharoen, S.

    1983-08-01

    A patient with known systemic lupus erythematosus had fever and symptoms of a lower urinary tract infection. Bone scintigraphy showed left ureteral perforation and necrosis with no demonstrable nephrolithiasis. It is speculated that this episode was due to lupus vasculitis.

  6. Intravenous immunoglobulin therapy and systemic lupus erythematosus.

    Science.gov (United States)

    Zandman-Goddard, Gisele; Levy, Yair; Shoenfeld, Yehuda

    2005-12-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with diverse manifestations. We suggest that intravenous immunoglobulin (IVIg) therapy may be beneficial and safe for various manifestations in SLE. A structured literature search of articles published on the efficacy of IVIg in the treatment of SLE between 1983 and 2005 was conducted. We searched the terms "IVIg," "intravenous immunoglobulin," "lupus," "SLE," and "systemic lupus erythematosus." The various clinical manifestations of SLE that were reported to be successfully treated by IVIg in case reports include autoimmune hemolytic anemia, acquired factor VIII inhibitors, acquired von Willebrand disease, pure red cell aplasia, thrombocytopenia, pancytopenia, myelofibrosis, pneumonitis, pleural effusion, pericarditis, myocarditis, cardiogenic shock, nephritis, end-stage renal disease, encephalitis, neuropsychiatric lupus, psychosis, peripheral neuropathy, polyradiculoneuropathy, and vasculitis. The most extensive experience is with lupus nephritis. There are only a few case series of IVIg use in patients with SLE with various manifestations, in which the response rate to IVIg therapy ranged from 33 to 100%. We suggest that IVIg devoid of sucrose, at a dose of 2 g/kg over a 5-d period given uniformly and at a slow infusion rate in patients without an increased risk for thromboembolic events or renal failure, is a safe and beneficial adjunct therapy for cases of SLE that are resistant to or refuse conventional treatment. The duration of therapy is yet to be established. Controlled trials are warranted.

  7. [Pregnancy and systemic lupus erythematosus: compatible?].

    Science.gov (United States)

    Jason, M; von Frenckell, C; Emonts, P

    2012-11-01

    Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease that predominantly occurs in women of childbearing age. The risk of obstetric complications in lupus parturients is significant. In addition, pregnancy may be associated with flares of the disease requiring immunosuppressive therapy. For these reasons, SLE pregnancies are considered high risk and involve careful collaboration of the obstetrician and rheumatologist. Through the latter and medical advances including a better and better understanding of the binomial mother-child, most pregnancies end in a success.

  8. Systemic lupus erythematosus: Is it one disease?

    Science.gov (United States)

    Rivas-Larrauri, Francisco; Yamazaki-Nakashimada, Marco Antonio

    2016-01-01

    Systemic lupus erythematosus (SLE) is a multisystemic disease with a variety of clinical presentations. Monogenic predisposing conditions to the development of this disease have been described. As examples, an impaired expression of interferon-α regulated genes or complement deficiencies have been reported in patients with SLE, with particular clinical presentations. Those defects present particular presentations and a different severity, making an argument that lupus is not a single disease but many. Treatment could be individualized depending on the underlying defect generating the subtype of the disease.

  9. Anastrozole-induced subacute cutaneous lupus erythematosus.

    Science.gov (United States)

    Fisher, Juliya; Patel, Mital; Miller, Michael; Burris, Katy

    2016-08-01

    Drug-induced subacute cutaneous lupus erythematosus (DI-SCLE) has been associated with numerous drugs, but there are limited reports of its association with aromatase inhibitor anastrozole. We report the case of a patient undergoing treatment with anastrozole for breast cancer who presented with clinical, serological, and histological evidence consistent with DI-SCLE. Her condition quickly began to improve after the use of anastrozole was discontinued and hydroxychloroquine therapy was initiated. Cases such as ours as well as several others that implicate antiestrogen drugs in association with DI-SCLE seem to be contradictory to studies looking at the usefulness of treating systemic lupus erythematosus (SLE) with antiestrogen therapy. Further research on this relationship is warranted.

  10. Subacute cutaneous lupus erythematosus presenting as poikiloderma.

    LENUS (Irish Health Repository)

    Hughes, R

    2012-02-01

    Subacute cutaneous lupus erythematosus (SCLE) is a recognised variant of lupus erythematosus (LE), which accounts for 10-15% of all cases of cutaneous LE, occurring most commonly in young to middle-aged white women. Diagnosis is based on the detection of anti-Ro\\/SS-A antibodies in the skin and serum, characteristic clinical and histological cutaneous involvement, and relatively mild systemic involvement. Several unusual variants of SCLE have been reported including erythrodermic SCLE, SCLE with vitiligo-like lesions, acral SCLE and bullous SCLE. Poikoilodermatous SCLE is a recognised but rare variant of SCLE. There are currently only two case reports, comprising five individual cases, in the literature. We present a case of SCLE in which the main clinical findings were an extensive photodistributed poikilodermatous rash and alopecia.

  11. Gestational outcomes in patients with neuropsychiatric systemic lupus erythematosus.

    Science.gov (United States)

    de Jesus, G R; Rodrigues, B C; Lacerda, M I; Dos Santos, F C; de Jesus, N R; Klumb, E M; Levy, R A

    2017-04-01

    This study analyzed maternal and fetal outcomes of pregnancies of neuropsychiatric systemic lupus erythematosus patients followed in a reference unit. This retrospective cohort study included 26 pregnancies of patients seen between 2011 and 2015 included with history and/or active neuropsychiatric systemic lupus erythematosus among 135 pregnancies. Three patients had active neuropsychiatric systemic lupus erythematosus at conception, but only one remained with neurological activity during gestation, characteristically related to the inadvertent suspension of medications. Twenty six percent of the newborns were small for gestational age and 40% of live births were premature, with no neonatal death or early complications of prematurity. Preeclampsia was diagnosed in nine pregnancies, with two cases of early severe form that resulted in intrauterine fetal death. Patients with neuropsychiatric systemic lupus erythematosus had more prematurity and preeclampsia compared to patients without neuropsychiatric disease. However, when concomitant lupus nephritis was excluded, the gestational results of neuropsychiatric systemic lupus erythematosus patients were more favorable.

  12. Sweet syndrome revealing systemic lupus erythematosus.

    LENUS (Irish Health Repository)

    Quinn, N

    2015-02-01

    Sweet Syndrome is an acute inflammatory skin eruption which is rare in children. We report a case of childhood Systemic Lupus Erythematosus (SLE) that presented with Sweet syndrome. This case is a unique presentation of a common disorder which provides a new facet for the differential diagnosis of SLE in children. It is also the first paediatric case to be reported in a Caucasian child.

  13. Uncommon cutaneous manifestations of lupus erythematosus.

    Science.gov (United States)

    Mascaro, J M; Herrero, C; Hausmann, G

    1997-01-01

    Cutaneous manifestations of lupus erythematosus (LE) are, usually, characteristic enough to permit an easy diagnosis. However, some patients may present less typical lesions, associated or not to the classic ones. Therefore, irrespectively of the variety of LE (acute, subacute and chronic), in absence of the typical butterfly rash, erythematosquamous papules or plaques, or any of the characteristic cutaneous alterations, it is important (even though not always easy) to recognize the uncommon and/or atypical changes of the skin.

  14. Intraveous gammaglobulin in systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Badillo Tenorio Rocio Elizabeth

    2014-07-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic autoinmune multisystemic disease. Intravenous immunoglobulins (IVIg have been previously used for the treatment of primary immunodefi- ciency diseases. In addition IVIg is used for a few autoimmune diseases, such as immune thrombocytopenic purpura. IVIg is an immunomodulatory agent capable of modulating autoinmu- nity and also provide defense against infection, IVIg has been used successfully in SLE. We review the role of IVIg in SLE as a therapeutic option.

  15. Cutaneous lupus erythematosus and systemic lupus erythematosus are associated with clinically significant cardiovascular risk

    DEFF Research Database (Denmark)

    Hesselvig, J Halskou; Ahlehoff, O; Dreyer, L

    2017-01-01

    Systemic lupus erythematosus (SLE) is a well-known cardiovascular risk factor. Less is known about cutaneous lupus erythematosus (CLE) and the risk of developing cardiovascular disease (CVD). Therefore, we investigated the risk of mortality and adverse cardiovascular events in patients diagnosed....... The corresponding HRs for all-cause mortality were 1.32 (95% CI 1.20-1.45) for CLE and 2.21 (95% CI 2.03-2.41) for SLE. CLE and SLE were associated with a significantly increased risk of CVD and all-cause mortality. Local and chronic inflammation may be the driver of low-grade systemic inflammation....

  16. Cardiac tamponade as an initial manifestation of systemic lupus erythematosus.

    Science.gov (United States)

    Carrion, Diego M; Carrion, Andres F

    2012-06-12

    Clinical manifestations of pericardial disease may precede other signs and symptoms associated with systemic lupus erythematosus. Although pericardial effusion is one of the most common cardiac problems in patients with systemic lupus erythematosus, haemodynamically significant effusions manifesting as cardiac tamponade are rare and require prompt diagnosis and treatment.

  17. Chronic aseptic meningitis in a patient with systemic lupus erythematosus.

    Science.gov (United States)

    Lancman, M E; Mesropian, H; Granillo, R J

    1989-08-01

    Chronic aseptic meningitis is a rare manifestation of systemic lupus erythematosus. It may occur early in the course of the disease and sometimes may be the initial symptom. We report a patient with chronic aseptic meningitis associated with systemic lupus erythematosus. Magnetic resonance imaging showed several ischemic lesions and an appearance which was compatible with chronic inflammation of the ependyma of the lateral ventricles.

  18. Unique Protein Signature of Circulating Microparticles in Systemic Lupus Erythematosus

    DEFF Research Database (Denmark)

    Østergaard, Ole; Nielsen, Christoffer; Iversen, Line V

    2013-01-01

    To characterize the unique qualities of proteins associated with circulating subcellular material in systemic lupus erythematosus (SLE) patients compared with healthy controls and patients with other chronic autoimmune diseases.......To characterize the unique qualities of proteins associated with circulating subcellular material in systemic lupus erythematosus (SLE) patients compared with healthy controls and patients with other chronic autoimmune diseases....

  19. [Determinant Factors of Morbidity in Patients with Systemic Lupus Erythematosus].

    Science.gov (United States)

    Jacinto, Margarida; Silva, Eliana; Riso, Nuno; Moraes-Fontes, Maria Francisca

    2017-05-31

    Severity in systemic lupus erythematosus may vary from mild to even fatal consequences. There are no biomarkers to predict the disease's prognosis. The Systemic Lupus International Collaborating Clinics/ Systemic Damage Index defines systemic lupus erythematosus disease severity and is found to predict prognosis. To test damage determinants in a single-centre systemic lupus erythematosus cohort. Retrospectively followed systemic lupus erythematosus female patients (defined by the identification of at least four systemic lupus erythematosus American College of Rheumatology criteria - fulfillment 100%, n = 76) over the past five years. Age of onset, ethnicity, disease duration, number of American College of Rheumatology criteria at the end of follow-up, cumulative: renal, neuropsychiatric and articular phenotypes, hypertension, dyslipidaemia, smoking and Systemic Lupus Erythematosus Disease Activity Index 2K were correlated to the presence and degree of irreversible damage (Systemic Lupus International Collaborating Clinics Damage Index). Accumulation of American College of Rheumatology criteria was measured in a sub-group of patients followed from disease onset (within a year of the first symptom ascribed to systemic lupus erythematosus) (n = 39 - 51%); Systemic Lupus Erythematosus Disease Activity Index and Systemic Lupus International Collaborating Clinics Damage Index were performed. Statistical analysis was performed using Chi-square, Wilcoxon Mann-Whitney tests and Spearman correlation rho (Sig. 2-tailed p Systemic Lupus International Collaborating Clinics/Systemic Damage Index > 0 was present in 56.6% and significantly associated to a longer duration, a higher number of American College of Rheumatology criteria and a neuropsychiatric phenotype when compared with those with no damage. The final number of American College of Rheumatology criteria accrued was positively correlated to a higher disease activity over the past five years of follow-up (Spearman´s rho

  20. Bullous Systemic Lupus Erythematosus: Case report

    Directory of Open Access Journals (Sweden)

    Miziara, Ivan Dieb

    2014-01-01

    Full Text Available Introduction: Bullous systemic lupus erythematosus (BSLE is an autoantibody-mediated disease with subepidermal blisters. It is a rare form of presentation of SLE that occurs in less than 5% of cases of lupus. Case Report: A 27-year-old, female, FRS patient reported the appearance of painful bullous lesions in the left nasal wing and left buccal mucosa that displayed sudden and rapid growth. She sought advice from emergency dermatology staff 15 days after onset and was hospitalized with suspected bullous disease. Intravenous antibiotics and steroids were administered initially, but the patient showed no improvement during hospitalization. She displayed further extensive injuries to the trunk, axillae, and vulva as well as disruption of the bullous lesions, which remained as hyperemic scars. Incisional biopsy of a lesion in the left buccal mucosa was performed, and pathological results indicated mucositis with extensive erosion and the presence of a predominantly neutrophilic infiltrate with degeneration of basal cells and apoptotic keratinocytes. Under direct immunofluorescence, the skin showed anti-IgA, anti-IgM, and anti-IgG linear fluorescence on the continuous dermal side of the cleavage. Indirect immunofluorescence of the skin showed conjugated anti-IgA, was anti-IgM negative, and displayed pemphigus in conjunction with anti-IgG fluorescence in the nucleus of keratinocytes, consistent with a diagnosis of bullous lupus erythematosus. Discussion: BSLE is an acquired autoimmune bullous disease caused by autoantibodies against type VII collagen or other components of the junctional zone, epidermis, and dermis. It must be differentiated from the secondary bubbles and vacuolar degeneration of the basement membrane that may occur in acute and subacute cutaneous lupus erythematosus.

  1. Bullous Systemic Lupus Erythematosus: Case report

    Science.gov (United States)

    Miziara, Ivan Dieb; Mahmoud, Ali; Chagury, Azis Arruda; Alves, Ricardo Dourado

    2013-01-01

    Summary Introduction: Bullous systemic lupus erythematosus (BSLE) is an autoantibody-mediated disease with subepidermal blisters. It is a rare form of presentation of SLE that occurs in less than 5% of cases of lupus. Case Report: A 27-year-old, female, FRS patient reported the appearance of painful bullous lesions in the left nasal wing and left buccal mucosa that displayed sudden and rapid growth. She sought advice from emergency dermatology staff 15 days after onset and was hospitalized with suspected bullous disease. Intravenous antibiotics and steroids were administered initially, but the patient showed no improvement during hospitalization. She displayed further extensive injuries to the trunk, axillae, and vulva as well as disruption of the bullous lesions, which remained as hyperemic scars. Incisional biopsy of a lesion in the left buccal mucosa was performed, and pathological results indicated mucositis with extensive erosion and the presence of a predominantly neutrophilic infiltrate with degeneration of basal cells and apoptotic keratinocytes. Under direct immunofluorescence, the skin showed anti-IgA, anti-IgM, and anti-IgG linear fluorescence on the continuous dermal side of the cleavage. Indirect immunofluorescence of the skin showed conjugated anti-IgA, was anti-IgM negative, and displayed pemphigus in conjunction with anti-IgG fluorescence in the nucleus of keratinocytes, consistent with a diagnosis of bullous lupus erythematosus. Discussion: BSLE is an acquired autoimmune bullous disease caused by autoantibodies against type VII collagen or other components of the junctional zone, epidermis, and dermis. It must be differentiated from the secondary bubbles and vacuolar degeneration of the basement membrane that may occur in acute and subacute cutaneous lupus erythematosus. PMID:25992032

  2. Relationship between health-related quality of life, disease activity and disease damage in a prospective international multicenter cohort of childhood onset systemic lupus erythematosus patients.

    Science.gov (United States)

    Moorthy, L N; Baldino, M E; Kurra, V; Puwar, D; Llanos, A; Peterson, M G E; Hassett, A L; Lehman, T J A

    2017-03-01

    Previously, we described associations between health-related quality of life (HRQOL) and disease-related factors among childhood onset systemic lupus erythematosus (cSLE) patients. Here we determined the relationship between HRQOL, disease activity and damage in a large prospective international cohort of cSLE. We compared HRQOL, disease activity and disease damage across different continents and examined the relationship between children's and parents' assessments of HRQOL. Patients with cSLE and their parents completed HRQOL measures at enrollment and ≥4 follow-up visits. Physicians assessed disease activity and damage. The multinational cohort ( n = 467) had relatively low disease activity and damage. Patient and parent HRQOL scores were significantly correlated. Asian and European patients had the highest HRQOL, while South and North American patients had lower HRQOL scores. Renal, CNS, skin and musculoskeletal systems exhibited the highest levels of damage. North and South American and Asian patients were more likely to have disease damage and activity scores above median values, compared with Europeans. Asians were more likely to use cyclophosphamide/rituximab. Female gender, high disease activity and damage, non-White ethnicity, and use of cyclophosphamide and/rituximab were related to lower HRQOL. HRQOL domain scores continue to emphasize that SLE has widespread impact on all aspects of children's and parents' lives.

  3. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Petri, Michelle; Orbai, Ana-Maria; Alarcón, Graciela S

    2012-01-01

    The Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) classification criteria in order to improve clinical relevance, meet stringent methodology requirements, and incorporate new kno...

  4. The Euro-lupus project: epidemiology of systemic lupus erythematosus in Europe.

    Science.gov (United States)

    Cervera, R; Khamashta, M A; Hughes, G R V

    2009-09-01

    The Euro-lupus project provides updated information on the epidemiologic characteristics of systemic lupus erythematosus (SLE) at the change of the millennium and defines several clinical and immunological prognostic factors. The Euro-lupus cohort is composed of 1000 patients with SLE who have been followed prospectively since 1991. Among other findings, this project has shown that a) the age at onset of the disease, the gender and the autoantibody pattern, among other factors, modify the disease expression and define some specific SLE subsets; b) most of the SLE inflammatory manifestations are less common after long-term evolution of the disease, thus probably reflecting the effect of therapy as well as the progressive remission of the disease in many patients and c) a more prominent role of thrombotic events is becoming evident affecting both morbidity and mortality in SLE.

  5. Systemic lupus erythematosus in pregnancy: Case report

    Directory of Open Access Journals (Sweden)

    Radeka Gordana

    2005-01-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic inflammatory connective tissue disease commonly diagnosed after the age of 20, mostly around the age of 30 years. It is more common in women than in men, especially during the fertile period. Women with SLE are at higher risk for spontaneous abortions, intrauterine fetal death, preeclampsia and eclampsia, preterm delivery and intrauterine growth retardation. This paper is a case report of a pregnant woman with SLE complicated with preeclampsia, but it also discusses follow-up of such pregnancies.

  6. Management of Systemic Lupus Erythematosus During Pregnancy.

    Science.gov (United States)

    Sammaritano, Lisa R

    2017-01-14

    Reproductive issues including contraception, fertility, and pregnancy are important components of the comprehensive care of women with systemic lupus erythematosus (SLE). SLE pregnancies are complicated due to risk for maternal disease exacerbation and potential for fetal and neonatal complications. Pre-pregnancy assessment is important to identify patients with severe disease-related damage who should avoid pregnancy, counsel patients to conceive when disease has been stable and inactive on appropriate medications, and assess relevant risk factors including renal disease, antiphospholipid antibody, and anti-Ro/SS-A and anti-La/SS-B antibodies. With careful planning, monitoring, and care, most women with SLE can anticipate a successful pregnancy.

  7. Systemic lupus erythematosus: Clinical and experimental aspects

    Energy Technology Data Exchange (ETDEWEB)

    Smolen, J.S.

    1987-01-01

    This text covers questions related to the history, etiology, pathogenesis, clinical aspects and therapy of systematic lupus erythematosus (SLE). Both animal models and human SLE are considered. With regard to basic science, concise information on cellular immunology, autoantibodies, viral aspects and molecular biology in SLE is provided. Clinical topics then deal with medical, dermatologic, neurologic, radiologic, pathologic, and therapeutic aspects. The book not only presents the most recent information on clinical and experimental insights, but also looks at future aspects related to the diagnosis and therapy of SLE.

  8. Review of treatment for discoid lupus erythematosus.

    Science.gov (United States)

    Garza-Mayers, Anna Cristina; McClurkin, Michael; Smith, Gideon P

    2016-07-01

    Discoid lupus erythematosus (DLE) is a chronic cutaneous disease characterized by inflammatory plaques that, in the absence of prompt diagnosis and treatment, may lead to disfiguring scarring and skin atrophy. However, there is limited evidence for which treatments are most effective. Currently, no medications have been approved specifically for the treatment of DLE. Many of the drugs described in the literature were developed for use in other immune disorders. This review will summarize current therapeutic options for DLE and their supporting evidence with discussion of prevention, topical measures, physical modalities, and systemic therapies, including newer potential therapies.

  9. Systemic lupus erythematosus : abdominal radiologic findings

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Jae Cheon; Cho, On Koo; Lee, Yong Joo; Bae, Jae Ik; Kim, Yong Soo; Rhim, Hyun Chul; Ko, Byung Hee [Hanyang Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-06-01

    Systemic lupus erythematosus(SLE) is a systemic disease of unknown etiology. Its main pathology is vasculitis and serositis, due to deposition of the immune complex or antibodies. Most findings are nonspecific ; abdominal manifestations include enteritis, hepatomegaly, pancreatic enlargement, serositis, lymphadenopathy, splenomegaly, nephritis, interstitial cystitis, and thrombophlebitis. We described radiologic findings of various organ involvement of SLE; digestive system, serosa, reticuloendothelial system, urinary system, and venous system. Diagnosis of SLE was done according to the criteria of American Rheumatism Association. Understanding of the variable imaging findings in SLE may be helpful for the early detection of abdominal involvement and complications.

  10. Pulmonary manifestations of systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Yang, Kee Hyuk; Choi, Yo Won; Jeon, Seok Chol; Park, Choong Ki; Joo, Kyung Bin; Hahm, Chang Kok; Lee, Seung Ro [College of Medicine, Hanyang Univ., Seoul (Korea, Republic of)

    2004-02-01

    Pulmonary involvement is more common in systemic lupus erythematosus (SLE) than in any other connective tissue disease, and more than half of patients with SLE suffer from respiratory dysfunction during the course of their illness. Although sepsis and renal disease are the most common causes of death in SLE, lung disease is the predominant manifestation and is an indicator of overall prognosis. Respiratory disease may be due to direct involvement of the lung or as a secondary consequence of the effect of the disease on other organ systems.

  11. Collapsing glomerulopathy in systemic lupus erythematosus.

    Science.gov (United States)

    Abadeer, Kerolos; Alsaad, Ali A; Geiger, Xochiquetzal J; Porter, Ivan E

    2017-02-27

    Collapsing glomerulopathy (CG) is a rare disease that can be associated with multiple other disorders. It usually leads to poor prognosis with a high percentage of patients progressing to end-stage renal disease. In this article, we illustrate a clinical case of CG associated with systemic lupus erythematosus that had a prompt response to mycophenolate and prednisone. The condition started after sudden cessation of the already established mycophenolate treatment regimen. The patient then presented with acute kidney injury due to kidney biopsy-proven CG. In that circumstance, we hypothesised that mycophenolate may play a role in prevention and development of CG.

  12. Lichen planus and lupus erythematosus overlap syndrome

    Directory of Open Access Journals (Sweden)

    Chopra Adarsh

    1996-01-01

    Full Text Available A 45-year-old woman with livid plaques showing central atrophy and erythematous vesicular borders over both dorsa of feet and buttocks, and follicular and papular lesions over buttocks and lumbar area, was difficult to diagnose as either lichen planus (LP or lupus erythematosus (LE. The histological studies from two places showed features of both LE and LP. Laboratory findings were within normal limits first, but follow up studies for two years showed persistent albuminurea, leucopenia, arthritis and erythema over the exposed areas with same histology suggesting that eruption may be an unusual variant of LE.

  13. Novel therapeutic agents for systemic lupus erythematosus.

    Science.gov (United States)

    Gescuk, Bryan D; Davis, John C

    2002-09-01

    The last significant breakthrough in the treatment of systemic lupus erythematosus (SLE) was the use of cyclophosphamide and methylprednisolone in the treatment of lupus nephritis. Recent advances in immunology, oncology, and endocrinology have resulted in many potential therapies for SLE. These therapies include new immunosuppressants, biologic medications, tolerizing agents, immunoablation techniques, and hormonal medications. Each of these approaches will be discussed in this review. Some therapies are currently in use in clinical rheumatology practice (mycophenolate mofetil) and others are entering phase I trials (anti-BLyS monoclonal antibody). While some of these new therapies target specific inflammatory mechanisms in SLE (anti-CD40L monoclonal antibody), others work by nonspecific inhibition of the immune system (immunoablation).

  14. Cutaneous Manifestations of Systemic Lupus Erythematosus

    Science.gov (United States)

    Uva, Luís; Miguel, Diana; Pinheiro, Catarina; Freitas, João Pedro; Marques Gomes, Manuel; Filipe, Paulo

    2012-01-01

    Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. The skin is one of the target organs most variably affected by the disease. The American College of Rheumatology (ACR) established 11 criteria as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. Cutaneous lesions account for four of these 11 revised criteria of SLE. Skin lesions in patients with lupus may be specific or nonspecific. This paper covers the SLE-specific cutaneous changes: malar rash, discoid rash, photosensitivity, and oral mucosal lesions as well as SLE nonspecific skin manifestations, their pathophysiology, and management. A deeper thorough understanding of the cutaneous manifestations of SLE is essential for diagnosis, prognosis, and efficient management. Thus, dermatologists should cooperate with other specialties to provide optimal care of SLE patient. PMID:22888407

  15. Acquired enophthalmos with systemic lupus erythematosus.

    Science.gov (United States)

    Park, K R; Seo, M R; Ryu, H J; Chi, M J; Baek, H J; Choi, H J

    2016-01-01

    Ocular involvement sometimes occurs with systemic lupus erythematosus (SLE) but enophthalmos with SLE is rare. We report a case of enophthalmos with SLE. A 25-year-old male was admitted for two weeks of fever, sore throat, arthralgia, chest pain and right arm weakness with pain. We diagnosed him with SLE with malar rash, arthritis, pleural effusion, proteinuria, leukopenia, positive antinuclear antibody, anti-dsDNA, and lupus anticoagulant. The patient was prescribed high-dose prednisolone and hydroxychloroquine 400 mg. One week after discharge, he complained about a sensation of a sunken right eye. CT showed right enophthalmos, a post-inflammatory change and chronic inflammation. Proteinuria increased to 3.8 g/day after the patient stopped taking prednisolone. Cyclophosphamide therapy was administered for three months without improvement. We decided to restart prednisolone and change cyclophosphamide to mycophenolate mofetil. Proteinuria decreased but enophthalmos remains as of this reporting.

  16. Echocardiographic findings in asymptomatic systemic lupus erythematosus patients.

    Science.gov (United States)

    Mohammed, Abdel GaffarA; Alghamdi, Abdulaziz A; ALjahlan, Mohammad A; Al-Homood, Ibrahim A

    2017-03-01

    The aim of this study is to use transthoracic echocardiographic (TTE) imaging methods to identify cardiac dysfunction in asymptomatic systemic lupus erythematosus (SLE) patients and to determine the association between echocardiographic findings and serology. This is a prospective cross-sectional study where 50 patients with confirmed diagnoses of SLE were recruited from rheumatology outpatient clinics. Clinical and serological evaluation to confirm the diagnosis of lupus was done in all patients. Fifty SLE patients, 46 (92%) females and 4 (8%) males, were recruited. Anti-double-stranded DNA (Anti-dsDNA), anticardiolipin, lupus anticoagulant, and anti-β2-glycoproteins were positive in 52.1, 32.6, 13.3, and 15.6%, respectively. Transthoracic echocardiogram revealed mitral regurgitation in 16 patients (32%), pericardial effusion in16 patients (32%), aortic regurgitation in five patients (10%), and tricuspid regurgitation in 10 patients (20%). Eleven patients had left ventricular hypertrophy (22%), and eight patients had ventricular systolic dysfunction (16%). Only four patients had ventricular diastolic dysfunction (8%). A significant association between mitral and tricuspid valve regurgitation and positive anti-dsDNA (p lupus anticoagulant, and anti-β 2 glycoprotein antibodies were also associated with mitral valve regurgitation (p values 0.044, 0.006, and 0.023), respectively. Active disease assessed by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was found to be associated with increased risk of mitral valvular leaflet thickening (p value 0.028). Performing regular transthoracic echocardiogram in asymptomatic SLE patients is important for early detection and appropriate treatment of cardiac lesions. Clinically quiescent but serologically active disease and presence of antiphospholipid antibodies were associated with structural heart abnormalities.

  17. Epigenetics and Systemic Lupus Erythematosus: Unmet Needs.

    Science.gov (United States)

    Meroni, Pier Luigi; Penatti, Alessandra Emiliana

    2016-06-01

    Systemic lupus erythematosus (SLE) is a chronic relapsing-remitting autoimmune disease affecting several organs. Although the management of lupus patients has improved in the last years, several aspects still remain challenging. More sensitive and specific biomarkers for an early diagnosis as well as for monitoring disease activity and tissue damage are needed. Genome-wide association and gene mapping studies have supported the genetic background for SLE susceptibility. However, the relatively modest risk association and the studies in twins have suggested a role for environmental and epigenetic factors, as well as genetic-epigenetic interaction. Accordingly, there is evidence that differences in DNA methylation, histone modifications, and miRNA profiling can be found in lupus patients versus normal subjects. Moreover, impaired DNA methylation on the inactive X-chromosome was suggested to explain, at least in part, the female prevalence of the disease. Epigenetic markers may be help in fulfilling the unmet needs for SLE by offering new diagnostic tools, new biomarkers for monitoring disease activity, or to better characterize patients with a silent clinical disease but with an active serology. Anti-DNA, anti-phospholipid, and anti-Ro/SSA autoantibodies are thought to be pathogenic for glomerulonephritis, recurrent thrombosis and miscarriages, and neonatal lupus, respectively. However, tissue damage occurs occasionally or, in some patients, only in spite of the persistent presence of the antibodies. Preliminary studies suggest that epigenetic mechanisms may explain why the damage takes place in some patients only or at a given time.

  18. Gastrointestinal system involvement in systemic lupus erythematosus.

    Science.gov (United States)

    Li, Z; Xu, D; Wang, Z; Wang, Y; Zhang, S; Li, M; Zeng, X

    2017-10-01

    Systemic lupus erythematosus (SLE) is a multisystem disorder which can affect the gastrointestinal (GI) system. Although GI symptoms can manifest in 50% of patients with SLE, these have barely been reviewed due to difficulty in identifying different causes. This study aims to clarify clinical characteristics, diagnosis and treatment of the four major SLE-related GI system complications: protein-losing enteropathy (PLE), intestinal pseudo-obstruction (IPO), hepatic involvement and pancreatitis. It is a systematic review using MEDLINE and EMBASE databases and the major search terms were SLE, PLE, IPO, hepatitis and pancreatitis. A total of 125 articles were chosen for our study. SLE-related PLE was characterized by edema and hypoalbuminemia, with Technetium 99m labeled human albumin scintigraphy ((99m)Tc HAS) and alpha-1-antitrypsin fecal clearance test commonly used as diagnostic test. The most common site of protein leakage was the small intestine and the least common site was the stomach. More than half of SLE-related IPO patients had ureterohydronephrosis, and sometimes they manifested as interstitial cystitis and hepatobiliary dilatation. Lupus hepatitis and SLE accompanied by autoimmune hepatitis (SLE-AIH overlap) shared similar clinical manifestations but had different autoantibodies and histopathological features, and positive anti-ribosome P antibody highly indicated the diagnosis of lupus hepatitis. Lupus pancreatitis was usually accompanied by high SLE activity with a relatively high mortality rate. Early diagnosis and timely intervention were crucial, and administration of corticosteroids and immunosuppressants was effective for most of the patients.

  19. Characteristics of pleural effusions in systemic lupus erythematosus: differential diagnosis of lupus pleuritis.

    Science.gov (United States)

    Choi, B Y; Yoon, M J; Shin, K; Lee, Y J; Song, Y W

    2015-03-01

    We investigated the clinical characteristics of pleural effusion in systemic lupus erythematosus (SLE). A prospective analysis of 17 SLE patients with pleural effusion (seven lupus pleuritis, eight transudative effusions and two parapneumonic effusions) was performed. Thirty non-SLE patients with pleural effusion were recruited as controls. A pleural fluid ANA titer ≥1:160 was found in 8/17 (47.1%) SLE patients and none of the 30 non-SLE patients (p = 0.0001). Pleural fluid to serum C3 ratios were significantly lower in SLE than in non-SLE (median (minimum-maximum) 0.29 (0.03-0.43) versus 0.52 (0.26-0.73), p = 0.0002). Among SLE patients, pleural fluid ANA titers ≥1:160 were more frequently found in patients with lupus pleuritis than in those with pleural effusion from causes other than lupus itself (85.7% versus 20.0%, p = 0.0152). Serum CRP levels were significantly increased in patients with lupus pleuritis compared with SLE patients with transudative pleural effusion (2.30 (0.30-5.66) versus 0.7 (0.12-1.47) mg/dl, p = 0.0062). In conclusion, pleural fluid ANA titer and serum CRP levels are significantly increased in lupus pleuritis. © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  20. Therapeutic interventions of tissue specific autoimmune onset in systemic lupus erythematosus.

    Science.gov (United States)

    Dasgupta, Subhajit; Dasgupta, Shaoni

    2016-06-10

    Systemic lupus erythematosus (lupus) is a female predominant autoimmune disease. The auto reactive B cells and T helper cells together are known to develop self-reactive immune responses in different tissues like kidney, bone, cardiovascular and central nervous system. Progression of disease is associated with deposition of immune complex which initiates tissue damage. The therapy for lupus still includes corticosteroids to reduce allergic manifestations and inflammatory immune responses. Recent observations suggested that, mycophenolate mofetil and cyclophosphamide treatment in combination with corticosteroids have benefit in lupus therapy. The prospect of B cell depletion by CD20 targeted monoclonal antibody Rituximab has been demonstrated in lupus patients. The CD52 specific monoclonal antibody Alemtuzumab is another proposition for lupus therapy. The drug Belimumab inhibits B cell activation by altering BAFF/APRIL signal cascade. Recent discovery of the CD22 targeted Epratuzumab also shows therapeutic prospect. The researches on new generation drugs for autoimmune lupus include search for inhibitors of CD40-CD40Ligand interactions, CD86 activation, selective modulation of complement cascades. The choice of inhibitors of transcription factor NF-κBp65 and selective modulators for estrogen receptor alpha are proposed areas of lupus drug discovery research. Keeping a close eye on the mechanisms of disease onset, a comprehensive view is provided on recent therapy of systemic lupus erythematosus.

  1. Catatonia due to systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Francisco de Assis Pinto Cabral Júnior Rabello

    2014-07-01

    Full Text Available Objectives Discuss neuropsychiatric aspects and differential diagnosis of catatonic syndrome secondary to systemic lupus erythematosus (SLE in a pediatric patient. Methods Single case report. Result A 13-year-old male, after two months diagnosed with SLE, started to present psychotic symptoms (behavioral changes, hallucinations and delusions that evolved into intense catatonia. During hospitalization, neuroimaging, biochemical and serological tests for differential diagnosis with metabolic encephalopathy, neurological tumors and neuroinfections, among other tests, were performed. The possibility of neuroleptic malignant syndrome, steroid-induced psychosis and catatonia was also evaluated. A complete reversal of catatonia was achieved after using benzodiazepines in high doses, associated with immunosuppressive therapy for lupus, which speaks in favor of catatonia secondary to autoimmune encephalitis due to lupus. Conclusion Although catatonia rarely is the initial clinical presentation of SLE, the delay in recognizing the syndrome can be risky, having a negative impact on prognosis. Benzodiazepines have an important role in the catatonia resolution, especially when associated with parallel specific organic base cause treatment. The use of neuroleptics should be avoided for the duration of the catatonic syndrome as it may cause clinical deterioration.

  2. Bullous Systemic Lupus Erythematosus Associated with Esophagitis Dissecans Superficialis

    Science.gov (United States)

    Jaffe, Eric A.

    2015-01-01

    Bullous systemic lupus erythematosus is one of the rare autoantibody mediated skin manifestation of systemic lupus erythematosus (SLE) demonstrating subepidermal blistering with neutrophilic infiltrate histologically. We present a case of a 40-year-old Hispanic female who presented with a several months' history of multiple blistering pruritic skin lesions involving the face and trunk, a photosensitive rash over the face and neck, swelling of the right neck lymph node, and joint pain involving her elbows and wrist. Her malady was diagnosed as bullous systemic lupus erythematosus based on the immunological workup and biopsy of her skin lesions. The patient also complained of odynophagia and endoscopy revealed esophagitis dissecans superficialis which is a rare endoscopic finding characterized by sloughing of the esophageal mucosa. The bullous disorders typically associated with esophagitis dissecans superficialis are pemphigus and rarely bullous pemphigoid. However, this is the first reported case of bullous systemic lupus erythematosus associated with esophagitis dissecans superficialis. PMID:25821624

  3. Case Report: An atypical case of systemic lupus erythematosus ...

    African Journals Online (AJOL)

    Results: She had a favorable clinical response and continues to be followed up as an outpatient.Conclusion: Systemic lupus erythematosus can be difficult diagnosis to make as it may present with atypical features.

  4. Central nervous system involvement in systemic lupus erythematosus.

    Institute of Scientific and Technical Information of China (English)

    1994-01-01

    This paper deals with the clinical, immunological and pathological data of 5 eases of systemic lupus erythematosus (SLE). Each of the five cases has typical SLE damages on the skin and multiple organs. Among

  5. Purified umbilical cord derived mesenchymal stem cell treatment in a case of systemic lupus erythematosus.

    Science.gov (United States)

    Phillips, Christopher D; Wongsaisri, Pornpatcharin; Htut, Thein; Grossman, Terry

    2017-12-01

    Systemic lupus erythematosus (SLE) is a multiple organ system autoimmune disorder for which there is no known cure. We report a case of a young adult lady with SLE and Sjogren's with diagnostic and clinical resolution following purified umbilical cord derived mesenchymal stem cell (MSC) and globulin component protein macrophage activating factor (GcMAF) therapy in a combined multidisciplinary integrative medicine protocol. Our patient had complete reversal of all clinical and laboratory markers. We recommend a prospective randomized double blind study to assess the sustained efficacy of MSC and GcMAF in the treatment of autoimmune connective tissue diseases such as systemic lupus erythematosus.

  6. The Physiotherapy in Patients with Systemic Lupus Erythematosus

    OpenAIRE

    Hladíková, Alena

    2016-01-01

    Name: Alena Hladíková Supervisor: Mgr. Renáta Muchová Opponent: Title of bachelor thesis: The Physiotherapy in Patients with Systemic Lupus Erythematosus Abstract: This bachelor thesis concerns physiotherapy in patients with systemic lupus erythematosus diagnose. It mainly focuses on physiotherapeutic techniques indicated in this kind of patients. Another task is evaluating the effect of physiotherapy in acute issues caused by this disease. The thesis is divided into theoretical and practical...

  7. Lupus eritematoso discóide na infância Discoid lupus erythematosus in childhood

    Directory of Open Access Journals (Sweden)

    Maria Carolina de A. Sampaio

    2007-06-01

    Full Text Available OBJETIVO: Realizar revisão da literatura sobre o lupus eritematoso discóide (LED na infância. FONTES DE DADOS: Livros-texto e artigos de revistas indexadas pelo Medline e SciELO nos últimos dez anos, usando as seguintes palavras-chave: "discoid lupus erythematosus", "chronic cutaneous lupus erythematosus", "lupus erythematosus in childhood", "lupus erythematosus in children", "discoid lupus erythematosus in childhood", "discoid lupus erythematosus in children". SÍNTESEDOS DADOS: A idade de início da doença ocorre predominantemente entre cinco e dez anos e a história familiar de lupus eritematoso está presente em 11 a 35% dos casos. A relação gênero feminino/masculino varia de 1/1 a 2,4/1. Por sua vez, 24 a 27% dos pacientes com LED desenvolvem lupus eritematoso sistêmico (LES. Lesões discóides localizadas (que acometem cabeça e pescoço são observadas em 56 a 75% dos pacientes. A face é o local mais acometido. O LED localizado e o generalizado apresentam evolução semelhante. Os achados histológicos são típicos, mostrando dermatite de interface. IgM e IgG são os depósitos mais freqüentes na zona da membrana basal da epiderme. Os tratamentos geralmente utilizados são: fotoproteção, corticosteróides tópicos e antimaláricos. Imunossupressores, talidomida, dapsona e retinóides podem ser usados nos casos refratários. CONCLUSÕES: O LED da infância parece ter pequeno predomínio no gênero feminino, alta prevalência de história familiar de lupus eritematoso e elevada proporção que evolui para a forma sistêmica da doença, comparada ao LED do adulto. O LED localizado e o generalizado apresentam prognósticos semelhante. Os achados histológicos não foram diferentes daqueles descritos no LED do adulto.OBJECTIVE: To review the literature about discoid lupus erythematosus (DLE in childhood. DATA SOURCES: Textbooks and journals indexed for Medline and SciELO in the last ten years. The following key-words were

  8. Sjögren's syndrome associated with systemic lupus erythematosus.

    Science.gov (United States)

    Taşdemir, Mehmet; Hasan, Chiar; Ağbaş, Ayşe; Kasapçopur, Özgür; Canpolat, Nur; Sever, Lale; Çalışkan, Salim

    2016-09-01

    Systemic lupus erythematosus and Sjögren's syndrome are chronic auto- inflammatory disorders which can lead to serious organ damage. Although systemic lupus erythematosus and Sjögren's syndrome were previously considered two forms of the same disease because of presence of clinical coexistence of these two conditions, the view that they are two different conditions with mutual characteristics has become prominent in recent years. In this paper, we reported a 16 year-old girl who was followed up with a diagnosis of Sjögren's syndrome for six years and then was observed to have overlap of systemic lupus erythematosus. In the baseline, she did not have any clinical or serological evidence for systemic lupus erythematosus. After six year, massive proteinuria and serological findings developed and systemic lupus erythematosus nephritis was diagnosed by kidney biopsy. Currently, systemic lupus erythematosus and Sjögren's syndrome cannot be differentiated definetely. We need more valuable diagnostic and classification criteria to differentiate these two important conditions.

  9. Autoantibody profiling in systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Hsieh SC

    2013-08-01

    Full Text Available Song-Chou Hsieh,1 Chia-Li Yu1,2 1Department of Internal Medicine, 2Institute of Molecular Medicine, National Taiwan University College of Medicine, Taipei, Taiwan Abstract: Systemic lupus erythematosus (SLE is an archetype of systemic autoimmune disease characterized by the production of a broad spectrum of autoantibodies. More than 100 autoantibodies have been found in the sera of patients with SLE, including antibodies against nuclear, cytoplasmic, surface-membrane, and extracellular antigens. There has been considerable debate as to whether these antinuclear autoantibodies (ANAs are merely biomarkers for disease or are responsible for organ/tissue damage in SLE. In recent years, sufficient evidence has supported the hypothesis that many ANAs, such as anti-double-stranded DNA (anti-dsDNA, antiribosomal P, anti-Sm, antiribonucleoprotein (anti-RNP, and even anti-Sjögren's syndrome (SS-B/La antibodies not only act against specific nuclear antigens but also cross-react with different surface-expressed cognate molecules. The binding of autoantibodies to the cell surface leads to their penetration into the cell's interior to elicit cellular damage. There are at least four conceivable routes for ANAs to penetrate the cytoplasm: (1 nonspecific Fcγ receptor-mediated uptake, (2 cell-surface caveolae-mediated endocytosis, (3 electrostatic interactions between positively charged amino acids of the complementarity-determining regions of the antibody molecule and the negatively charged surface membrane, and (4 the binding of the autoantibody with its cross-reactive cell surface-expressed cognate molecule, and its subsequent endocytosis into the cytoplasm. In this review, we discuss in detail the immunopathogenic mechanisms of the commonly encountered ANAs, such as anti-dsDNA, antiribosomal P, and anti-SSB/La, that are associated with lupus pathogenesis. Additionally, the detrimental thromboembolism-inducing anticardiolipin antibodies in patients with SLE

  10. Systemic lupus erythematosus--2005 annus mirabilis?

    Science.gov (United States)

    Isenberg, David; Rahman, Anisur

    2006-03-01

    We are about to enter a new era in the treatment of patients with systemic lupus erythematosus (SLE). For the past 40 years hydroxychloroquine sulfate and corticosteroids, together with varying combinations of immunosuppressive drugs, have been the main treatments for SLE. Although effective for many patients, some patients fail to respond to these drugs and even more suffer from major side effects due to the generalized nature of the immunosuppression. In this article we review the remarkable confluence of new therapies ranging from newer immunosuppressive drugs with fewer side effects, such as mycophenolate mofetil, to the more targeted approaches offered by biological agents. These agents have been designed to block molecules such as CD20, CD22 and interleukin-10 that are thought to have an integral part in the development of SLE. This wolf might not yet be about to become extinct but its survival is increasingly under threat!

  11. Management of pregnancy in systemic lupus erythematosus.

    Science.gov (United States)

    Lateef, Aisha; Petri, Michelle

    2012-12-01

    Systemic lupus erythematosus (SLE) is an autoantibody-mediated systemic autoimmune disease, predominantly affecting young females. Pregnancy is increasingly common in the setting of SLE, as survival and quality of life of patients improve. Although live births can be achieved in the most cases, pregnancy in patients with SLE remains a high-risk condition. Maternal and fetal mortality and morbidity are considerably increased, compared with the general population. Aberrations in pregnancy-related maternal immune adaptations are likely contributors. Active maternal disease, renal involvement, specific autoantibody subsets and advanced organ damage are predictors of poor outcome. Therapeutic options are limited during pregnancy as maternal benefit has to be weighed against fetal risk. Prevention of preterm birth and refractory pregnancy loss, as well as management of established neonatal heart block remain unmet needs. Further research should address these important issues that affect young patients with SLE and their babies.

  12. [Current diagnosis of cutaneous lupus erythematosus].

    Science.gov (United States)

    Haust, M; Bonsmann, G; Kuhn, A

    2006-07-14

    Cutaneous lupus erythematosus (CLE) is a disease with different subtypes and the new classification system includes acute CLE (ACLE), subacute CLE (SCLE), chronic CLE (CCLE), and intermittent CLE (ICLE). The broad spectrum of skin involvement and the possibility of systemic organ manifestations at the beginning and during the course of CLE require specific diagnostic procedures. Clinical assessment of the cutaneous manifestations is necessary along with a detailed patient's history. The diagnosis of CLE is confirmed by histopathology and immunofluorescence microscopy. Selective laboratory screening and additional diagnostic procedures depending on clinical symptoms are recommended. Photoprovocation tests can be performed to assess photosensitivity in patients with CLE and to support the diagnosis. Recently, a scoring system for the activity of the cutaneous manifestations in CLE has been developed and is now evaluated in several clinical studies. In this review, the classification and the characteristic clinical criteria of the different CLE subtypes as well as the current diagnostic possibilities are emphasized.

  13. Systemic lupus erythematosus one disease or many?

    Science.gov (United States)

    Agmon-Levin, N; Mosca, M; Petri, M; Shoenfeld, Y

    2012-06-01

    Systemic lupus erythematosus (SLE) characterizes by a variety of clinical manifestations and the presence of a wide profile of autoantibodies. This clinical and serological heterogeneity raised the question: is SLE a single disease with varied phenotypes, or a similar phenotype shared by different diseases with diverse pathogenic mechanisms? Herein we debate the clinical, genetic, hormonal and serological differences typically observed in SLE on the one hand, and the numerous similarities between subtypes of this disease on the other. Leading to the conclusion that SLE may be considered not as a single disease but rather as a single syndrome, which defines by a set of signs, symptoms, or phenomena that occur together and suggest a particular abnormality. Additionally, the accumulated knowledge on gene expression pathways, autoantibodies clusters, hormonal and environmental factors associated with SLE may allow a better classification of this syndrome and updating of SLE criteria. This may further allow targeted biologics and other therapies as well as "personalized medicine" to begin.

  14. Ocular manifestations in systemic lupus erythematosus.

    Science.gov (United States)

    Silpa-archa, Sukhum; Lee, Joan J; Foster, C Stephen

    2016-01-01

    Systemic lupus erythematosus (SLE) can involve many parts of the eye, including the eyelid, ocular adnexa, sclera, cornea, uvea, retina and optic nerve. Ocular manifestations of SLE are common and may lead to permanent blindness from the underlying disease or therapeutic side effects. Keratoconjunctivitis sicca is the most common manifestation. However, vision loss may result from involvement of the retina, choroid and optic nerve. Ocular symptoms are correlated to systemic disease activity and can present as an initial manifestation of SLE. The established treatment includes prompt systemic corticosteroids, steroid-sparing immunosuppressive drugs and biological agents. Local ocular therapies are options with promising efficacy. The early recognition of disease and treatment provides reduction of visual morbidity and mortality.

  15. Central nervous system lupus erythematosus in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Yokota, Shumpei; Kimura, Kazue; Yoshida, Naotaka; Mitsuda, Toshihiro; Ibe, Masa-aki; Shimizu, Hiroko (Yokohama City Univ. (Japan). Faculty of Medicine)

    1989-12-01

    Clinical features of central nervous system (CNS) invlvement in childhood systemic lupus erythematosus (SLE) was investigated. Neuropsychiatric manifestations including seizures, chorea, headache, overt psychosis, tremor, increase of muscle spastisity, and disturbed memory were found in 47% of 15 patients with SLE. There was a well correlatin between CNS abnormalities and SLE disease activity judged by serum complement levels and anti-nuclear antibody and anti-DNA antibody titers. The administration of Prednisolon was effective for the treatment of these CNS abnormalities and steroid psychosis was rare in the present study. EEG abnormalities involving diffuse slowing and slowing bursts were found in 73% of the patients. Cranial CT scan revealed basel ganglia calcifications in 2 patients, and marked brain atrophy in 3 patients. This study indicated that in the long term following of SLE children CNS abnormalities need to be serially checked by EEG and cranial CT scans as well as serological investigations. (author).

  16. Biological Therapy in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Mariana Postal

    2012-01-01

    Full Text Available Systemic lupus erythematosus (SLE is a prototypic inflammatory autoimmune disorder characterized by multisystem involvement and fluctuating disease activity. Symptoms range from rather mild manifestations such as rash or arthritis to life-threatening end-organ manifestations. Despite new and improved therapy having positively impacted the prognosis of SLE, a subgroup of patients do not respond to conventional therapy. Moreover, the risk of fatal outcomes and the damaging side effects of immunosuppressive therapies in SLE call for an improvement in the current therapeutic management. New therapeutic approaches are focused on B-cell targets, T-cell downregulation and costimulatory blockade, cytokine inhibition, and the modulation of complement. Several biological agents have been developed, but this encouraging news is associated with several disappointments in trials and provide a timely moment to reflect on biologic therapy in SLE.

  17. Classification criteria for systemic lupus erythematosus: a review.

    Science.gov (United States)

    Petri, M; Magder, L

    2004-01-01

    The Systemic Lupus International Collaborating Clinics, in preparation for revising the ACR classification criteria for systemic lupus erythematosus, reviewed the current classification criteria. These critical reviews, discussed at the Lund, Sweden, meeting in 2003, will be useful to the clinician and to the researcher. This paper reviews and critiques previous classification attempts.

  18. Lupus erythematosus--a case of facial swelling.

    Science.gov (United States)

    Loescher, A; Edmondson, H D

    1988-04-01

    A case is reported of acute facial swelling following tooth extraction that failed to respond in a normal manner. The patient developed systemic signs and symptoms ultimately revealing the diagnosis of lupus erythematosus. The possibility of soft tissue lesions arising in some forms of lupus is emphasised by this report.

  19. T-cell-directed therapies in systemic lupus erythematosus.

    Science.gov (United States)

    Nandkumar, P; Furie, R

    2016-09-01

    Drug development for the treatment of systemic lupus erythematosus (SLE) has largely focused on B-cell therapies. A greater understanding of the immunopathogenesis of SLE coupled with advanced bioengineering has allowed for clinical trials centered on other targets for SLE therapy. The authors discuss the benefits and shortcomings of focusing on T-cell-directed therapies in SLE and lupus nephritis clinical trials.

  20. Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives

    NARCIS (Netherlands)

    C. Magro-Checa (César); E.J. Zirkzee (Elisabeth J.); T.W.J. Huizinga (Tom); G.M. Steup-Beekman (G.)

    2016-01-01

    textabstractNeuropsychiatric systemic lupus erythematosus (NPSLE) is a generic definition referring to a series of neurological and psychiatric symptoms directly related to systemic lupus erythematosus (SLE). NPSLE includes heterogeneous and rare neuropsychiatric (NP) manifestations involving both t

  1. Cryptococcal meningitis in systemic lupus erythematosus patients : pooled analysis and systematic review

    NARCIS (Netherlands)

    Fang, Wenjie; Chen, Min; Liu, Jia; Hagen, Ferry; Ms, Abdullah; Al-Hatmi, A M S; Zhang, Peilian; Guo, Yun; Boekhout, Teun; Deng, Danqi; Xu, Jianping; Pan, Weihua; Liao, Wanqing

    2016-01-01

    Cryptococcal meningitis is an important fungal infection among systemic lupus erythematosus patients. We conducted a pooled analysis and systematic review to describe the epidemiological and clinical profile of cryptococcal meningitis in systemic lupus erythematosus patients. From two hospitals in C

  2. Systemic lupus erythematosus presenting with eosinophilic enteritis: a case report

    OpenAIRE

    Kalany Mohammad; Abdollahi Pejman; Asadi Gharabaghi Mehrnaz; Sotoudeh Masoud

    2011-01-01

    Abstract Introduction Systemic lupus erythematosus (SLE) is a multisystem disorder that may present with various symptoms. It may involve the gastrointestinal tract in a variety of ways; some of the most well-known ones are transaminitis, lupus mesenteric vasculitis, lupus enteritis and mesenteric vascular leakage. We describe a case of a patient with SLE who presented with a five-month history of diarrhea caused by eosinophilic enteritis. To the best of our knowledge, there are few cases rep...

  3. Toe walking as a presenting sign of systemic lupus erythematosus.

    Science.gov (United States)

    Basiaga, M; Sherry, D

    2015-10-01

    Toe walking is a previously unreported presentation of systemic lupus erythematosus (SLE). We describe a patient who presented with profound multisystem involvement that was preceded by one month of toe walking and multiple flexion contractures without arthritis. Her lupus is now under control after aggressive therapy, yet she continues to struggle with tendinopathy despite continued physical and occupational therapy. Lupus should be considered in the appropriate clinical context in children who have new-onset contractures due to tight tendons.

  4. Lupus erythematosus: considerations about clinical, cutaneous and therapeutic aspects*

    OpenAIRE

    Moura Filho,Jucelio Pereira; Peixoto,Raiza Luna; Martins,Livia Gomes; de Melo, Sillas Duarte; Carvalho,Ligiana Leite de; Pereira,Ana Karine F. da Trindade C.; Freire,Eutilia Andrade Medeiros

    2014-01-01

    Systemic Lupus Erythematosus is a chronic inflammatory disease with multifactorial etiology. Although clinical manifestations are varied, the skin is an important target-organ, which contributes to the inclusion of skin lesions in 4 out of the 17 new criteria for the diagnosis of the disease, according to the Systemic Lupus International Collaborating Clinics. The cutaneous manifestations of lupus are pleomorphic. Depending on their clinical characteristics, they can be classified into Acute ...

  5. Glaucoma secondary to systemic lupus erythematosus

    Institute of Scientific and Technical Information of China (English)

    Zhang Jing; Huang Wenbin; Gao Xinbo; Che Huixin; Yu Keming; Zhang Xiulan

    2014-01-01

    Background Glaucoma secondary to systemic lupus erythematosus (SLE) (") an uncommon but serious complication that threatens vision and therefore cannot be neglected.A few cases of secon(ca)ry glaucoma resulting from lupus-induced or iatrogenic ocular impairments have been reported in association with SLE.However,a systematic analysis of the diagnosis and treatment of glaucoma secondary to SLE has not been reported in the literature.The aim of this study is to further investigate the relationship between glaucoma and SLE.Methods In this study,we reviewed nine eyes of five patients diagnosed with secondary glaucoma associated with SLE,including one case of neovascular glaucoma and four cases of steroid-induced glaucoma.Results Neovascular glaucoma was successfully treated by Ahmed glaucoma valve implantation surgery with adjunctive ranibizumab intravitreal injection,followed by panretinal photocoagulation (PRP).The steroid-induced glaucoma in eight eyes of four cases were controlled by trabeculectomy along with antiproliferative agents.Conclusion Regular follow-up ocular examinations should be conducted to ensure early diagnosis and treatment of secondary glaucoma in SLE patients to improve the prognosis of vision.

  6. Periorbital discoid lupus: a rare localization in a patient with systemic lupus erythematosus.

    Science.gov (United States)

    Cakici, Ozgur; Karadag, Remzi; Bayramlar, Huseyin; Ozkanli, Seyma; Uzuncakmak, Tugba Kevser; Karadag, Ayse Serap

    2016-01-01

    A 40-year-old female patient with a 5-year history of systemic lupus erythematosus was referred to our policlinic with complaints of erythema, atrophy, and telangiectasia on the upper eyelids for 8 months. No associated mucocutaneous lesion was present. Biopsy taken by our ophthalmology department revealed discoid lupus erythematosus. Topical tacrolimus was augmented to the systemic therapeutic regimen of the patient, which consisted of continuous antimalarial treatment and intermittent corticosteroid drugs. We observed no remission in spite of the 6-month supervised therapy. Periorbital discoid lupus erythematosus is very unusual and should be considered in the differential diagnosis of erythematous lesions of the periorbital area..

  7. Periorbital discoid lupus: a rare localization in a patient with systemic lupus erythematosus*

    Science.gov (United States)

    Cakici, Ozgur; Karadag, Remzi; Bayramlar, Huseyin; Ozkanli, Seyma; Uzuncakmak, Tugba Kevser; Karadag, Ayse Serap

    2016-01-01

    A 40-year-old female patient with a 5-year history of systemic lupus erythematosus was referred to our policlinic with complaints of erythema, atrophy, and telangiectasia on the upper eyelids for 8 months. No associated mucocutaneous lesion was present. Biopsy taken by our ophthalmology department revealed discoid lupus erythematosus. Topical tacrolimus was augmented to the systemic therapeutic regimen of the patient, which consisted of continuous antimalarial treatment and intermittent corticosteroid drugs. We observed no remission in spite of the 6-month supervised therapy. Periorbital discoid lupus erythematosus is very unusual and should be considered in the differential diagnosis of erythematous lesions of the periorbital area..

  8. Systemic lupus erythematosus flare triggered by a spider bite.

    Science.gov (United States)

    Martín Nares, Eduardo; López Iñiguez, Alvaro; Ontiveros Mercado, Heriberto

    2016-01-01

    Systemic lupus erythematosus is a chronic autoimmune disease with a relapsing and remitting course characterized by disease flares. Flares are a major cause of hospitalization, morbidity and mortality in patients with systemic lupus erythematosus. Some triggers for these exacerbations have been identified, including infections, vaccines, pregnancy, environmental factors such as weather, stress and drugs. We report a patient who presented with a lupus flare with predominantly mucocutaneous, serosal and cardiac involvement after being bitten by a spider and we present the possible mechanisms by which the venom elicited such a reaction. To the best of our knowledge, this is the first such case reported in the literature.

  9. [Papular mucinosis associated with lupus erythematosus. A case presentation and review of the literature].

    Science.gov (United States)

    Revier, J; Kienzler, J L; Blanc, D; Coulon, G; Saint-Hillier, Y; Laurent, R

    1982-01-01

    A 36-year-old woman presented with a widespread papulonodular eruption followed by cutaneous and systemic manifestations of lupus erythematosus. Both conditions, papular mucinosis and lupus erythematosus were investigated by histopathology, immunofluorescence and electron microscopy. Referring to seven other cases reported in the literature, the possible relationship between lupus erythematosus and cutaneous mucinosis is discussed. It seemed that the cutaneous deposits of mucine were secondary to lupus erythematosus and not a simultaneous occurrence of the two diseases.

  10. Treatment Algorithms in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Muangchan, Chayawee; van Vollenhoven, Ronald F; Bernatsky, Sasha R; Smith, C Douglas; Hudson, Marie; Inanç, Murat; Rothfield, Naomi F; Nash, Peter T; Furie, Richard A; Senécal, Jean-Luc; Chandran, Vinod; Burgos-Vargas, Ruben; Ramsey-Goldman, Rosalind; Pope, Janet E

    2015-09-01

    To establish agreement on systemic lupus erythematosus (SLE) treatment. SLE experts (n = 69) were e-mailed scenarios and indicated preferred treatments. Algorithms were constructed and agreement determined (≥50% respondents indicating ≥70% agreement). Initially, 54% (n = 37) responded suggesting treatment for scenarios; 13 experts rated agreement with scenarios. Fourteen of 16 scenarios had agreement as follows: discoid lupus: first-line therapy was topical agents and hydroxychloroquine and/or glucocorticoids then azathioprine and subsequently mycophenolate (mofetil); uncomplicated cutaneous vasculitis: initial treatment was glucocorticoids ± hydroxychloroquine ± methotrexate, followed by azathioprine or mycophenolate and then cyclophosphamide; arthritis: initial therapy was hydroxychloroquine and/or glucocorticoids, then methotrexate and subsequently rituximab; pericarditis: first-line therapy was nonsteroidal antiinflammatory drugs, then glucocorticoids with/without hydroxychloroquine, then azathioprine, mycophenolate, or methotrexate and finally belimumab or rituximab, and/or a pericardial window; interstitial lung disease/alveolitis: induction was glucocorticoids and mycophenolate or cyclophosphamide, then rituximab or intravenous gamma globulin (IVIG), and maintenance followed with azathioprine or mycophenolate; pulmonary hypertension: glucocorticoids and mycophenolate or cyclophosphamide and an endothelin receptor antagonist were initial therapies, subsequent treatments were phosphodiesterase-5 inhibitors and then prostanoids and rituximab; antiphospholipid antibody syndrome: standard anticoagulation with/without hydroxychloroquine, then a thrombin inhibitor for venous thrombosis, versus adding aspirin or platelet inhibition drugs for arterial events; mononeuritis multiplex and central nervous system vasculitis: first-line therapy was glucocorticoids and cyclophosphamide followed by maintenance with azathioprine or mycophenolate, and

  11. Pregnancy and contraception in systemic and cutaneous lupus erythematosus.

    Science.gov (United States)

    Guettrot-Imbert, G; Morel, N; Le Guern, V; Plu-Bureau, G; Frances, C; Costedoat-Chalumeau, N

    2016-10-01

    A causal link has long been described between estrogen and systemic lupus erythematosus activity. Contraceptive and pregnancy management is now common for lupus patients, but pregnancy continues to be associated with higher maternal and fetal mortality/morbidity in systemic lupus erythematosus patients than among the general population. Potential complications include lupus flares, obstetric complications (fetal loss, in utero growth retardation, premature birth) and neonatal lupus syndrome. Association with antiphospholipid antibodies or antiphospholipid syndrome increases the risk of obstetric complications. Anti-SSA and/or anti-SSB antibodies put fetuses at risk for neonatal lupus. Improving the outcome of such pregnancies depends upon optimal systematic planning of pregnancy at a preconception counseling visit coupled with a multidisciplinary approach. Absence of lupus activity, use of appropriate medication during pregnancy based on the patient's medical history and risk factors, and regular monitoring constitute the best tools for achieving a favorable outcome in such high-risk pregnancies. The aim of this review is to provide an update on the management of contraception and pregnancy in systemic lupus erythematosus, cutaneous lupus and/or antiphospholipid syndrome in order to reduce the risk of complications and to ensure the best maternal and fetal prognosis.

  12. Renal biopsy in patients with systemic lupus erythematosus: Not just lupus glomerulonephritis!

    Science.gov (United States)

    Howell, David N

    2017-01-01

    Kidney biopsy is a mainstay in the diagnosis and management of renal disease in patients with systemic lupus erythematosus. Though biopsies from patients with lupus typically show various forms of immune complex glomerulonephritis, other pathologies are occasionally encountered, including unusual lupus-related nephropathies, other forms of autoimmune disease, and occasional renal disorders without any direct connection with lupus or autoimmunity. Electron microscopy is a powerful tool for detecting and classifying these unusual conditions, which frequently have important therapeutic and prognostic implications.

  13. Vesiculobullous systemic lupus erythematosus. A report of four cases.

    Science.gov (United States)

    Camisa, C

    1988-01-01

    A vesiculobullous eruption is now recognized as a specific but rare cutaneous complication of systemic lupus erythematosus. Four additional cases are reported in whom the five previously proposed criteria were met. Increased activity of systemic lupus erythematosus affecting other organ systems was documented in three of four cases. All four patients demonstrated a positive lupus band, and three of four showed granular deposits of IgA along the basement membrane zone (BMZ). Evidence of glomerulonephritis was obtained in three of four cases, which resulted in death in one. The higher than expected incidence of IgA deposits in skin and renal disease in patients with vesiculobullous eruption of systemic lupus erythematosus is again confirmed. The eruption cleared in all four cases with either dapsone, 50 mg daily, or high doses of corticosteroids and immunosuppressive agents.

  14. 75 FR 35493 - Guidance for Industry on Systemic Lupus Erythematosus-Developing Medical Products for Treatment...

    Science.gov (United States)

    2010-06-22

    ... Systemic Lupus Erythematosus--Developing Medical Products for Treatment; Availability AGENCY: Food and Drug... availability of a guidance for industry entitled ``Systemic Lupus Erythematosus--Developing Medical Products..., therapeutic biological products, and medical devices for the treatment of systemic lupus erythematosus (SLE...

  15. Lupus

    Science.gov (United States)

    What is lupus? Lupus is an autoimmune disease. This means that your immune system attacks healthy cells and tissues by mistake. This can ... vessels, and brain. There are several kinds of lupus Systemic lupus erythematosus (SLE) is the most common ...

  16. Role of Vitamin D in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Rada Miskovic

    2014-12-01

    Full Text Available Vitamin D is a steroid hormone that in addition to its well known role in the metabolism of calcium and phosphorus exerts immunoregulatory properties. Data from animal studies and from prospective clinical trials on patients with rheumatoid arthritis, multiple sclerosis and type 1 diabetes point to the potential role of vitamin D as important environmental factor in the development of autoimmune diseases. Such role of vitamin D in systemic lupus erythematosus (SLE has not yet been sufficiently studied. This review shows the sources, metabolism and mechanism of action of vitamin D, its effect on the cells of the immune system, prevalence and causes of vitamin D deficiency in patients with SLE, the link between vitamin D status and disease activity as well as recommendations for vitamin D supplementation.

  17. [Systemic lupus erythematosus and antiphospholipid syndrome: How to manage pregnancy?].

    Science.gov (United States)

    Guettrot-Imbert, G; Le Guern, V; Morel, N; Vauthier, D; Tsatsaris, V; Pannier, E; Piette, J-C; Costedoat-Chalumeau, N

    2015-03-01

    Pregnancy in systemic lupus erythematosus patients is a common situation that remains associated with higher maternal and fetal mortality/morbidity than in the general population. Complications include lupus flares, obstetrical complications (fetal loss, in utero growth retardation, prematurity) and neonatal lupus syndrome. The association with antiphospholipid antibodies or antiphospholipid syndrome increases the risk of obstetrical complications. Improving the care of these pregnancies depends upon a systematic pregnancy planning, ideally during a preconception counseling visit and a multidisciplinary approach (internist/rheumatologist, obstetrician and anesthetist). The absence of lupus activity, the use of appropriate medications during pregnancy adjusted to the patient's medical history and risk factors, and a regular monitoring are the best tools for a favorable outcome for these high-risk pregnancies. The aim of this review article is to perform an update on the medical care of pregnancy in systemic lupus erythematosus or antiphospholipid syndrome to reduce the risk of complications and to ensure the best maternal and fetal prognosis.

  18. An update on childhood-onset systemic lupus erythematosus.

    Science.gov (United States)

    Barsalou, Julie; Levy, Deborah M; Silverman, Earl D

    2013-09-01

    This manuscript will provide a review of studies published in the last year examining the major disease manifestations, comorbidities, biomarkers and therapeutic trials involving childhood-onset systemic lupus erythematosus (cSLE) patients. Recent multicenter prospective cohort studies supported previous findings that cSLE patients accrue damage early on their disease. Four studies showed that ethnicity altered disease severity and incidence of both cSLE and lupus nephritis. Description of clinical features and response to therapy in a large group of cSLE patients with neuropsychiatric involvement provided useful information on this feature. Advancement in the field of biomarkers was seen but the new biomarkers are not yet ready for clinical use. A randomized placebo-controlled trial of statins to prevent atherosclerosis progression did not meet its primary endpoint but did show a trend in improvement of carotid intima-media thickness, a surrogate marker of atherosclerosis. No other prospective interventional treatment trials designed specifically for cSLE patients were reported in the past year. There is an urgent need to better characterize the long-term outcome of cSLE patients and identify early on those at risk of a worse outcome. Advances in the field of biologics and small molecules will hopefully allow better targeted therapies of the cSLE population.

  19. Genome-Wide Association Study in African-Americans with Systemic Lupus Erythematosus

    Science.gov (United States)

    2013-09-01

    Americans with Systemic Lupus Erythematosus PRINCIPAL INVESTIGATOR: John Harley, M.D., Ph.D...September 2012 – 31 August 2013 4. TITLE AND SUBTITLE 5a. CONTRACT NUMBER Genome-Wide Association Study in African-Americans with Systemic Lupus ...SUPPLEMENTARY NOTES 14. ABSTRACT Systemic lupus erythematosus ( lupus ) is a potentially deadly systemic autoimmune disease that disproportionately

  20. Novel treatments for systemic lupus erythematosus.

    Science.gov (United States)

    Gayed, Mary; Gordon, Caroline

    2010-11-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease that is associated with the production of autoantibodies, and with considerable morbidity and mortality. There has been much interest in developing more specific therapies for this disease, which is currently managed with immunosuppressive drugs, predominantly corticosteroids, azathioprine, methotrexate and cyclophosphamide, in combination with hydroxychloroquine. Mycophenolate mofetil has been demonstrated to be as efficacious as cyclophosphamide in patients with lupus nephritis, and is being used increasingly in the clinic despite not being licensed for this indication. Novel methods of reducing autoantibody formation in SLE include the use of mAbs that modulate and/or deplete B-cells (anti-CD22 and anti-CD20 antibodies, respectively), or that interfere with the stimulatory effects of the soluble factor B-lymphocyte stimulator (anti-BLys antibodies). Alternative approaches include the use of atacicept (Merck Serono), a transmembrane activator and calcium modulator ligand interactor (TACI)-Ig fusion protein, which inhibits B-cell stimulation by binding to BLys and a profileration-inducing ligand (APRIL), or toleragens such as abetimus. Blocking costimulatory molecule interactions, such as the CD40-CD40 ligand interaction with mAbs and the CD28-B7 interaction with a soluble cytotoxic T-lymphocyte antigen 4 (CTLA-4)-IgG1 construct (abatacept), has also been attempted as a therapeutic strategy for SLE. The most promising strategy for a new drug for SLE is belimumab (Human Genome Sciences/GlaxoSmithKline), an anti-BLys antibody, as two phase III clinical trials with this drug recently met their primary endpoints. In this review, these novel approaches to the treatment of SLE, including the potential of targeting cytokine pathways involved in autoimmunity, are discussed.

  1. Value of multidisciplinary reassessment in attribution of neuropsychiatric events to systemic lupus erythematosus: prospective data from the Leiden NPSLE cohort.

    Science.gov (United States)

    Magro-Checa, César; Zirkzee, Els J; Beaart-van de Voorde, Liesbeth J J; Middelkoop, Huub A; van der Wee, Nic J; Huisman, Menno V; Eikenboom, Jeroen; Kruyt, Nyika D; van Buchem, Mark A; Huizinga, Tom W J; Steup-Beekman, Gerda M

    2017-10-01

    To determine the contribution of reassessment in the attribution process of neuropsychiatric (NP) events to SLE or other aetiologies in a large, prospective and multidisciplinary assessed NPSLE cohort and to compare these results with other available attribution models for NP events occurring in SLE. Three hundred and four consecutive SLE patients presenting NP events were evaluated. All subjects underwent standardized multidisciplinary medical, neuropsychological, laboratory and radiological examination on the inclusion and reassessment dates. Diagnosis was always established by multidisciplinary consensus. The final diagnosis after reassessment also took into account disease course and response to treatment. These data were compared with currently available attribution models for NP events in SLE. A total of 463 NP events were established. After reassessment, attribution to SLE was discordant in 64 (13.8%) NP events when compared with the first visit. We show that 14.5% of NP events previously attributed to SLE reclassified as non-NPSLE. In 86.4% of these patients immunosuppressive therapy was started after the first visit. When reassessment and available attribution models were compared, NPSLE cases overlapped considerably. Although specificity was high for all comparisons (0.81-0.95), an important variation in sensitivity (0.39-0.83) and agreement estimates (κ = 0.29-0.68) was observed. The Italian algorithm showed the highest sensitivity and specificity (>0.80) and moderate agreement (0.59-0.64). In clinical practice NP events presenting in SLE are too often attributed to an immune-mediated origin. Multidisciplinary reassessment avoids misclassification in NPSLE. Multidisciplinary reassessment is the reference standard in NP events presenting in SLE and cannot be replaced by available attribution models.

  2. Systemic lupus erythematosus and vitamin D

    Directory of Open Access Journals (Sweden)

    N. G. Klyukvina

    2015-01-01

    Full Text Available The review presents the data available in the literature on the rate of hypovitaminosis D in systemic lupus erythematosus (SLE, analyzes the associations between the clinical and laboratory parameters of the disease and the levels of vitamin D, and considers the possibilities of the therapeutic use of its metabolites.Vitamin D deficiency is a very common pathological condition that creates prerequisites for the development of a wide range of diseases. The low serum level of vitamin D may be associated with insufficient solar exposure, genetic predisposition (vitamin D receptor polymorphism, and alimentary factors and may accompany autoimmune diseases. The very recently revealed immunomodulatory properties of vitamin D are of interest with respect to the possible implication of this hormone in the pathogenesis of autoimmune (including rheumatic diseases. A number of investigators propose to regard vitamin D as a modifying environmental factor involved in the development of autoimmune diseases. There is evidence for the association of low serum 25(ОНD levels with a risk for some rheumatic diseases (primarily rheumatoid arthritis and SLE, their activity, severity, and prognosis, which calls for further investigation. The antiresorptive, anti-inflammatory, and immunomodulatory effects of vitamin D metabolites substantiate that the latter should be used in combination with traditional disease-modifying agents to treatchronic inflammatory diseases.

  3. Psychiatric disorders in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Hutchinson, G A; Nehall, J E; Simeon, D T

    1996-06-01

    The symptoms of Systemic Lupus Erythematosus (SLE) may include altered mental function. The present study sought to determine whether the psychiatric disorders are due to the disease itself or to the stress of having a chronic disease. Forty-five SLE patients attending outpatient clinics at the Port-of-Spain General Hospital in Trinidad were compared with two control groups: patients with chronic debilitating diseases similar to SLE in terms of chronicity and treatment (n = 44) and non-diseased individuals (n = 48). The Structured Clinical Interview for DSM III-R was used to identify psychiatric disorders. Both the SLE and the chronic illness groups had more psychiatric illness (44% and 39%, respectively) when compared with the non-diseased controls (2%) (p < 0.001). Major depression was the most common diagnosis among both diseased groups. However, psychotic illnesses (schizophrenic-type psychosis and bipolar disorders) were more prevalent in the SLE group (11.1% vs 0%, p = 0.02). These results indicate that major depression in SLE may be related more to the effects of a chronic illness than to SLE itself. However, the occurrence of psychotic symptoms may be related to SLE disease and needs further study.

  4. Biotherapies in systemic lupus erythematosus: New targets.

    Science.gov (United States)

    Lazaro, Estibaliz; Scherlinger, Marc; Truchetet, Marie-Elise; Chiche, Laurent; Schaeverbeke, Thierry; Blanco, Patrick; Richez, Christophe

    2016-09-20

    Systemic lupus erythematosus (SLE) is an autoimmune disease with a polymorphic presentation. The variability in the clinical expression and severity of SLE makes new treatments both essential and challenging to develop. Several biotherapies targeting different pathophysiological pathways have been developed over the past 15 years. The results of Phase II trials were encouraging but rarely borne out by Phase III trials. Recent data, which are discussed in detail in this review, allowed belimumab - a monoclonal antibody against BLyS (B-lymphocyte stimulator) - to become the first biotherapy approved for use in SLE. Other molecules targeting B cells include the two anti-BLyS antibodies tabalumab and blisibimod; atacicept, which targets both BLyS and APRIL (a proliferation-inducing ligand); and the monoclonal antibody to CD22 epratuzumab. The rekindling of interest in the B-cell pathway has also driven new clinical research into rituximab, a monoclonal antibody targeting CD20 with evaluations of new strategies. A new and promising approach is the use of inhibitors of the type 1 interferon (IFN) pathway, of which the most promising is anifrolumab, a monoclonal antibody targeting the type 1 IFN receptor. In this review, we discuss study findings and their clinical relevance, present the most promising targets, and analyze possible explanations to negative results, such as inappropriate patient selection and treatment response criteria or the erratic use of high-dose glucocorticoid therapy.

  5. Unmet medical needs in systemic lupus erythematosus

    Science.gov (United States)

    2012-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease of diverse manifestations, with onset usually in young women in the third to fourth decade of life. The chronic nature of this relapsing remitting disease leads to organ damage accrual over time. Mortality and morbidity are increased in patients with SLE compared with the general population. Therapeutic advances over the last few decades have led to significant improvements in patient outcomes. Five-year survival has improved to over 90% from a low of 50% in the 1950s. However, multiple aspects of the management of SLE patients are still far from optimal. Early diagnosis remains a challenge; diagnostic delays leading to delay in definitive treatment are common. Monitoring treatment remains problematic due to the paucity of sensitive biomarkers. Current treatment regimens rely heavily on corticosteroids, even though corticosteroids are well known to cause organ damage. Treatment of refractory disease manifestations such as nephritis, recalcitrant cutaneous lesions and neurological involvement require new approaches with greater efficacy. Cognitive dysfunction is common in SLE patients, but early recognition and adequate treatment are yet to be established. Premature accelerated atherosclerosis remains a leading cause of morbidity and mortality. Fatigue is one of the most disabling symptoms, and contributes to the poor quality of life in patients with SLE. Ongoing research in SLE faces many challenges, including enrollment of homogeneous patient populations, use of reliable outcome measures and a standard control arm. The current review will highlight some of the outstanding unmet challenges in the management of this complex disease. PMID:23281889

  6. Juvenile systemic lupus erythematosus in Nigeria.

    Science.gov (United States)

    Adelowo, O O; Olaosebikan, B H; Animashaun, B A; Akintayo, R O

    2017-03-01

    Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disorder of unknown cause. It accounts for about one in five cases of SLE. The tendency for SLE to run a fulminant course when it starts in childhood has made JSLE a potentially more severe disease than adult SLE. Reports of JSLE from sub-Saharan Africa are scanty in spite of the increasing reports of adult SLE. We conducted a 4-year retrospective study of JSLE cases seen at the Lagos State University Teaching Hospital between January 2010 and December 2014. Out of the 12 patients studied, eight were girls and four were boys. All patients had positive antinuclear antibody and extractable nuclear antibody tests. Anti-dsDNA antibody was positive in 10 patients. Eight patients had renal disease while four patients had neuropsychiatric manifestations. Haematological abnormalities and constitutional symptoms were present in all patients. Patients were treated with pulse methylprednisolone, oral prednisolone, hydroxychloroquine and azathioprine. Three patients also received rituximab. In conclusion, JSLE exists in Nigeria and exhibits clinical and immunological characteristics similar to its pattern in other parts of the world. It is, however, diagnosed late and is possibly being underdiagnosed as there is no paediatric rheumatologist in the country.

  7. Incidence of cervical human papillomavirus infection in systemic lupus erythematosus women.

    Science.gov (United States)

    Mendoza-Pinto, C; García-Carrasco, M; Vallejo-Ruiz, V; Méndez-Martínez, S; Taboada-Cole, A; Etchegaray-Morales, I; Muñóz-Guarneros, M; Reyes-Leyva, J; López-Colombo, A

    2017-08-01

    Objectives Our objective was to study the incidence, persistence and clearance of human papillomavirus infection in systemic lupus erythematosus women and assess risk factors for persistence of human papillomavirus infection. Methods We carried out a prospective, observational cohort study of 127 systemic lupus erythematosus women. Patients were evaluated at baseline and at three years. Traditional and systemic lupus erythematosus women-related disease risk factors were collected. Gynaecological evaluations and cervical cytology screening were made. Human papillomavirus detection and genotyping were made by polymerase chain reaction and linear array. Results The cumulative prevalence of human papillomavirus infection increased from 22.8% at baseline to 33.8% at three years; p = systemic lupus erythematosus women, the cumulative prevalence of human papillomavirus infection, including high risk-human papillomavirus and multiple human papillomavirus infections, may increase over time. Most persistent infections were low risk-human papillomavirus. The number of lifetime sexual partners and the cumulative cyclophosphamide dose were independently associated with incident human papillomavirus infection.

  8. Pregnancy complications in a patient with systemic lupus erythematosus and lupus nephritis

    DEFF Research Database (Denmark)

    Bisgaard, Helene; Jacobsen, Søren; Tvede, Niels

    2014-01-01

    A woman with systemic lupus erythematosus (SLE) and lupus nephritis had two pregnancies which both resulted in complications known to be associated with SLE, i.e. late abortion, preterm delivery and pre-eclampsia. We conclude that disease quiescence is important for a successful outcome...

  9. [Pregnancy complications in a patient with systemic lupus erythematosus and lupus nephritis].

    Science.gov (United States)

    Bisgaard, Helene; Jacobsen, Søren; Tvede, Niels; Langhoff-Roos, Jens

    2014-07-14

    A woman with systemic lupus erythematosus (SLE) and lupus nephritis had two pregnancies which both resulted in complications known to be associated with SLE, i.e. late abortion, preterm delivery and pre-eclampsia. We conclude that disease quiescence is important for a successful outcome and that pregnant women with SLE should be followed in a multidisciplinary setting.

  10. Graviditetskomplikationer hos en patient med systemisk lupus erythematosus og lupus nefritis

    DEFF Research Database (Denmark)

    Bisgaard, Helene; Jacobsen, Søren; Tvede, Niels

    2014-01-01

    A woman with systemic lupus erythematosus (SLE) and lupus nephritis had two pregnancies which both resulted in complications known to be associated with SLE, i.e. late abortion, preterm delivery and pre-eclampsia. We conclude that disease quiescence is important for a successful outcome...

  11. Ethylenediaminetetraacetic acid-dependent pseudothrombocytopenia in a patient with systemic lupus erythematosus and lupus nephritis

    OpenAIRE

    Akyol, Lütfi; Önem, Soner; Özgen, Metin; Sayarlıoğlu, Mehmet

    2015-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by several immunological abnormalities. We wish to communicate the case of a patient with SLE and lupus nephritis (LN) who developed pseudothrombocytopenia. Pseudothrombocytopenia can occur in patients with SLE and LN and should be considered when diagnosing patients with thrombocytopenia without bleeding.

  12. Therapeutic strategies evaluated by the European Society of Cutaneous Lupus Erythematosus (EUSCLE) Core Set Questionnaire in more than 1000 patients with cutaneous lupus erythematosus.

    Science.gov (United States)

    Sigges, Johanna; Biazar, Cyrus; Landmann, Aysche; Ruland, Vincent; Patsinakidis, Nikolaos; Amler, Susanne; Bonsmann, Gisela; Kuhn, Annegret

    2013-05-01

    The aim of this prospective, cross-sectional, multicentre study performed by the European Society of Cutaneous Lupus Erythematosus (EUSCLE) was to investigate different therapeutic strategies and their efficacies in cutaneous lupus erythematosus (CLE) throughout Europe. Using the EUSCLE Core Set Questionnaire, topical and systemic treatment options were analysed in a total of 1002 patients (768 females and 234 males) with different CLE subtypes. The data were correlated with the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and the criteria of the American College of Rheumatology (ACR) for the classification of systemic lupus erythematosus. Sunscreens were applied by 84.0% of the study cohort and showed a high efficacy in preventing skin lesions in all disease subtypes, correlating with a lower CLASI activity score. Topical steroids were used in 81.5% of the patients, with an efficacy of 88.4%, whereas calcineurin inhibitors were applied in 16.4% of the study population and showed an efficacy of 61.7%. Systemic agents including antimalarials and several immunomodulating/-suppressive drugs, such as systemic steroids and methotrexate, were applied in 84.4% of the 1002 patients. The CLASI activity and damage score was higher in treated CLE patients compared to untreated patients, regardless of therapy with topical or systemic agents. In summary, preventive and therapeutic strategies of 1002 patients with different subtypes of CLE were analysed in this prospective, multicentre, Europe-wide study. Sunscreens were confirmed to be successful as preventive agents, and topical steroids showed a high efficacy, whereas antimalarials were used as first-line systemic treatment.

  13. Extracellular Vesicles as Biomarkers of Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Javier Perez-Hernandez

    2015-01-01

    Full Text Available Systemic lupus erythematosus is an autoimmune disease that predominantly affects women and typically manifests in multiple organs. The damage caused by this disorder is characterized by a chronic inflammatory state. Extracellular vesicles (EVs, including microvesicles (also known as microparticles, apoptotic bodies, and exosomes, are recognized vehicles of intercellular communication, carrying autoantigens, cytokines, and surface receptors. Therefore, the evidence of EVs and their cargo as biomarkers of autoimmune disease is rapidly expanding. This review will focus on biogenesis of extracellular vesicles, their pathophysiological roles, and their potential as biomarkers and therapeutics in inflammatory disease, especially in systemic lupus erythematosus.

  14. Coincident systemic lupus erythematosus and psoriasis vulgaris: a case report.

    Science.gov (United States)

    Wang, Y; Da, G; Yu, Y; Han, J; Li, H

    2015-12-01

    Psoriasis vulgaris is an autoimmune chronic inflammatory skin disease, but its association with other typical autoimmune disease such as systemic lupus erythematosus has only occasionally been reported. We presented a 25-year-old female who developed systemic lupus erythematosus associated with psoriasis vulgaris. Her conditions were in good control after she got administration of prednisolone (5 mg/day) and Tripterygium Wilfordii Hook (20 mg/day). It is necessary to integrate past history and physical examination to diagnose coincident SLE and psoriasis, and combined treatment with prednisolone and Tripterygium Wilfordii Hook proves effective.

  15. Depressive symptoms and associated factors in systemic lupus erythematosus.

    Science.gov (United States)

    Karol, David E; Criscione-Schreiber, Lisa G; Lin, Min; Clowse, Megan E B

    2013-01-01

    Depressive symptoms affect anywhere from 11% to 71% of patients with systemic lupus erythematosus (SLE), which may be related to SLE disease activity, other clinical variables, or sociodemographic factors. We aimed to measure the rate of depressive symptoms in our cohort of patients with SLE and to identify modifiable factors associated with depressive symptoms. Patients in our university-based SLE registry completed the Beck Depression Inventory-II (BDI-II), pain scores, and demographic information. Disease activity was measured using the physician's global assessment (PGA) and Selena-SLE disease activity index (Selena-systemic lupus erythematosus disease activity index (SLEDAI)). Patients were identified as having moderate or severe depressive symptoms (BDI-II ≥ 18) or not (BDI-II lupus arthritis (P lupus arthritis, may result in alleviation of depressive symptoms in patients with SLE. Copyright © 2013 The Academy of Psychosomatic Medicine. Published by Elsevier Inc. All rights reserved.

  16. Review of classification criteria for systemic lupus erythematosus.

    Science.gov (United States)

    Petri, Michelle

    2005-05-01

    The American College of Rheumatology classification criteria were devised in 1982. In 1997, the immunologic disorder criterion was revised by a committee (without validation). All 11 criteria in the American College of Rheumatology criteria set have limitations. One of the most important laboratory tests, hypocomplementemia, was excluded entirely. Other classification criteria, emphasizing weighting or recursive partitioning, exist, but they are more cumbersome. Revised criteria are needed, not just for systemic lupus erythematosus, but also for chronic cutaneous lupus.

  17. B cell depleting therapy in systemic lupus erythematosus

    OpenAIRE

    Jónsdóttir, Thórunn

    2009-01-01

    Systemic Lupus Erythematosus (SLE) is a chronic autoimmune inflammatory disease characterized by multiple organ involvement, production of a wide range of autoantibodies and local formation or tissue deposition of immune complexes in the affected organs. Lupus nephritis (LN) is a common and serious organ involvement in patients with SLE. Despite better treatment and care of patients during the last decades there is still a great unmet need in the treatment with many patients...

  18. Validation of the LupusPRO in Chinese patients from Hong Kong with systemic lupus erythematosus.

    Science.gov (United States)

    Mok, Chi Chiu; Kosinski, Mark; Ho, Ling Yin; Chan, Kar Li; Jolly, Meenakshi

    2015-02-01

    LupusPRO is a disease-targeted, patient-reported outcome (PRO) measure that was developed and validated for assessment of quality of life in US patients with systemic lupus erythematosus (SLE). We present results of adapting the LupusPRO into Chinese and testing its psychometric properties in Chinese patients with SLE. LupusPRO was translated into "traditional" Chinese, followed by pretesting among native Cantonese Chinese speakers. The translation version was revised based on the feedback obtained. The Chinese language LupusPRO tool was administered along with a generic PRO tool (the Short Form 36 health survey [SF-36]) to ethnic Chinese SLE patients. At the same time, demographic information, clinical data, disease activity (Safety of Estrogens in Lupus Erythematosus National Assessment [SELENA] version of the Systemic Lupus Erythematosus Disease Activity Index [SLEDAI]), and damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index [SDI]) were obtained. We performed confirmatory factor analysis of the Chinese LupusPRO and evaluated internal consistency reliability, as well as convergent and criterion validity. Among the 463 SLE patients (95% women) with a mean ± SD age of 42.3 ± 13.5 years, the mean ± SD physician global assessment score was 0.48 ± 0.45, the mean ± SD SELENA-SLEDAI score was 2.9 ± 3.0, and the mean ± SD SDI score was 0.7 ± 1.2. Results of factor analysis conformed to the original LupusPRO model with only minor modifications. The reliability of the LupusPRO domains ranged from 0.60-0.94. LupusPRO domains had correlations as expected with the corresponding SF-36 domains. A significant but weak correlation with disease activity was noted for criterion validity as expected. The Chinese language LupusPRO has fair psychometric properties and may be used in SLE clinical trials. Copyright © 2015 by the American College of Rheumatology.

  19. Systemic lupus erythematosus associated with Wells' syndrome.

    Science.gov (United States)

    Yin, Geng; Xie, Qibing

    2012-04-01

    Wells' syndrome is a multifaceted dermatosis with a wide morphological spectrum, ranging from characteristic cellulitis-like erythema and papula to an unusual presentation of vesicles and pustules. The most important elements for diagnosis are erythemal plaques and histological picture of eosinophilic infiltration of the dermis with 'flame figures' (Plotz et al., in Hautarzt 51:182-186, 2000). Because of its original description as a distinct entity, it has come to be regarded as an abnormal eosinophilic response to a number of causative agents such as herpes simplex virus 2(HSV-2) and toxocara (Ludwig et al., in J Am Acad Dermatol 48:S60-S61, 2003; Bassukas et al., in Cases J 1:356, 2008). Concurrence of WS and malignant diseases as colon cancer, trachea squamous carcinoma, nasopharyngeal carcinoma or angioimmunoblastic lymphadenopathy has been reported (Hirsch et al., in J Dtsch Dermatol Ges 3:530-531, 2005; Renner et al., in Acta Derm Venereol 87:525-528, 2007). Autoimmune diseases, including Systemic lupus erythematosus (SLE) are multi-system disorders of unknown cause and are commonly characterized by protean cutaneous manifestations. To date, few autoimmne disease was found associated with WS except four previous reports of Churg-Strauss syndrome (CSS) and one case of ulcerative colitis (Fujimoto et al., in Clin Exp Dermatol, 2010; Sakaria et al., in J Gastroenterol 42:250-252, 2007). The coexistence of SLE and WS in one patient was not found in literature and our case is the first. Here we described the rare combination and discussed the treatment strategy for this condition.

  20. Bone Marrow Involvement in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Chalayer, Emilie; Costedoat-Chalumeau, Nathalie; Beyne-Rauzy, Odile; Ninet, Jacques; Durupt, Stephane; Tebib, Jacques; Asli, Bouchra; Lambotte, Olivier; Ffrench, Martine; Vasselon, Christian; Cathébras, Pascal

    2017-05-19

    Besides peripheral cytopenias, bone marrow abnormalities, such as fibrosis, pure red cell aplasia, and aplastic anemia have been reported in patients with systemic lupus erythematosus (SLE), suggesting that bone marrow may be a target organ in SLE. Our objective was to describe this bone marrow involvement. This registry is a nationwide retrospective study. Centers provided data concerning medical history, SLE manifestations, type of hematologic disorder, treatments and outcome. Bone marrow aspirations and/or biopsies were transferred for centralized review. Thirty patients from 19 centers were included. Central hematologic manifestations comprised bone marrow fibrosis (n=17; 57%), pure red cell aplasia (n=8; 27%), myelodysplastic syndrome (n=3; 10%), aplastic anemia and agranulocytosis (n=1; 3% each). Bone marrow involvement was diagnosed concomitantly with SLE in 12 patients. Bone marrow biopsies showed fibrosis in 19 cases, including one case of pure red cell aplasia and one case of agranulocytosis and variable global marrow cellularity. Treatments included corticosteroids (90%), hydroxychloroquine (87%), rituximab (33%), intravenous immunoglobulins (30%), mycophenolate mofetil (20%) and ciclosporine (20%). After a median follow-up of 27 months (range: 1-142), 24 patients manifested complete improvement. No patient died. This registry comprises the largest series of SLE patients with bone marrow involvement. It demonstrates the strong link between SLE and bone marrow fibrosis. Patients with atypical or refractory cytopenia associated with SLE should undergo bone marrow examination to enable appropriate, and often effective, treatment. Long-term prognosis is good. © The Author 2017. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  1. Novel approaches to therapy for systemic lupus erythematosus: update 2005.

    Science.gov (United States)

    Zandman-Goddard, Gisele; Orbach, Hedi; Shoenfeld, Yehuda

    2005-07-01

    This review covers the major advances in the therapeutic potentials related to systemic lupus erythematosus published in Medline between 2000 and February 2005. Controlled, open and Phase I-III trials were included. Anecdotal reports were excluded. Several trials have defined the role of cyclophosphamide, methotrexate, antimalarials, hormonal treatment and mycophenolate mofetil (Cellcept) in the management of systemic lupus erythematosus. The aims of novel biologics for systemic lupus erythematosus are to target the autoimmune disease at different points: B-cell depletion (rituximab [Rituxan], anti-BLys antibodies [Lymphostat-B]), inhibition of T-B interaction (rituximab), blockade of cytokines (anti-interleukin-10 antibodies), manipulation of idiotypes (intravenous immunoglobulin), tolerance induction to DNA and immunoglobulin-peptides and peptide therapy (abetimus sodium [Riquent]). Low-dose intravenous cyclophosphamide (Euro-Lupus protocol) is as effective as the conventional National Institutes of Health protocol and is also associated with less toxicity. Stem cell transplantation for severe disease induces remission in most patients, however, the relapse rate in a third of patients and the associated morbity and mortality restricts its use to selected patients with life-threatening disease. Intravenous immunoglobulin, although utilized in open trials, is effective and safe for various manifestations of systemic lupus erythematosus. Major advances have been associated with mycophenolate mofetil and rituximab. Mycophenolate mofetil is effective for induction and maintenance therapy of lupus proliferative glomerulonephritis and is associated with fewer adverse events than monthly intravenous cyclophosphamide. Rituximab is a promising agent, and although its utilization is presently limited, it appears to be effective for lupus patients with severe disease.

  2. Pulmonary arterial hypertension as a manifestation of lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Stark, P.; Sargent, E.N.; Boylen, T.; Jaramillo, D.

    1987-08-01

    We present five patients with systemic lupus erythematosus (SLE) who developed pulmonary arterial hypertension and cor pulmonale in the course of their disease. The clinical features, as well as, the radiological manifestations of this rare manifestation of SLE are discussed. A vasculitic process is the most likely cause of this complication. Therapy is ineffective and the prognosis is poor.

  3. Influenza vaccination in systemic lupus erythematosus : Safe and protective?

    NARCIS (Netherlands)

    Holvast, Bert; Huckriede, Anke; Kallenberg, Cees G. M.; Bijl, Marc

    2007-01-01

    Patients with systemic lupus erythematosus (SLE) show decreased immune responsiveness and are vulnerable for infectious diseases, due to the underlying disease and the frequent use of immunosuppressive drugs. Influenza has a high incidence in the population and is associated with increased morbidity

  4. Aquaporin-4-autoimmunity in patients with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Asgari, Nasrin; Jarius, Sven; Laustrup, Helle

    2017-01-01

    BACKGROUND: Serum immunoglobulin G targeting the astrocyte water channel aquaporin-4 (AQP4) in the central nervous system (CNS) is a biomarker for neuromyelitis optica spectrum disease (NMOSD). Co-existence of NMOSD with systemic lupus erythematosus (SLE) putatively suggests susceptibility to ant...

  5. Acute Pancreatitis as the Initial Presentation of Systematic Lupus Erythematosus

    OpenAIRE

    Yi Jia; Arleen Ortiz; Richard Mccallum; Hasan Salameh; Pedro Serrato

    2014-01-01

    Systematic lupus erythematosus (SLE) is a multisystem disease, including the gastrointestinal system in about half of SLE patients. As a rare complication of SLE, acute pancreatitis presents as generalized flare-ups in most cases of patients previously diagnosed with SLE. Here we report a rare case of acute pancreatitis as the initial presentation with later diagnosis of SLE.

  6. Acute Pancreatitis as the Initial Presentation of Systematic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Yi Jia

    2014-01-01

    Full Text Available Systematic lupus erythematosus (SLE is a multisystem disease, including the gastrointestinal system in about half of SLE patients. As a rare complication of SLE, acute pancreatitis presents as generalized flare-ups in most cases of patients previously diagnosed with SLE. Here we report a rare case of acute pancreatitis as the initial presentation with later diagnosis of SLE.

  7. Transancestral mapping and genetic load in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Langefeld, Carl D; Ainsworth, Hannah C; Graham, Deborah S Cunninghame

    2017-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with marked gender and ethnic disparities. We report a large transancestral association study of SLE using Immunochip genotype data from 27,574 individuals of European (EA), African (AA) and Hispanic Amerindian (HA) ancestry. We identify...

  8. Circulating surfactant protein D is decreased in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Hoegh, Silje Vermedal; Voss, Anne; Sorensen, Grith Lykke

    2009-01-01

    Objective. Deficiencies of innate immune molecules like mannan binding lectin (MBL) have been implicated in the pathogenesis of systemic lupus erythematosus (SLE). Surfactant protein D (SP-D) and MBL belong to the same family of innate immune molecules - the collectins, which share important...

  9. Crusted scabies in a chid with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Nurimar C.F. Wanke

    1992-03-01

    Full Text Available A child with systemic lupus erythematosus who has been treated with prednisone for three years, developed crusted scabies. Scrapings from lesions revealed Sarcoptes scabiei adult mites mad eggs. The patient died with septicemia and renal failure soon after starting topical 20% sulfur. A marked improvement was observed in the cutaneous lesions.

  10. Treat-to-target in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Mosca, Marta; Boumpas, Dimitrios T; Bruce, Ian N

    2013-01-01

    on May 8, 2012 to discuss whether a treat-to-target approach could be applied in the treatment of systemic lupus erythematosus (SLE) (T2T/SLE), define a research agenda, and establish a plan for moving forward. In the present paper, observations raised at the meeting and literature data on potential...

  11. Systemic lupus erythematosus: A possible cause of non-alcoholic ...

    African Journals Online (AJOL)

    We report a young woman with systemic lupus erythematosus (SLE) and an ... The clinical triad of mild confusion, ataxia and ophthalmoplegia also raised ... The diagnosis of WE was further supported by the magnetic resonance imaging features. ... Gastrointestinal manifestations of SLE are described as being common in ...

  12. Treat-to-target in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Mosca, Marta; Boumpas, Dimitrios T; Bruce, Ian N

    2013-01-01

    on May 8, 2012 to discuss whether a treat-to-target approach could be applied in the treatment of systemic lupus erythematosus (SLE) (T2T/SLE), define a research agenda, and establish a plan for moving forward. In the present paper, observations raised at the meeting and literature data on potential...

  13. Transancestral mapping and genetic load in systemic lupus erythematosus

    NARCIS (Netherlands)

    Langefeld, Carl D.; Ainsworth, Hannah C.; Graham, Deborah S. Cunninghame; Kelly, Jennifer A.; Comeau, Mary E.; Marion, Miranda C.; Howard, Timothy D.; Ramos, Paula S.; Croker, Jennifer A.; Morris, David L.; Sandling, Johanna K.; Almlof, Jonas Carlsson; Acevedo-Vasquez, Eduardo M.; Alarcon, Graciela S.; Babini, Alejandra M.; Baca, Vicente; Bengtsson, Anders A.; Berbotto, Guillermo A.; Bijl, Marc; Brown, Elizabeth E.; Brunner, Hermine I.; Cardiel, Mario H.; Catoggio, Luis; Cervera, Ricard; Cucho-Venegas, Jorge M.; Dahlqvist, Solbritt Rantapaa; D'Alfonso, Sandra; Da Silva, Berta Martins; de la Rua Figueroa, Inigo; Doria, Andrea; Edberg, Jeffrey C.; Endreffy, Emoke; Esquivel-Valerio, Jorge A.; Fortin, Paul R.; Freedman, Barry I.; Frostegard, Johan; Garcia, Mercedes A.; Garcia de la Torre, Ignacio; Gilkeson, Gary S.; Gladman, Dafna D.; Gunnarsson, Iva; Guthridge, Joel M.; Huggins, Jennifer L.; James, Judith A.; Kallenberg, Cees G. M.; Kamen, Diane L.; Karp, David R.; Kaufman, Kenneth M.; Kottyan, Leah C.; Kovacs, Laszlo; Laustrup, Helle; Lauwerys, Bernard R.; Li, Quan-Zhen; Maradiaga-Cecena, Marco A.; Martin, Javier; McCune, Joseph M.; McWilliams, David R.; Merrill, Joan T.; Miranda, Pedro; Moctezuma, Jose F.; Nath, Swapan K.; Niewold, Timothy B.; Orozco, Lorena; Ortego-Centeno, Norberto; Petri, Michelle; Pineau, Christian A.; Pons-Estel, Bernardo A.; Pope, Janet; Raj, Prithvi; Ramsey-Goldman, Rosalind; Reveille, John D.; Russell, Laurie P.; Sabio, Jose M.; Aguilar-Salinas, Carlos A.; Scherbarth, Hugo R.; Scorza, Raffaella; Seldin, Michael F.; Sjowall, Christopher; Svenungsson, Elisabet; Thompson, Susan D.; Toloza, Sergio M. A.; Truedsson, Lennart; Tusie-Luna, Teresa; Vasconcelos, Carlos; Vila, Luis M.; Wallace, Daniel J.; Weisman, Michael H.; Wither, Joan E.; Bhangale, Tushar; Oksenberg, Jorge R.; Rioux, John D.; Gregersen, Peter K.; Syvanen, Ann-Christine; Ronnblom, Lars; Criswell, Lindsey A.; Jacob, Chaim O.; Sivils, Kathy L.; Tsao, Betty P.; Schanberg, Laura E.; Behrens, Timothy W.; Silverman, Earl D.; Alarcon-Riquelme, Marta E.; Kimberly, Robert P.; Harley, John B.; Wakeland, Edward K.; Graham, Robert R.; Gaffney, Patrick M.; Vyse, Timothy J.

    2017-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with marked gender and ethnic disparities. We report a large transancestral association study of SLE using Immunochip genotype data from 27,574 individuals of European (EA), African (AA) and Hispanic Amerindian (HA) ancestry. We identify 58

  14. Acute pancreatitis as the initial presentation of systematic lupus erythematosus.

    Science.gov (United States)

    Jia, Yi; Ortiz, Arleen; Mccallum, Richard; Salameh, Hasan; Serrato, Pedro

    2014-01-01

    Systematic lupus erythematosus (SLE) is a multisystem disease, including the gastrointestinal system in about half of SLE patients. As a rare complication of SLE, acute pancreatitis presents as generalized flare-ups in most cases of patients previously diagnosed with SLE. Here we report a rare case of acute pancreatitis as the initial presentation with later diagnosis of SLE.

  15. Total lymphoid irradiation in refractory systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Ben-Chetrit, E.; Gross, D.J.; Braverman, A.; Weshler, Z.; Fuks, Z.; Slavin, S.; Eliakim, M.

    1986-07-01

    In two patients with systemic lupus erythematosus, conventional therapy was considered to have failed because of persistent disease activity and unacceptable side effects. Both were treated with total lymphoid irradiation without clinical benefit, despite adequate immunosuppression as documented by markedly reduced numbers of circulating T lymphocytes and T-lymphocyte-dependent proliferative responses in vitro. The first patient developed herpes zoster, gram-negative septicemia, neurologic symptoms, and deterioration of lupus nephritis. The second patient developed massive bronchopneumonia, necrotic cutaneous lesions, and progressive nephritis and died 2 weeks after completion of radiotherapy. These observations, although limited to two patients, indicate that total lymphoid irradiation in patients with severe systemic lupus erythematosus should be regarded as strictly experimental.

  16. Chinese Herbal Medicine (Zi Shen Qing for Mild-to-Moderate Systematic Lupus Erythematosus: A Pilot Prospective, Single-Blinded, Randomized Controlled Study

    Directory of Open Access Journals (Sweden)

    Linda L. D. Zhong

    2013-01-01

    Full Text Available Objective. The aim of this study is to investigate the effectiveness and safety of a Chinese herbal formula Zi Shen Qing (ZSQ in the treatment of systematic lupus erythematosus (SLE in Chinese patients. Methods. A randomized controlled trial was conducted over 12 weeks in 84 Chinese patients who reported total scores of SLE Disease Activity Index-2000 (SLEDAI-2000 was from 5 to 14. The primary outcome was the changes of the SLEDAI-2000. The secondary outcomes included score changes of Chinese Medicine Syndromes (CMS, the changes of steroid dosage, the incidence of disease flare-up and biologic markers. Results. ZSQ significantly reduced SLEDAI-2000, the total scores of CMS in the treatment group compared with the controlled group (P<0.05. Superiority of ZSQ over controlled group was also observed with greater improvement in the withdrawal dosage of corticosteroids and the incidence of disease flare-up (P<0.05. There were no serious adverse events, and safety indices of whole blood counts, renal and liver functions were normal, both before and after the treatment. Conclusion. ZSQ is safe and effective for decreasing SLE disease activity and withdrawal dosage of corticosteroids in the mild to moderate SLE patients with “Deficiency of Qi and Yin” Pattern.

  17. Noninvasive cardiac assessment in children of women with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Maria de Fátima Monteitro Pereira Leite

    2003-11-01

    Full Text Available OBJECTIVE: Noninvasive cardiac assessment of newborns and infants of women with systemic lupus erythematosus. The children had no congenital total atrioventricular block and were compared with the children of healthy women. METHODS: We prospectively assessed 13 newborns and infants aged 1 to 60 days, children of women with systemic lupus erythematosus and without congenital total atrioventricular block. These children were compared with 30 children of women who had no lupus or anti-Ro/SSA antibodies, and no risk factors for congenital heart disease either. Their age groups matched. The following examinations were performed: cardiological physical examination, electrocardiography, echocardiography, and signal-averaged electrocardiography. RESULTS: The statistical analysis showed no significant difference in ventricular function or in the cardiac conduction system between the groups. CONCLUSION: In regard to the conduction system and ventricular function in the absence of total atrioventricular block, no statistically significant difference was observed between the children of women with systemic lupus erythematosus and children of healthy women.

  18. Cardiovascular Events in Systemic Lupus Erythematosus

    Science.gov (United States)

    Fernández-Nebro, Antonio; Rúa-Figueroa, Íñigo; López-Longo, Francisco J.; Galindo-Izquierdo, María; Calvo-Alén, Jaime; Olivé-Marqués, Alejandro; Ordóñez-Cañizares, Carmen; Martín-Martínez, María A.; Blanco, Ricardo; Melero-González, Rafael; Ibáñez-Rúan, Jesús; Bernal-Vidal, José Antonio; Tomero-Muriel, Eva; Uriarte-Isacelaya, Esther; Horcada-Rubio, Loreto; Freire-González, Mercedes; Narváez, Javier; Boteanu, Alina L.; Santos-Soler, Gregorio; Andreu, José L.; Pego-Reigosa, José M.

    2015-01-01

    Abstract This article estimates the frequency of cardiovascular (CV) events that occurred after diagnosis in a large Spanish cohort of patients with systemic lupus erythematosus (SLE) and investigates the main risk factors for atherosclerosis. RELESSER is a nationwide multicenter, hospital-based registry of SLE patients. This is a cross-sectional study. Demographic and clinical variables, the presence of traditional risk factors, and CV events were collected. A CV event was defined as a myocardial infarction, angina, stroke, and/or peripheral artery disease. Multiple logistic regression analysis was performed to investigate the possible risk factors for atherosclerosis. From 2011 to 2012, 3658 SLE patients were enrolled. Of these, 374 (10.9%) patients suffered at least a CV event. In 269 (7.4%) patients, the CV events occurred after SLE diagnosis (86.2% women, median [interquartile range] age 54.9 years [43.2–66.1], and SLE duration of 212.0 months [120.8–289.0]). Strokes (5.7%) were the most frequent CV event, followed by ischemic heart disease (3.8%) and peripheral artery disease (2.2%). Multivariate analysis identified age (odds ratio [95% confidence interval], 1.03 [1.02–1.04]), hypertension (1.71 [1.20–2.44]), smoking (1.48 [1.06–2.07]), diabetes (2.2 [1.32–3.74]), dyslipidemia (2.18 [1.54–3.09]), neurolupus (2.42 [1.56–3.75]), valvulopathy (2.44 [1.34–4.26]), serositis (1.54 [1.09–2.18]), antiphospholipid antibodies (1.57 [1.13–2.17]), low complement (1.81 [1.12–2.93]), and azathioprine (1.47 [1.04–2.07]) as risk factors for CV events. We have confirmed that SLE patients suffer a high prevalence of premature CV disease. Both traditional and nontraditional risk factors contribute to this higher prevalence. Although it needs to be verified with future studies, our study also shows—for the first time—an association between diabetes and CV events in SLE patients. PMID:26200625

  19. Ethnic differences in the epidemiology of cutaneous lupus erythematosus in New Zealand.

    Science.gov (United States)

    Jarrett, P; Thornley, S; Scragg, R

    2016-11-01

    Background The prevalence and variation by ethnicity of cutaneous lupus in New Zealand is not known. Therefore, a cross-sectional study to determine the prevalence and variation by ethnicity of cutaneous lupus in the ethnically diverse community of South Auckland, New Zealand, was undertaken. Methods Multiple sources were examined to determine the prevalence of acute cutaneous lupus erythematosus, subacute cutaneous erythematosus and discoid lupus erythematosus. Ethnicities examined were European, Māori/Pacific and Indian/Asian. Capture-recapture was used to determine the overall population prevalence of cutaneous lupus. Results A total of 145 cases of cutaneous lupus were identified. There were 22 men and 123 women, with an average age (standard deviation), respectively, of 46.4 (±21.5) and 43.1 (±14.8) years. There were 53 cases of acute cutaneous lupus erythematosus, 19 cases of subacute cutaneous erythematosus and 66 cases of discoid lupus erythematosus. The age and sex adjusted relative risk (95% confidence interval; CI) of Māori/Pacific compared to the European population was 2.47 (95% CI 1.67-3.67) for all types of cutaneous lupus, 1.60 (95% CI 0.84-3.18) for acute cutaneous lupus erythematosus, 0.09 (95% CI 0.01-1.1) for subacute cutaneous erythematosus and 5.96 (95% CI 3.06-11.6) for discoid lupus erythematosus. The overall prevalence of cutaneous lupus was 30.1 (95% CI 25.5-35.4) per 100,000. However, capture-recapture estimated the unadjusted prevalence of cutaneous lupus to be 86.0 (95% CI 78.1-94.7) per 100,000. Conclusion Māori and Pacific people in Auckland, New Zealand, have a greater relative risk of all types of cutaneous lupus compared to the European population and a particularly high risk of discoid lupus erythematosus.

  20. Successful Pregnancy Following Assisted Reproduction in Woman With Systemic Lupus Erythematosus and Hypertension

    OpenAIRE

    de Macedo, José Fernando; de Macedo, Gustavo Capinzaiki; Campos, Luciana Aparecida; Baltatu, Ovidiu Constantin

    2015-01-01

    Abstract Patients with systemic lupus erythematosus have a poor prognosis of pregnancy, since it is associated with significant maternal and fetal morbidity, including spontaneous miscarriage, pre-eclampsia, intrauterine growth restriction, fetal death and pre-term delivery. We report a case with successful pregnancy in a patient with systemic lupus erythematosus and hypertension. A 39-year-old nulliparous woman presented with systemic lupus erythematosus with antinuclear and antiphospholipid...

  1. Is chronic periodontitis premature in systemic lupus erythematosus patients?

    Science.gov (United States)

    Calderaro, Débora Cerqueira; Ferreira, Gilda Aparecida; Corrêa, Jôice Dias; Mendonça, Santuza Maria Souza; Silva, Tarcília Aparecida; Costa, Fernando Oliveira; Lúcio Teixeira, Antônio

    2017-03-01

    The aim of this study was to compare the frequency and severity of chronic periodontitis (CP) in systemic lupus erythematosus (SLE) patients with individuals without rheumatic diseases. Seventy-five patients with SLE were compared to 75 individuals without rheumatic diseases (control group) matched for age, educational level, and income. The activity of SLE was assessed with the Systemic Lupus Erythematosus Disease Activity Index 2000. Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index for Systemic Lupus Erythematosus evaluated SLE-related damage. Dental evaluation included measuring plaque index and parameters of periodontal disease (probing depth, clinical attachment level, and bleeding on probing). Fifty-one (68 %) SLE patients and 42 (56 %) control individuals had CP (p = 0.13). Periodontal status was similar in both groups. Considering only individuals with CP, SLE patients were younger than controls (40.7 ± 9.8 versus 46.14 ± 12.5 years of age, p = 0.02). CP was not associated with activity or therapeutics in SLE patients. Severity of periodontal parameters was similar in SLE patients and control subjects; however, CP occurred earlier in SLE patients.

  2. Contemporary concepts for the clinical and laboratory evaluation of systemic lupus erythematosus and "lupus-like" syndromes.

    Science.gov (United States)

    Nakamura, R M; Bylund, D J

    1994-01-01

    Systemic lupus erythematosus (SLE) is a nonorgan-specific autoimmune disease which affects multiple organ systems and is multifactorial in etiology. SLE is the prototypic systemic rheumatic disease with immune dysregulation characterized by (1) polyclonal activation of B-cells and (2) production of a large spectrum of autoantibodies with a marked preference for nuclear and intracellular antigens. The clinical and laboratory manifestations and criteria for classification and diagnosis of systemic lupus erythematosus, lupus-like syndromes, and various subsets of systemic lupus erythematosus, are reviewed. The differential diagnosis of SLE and related diseases is described with correlation of specific intracellular autoantibodies.

  3. A Prospective Study of the Impact of Current Poverty, History of Poverty, and Exiting Poverty on Accumulation of Disease Damage in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Yelin, Edward; Trupin, Laura; Yazdany, Jinoos

    2017-08-01

    To estimate the effect of current poverty, number of years in poverty, and exiting poverty on disease damage accumulation in systemic lupus erythematosus (SLE). For this study, 783 patients with SLE were followed up from 2003 to 2015 through annual structured interviews. Respondents were categorized in each year by whether they had a household income of ≤125% of the US federal poverty level. Linear and logistic regression analyses were used to assess the impact of poverty in 2009, number of years in poverty between 2003 and 2009, and permanent exits from poverty as of 2009 on the extent of disease damage (according to the Brief Index of Lupus Damage [BILD] score) or accumulation of a clinically meaningful increase in disease damage (defined as a minimum 2-point increase in the BILD damage score) by 2015. After adjustment for sociodemographic features, health care characteristics, and health behaviors, poverty in 2009 was associated with an increased level of accumulated disease damage in 2015 (mean difference in BILD damage score between poor and non-poor 0.62 points, 95% confidence interval [95% CI] 0.25-0.98) and increased odds of a clinically important increase in damage (odds ratio [OR] 1.67, 95% CI 0.98-2.85). Being poor in every year between 2003 and 2009 was associated with greater damage (mean change in BILD score 2.45, 95% CI 1.88-3.01) than being poor for one-half or more of those years (mean change in BILD score 1.45, 95% CI 0.97-1.93), for fewer than one-half of those years (mean change in BILD score 1.49, 95% CI 1.10-1.88), or for none of those years (mean change in BILD score 1.34, 95% CI 1.20-1.49). Those exiting poverty permanently had similar increases in disease damage (mean change in BILD score 1.30, 95% CI 0.90-1.69) as those who were never in poverty (mean change in BILD score 1.36, 95% CI 1.23-1.50) but much less damage than those who remained in poverty (mean change in BILD score 1.98, 95% CI 1.59-2.38). The effects of current poverty

  4. Systemic lupus erythematosus in pregnancy - intricate, but wieldy

    Directory of Open Access Journals (Sweden)

    Ritin Mohindra

    2015-04-01

    Full Text Available Systemic Lupus Erythematosus (SLE predominantly afflicts young women in reproductive age. In this context, it is only rational that pregnancy and its outcome becomes an imperative concern for the lupus patients and their health care providers. Queries regarding the risk of disease flares during pregnancy, chance of fetal loss, and the safety of various drugs are often raised. However the present day availability of effective treatment regimens has meant that many patients achieve protracted remissions and better disease control. Indeed, a high proportion of lupus patients, with professional care, can thus look forward to a successful pregnancy.1 This article reviews the contemporary concepts regarding SLE and pregnancy, especially regarding the fertility rate, optimal timing of conception, risk of disease flares during lupus pregnancy, pregnancy course, fetal outcome, safety of various drugs used for disease control during pregnancy and lactation, and contraceptive advice. [Int J Reprod Contracept Obstet Gynecol 2015; 4(2.000: 295-300

  5. Postextraction hemorrhage in a young male patient with systemic lupus erythematosus.

    Science.gov (United States)

    Schwartz, S; Esseltine, D W

    1984-03-01

    A case of a 13-year-old boy with prolonged bleeding after tooth extraction is reported. This was the first manifestation of systemic lupus erythematosus found to be associated with circulating anticoagulants, including the "lupus anticoagulant," and possible hypoprothrombinemia.

  6. CHANGES IN LEVELS OF SOLUBLE T-CELL ACTIVATION MARKERS, SIL-2R, SCD4 AND SCD8, IN RELATION TO DISEASE EXACERBATIONS IN PATIENTS WITH SYSTEMIC LUPUS-ERYTHEMATOSUS - A PROSPECTIVE-STUDY

    NARCIS (Netherlands)

    SPRONK, P.E.; TERBORG, E.J.; HUITEMA, M.G.; Limburg, Piet; Kallenberg, Cees

    1994-01-01

    Objectives-To assess serial activation of T-cell subsets in relation to auto-antibody production and the occurrence of disease exacerbations in patients with systemic lupus erythematosus (SLE). Methods-To study the possible role of T-cells in the pathophysiology of the disease, 16 consecutive exacer

  7. Lupus eritematoso neonatal: reporte de cuatro casos Neonatal lupus erythematosus: a report of four cases

    Directory of Open Access Journals (Sweden)

    Maria Fernanda Perez

    2011-04-01

    Full Text Available El lupus eritematoso neonatal es una enfermedad poco frecuente, caracterizada clínica mente por alteraciones cutáneas semejantes al lupus subagudo o discoide y/o bloqueo cardíaco congénito. Generalmente, cuando los pacientes presentan manifestaciones cutáneas, no tienen anormalidades cardiológicas y viceversa, aunque en un 10% de los casos ambas manifestaciones pueden coexistir. Puede acompañarse también de alteraciones hematológicas, hepáticas y neurológicas. Es causado por el pasaje trasplacentario de anticuerpos maternos anti Ro (95%, anti La y menos frecuentemente anti U1RNP. Presentamos cuatro pacientes con hallazgos clínicos, histopatológicos e inmunológicos compatibles con lupus eritematoso neonatal, su tratamiento y evolución.Neonatal lupus erythematosus is a very rare disease, clinically characterized by skin lesions that resemble those of subacute or discoid lupus erythematosus and/or congenital heart block. Generally, when patients have skin manifestations, they have no cardiac defects and vice-versa; however, in 10% of cases these manifestations may coexist. Other findings may include hematologic, hepatic and neurological abnormalities. This condition is caused by the transplacental passage of maternal autoantibodies against Ro (95%, La and, less frequently, U1-ribonucleoprotein (U1-RNP. The present case report describes four patients with clinical, histopathological and immunological findings compatible with neonatal lupus erythematosus, their treatment and progress.

  8. Renal histology and pregnancy performance in systemic lupus erythematosus.

    Science.gov (United States)

    Devoe, L D; Loy, G L; Spargo, B H

    1983-01-01

    Previous reports indicate that maternal and fetal outcome in pregnancies complicated by systemic lupus erythematosus (SLE) may be strongly influenced by the presence of renal disease. As the relationship between renal histology and clinical function in SLE is not consistent, prospective data on the outcomes of such pregnancies would aid patient counselling. Fifteen women with SLE had 18 pregnancies subsequent to renal biopsies, performed from 3 months to 8 years prior to conception. Their renal function was evaluated before, during and after pregnancy. Fourteen of 15 patients had evidence of renal involvement, based on by light and electron microscopic sections: 7 had mesangial involvement (WHO Class II); 5 had active focal or diffuse glomerulonephritis (Classes III and IV); two had membranous involvement (Class V); 1, no evident disease. Perinatal outcome was similar whether lesions were milder (8 continuing pregnancies, 4 term deliveries) or more severe (6 continuing pregnancies, 3 term deliveries). Clinical renal function was normal in all but 3 cases at the beginning of pregnancy; 2 additional patients experienced moderate deteriorations in renal function during pregnancy but recovered normal function in the puerperium. Fetal outcome was abnormal (3 premature deliveries, 1 neonatal death, 1 spontaneous abortion) in all cases where renal function was decreased, while 10 of 13 pregnancies in patients with normal renal function ended in term deliveries. The data suggest that currently preconceptual renal histology provides a less accurate basis for perinatal counselling than does the assessment of clinical renal function.

  9. The clinical significance of antiphospholipid antibodies in systemic lupus erythematosus

    Science.gov (United States)

    Ünlü, Ozan; Zuily, Stephane; Erkan, Doruk

    2016-01-01

    Antiphospholipid syndrome (APS) is the association of thrombosis and/or pregnancy morbidity with antiphospholipid antibodies (aPL). Thirty to forty percent of systemic lupus erythematosus (SLE) patients are tested positive for aPL, which may have an impact on the SLE presentation, management, and prognosis. Compared with SLE patients without aPL, those with aPL have a higher prevalence of thrombosis, pregnancy morbidity, valve disease, pulmonary hypertension, livedo reticularis, thrombocytopenia, hemolytic anemia, acute/chronic renal vascular lesions, and moderate/severe cognitive impairment; worse quality of life; and higher risk of organ damage. The use of low-dose aspirin (LDA) is controversial for primary thrombosis and pregnancy morbidity prevention because of the lack of strong prospective controlled data. Similarly, the use of anticoagulation is controversial for patients with an aPL-related nephropathy. Until further studies are available, physicians should discuss the risk/benefits of LDA or anticoagulation as well as the available literature with patients. PMID:27708976

  10. The Importance of an Early Diagnosis in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Sebastiani, Gian D; Prevete, Immacolata; Iuliano, Annamaria; Minisola, Giovanni

    2016-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with a high degree of variability at onset, making it difficult to reach a correct and prompt diagnosis. To present the difficulties faced by the clinician in making a SLE diagnosis, based on the characteristics at study entry of an Italian cohort of SLE patients with recent onset as compared to two similar cohorts. Beginning on 1 January 2012 all patients with a diagnosis of SLE (1997 ACR criteria) and disease duration of less than 12 months were consecutively enrolled in a multicenter prospective study. Information on clinical and serological characteristics was collected at study entry and every 6 months thereafter. Our cohort consisted of 122 patients, of whom 103 were females. Among the manifestations included in the 1997 American College of Rheumatology (ACR) criteria, cutaneous, articular and hematologic symptoms were the most prevalent symptoms at study entry. Data from the literature confirm that the diagnosis of SLE is challenging, and that SLE is a severe disease even at onset when a prompt diagnosis is necessary for initiating the appropriate therapy.

  11. Caesarean section in a case of systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Varsha Vyas

    2014-01-01

    Full Text Available Systemic lupus erythematosus (SLE is an autoimmune disease most frequently found in women of child bearing age and may co-exist with pregnancy. Disease exacerbation, increased foetal loss, neonatal lupus and an increased incidence of pre-eclampsia are the major challenges. Its multisystem involvement and therapeutic interventions like anticoagulants, steroids and immunosuppressive agents pose a high risk for both surgery and anaesthesia. We describe successful management of an antinuclear antibody (ANA positive parturient with bad obstetric history who underwent elective caesarean section under spinal anaesthesia.

  12. Vitamin D and Systemic Lupus Erythematosus: Myth or Reality?

    Science.gov (United States)

    Watad, Abdulla; Neumann, Shana G; Soriano, Alessandra; Amital, Howard; Shoenfeld, Yehuda

    2016-01-01

    There is growing interest in the contribution of vitamin D deficiency to autoimmunity. Several studies have shown an association between low levels of vitamin D and autoimmune disorders, including multiple sclerosis, rheumatoid arthritis, type 1 diabetes, autoimmune thyroid diseases, celiac disease, and systemic lupus erythematosus (SLE). Vitamin D receptor ligands can mediate immunosuppressive effects. It has been suggested that low levels of this hormone contribute to the immune activation in lupus and other autoimmune diseases. This review updates and summarizes the literature on the association between vitamin D and SLE, and discusses the various correlations between vitamin D and SLE activity, clinical expressions, serology, and gene polymorphisms of vitamin D receptors.

  13. Pregnancies in women with systemic lupus erythematosus and antiphospholipid antibodies.

    Science.gov (United States)

    Schreiber, K

    2016-04-01

    Systemic lupus erythematosus (SLE) has preponderance in women in their childbearing years; consequently pregnancy has always been an important issue of concern for the patient and the treating physician. Based upon numerous reports on successful pregnancy outcomes in the past decades, the initial advice against pregnancy in the 1950s has been replaced by a common understanding that women with SLE often have successful pregnancy outcomes, and clinicians therefore advise on pregnancy planning, including possible drug adjustments, timing and close surveillance. The recently published Predictors of Pregnancy Outcome: Biomarkers in Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus (PROMISSE) study, so far the largest multicentre cohort study of pregnant women with underlying stable SLE, has given some important answers to long-discussed questions. Future studies on data collected from the PROMISSE cohort will hopefully identify serological biomarkers, possibly genes, and in addition, give valuable information about underlying disease mechanisms. © The Author(s) 2016.

  14. 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.

  15. Pregnancies in women with systemic lupus erythematosus and antiphospholipid antibodies

    DEFF Research Database (Denmark)

    Schreiber, K

    2016-01-01

    Systemic lupus erythematosus (SLE) has preponderance in women in their childbearing years; consequently pregnancy has always been an important issue of concern for the patient and the treating physician. Based upon numerous reports on successful pregnancy outcomes in the past decades, the initial...... of Pregnancy Outcome: Biomarkers in Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus (PROMISSE) study, so far the largest multicentre cohort study of pregnant women with underlying stable SLE, has given some important answers to long-discussed questions. Future studies on data collected from...... advice against pregnancy in the 1950s has been replaced by a common understanding that women with SLE often have successful pregnancy outcomes, and clinicians therefore advise on pregnancy planning, including possible drug adjustments, timing and close surveillance. The recently published Predictors...

  16. Echolalia as a novel manifestation of neuropsychiatric systemic lupus erythematosus.

    Science.gov (United States)

    Zapor, M; Murphy, F T; Enzenauer, R

    2001-01-01

    "That tongue of yours, by which I have been tricked, shall have its power curtailed and enjoy the briefest use of speech." With these words, Hera, of Greek mythology, deprived the nymph Echo of spontaneous speech, constraining her instead to merely repeating the words of others. Echolalia, which derives from the word "echo," is disordered speech in which an individual persistently repeats what is heard. Echolalia has been described in patients with a number of neuropsychiatric illnesses including autism and Tourette's syndrome. Neuropsychiatric systemic lupus erythematosus (NPSLE) is a heterogeneous disease with protean manifestations that may occur in approximately 25% to 50% of patients with systemic lupus erythematosus (SLE). Although the most common manifestations include cognitive dysfunction (50%) and seizures (20%), NPSLE may also present as peripheral neuropathy (15%), psychosis (10%), or other central nervous system abnormalities. We report the case of a 57-year-old woman with SLE and echolalia.

  17. Streptococcus pneumoniae necrotizing fasciitis in systemic lupus erythematosus.

    Science.gov (United States)

    Sánchez, A; Robaina, R; Pérez, G; Cairoli, E

    2016-04-01

    Necrotizing fasciitis is a rapidly progressive destructive soft tissue infection with high mortality. Streptococcus pneumoniae as etiologic agent of necrotizing fasciitis is extremely unusual. The increased susceptibility to Streptococcus pneumoniae infection in patients with systemic lupus erythematosus is probably a multifactorial phenomenon. We report a case of a patient, a 36-year-old Caucasian female with 8-year history of systemic lupus erythematosus who presented a fatal Streptococcus pneumoniae necrotizing fasciitis. The role of computed tomography and the high performance of blood cultures for isolation of the causative microorganism are emphasized. Once diagnosis is suspected, empiric antibiotic treatment must be prescribed and prompt surgical exploration is mandatory. © The Author(s) 2015.

  18. Cognitive functions and autoantibodies in patients with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Anna Bogaczewicz

    2016-06-01

    Full Text Available Introduction: Autoantibodies may occur in the course of various diseases. In the case of systemic lupus erythematosus the presence of specific autoantibodies is included in the classification criteria of the disease. The aim of the study was to investigate whether the presence of the serologic markers of systemic lupus erythematosus, i.e. anti-dsDNA, anti-Sm and anticardiolipin antibodies of the class IgM and IgG are linked with the results of neuropsychological tests evaluating selected cognitive functions in patients without overt neuropsychiatric lupus and without antiphospholipid syndrome. Material and methods: The study included 22 patients with systemic lupus erythematosus. For the assessment of anti-dsDNA, anti-Sm and anticardiolipin antibodies the immunoenzymatic method was used. For neuropsychological estimation of the selected cognitive functions the attention switching test and the choice reaction time were applied, in which the results are expressed as the average delay i.e. mean correct latency, using the computer-based Cambridge Neuropsychological Test Automated Battery (CANTAB. Results: The results of attention switching test in patients with anti-Sm antibodies were lower, but not significantly different from those obtained by the patients without such antibodies: 75.0 (73.12–88.12 vs. 92.5 (85–95. Choice reaction time was significantly longer in patients with anti-Sm antibodies in comparison to the patients without antiSm antibodies: 614.9 (520.6–740.8 vs. 476.7 (396.6–540 (p = 0.01. No significant difference was demonstrated in the results of attention switching test and choice reaction time with regard to the presence of anti-dsDNA antibodies. The results of attention switching test and choice reaction time were not different between the groups of patients with and without anticardiolipin antibodies in the IgM and IgG class. Conclusions: Anti-Sm antibodies seem to contribute to

  19. Quality of life in patients with systemic lupus erythematosus

    OpenAIRE

    Елена Александровна Асеева; V. N. Amirdzhanova; ЛИСИЦЫНА Т.А.; M V Zavalskaya

    2013-01-01

    The recent rise in survival rates and therefore in the degree of organ damages due to both disease itself and performed therapy increasingly gives a reason to think about health-related quality of life (QL) in patients with systemic lupus erythematosus (SLE). Numerous studies initiated in the 1980s to asses QL in patients with SLE have revealed considerable impairments in physical, emotional functioning in this category of patients. SLE leads to social dysadaptation, chronic stress, anxiety, ...

  20. Systemic lupus erythematosus: strategies to improve pregnancy outcomes

    OpenAIRE

    Yamamoto Y; Aoki S

    2016-01-01

    Yuriko Yamamoto, Shigeru Aoki Perinatal Center for Maternity and Neonate, Yokohama City University Medical Center, Yokohama, Kanagawa Prefecture, Japan Abstract: Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease with a high prevalence in females of childbearing age. Thus, reproduction in SLE patients is a major concern for clinicians. In the past, SLE patients were advised to defer pregnancy because of poor pregnancy outcomes and fear of SLE flares during pregn...

  1. Vitamin D and systemic lupus erythematosus: continued evolution.

    Science.gov (United States)

    Yap, Kristy S; Morand, Eric F

    2015-02-01

    Vitamin D is a steroid hormone that has well-established roles in calcium and bone metabolism. Vitamin D has more recently become recognized for its role in the immune response and its potential immunomodulatory effects in autoimmune diseases, including systemic lupus erythematosus (SLE). This review provides a summary of the recent literature regarding vitamin D and SLE, as well as current recommendations for vitamin D supplementation in patients with SLE.

  2. Antinuclear antibody-negative systemic lupus erythematosus-how common?

    OpenAIRE

    1982-01-01

    A review of five years' DNA-binding antibody results in a routine service laboratory revealed 38 patients who had high DNA-binding capacity (DNA-bc) but no antinuclear antibodies (ANA). On retrospective case note analysis, 22 patients (58%) were thought to have systemic lupus erythematosus (SLE), although only six (16%) fulfilled the preliminary classification criteria of the American Rheumatism Association (ARA). Our findings indicate that ANA-negative SLE is commoner than generally realised...

  3. Aortic valve replacement in a patient with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Bhuvnesh Kansara

    2013-01-01

    Full Text Available Valvular heart disease in systemic lupus erythematosus (SLE is associated with substantial morbidity and mortality. Current therapy includes symptomatic measures and valve replacement. SLE can present major challenges because of accrued organ damage, coagulation defects and complex management regimes. The peri-operative goals are to maintain strict asepsis, avoid use of nephrotoxic drugs and thereby renal insult, and to promote early ambulation post-operatively.

  4. Novel molecular targets in the treatment of systemic lupus erythematosus

    Science.gov (United States)

    Crispín, JoséC; Tsokos, George C.

    2009-01-01

    T cells from patients with systemic lupus erythematosus (SLE) display a number of biochemical abnormalities which include altered expression of key signaling molecules, heightened calcium responses, and skewed expression of transcription factors. These defects are involved in the altered behavior of SLE T cells and are probably central in the disease pathogenesis. The aim of this communication is to review the defects that have been consistently documented in SLE T cells, highlighting molecules and pathways that represent therapeutic targets. PMID:18190888

  5. Intracranial Aneurysm with Systemic Lupus Erythematosus Treated by Endovascular Intervention

    Directory of Open Access Journals (Sweden)

    P Shrestha

    2010-03-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic disease with multiple pathologies that can affect every organ system of the body including central nervous system. Intracerebral aneurysms and subarachnoid hemorrhage (SAH are one of comparatively rarer manifestations of central nervous system SLE. Here we present a case of known SLE complicated by the rupture of intra cerebral aneurysm at basilar artery tip which was successfully treated with endovascular coiling. Keywords: cerebral aneurysm, endovascular surgery, SAH, SLE

  6. Quality of life in patients with systemic lupus erythematosus

    OpenAIRE

    Елена Александровна Асеева; V N Amirdzhanova; Т А Лисицына; M V Zavalskaya

    2013-01-01

    The recent rise in survival rates and therefore in the degree of organ damages due to both disease itself and performed therapy increasingly gives a reason to think about health-related quality of life (QL) in patients with systemic lupus erythematosus (SLE). Numerous studies initiated in the 1980s to asses QL in patients with SLE have revealed considerable impairments in physical, emotional functioning in this category of patients. SLE leads to social dysadaptation, chronic stress, anxiety, ...

  7. Multiple keratoacanthomas of hands simulating discoid lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Chopra Adarsh

    2000-01-01

    Full Text Available A 38-year-old woman presented with multiple, well-defined, erythematous, scaly, slightly pain-ful nodules progressing to plaques on the palms, dorsa of fingers of both hands since 6 years. Healed lesions had well-defined hyperpigmented margins with slight central atrophy. Clinical diagnosis of dis-coid lupus erythematosus was made, but histopatholgoy confirmed the diagnosis of keratoacanthomas

  8. DETECTION OF LUNG INVOLVEMENT BY HRCT IN SYSTEMIC LUPUS ERYTHEMATOSUS

    Institute of Scientific and Technical Information of China (English)

    沈加林; 陈克敏; 蒋蕴毅; 丁小龙

    2002-01-01

    Objective To assess the sensitivity of high-resolution CT (HRCT) in detecting pulmonary involvement attributed to systemic lupus erythematosus (SLE). Methods Plain chest radiography, HRCT, and pulmonary function testing of 36 patients with SLE were analyzed. Results The sensitivity of the pulmonary involvement by HRCT, plain chest radiography and pulmonary function testing were 88.9%,36.1%,and 33.3%, respectively. Conclusion HRCT played an important role in detecting pulmonary involvemen due to SLE, especially in early and mild cases.

  9. Management of systemic lupus erythematosus during pregnancy: challenges and solutions

    OpenAIRE

    Knight,Caroline; Nelson-Piercy, Catherine

    2017-01-01

    Caroline L Knight, Catherine Nelson-Piercy Division of Women’s Health, Women’s Health Academic Centre, King’s College London and King’s Health Partners, St Thomas’ Hospital, London, UK Abstract: Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease predominantly affecting women, particularly those of childbearing age. SLE provides challenges in the prepregnancy, antenatal, intrapartum, and postpartum periods ...

  10. Management of systemic lupus erythematosus during pregnancy: challenges and solutions

    OpenAIRE

    Knight CL; Nelson-Piercy C

    2017-01-01

    Caroline L Knight, Catherine Nelson-Piercy Division of Women’s Health, Women’s Health Academic Centre, King’s College London and King’s Health Partners, St Thomas’ Hospital, London, UK Abstract: Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease predominantly affecting women, particularly those of childbearing age. SLE provides challenges in the prepregnancy, antenatal, intrapartum, and postpartum periods for these women, and for...

  11. Psoriatic Alopecia in a Patient with Systemic Lupus Erythematosus

    OpenAIRE

    Wimolsiri Iamsumang; Tueboon Sriphojanart; Poonkiat Suchonwanit

    2017-01-01

    Psoriasis is a chronic, recurrent, and relatively common inflammatory dermatologic condition, which demonstrates various clinical manifestations including hair loss. It was once believed that alopecia was not a presentation of scalp psoriasis, but it is now widely accepted that psoriatic alopecia exists. Although the majority of patients get hair regrowth, it can potentially lead to permanent hair loss. Herein, we report a case of 26-year-old female patient with systemic lupus erythematosus w...

  12. Lupus pneumonitis as the initial presentation of systemic lupus erythematosus: case series from a single institution.

    Science.gov (United States)

    Wan, S A; Teh, C L; Jobli, A T

    2016-11-01

    Objective The aim of this study was to examine the clinical features, treatment and outcome of systemic lupus erythematosus (SLE) patients in our centre who presented with lupus pneumonitis as the initial manifestation. Methods We performed a retrospective review of all patients who presented with lupus pneumonitis during the initial SLE manifestation from March 2006 to March 2015. Results There were a total of five patients in our study who presented with fever and cough as the main clinical features. All patients had pulmonary infiltrates on chest radiographs. High-resolution computed tomography, which was performed in two patients, showed ground glass opacities with patchy consolidations bilaterally. All patients received high-dose steroids, 80% received intravenous cyclophosphamide and 60% received intravenous immunoglobulin. Two patients died from severe lupus pneumonitis within 2 weeks of admission despite treatment with ventilation, steroids, cyclophosphamide and intravenous immunoglobulin. Conclusions Acute lupus pneumonitis is an uncommon presentation of SLE. Mortality in this case series is 40%.

  13. Advances in the treatment of cutaneous lupus erythematosus.

    Science.gov (United States)

    Kuhn, A; Landmann, A; Wenzel, J

    2016-07-01

    Lupus erythematosus (LE) is a multifactorial autoimmune disease with clinical manifestations of differing severity which may present with skin manifestations as primary sign of the disease (cutaneous lupus erythematosus, CLE) or as part of a disease spectrum (systemic lupus erythematosus, SLE). To date, no drugs are approved specifically for the treatment of CLE and only single agents have been applied in randomized controlled trials. Therefore, topical and systemic agents are used "off-label", primarily based on open-label studies, case series, retrospective analyses, and expert opinions. In contrast, several agents, such as hydroxychloroquine, chloroquine, cyclophosphamide, azathioprine, and belimumab, are approved for the treatment of SLE. Recent approaches in the understanding of the molecular pathogenesis of LE enabled the development of further new agents, which target molecules such as interleukin 6 (IL-6) and interferon (IFN). Only single trials, however, applied these new agents in patients with cutaneous involvement of the disease and/or included endpoints which evaluated the efficacy of these agents on skin manifestations. This article provides an updated review on new and recent approaches in the treatment of CLE.

  14. Systemic lupus erythematosus presenting with eosinophilic enteritis: a case report

    Directory of Open Access Journals (Sweden)

    Kalany Mohammad

    2011-06-01

    Full Text Available Abstract Introduction Systemic lupus erythematosus (SLE is a multisystem disorder that may present with various symptoms. It may involve the gastrointestinal tract in a variety of ways; some of the most well-known ones are transaminitis, lupus mesenteric vasculitis, lupus enteritis and mesenteric vascular leakage. We describe a case of a patient with SLE who presented with a five-month history of diarrhea caused by eosinophilic enteritis. To the best of our knowledge, there are few cases reported in the literature of patients with SLE who initially present with chronic diarrhea due to eosinophilic enteritis. Case presentation A 38-year-old Persian Iranian woman was admitted with a five-month history of diarrhea and abdominal pain. A physical examination showed nothing abnormal. Initially, she had only lymphopenia and mild eosinophilia. No autoimmune or infectious etiology was detected to justify these abnormalities. A thorough evaluation was not helpful in finding the etiology, until she developed a scalp lesion similar to discoid lupus erythematosus. Computed tomography showed small bowel wall thickening. Briefly, she manifested full-blown SLE, and it was revealed that the diarrhea was caused by eosinophilic enteritis. Conclusion Considering SLE in a patient who presents with chronic diarrhea and lymphopenia may be helpful in earlier diagnosis and therapy. This is an original case report of interest to physicians who practice internal medicine, family medicine and gastroenterology.

  15. DEPRESSION--A FELLOW TRAVELER WITH SYSTEMIC LUPUS ERYTHEMATOSUS.

    Science.gov (United States)

    Cojocaru, Doina-Clementina; Costin, Melania; Bădeanu, Lucia Elena; Negru, R D; Aursulesei, Viviana

    2015-01-01

    Systemic lupus erythematosus (SLE) is a chronic multisystem inflammatory disorder that occurs primarily in women of childbearing age, immunologic abnormalities being a prominent feature of the disease. Psychiatric disorders frequently coexist, depression being the most common mood disorder in neuropsychiatric lupus. This literature review was performed through searching MEDLINE database for full-text English-language articles--original research, systematic review and updates published in the last five years (2010-2015), using the keywords "depression and systemic lupus erythematosus". The main outcomes identified were prevalence and predictors of depression in various cultural and ethnic groups, depression-related clinical issues (suicidal ideation, cognitive impairment, altered body image, sleep and sexual disturbances, influence of SLE treatment), and influence on quality of life. A multidisciplinary approach that takes into account the polymorphism and individual variability of the SLE clinical manifestations helps to improve early detection of depression, which is responsible for the increased risk of comorbidities, suicidal attempts, decreased treatment adherence, and impaired quality of life. Physicians across all specialties involved in the care for lupus patients should be aware of the major prevalence of this condition, while helping patients to cope with their disabling disease.

  16. Pyomyositis in childhood-systemic lupus erythematosus.

    Science.gov (United States)

    Blay, Gabriela; Ferriani, Mariana P L; Buscatti, Izabel M; França, Camila M P; Campos, Lucia M A; Silva, Clovis A

    2016-01-01

    Pyomyositis is a pyogenic infection of skeletal muscle that arises from hematogenous spread and usually presents with localized abscess. This muscle infection has been rarely reported in adult-onset systemic lupus erythematous and, to the best of our knowledge, has not been diagnosed in pediatric lupus population. Among our childhood-onset systemic lupus erythematous population, including 289 patients, one presented pyomyositis. This patient was diagnosed with childhood-onset systemic lupus erythematous at the age of 10 years-old. After six years, while being treated with prednisone, azathioprine and hydroxychloroquine, she was hospitalized due to a 30-day history of insidious pain in the left thigh and no apparent trauma or fever were reported. Her physical examination showed muscle tenderness and woody induration. Laboratory tests revealed anemia, increased acute phase reactants and normal muscle enzymes. Computer tomography of the left thigh showed collection on the middle third of the vastus intermedius, suggesting purulent stage of pyomyositis. Treatment with broad-spectrum antibiotic was initiated, leading to a complete clinical resolution. In conclusion, we described the first case of pyomyositis during childhood in pediatric lupus population. This report reinforces that the presence of localized muscle pain in immunocompromised patients, even without elevation of muscle enzymes, should raise the suspicion of pyomyositis. A prompt antibiotic therapy is strongly recommended.

  17. Quality of life in patients with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Елена Александровна Асеева

    2013-01-01

    Full Text Available The recent rise in survival rates and therefore in the degree of organ damages due to both disease itself and performed therapy increasingly gives a reason to think about health-related quality of life (QL in patients with systemic lupus erythematosus (SLE. Numerous studies initiated in the 1980s to asses QL in patients with SLE have revealed considerable impairments in physical, emotional functioning in this category of patients. SLE leads to social dysadaptation, chronic stress, anxiety, and depression. Timely detection of worse QL in patients and correction of depressive disorders improve patient compliance. Six SLE questionnaires were validated to study health-related QL; these were Medical Outcomes Study 36-Item Short Form Health Survey (MOS SF-36 SLE Symptom checklist, SLE QL Questionnaire (SLEQol, Lupus QL Questionnaire (LupusQol, LQol, and Lupus Patient Reported Outcome Tool (LupusPRO. The general MOS SF-36 and specific Lupus Qol that has been developed for SLE patients are available in Russian. To study QL in the patients at the present stage of rheumatology development is an important component of SLE cura-tion both in real clinical practice and during international clinical trials as one of the components of therapy assessment.

  18. The Pathology of T Cells in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Anselm Mak

    2014-01-01

    Full Text Available Systemic lupus erythematosus (SLE is characterized by the production of a wide array of autoantibodies. Thus, the condition was traditionally classified as a “B-cell disease”. Compelling evidence has however shown that without the assistance of the helper T lymphocytes, it is indeed difficult for the “helpless” B cells to become functional enough to trigger SLE-related inflammation. T cells have been recognized to be crucial in the pathogenicity of SLE through their capabilities to communicate with and offer enormous help to B cells for driving autoantibody production. Recently, a number of phenotypic and functional alterations which increase the propensity to trigger lupus-related inflammation have been identified in lupus T cells. Here, potential mechanisms involving alterations in T-cell receptor expressions, postreceptor downstream signalling, epigenetics, and oxidative stress which favour activation of lupus T cells will be discussed. Additionally, how regulatory CD4+, CD8+, and γδ T cells tune down lupus-related inflammation will be highlighted. Lastly, while currently available outcomes of clinical trials evaluating therapeutic agents which manipulate the T cells such as calcineurin inhibitors indicate that they are at least as efficacious and safe as conventional immunosuppressants in treating lupus glomerulonephritis, larger clinical trials are undoubtedly required to validate these as-yet favourable findings.

  19. Anti-C1q antibodies in systemic lupus erythematosus.

    Science.gov (United States)

    Orbai, A-M; Truedsson, L; Sturfelt, G; Nived, O; Fang, H; Alarcón, G S; Gordon, C; Merrill, Jt; Fortin, P R; Bruce, I N; Isenberg, D A; Wallace, D J; Ramsey-Goldman, R; Bae, S-C; Hanly, J G; Sanchez-Guerrero, J; Clarke, A E; Aranow, C B; Manzi, S; Urowitz, M B; Gladman, D D; Kalunian, K C; Costner, M I; Werth, V P; Zoma, A; Bernatsky, S; Ruiz-Irastorza, G; Khamashta, M A; Jacobsen, S; Buyon, J P; Maddison, P; Dooley, M A; Van Vollenhoven, R F; Ginzler, E; Stoll, T; Peschken, C; Jorizzo, J L; Callen, J P; Lim, S S; Fessler, B J; Inanc, M; Kamen, D L; Rahman, A; Steinsson, K; Franks, A G; Sigler, L; Hameed, S; Pham, N; Brey, R; Weisman, M H; McGwin, G; Magder, L S; Petri, M

    2015-01-01

    Anti-C1q has been associated with systemic lupus erythematosus (SLE) and lupus nephritis in previous studies. We studied anti-C1q specificity for SLE (vs rheumatic disease controls) and the association with SLE manifestations in an international multicenter study. Information and blood samples were obtained in a cross-sectional study from patients with SLE (n = 308) and other rheumatologic diseases (n = 389) from 25 clinical sites (84% female, 68% Caucasian, 17% African descent, 8% Asian, 7% other). IgG anti-C1q against the collagen-like region was measured by ELISA. Prevalence of anti-C1q was 28% (86/308) in patients with SLE and 13% (49/389) in controls (OR = 2.7, 95% CI: 1.8-4, p lupus nephritis. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  20. Refractory Angioedema in a Patient with Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Zahra Habibagahi

    2015-07-01

    Full Text Available Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless edema, but edema of the genital area, gastrointestinal and laryngeal tracts have also been reported. In lupus patients, angioedema may be the result of an acquired type of C1 inhibitor deficiency, most probably due to antibody formation directed against the C1 inhibitor molecule. Herein we report a new case of lupus nephritis that developed angioedema and a rapid course of disease progression with acute renal failure and alveolar hemorrhage without response to high dose steroid and plasmapheresis.

  1. Acute macular neuroretinopathy associated with systemic lupus erythematosus.

    Science.gov (United States)

    Lee, D H; Lee, S C; Kim, M

    2016-04-01

    Acute macular neuroretinopathy (AMN) is a rare disorder that presents with abrupt visual change with wedge-shaped or flower-like lesions pointing towards the fovea. Ischemic insults to the retinal capillary plexus may be important for development of this disease. While many case reports have been published on AMN, none have described AMN in association with systemic lupus erythematosus (SLE). Here, we report a case of AMN associated with newly-diagnosed SLE. We speculate that in patients with lupus flares, immune complex-mediated vascular injury and microvascular thrombosis may disrupt the deep retinal capillary network, causing ischemic damages to the outer retina and leading to the development of AMN. AMN can develop in patients with lupus flares, and must be considered as an SLE-associated ophthalmologic complication. To the best of our knowledge, this is the first case report of AMN associated with SLE. © The Author(s) 2015.

  2. Association of Sweet's Syndrome and Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    J. L. Barton

    2011-01-01

    Full Text Available Sweet's syndrome is an acute febrile neutrophilic dermatosis which usually presents as an idiopathic disorder but can also be drug induced, associated with hematopoetic malignancies and myelodysplastic disorders, and more, infrequently, observed in autoimmune disorders. Sweet's syndrome has been reported in three cases of neonatal lupus, three cases of hydralazine-induced lupus in adults, and in nine pediatric and adult systemic lupus erythematosus (SLE patients. We describe three additional adult cases of Sweet's associated with SLE and provide a focused review on nondrug-induced, nonneonatal SLE and Sweet's. In two of three new cases, as in the majority of prior cases, the skin rash of Sweet's paralleled underlying SLE disease activity. The pathogenesis of Sweet's remains elusive, but evidence suggests that cytokine dysregulation may be central to the clinical and pathological changes in this condition, as well as in SLE. Further research is needed to define the exact relationship between the two conditions.

  3. Experience of long-term belimumab use in patients with systemic lupus erythematosus (a case report

    Directory of Open Access Journals (Sweden)

    Natalia Gennadyevna Klyukvina

    2013-01-01

    Full Text Available In the past years considerable progress has been made in the treatment of systemic lupus erythe-matosus; however, not all questions have been answered. The range of medications has substan-tially increased. The paper describes a case of the long-term use of the new genetically engineered agent belimumab in a patient with systemic lupus erythematosus.

  4. Mannose-binding lectin polymorphisms and susceptibility to infection in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Garred, P; Madsen, H O; Halberg, P

    1999-01-01

    To determine whether variant alleles in the coding portion of the mannose-binding lectin (MBL) gene are associated with increased susceptibility to systemic lupus erythematosus (SLE) and concomitant infections.......To determine whether variant alleles in the coding portion of the mannose-binding lectin (MBL) gene are associated with increased susceptibility to systemic lupus erythematosus (SLE) and concomitant infections....

  5. Dietary micronutrient intake and atherosclerosis in systemic lupus erythematosus.

    Science.gov (United States)

    Lourdudoss, C; Elkan, A-C; Hafström, I; Jogestrand, T; Gustafsson, T; van Vollenhoven, R; Frostegård, J

    2016-12-01

    The aim of this study was to investigate the role of dietary micronutrient intake in systemic lupus erythematosus (SLE). This study included 111 SLE patients and 118 age and gender-matched controls. Data on diet (food frequency questionnaires) were linked with data on Systemic Lupus Activity Measure, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and carotid atherosclerotic/echolucent plaque (B-mode ultrasound). Dietary micronutrient intake were compared between SLE patients and controls and in relation to lupus activity and atherosclerosis in SLE. Associations between micronutrient intake and plaque were analyzed through logistic regression, adjusted for potential confounders. Micronutrient intake did not differ between patients and controls, and between lower and higher lupus activity, apart from the fact that phosphorus was associated with SLEDAI > 6. In SLE patients, some micronutrients were associated with atherosclerotic plaque, left side. Lower intake of riboflavin and phosphorus was associated with atherosclerotic plaque, left side (odds ratio (OR) 3.06, 95% confidence interval (CI) 1.12-8.40 and OR 4.36, 95% CI 1.53-12.39, respectively). Higher intake of selenium and thiamin was inversely associated with atherosclerotic plaque, left side (OR 0.28, 95% CI 0.09-0.89 and OR 0.26, 95% CI 0.08-0.80, respectively). In addition, higher intake of thiamin was inversely associated with echolucent plaque, left side (OR 0.22, 95% CI 0.06-0.84). Lower intake of folate was inversely associated with bilateral echolucent plaque (OR 0.36, 95% CI 0.13-0.99). SLE patients did not have different dietary micronutrient intake compared to controls. Phosphorus was associated with lupus activity. Riboflavin, phosphorus, selenium and thiamin were inversely associated with atherosclerotic plaque, left side in SLE patients, but not in controls. Dietary micronutrients may play a role in atherosclerosis in SLE. © The Author(s) 2016.

  6. Massive intracranial calcifications in a patient with systemic lupus erythematosus; Calcificacoes intracranianas macicas em um paciente com lupus eritematoso sistemico

    Energy Technology Data Exchange (ETDEWEB)

    Gasparetto, Emerson L.; Carvalho Neto, Arnolfo de [Parana Univ., Curitiba, PR (Brazil). Dept. de Clinica Medica. Servico de Radiologia Medica]. E-mail: gasparetto@hotmail.com; Ono, Sergio E. [Parana Univ., Curitiba, PR (Brazil). Faculdade de Medicina

    2004-12-01

    Central nervous system involvement is frequently reported in patients with systemic lupus erythematosus. Computed tomography and magnetic resonance imaging studies usually show brain atrophy, cerebral infarction and/or intracranial bleeding. Extensive intracranial calcification in patients with systemic lupus erythematosus is rare. We report a case of a patient with systemic lupus erythematosus who presented with seizures and massive basal ganglia calcification and mild calcifications in the frontal lobes, seen on the brain computed tomography scan. Magnetic resonance imaging showed hyperintensity on FLAIR images and hypointense signals on T2{sup *} gradient echo images in the basal ganglia. (author)

  7. Retinal nerve fiber layer thickness and neuropsychiatric manifestations in systemic lupus erythematosus.

    Science.gov (United States)

    Shulman, S; Shorer, R; Wollman, J; Dotan, G; Paran, D

    2017-01-01

    Background Cognitive impairment is frequent in systemic lupus erythematosus. Atrophy of the corpus callosum and hippocampus have been reported in patients with systemic lupus erythematosus, and diffusion tensor imaging studies have shown impaired white matter integrity, suggesting that white matter damage in systemic lupus erythematosus may underlie the cognitive impairment as well as other neuropsychiatric systemic lupus erythematosus manifestations. Retinal nerve fiber layer thickness, as assessed by optical coherence tomography, has been suggested as a biomarker for white matter damage in neurologic disorders such as multiple sclerosis, Alzheimer's disease and Parkinson's disease. Retinal nerve fiber layer thinning may occur early, even in patients with mild clinical symptoms. Aim The objective of this study was to assess the association of retinal nerve fiber layer thickness, as a biomarker of white matter damage in systemic lupus erythematosus patients, with neuropsychiatric systemic lupus erythematosus manifestations, including cognitive impairment. Methods Twenty-one consecutive patients with systemic lupus erythematosus underwent neuropsychological testing using a validated computerized battery of tests as well as the Rey-Auditory verbal learning test. All 21 patients, as well as 11 healthy, age matched controls, underwent optical coherence tomography testing to assess retinal nerve fiber layer thickness. Correlations between retinal nerve fiber layer thickness and results in eight cognitive domains assessed by the computerized battery of tests as well as the Rey-Auditory verbal learning test were assessed in patients with systemic lupus erythematosus, with and without neuropsychiatric systemic lupus erythematosus, and compared to retinal nerve fiber layer thickness in healthy controls. Results No statistically significant correlation was found between retinal nerve fiber layer thickness in patients with systemic lupus erythematosus as compared to healthy

  8. Neurodevelopmental disorders in children born to mothers with systemic lupus erythematosus.

    Science.gov (United States)

    Vinet, É; Pineau, C A; Clarke, A E; Fombonne, É; Platt, R W; Bernatsky, S

    2014-10-01

    Children born to women with systemic lupus erythematosus seem to have a potentially increased risk of neurodevelopmental disorders compared to children born to healthy women. Recent experimental data suggest in utero exposure to maternal antibodies and cytokines as important risk factors for neurodevelopmental disorders. Interestingly, women with systemic lupus erythematosus display high levels of autoantibodies and cytokines, which have been shown, in animal models, to alter fetal brain development and induce behavioral anomalies in offspring. Furthermore, subjects with systemic lupus erythematosus and neurodevelopmental disorders share a common genetic predisposition, which could impair the fetal immune response to in utero immunologic insults. Moreover, systemic lupus erythematosus pregnancies are at increased risk of adverse obstetrical outcomes and medication exposures, which have been implicated as potential risk factors for neurodevelopmental disorders. In this article, we review the current state of knowledge on neurodevelopmental disorders and their potential determinants in systemic lupus erythematosus offspring.

  9. Frontal Fibrosing Alopecia Coexisting with Lupus Erythematosus: Poor Response to Hydroxychloroquine.

    Science.gov (United States)

    Contin, Letícia Arsie; Martins da Costa Marques, Elisa Raquel; Noriega, Leandro

    2017-01-01

    Lupus erythematosus, especially the discoid form, and lichen planopilaris may be associated and can occur in different topographies (coexistence) or in the same lesion (lupus eythematosus/lichen planus overlap syndrome). Frontal fibrosing alopecia is considered a variant form of lichen planopilaris and is characterized by frontotemporal hairline and eyebrow involvement. Of the association with lupus erythematosus we have only a few descriptions. Hydroxychloroquine and chloroquine diphosphate are antimalarial drugs described as viable treatment options for both diseases, due to an antilymphocytic effect. The association between frontal fibrosing alopecia and lupus erythematosus (discoid or systemic) is reported in this article, showing a progressive alopecia in the frontotemporal hairline despite treatment with hydroxychloroquine.

  10. Hydroxychloroquine and pregnancy on lupus flares in Korean patients with systemic lupus erythematosus.

    Science.gov (United States)

    Koh, J H; Ko, H S; Kwok, S-K; Ju, J H; Park, S-H

    2015-02-01

    We investigated the clinical and laboratory characteristics of pregnancies with systemic lupus erythematosus (SLE) and identified lupus flare predictors during pregnancy. Additionally, we examined lupus activity and pregnancy outcomes in SLE patients who continued, discontinued or underwent no hydroxychloroquine (HCQ) treatment during pregnancy. We retrospectively analyzed 179 pregnancies in 128 SLE patients at Seoul St. Mary's Hospital, Korea, between 1998 and 2012 and then assessed the clinical profiles and maternal and fetal outcomes. Overall, 90.5% of pregnancies resulted in a successful delivery and were divided into two groups: those who experienced lupus flares (80 pregnancies, 44.7%) and those who did not (99 pregnancies, 55.3%). Increased preeclampsia, preterm births, low birth weight, intrauterine growth restriction (IUGR), and low 1-minute Apgar scores occurred in pregnancies with lupus flares compared to pregnancies in quiescent disease. Lupus flares were predicted by HCQ discontinuation, a history of lupus nephritis, high pre-pregnancy serum uric acid and low C4 levels. Our study indicates that achieving pre-pregnancy remission and continuing HCQ treatment during pregnancy are important for preventing lupus flares.

  11. Dengue fever triggering systemic lupus erythematosus and lupus nephritis: a case report

    Directory of Open Access Journals (Sweden)

    Talib SH

    2013-10-01

    Full Text Available SH Talib, SR Bhattu, R Bhattu, SG Deshpande, DB Dahiphale Department of Medicine and Nephrology, MGM Medical College and Hospital, Aurangabad, Maharashtra, India Abstract: We report a rare case of dengue fever triggering systemic lupus erythematosus and lupus nephritis. The patient presented herself during a large outbreak of dengue fever in December 2012 in Maharashtra, India. The diagnosis of dengue fever was confirmed by the presence of NS-1 antigen during the first few days of febrile illness. Eight weeks later, kidney tissue biopsy studies revealed evidence of lupus nephritis on microscopic examination and immunofluorescence. The report interpreted it as focal proliferative glomerulonephritis and segmental sclerosis (Stage IIIC. The case was also found positive for perinuclear antineutrophil cytoplasmic antibodies by indirect immunofluorescence assay. An active and effective management of a case essentially calls for clear perception of differentiating dengue-induced lupus flare, antineutrophil cytoplasmic antibody-related nephropathy, and/or dengue-induced de-novo lupus disease. Dengue viremia may be the trigger for immune complex formation in patients who are predisposed to developing autoimmune diseases. The present case explains the importance of considering the diagnosis of dengue-related lupus nephritis as an atypical occurrence in appropriate situations, as in this case. It would not be improper to regard this escalating disease as an expanded feature of dengue. Keywords: kidney biopsy, glomerulonephritis, segmental sclerosis, lupus flare, dengue viremia, autoimmune, de-novo lupus nephritis

  12. Risk factors of systemic lupus erythematosus flares during pregnancy.

    Science.gov (United States)

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

    2014-12-01

    This review examines the risk factors for the development of systemic lupus erythematosus (SLE) flares during pregnancy. In preconception, anti-DNA, hypocomplementemia, previous thrombosis, triple antiphospholipid (aPL) antibody positivity, active lupus nephritis and discontinuation of medications such as hydroxychloroquine and azathioprine are factors associated with pregnancy failure. During pregnancy, SLE flares are associated with aPL antibodies, synergic changes of pregnancy on Th1 and TH2 cytokines, other cytokines and chemokines that interact with hormones such as estrogen and prolactin that amplify the inflammatory effect. From the clinical point of view, SLE activity at pregnancy onset, thrombocytopenia, lupus nephritis, arterial hypertension, aPL syndromes, preeclampsia is associated with lupus flares and fetal complications. In puerperium, the risk factors of flares are similar to pregnancy. Hyperactivity of immune system, autoantibodies, hyperprolactinemia, active lupus nephritis, decrease in TH2 cytokines with increase in TH1 cytokines probably participate in SLE flare. The SLE flares during pregnancy make the difference between an uncomplicated pregnancy and pregnancy with maternal and fetal complications. Therefore, the knowledge of risk factors leads the best treatment strategies to reduce flares and fetal complications in SLE patients.

  13. Prolactin has a pathogenic role in systemic lupus erythematosus.

    Science.gov (United States)

    Jara, Luis J; Medina, Gabriela; Saavedra, Miguel A; Vera-Lastra, Olga; Torres-Aguilar, Honorio; Navarro, Carmen; Vazquez Del Mercado, Monica; Espinoza, Luis R

    2017-01-28

    Prolactin, a 23-kDa peptide hormone, is produced by the anterior pituitary gland and extrapituitary sites including the immune cells. Prolactin (PRL) participates in innate and adaptive immune response. PRL stimulates the immune cells by binding to receptor (PRL-R). Binding of PRL to its receptor activates the Janus kinase-signal transducer (JAK-STAT). Activation of these cascades results in endpoints such as immunoestimulator and immunosupressor action. Prolactin belongs to the network of immune-neuroendocrine interaction. Hyperprolactinemia has been found in patients with systemic lupus erythematosus (SLE), and new evidence has confirmed a significant correlation between serum PRL levels and disease activity. PRL participates in activation of SLE during pregnancy and in pathogenesis of lupus nephritis, neuropsychiatric, serosal, hematologic, articular, and cutaneous involvement. Hyperprolactinemia was associated with increase IgG concentrations, anti-DNA antibodies, immune complex, glomerulonephritis, and accelerated mortality in murine lupus. Bromocriptine, a dopamine analog that suppresses PRL secretion, was associated with decreased lupus activity, prolonged lifespan, and restoration of immune competence in experimental model. In clinical trials, bromocriptine and derivative drugs showed beneficial therapeutic effect in treating human lupus, including pregnancy. Taken together, clinical and experimental results leave little doubt that PRL indeed contributes to the pathogenesis and clinical expression of SLE.

  14. Risk of infective endocarditis in patients with systemic lupus erythematosus in Taiwan: a nationwide population-based study.

    Science.gov (United States)

    Chang, Y S; Chang, C C; Chen, Y H; Chen, W S; Chen, J H

    2017-10-01

    Objectives Patients with systemic lupus erythematosus are considered vulnerable to infective endocarditis and prophylactic antibiotics are recommended before an invasive dental procedure. However, the evidence is insufficient. This nationwide population-based study evaluated the risk and related factors of infective endocarditis in systemic lupus erythematosus. Methods We identified 12,102 systemic lupus erythematosus patients from the National Health Insurance research-oriented database, and compared the incidence rate of infective endocarditis with that among 48,408 non-systemic lupus erythematosus controls. A Cox multivariable proportional hazards model was employed to evaluate the risk of infective endocarditis in the systemic lupus erythematosus cohort. Results After a mean follow-up of more than six years, the systemic lupus erythematosus cohort had a significantly higher incidence rate of infective endocarditis (42.58 vs 4.32 per 100,000 person-years, incidence rate ratio = 9.86, p systemic lupus erythematosus cohort had lower risk (adjusted hazard ratio 11.64) than that of the younger-than-60-years systemic lupus erythematosus cohort (adjusted hazard ratio 15.82). Cox multivariate proportional hazards analysis revealed heart disease (hazard ratio = 5.71, p systemic lupus erythematosus patients. Conclusions A higher risk of infective endocarditis was observed in systemic lupus erythematosus patients. Risk factors for infective endocarditis in the systemic lupus erythematosus cohort included heart disease, chronic kidney disease, steroid pulse therapy within 30 days, and a recent invasive dental procedure within 30 days.

  15. CARDIOVASCULAR COMPLICATIONS IN SYSTEMIC LUPUS ERYTHEMATOSUS : A STUDY IN A TERTIARY CARE HOSPITAL

    Directory of Open Access Journals (Sweden)

    Purushottam Rao

    2015-08-01

    Full Text Available INTRODUCTION: Systemic Lupus Erythematosus is an autoimmune disorder in which organs and cells undergo damage mediated by tissue - binding auto antibodies and immune complexes 1 . Systemic Lupus Erythematosus is a multigenic disease . 1 People of all sexes, all ages and all ethnic groups are susceptible. Lupus nephrits, infection and thromboembolism contribute for mortality. Cardiac manifestations are not uncommon in systemic lupus erythematosus. It involves all the layers of the heart, pericardium, myocardium and endocardium as well as coronary arteries. AIM AND OBJECTIVES: To find out the prevalence of cardiac manifestations in patients with Systemic Lupus Erythematosus. METHODOLOGY: The study was conducted in t he Department of Medicine, Andhra Medical College, King George Hospital, Visakhapatnam, Andhra Pradesh. It is a tertiary care hospital. It was a study done on a selected population of Systemic Lupus Erythematosus based on the 1997 update of the 1982 Americ an College of Rheumatology classification criteria for Systemic Lupus Erythematosus. RESULTS: In this study consisting of fifty patients, changes suggestive of cardiac disease were seen in 74% of patients. CONCLUSION: Systemic lupus erythematosus is a mult isystem disease. Prevalence of cardiac disease is not uncommon . Pericarditis or pericardial effusion is the most common followed by valvular heart disease

  16. Systemic lupus erythematosus: strategies to improve pregnancy outcomes

    Directory of Open Access Journals (Sweden)

    Yamamoto Y

    2016-07-01

    Full Text Available Yuriko Yamamoto, Shigeru Aoki Perinatal Center for Maternity and Neonate, Yokohama City University Medical Center, Yokohama, Kanagawa Prefecture, Japan Abstract: Systemic lupus erythematosus (SLE is a chronic autoimmune inflammatory disease with a high prevalence in females of childbearing age. Thus, reproduction in SLE patients is a major concern for clinicians. In the past, SLE patients were advised to defer pregnancy because of poor pregnancy outcomes and fear of SLE flares during pregnancy. Investigations to date show that maternal and fetal risks are higher in females with SLE than in the general population. However, with appropriate management of the disease, sufferers may have a relatively uncomplicated pregnancy course. Factors such as appropriate preconception counseling and medication adjustment, strict disease control prior to pregnancy, intensive surveillance during and after pregnancy by both the obstetrician and rheumatologist, and appropriate interventions when necessary play a key role. This review describes the strategies to improve pregnancy outcomes in SLE patients at different time points in the reproduction cycle (preconception, during pregnancy, and postpartum period and also details the neonatal concerns. Keywords: systemic lupus erythematosus, pregnancy outcomes, lupus flare

  17. Gestational weight gain in women with systemic lupus erythematosus.

    Science.gov (United States)

    Eudy, A M; Siega-Riz, A M; Engel, S M; Franceschini, N; Howard, A G; Clowse, M E B; Petri, M

    2017-05-01

    Objective The objective of this study was to estimate the proportion of pregnant women with systemic lupus erythematosus meeting Institute of Medicine guidelines for gestational weight gain and determine correlates of adherence to guidelines. Methods Singleton, live births in the Hopkins Lupus Pregnancy Cohort 1987-2015 were included. Pre-pregnancy weight was the weight recorded 12 months prior to pregnancy/first trimester. Final weight was the last weight recorded in the third trimester. Adherence to Institute of Medicine guidelines (inadequate, adequate, or excessive) was based on pre-pregnancy body mass index. Fisher's exact test and analysis of variance determined factors associated with not meeting guidelines. Stepwise selection estimated predictors of gestational weight gain. Results Of the 211 pregnancies, 34%, 24% and 42% had inadequate, adequate and excessive gestational weight gain, respectively. In exploratory analyses, differences in Institute of Medicine adherence were observed by pre-pregnancy body mass index, race, elevated creatinine during pregnancy and pre-pregnancy blood pressure. Odds of inadequate and excessive gestational weight gain increased 12% with each 1 kg/m(2) increase in pre-pregnancy body mass index. Lower maternal education was associated with increased odds of inadequate and excessive gestational weight gain. Conclusions As in the general population, most women with systemic lupus erythematosus did not meet Institute of Medicine guidelines. Our results identified predictors of gestational weight gain to aid in targeted interventions to improve guideline adherence in this population.

  18. [Dyslipidaemia and atherogenic risk in patients with systemic lupus erythematosus].

    Science.gov (United States)

    Batún Garrido, José Antonio de Jesús; Radillo Alba, Hugo Alberto; Hernández Núñez, Éufrates; Olán, Francisco

    2016-07-15

    Dyslipidaemia is a common comorbidity in patients with systemic lupus erythematosus. Fifty-one patients were included. Variables associated with the disease and the drugs used were recorded. Atherogenic risk was calculated. Chi square was used for categorical variables. ANOVA was performed and a logistic regression model to determine the association of the variables with the presence of dyslipidaemia. A percentage of 68.6 had dyslipidaemia. A significant difference between the presence of dyslipidaemia and activity index measured by SLEDAI was found, the presence of lupus nephritis, use of prednisone≥20mg/day, evolution of the disease<3 years. Significance between the absence of dyslipidaemia and use of hydroxychloroquine was found. SLEDAI≥4 and the use of prednisone≥20mg/day were independently associated with the presence of dyslipidaemia. The average of Castelli rate was 5.02, the Kannel index was 2.97 and triglyceride/HDL-C ratio was 5.24. Patients with systemic lupus erythematosus have a high prevalence of dyslipidaemia and a high atherogenic rate, which increases cardiovascular risk significantly. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  19. Psoriasis in systemic lupus erythematosus: a single-center experience.

    Science.gov (United States)

    Tselios, Konstantinos; Yap, Kristy Su-Ying; Pakchotanon, Rattapol; Polachek, Ari; Su, Jiandong; Urowitz, Murray B; Gladman, Dafna D

    2017-04-01

    The coexistence of psoriasis with systemic lupus erythematosus (SLE) has been reported in limited case series, raising hypotheses about shared pathogenetic mechanisms. Nevertheless, important differences regarding treatment do exist. The aim of the present study was to determine the prevalence and characteristics of psoriasis in a defined cohort of lupus patients. Patients with psoriasis were retrieved from the University of Toronto Lupus Clinic from its inception in 1970 up to 2015. Charts were hand-searched to collect information concerning demographic, clinical, and therapeutic variables. Patients were matched with non-psoriasis lupus patients to identify the impact of supervening psoriasis on lupus activity, damage accrual, and venous thromboembolic (VTEs) and cardiovascular events (CVEs). Psoriasis was diagnosed in 63 patients (49 females, 14 males) for a prevalence of 3.46% (63/1823). The male-to-female ratio was significantly higher in non-psoriasis patients (0.286 vs. 0.138, p = 0.017). Plaque psoriasis was the most prominent type (55/63, 87.3%) whereas three patients had pustular disease; one had psoriatic arthritis. Nine patients (14.3%) were administered systemic treatment with methotrexate (n = 5), azathioprine (n = 1), ustekinumab (n = 3), and etanercept (n = 1). Psoriasis was definitely deteriorated by hydroxychloroquine in one patient. There was no significant impact of psoriasis on disease activity, damage accrual, VTEs, and CVEs. The prevalence of psoriasis was twice as high as that of the general Canadian population in this lupus cohort. Plaque psoriasis was the most prominent subtype, and topical treatment was adequate in the majority of patients. Supervening psoriasis had no significant impact on lupus activity and damage accrual.

  20. Management of systemic lupus erythematosus during pregnancy: challenges and solutions

    Directory of Open Access Journals (Sweden)

    Knight CL

    2017-03-01

    Full Text Available Caroline L Knight, Catherine Nelson-Piercy Division of Women’s Health, Women’s Health Academic Centre, King’s College London and King’s Health Partners, St Thomas’ Hospital, London, UK Abstract: Systemic lupus erythematosus (SLE is a chronic, multisystem autoimmune disease predominantly affecting women, particularly those of childbearing age. SLE provides challenges in the prepregnancy, antenatal, intrapartum, and postpartum periods for these women, and for the medical, obstetric, and midwifery teams who provide their care. As with many medical conditions in pregnancy, the best maternal and fetal–neonatal outcomes are obtained with a planned pregnancy and a cohesive multidisciplinary approach. Effective prepregnancy risk assessment and counseling includes exploration of factors for poor pregnancy outcome, discussion of risks, and appropriate planning for pregnancy, with consideration of discussion of relative contraindications to pregnancy. In pregnancy, early referral for hospital-coordinated care, involvement of obstetricians and rheumatologists (and other specialists as required, an individual management plan, regular reviews, and early recognition of flares and complications are all important. Women are at risk of lupus flares, worsening renal impairment, onset of or worsening hypertension, preeclampsia, and/or venous thromboembolism, and miscarriage, intrauterine growth restriction, preterm delivery, and/or neonatal lupus syndrome (congenital heart block or neonatal lupus erythematosus. A cesarean section may be required in certain obstetric contexts (such as urgent preterm delivery for maternal and/or fetal well-being, but vaginal birth should be the aim for the majority of women. Postnatally, an ongoing individual management plan remains important, with neonatal management where necessary and rheumatology follow-up. This article explores the challenges at each stage of pregnancy, discusses the effect of SLE on pregnancy and

  1. Ocular Complications in Cutaneous Lupus Erythematosus: A Systematic Review with a Meta-Analysis of Reported Cases

    OpenAIRE

    Arrico, L; A. Abbouda; I. Abicca; Malagola, R.

    2015-01-01

    Ocular complications associated with cutaneous lupus erythematosus (CLE) are less studied compared with those ones associated with systemic lupus erythematosus (SLE). The main ocular sites involved in patients affected by discoid lupus erythematosus (DLE) are eyelids followed by orbit and periorbit, the least being cornea. The most common complications are blepharitis usually affecting the lower lid and associated with some type of lid lesion such as plaque or erythematosus patches and madaro...

  2. The economic burden of systemic lupus erythematosus in Asia: the current state.

    Science.gov (United States)

    Mak, A

    2010-10-01

    Systemic lupus erythematosus (SLE) is a chronic systemic inflammatory disorder which imposes considerable negative impact on patients' function and quality of life, and it appears to entail substantial loss of work productivity and healthcare cost. Although much has been studied regarding the epidemiology, pathogenesis, disease activity, disease damage and pharmacological treatment of SLE, publications on the economic burden of lupus are scarce. As the majority of lupus patients are residing in the Asia Pacific region where many are financially and socially deprived, and, from what we know from the current literature, work disability of lupus patients in Asia is substantial, cost-of-illness studies on SLE are thus particularly relevant in countries around the region. Reliable data from properly conducted prospective SLE cost studies are imperative for policymakers to efficiently distribute healthcare resources, especially in Asia where limited resources are unable to cope with the huge population. In this paper, we review the current state of cost-of-illness research on lupus in Asia and analyze the reasons why such studies are urgently required in the Asia Pacific region.

  3. Lupus cystitis in Korean patients with systemic lupus erythematosus: risk factors and clinical outcomes.

    Science.gov (United States)

    Koh, J H; Lee, J; Jung, S M; Ju, J H; Park, S-H; Kim, H-Y; Kwok, S-K

    2015-10-01

    This study was performed to investigate the clinical characteristics of lupus cystitis and determine the risk factors and clinical outcomes of lupus cystitis in patients with systemic lupus erythematosus (SLE). We retrospectively reviewed 1064 patients at Seoul St. Mary's Hospital in Seoul, Korea, from 1998 to 2013. Twenty-four patients had lupus cystitis. Lupus cystitis was defined as unexplained ureteritis and/or cystitis as detected by imaging studies, cystoscopy, or bladder histopathology without urinary microorganisms or stones. Three-fourths of patients with lupus cystitis had concurrent lupus mesenteric vasculitis (LMV). The initial symptoms were gastrointestinal in nature for most patients (79.2%). High-dose methylprednisolone was initially administered to most patients (91.7%) with lupus cystitis. Two patients (8.3%) died of urinary tract infections. Sixty-five age- and sex-matched patients with SLE who were admitted with other manifestations were included as the control group. Patients with lupus cystitis showed a lower C3 level (p = 0.031), higher SLE Disease Activity Index score (p = 0.006), and higher ESR (p = 0.05) upon admission; more frequently had a history of LMV prior to admission (p lupus (p = 0.031) than did patients with SLE but without lupus cystitis. The occurrence of lupus cystitis was associated with a history of LMV (OR, 21.794; 95% CI, 4.061-116.963). The median follow-up period was 3.4 years, and the cumulative one-year mortality rate was 20%. Complications developed in 33.3% of patients with lupus cystitis and were related to survival (log-rank p = 0.021). Our results suggest that the possibility of lupus cystitis should be considered when a patient with SLE and history of LMV presents with gastrointestinal symptoms or lower urinary tract symptoms. Development of complications in patients with lupus cystitis can be fatal. Thus, intensive treatment and follow-up are needed, especially in the presence of

  4. A complicated multisystem flare of systemic lupus erythematosus during pregnancy.

    Science.gov (United States)

    Webster, Philip; Nelson-Piercy, Catherine; Lightstone, Liz

    2017-02-08

    We report a case of systemic lupus erythematosus (SLE) in a young woman who became pregnant amid a severe flare. She continued to have active disease in the face of aggressive treatments complicated by several side effects of immunosuppressive drugs including recurrent sepsis and gestational diabetes. Her fetus was at risk for congenital heart block during the second and third trimesters. Despite an extremely guarded prognosis, she delivered a healthy baby girl. This case highlights the complexities of SLE management during pregnancy. We discuss the therapeutic options available in pregnancy, and highlight the importance of cross-specialty multidisciplinary care in these women.

  5. [The influence of pregnancy on the systemic lupus erythematosus].

    Science.gov (United States)

    Basheva, S; Nikolov, A; Monov, C; Shumnalieva, R; Monova, D; Rashkov, R

    2014-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease caused by the interaction between genetic and environment factors which leads to abnormal immune responses. SLE affects more commonly women of childbearing age which raises the following questions--the influence of the disease activity on pregnancy and the influence of pregnancy on disease activity. On the one hand physiological changes occurring during pregnancy could lead to increased SLE activity, on the other hand the latter could mimic SLE activity. Differentiating these manifestations is important for the clinical practice--pregnancy and delivering guidance and SLE therapy.

  6. New onset of systemic lupus erythematosus during pregnancy

    Directory of Open Access Journals (Sweden)

    Laura Keisa

    2016-04-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic inflammatory disease that frequently affects young women. If SLE is first suspected during pregnancy, the diagnostic criterion is the same as for nonpregnant women and the treatment should be started as soon as the diagnosis is made. This study describes a 31-year-old pregnant woman who was hospitalized with shortness of breath, pain, and stiffness in phalangeal joints. The new onset of SLE was diagnosed. The disease was controlled by medical treatment. The patient had successful pregnancy and a healthy baby was delivered. [Int J Reprod Contracept Obstet Gynecol 2016; 5(4.000: 1221-1224

  7. Vitamin D and systemic lupus erythematosus: state of the art.

    Science.gov (United States)

    Schneider, Laiana; Dos Santos, Amanda Senna Pereira; Santos, Marcele; da Silva Chakr, Rafael Mendonça; Monticielo, Odirlei Andre

    2014-08-01

    Systemic lupus erythematosus (SLE) is a systemic inflammatory disease associated with genetic, environmental, hormonal, and immunological factors. One of these factors is vitamin D deficiency. Vitamin D plays many roles in the immune system. Several studies have suggested a potential role in the development of autoimmune diseases. SLE patients have low serum levels of vitamin D, which increase the possibility of an association between vitamin deficiency and disease onset and evolution. This review of the literature presents an analysis of the aspects related to the immunoregulatory effects of vitamin D and its importance for SLE, as well as the recommendations for vitamin D supplementation in these patients.

  8. Vaccination of Adult Patients with Systemic Lupus Erythematosus in Portugal

    Directory of Open Access Journals (Sweden)

    Maria Francisca Moraes-Fontes

    2016-01-01

    Full Text Available In the wake of the Portuguese vaccination program 50th anniversary it seems appropriate to review vaccination in patients with systemic lupus erythematosus. Controversial issues as regards the association between autoimmune diseases, infections, and vaccines are discussed as well as vaccine safety and efficacy issues as regards chronic immunosuppressant (IS drug therapy. After a brief overview of national policies, specific recommendations are made as regards vaccination for adult patients with SLE with a particular focus on current IS therapy and unmet needs.

  9. GLUCOCORTICOIDS IN THE THERAPY OF SYSTEMIC LUPUS ERYTHEMATOSUS

    Directory of Open Access Journals (Sweden)

    T. M. Reshetnyak

    2014-07-01

    Full Text Available Despite a more than fifty-year period after the introduction of glucocorticoids (GCs into therapeutic practice; they have headed the list of anti-inflammatory drugs so far. The paper analyzes current views on the terminology of GCs, mechanism of action, standardization of indications for their use in systemic lupus erythematosus, choice of a dose and a regimes of the drugs, as well as the rates of their decrease; the authors also review Russian and foreign literature on this problem and their own data.

  10. Chronic meningitis in systemic lupus erythematosus: An unusual etiology

    Directory of Open Access Journals (Sweden)

    Anu Gupta

    2014-01-01

    Full Text Available Chronic aseptic meningitis is a rare manifestation of systemic lupus erythematosus (SLE. Apart from immunological causes and drugs, the aseptic meningitis group can include some unidentified viral infections that cannot be detected by routine microbiological testing. It is imperative to do complete cerebrospinal fluid (CSF workup before implicating the symptoms to disease activity or drugs, as untreated infections cause significant mortality in SLE. We present a case of young female with SLE who presented with chronic meningitis of an uncommon etiology.

  11. GLUCOCORTICOIDS IN THE THERAPY OF SYSTEMIC LUPUS ERYTHEMATOSUS

    Directory of Open Access Journals (Sweden)

    T. M. Reshetnyak

    2013-01-01

    Full Text Available Despite a more than fifty-year period after the introduction of glucocorticoids (GCs into therapeutic practice; they have headed the list of anti-inflammatory drugs so far. The paper analyzes current views on the terminology of GCs, mechanism of action, standardization of indications for their use in systemic lupus erythematosus, choice of a dose and a regimes of the drugs, as well as the rates of their decrease; the authors also review Russian and foreign literature on this problem and their own data.

  12. Diffusion tensor imaging in neuropsychiatric systemic lupus erythematosus

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    Qualls Clifford R

    2010-07-01

    Full Text Available Abstract Background Neuropsychiatric systemic lupus erythematosus (NPSLE is associated with increased morbidity and mortality. Methods We used Diffusion Tensor Imaging (DTI to assess white matter abnormalities in seventeen NPSLE patients, sixteen SLE patients without NPSLE, and twenty age- and gender-matched controls. Results NPSLE patients differed significantly from SLE and control patients in white matter integrity of the body of the corpus callosum, the left arm of the forceps major and the left anterior corona radiata. Conclusions Several possible mechanisms of white matter injury are explored, including vascular injury, medication effects, and platelet or fibrin macro- or microembolism from Libman-Sacks endocarditis.

  13. Acquired hemophilia A in a patient with systemic lupus erythematosus.

    Science.gov (United States)

    Ishikawa, T; Tsukamoto, N; Suto, M; Uchiumi, H; Mitsuhashi, H; Yokohama, A; Maesawa, A; Nojima, Y; Naruse, T

    2001-06-01

    A patient with systemic lupus erythematosus (SLE) developed acquired hemophilia A. The patient, a 24-year-old Japanese woman, was referred to our hospital because of uncontrollable bleeding following a tooth extraction. Laboratory examination revealed prolonged APTT (116 seconds), reduced factor VIII activity (2.8 %) and the presence of factor VIII inhibitor at a titer of 46.5 Bethesda units/ml. Transfusion of prothrombin complex concentrate and activated prothrombin complex concentrate followed by administration of prednisolone and cyclophosphamide successfully arrested bleeding and reduced the factor VIII inhibitor level. Acquired hemophilia A is a rare but lethal condition. Rapid diagnosis and introduction of adequate therapies are critical.

  14. Alveolar hemorrhage as the initial presentation of systemic lupus erythematosus.

    Science.gov (United States)

    de Holanda, Bruna A; Barreto, Isabela G Menna; de Araujo, Isadora S Gomes; de Araujo, Daniel B

    2016-01-01

    Alveolar hemorrhage (AH) is a rare syndrome that can often occur in autoimmune diseases, blood clotting disorders, infection or by acute inhalation injury, presenting rapid evolution and high mortality, especially with late diagnosis and treatment. Among the autoimmune diseases, there are reported cases in patients with primary antiphospholipid syndrome (PAPS), vasculitis and systemic lupus erythematosus (SLE). An early diagnosis is an essential tool in the successful management of this complication, requiring aggressive treatment based on vigorous immunosuppression and broad-spectrum antibiotic. We describe here a case of alveolar hemorrhage associated with glomerulonephritis as the open presentation in a patient with SLE.

  15. Vaccination of Adult Patients with Systemic Lupus Erythematosus in Portugal

    Science.gov (United States)

    Moraes-Fontes, Maria Francisca; Antunes, Ana Margarida; Gruner, Heidi; Riso, Nuno

    2016-01-01

    In the wake of the Portuguese vaccination program 50th anniversary it seems appropriate to review vaccination in patients with systemic lupus erythematosus. Controversial issues as regards the association between autoimmune diseases, infections, and vaccines are discussed as well as vaccine safety and efficacy issues as regards chronic immunosuppressant (IS) drug therapy. After a brief overview of national policies, specific recommendations are made as regards vaccination for adult patients with SLE with a particular focus on current IS therapy and unmet needs. PMID:27069477

  16. Disseminated tuberculosis in a patient with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    J. Harikrishna

    2012-10-01

    Full Text Available Systemic lupus erythematosus (SLE is a systemic autoimmune disorder. Intercurrent infections and nephritis are important causes of mortality in SLE. Among infections, tuberculosis (TB is of particular importance as SLE patients are more susceptible to develop active TB, prior TB can precipitate SLE in genetically susceptible individuals and similar clinical presentations of SLE flare and TB may lead to delayed diagnosis. We report a patient with SLE, who developed disseminated TB. The present case highlights the importance of a high index of suspicion and focussed evaluation in the diagnosis of intercurrent infections, particularly TB in patients with SLE.

  17. Systemic lupus erythematosus in the elderly: clinical and immunological characteristics.

    OpenAIRE

    Font, J.; Pallarés, L.; Cervera, R; López-Soto, A; Navarro, M.; Bosch, X.; Ingelmo, M

    1991-01-01

    Systemic lupus erythematosus (SLE) predominantly affects young women in their 20s. In 40 out of 250 (16%) patients with SLE seen in our hospital disease onset occurred after the age of 50. The interval between the time of onset and diagnosis was five years in this older group compared with three years in the younger group. Arthritis, as a first symptom, was less common in the older onset group. During the follow up a lower incidence of arthritis, malar rash, photosensitivity, and nephropathy ...

  18. Alveolar hemorrhage as the initial presentation of systemic lupus erythematosus

    Science.gov (United States)

    de Holanda, Bruna A.; Barreto, Isabela G. Menna; de Araujo, Isadora S. Gomes

    2016-01-01

    Alveolar hemorrhage (AH) is a rare syndrome that can often occur in autoimmune diseases, blood clotting disorders, infection or by acute inhalation injury, presenting rapid evolution and high mortality, especially with late diagnosis and treatment. Among the autoimmune diseases, there are reported cases in patients with primary antiphospholipid syndrome (PAPS), vasculitis and systemic lupus erythematosus (SLE). An early diagnosis is an essential tool in the successful management of this complication, requiring aggressive treatment based on vigorous immunosuppression and broad-spectrum antibiotic. We describe here a case of alveolar hemorrhage associated with glomerulonephritis as the open presentation in a patient with SLE. PMID:27994272

  19. Genetic Factors in Systemic Lupus Erythematosus: Contribution to Disease Phenotype

    Science.gov (United States)

    Ceccarelli, Fulvia; Perricone, Carlo; Borgiani, Paola; Ciccacci, Cinzia; Rufini, Sara; Cipriano, Enrica; Alessandri, Cristiano; Spinelli, Francesca Romana; Sili Scavalli, Antonio; Novelli, Giuseppe; Valesini, Guido; Conti, Fabrizio

    2015-01-01

    Genetic factors exert an important role in determining Systemic Lupus Erythematosus (SLE) susceptibility, interplaying with environmental factors. Several genetic studies in various SLE populations have identified numerous susceptibility loci. From a clinical point of view, SLE is characterized by a great heterogeneity in terms of clinical and laboratory manifestations. As widely demonstrated, specific laboratory features are associated with clinical disease subset, with different severity degree. Similarly, in the last years, an association between specific phenotypes and genetic variants has been identified, allowing the possibility to elucidate different mechanisms and pathways accountable for disease manifestations. However, except for Lupus Nephritis (LN), no studies have been designed to identify the genetic variants associated with the development of different phenotypes. In this review, we will report data currently known about this specific association. PMID:26798662

  20. Immunosuppression in pregnant women with systemic lupus erythematosus.

    Science.gov (United States)

    Ponticelli, Claudio; Moroni, Gabriella

    2015-05-01

    Most pregnancies are successful in women with systemic lupus erythematosus, particularly if the disease is quiescent and there are no signs of active nephritis. There is no major impact of immunosuppression on maternal outcome. However, high doses of cyclosporine and glucocorticoids are used which may favor development of hypertension or preeclampsia. Some immunosuppressive drugs may exert toxic effects on the fetus. Glucocorticoids may cause small birth weight, and azathioprine and calcineurin inhibitors may be associated with lower birth weight, gestational age and prematurity. Cyclophosphamide may cause fetal malformation when given in the first trimester. Mycophenolate and leflunomide are teratogenic drugs and should be withdrawn before conception in case of programmed pregnancy or should be rapidly discontinued in case of unexpected pregnancy. Option counseling for pregnancy and correct use of immunosuppressive drugs are prerequisites for a successful pregnancy in women with lupus.

  1. Cardiac tamponade as initial presentation in systemic lupus erythematosus.

    Science.gov (United States)

    Jawaid, Ambreen; Almas, Aysha

    2014-05-01

    Systemic Lupus Erythematosus (SLE) is one of the many diseases known as 'the great imitators' because it can have diverse presentations and so is misunderstood for other illnesses. This case illustrates a 19 years old girl with SLE who presented as cardiac tamponade which is a rare feature of lupus pericarditis requiring medical and surgical treatment. Even after pericardiocentesis and steroid therapy there was a re-accumulation of the pericardial fluid resulting in cardiac tamponade which led to pericardial window formation. This case draws attention to the need to consider the diagnosis of tamponade in patients with connective tissue disease and dyspnea or hemodynamic compromise. It also outlines the treatment options available so that surgical referral, if needed, can be done timely for this rare but life threatening manifestation of SLE.

  2. Unmet Needs of Patients with Systemic Lupus Erythematosus

    Science.gov (United States)

    Danoff-Burg, Sharon; Friedberg, Fred

    2009-01-01

    The authors' goal was to assess unmet needs of patients with systemic lupus erythematosus (SLE). Participants (N = 112), who were recruited through the mailing list and support group meetings of a Lupus Alliance of America Affiliate, completed a survey based on prior research. All participants perceived at least 1 unmet need. The most frequently reported unmet needs were in the physical symptoms domain. Older patients were more likely than younger patients to have higher levels of unmet needs related to physical and psychological functioning. African American patients were more likely than white patients to have higher levels of unmet needs related to health services and information. Our findings document the high prevalence and variety of unmet needs among these patients, as well as variations among demographic groups. To address unmet needs of SLE patients, targeted referrals to patient educators, mental health professionals, and support organizations are important adjuncts to medical treatment. PMID:19297299

  3. Genetically engineered biological agents in therapy for systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Elena Aleksandrovna Aseeva

    2013-01-01

    Full Text Available Systemic lupus erythematosus (SLE is a prototype for chronic autoimmune disease. Its prevalence is 20 to 70 cases per 100,000 women and varies by race and ethnicity. Despite considerable progress in traditional therapy, many problems associated with the management of these patients need to be immediately solved: thus, 50-80% are found to have activity signs and/or frequent exacerbations and about 30% of the patients have to stop work; Class IV lupus nephritis increases the risk of terminalrenal failure. In the past 20 years great progress has been made in studying the pathogenesis of SLE: biological targets to affect drugs have been sought and fundamentally new therapeutic goals defined. Belimumab is the first genetically biological agent specially designed to treat SLE, which is rightly regarded as one of the most important achievements of rheumatology in the past 50 years.

  4. [Tapering and termination of immunosuppressive therapy : Systemic lupus erythematosus].

    Science.gov (United States)

    Aringer, M; Leuchten, N; Fischer-Betz, R

    2017-02-01

    Similar to patients with other rheumatic diseases, patients with systemic lupus erythematosus (SLE) nowadays can also have the desire to terminate immunosuppressive and immunomodulatory medications. In order to provide appropriate advice to patients, the two main issues are the risk of severe adverse events under long-term therapy with any drug and the perceived risk of a flare, in particular of severe flares. The risks of long-term therapy vary greatly between drugs, ranging from severe unacceptable risks with cyclophosphamide and higher dose glucocorticoids to low risks usually outweighed by long-term benefits with hydroxychloroquine. The individual risk of flares is often difficult to estimate but clinical remission and at least 3 years of immunosuppression are recommended for lupus nephritis. The duration of remission can also be shorter in cases of milder forms of disease. This review article tries to put the available evidence into a clinical perspective and to derive concrete recommendations.

  5. Mitral Valve Surgery in Patients with Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Mahnoosh Foroughi

    2014-01-01

    Full Text Available Valvular heart disease is the common cardiac manifestation of systemic lupus erythematosus (SLE with a tendency for mitral valve regurgitation. In this study we report a case of mitral valve replacement for mitral stenosis caused by Libman-Sacks endocarditis in the setting of SLE. In addition, we provide a systematic review of the literature on mitral valve surgery in the presence of Libman-Sacks endocarditis because its challenge on surgical options continues. Surgical decision depends on structural involvement of mitral valve and presence of active lupus nephritis and antiphospholipid antibody syndrome. Review of the literature has also shown that outcome is good in most SLE patients who have undergone valvular surgery, but association of antiphospholipid antibody syndrome with SLE has negative impact on the outcome.

  6. Targeting BLyS in systemic lupus erythematosus.

    Science.gov (United States)

    Liu, Yaoyang; La Cava, Antonio

    2012-05-01

    Systemic lupus erythematosus (SLE) is a chronic disabling autoimmune disease that significantly impacts the quality of life of patients, and can associate with several complications including end-stage renal disease and shortened lifespan. A central component in the pathogenesis of SLE is the B-cell production of autoantibodies to multiple self-antigens. Since, B lymphocyte stimulator (BLyS) plays a key role in the selection, differentiation and survival of most B cells, it has been studied as a therapeutic target in SLE. After a gap of more than fifty years without new drugs being approved for this disease, the human neutralizing anti-BLyS monoclonal antibody belimumab has recently been approved by the FDA for SLE therapy. This review provides an overview on the targeting of BLyS in lupus animal models, the use of belimumab in human SLE, and relevant patents.

  7. Hydrocephalus in an elderly man with systemic lupus erythematosus.

    Science.gov (United States)

    Chen, Wei-Sheng; Wu, Tsai-Hung; Chou, Chung-Tei; Tsai, Chang-Youh

    2009-06-01

    A 71-year-old man presented with quadriplegia, seizures, dysarthria, motor aphasia and urinary incontinence lasting for several years. The development of proteinuria and increased susceptibility to infections brought the physician's attention to possible underlying autoimmune diseases. Laboratory investigations revealed evidence for systemic lupus erythematosus (SLE) and antiphospholipid syndrome. Imaging studies showed obstructive hydrocephalus. Several courses of methylprednisolone therapies followed by maintenance therapy with low-dose steroid, ventriculoperitoneal shunt, and antihypertensives improved the proteinuria and dysarthria but not the urinary incontinence or dementia. A thromboembolic event in the central nervous system secondary to phospholipid antibodies or lupus activity may represent a pathogenetic basis for hydrocephalus. When encountering a patient with hydrocephalus but without apparent predisposing factors, it is always important to include SLE as a differential diagnosis.

  8. Aplastic anemia as a feature of systemic lupus erythematosus: a case report and literature review.

    Science.gov (United States)

    Chalayer, Émilie; Ffrench, Martine; Cathébras, Pascal

    2015-06-01

    Peripheral cytopenias are common in systemic lupus erythematosus, but bone marrow involvement is rarely reported. Aplastic anemia is the result of immune-mediated destruction of hematopoietic stem cells causing pancytopenia and characterized by an empty bone marrow. This rare but serious disease has been described as an unusual manifestation of systemic lupus erythematosus. We reviewed the 25 cases published in the English language literature and discuss the clinical presentation, outcome, treatment, and pathophysiology of aplastic anemia as a complication of systemic lupus erythematosus. We report here the first case of aplastic anemia associated with systemic lupus erythematosus treated with an allogeneic hematopoietic stem cell transplant. Over one half of patients received concomitantly the diagnoses of systemic lupus erythematosus and aplastic anemia. No clinical or histological features can distinguish primary aplastic anemia from aplastic anemia occurring in systemic lupus erythematosus patients. The overall mortality is about 15% and corticosteroid-based therapy alone or in combination with other immunomodulatory drugs can restore bone marrow function. Systemic lupus erythematosus may be complicated by bone marrow involvement. The diagnosis of peripheral cytopenias should be confirmed by bone marrow aspiration. All these patients should receive cortisone as a first treatment. Plasma exchanges seem to have some efficacy. Other different immunomodulatory therapies were used with variable results.

  9. Management of systemic lupus erythematosus during pregnancy: challenges and solutions.

    Science.gov (United States)

    Knight, Caroline L; Nelson-Piercy, Catherine

    2017-01-01

    Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease predominantly affecting women, particularly those of childbearing age. SLE provides challenges in the prepregnancy, antenatal, intrapartum, and postpartum periods for these women, and for the medical, obstetric, and midwifery teams who provide their care. As with many medical conditions in pregnancy, the best maternal and fetal-neonatal outcomes are obtained with a planned pregnancy and a cohesive multidisciplinary approach. Effective prepregnancy risk assessment and counseling includes exploration of factors for poor pregnancy outcome, discussion of risks, and appropriate planning for pregnancy, with consideration of discussion of relative contraindications to pregnancy. In pregnancy, early referral for hospital-coordinated care, involvement of obstetricians and rheumatologists (and other specialists as required), an individual management plan, regular reviews, and early recognition of flares and complications are all important. Women are at risk of lupus flares, worsening renal impairment, onset of or worsening hypertension, preeclampsia, and/or venous thromboembolism, and miscarriage, intrauterine growth restriction, preterm delivery, and/or neonatal lupus syndrome (congenital heart block or neonatal lupus erythematosus). A cesarean section may be required in certain obstetric contexts (such as urgent preterm delivery for maternal and/or fetal well-being), but vaginal birth should be the aim for the majority of women. Postnatally, an ongoing individual management plan remains important, with neonatal management where necessary and rheumatology followup. This article explores the challenges at each stage of pregnancy, discusses the effect of SLE on pregnancy and vice versa, and reviews antirheumatic medications with the latest guidance about their use and safety in pregnancy. Such information is required to effectively and safely manage each stage of pregnancy in women with SLE.

  10. Management of systemic lupus erythematosus during pregnancy: challenges and solutions

    Science.gov (United States)

    Knight, Caroline L; Nelson-Piercy, Catherine

    2017-01-01

    Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease predominantly affecting women, particularly those of childbearing age. SLE provides challenges in the prepregnancy, antenatal, intrapartum, and postpartum periods for these women, and for the medical, obstetric, and midwifery teams who provide their care. As with many medical conditions in pregnancy, the best maternal and fetal–neonatal outcomes are obtained with a planned pregnancy and a cohesive multidisciplinary approach. Effective prepregnancy risk assessment and counseling includes exploration of factors for poor pregnancy outcome, discussion of risks, and appropriate planning for pregnancy, with consideration of discussion of relative contraindications to pregnancy. In pregnancy, early referral for hospital-coordinated care, involvement of obstetricians and rheumatologists (and other specialists as required), an individual management plan, regular reviews, and early recognition of flares and complications are all important. Women are at risk of lupus flares, worsening renal impairment, onset of or worsening hypertension, preeclampsia, and/or venous thromboembolism, and miscarriage, intrauterine growth restriction, preterm delivery, and/or neonatal lupus syndrome (congenital heart block or neonatal lupus erythematosus). A cesarean section may be required in certain obstetric contexts (such as urgent preterm delivery for maternal and/or fetal well-being), but vaginal birth should be the aim for the majority of women. Postnatally, an ongoing individual management plan remains important, with neonatal management where necessary and rheumatology followup. This article explores the challenges at each stage of pregnancy, discusses the effect of SLE on pregnancy and vice versa, and reviews antirheumatic medications with the latest guidance about their use and safety in pregnancy. Such information is required to effectively and safely manage each stage of pregnancy in women with SLE

  11. Ultraviolet-A1 irradiation therapy for systemic lupus erythematosus

    Science.gov (United States)

    2017-01-01

    Systemic lupus erythematosus (lupus, SLE) is a chronic autoimmune disease characterized by the production of autoantibodies, which bind to antigens and are deposited within tissues to fix complement, resulting in widespread systemic inflammation. The studies presented herein are consistent with hyperpolarized, adenosine triphosphate (ATP)-deficient mitochondria being central to the disease process. These hyperpolarized mitochondria resist the depolarization required for activation-induced apoptosis. The mitochondrial ATP deficits add to this resistance to apoptosis and also reduce the macrophage energy that is needed to clear apoptotic bodies. In both cases, necrosis, the alternative pathway of cell death, results. Intracellular constituents spill into the blood and tissues, eliciting inflammatory responses directed at their removal. What results is “autoimmunity.” Ultraviolet (UV)-A1 photons have the capacity to remediate this aberrancy. Exogenous exposure to low-dose, full-body, UV-A1 radiation generates singlet oxygen. Singlet oxygen has two major palliative actions in patients with lupus and the UV-A1 photons themselves have several more. Singlet oxygen depolarizes the hyperpolarized mitochondrion, triggering non-ATP-dependent apoptosis that deters necrosis. Next, singlet oxygen activates the gene encoding heme oxygenase (HO-1), a major governor of systemic homeostasis. HO-1 catalyzes the degradation of the oxidant heme into biliverdin (converted to bilirubin), Fe, and carbon monoxide (CO), the first three of these exerting powerful antioxidant effects, and in conjunction with a fourth, CO, protecting against injury to the coronary arteries, the central nervous system, and the lungs. The UV-A1 photons themselves directly attenuate disease in lupus by reducing B cell activity, preventing the suppression of cell-mediated immunity, slowing an epigenetic progression toward SLE, and ameliorating discoid and subacute cutaneous lupus. Finally, a combination of

  12. Mechanisms of Dyslipoproteinemias in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Eduardo F. Borba

    2006-01-01

    Full Text Available Autoimmunity and inflammation are associated with marked changes in lipid and lipoprotein metabolism in SLE. Autoantibodies and cytokines are able to modulate lipoprotein lipase (LPL activity, a key enzyme in lipid metabolism, with a consequent “lupus pattern” of dyslipoproteinemia characterized by elevated levels of very low-density lipoprotein cholesterol (VLDL and triglycerides (TG and lower high-density lipoprotein cholesterol (HDL levels. This pattern favors an enhanced LDL oxidation with a subsequent deleterious foam cell formation. Autoantibodies and immunocomplexes may aggravate this oxidative injury by inducing accumulation and deposition of oxLDL in endothelial cells. Drugs and associated diseases usually magnify the close interaction of these factors and further promote the proatherogenic environment of this disease.

  13. Sharing experiences and social support requests in an Internet forum for patients with systemic lupus erythematosus.

    Science.gov (United States)

    Mazzoni, Davide; Cicognani, Elvira

    2014-05-01

    Internet forums represent a useful but understudied resource to understand psychosocial aspects of living with systemic lupus erythematosus. This study was aimed to describe the demand/supply of social support through the Internet in relation with the description of personal illness experiences. All the posts (118) from an Italian forum for systemic lupus erythematosus patients were collected and analyzed combining qualitative content analysis with statistical textual analysis. The results showed different purposes for posts: starting new relationships, seeking information, receiving emotional support, and giving a contribution. Lexical analysis identified three ways of describing patients' experiences. Discussion focuses on the relationship between the requested/offered support and systemic lupus erythematosus experiences.

  14. A case of subacute cutaneous lupus erythematosus as a result of ranibizumab (Lucentis treatment

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    Marko Andric

    2013-01-01

    Full Text Available Cutaneous lupus erythematosus is a previously undiagnosed side-effect of ranibizumab. Here, we present a case of an 82-year-old female Caucasian patient with wet age-related macular degeneration. Following a single intraocular injection of Lucentis (ranibizumab, she developed a subacute cutaneous lupus erythematosus which, with treatment, took nearly 12 months to resolve. This shows that cutaneous lupus erythematosus is a potential side-effect of many medications, including ranibizumab, as in our case and, in an aging population where polypharmacy is a growing reality, clinicians should be aware of how to diagnose and best manage such cases.

  15. Acute closed lock in a patient with lupus erythematosus: case review.

    Science.gov (United States)

    Bade, D M; Lovasko, J H; Montana, J; Waide, F L

    1992-01-01

    Collagen failure has been shown to result in synovitis, joint adhesions, and internal joint derangement. This case report illustrates the similarities between patients with systemic lupus erythematosus and an internally deranged temporomandibular joint and patients with internal derangement with no lupus erythematosus. If abnormalities in intra-articular collagen tissue lead to adhesion formation and restrict normal mobility during translatory movements, joint mechanics would be compromised. Arthritic changes, vasculitis, and synovitis of systemic lupus erythematosus appear to be contributory factors in this pathophysiologic process. Diagnostic and therapeutic arthroscopic surgery was performed. Acute and chronic signs of synovitis were observed during surgery, and tissue samples were obtained for histologic interpretation.

  16. Illness perceptions in patients with systemic lupus erythematosus and proliferative lupus nephritis.

    Science.gov (United States)

    Daleboudt, G M N; Broadbent, E; Berger, S P; Kaptein, A A

    2011-03-01

    This study investigated the illness perceptions of patients with systemic lupus erythematosus (SLE) and whether perceptions are influenced by type of treatment for proliferative lupus nephritis. In addition, the illness perceptions of SLE patients were compared with those of patients with other chronic illnesses. Thirty-two patients who had experienced at least one episode of proliferative lupus nephritis were included. Patients were treated with either a high or low-dose cyclophosphamide (CYC) regimen (National Institutes of Health [NIH] vs. Euro-Lupus protocol). Illness perceptions were measured with the Brief Illness Perception Questionnaire (B-IPQ) and a drawing assignment. The low-dose CYC group perceived their treatment as more helpful than the high-dose CYC group. In comparison with patients with asthma, SLE patients showed more negative illness perceptions on five of the eight illness perception domains. Drawings of the kidney provided additional information about perceptions of treatment effectiveness, kidney function and patients' understanding of their illness. Drawing characteristics showed associations with perceptions of consequences, identity, concern and personal control. These findings suggest that the type of treatment SLE patients with proliferative lupus nephritis receive may influence perceptions of treatment effectiveness. In addition, patients' drawings reveal perceptions of damage caused by lupus nephritis to the kidneys and the extent of relief provided by treatment. The finding that SLE is experienced as a more severe illness than other chronic illnesses supports the need to more frequently assess and aim to improve psychological functioning in SLE patients.

  17. Low prevalence of Pneumocystis pneumonia in hospitalized patients with systemic lupus erythematosus: review of a clinical data warehouse.

    Science.gov (United States)

    Kapoor, T M; Mahadeshwar, P; Nguyen, S; Li, J; Kapoor, S; Bathon, J; Giles, J; Askanase, A

    2017-01-01

    Objective In the era of powerful immunosuppression, opportunistic infections are an increasing concern in systemic lupus erythematosus. One of the best-studied opportunistic infections is Pneumocystis pneumonia; however, the prevalence of Pneumocystis pneumonia in systemic lupus erythematosus is not clearly defined. This study evaluates the prevalence of Pneumocystis pneumonia in hospitalized systemic lupus erythematosus patients, with a focus on validating the Pneumocystis pneumonia and systemic lupus erythematosus diagnoses with clinical information. Methods This retrospective cohort study evaluates the prevalence of Pneumocystis pneumonia in all systemic lupus erythematosus patients treated at Columbia University Medical Center-New York Presbyterian Hospital between January 2000 and September 2014, using electronic medical record data. Patients with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) and patients with renal transplants (including both early and late post-transplant patients) represented immunocompromised control groups. Patients with systemic lupus erythematosus, Pneumocystis pneumonia, HIV/AIDS, or renal transplant were identified using diagnostic codes from the International Classification of Diseases, Ninth Revision (ICD-9). Results Out of 2013 hospitalized systemic lupus erythematosus patients, nine had presumed Pneumocystis pneumonia, yielding a low prevalence of Pneumocystis pneumonia in systemic lupus erythematosus of 0.45%. Three of the nine Pneumocystis pneumonia cases were patients with concomitant systemic lupus erythematosus and HIV/AIDS. Only one of these nine cases was histologically confirmed as Pneumocystis pneumonia, in a patient with concomitant systemic lupus erythematosus and HIV/AIDS and a CD4 count of 13 cells/mm(3). The prevalence of Pneumocystis pneumonia in renal transplant patients and HIV/AIDS patients was 0.61% and 5.98%, respectively. Conclusion Given the reported high rate of adverse effects

  18. The Avise Lupus Test and Cell-bound Complement Activation Products Aid the Diagnosis of Systemic Lupus Erythematosus

    OpenAIRE

    2016-01-01

    Background: Systemic lupus erythematosus (SLE) is a multifaceted disease, and its diagnosis may be challenging. A blood test for the diagnosis of SLE, the Avise Lupus test, has been recently commercialized and validated in clinical studies. Objectives: To evaluate the use of the Avise Lupus test by community rheumatologists. Methods: The study is a longitudinal, case-control, retrospective review of medical charts. Cases had a positive test result, and controls had a negative result; all pati...

  19. Experience of living with Systemic Lupus Erythematosus: A phenomenological study

    Directory of Open Access Journals (Sweden)

    Mazhari Azad F

    2015-05-01

    Full Text Available Background and Objective: Systemic Lupus Erythematosus (SLE is a chronic autoimmune disease. Addition to various clinical manifestations it has spiritual, psycho-social and economic consequences. The present research was conducted aimed to describe the lived experiences of patients living with systemic lupus erythematosus.  Materials and Method: A qualitative study, using phenomenology approach was used. Ten patients with SLE who referred to rheumatology clinic in Bandar Abbas were selected through purposive sampling in 2012. Data generation was done through in-depth unstructured interview. Colaizzi's method of data analysis was used for analysis.  Results: The results of study demonstrated four main themes including Mental-emotional consequences, pain of recurrence of disease, forgotten socio-economic needs and inability to perform duties.  Conclusion: According to results, patients experience the wide range of problems and they need to take care of family and society concerning the physical aspects and receiving psychological support in psychological aspects. Because of the medical expenses and work-related disability, economic pressures propel them to dependence and challenge in meeting the needs of health care. Some of their needs remain unanswered

  20. Cerebrospinal fluid cytology studies of neuropsychiatric systemic lupus erythematosus

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    CHEN Lin

    2013-02-01

    Full Text Available Background Neuropsychiatric systemic lupus erythematosus (NP-SLE is the central nervous system (CNS involvement of systemic lupus erythematosus (SLE. The diagnosis of NP-SLE may be difficult due to the lack of specific biomarker. CNS cerebrospinal fluid (CSF cytology is diagnostic significant to CNS autoimmune disease. This paper described the characteristics of CSF cytology and evaluated its diagnostic value in NP-SLE. Methods Seventy-six eligible patients with clear diagnosis of NP-SLE were collected for CSF cytological examinations. Results The CSF cytology findings of 25 cases in 76 were abnormal, among which 16 cases showed lymphocytic inflammatory reactions; 8 cases had slight increase of neutrophile granulocyte percent; 9 cases showed lymphocyte-neutrophile inflammation. Activated lymphocytes together with monocytes were present in 24 cases. Among those cases, abnormal endocytosis of monocytes, which presented as monocytes phagocytosing lymphocytes or plasmocytes, was shown in 17 cases; plasmocytes were found in 17 cases. Conclusion The diagnosis of NP-SLE is based on clinical, neuroimaging and CSF studies. Among these methods, the CSF cytology findings are quite useful in practice, since the CSF cytological inflammatory reactions, especially the presentation of abnormal phagocytes in CSF is typical in NP-SLE and indicates its vasculitic mechanism.

  1. [Angina Pectoris in a Young Woman with Lupus Erythematosus].

    Science.gov (United States)

    Braumann, Simon; Bartram, Malte P; Pfister, Roman; Michels, Guido

    2017-09-01

    History and clinical findings We present a 31-year old woman with a 6-year history of cutaneous lupus erythematosus (CLE) who presented to the emergency room with typical chest pain. ECG and transthoracic echocardiography were normal. Her working diagnosis of pericarditis was made due to systemic progression of her lupus erythematosus (LE). Treatment with NSAIDs was initiated and her immunosuppressive regimen intensified. The patient was discharged after resolution of her symptoms. A week later, the patient was seen at the rheumatology clinic with recurrence and aggravation of her symptoms. She was found to have elevated troponin and cardiac enzymes and therefore underwent cardiac catheterization, revealing three vessel coronary artery disease. Therapy and course The patient underwent urgent open surgical myocardial revascularization. Despite the immunosuppressive therapy the postoperative course was uneventful. Conclusions The risk for coronary artery disease in LE patients is very high. Particularly in young women presenting with chest pain, regardless of typical cardiopulmonary manifestations of LE such as pericarditis and pleurisy, acute coronary syndrome should always be considered. © Georg Thieme Verlag KG Stuttgart · New York.

  2. A Patient With Plaque Type Morphea Mimicking Systemic Lupus Erythematosus

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    Wardhana Wardhana

    2015-04-01

    Full Text Available Morphea is an uncommon connective tissue disease with the most prominent feature being thickening or fibrosis of the dermal without internal organ involvement. It is also known as a part of localized scleroderma. Based on clinical presentation and depth of tissue involvement, morphea is classified into several forms, and about two thirds of adults with morphea have plaque type. Overproduction of collagen production by fibroblast is the cause of abnormality in morphea, and the hyperactivity mechanism of fibroblast is still unknown, although there are several mechanisms already proposed. Plaque type morphea is actually a benign and self limited. Plaque type morphea that mimicking systemic lupus erythematosus in clinical appearance, such as alopecia and oral mucosal ulcers, is uncommon. A case of plaque type morphea mimicking systemic lupus erythematosus in a 20 year old woman was discussed. The patient was treated with local and systemic immunosuppressant and antioxydant. The patient’s condition is improved without any significant side effects. Key words: morphea, plaque type.

  3. Bone mineral density in juvenile systemic lupus erythematosus

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    Castro T.C.M.

    2002-01-01

    Full Text Available We evaluated spine bone mineral density (BMD in Brazilian children with juvenile systemic lupus erythematosus (JSLE in order to detect potential predictors of reduction in bone mass. A cross-sectional study of BMD at the lumbar spine level (L2-L4 was conducted on 16 female JSLE patients aged 6-17 years. Thirty-two age-matched healthy girls were used as control. BMD at the lumbar spine was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in patients and controls. Disease duration, mean daily steroid doses, mean cumulative steroid doses and JSLE activity measured by the systemic lupus erythematosus disease activity index (SLEDAI were determined for all JSLE patients based on their medical charts. All parameters were used as potential determinant factors for bone loss. Lumbar BMD tended to be lower in the JSLE patients, however, this difference was not statistically significant (P = 0.10. No significant correlation was observed in JSLE girls between BMD and age, height, Tanner stage, disease duration, corticosteroid use or disease activity. We found a weak correlation between BMD and weight (r = 0.672. In the JSLE group we found no significant parameters to correlate with reduced bone mass. Disease activity and mean cumulative steroid doses were not related to BMD values. We did not observe reduced bone mass in female JSLE.

  4. Does parvovirus infection have a role in systemic lupus erythematosus?

    Science.gov (United States)

    Hod, Tami; Zandman-Goddard, Giselle; Langevitz, Pnina; Rudnic, Hagit; Grossman, Zehava; Rotman-Pikielny, Pnina; Levy, Yair

    2017-01-23

    We sought to evaluate a possible link between parvovirus B19 infection and the clinical and laboratory expression of systemic lupus erythematosus (SLE). SLE patients were examined to evaluate their clinical status and disease activity. A complete Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score was obtained for each patient. In addition, we determined the level of systemic involvement throughout the course of the disease. Blood levels of IgM and IgG antibodies to parvovirus B19, levels of anti-dsDNA, C3, and C4 were measured. A PCR real-time assay was used to determine the presence of parvovirus B19 genetic material. The viral genome was found in sera of 2 of 51(3.9%) patients with SLE. There was no correlation between viral serology and the clinical and serological parameters of the disease. More SLE patients with secondary antiphospholipid syndrome (APS) had IgG and IgM antibodies to the virus (p < 0.029 and p < 0.018, respectively). These patients also had a higher titer of IgG antibodies to parvovirus B19 compared to SLE patients without APS. In this group of SLE patients, no association was found between parvovirus infection and the presence or activity of SLE. The results of the study suggest an association between parvovirus infection and antibody production directed against phospholipids.

  5. [Cardiovascular manifestations in systemic lupus erythematosus in Dakar: Descriptive study about 50 cases].

    Science.gov (United States)

    Ngaïdé, A A; Ly, F; Ly, K; Diao, M; Kane, Ad; Mbaye, A; Lèye, M; Aw, F; Sarr, S A; Dioum, M; Ndao, C T; Gaye, N D; Ndiaye, M B; Bodian, M; Bah, M B; Ndiaye, M; Cissé, A F; Kouamé, I; Tabane, A; Mingou, J S; Thiombiano, P; Kane, A; Bâ, S A

    2016-12-01

    Systemic lupus erythematosus is a non-specific inflammatory disorder of an organ of unknown cause and autoimmune origin. Visceral injuries, including those cardiovascular, determine the prognosis of this disease primarily affecting women. The objectives of this study were to determine the frequency and describe the cardiovascular manifestations in systemic lupus erythematosus in a lupus population of the Dakar region. This is a multicenter prospective study descriptive and analytical conducted in the region of Dakar (Senegal) from 14 February 2011 to 2 July 2012. Patients were either hospitalized or monitored as outpatients. Included were all patients with lupus and meeting at least four criteria of the American College of Rheumatology of lupus disease classification 1997. All patients underwent physical examination, an electrocardiogram and an echocardiogram looking for cardiovascular damage. The collected data were entered into the Epi Info version 3.5.1 and processed with SPSS 16.0 software. Quantitative variables are described in the median and the qualitative workforce, percentage and frequency. We have included 50 patients. The average age of the population was 36.18 years. A female predominance is noted with a sex ratio man/woman of 0.09. Cardiovascular functional symptoms were dominated by dyspnea stage II to IV NYHA (26%) and palpitations (22%). The physical signs we have found were mainly tachycardia (40%), spontaneous turgor of the jugular veins (29%), a muffling of the heart sounds (29%) and a infandibulopulmonairy shock (18%). The frequency of cardiovascular events was 46%. Electrical cardiac events were dominated by sinus tachycardia (40%) of repolarization disorders (16.3%) type of ischemia, injury, ischemia injury, necrosis and hypertrophy with 18% atrial and left ventricular hypertrophy each. Furthermore, one case of BAV first degree at 280 ms was recorded. We found 19 cases of pericarditis including 2 tamponade, 3 cases of dilated cardiomyopathy

  6. Neurological Manifestations in Systemic Lupus Erythematosus: A Single Centre Study from North East India

    Science.gov (United States)

    Kakati, Sanjeeb; Ahmed, Sobur U; Hussain, Masaraf

    2017-01-01

    Introduction Neurological manifestations although common in Systemic Lupus Erythematosus (SLE), are often not recognized due to their diversed and varied presentation. Therefore, the study was planned to highlight the pattern of neurological involvement in SLE to help in early recognition. Aim To study the pattern of neurological involvement in SLE and its correlation with disease activity and different investigation. Materials and Methods This hospital based prospective observational study was carried out from August 2009 to July 2010. Diagnosed cases of SLE [based upon American Rheumatism Association (ARA) criteria] who presented with neurological manifestations at the time of diagnosis or develop during the course of the disease were included in the study. They were assessed clinically and investigated with neuroimaging and neurophysiological tests as applicable. Results In total, 52 consecutive patients with SLE were evaluated, 92% were female. The most common age group was 21 to 25 years. Nervous system involvement was found in 19 (36.54%) patients. Cognitive impairment was the most frequent manifestation, present in 11 (57.89%) patients followed by seizure disorder in eight patients (42.1%). Peripheral neuropathy was diagnosed in eight (42.1%), acute confusional state in six (31.57%) and headache and depression was diagnosed in five (26.31%) patients each. Less common manifestations were psychosis, movement disorder and aseptic meningitis. Percentage of neurological manifestations directly correlated with disease activity. A significant difference was found in Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score between the patients with Neuro Psychiatric Systemic Lupus Erythematosus (NPSLE) and those without NPSLE (32.42±16.34 Vs 17.3±10.6). Conclusion Neurological involvement in SLE is seen relatively early in the course of the disease with cognitive impairment being the most common manifestation and correlate with disease activity.

  7. Serum interleukin-17 levels are associated with nephritis in childhood-onset systemic lupus erythematosus

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    Karina de Oliveira Peliçari

    2015-05-01

    Full Text Available OBJECTIVES: To determine the serum interleukin-17 (IL-17 levels in childhood-onset systemic lupus erythematosus patients and to evaluate the association between IL-17 and clinical manifestations, disease activity, laboratory findings and treatment. METHODS: We included 67 consecutive childhood-onset systemic lupus erythematosus patients [61 women; median age 18 years (range 11-31], 55 first-degree relatives [50 women; median age 40 years (range 29-52] and 47 age- and sex-matched healthy controls [42 women; median age 19 years (range 6-30]. The childhood-onset systemic lupus erythematosus patients were assessed for clinical and laboratory systemic lupus erythematosus manifestations, disease activity [Systemic Lupus Erythematosus Disease Activity Index (SLEDAI], cumulative damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR Damage Index] and current drug use. Serum IL-17 levels were measured by an enzyme-linked immunosorbent assay using commercial kits. RESULTS: The median serum IL-17 level was 36.3 (range 17.36-105.92 pg/mL in childhood-onset systemic lupus erythematosus patients and 29.47 (15.16-62.17 pg/mL in healthy controls (p=0.009. We observed an association between serum IL-17 levels and active nephritis (p=0.01 and migraines (p=0.03. Serum IL-17 levels were not associated with disease activity (p=0.32, cumulative damage (p=0.34, or medication use (p=0.63. CONCLUSION: IL-17 is increased in childhood-onset systemic lupus erythematosus and may play a role in the pathogenesis of neuropsychiatric and renal manifestations. Longitudinal studies are necessary to determine the role of IL-17 in childhood-onset systemic lupus erythematosus.

  8. A case of "refractory" lupus erythematosus profundus responsive to rituximab [case report].

    LENUS (Irish Health Repository)

    McArdle, Adrian

    2012-02-01

    Lupus erythematosus profundus is a rare complication of systemic lupus erythematosus characterized by the presence of deep, tender subcutaneous nodules. A 22-year-old African-American female with extensive lupus profundus resistant to conventional therapies was treated with two infusions of the anti-CD20 monoclonal antibody, rituximab, at a dosage of 1,000 mg each. The patient demonstrated a remarkable clinical response as indicated by the disappearance of the nodules. B-cell depletion therapy with rituximab used alone or in combination with other therapies may be a viable option in patients with lupus profundus refractory to current therapies.

  9. Dry Eye and Corneal Langerhans Cells in Systemic Lupus Erythematosus

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    Miklós D. Resch

    2015-01-01

    Full Text Available Purpose. Investigation of dry eye and corneal Langerhans cells (LCs in systemic lupus erythematosus (SLE. Methods. Prospective consecutive case series of 27 SLE patients and 27 control subjects. Dry eye was evaluated by lid-parallel conjunctival folds (LIPCOF, Schirmer test, tear break-up time (TBUT, and ocular surface disease index (OSDI questionnaire. In vivo investigation of corneal LCs density and morphology (LCM was performed with confocal corneal microscopy (Heidelberg Retina Tomograph with Rostock Cornea Module. Results. Tear production and stability were pathological in SLE subjects compared to control (Schirmer: 8.45 ± 9.82 mm/5 min versus 11.67 ± 3.21 mm/5 min; TBUT: 6.86 ± 3.53 s versus 11.09 ± 3.37 s. OSDI was significantly greater in SLE patients (25.95 ± 17.92 than in controls (11.06 ± 7.18. Central LC density was greater in SLE patients (43.08 ± 48.67 cell/mm2 than in controls (20.57 ± 21.04 cell/mm2. There was no difference in the peripheral LC density (124.78 ± 165.39 versus 78.00 ± 39.51 cell/mm2. LCM was higher in SLE patients in the centre (1.43 ± 0.79 and in the periphery (2.89 ± 0.42 compared to controls (centre: 1.00 ± 0.69, periphery: 2.35 ± 0.54. Conclusions. Significant changes in dry eye parameters and marked increase of central LCs could be demonstrated in SLE patients. SLE alters not only the LC density but also the morphology, modifies corneal homeostasis, and might contribute to the development of dry eye.

  10. BK viruria and viremia in children with systemic lupus erythematosus.

    Science.gov (United States)

    Gupta, Nirupama; Nguyen, Cuong Q; Modica, Renee F; Elder, Melissa E; Garin, Eduardo H

    2017-04-11

    BK virus (BKV) is a ubiquitous polyoma virus that lies dormant in the genitourinary tract once acquired in early childhood. In states of cellular immunodeficiency, the virus can reactivate to cause hemorrhagic cystitis and nephritis. Children with systemic lupus erythematosus (SLE) have an increased risk of developing infectious complications secondary to their immunocompromised state from the administration of several immuno-modulatory drugs. Currently, there are no data regarding the prevalence of BK viruria or viremia in children with SLE. We conducted a prospective cohort study involving children with SLE of 18 years and younger. We obtained urine and blood samples at baseline and every 3 months up to 1 year for BK virus detection by real-time, quantitative polymerase chain reaction analysis. A comprehensive review of demographic information, clinical characteristics and medication history was also obtained. Thirty-two pediatric patients (26 females and 6 males) with SLE were enrolled. Median age at the time of SLE diagnosis and enrollment into study was 13.6 years and 16.0 years old, respectively. The prevalence at enrollment was 3.1% (1/32) for BK viruria and 6.2% (2/32) for BK viremia. During the study period, 3 patients had viruria, 5 had viremia and 4 had both viruria and viremia. Of the 12 patients with BKV reactivation, only one was positive for microscopic hematuria, all others were asymptomatic. A total of nine of 97(9.2%) urine samples and 10 of 96(10.4%) blood samples were positive for BK virus. The most commonly utilized biologics in this cohort group were Rituximab (90.6%), Abatacept (12.5%), and Belimumab (9.3%). The type of medication exposure and clinical characteristics did not statistically differ between the groups that did or did not have BK viruria and/or viremia. Our study suggests that pediatric patients with SLE have BK viremia and/or viruria at a higher rate than the general healthy population, although the significance of the

  11. Cutaneous lupus erythematosus and systemic lupus erythematosus are associated with clinically significant cardiovascular risk: a Danish nationwide cohort study.

    Science.gov (United States)

    Hesselvig, J Halskou; Ahlehoff, O; Dreyer, L; Gislason, G; Kofoed, K

    2017-01-01

    Systemic lupus erythematosus (SLE) is a well-known cardiovascular risk factor. Less is known about cutaneous lupus erythematosus (CLE) and the risk of developing cardiovascular disease (CVD). Therefore, we investigated the risk of mortality and adverse cardiovascular events in patients diagnosed with SLE and CLE. We conducted a cohort study of the entire Danish population aged ≥ 18 and ≤ 100 years, followed from 1997 to 2011 by individual-level linkage of nationwide registries. Multivariable adjusted Cox regression models were used to estimate the hazard ratios (HRs) for a composite cardiovascular endpoint and all-cause mortality, for patients with SLE and CLE. A total of 3282 patients with CLE and 3747 patients with SLE were identified and compared with 5,513,739 controls. The overall HR for the composite CVD endpoint was 1.31 (95% CI 1.16-1.49) for CLE and 2.05 (95% CI 1.15-3.44) for SLE. The corresponding HRs for all-cause mortality were 1.32 (95% CI 1.20-1.45) for CLE and 2.21 (95% CI 2.03-2.41) for SLE. CLE and SLE were associated with a significantly increased risk of CVD and all-cause mortality. Local and chronic inflammation may be the driver of low-grade systemic inflammation.

  12. Pregnancy-related issues in women with systemic lupus erythematosus.

    Science.gov (United States)

    Singh, Abha G; Chowdhary, Vaidehi R

    2015-02-01

    While fertility is preserved in females with systemic lupus erythematosus (SLE), it is well established that pregnancy in these patients is associated with adverse maternal and fetal outcomes, including pregnancy loss, pre-eclampsia, preterm delivery and intrauterine growth retardation, as well as neonatal mortality. Mechanisms underlying these adverse outcomes are poorly understood, and better understanding of these would allow development of targeted and personalized treatment strategies. Established risk factors for adverse pregnancy outcomes include active disease within 6 months prior to conception and during pregnancy, active nephritis, maternal hypertension, antiphospholipid antibodies and hypocomplementemia. While intensive monitoring is recommended, the comparative effectiveness of appropriate management strategies is unclear. While current strategies are able to achieve live births in 85-90% of pregnancies, certain aspects such as prevention of preterm birth, treatment of congenital heart block due to neonatal lupus and recurrent pregnancy loss despite best management, remains challenging. Pregnancy is also associated with an increased risk of flare of lupus, particularly in patients with active disease at time of conception or within 6 months prior to conception. Pregnant patients with SLE should be followed in a high-risk obstetric clinic, and care should be closely coordinated between the obstetrician and rheumatologist.

  13. New-onset systemic lupus erythematosus during pregnancy.

    Science.gov (United States)

    Zhao, Chunmei; Zhao, Jijun; Huang, Yuefang; Wang, Zilian; Wang, Hongyue; Zhang, Hui; Xu, Hanshi; Yang, Niansheng

    2013-06-01

    Few studies have been published focusing on the clinical features of new-onset systemic lupus erythematosus (SLE) during pregnancy. This study examined the clinical characteristics of SLE during pregnancy or puerperium. The clinical characteristics and serological parameters of 48 patients with onset of SLE during pregnancy were retrospectively compared with those of age-matched new-onset SLE patients who were diagnosed in a period of more than 12 months without pregnancy (n = 65) and age-matched preeclampsia patients (n = 48). SLE tended to occur during the first and second trimesters (33 and 42 %, respectively). Lupus nephritis (LN) and severe thrombocytopenia were more commonly seen in new-onset SLE during pregnancy than in patients without pregnancy (68.8 vs 35.4 % and 25 vs 9.2 %, respectively, p pregnancy (n = 23), LN patients with pregnancy (n = 33) had more prominent proteinuria and nephrotic syndrome (p pregnancy had early onset of symptoms during gestation and were characterized by presence of fever, malar lesion, autoantibodies, hypocomplementemia, hyperuricemia, active urinary sediment, and multi-organ involvement. In conclusion, patients with their first onset of lupus during pregnancy generally have more severe disease with higher prevalence of renal and platelet involvement.

  14. Gastrointestinal system manifestations in juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Sönmez, Hafize Emine; Karhan, Asuman Nur; Batu, Ezgi Deniz; Bilginer, Yelda; Gümüş, Ersin; Demir, Hülya; Yüce, Aysel; Özen, Seza

    2017-02-16

    Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18 years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68.4%). The GIS manifestations due to SLE were autoimmune hepatitis (AIH) (n = 8) and lupus enteritis (n = 1). Manifestations associated with SLE were hepatomegaly and hypertransaminasemia due to macrophage activation syndrome (MAS) (n = 3) and hepatic steatosis (n = 1). GIS manifestations as a result of the adverse events of drugs were as follows: toxic hepatitis (n = 3; associated with methotrexate and nonsteroidal anti-inflammatory drugs in one, methotrexate in another, and azathioprine in another patient), azathioprine-induced cholestatic hepatitis (n = 1), and gastritis associated with corticosteroid (n = 1). In one patient, acute appendicitis occurred as a coincidence. In this study, one of every five pediatric SLE patients had GIS-related manifestations. GIS involvement may occur as an initial manifestation of the disease.

  15. Risk factors for neuropsychiatric manifestations in children with systemic lupus erythematosus: case-control study.

    Science.gov (United States)

    Zuniga Zambrano, Yenny Carolina; Guevara Ramos, Juan David; Penagos Vargas, Nathalia Elena; Benitez Ramirez, Diana Carol; Ramirez Rodriguez, Sandra Milena; Vargas Niño, Adriana Carolina; Izquierdo Bello, Alvaro Hernando

    2014-09-01

    Neuropsychiatric symptoms in children with systemic lupus erythematosus cause high morbidity and disability. This study analyzed risk factors associated with neuropsychiatric presentation in patients with systemic lupus erythematosus aged information of patients with a diagnosis of systemic lupus erythematosus who were hospitalized with or without neuropsychiatric symptoms was collected between March 2007 and January 2012. Clinical variables, laboratory examinations, neuroimages, and disease activity (Systemic Erythematosus Lupus Disease Activity Index) and damage (Systemic Lupus International Collaborating Clinics) indices were analyzed. A total of 90 patients were selected, 30 with neuropsychiatric symptoms. The patients' average age was 12.2 years. The most common neuropsychiatric symptoms were seizures, migraine, and depression. The average Systemic Erythematosus Lupus Disease Activity Index was 19.86 (S.D. 10.83) and the average Systemic Lupus International Collaborating Clinics index was 2.02 (S.D. 2.43), with higher values in patients with neuropsychiatric symptoms (P = 0.001). The levels of complement C3 and C4 were significantly higher in patients with a neuropsychiatric disorder (P = 0.003). Lupus anticoagulant was found in 51.5% of patients with neuropsychiatric symptoms (odds ratio, 3.7; 95% confidence interval, 1.3-10.0). Immunosuppression with azathioprine, rituximab, or cyclophosphamide delayed the time to neuropsychiatric systemic lupus erythematosus development by 18.5 months (95% confidence interval, 10.6-26.5) compared to patients who did not receive these agents. The presence of lupus anticoagulant was a risk factor in our patients. The use of immunosuppressants, such as cyclophosphamide, rituximab, and azathioprine, delayed the presentation of neuropsychiatric manifestations of lupus. Copyright © 2014 Elsevier Inc. All rights reserved.

  16. Photoprotective behaviour and sunscreen use: impact on vitamin D levels in cutaneous lupus erythematosus.

    LENUS (Irish Health Repository)

    Cusack, Caitriona

    2008-10-01

    Sun exposure of the skin, independent of dietary sources, may provide sufficient vitamin D in healthy individuals. A recent study of patients with cutaneous lupus erythematosus concluded that over 70% of them restrict their sun exposure.

  17. Successful Pregnancy Following Assisted Reproduction in Woman With Systemic Lupus Erythematosus and Hypertension: A Case Report.

    Science.gov (United States)

    de Macedo, José Fernando; de Macedo, Gustavo Capinzaiki; Campos, Luciana Aparecida; Baltatu, Ovidiu Constantin

    2015-09-01

    Patients with systemic lupus erythematosus have a poor prognosis of pregnancy, since it is associated with significant maternal and fetal morbidity, including spontaneous miscarriage, pre-eclampsia, intrauterine growth restriction, fetal death and pre-term delivery. We report a case with successful pregnancy in a patient with systemic lupus erythematosus and hypertension. A 39-year-old nulliparous woman presented with systemic lupus erythematosus with antinuclear and antiphospholipid antibodies, hypertension and recurrent pregnancy loss presented for assisted reproduction. The patient responded well to enoxaparin and prednisone during both assisted reproduction and prenatal treatment. This case report indicates that prescription of immunosuppressant and blood thinners can be safely recommended throughout the whole prenatal period in patients with systemic lupus erythematosus. Enoxaparin and prednisone may be prescribed concurrently during pregnancy.

  18. Systemisk lupus erythematosus i Fyns Amt. En epidemiologisk undersøgelse

    DEFF Research Database (Denmark)

    Voss, A B; Green, A; Junker, P

    1999-01-01

    The incidence and prevalence of systemic lupus erythematosus (SLE) has never been investigated in Denmark, whereas international studies have reached divergent results. In the study patients were ascertained from diagnosis-based registers of inpatients and outpatients, notifications from physicians...

  19. Systemic lupus erythematosus and the economic perspective: a systematic literature review and points to consider

    OpenAIRE

    Turchetti, G.; Yazdany, J; Palla, I.; Yelin, E; Mosca, M.

    2012-01-01

    Systemic lupus erythematosus (SLE) is a chronic, disabling, progressive disease, with many associated comorbidities, affecting patients during prime working years resulting in a high economic burden on society, producing high direct, indirect and intangible costs.

  20. Magnetic resonance imaging in neuropsychiatric systemic lupus erythematosus: current state of the art and novel approaches.

    Science.gov (United States)

    Postal, M; Lapa, A Tamires; Reis, F; Rittner, L; Appenzeller, S

    2017-04-01

    Systemic lupus erythematosus is a chronic, inflammatory, immune-mediated disease affecting 0.1% of the general population. Neuropsychiatric manifestations in systemic lupus erythematosus have been more frequently recognized and reported in recent years, occurring in up to 75% of patients during the disease course. Magnetic resonance imaging is known to be a useful tool for the detection of structural brain abnormalities in neuropsychiatric systemic lupus erythematosus patients because of the excellent soft-tissue contrast observed with MRI and the ability to acquire multiplanar images. In addition to conventional magnetic resonance imaging techniques to evaluate the presence of atrophy and white matter lesions, several different magnetic resonance imaging techniques have been used to identify microstructural or functional abnormalities. This review will highlight different magnetic resonance imaging techniques, including the advanced magnetic resonance imaging methods used to determine central nervous system involvement in systemic lupus erythematosus.

  1. Antibodies to early EBV, CMV, and HHV6 antigens in systemic lupus erythematosus patients

    DEFF Research Database (Denmark)

    Rasmussen, N S; Draborg, A H; Nielsen, C T

    2015-01-01

    OBJECTIVES: We investigated the antibody levels against early antigens of Epstein-Barr virus (EBV), cytomegalovirus (CMV), and human herpesvirus 6 (HHV6) in systemic lupus erythematosus (SLE) patients and healthy controls, and further correlated these antibodies to haematology...

  2. Thrombocytopenia in Systemic Lupus Erythematosus: Clinical Manifestations, Treatment, and Prognosis in 230 Patients

    National Research Council Canada - National Science Library

    Jung, Jin-Hee; Soh, Moon-Seung; Ahn, Young-Hwan; Um, Yoo-Jin; Jung, Ju-Yang; Suh, Chang-Hee; Kim, Hyoun-Ah

    2016-01-01

    The aim of the study was to examine the clinical characteristics and prognosis according to severity of thrombocytopenia and response to treatment for thrombocytopenia in patients with systemic lupus erythematosus (SLE...

  3. Flares of systemic lupus erythematosus during pregnancy and the puerperium: prevention, diagnosis and management.

    Science.gov (United States)

    Stojan, George; Baer, Alan N

    2012-07-01

    Systemic lupus erythematosus is a systemic autoimmune disease that primarily affects women in their reproductive age years. Pregnancy in systemic lupus erythematosus now has favorable outcomes for the majority of women. However, flares of disease activity, preeclampsia, fetal loss, intrauterine growth retardation and preterm birth are established risks of such pregnancies. Active lupus nephritis at the time of conception poses the greatest risk for disease flares and poor obstetric outcomes. Patients should delay conception until their lupus has been in remission for at least 6 months. In addition, certain lupus medications are potentially teratogenic and need to be stopped before conception. The signs and symptoms of a lupus flare may mimic those of normal pregnancy, impeding its recognition during pregnancy. Hydroxychloroquine, low-dose prednisone, pulse intravenous methylprednisolone and azathioprine are commonly used to treat lupus flares during pregnancy.

  4. Lupus-related advanced liver involvement as the initial presentation of systemic lupus erythematosus.

    Science.gov (United States)

    Lu, Ming-Chi; Li, Ko-Jen; Hsieh, Song-Chou; Wu, Cheng-Han; Yu, Chia-Li

    2006-12-01

    Systemic lupus erythematosus (SLE), a prototype of systemic autoimmune disease characterized by multiorgan involvement with diverse clinical and serological manifestations, principally affects women in their child-bearing years. Clinically significant hepatic abnormality as the initial presentation of SLE has rarely been reported. Eleven patients with lupus with initial presentation of lupus-related hepatitis were included in this retrospective review. Clinical manifestation, immunological profiles, and risk factors for poor prognosis were analyzed. The most commonly associated clinical manifestations were found to be thrombocytopenia, leukopenia, advancing age, and presence of anti-SSA/Ro antibody and anti-thyroid antibodies. The diagnosis of SLE was delayed due to dominant hepatic abnormalities. Age greater than 50 years and marked hepatic decompensation in accordance with Child classification B and C might suggest poor prognosis (p=0.06). However, the p value was not statistically significant because of the small sample size. Lupus-related hepatitis, particularly in late-onset lupus, is common. In addition, the presence of anti-SSA, anti-thyroglobulin, and anti-microsomal antibodies is indicative of hepatic involvement in patients with SLE.

  5. Immunological aspects of biopsy-proven lupus nephritis in Bahraini patients with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Eman M Farid

    2013-01-01

    Full Text Available Lupus nephritis (LN is a frequent and potentially serious complication of systemic lupus erythematosus (SLE that may influence morbidity and mortality. Immunological investigations are aiding tools to the kidney biopsy findings in early diagnosis, in addition to monitoring the effect of therapy. The aim of the present study is to highlight the role of these investigations in a group of Bahraini patients and to determine whether there is any positive association between these findings and the outcome of LN. The current study is a retrospective case-control study of randomly selected 88 SLE patients, 44 with biopsyproven LN and 44 without, acting as controls. All renal biopsies performed during the period from 1996 to 2012 were classified according to the World Health Organization classification. Immunological investigations analyzed are: Antinuclear antibodies (ANA, anti-ds DNA, anti-ENA, anti-cardiolipin antibodies (abs and complement components C3, C4. Human leukocyte antigen (HLA typing class II was performed on selected cases. All patients had positive ANA (100%. A significantly high frequency of anti-Smith abs among the non-LN group (43.18% compared with the LN group (18.18% was found (P <0.001. On the other hand, the anti-Ro/SSA abs in the non-LN group was also found at a statistically higher frequency (20.45% compared with that in the LN group (4.54% (P <0.01. Anti-ds-DNA abs were found to be higher in the LN group (84.09% compared with the non-LN group (70.45%, but the difference was not statistically significant (P = 0.082. There was a positive association of ANA positivity and low C3 and or C4 in the studied group. In our study, 88.2% of the HLA typed patients had HLADR2, DR3 or both. In conclusion, in our Arabic Bahraini SLE patients, the presence of anti-Smith, anti-Ro/SSA and anti-RNP antibodies and the absence of anti-dsDNA antibodies are independent predictive markers for renal involvement. However, more prospective studies with a

  6. Early Lupus Project - A multicentre Italian study on systemic lupus erythematosus of recent onset.

    Science.gov (United States)

    Sebastiani, G D; Prevete, I; Piga, M; Iuliano, A; Bettio, S; Bortoluzzi, A; Coladonato, L; Tani, C; Spinelli, F R; Fineschi, I; Mathieu, A

    2015-10-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with a high degree of variability at onset that is problematic for a correct and prompt diagnosis. We undertook this project with the purpose of collecting an inception cohort of Italian patients with recent-onset SLE, in order to obtain information on the main clinical and serological characteristics at the beginning of the disease. In this first report we describe the characteristics of this cohort at study entry. All patients with a diagnosis of SLE (1997 ACR criteria) and a disease duration less than 12 months were consecutively enrolled between 1 January 2012 and 31 December 2013 in a multicentre prospective study. Information on clinical and serological characteristics at study entry and then every six months was collected into a specific electronic database. Statistical analysis was performed by means of the Openstat program. Among 122 patients enrolled (103 F) 94.3% were Caucasians. Mean age (SD) of patients at study entry was 37.3 (14.3) years, mean age at disease onset was 34.8 (14.3) years, mean age at diagnosis was 36.9 (14.3) years, and mean disease duration was 2.9 (3.9) months. The frequency of the manifestations included in the 1997 ACR criteria was as follows: ANA 97.5%, immunologic disorders (anti-dsDNA, anti-Sm, antiphospholipid antibodies) 85.2%, arthritis 61.8%, haematologic disorders 55.7%, malar rash 31.1%, photosensitivity 29.5%, serositis 27%, renal disorders 27%, oral/nasal ulcers 11.5%, neurologic disorders 8.2%, and discoid rash 5.7%. The cumulative frequency of mucocutaneous symptoms was 77.8%. At enrolment, autoantibody frequency was: ANA 100%, anti-dsDNA 83.6%, anti-SSA 28%, anticardiolipin 24.5%, anti-nRNP 20.4%, anti-beta2GPI 17.2%, lupus anticoagulant 16.3%, anti-Sm 16%, and anti-SSB 13.1%. In this paper we describe the main clinical and serological characteristics of an Italian inception cohort of patients with recent-onset SLE. At disease onset, mucocutaneous

  7. Bullous Systemic Lupus Erythematosus and Lupus Nephritis in a Young Girl

    Directory of Open Access Journals (Sweden)

    Tooba Momen

    2016-11-01

    Full Text Available Bullous systemic lupus erythematosus (BSLE is an autoimmune blistering disease occurring in patients with systemic lupus erythematosus (SLE. It is a rare disease, especially in children. A 14-year-old girl initially presented with fatigue, generalized vesiculobullous skin lesions, and ulcers over the hard palate and oral mucosa. Clinical investigations revealed hematuria and proteinuria, a high erythrocyte sedimentation rate and titer of antinuclear antibody, and anti-double-stranded DNA. Skin biopsy findings were suggestive of BSLE. A renal biopsy confirmed the features of class V lupus nephritis. Based on the clinical features and investigations, a diagnosis of BSLE with nephritis was made. She received methylprednisolone pulse therapy and hydroxychloroquine; however, it did not alleviate the vesiculobullous eruption, so treatment with dapsone started and resulted in the dramatic disappearance of the lesions. Interruption of dapsone due to hemolysis did not aggravate the bullous disease. During follow-up, she had multiple flare-ups of disease and nephritis without rebound of bullous lesions. BSLE is a rare presentation of SLE in children. Differentiating it from other skin bullous diseases and SLE with blister is important for the correct management. The unusual presentation of this disease may delay the diagnosis and therefore requires a high index of clinical suspicion.

  8. Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives

    OpenAIRE

    Magro-Checa, César; Zirkzee, Elisabeth J.; Huizinga, Tom; Steup-Beekman, G

    2016-01-01

    textabstractNeuropsychiatric systemic lupus erythematosus (NPSLE) is a generic definition referring to a series of neurological and psychiatric symptoms directly related to systemic lupus erythematosus (SLE). NPSLE includes heterogeneous and rare neuropsychiatric (NP) manifestations involving both the central and peripheral nervous system. Due to the lack of a gold standard, the attribution of NP symptoms to SLE represents a clinical challenge that obligates the strict exclusion of any other ...

  9. Toxic Epidermal Necrolysis-Like Lesions and Systemic Lupus Erythematosus Possibly Triggered by Sulfasalazine

    DEFF Research Database (Denmark)

    Krabbe, Simon; Gül, Cigdem; Andersen, Bjarne

    2016-01-01

    elevated ferritin, and muscle wasting. A diagnosis of systemic lupus erythematosus was made, and mycophenolate mofetil and systemic glucocorticoids brought this severe disease under control. Toxic epidermal necrolysis-like lesions and hemophagocytic syndrome have been reported as manifestations of systemic...... lupus erythematosus. This patient possibly had spondyloarthritis or an undifferentiated connective tissue disease at presentation, and we suggest, based on the timing of events, that sulfasalazine may have acted as a trigger of the severe disease manifestations....

  10. Sjögren’s syndrome associated with systemic lupus erythematosus

    OpenAIRE

    Taşdemir, Mehmet; Hasan, Chiar; Ağbaş, Ayşe; Kasapçopur, Özgür; Canpolat, Nur; Sever, Lale; Çalışkan, Salim

    2016-01-01

    Systemic lupus erythematosus and Sjögren’s syndrome are chronic auto- inflammatory disorders which can lead to serious organ damage. Although systemic lupus erythematosus and Sjögren’s syndrome were previously considered two forms of the same disease because of presence of clinical coexistence of these two conditions, the view that they are two different conditions with mutual characteristics has become prominent in recent years. In this paper, we reported a 16 year-old girl who was followed ...

  11. Systemic lupus erythematosus and pregnancy outcomes: an update and review of the literature.

    Science.gov (United States)

    Peart, Erica; Clowse, Megan E B

    2014-03-01

    This review synthesizes new data from the studies published between 2011 and 2013, with particular focus on the different information gleaned by various study types. Population-based cohorts have demonstrated that women with systemic lupus erythematosus (SLE) have fewer live births and more pregnancy complications, but can have successful live births after having a poor outcome. A retrospective study suggests that only 4 months, not the traditional 6 months of disease quiescent SLE prior to pregnancy improves outcomes. Prospective studies identified several novel predictors of poor pregnancy outcomes, including uterine Doppler and laboratory findings. A prospective study found great success in transitioning to azathioprine from mycophenolate mofetil prior to pregnancy in patients with quiet lupus nephritis. Two retrospective analyses suggest that hydroxychloroquine may prevent congenital heart block in pregnancies exposed to SSA/Ro antibodies. Finally, the initial pregnancy data for belimumab suggest a high degree of transplacental transfer, but thus far no definitive link between belimumab and congenital abnormalities. Recent studies suggest both novel markers of poor pregnancy outcomes and new approaches to the management of lupus during pregnancy.

  12. Sjögren’s syndrome associated with systemic lupus erythematosus

    Science.gov (United States)

    Taşdemir, Mehmet; Hasan, Chiar; Ağbaş, Ayşe; Kasapçopur, Özgür; Canpolat, Nur; Sever, Lale; Çalışkan, Salim

    2016-01-01

    Systemic lupus erythematosus and Sjögren’s syndrome are chronic auto- inflammatory disorders which can lead to serious organ damage. Although systemic lupus erythematosus and Sjögren’s syndrome were previously considered two forms of the same disease because of presence of clinical coexistence of these two conditions, the view that they are two different conditions with mutual characteristics has become prominent in recent years. In this paper, we reported a 16 year-old girl who was followed up with a diagnosis of Sjögren’s syndrome for six years and then was observed to have overlap of systemic lupus erythematosus. In the baseline, she did not have any clinical or serological evidence for systemic lupus erythematosus. After six year, massive proteinuria and serological findings developed and systemic lupus erythematosus nephritis was diagnosed by kidney biopsy. Currently, systemic lupus erythematosus and Sjögren’s syndrome cannot be differentiated definetely. We need more valuable diagnostic and classification criteria to differentiate these two important conditions. PMID:27738403

  13. Survival in systemic lupus erythematosus, 1995-2010

    DEFF Research Database (Denmark)

    Voss, A; Laustrup, H; Hjelmborg, J

    2013-01-01

    ObjectiveThe objective of this paper is to investigate survival and causes of death in a Danish lupus population.MethodsTwo hundred and fifteen SLE patients (94% Caucasians) were followed prospectively for up to 16 years. Thirty-eight patients died. Survival rate and causes of death were analysed......%) and malignancies (13%). Deaths due to infections and active SLE were rare and predominated within the first seven years after diagnosis and before age 40, while cardiovascular deaths prevailed after 20 years' follow-up.ConclusionThis study shows that despite progress in lupus management, including direct access...... to specialized hospital care and increased use of hydroxychloroquine, mortality in lupus patients is still increased. Main causes of death were active disease and infections among the young and newly diagnosed, while cardiovascular deaths prevailed in longstanding disease....

  14. Quality of care in systemic lupus erythematosus: the association between process and outcome measures in the Lupus Outcomes Study.

    Science.gov (United States)

    Yazdany, Jinoos; Trupin, Laura; Schmajuk, Gabriela; Katz, Patricia P; Yelin, Edward H

    2014-08-01

    Although process measures to assess quality of care in systemic lupus erythematosus (SLE) are available, their relationship to long-term outcomes has not been studied. Using a prospective, longitudinal cohort study, we examined the associations between high-quality care and two important SLE outcomes, disease activity and damage. Data were derived from the University of California, San Francisco Lupus Outcomes Study. Participants were followed from 2009 through 2013, responding to yearly surveys. Primary outcomes in this study were clinically meaningful increases in disease activity and damage, assessed by the Systemic Lupus Activity Questionnaire (SLAQ) and the Brief Index of Lupus Damage (BILD), respectively. Using multivariable regression, we examined the relationship between high performance on 13 validated quality measures (receipt of ≥85% of quality measures), and disease outcomes, adjusting for disease status, sociodemographic characteristics, healthcare services and follow-up time. The 737 participants were eligible for a mean of five quality measures (SD 2, range 2-12). There were 155 and 162 participants who had clinically meaningful increases in SLAQ and BILD, respectively. In our models, we found no statistically significant relationship between performance on quality measures and changes in SLAQ. However, receiving higher-quality SLE care was significantly protective against increased disease damage (adjusted OR 0.4, 95% CI 0.4 to 0.7), even after adjusting for covariates. In this community-based cohort, we illustrate for the first time a strong link between processes of care, defined by SLE quality measures, and the subsequent accumulation of disease damage, an important outcome. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  15. Systemic lupus erythematosus following HPV immunization or infection?

    Science.gov (United States)

    Soldevilla, H F; Briones, S F R; Navarra, S V

    2012-02-01

    The link between autoimmunity and infectious agents has been strongly suggested by reports of lupus or lupus-like syndromes following immunization. This report describes three patients with either newly diagnosed systemic lupus erythematosus (SLE) or SLE flare, following vaccination for human papilloma virus (HPV). CASE 1: A 17-year-old female completed two doses of HPV vaccine uneventfully. Two months later, she developed arthralgias with pruritic rashes on both lower extremities, later accompanied by livedo reticularis, bipedal edema with proteinuria, anemia, leucopenia, hypocomplementemia and high titers of anti-nuclear antibody (ANA) and anti-double-stranded DNA (anti-dsDNA). Kidney biopsy showed International Society of Nephrology/Renal Pathology Society Class III lupus nephritis. She was started on high dose steroids followed by pulse cyclophosphamide therapy protocol for lupus nephritis, and subsequently went into remission. CASE 2: A 45-year-old housewife, previously managed for 11 years as having rheumatoid arthritis, had been in clinical remission for a year when she received two doses of HPV immunization. Four months later, she developed fever accompanied by arthritis, malar rash, oral ulcers, recurrent ascites with intestinal pseudo-obstruction, and behavioral changes. Cranial MRI showed vasculitic lesions on the frontal and parietal lobes. Laboratory tests showed anemia with leucopenia, hypocomplementemia, proteinuria, ANA positive at 1:320, and antibodies against dsDNA, Ro/SSA, La/SSB and histone. She improved following pulse methylprednisolone with subsequent oral prednisone combined with hydroxychloroquine. CASE 3: A 58-year-old housewife diagnosed with SLE had been in clinical remission for 8 years when she received two doses of HPV immunization. Three months later, she was admitted to emergency because of a 1-week history of fever, malar rash, easy fatigability, cervical lymph nodes, gross hematuria and pallor. Laboratory exams showed severe

  16. Systemic Lupus Erythematosus Associated Gastrointestinal System Vasculopathy in a Patient with Lupus Nephropathy

    Directory of Open Access Journals (Sweden)

    Özkan ULUTAŞ

    2011-09-01

    Full Text Available Gastrointestinal system (GIS manifestations are common in systemic lupus erythematosus (SLE patients. The gastrointestinal vasculopathy of SLE is a unique clinical entity and reviewed infrequently but it should be kept in mind to give the necessary treatment with corticosteroids and other immunosuppressive agents and to avoid non-essential surgical interventions. Here we report a 33-yearold SLE patient that presented with surgical acute abdomen symptoms, was followed-up in a Nephrology Department, and was well managed with high doses of corticosteroids and cyclophosphamide after the diagnosis of GIS vasculopathy.

  17. Systemic lupus erythematosus: strategies to improve pregnancy outcomes

    Science.gov (United States)

    Yamamoto, Yuriko; Aoki, Shigeru

    2016-01-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease with a high prevalence in females of childbearing age. Thus, reproduction in SLE patients is a major concern for clinicians. In the past, SLE patients were advised to defer pregnancy because of poor pregnancy outcomes and fear of SLE flares during pregnancy. Investigations to date show that maternal and fetal risks are higher in females with SLE than in the general population. However, with appropriate management of the disease, sufferers may have a relatively uncomplicated pregnancy course. Factors such as appropriate preconception counseling and medication adjustment, strict disease control prior to pregnancy, intensive surveillance during and after pregnancy by both the obstetrician and rheumatologist, and appropriate interventions when necessary play a key role. This review describes the strategies to improve pregnancy outcomes in SLE patients at different time points in the reproduction cycle (preconception, during pregnancy, and postpartum period) and also details the neonatal concerns. PMID:27468250

  18. Toward new criteria for systemic lupus erythematosus-a standpoint.

    Science.gov (United States)

    Aringer, M; Dörner, T; Leuchten, N; Johnson, S R

    2016-07-01

    While clearly different in their aims and means, classification and diagnosis both try to accurately label the disease patients are suffering from. For systemic lupus erythematosus (SLE), this is complicated by the multi-organ nature of the disease and by our incomplete understanding of its pathophysiology. Hallmarks of SLE are the presence of antinuclear antibodies (ANA), and multiple immune-mediated organ symptoms that are largely independent. In an attempt to overcome limitations of the current sets of SLE classification criteria, a new four-phase approach is being developed, which is jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). This review attempts to delineate the performance of the current sets of criteria, the reasons for the decision for classification, and not diagnostic, criteria, and to provide a background of the current approach taken.

  19. Successful treatment of systemic lupus erythematosus with subcutaneous immunoglobulin.

    Science.gov (United States)

    Brasileiro, A; Fonseca Oliveira, J; Pinheiro, S; Paiva-Lopes, M J

    2016-05-01

    The therapeutic efficacy of high-dose intravenous immunoglobulin in systemic lupus erythematosus (SLE) patients is well established. However, side effects might limit its use and lead to the consideration of therapeutic alternatives, such as the subcutaneous formulation of immunoglobulin, which has been used in some patients with other autoimmune diseases. We report a case of SLE refractory to classical therapies. High-dose intravenous immunoglobulin was effective, but gave rise to significant side effects. The patient was successfully treated with subcutaneous human immunoglobulin, achieving and maintaining clinical and laboratory remission. A lower immunoglobulin dose was needed and no side effects were observed, compared to the intravenous administration. Subcutaneous immunoglobulin could be a better-tolerated and cost-saving therapeutic option for select SLE patients.

  20. Systemic lupus erythematosus, pregnancy and carcinoma of the tongue.

    Science.gov (United States)

    Unsworth, Jeffrey David; Baldwin, Andrew; Byrd, Louise

    2013-05-31

    We present a case which describes a 29-year-old woman with systemic lupus erythematosus who was treated aggressively with cytotoxic immunosuppression. Five years later and approximately 12 weeks pregnant, she is confirmed as having carcinoma of the tongue. Not wishing to consider termination of her pregnancy, she underwent surgical resection, which included partial glossectomy with microvascular reconstruction. Good oral function (speech and swallowing) was restored within 2 weeks. The pregnancy proceeded relatively uneventfully to 37 weeks gestation when proteinuric hypertension necessitated induction of labour. She remains well with no evidence of recurrence. This case highlights the options available in the treatment of carcinoma of the tongue during pregnancy together with the ethical considerations required, balanced against optimising maternal outcomes.

  1. Cytokine profile in two siblings with neonatal lupus erythematosus.

    Science.gov (United States)

    Shimozawa, Hironori; Kono, Yumi; Matano, Miyuki; Suzuki, Yume; Koike, Yasunori; Yada, Yukari; Yamagata, Takanori; Takahashi, Naoto

    2015-12-01

    We studied the cytokine profile of two siblings with neonatal lupus erythematosus (NLE) born to a mother positive for serum anti-Ro and -La antibodies, who did not receive any medication during the two pregnancies. The first sibling was found to have complete atrioventricular block in utero and became severely ill after birth. He fulfilled the diagnostic criteria for hemophagocytic lymphohistiocytosis on day 2. The second sibling did not have any fetal symptoms. He was generally stable after birth, but with typical skin rash. Laboratory data suggested that they both had hypercytokinemia during the neonatal period, requiring corticosteroid treatment. Interleukin (IL)-6, interferon-γ, IL-8 and monocyte chemotactic protein-1 were elevated in both cases, while IL-12, IL-13 and IL-17 were elevated only in the second sibling. Comparison of the cytokine profiles suggests the potential roles of different cytokines in the onset and clinical manifestations of NLE.

  2. Contraception in patients with systemic lupus erythematosus and antiphospholipid syndrome.

    Science.gov (United States)

    Sammaritano, L R

    2014-10-01

    Contraceptive choice in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) is challenging but important. Long-acting forms of contraception such as the progesterone intrauterine device (IUD) or subdermal implant are preferable for most patients. Estrogen-containing hormonal contraceptives may be used in stable, inactive SLE patients but are contraindicated in patients with positive antiphospholipid antibodies (aPL). The levonorgestrel IUD is a good alternative for many APS patients and often decreases menstrual blood loss. It is prudent to avoid depot medroxyprogesterone acetate (DMPA) in corticosteroid-treated or other patients at risk for osteoporosis because of the inhibition of ovulation. Effective and safe contraception in patients with SLE and APS permits planning for pregnancy during inactive disease and while on pregnancy-compatible medications, preventing a poorly timed pregnancy that may jeopardize maternal and/or fetal health.

  3. Systemic lupus erythematosus: strategies to improve pregnancy outcomes.

    Science.gov (United States)

    Yamamoto, Yuriko; Aoki, Shigeru

    2016-01-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease with a high prevalence in females of childbearing age. Thus, reproduction in SLE patients is a major concern for clinicians. In the past, SLE patients were advised to defer pregnancy because of poor pregnancy outcomes and fear of SLE flares during pregnancy. Investigations to date show that maternal and fetal risks are higher in females with SLE than in the general population. However, with appropriate management of the disease, sufferers may have a relatively uncomplicated pregnancy course. Factors such as appropriate preconception counseling and medication adjustment, strict disease control prior to pregnancy, intensive surveillance during and after pregnancy by both the obstetrician and rheumatologist, and appropriate interventions when necessary play a key role. This review describes the strategies to improve pregnancy outcomes in SLE patients at different time points in the reproduction cycle (preconception, during pregnancy, and postpartum period) and also details the neonatal concerns.

  4. Regulatory T cells in systemic lupus erythematosus and pregnancy.

    Science.gov (United States)

    Tower, Clare; Mathen, Stephy; Crocker, Ian; Bruce, Ian N

    2013-06-01

    Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disorder that predominantly affects women of reproductive age. As clinical outcomes improve, pregnancy in these women is becoming more common. Although epidemiological data have documented an improvement in the prognosis of pregnancy in these women over recent years, they are still at significantly increased risk of pregnancy complications, such as miscarriage, stillbirth, pre-eclampsia and impaired foetal growth. The pathogenesis of SLE involves marked immune dysfunction, and in particular, the function of immunosuppressive elements of the immune system is impaired, including regulatory T-cell function. Because regulatory T cells are likely to be the key cell-modulating feto-maternal tolerance, this review overviews the possibility that regulatory T-cell impairments contribute to pregnancy pathology in women with SLE and contribute to the clinical challenge of managing these women during pregnancy.

  5. Vitamin D and systemic lupus erythematosus: an update.

    Science.gov (United States)

    Mok, Chi Chiu

    2013-05-01

    Vitamin D is a steroid hormone that, in addition to its actions on calcium and bone metabolism, exhibits a plethora of regulatory effects on growth, proliferation, apoptosis and function of the cells of the immune system that are relevant to the pathophysiology of systemic lupus erythematosus (SLE). Hypovitaminosis D is highly prevalent in SLE as a result of avoidance of sunshine, photoprotection, renal insufficiency and the use of medications such as glucocorticoids, anticonvulsants, antimalarials and the calcineurin inhibitors, which alter the metabolism of vitamin D or downregulate the functions of the vitamin D receptor. Low levels of vitamin D correlate with disease activity, and is associated with osteoporosis, fatigue and certain cardiovascular risk factors in SLE patients. This review updates the recent evidence on the relationship between vitamin D status and the onset, activity and complications of SLE, and summarizes the recommendations for vitamin D supplementation.

  6. A Unique Case of Systemic Lupus Erythematosus Pelvic Vasculitis

    Directory of Open Access Journals (Sweden)

    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.

  7. The in vitro photosensitivity of systemic lupus erythematosus skin fibroblasts.

    Science.gov (United States)

    Zamansky, G B; Minka, D F; Deal, C L; Hendricks, K

    1985-03-01

    To investigate the role of DNA damage in the pathogenesis of systemic lupus erythematosus (SLE), we studied the ability of skin fibroblasts derived from SLE patients to recover from ultraviolet (UV) light radiation of varying wavelengths. Four of five SLE cell strains were more sensitive to UV-C (254 nm), sun lamp, and UV-A (320 to 400 nm) light than were normal cells. SLE cellular recovery was most sensitive to broad spectrum, long wavelength light. This hypersensitivity did not appear to result from the UV light activation of a clastogenic factor. Experiments which examined the DNA repair capacity of irradiated cells indicated that SLE fibroblasts may be able to excise certain DNA lesions as well as normal cells. The mechanisms responsible for the hypersensitivity of SLE cells remain under investigation.

  8. The incidence of systemic lupus erythematosus in North American Indians.

    Science.gov (United States)

    Morton, R O; Gershwin, M E; Brady, C; Steinberg, A D

    1976-06-01

    The annual incidence (AI) of systemic lupus erythematosus (SLE) was determined in 75 highly inbred North American Indian tribes, a total of approximately 800,000 people, during the fiscal years 1971-1975. Seventy-two of the Indian tribes had an AI of SLE which was of similar magnitude to previously published studies from Sweden, Rochester (Minn.), Alabama, New York City, and San Francisco. However, Three tribes, the Crow, Arapahoe, and Sioux Indians, had a markedly elevated AI of SLE. These three tribes share common historical, geographic, and cultural characteristics. Further, they all reside in the northern half of the United States, in states that do not receive intense sun exposure, thereby eliminating photosensitivity as a major determinant of this increased prevalence. Finally, the AI of SLE in the Sioux Indians was highest for "full-blooded" members and lowest for genetic admixtures.

  9. Neurocognitive impairment in children and adolescents with systemic lupus erythematosus.

    Science.gov (United States)

    Levy, Deborah M; Ardoin, Stacy P; Schanberg, Laura E

    2009-02-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, in which neuropsychiatric manifestations are a common cause of significant morbidity. The American College of Rheumatology has identified 19 distinct neuropsychiatric syndromes associated with SLE, although the 1982 American College of Rheumatology classification criteria for SLE recognize only two: seizures and psychosis. Neurocognitive impairment (NCI) is one of the most common and clinically challenging manifestations of SLE, but its pathophysiology remains poorly understood. This Review examines the epidemiology and pathophysiology of NCI in children and adolescents with SLE, as well as the diagnostic and therapeutic approaches that are available for these patients. As few published studies specifically address NCI in pediatric SLE, new directions for research are also discussed.

  10.  Biological therapies in systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Dorota Suszek

    2012-08-01

    Full Text Available  The prevention of chronic organic damage and complete inhibition of inflammatory activity of the disease are the main goals in the treatment of systemic lupus erythematosus (SLE. Current therapies of SLE are not effective enough and they may cause various serious side effects. Biological therapies, affecting important pathogenetic disturbances in the immunological system of SLE patients, give hope for the development of a new treatment for SLE. Currently the most advanced clinical trials are being conducted with anti-lymphocyte B drugs, such as rituximab, belimumab and epratuzumab. Belimumab as the first biological agent was registered for treatment of the active, seropositive form of SLE. The advances in immunology and rheumatology nowadays raise the hope of finding effective and safe treatment for SLE. In our article we present an overview of data concerning perspectives of biological treatment in SLE.

  11. Dexamethasone-cyclophosphamide pulse therapy in systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Dhabhai Ravindra

    2005-01-01

    Full Text Available BACKGROUND AND AIMS: Therapy systemic lupus erythematosus (SLE has been generally discouraging. Methyl-prednisolone pulse therapy has been used for various connective tissue disorders. We used intravenous dexamethasone cyclophosphamide pulse therapy to treat SLE. METHODS: Fourteen patients (10 females and 4 males between the age of 15-48 years with definite or classical clinical criteria laid by American Rheumatism Association criteria were treated by Dexamethasone-Cyclophosphamide pulse (DCP therapy at our center. RESULTS: It was possible to induce a complete clinical remission with DCP therapy in most of the patients thereby offering them life free from disease and drugs. The side effects commonly observed with conventional daily dose regimen of corticosteroids were not present or were mild. CONCLUSIONS: Almost all patients had good response after 3-4 pulses to allow them a normal life style. Fever, malar rash and oral ulceration responded early but photosensitivity, discoid rash, alopecia and joint pains took some more time.

  12. Tuberculosis in patients with systemic lupus erythematosus: Spain's situation.

    Science.gov (United States)

    Arenas Miras, María del Mar; Hidalgo Tenorio, Carmen; Jimenez Alonso, Juan

    2013-01-01

    There has recently been an increase in the incidence of patients with systemic lupus erythematosus (SLE) due mainly to earlier diagnosis, and increased survival. Tuberculosis in our country is one of the most prevalent infectious diseases, and one of the underlying causes would be HIV infection and increased immigration from areas with high tuberculosis prevalence; this phenomenon is truly important in patients with autoimmune diseases, as clinical presentation, severity and prognosis of tuberculosis are often different to that of immunocompetent patients. Studies of tuberculosis in patients with SLE are scarce and inconclusive, with many doubts existing about the performance or non-tuberculous prophylaxis in this population and the absence of a protocol due to lack of conclusive studies. New techniques for diagnosis of tuberculosis (IGRAs) may be useful in this population due to higher sensitivity than Mantoux, helping avoid false negatives. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  13. Systemic Lupus Erythematosus with Hepatosplenic Granuloma: A Rare Case

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    Anju Bharti

    2014-01-01

    Full Text Available Background. Systemic lupus erythematosus (SLE is an autoimmune disease which is known to present with a wide variety of clinical manifestations. Case Report. A 15-year-old male presented with complaints of moderate grade fever and generalized body swelling. There was no history of cough, weight loss, joint pain, oral ulcerations, skin rash, photosensitivity, loss of hair, pain abdomen, jaundice, or any significant illness in the past. Contrast enhanced computerized tomography of the abdomen revealed hypodense lesions in both liver and spleen (without contrast enhancement, suggestive of granulomas along with few retroperitoneal and mesenteric lymph nodes. On the basis of immunological tests and renal biopsy report, SLE with hepatosplenic granulomatosis diagnosis was made. He was given pulse methylprednisolone 500 mg, for 3 days and he showed dramatic improvement clinically. Conclusion. Hepatic and splenic granulomas are not common in SLE, but this should be kept in differential diagnosis.

  14. The need to define treatment goals for systemic lupus erythematosus.

    Science.gov (United States)

    Franklyn, Kate; Hoi, Alberta; Nikpour, Mandana; Morand, Eric F

    2014-09-01

    In the current therapeutic climate, mortality rates from systemic lupus erythematosus (SLE) remain unacceptably high. Although new therapies are on the horizon, pending their emergence and availability, optimization of the currently available therapies is potentially achievable. A 'treat-to-target' approach is now considered routine for many diseases, including rheumatoid arthritis, for which it has substantially improved patient outcomes. The heterogeneity of SLE, as well as lack of universal agreement over methods to measure disease activity and treatment responses, has impeded the development of such an approach for this disease. In this article, the potential benefits of a treatment-target definition are explored, obstacles to the development of a treatment target in SLE are identified, and possible strategies to achieve this goal are discussed.

  15. Systemic lupus erythematosus following total body irradiation for malignant lymphoma.

    Science.gov (United States)

    Spinozzi, F; Capodicasa, E; Gerli, R; Bertotto, A; Rambotti, P; Grignani, F

    1986-01-01

    A case of a 63-year old man, who developed systemic lupus erythematosus three years after an initial diagnosis of small-cleaved centrofollicular lymphoma is described. The diagnosis of SLE was made on the basis of the accepted "1982 revised criteria for the classification of SLE". The autoimmune disease arose after a cycle of total body irradiation, despite the treatment with combination chemotherapeutic doses such a CVP or COAP or Cyclophosphamide, Vincristine, VM-26 and Prednisone. Genetic, immunological and exogenous environmental factors may co-exist and might equally be implicated in the pathogenesis of SLE and malignant lymphoma. However, the onset of SLE after total body irradiation could have been caused by the inactivation of suppressor T lymphocytes, which are known to be sensitive to radiations in vitro.

  16. Relapsed hydroxychloroquine induced thrombocytopenia in a systemic lupus erythematosus patient.

    Science.gov (United States)

    Antón Vázquez, Vanesa; Pascual, Luis; Corominas, Héctor; Giménez Torrecilla, Isabel

    Hydroxychloroquine is used in the long-term therapy of systemic lupus erythematosus (SLE). Although considered to be a safe treatment, side effects have been documented. An uncommon side effect is thrombocytopenia. In order to establish the diagnosis of thrombocytopenia secondary to Hydroxychloroquine, non-pharmacological causes must be ruled out and it is necessary to determine a recurrence after re-exposure to the drug. We present one case of severe thrombocytopenia occurring in a patient with SLE undergoing treatment with Hydroxychloroquine. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  17. Distinct proteome pathology of circulating microparticles in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Østergaard, Ole; Nielsen, Christoffer Tandrup; Tanassi, Julia Tanas

    2017-01-01

    BACKGROUND: The pathogenesis of systemic lupus erythematosus (SLE) is poorly understood but has been linked to defective clearance of subcellular particulate material from the circulation. This study investigates the origin, formation, and specificity of circulating microparticles (MPs) in patients...... with SLE based on comprehensive MP proteome profiling using patients with systemic sclerosis (SSc) and healthy donors (HC) as controls. METHODS: We purified MPs from platelet-poor plasma using differential centrifugation of samples from SLE (n = 45), SSc (n = 38), and two sets of HC (n = 35, n = 25). MP......-MPs (which we propose to call luposomes) are highly specific for SLE, i.e. not found in MP preparations from HC or patients with another autoimmune, systemic disease, SSc. In SLE-MPs platelet proteins and mitochondrial proteins are significantly diminished, cytoskeletal proteins deranged, and glycolytic...

  18. [A case of skin cryptococcosis in systemic lupus erythematosus].

    Science.gov (United States)

    Halweg, H; Korzeniewska-Koseła, M; Podsiadło, B; Krakówka, P

    1990-01-01

    Here is presented a case of woman treated by immunosuppressive preparations because of systemic lupus erythematosus with ski manifestations as tubercles and ulcerations on skin of trunk and extremities. On the basis of histological examination of tubercle skin specimens and mycological examinations of material obtained from skin ulcerations cryptococcosis was diagnosed. Disease was limited to skin that was an entry of infection. Patient was treated by Amphotericin B administered intravenously and Flucitosine per os. Amphotericin B was also applied topically. The results of cultures became gradually negative, up to total disappearance of fungus cells in direct specimens, prepared from examined material. After treatment continuing for 5 months only discoloured scars were observed on sick skin.

  19. The Real Culprit in Systemic Lupus Erythematosus: Abnormal Epigenetic Regulation

    Directory of Open Access Journals (Sweden)

    Haijing Wu

    2015-05-01

    Full Text Available Systemic lupus erythematosus (SLE is an autoimmune disease involving multiple organs and the presence of anti-nuclear antibodies. The pathogenesis of SLE has been intensively studied but remains far from clear. B and T lymphocyte abnormalities, dysregulation of apoptosis, defects in the clearance of apoptotic materials, and various genetic and epigenetic factors are attributed to the development of SLE. The latest research findings point to the association between abnormal epigenetic regulation and SLE, which has attracted considerable interest worldwide. It is the purpose of this review to present and discuss the relationship between aberrant epigenetic regulation and SLE, including DNA methylation, histone modifications and microRNAs in patients with SLE, the possible mechanisms of immune dysfunction caused by epigenetic changes, and to better understand the roles of aberrant epigenetic regulation in the initiation and development of SLE and to provide an insight into the related therapeutic options in SLE.

  20. Basal ganglia calcification on computed tomography in systemic lupus erythematosus

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    Nagaoka, Shohei; Tani, Kenji; Ishigatsubo, Yoshiaki and others

    1988-09-01

    The development of basal ganglia calcification was studied in 85 patients with systemic lupus erythematosus (SLE) by computed tomography (CT). Bilateral calcification of the basal ganglia was found to occur in 5 patients (5.9 %) with SLE, but was not seen in patients with rheumatoid arthritis and progressive systemic sclerosis. All were female with a mean age of 42 years (range 29 - 49). The patients with calcification of the basal ganglia had neurological symptoms, such as psychiatric problems (3 cases), grand mal seizures (1 case), CSF abnormalities (2 cases), and EEG changes (4 cases). There were significantly higher incidences of alopecia, cutaneous vasculitis, leukopenia, and thrombocytopenia in the group with calcifications than those in the group with normal CT findings. Circulating immune complexes were detected and LE tests were positive in 2 patients. Endocrinological examination showed no abnormality in any. We suggest that basal ganglia calcification in SLE might be related to cerebral vasculitis.

  1. White matter correlates of neuropsychological dysfunction in systemic lupus erythematosus.

    Directory of Open Access Journals (Sweden)

    Rex E Jung

    Full Text Available Patients diagnosed with Systemic Lupus Erythematosus have similar levels of neuropsychological dysfunction (i.e., 20-50% as those with Neuropsychiatric Systemic Lupus Erythematosus (NPSLE. We hypothesized a gradient between cognition and white matter integrity, such that strongest brain-behavior relationships would emerge in NPSLE, intermediate in non-NPSLE, and minimal in controls. We studied thirty-one patients (16 non-NPSLE; 15 NPSLE, ranging in age from 18 to 59 years old (100% female, and eighteen age and gender matched healthy controls. DTI examinations were performed on a 1.5T scanner. A broad neuropsychological battery was administered, tapping attention, memory, processing speed, and executive functioning. The Total z-score consisted of the combined sum of all neuropsychological measures. In control subjects, we found no significant FA-Total z-score correlations. NPSLE, non-NPSLE, and control subjects differed significantly in terms of Total z-score (NPSLE = -2.25+/-1.77, non-NPSLE = -1.22+/-1.03, Controls = -0.10+/-.57; F = 13.2, p<.001. In non-NPSLE subjects, FA within the right external capsule was significantly correlated with Total z-score. In NPSLE subjects, the largest FA-Total z-score clusters were observed within the left anterior thalamic radiation and right superior longitudinal fasciculus. In subsequent analyses the largest number of significant voxels linked FA with the Processing Speed z-score in NPSLE. The current results reflect objective white matter correlates of neuropsychological dysfunction in both NPSLE and (to a lesser degree in non-NPSLE. non-NPSLE and NPSLE subjects did not differ significantly in terms of depression, as measured by the GDI; thus, previous hypotheses suggesting moderating effects of depression upon neuropsychological performance do not impact the current FA results.

  2. Posterior reversible encephalopathy syndrome and association with systemic lupus erythematosus.

    Science.gov (United States)

    Ferreira, T S; Reis, F; Appenzeller, S

    2016-10-01

    Posterior reversible encephalopathy syndrome (PRES) is a neurological complex disorder with many clinical associations and causative factors. It is important to recognize this condition because early diagnosis and treatment usually result in its complete resolution, radiological imaging becoming the key for the correct diagnosis. We retrospectively reviewed charts and magnetic resonance imaging findings in the University of Campinas from January 2005 to July 2015, selecting three cases of patients with systemic lupus erythematosus syndrome who developed PRES, for whom risk factors, characteristics, magnetic resonance imaging findings and neurological resolution were analyzed. We also conducted a review of the English-language literature. The three cases had neurological symptoms like acute onset of headache, altered mental status, cortical blindness and seizures. Brain magnetic resonance imaging demonstrated posterior cortical and white matter alterations involving posterior brain territories, which were more conspicuous on T2-weighted and fluid-attenuated inversion recovery. Spectroscopy, diffusion-weighted imaging and susceptibility-weighted imaging were also important for neuroradiological evaluation. Immunosuppressive drugs were taken in all cases. Partial clinical and radiological recovery was observed in two cases, and complete resolution was observed in the third patient. We found 52 cases of PRES in systemic lupus erythematosus patients. Almost all patients were women 94%, ranging from 8 to 62 years old. Posterior brain territory involvements were found in 98% of patients. Hemorrhagic complications involved 26% of patients, becoming a risk factor for clinical sequels. The total percentage of patients with no complete resolution of radiological findings on follow-up images was 27.5%. In patients with autoimmune disorders, endothelial dysfunction may occur secondary to autoimmunity and the use of cytotoxic drugs, supposedly facilitating the occurrence of more

  3. Epidemiological characteristics of patients with cutaneous lupus erythematosus.

    Science.gov (United States)

    Avilés Izquierdo, J A; Cano Martínez, N; Lázaro Ochaita, P

    2014-01-01

    Lupus erythematosus is a chronic autoimmune inflammatory disease with a wide clinical spectrum and variable clinical course. Few studies have analyzed the characteristics of patients with only cutaneous lupus erythematosus (CLE). The aim of this study was to describe the epidemiological, clinical, and immunological characteristics of a series of patients diagnosed with CLE. An analysis was performed of the data from all patients over 18 years of age with a diagnosis of CLE and seen between January 1, 2007 and December 31, 2011 in a tertiary hospital. One hundred- one patients were included in the study. The mean age at diagnosis was 40 years and 84% were women. Subacute forms of presentation were observed in 94% of patients. The chronic discoid forms were localized to the head and neck in 79% of cases, whereas the subacute forms were on the trunk in 97% of cases. Patients with acute forms were positive for antinuclear, anti-DNA, anti-smooth muscle, and anti-RNP (ribonucleoprotein) antibodies, whereas anti-Ro and anti-La antibodies predominated in patients with subacute forms. Seventeen patients presented more than 1 subtype of CLE. Fifteen (88%) of these patients received immunosuppressor treatment versus 44 (52%) of the other 84 patients with only 1 subtype of CLE. Patients with distinct subtypes of CLE present different clinical and immunological characteristics. Oral immunosuppressants are often needed to control the disease in a large proportion of patients with different subtypes of CLE. Copyright © 2013 Elsevier España, S.L. and AEDV. All rights reserved.

  4. Persistent scarring, atrophy, and dyspigmentation in a preteen girl with neonatal lupus erythematosus.

    Science.gov (United States)

    High, Whitney A; Costner, Melissa I

    2003-04-01

    Neonatal lupus erythematosus is an uncommon autoimmune disease with distinctive cutaneous findings. Descriptions of chronic cutaneous sequelae are rare. We describe a 12-year-old girl with persistent dyspigmentation, scarring, and atrophy as a result of neonatal lupus occurring during infancy.

  5. The serum levels of connective tissue growth factor in patients with systemic lupus erythematosus and lupus nephritis.

    Science.gov (United States)

    Wang, F-M; Yu, F; Tan, Y; Liu, G; Zhao, M-H

    2014-06-01

    The expression of connective tissue growth factor mRNA in human kidneys may serve as an early marker for lupus nephritis progression. Therefore, we speculated that connective tissue growth factor may be involved in the pathogenesis of systemic lupus erythematosus and lupus nephritis. In this study, we set out to investigate the associations between serum connective tissue growth factor levels and clinicopathological features of patients with systemic lupus erythematosus and lupus nephritis. Serum samples from patients with non-renal systemic lupus erythematosus, renal biopsy-proven lupus nephritis and healthy control subjects were detected by enzyme-linked immunosorbent assay for serum connective tissue growth factor levels. The associations between connective tissue growth factor levels and clinicopathological features of the patients were further analysed. The levels of serum connective tissue growth factor in patients with non-renal systemic lupus erythematosus and lupus nephritis were both significantly higher than those in the normal control group (34.14 ± 12.17 ng/ml vs. 22.8 ± 3.0 ng/ml, psystemic lupus erythematosus and lupus nephritis group (34.14 ± 12.17 ng/ml vs. 44.1 ± 46.8 ng/ml, p = 0.183). Serum connective tissue growth factor levels were significantly higher in lupus nephritis patients with the following clinical manifestations, including anaemia (51.3 ± 51.4 ng/ml vs. 23.4 ± 9.7 ng/ml, plupus nephritis (63.3 ± 63.4 ng/ml vs. 38.3 ± 37.9 ng/ml, p = 0.035, respectively). Serum connective tissue growth factor levels were negatively associated with estimated glomerular filtration rate (r = -0.46, plupus nephritis (plupus and correlated with chronic renal interstitial injury and doubling of serum creatinine in patients with lupus nephritis. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  6. Abatacept for the treatment of systemic lupus erythematosus.

    Science.gov (United States)

    Pimentel-Quiroz, Victor R; Ugarte-Gil, Manuel F; Alarcón, Graciela S

    2016-01-01

    Due to improvements in our understanding of the pathogenesis of systemic lupus erythematosus (SLE), several target drugs have been and are being developed. One of the possible targets in SLE is co-stimulation between antigen-presenting cells and T cells. Abatacept is a co-stimulation moderator approved for the treatment of several autoimmune diseases. There is an unmet need for drugs with a better efficacy and safety profile when treating patients with SLE. In this review, the authors discuss the mechanism of action of abatacept including its role in the immune system and glomeruli, and relevant information about its clinical efficacy and safety. Possible explanations for the failure of previous randomized clinical trials are also discussed. Abatacept has demonstrated efficacy in other autoimmune diseases, but in SLE, randomized clinical trials have failed to achieve their primary outcome. Despite these disappointing results and based on its mechanism of action, abatacept seems to have a role in lupus nephritis and arthritis. This should be corroborated with new trials which hopefully will overcome the design pitfalls of the ones conducted to date.

  7. Altered B cell receptor signaling in human systemic lupus erythematosus

    Science.gov (United States)

    Jenks, Scott A.; Sanz, Iñaki

    2009-01-01

    Regulation of B cell receptor signaling is essential for the development of specific immunity while retaining tolerance to self. Systemic lupus erythematosus (SLE) is characterized by a loss of B cell tolerance and the production of anti-self antibodies. Accompanying this break down in tolerance are alterations in B cell receptor signal transduction including elevated induced calcium responses and increased protein phosphorylation. Specific pathways that negatively regulate B cell signaling have been shown to be impaired in some SLE patients. These patients have reduced levels of the kinase Lyn in lipid raft microdomains and this reduction is inversely correlated with increased CD45 in lipid rafts. Function and expression of the inhibitory immunoglobulin receptor FcγRIIB is also reduced in Lupus IgM- CD27+ memory cells. Because the relative contribution of different memory and transitional B cell subsets can be abnormal in SLE patients, we believe studies targeted to well defined B cell subsets will be necessary to further our understanding of signaling abnormalities in SLE. Intracellular flow cytometric analysis of signaling is a useful approach to accomplish this goal. PMID:18723129

  8. Entheseal involvement in systemic lupus erythematosus: are we missing something?

    Science.gov (United States)

    Di Matteo, A; Satulu, I; Di Carlo, M; Lato, V; Filippucci, E; Grassi, W

    2017-03-01

    Background Musculoskeletal involvement is extremely common in patients with systemic lupus erythematosus (SLE). Continuing the research initiated in patients with inflammatory arthritis, recent studies have shown the potential role of musculoskeletal ultrasound (MSUS) in the evaluation of clinical and subclinical lupus synovitis. The inflammatory process in SLE is traditionally considered to be localized at synovial tissue areas while enthesis is not included among the possible targets of the disease. Patients and methods Entheses included in the Glasgow Ultrasound Enthesitis Scoring System were scanned in a cohort of 20 SLE patients serving as disease controls in an MSUS study aimed at assessing enthesitis in patients with psoriatic arthritis. We describe in detail four cases with unexpected and unequivocal expressions of MSUS enthesitis according to the OMERACT definition. Three out of four patients had no predisposing factors for enthesopathy. Case no. 2 was treated with a variable-dose prednisone regimen. Results In the four cases MSUS examination revealed relevant grey-scale and power Doppler abnormalities at the entheseal level, most commonly at the distal insertion of the patellar tendon. Signs of clinical enthesitis were detected in only one patient. Conclusions This case series shows for the first time the presence of clearly evident MSUS findings indicative of enthesitis in four out of 20 SLE patients (20%), raising the hypothesis that enthesis could be a missing target in the clinical evaluation of SLE patients. Our case series justifies further investigations for a better evaluation of the prevalence, characteristics and clinical relevance of entheseal involvement in SLE.

  9. Systemic lupus erythematosus presenting as cryptogenic organizing pneumonia: case report

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    Jedd A

    2015-02-01

    Full Text Available No abstract available. Article truncated after 150 words. Introduction: Systemic Lupus Erythematosus (SLE is a systemic disease with multiorgan involvement. In the respiratory system, SLE can involve the lung parenchyma and pleura with intrathoracic manifestations of pleuritis, alveolar hemorrhage and pulmonary fibrosis. Cryptogenic organizing pneumonia (COP is a rare complication of SLE. We describe a case of newly diagnosed lupus presenting as COP. Case Report: An 18-year-old woman with no significant past medical history presented to the Emergency Department complaining of generalized malaise, cough and fever for 4 days. Her cough was productive with white to brownish sputum. She complained of chest heaviness/pressure with inability to take deep breaths. Her only reported sick contact was her mother who had the “flu” one week prior to the onset of her illness. She denied any illicit drug use, alcohol or smoking, as well as any recent travel or exotic pet exposure. On admission, her vital signs were: temperature 38.4°C, pulse ...

  10. Imaging Assessment of Cardiovascular Disease in Systemic Lupus Erythematosus

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    Sara C. Croca

    2012-01-01

    Full Text Available Systemic lupus erythematosus is a multisystem, autoimmune disease known to be one of the strongest risk factors for atherosclerosis. Patients with SLE have an excess cardiovascular risk compared with the general population, leading to increased cardiovascular morbidity and mortality. Although the precise explanation for this is yet to be established, it seems to be associated with the presence of an accelerated atherosclerotic process, arising from the combination of traditional and lupus-specific risk factors. Moreover, cardiovascular-disease associated mortality in patients with SLE has not improved over time. One of the main reasons for this is the poor performance of standard risk stratification tools on assessing the cardiovascular risk of patients with SLE. Therefore, establishing alternative ways to identify patients at increased risk efficiently is essential. With recent developments in several imaging techniques, the ultimate goal of cardiovascular assessment will shift from assessing symptomatic patients to diagnosing early cardiovascular disease in asymptomatic patients which will hopefully help us to prevent its progression. This review will focus on the current status of the imaging tools available to assess cardiac and vascular function in patients with SLE.

  11. Peripheral Gangrene Complicating Systemic Lupus Erythematosus in a Patient with Spina Bifida: A Case Report.

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    Vijay, S; Imthiaz, V K; Hitesh, S

    2017-03-01

    An adolescent girl, a known case of spina bifida with systemic lupus, presented with bluish discolouration of three toes of the right foot. She had thrombosis of bilateral popliteal arteries. She underwent percutaneous transluminal angioplasty (PTA) of both legs and Chopart amputation of the right foot. Systemic lupus erythematosus (SLE) occurring in a patient with spina bifida has not been previously reported. Weakness, sensory loss, lack of normal ambulation, endarteritis, antiphospholipid antibody syndrome are common contributory factors for peripheral gangrene in patients with spina bifida with systemic lupus erythematosus.

  12. The rate of and risk factors for frequent hospitalization in systemic lupus erythematosus: results from the Korean lupus network registry.

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    Lee, J W; Park, D J; Kang, J H; Choi, S E; Yim, Y R; Kim, J E; Lee, K E; Wen, L; Kim, T J; Park, Y W; Sung, Y K; Lee, S S

    2016-11-01

    Objectives The survival rate of patients with systemic lupus erythematosus has improved in the last few decades, but the rate of hospitalization and health care costs for these patients remain higher than in the general population. Thus, we evaluated the rate of hospitalization and associated risk factors in an inception cohort of Korean patients with lupus. Methods Of the 507 patients with systemic lupus erythematosus enrolled in the KORean lupus NETwork, we investigated an inception cohort consisting of 196 patients with systemic lupus erythematosus presenting within 6 months of diagnosis based on the American College of Rheumatology classification criteria. We evaluated the causes of hospitalization, demographic characteristics, and laboratory and clinical data at the time of systemic lupus erythematosus diagnosis of hospitalized patients and during a follow-up period. We calculated the hospitalization rate as the number of total hospitalizations divided by the disease duration, and defined "frequent hospitalization" as hospitalization more than once per year. Results Of the 196 patients, 117 (59.6%) were admitted to hospital a total of 257 times during the 8-year follow-up period. Moreover, 22 (11.2%) patients were hospitalized frequently. The most common reasons for hospitalization included disease flares, infection, and pregnancy-related morbidity. In the univariate regression analysis, malar rash, arthritis, pericarditis, renal involvement, fever, systemic lupus erythematosus disease activity index > 12, hemoglobin level < 10 mg/dl, albumin level < 3.5 mg/dl, and anti-Sjögren's syndrome A positivity were associated with frequent hospitalization. Finally, multivariate analysis showed that arthritis, pericarditis, and anti-Sjögren's syndrome A antibody positivity at the time of diagnosis were risk factors for frequent hospitalization. Conclusions Our results showed that frequent hospitalization occurred in 11.2% of hospitalized patients and

  13. Pharmacological Management of Childhood-Onset Systemic Lupus Erythematosus.

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    Thorbinson, Colin; Oni, Louise; Smith, Eve; Midgley, Angela; Beresford, Michael W

    2016-06-01

    Systemic lupus erythematosus (SLE) is a rare, severe, multisystem autoimmune disorder. Childhood-onset SLE (cSLE) follows a more aggressive course with greater associated morbidity and mortality than adult-onset SLE. Its aetiology is yet to be fully elucidated. It is recognised to be the archetypal systemic autoimmune disease, arising from a complex interaction between the innate and adaptive immune systems. Its complexity is reflected by the fact that there has been only one new drug licensed for use in SLE in the last 50 years. However, biologic agents that specifically target aspects of the immune system are emerging. Immunosuppression remains the cornerstone of medical management, with glucocorticoids still playing a leading role. Treatment choices are led by disease severity. Immunosuppressants, including azathioprine and methotrexate, are used in mild to moderate manifestations. Mycophenolate mofetil is widely used for lupus nephritis. Cyclophosphamide remains the first-line treatment for patients with severe organ disease. No biologic therapies have yet been approved for cSLE, although they are being used increasingly as part of routine care of patients with severe lupus nephritis or with neurological and/or haematological involvement. Drugs influencing B cell survival, including belimumab and rituximab, are currently undergoing clinical trials in cSLE. Hydroxychloroquine is indicated for disease manifestations of all severities and can be used as monotherapy in mild disease. However, the management of cSLE is hampered by the lack of a robust evidence base. To date, it has been principally guided by best-practice guidelines, retrospective case series and adapted adult protocols. In this pharmacological review, we provide an overview of current practice for the management of cSLE, together with recent advances in new therapies, including biologic agents.

  14. [Treatment of systemic lupus erythematosus: myths, certainties and doubts].

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    Ruiz-Irastorza, Guillermo; Danza, Alvaro; Khamashta, Munther

    2013-12-21

    Systemic lupus erythematosus (SLE) is a complex disease with different clinical forms of presentation, including a wide range of severity and organic involvement. Such circumstance, along with the fact of the uncommon nature of the disease and the absence of clinically representative response criteria, make it difficult to design controlled clinical trials in SLE patients. As a result, observational studies have a special relevance, being a source of valuable information of SLE prognosis and outcome as well as of the efficacy and adverse effects of the different therapies. Herein we update some of the main treatments used in SLE. Steroids may have more risks than benefits if used at high doses. New mechanisms of action have been described, supporting the use of lower doses, possibly with the same efficacy and less adverse effects. Intravenous pulses of cyclophosphamide still have a role in the treatment of proliferative lupus nephritis and other serious SLE manifestations. Mycophenolate mofetil has shown its efficacy both as induction and maintenance therapy of selected cases of lupus nephritis. Biological therapies have emerged as new promising options. Although clinical trials have not confirmed a clear superiority of rituximab in SLE, observational studies have shown good response rates in severe SLE manifestations or refractory forms. Belimumab has recently been added to the therapeutic armamentarium of SLE; although its place in clinical practice is not well-defined, it may be recommended in active patients with no response or good tolerance to standard therapies. Hydroxichloroquine improves survival, decreases the risk of thrombosis and flares and is safe in pregnancy, and should be considered the baseline therapy in most SLE patients.

  15. Lupus Tumidus: underreported variant of lupus erythematosus (a case report and review of the literature

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    Viktoryia Kazlouskaya

    2014-01-01

    Full Text Available Introduction: Lupus erythematosus tumidus (LET is an underreported variant of lupus erythematosus (LE that is characterized by soft urticarial-like elements usually located on the sun exposed skin. LET is featured with high photosensitivity. Lesions of LET resolve without scarring and do not cause disfigurement as seen in the discoid LE variants. Lesions of LET may co-exist with other variants of LE: discoid or systemic. Main observations: The case presents a female patient with LET localized on the lateral infraorbital areas of her face and cheeks. Histopathological evaluation showed a lymphocytic infiltrate in the middle and deeper parts of the dermis. This article also presents a contemporary review of the clinical variants of LET, histopathological features and approaches to the treatment of LET. Conclusions: LET should be considered in urticarial-like lesions on the skin of the face and other skin exposed areas. Histopathological examination is needed to make a definitive diagnosis of the condition and helps in cases when the clinical presentation is subtle or non-specific.

  16. [A case of systemic lupus erythematosus presenting with jaundice and lupus pneumonia].

    Science.gov (United States)

    Kawai, Takako; Tominaga, Sizuo; Okouchi, Akiko; Kudo, Makoto; Katoh, Kiyoshi; Shoda, Masataka; Fujino, Masayuki A

    2005-02-01

    A 27-year-old Japanese woman was referred to our hospital for acute hepatitis in April 2002. She had been suffering from low grade fever and fatigue for a week. She also presented with dyspnea. On admission, ALT and AST were 857 U/l and 473 U/l respectively. Urine protein was 2 g/day. Chest radiograph showed bilateral infiltrative shadow and pleural effusion. She developed jaundice and her level of total bilirubin was increased to 9.6 mg/dl on May 9. Antibodies to hepatitis viruses were not detected. Testing for antimitochondrial antibodies, antismooth muscle antibodies, and antiribosomal P antibodies showed all negative. However, antinuclear antibodies were positive at titer 1:160 and anti-double stranded DNA antibodies were 130 U/ml. A diagnosis of systemic lupus erythematosus was made and oral administration of 60 mg/day prednisolon was started on May 10. Serum levels of ALT, AST and bilirubin were reduced to within normal range and pulmonary lesions were also improved. We conclude that this is a rare case of systemic lupus erythematosus presenting with acute hepatitis and jaundice.

  17. Autoantibodies as Biomarkers for the Prediction of Neuropsychiatric Events in Systemic Lupus Erythematosus

    Science.gov (United States)

    Hanly, J G; Urowitz, M B; Su, L; Bae, S-C; Gordon, C; Sanchez-Guerrero, J; Clarke, A; Bernatsky, S; Vasudevan, A; Isenberg, D; Rahman, A; Wallace, D J; Fortin, P R; Gladman, D; Dooley, M A; Bruce, I; Steinsson, K; Khamashta, M; Manzi, S; Ramsey-Goldman, R; Sturfelt, G; Nived, O; van Vollenhoven, R; Ramos-Casals, M; Aranow, C; Mackay, M; Kalunian, K; Alarcón, G S; Fessler, B J; Ruiz-Irastorza, G; Petri, M; Lim, S; Kamen, D; Peschken, C; Farewell, V; Thompson, K; Theriault, C; Merrill, J T

    2015-01-01

    Objective Neuropsychiatric (NP) events occur unpredictably in systemic lupus erythematosus (SLE) and most biomarker associations remain to be prospectively validated. We examined a disease inception cohort of 1047 SLE patients to determine which autoantibodies at enrollment predicted subsequent NP events. Methods Patients with recent SLE diagnosis were assessed prospectively for up to 10 years for NP events using ACR case definitions. Decision rules of graded stringency determined whether NP events were attributable to SLE. Associations between the first NP event and baseline autoantibodies (lupus anticoagulant, anticardiolipin, anti-β2 glycoprotein-I, anti-ribosomal P and anti-NR2 glutamate receptor) were tested by Cox proportional hazards regression. Results Disease duration at enrollment was 5.4±4.2 months, followup was 3.6±2.6 years. Patients were 89.1% female with mean (±SD) age 35.2±13.7 years. 495/1047 (47.3%) developed ≥1 NP event (total 917 events). NP events attributed to SLE were 15.4% (model A) and 28.2% (model B). At enrollment 21.9% of patients had lupus anticoagulant, 13.4% anticardiolipin, 15.1% anti-β2 glycoprotein-I, 9.2% anti-ribosomal P and 13.7% anti-NR2 antibodies. Lupus anticoagulant at baseline was associated with subsequent intracranial thrombosis (total n=22) attributed to SLE (model B) (Hazard ratio, HR 2.54 (95% CI: 1.08–5.94). Anti-ribosomal P antibody was associated with subsequent psychosis (total n=14) attributed to SLE (model B) (HR: 3.92 (95% CI:1.23–12.5); p=0.02). Other autoantibodies did not predict NP events. Conclusion In a prospective study of 1047 recently diagnosed SLE patients, lupus anticoagulant and anti-ribosomal P antibodies are associated with an increased future risk for intracranial thrombosis and lupus psychosis respectively PMID:21893582

  18. Lupus eritematoso sistêmico associado a miastenia gravis: relato de caso Systemic lupus erythematosus and myasthenia gravis: case report

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    MARCIO F. DE CARVALHO

    1998-03-01

    Full Text Available Os autores descrevem o caso de uma mulher branca de 24 anos de idade admitida com lupus eritematoso sistêmico (com 4 anos de evolução de doença e início recente de miastenia gravis. São discutidos os principais diagnósticos diferenciais para a fraqueza muscular e a fadiga apresentadas por esta paciente. Uma revisão de literatura abordando a associação de miastenia gravis e lupus eritematoso é feita, com ênfase às características clínicas desses pacientes e ao papel do timoma e timectomia no desenvolvimento de lupus eritematoso em pacientes previamente miastênicos.We report the case of a 24-year-old white woman admitted with a four year diagnosis of systemic lupus erythematosus and the recent onset of myasthenia gravis discussing the main differential diagnosis of weakness and fatigue in this patient. A review of literature approaching the association of myasthenia gravis and systemic lupus erythematosus is also done with emphasis on the clinical characteristics of these patients and the role of thymoma and thymectomy in the development of systemic lupus erythematosus in myasthenic patients.

  19. Correlation between the Modified Systemic Lupus Erythematosus Disease Activity Index 2000 and the European Consensus Lupus Activity Measurement in juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Sato, J O; Corrente, J E; Saad-Magalhães, C

    2016-11-01

    Objective The objective of this study was to assess Modified Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and European Consensus Lupus Activity Measurement (ECLAM) disease activity correlation in addition to their respective correlation to Pediatric Systemic Lupus International Collaborative Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (Ped-SDI), in juvenile systemic lupus erythematosus (JSLE). Methods The activity indices were scored retrospectively and summarized by adjusted means during follow-up. The Ped-SDI was scored during the last visit for those with more than six months follow-up. Pearson correlation between the Modified SLEDAI-2K and ECLAM, as well as Spearman correlations between the Modified SLEDAI-2K, ECLAM, and Ped-SDI were calculated. The receiver operating characteristic (ROC) curve was calculated for both activity indices discriminating damage measured by Ped-SDI. Results Thirty-seven patients with mean age at diagnosis 11 ± 2.9 years and mean follow-up time 3.2 ± 2.4 years were studied. The Modified SLEDAI-2K and ECLAM adjusted means were highly correlated ( r = 0.78, p  0.7, p < 0.001), but Modified SLEDAI-2K and ECLAM correlation with Ped-SDI was only moderate. ROC analysis discriminant performance for both activity indices resulted in area under curve (AUC) of 0.74 and 0.73 for Modified SLEDAI-2K and ECLAM, respectively. Conclusion The high correlation found between the Modified SLEDAI-2K and ECLAM adjusted means indicated that both tools can be equally useful for longitudinal estimates of JSLE activity.

  20. HLA-G genotype and HLA-G expression in systemic lupus erythematosus: HLA-G as a putative susceptibility gene in systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Rizzo, R; Hviid, T V F; Govoni, M

    2008-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease mainly mediated by the deposit of immune complexes and defects in T lymphocytes and antigen-presenting cells along with a high production of T-helper 2 cytokines. A tolerance-inducible function of nonclassical class Ib human leukocyte...

  1. Organ damage accrual and distribution in systemic lupus erythematosus patients followed-up for more than 10 years.

    Science.gov (United States)

    Taraborelli, M; Cavazzana, I; Martinazzi, N; Lazzaroni, M Grazia; Fredi, M; Andreoli, L; Franceschini, F; Tincani, A

    2017-10-01

    Objective The aim of this study was to determine the prevalence, predictors and progression of organ damage in a monocentric cohort of systemic lupus erythematosus patients with a long follow-up. Organ damage was assessed by the Systemic Lupus International Collaborating Clinics Damage Index one year after diagnosis and every five years. Disease activity was measured by the systemic lupus erythematosus disease activity index (SLEDAI)-2K at the beginning of the follow-up. Univariate and multivariable analyses were used to detect items associated with damage. A total of 511 systemic lupus erythematosus patients (92% females, 95% Caucasian), prospectively followed from 1972 to 2014, were included. Results After a mean disease duration of 16 years (SD: 9.5) and a mean follow-up of 12.9 years (SD: 8.8), 354 patients (69.3%) had accrued some damage: 49.7% developed mild/moderate damage, while 19.5% showed severe damage. Damage was evident in 40% of 511 patients one year after diagnosis, and its prevalence linearly increased over time. Longer disease duration, higher SLEDAI, severe Raynaud's, chronic alopecia and cerebral ischaemia were significantly associated with organ damage. No associations between damage and autoantibodies, including anti-dsDNA, anti-Sm or antiphospholipid antibodies, were observed. Anyway, antiphospholipid syndrome and anticardiolipin antibodies predicted the development of neuropsychiatric damage. The ocular, musculoskeletal and neuropsychiatric systems were the most frequently damaged organs, with a linear increase during follow-up. Conclusion A high rate of moderate and severe damage has been detected early in a wide cohort of young lupus patients, with a linear trend of increase over time. Disease activity and long duration of disease predict damage, while antiphospholipid antibodies play a role in determining neuropsychiatric damage.

  2. The lupus impact tracker is responsive to changes in clinical activity measured by the systemic lupus erythematosus responder index.

    Science.gov (United States)

    Devilliers, H; Bonithon-Kopp, C; Jolly, M

    2017-04-01

    Objective The lupus impact tracker (LIT) is a 10-item patient reported outcome tool to measure the impact of systemic lupus erythematosus or its treatment on patients' daily lives. Herein, we describe the responsiveness of the LIT and LupusQoL to changes in disease activity, using the systemic lupus erythematosus responder index (SRI). Methods A total of 325 adult systemic lupus erythematosus patients were enrolled in an observational, longitudinal, multicentre study, conducted across the USA and Canada. Data (demographics, LIT, LupusQoL, BILAG, SELENA-SLEDAI) were obtained three months apart. Modified SRI was defined as: a decrease in SELENA-SLEDAI (4 points); no new BILAG A, and no greater than one new BILAG B; and no increase in the physician global assessment. Standardised response mean and effect size for LIT and LupusQoL domains were calculated among SRI responders and non-responders. Wilcoxon's test was used to compare the LIT and LupusQoL variation by SRI responder status. Results Of the participants 90% were women, 53% were white, 33% were of African descendant and 17% were Hispanic. Mean (SD) age and SELENA-SLEDAI at baseline were 42.3 (16.2) years and 4.3 (3.8), respectively. Mean (SD) LIT score at baseline was 39.4 (22.9). LIT standardised response mean (effect size) among SRI responders and non-responders were -0.69 (-0.36) and -0.20 (-0.12), respectively ( P = 0.02). For LupusQoL, two domains were responsive to SRI: standardised response mean (effect size) for physical health and pain domains were 0.42 (0.23) and 0.65 (0.44), respectively. Conclusions LIT is moderately responsive to SRI in patients with systemic lupus erythematosus. Inclusion of this tool in clinical care and clinical trials may provide further insights into its responsiveness. This is the first systemic lupus erythematosus patient reported outcome tool to be evaluated against composite responder index (SRI) used in clinical trials.

  3. Infections Increase Risk of Arterial and Venous Thromboses in Danish Patients with Systemic Lupus Erythematosus

    DEFF Research Database (Denmark)

    Baronaite Hansen, Renata; Jacobsen, Søren

    2014-01-01

    OBJECTIVE: Infections and thromboses are known complications of systemic lupus erythematosus (SLE). We investigated if infectious episodes in patients with SLE were followed by an increased risk of thrombotic events. METHODS: A cohort of 571 patients with prevalent or incident SLE was followed...... for a mean of 8.9 ± 7.6 years. All episodes of hospitalized infections or episodes of cutaneous herpes zoster as well as arterial and venous thrombotic events were identified by retrospective chart review and prospective updating of a clinical database. For time-dependent analyses adjusted for age, sex...... was increased [relative rate (RR) 2.5, 95% CI 1.4-4.6, and RR 2.8, 95% CI 1.3-5.9, respectively]. Venous thromboses were in particular more prevalent after respiratory infections (RR 5.4, 95% CI 2.3-13). CONCLUSION: The temporal associations observed in this study indicate that infections could be risk factors...

  4. Epstein-Barr virus early antigen diffuse (EBV-EA/D)-directed immunoglobulin A antibodies in systemic lupus erythematosus patients

    DEFF Research Database (Denmark)

    Draborg, A H; Jørgensen, J M; Müller, H

    2012-01-01

    We sought to determine whether the serological response towards lytic cycle antigens of Epstein-Barr virus (EBV) is altered in systemic lupus erythematosus (SLE) patients.......We sought to determine whether the serological response towards lytic cycle antigens of Epstein-Barr virus (EBV) is altered in systemic lupus erythematosus (SLE) patients....

  5. Early discoid lupus erythematosus protects against renal disease in patients with systemic lupus erythematosus: longitudinal data from a large Latin American cohort.

    Science.gov (United States)

    Pons-Estel, G J; Aspey, L D; Bao, G; Pons-Estel, B A; Wojdyla, D; Saurit, V; Alvarellos, A; Caeiro, F; Haye Salinas, M J; Sato, E I; Soriano, E R; Costallat, L T L; Neira, O; Iglesias-Gamarra, A; Reyes-Llerena, G; Cardiel, M H; Acevedo-Vásquez, E M; Chacón-Díaz, R; Drenkard, C

    2017-01-01

    The objective of this study was to examine whether early discoid lupus erythematosus (DLE) would be a protective factor for further lupus nephritis in patients with systemic lupus erythematosus (SLE). We studied SLE patients from GLADEL, an inception longitudinal cohort from nine Latin American countries. The main predictor was DLE onset, which was defined as physician-documented DLE at SLE diagnosis. The outcome was time from the diagnosis of SLE to new lupus nephritis. Univariate and multivariate survival analyses were conducted to examine the association of DLE onset with time to lupus nephritis. Among 845 GLADEL patients, 204 (24.1%) developed lupus nephritis after SLE diagnosis. Of them, 10 (4.9%) had DLE onset, compared to 83 (12.9%) in the group of 641 patients that remained free of lupus nephritis (hazard ratio 0.39; P = 0.0033). The cumulative proportion of lupus nephritis at 1 and 5 years since SLE diagnosis was 6% and 14%, respectively, in the DLE onset group, compared to 14% and 29% in those without DLE (P = 0.0023). DLE onset was independently associated with a lower risk of lupus nephritis, after controlling for sociodemographic factors and disease severity at diagnosis (hazard ratio 0.38; 95% confidence interval 0.20-0.71). Our data indicate that DLE onset reduces the risk of further lupus nephritis in patients with SLE, independently of other factors such as age, ethnicity, disease activity, and organ damage. These findings have relevant prognosis implications for SLE patients and their clinicians. Further studies are warranted to unravel the biological and environmental pathways associated with the protective role of DLE against renal disease in patients with SLE. © The Author(s) 2016.

  6. Neonatal lupus in triplet pregnancy of a patient with undifferentiated connective tissue disease evolving to systemic lupus erythematosus.

    Science.gov (United States)

    Demaestri, M; Sciascia, S; Kuzenko, A; Bergia, R; Barberis, L; Lanza, M G; Bertero, M T

    2009-04-01

    Pregnancy in patients suffering from undifferentiated connective tissue disease (UCTD) represents a risk situation for both the mother and the child. SSA/SSB autoantibodies can determine neonatal lupus (NL) in the foetus, regardless of the maternal disease. Furthermore, pregnancy increases the risk of flares and evolution to differentiated connective tissue disease (CTD). We report an uncommon case in which these complications occurred in a mother and in her foetuses. A 37-year-old woman affected by UCTD developed systemic lupus erythematosus (SLE) after her triplet pregnancy. The only manifestation of neonatal lupus we observed in the three newborns was SSA positivity associated with asymptomatic transient neutropenia.

  7. Aplastic anemia associated to systemic lupus erythematosus in an AIDS patient: a case report.

    Science.gov (United States)

    de Oliveira, Leonardo Rodrigues; Ferreira, Thaís Camargos; Neves, Fernando de Freitas; Meneses, Antônio Carlos de Oliveira

    2013-01-01

    Aplastic anemia is a bone marrow failure syndrome characterized by peripheral cytopenias and hypocellular bone marrow. Although aplastic anemia is idiopathic in most cases, rheumatic diseases such as systemic lupus erythematosus are recognized as causes of aplastic anemia, with their possible etiological mechanisms being T and B lymphocyte dysfunction and pro-inflammatory cytokines and autoantibody production directed against bone marrow components. In the course of the human immunodeficiency virus infection/acquired immunodeficiency syndrome, the identification of autoantibodies and the occurrence of rheumatic events, in addition to the natural course of systemic lupus erythematosus which is modified by immune changes that are characteristic of human immunodeficiency virus infection/acquired immunodeficiency syndrome, make the diagnosis of systemic lupus erythematosus challenging. This study reports the case of a woman with acquired immunodeficiency syndrome treated with a highly active antiretroviral therapy, who had prolonged cytopenias and hypocellular bone marrow consistent with aplastic anemia. The clinical picture, high autoantibodies titers, and sustained remission of the patient's hematological status through immunosuppression supported the diagnosis of systemic lupus erythematosus-associated aplastic anemia. This is the first report of aplastic anemia concurrent with systemic lupus erythematosus and acquired immunodeficiency syndrome, providing additional evidence that immune dysfunction is a key part of the pathophysiological mechanism of aplastic anemia.

  8. Cytokines in relation to autoantibodies before onset of symptoms for systemic lupus erythematosus.

    Science.gov (United States)

    Eriksson, C; Rantapää-Dahlqvist, S

    2014-06-01

    A number of cytokines and chemokines were analysed and related to autoantibodies in blood samples pre-dating the onset of symptoms of systemic lupus erythematosus. Thirty-five patients with systemic lupus erythematosus (American College of Rheumatology criteria) were identified as having donated blood samples, prior to symptom onset, to the Biobank of northern Sweden. Altogether, 140 age- and sex-matched controls were also identified. The concentrations of interferon-α, interleukin-4, interleukin-9, interleukin-10, interferon inducible protein-10 and monocyte chemotactic protein-1 were analysed using multiplex technology and related to autoantibodies (ANA, ENA, anti-dsDNA and anti-histone antibodies) analysed from the same blood sample. The interferon-γ inducible protein-10 levels were higher in the pre-symptomatic individuals than in controls (p systemic lupus erythematosus. An increased concentration of interferon-γ inducible protein-10 pre-dated the onset of systemic lupus erythematosus and was related to autoantibodies before the onset of disease. The levels of interferon-γ inducible protein-10 and interferon-α were correlated. These findings support the proposal that the interferon system is important early in the pathogenesis of systemic lupus erythematosus and autoantibody formation. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  9. Management of cardiovascular risk in systemic lupus erythematosus: a systematic review.

    Science.gov (United States)

    Andrades, C; Fuego, C; Manrique-Arija, S; Fernández-Nebro, A

    2017-01-01

    Systemic lupus erythematosus is associated with accelerated atherosclerosis and increased risk of cardiovascular complications. The aim of this study was to review the effectiveness of interventions for primary and secondary prevention of cardiovascular events and mortality and to review the effectiveness of interventions for cardiovascular risk factor reduction in systemic lupus erythematosus patients. A systematic review was conducted. Electronic databases Medline and Embase (1961-2015) were searched. Nineteen articles met the inclusion criteria and were selected. Low-calorie and/or low glycaemic index calories may be a useful option for secondary prevention in obese patients with systemic lupus erythematosus, and exercise would be useful in improving the endothelial function measured by flow-mediated dilation in this group of patients. The use of lipid-lowering drugs may improve the lipid profile in patients with systemic lupus erythematosus and hyperlipidaemia, but the effect of this treatment on overall cardiovascular mortality remains unknown. Antiplatelets, anticoagulants, antimalarials and lipid-lowering drugs may be effective in the primary and secondary prevention of major cardiovascular events, such as acute myocardial infarction or stroke. Similarly, lipid-lowering drugs and antimalarial drugs appear to reduce the serum levels of total cholesterol, low-density lipoprotein, glucose, diastolic blood pressure and calcium deposition at the coronary arteries. They may also improve insulin resistance and the level of high-density lipoproteins. It appears that treatment with antihypertensive drugs reduces blood pressure in patients with systemic lupus erythematosus, but the available studies are of low quality.

  10. The Pediatric Rheumatology International Trials Organization/American College of Rheumatology provisional criteria for the evaluation of response to therapy in juvenile systemic lupus erythematosus: prospective validation of the definition of improvement

    DEFF Research Database (Denmark)

    Ruperto, N; Ravelli, A; Oliviera, S

    2006-01-01

    OBJECTIVE: To use the Pediatric Rheumatology International Trials Organization (PRINTO) core set of outcome measures to develop a validated definition of improvement for the evaluation of response to therapy in juvenile systemic lupus erythematosus (SLE). METHODS: Thirty-seven experienced pediatric...... rheumatologists from 27 countries, each of whom had specific experience in the assessment of juvenile SLE patients, achieved consensus on 128 patient profiles as being clinically improved or not improved. Using the physicians' consensus ratings as the gold standard measure, the chi-square, sensitivity......, specificity, false-positive and false-negative rates, area under the receiver operating characteristic curve, and kappa level of agreement for 597 candidate definitions of improvement were calculated. Only definitions with a kappa value greater than 0.7 were retained. The top definitions were selected based...

  11. Are There Various Forms of Lupus?

    Science.gov (United States)

    ... lupus Understanding lupus Are there various forms of lupus? Systemic Lupus Erythematosus Systemic lupus is the most common form ... and tissues at “ Lupus and the Body ”. Cutaneous Lupus Erythematosus This form of lupus is limited to ...

  12. Noma management in a child with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Irna Sufiawati

    2010-03-01

    Full Text Available Background: Noma, also known as cancrum oris, is an orofacial gangrene, which during its fulminating stage causes progressive and mutilating destruction of the infected tissues. The disease occurs mainly in children with malnutrition, poor oral hygiene and debilitating concurrent illness. Purpose: The aim of this paper was to report a unique case of noma associated with systemic lupus erythematosus in an 8-year-old boy. Case: An 8-year-old boy referred to Oral Medicine Department complaining about an ulcer at the left corner of his mouth for 1 month, painful and difficulty in opening the mouth. The patient was diagnosed systemic lupus erythematosus since 14 months before and had been given immunosuppressive therapy. The patient was also diagnosed severe malnutrition. Haematologic investigations revealed anemia. Case management: Panoramic radiography was performed to check for dental or periodontal foci of infection, but no abnormalities were present. The microbiology examination revelaed Fusobacterium necrophorum, Staphylococcus aureus, and Klabsiella. The patient has been treated with oral irrigation using hydrogen peroxide, saline and 0.2% chlorhexidine, thus helped to slough the necrotic tissue. Oral antibiotics and analgesics were prescribed. The patient was admitted to hospital under the care of a pediatrician, allergy and immunology specialist, and a nutritionist. The result of the comprehensive disease management showed that the lesion healed completely, but leaving a scar on his corner of the mouth. Its physical effects are permanent and may require reconstructive surgery to be repaired by oral surgeon. Conclusion: Noma is not a primary disease, there are various predisposing factors usually precede its occurrence. The management of noma requires a multidisciplinary approach.Latar Belakang: Noma, dikenal sebagai cancrum oris, adalah gangren pada daerah orofasial, yang menyebabkan kerusakan progresif dari jaringan yang terinfeksi

  13. Venous and Arterial Thrombotic Events in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Hinojosa-Azaola, Andrea; Romero-Diaz, Juanita; Vargas-Ruiz, Angel Gabriel; Nuñez-Alvarez, Carlos A; Cicero-Casarrubias, Alba; Ocampo-Torres, Mario C; Sanchez-Guerrero, Jorge

    2016-03-01

    The incidence of thrombosis in patients with systemic lupus erythematosus (SLE) is 25 to 50-fold higher than in the general population; we aimed to define the characteristics of venous thrombotic events (VTE) and arterial thrombotic events (ATE) to identify the patients at highest risk. The study included 219 patients with recent-onset SLE. At baseline, standardized medical history and laboratory tests were done. Followup visits occurred quarterly, and information about damage accrual, comorbidities, and cardiovascular risk factors was updated annually. Main outcome was development of TE after SLE diagnosis. Thirty-five patients (16%) developed TE (27 VTE, 8 ATE) during 5.21 years of followup; incidence rate 31/1000 patient-years. Most events (57%) developed within the first year of diagnosis, and 69% were not associated with lupus anticoagulant (LAC), determined with 1 method. VTE developed earlier than ATE (2.0 vs 57.5 mos, p = 0.02). In the multivariate analysis, variables preceding VTE included cutaneous vasculitis, nephrotic syndrome, dose of prednisone, and LAC in combination with anti-RNP/Sm antibodies (p < 0.03). Patients with ATE were older (median age 44 vs 29 yrs, p = 0.04), smokers, and had hypertension, diabetes mellitus, dyslipidemia, at least 2 traditional risk factors, nephrotic syndrome, chronic damage, and a higher cumulative dose of prednisone (p < 0.05). LAC in combination with anti-RNP/Sm antibodies was associated with VTE and improved the accuracy for predicting it. Our study suggests that in SLE, VTE and ATE have different risk factors. Understanding these differences is helpful for identifying patients at highest risk. The use of LAC plus anti-RNP/Sm for predicting VTE deserves further study.

  14. Pregnancy implications for systemic lupus erythematosus and the antiphospholipid syndrome.

    Science.gov (United States)

    Andreoli, Laura; Fredi, Micaela; Nalli, Cecilia; Reggia, Rossella; Lojacono, Andrea; Motta, Mario; Tincani, Angela

    2012-05-01

    Multidisciplinary approach and patient counselling have been the key points in the improvement of the management of pregnancy in women with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Most of these women can have successful pregnancy when thoroughly informed and instructed on several different issues. Disease activity should be in stable remission prior to pregnancy in order to reduce the chance for flare during pregnancy. To this purpose, medications must be modulated: "safe" drugs should be continued throughout pregnancy, embryotoxic/foetotoxic drugs should be withdrawn timely, and beneficial drugs such as low dose aspirin and heparin should be added for prophylaxis of maternal and foetal outcome, especially in the presence of antiphospholipid antibodies. The safety profile of anti-rheumatic drugs during pregnancy and breastfeeding should be kept constantly updated, as new data from inadvertent exposure accumulates and new drugs (especially biological agents) are available. Patients may carry autoantibodies that can negatively affect the baby, being neonatal lupus the prototypical case of passively acquired autoimmunity. Research has been greatly active in this field and more information on risk stratification and management are now available for counselling. The effect of both autoantibodies and drug exposure has been evaluated in the offspring: some concerns about learning disabilities have been raised, but these are treatable conditions that are likely to be overcome. To counsel a woman with SLE/APS during childbearing age means also to deal with contraception. Despite the "preferred choice" - combined oral contraceptive - may not be suitable for most of the patients, other options are available and should be offered and discussed with the patient. Fertility is not generally affected in SLE/APS patients, but those cases who require assisted reproduction techniques should be carefully evaluated and managed.

  15. Variables associated to fetal microchimerism in systemic lupus erythematosus patients.

    Science.gov (United States)

    da Silva Florim, Greiciane Maria; Caldas, Heloisa Cristina; Pavarino, Erika Cristina; Bertollo, Eny Maria Goloni; Fernandes, Ida Maria Maximina; Abbud-Filho, Mario

    2016-01-01

    In the present study, we sought to identify the factors during the pregnancy of systemic lupus erythematosus (SLE) patients that could be linked to the presence and proliferation of male fetal cells (MFC) and the possible relation between these factors and development of lupus nephritis (LN). We evaluated 18 healthy women (control group) and 28 women affected by SLE. Genomic DNA was extracted from peripheral blood and quantified using the technique of quantitative real-time polymerase chain reaction (qPCR) for specific Y chromosome sequences. The amount of MFC was significantly higher in the SLE group compared with the controls (SLE 252 ± 654 vs control 2.13 ± 3.7; P = 0.029). A higher amount of MFC was detected among multiparous SLE patients when compared with the control group (SLE 382 ± 924 vs control 0.073 ± 0.045; P = 0.019). LN was associated with reduced amount of MFC (LN 95.5 ± 338 vs control 388 ± 827; P = 0.019) especially when they have delivered their child before age 18 (LN 0.23 ± 0.22 vs control 355 ± 623; P = 0.028). SLE patients present a higher amount of MFC, which may increase with the time since birth of the first male child. LN patients showed an inverse correlation with MFC, suggesting that the role of the cells may be ambiguous during the various stages of development of the disease.

  16. Immunogenetics of systemic lupus erythematosus: A comprehensive review.

    Science.gov (United States)

    Ghodke-Puranik, Yogita; Niewold, Timothy B

    2015-11-01

    Our understanding of the genetic basis of systemic lupus erythematosus has progressed rapidly in recent years. While many genetic polymorphisms have been associated with disease susceptibility, the next major step involves integrating these genetic polymorphisms into the molecular mechanisms and cellular immunology of the human disease. In this review, we summarize some recent work in this area, including the genetics of the type I IFN response in SLE, including polygenic and monogenic factors, as well as epigenetic influences. Contributions of both HLA and non-HLA polymorphisms to the complex genetics of SLE are reviewed. We also review recent reports of specific gene deficits leading to monogenic SLE-like syndromes. The molecular functions of common SLE-risk variants are reviewed in depth, including regulatory variations in promoter and enhancer elements and coding-change polymorphisms, and studies which are beginning to define the molecular and cellular functions of these polymorphisms in the immune system. We discuss epigenetic influences on lupus, with an emphasis on micro-RNA expression and binding, as well as epigenetic modifications that regulate the expression levels of various genes involved in SLE pathogenesis and the ways epigenetic marks modify SLE susceptibility genes. The work summarized in this review provides a fascinating window into the biology and molecular mechanisms of human SLE. Understanding the functional mechanisms of causal genetic variants underlying the human disease greatly facilitates our ability to translate genetic associations toward personalized care, and may identify new therapeutic targets relevant to human SLE disease mechanisms. Copyright © 2015 Elsevier Ltd. All rights reserved.

  17. Pathogenic inflammation and its therapeutic targeting in systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Timothy Andrew Gottschalk

    2015-10-01

    Full Text Available Systemic Lupus Erythematosus (SLE, lupus is a highly complex and heterogeneous autoimmune disease that most often afflicts women in their child-bearing years. It is characterized by circulating self-reactive antibodies that deposit in tissues including skin, kidneys and brain, and the ensuing inflammatory response can lead to irreparable tissue damage. Over many years, clinical trials in SLE have focused on agents that control B and T lymphocyte activation, and, with the single exception of an agent known as Belimumab which targets the B cell survival factor BAFF, they have been disappointing. At present, standard therapy for SLE with mild disease is the agent hydroxychloroquine. During disease flares, steroids are often used, while the more severe manifestations with major organ involvement warrant potent, broad-spectrum immuno-suppression with cyclophosphamide or mycophenolate. Current treatments have severe and dose-limiting toxicities and thus a more specific therapy targeting a causative factor or signaling pathway would be greatly beneficial in SLE treatment. Moreover, the ability to control inflammation alongside B cell activation may be a superior approach for disease control. There has been a recent focus on the innate immune system and associated inflammation, which has uncovered key players in driving the pathogenesis of SLE. Delineating some of these intricate inflammatory mechanisms has been possible with studies using spontaneous mouse mutants and genetically engineered mice. These strains, to varying degrees, exhibit hallmarks of the human disease and therefore have been utilized to model human SLE and to test new drugs. Developing a better understanding of the initiation and perpetuation of disease in SLE may uncover suitable novel targets for therapeutic intervention. Here we discuss the involvement of inflammation in SLE disease pathogenesis, with a focus on several key proinflammatory cytokines and myeloid growth factors, and

  18. Borderline tuberculoid leprosy in childhood onset systemic lupus erythematosus patient.

    Science.gov (United States)

    Lopes, V A P; Lourenço, D M R; Guariento, A; Trindade, M A; Avancini, J; Silva, C A

    2015-11-01

    Leprosy is a contagious and chronic systemic granulomatous disease caused by the bacillus Mycobacterium leprae. To our knowledge, no case of leprosy in a childhood-onset systemic lupus erythematosus (c-SLE) patient has been reported. For a period of 31 years, 312 c-SLE patients were followed at the Pediatric Rheumatology Unit of our University Hospital. One of them (0.3%) had tuberculoid leprosy skin lesions during the disease course and is here reported. A 10-year-old boy from Northwest of Brazil was diagnosed with c-SLE based on malar rash, photosensitivity, oral ulcers, lymphopenia, proteinuria, positive antinuclear antibodies, anti-double-stranded DNA, anti-Sm and anti-Ro/SSA autoantibodies. He was treated with prednisone, hydroxychloroquine and intravenous cyclophosphamide, followed by mycophenolate mofetil. At 12-years-old, he presented asymmetric skin lesions characterized by erythematous plaques with elevated external borders and hypochromic center with sensory loss. Peripheral nerve involvement was not evidenced. No history of familial cases of leprosy was reported, although the region where the patient resides is considered to be endemic for leprosy. Skin biopsy revealed a well-defined tuberculoid form. A marked thickening of nerves was observed, often destroyed by granulomas, without evidence of Mycobacterium leprae bacilli. At that time, the SLEDAI-2K score was 4 and he had been receiving prednisone 15 mg/day, hydroxychloroquine 200 mg/day and mycophenolate mofetil 3 g/day. Paucibacillary treatment for leprosy with dapsone and rifampicine was also introduced. In conclusion, we have reported a rare case of leprosy in the course of c-SLE. Leprosy should always be considered in children and adolescents with lupus who present skin abnormalities, particularly with hypoesthesic or anesthesic cutaneous lesions. © The Author(s) 2015.

  19. Modified Framingham Risk Factor Score for Systemic Lupus Erythematosus.

    Science.gov (United States)

    Urowitz, Murray B; Ibañez, Dominique; Su, Jiandong; Gladman, Dafna D

    2016-05-01

    The traditional Framingham Risk Factor Score (FRS) underestimates the risk for coronary artery disease (CAD) in patients with systemic lupus erythematosus (SLE). We aimed to determine whether an adjustment to the FRS would more accurately reflect the higher prevalence of CAD among patients with SLE. Patients with SLE without a previous history of CAD or diabetes followed regularly at the University of Toronto Lupus Clinic were included. A modified FRS (mFRS) was calculated by multiplying the items by 1.5, 2, 3, or 4. In the first part of the study, using one-third of all eligible patients, we evaluated the sensitivity and specificity of the FRS and the different multipliers for the mFRS. In the second part of the study, using the remaining 2/3 of the eligible patients, we compared the predictive ability of the FRS to the mFRS. In the third part of the study, we assessed the prediction for CAD in a time-dependent analysis of the FRS and mFRS. There were 905 women (89.3%) with a total of 95 CAD events included. In part 1, we determined that a multiplier of 2 provided the best combination of sensitivity and specificity. In part 2, 2.4% of the patients were classified as moderate/high risk based on the classic FRS and 17.3% using the 2FRS (the FRS with a multiplier of 2). In part 3, a time-dependent covariate analysis for the prediction of the first CAD event revealed an HR of 3.22 (p = 0.07) for the classic FRS and 4.37 (p mFRS in which each item is multiplied by 2 more accurately predicts CAD in patients with SLE.

  20. Obesity and Cytokines in Childhood-Onset Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Nailú Angélica Sinicato

    2014-01-01

    Full Text Available Background. In systemic lupus erythematosus (SLE, atherosclerosis is attributed to traditional and lupus related risk factors, including metabolic syndrome (MetS, obesity, and inflammation. Objective. To evaluate the association between obesity, measures of body fat content, serum tumor necrosis factor alpha (TNF-α, and interleukin (IL-6 and -10 levels in childhood-onset SLE (cSLE. Methods. We screened consecutive cSLE patients followed up in the Pediatric Rheumatology Outpatient Clinic of the State University of Campinas. cSLE patients were assessed for disease and damage. Obesity was definite as body mass index (BMI ≥30 kg/m2. Serum TNF-α, IL-6, and IL-10 levels were measured by ELISA. Dual-energy X-ray absorptiometry was used to determine total fat mass, lean mass, and percent of body fat. Results. We included 52 cSLE patients and 52 controls. cSLE patients had higher serum TNF-α  (P=0.004, IL-6 (P=0.002, and IL-10 (P<0.001 levels compared to controls. We observed higher serum TNF-α  (P=0.036 levels in cSLE patients with obesity. An association between serum TNF-α levels and body fat percent (P=0.046 and total fat mass on trunk region (P=0.035 was observed. Conclusion. Serum TNF-α levels were associated with obesity and body fat content in cSLE. Our finding suggests that obesity may contribute to the increase of serum TNF-α levels in cSLE.

  1. Pathogenic Inflammation and Its Therapeutic Targeting in Systemic Lupus Erythematosus

    Science.gov (United States)

    Gottschalk, Timothy A.; Tsantikos, Evelyn; Hibbs, Margaret L.

    2015-01-01

    Systemic lupus erythematosus (SLE, lupus) is a highly complex and heterogeneous autoimmune disease that most often afflicts women in their child-bearing years. It is characterized by circulating self-reactive antibodies that deposit in tissues, including skin, kidneys, and brain, and the ensuing inflammatory response can lead to irreparable tissue damage. Over many years, clinical trials in SLE have focused on agents that control B- and T-lymphocyte activation, and, with the single exception of an agent known as belimumab which targets the B-cell survival factor BAFF, they have been disappointing. At present, standard therapy for SLE with mild disease is the agent hydroxychloroquine. During disease flares, steroids are often used, while the more severe manifestations with major organ involvement warrant potent, broad-spectrum immunosuppression with cyclophosphamide or mycophenolate. Current treatments have severe and dose-limiting toxicities and thus a more specific therapy targeting a causative factor or signaling pathway would be greatly beneficial in SLE treatment. Moreover, the ability to control inflammation alongside B-cell activation may be a superior approach for disease control. There has been a recent focus on the innate immune system and associated inflammation, which has uncovered key players in driving the pathogenesis of SLE. Delineating some of these intricate inflammatory mechanisms has been possible with studies using spontaneous mouse mutants and genetically engineered mice. These strains, to varying degrees, exhibit hallmarks of the human disease and therefore have been utilized to model human SLE and to test new drugs. Developing a better understanding of the initiation and perpetuation of disease in SLE may uncover suitable novel targets for therapeutic intervention. Here, we discuss the involvement of inflammation in SLE disease pathogenesis, with a focus on several key proinflammatory cytokines and myeloid growth factors, and review the known

  2. Incidence and prevalence of major central nervous system involvement in systemic lupus erythematosus: a 3-year prospective study of 370 patients.

    Directory of Open Access Journals (Sweden)

    Eleni I Kampylafka

    Full Text Available BACKGROUND: The incidence and prevalence of CNS involvement in SLE remains unclear owing to conflicting results in the published studies. The aim of the study was to evaluate the incidence and prevalence of major definite CNS events in SLE patients. METHODS: 370 SLE patients with no previous history of CNS involvement were prospectively evaluated in a tertiary hospital referral center for 3 years. Major CNS manifestations were codified according to ACR definitions, including chorea, aseptic meningitis, psychosis, seizures, myelopathy, demyelinating syndrome, acute confusional state and strokes. Minor CNS events were excluded. ECLAM and SLEDAI-SELENA Modification scores were used to evaluate disease activity and SLICC/ACR Damage Index was used to assess accumulated damage. RESULTS: 16/370 (4.3% patients presented with a total of 23 major CNS events. These included seizures (35%, strokes (26%, myelopathy (22%, optic neuritis (8.7%, aseptic meningitis (4.3% and acute psychosis (4.3%. Incidence was 7.8/100 person years. Among hospitalizations for SLE, 13% were due to CNS manifestations. Epileptic seizures were associated with high disease activity, while myelopathy correlated with lower disease activity and NMO-IgG antibodies (P≤0.05. Stroke incidence correlated with APS coexistence (P = 0.06. Overall, CNS involvement correlated with high ECLAM and SLEDAI scores (P<0.001. CONCLUSIONS: Clinically severe CNS involvement is rare in SLE patients, accounting for 7.8/100 person years. CNS involvement correlates with high disease activity and coexistence of specific features that define the respective CNS syndromes.

  3. Alcohol, smoking and illicit drug use in pediatric systemic lupus erythematosus patients

    Directory of Open Access Journals (Sweden)

    Marlon van Weelden

    2016-06-01

    Full Text Available Abstract Objective To evaluate alcohol, smoking and/or illicit drug use, and history of bullying in adolescent childhood-onset systemic lupus erythematosus and healthy controls. Methods 174 adolescents with pediatric rheumatic diseases were selected. All of the 34 childhood-onset systemic lupus erythematosus patients and 35 healthy controls participated in this study. A cross-sectional study included demographic/anthropometric data and puberty markers assessments; structured questionnaire and CRAFFT screening interview. Results McNemar tests indicated an excellent test–retest reliability of the structured questionnaire (p = 1.0. The median current age was similar between childhood-onset systemic lupus erythematosus patients and controls [15 (12–18 vs. 15 (12–18 years, p = 0.563]. The median of menarche age was significantly higher in childhood-onset systemic lupus erythematosus patients compared to controls [12 (10–15 vs. 11.5 (9–15 years, p = 0.041], particularly in those that lupus had occurred before first menstruation [13 (12–15 vs. 11.5(9–15 years, p = 0.007]. The other puberty marker and sexual function parameters were similar in both groups (p > 0.05. Alcohol use was similar in both childhood-onset systemic lupus erythematosus patients and controls (38% vs. 46%, p = 0.628. A trend of lower frequency of CRAFFT score ≥2 (high risk for substance abuse/dependence was evidenced in childhood-onset systemic lupus erythematosus patients compared to controls (0% vs. 15%, p = 0.053. Bullying was reported similarly for the two groups (43% vs. 44%, p = 0.950. Further analysis in lupus patients regarding alcohol/smoking/illicit drug use showed no differences in demographic data, puberty markers, history of bullying, sexual function, contraceptive use, disease activity/damage scores, clinical/laboratorial features and treatments (p > 0.05. Conclusion This study showed high frequencies of early alcohol use in lupus adolescents and

  4. Lupus erythematosus/lichen planus overlap syndrome: successful treatment with acitretin.

    Science.gov (United States)

    Lospinoso, D J; Fernelius, C; Edhegard, K D; Finger, D R; Arora, N S

    2013-07-01

    Lupus erythematosus/lichen planus overlap syndrome is a rare disorder combining the clinical, histological and immunopathological features of both lupus erythematosus (LE) and lichen planus (LP). Cutaneous lesions mostly affect the distal arms, legs, face and trunk. Palmoplantar involvement is felt to be characteristic of this condition. Plaques are often painful, centrally atrophic, bluish-red to hypopigmented in color, large, and scaly. On biopsy of clinically ambiguous lesions, histopathological features of one or both processes can be found, obscuring the diagnosis and complicating prognosis and treatment. Thus, direct immunofluorescence has become an essential tool in helping to diagnose this condition. In this report we describe the unique clinical and immunohistopathological manifestations of lupus erythematosus/lichen planus overlap syndrome along with a successful response to treatment with acitretin.

  5. Achados capilaroscópicos no lúpus eritematoso Capillaroscopy findings in lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Anamaria da Silva Facina

    2006-12-01

    Full Text Available FUNDAMENTOS: A capilaroscopia é método não invasivo e reprodutível capaz de analisar diretamente os capilares na região periungueal, auxiliando no diagnóstico diferencial das doenças do tecido conectivo. OBJETIVOS: Estudar, por meio da capilaroscopia periungueal, pacientes com lúpus eritematoso cutâneo crônico, lúpus eritematoso sistêmico e grupo controle. MÉTODOS: Foram analisados 70 pacientes pela capilaroscopia periungueal, sendo 37 com lúpus eritematoso cutâneo crônico e 33 com forma sistêmica, comparados a 32 indivíduos sadios. RESULTADOS: A presença de capilares ectasiados (p=0,027; p=0,001, enovelados (p=0,001; p=0,007 e em saca-rolhas (p=0,011;p=0,005, além de hemorragias capilares (p=0,004; p=0,001 foram parâmetros capazes de discriminar os dois grupos de pacientes do grupo controle. A variável capilar enovelado demonstrou ser preditiva para o diagnóstico de lúpus eritematoso sistêmico (OR=8,308. As variáveis independentes capilares ectasiados (OR=12,164 e hemorragias capilares (OR=5,652 foram preditoras para lúpus eritematoso cutâneo crônico. CONCLUSÃO: A capilaroscopia é útil na prática clínica, pois pacientes com alterações capilaroscópicas específicas parecem ter maior probabilidade de desenvolver lúpus eritematoso. As variáveis preditoras independentes para lúpus eritematoso sistêmico foram capilares enovelados e para lúpus eritematoso cutâneo crônico foram capilares ectasiados e hemorragias capilares.BACKGROUND: Capillaroscopy is an useful diagnostic tool that is non-invasive, reproducible, able to assess the capillaries in the periungal region and that assists in the differential diagnosis of connective tissue diseases. OBJETIVES: The aim of the study was to distinguish chronic cutaneous lupus erythematosus and systemic lupus erythematosus from controls assessed by nailfold capillaroscopy. METHODS: Seventy patients with lupus erythematosus (37 with chronic cutaneous lupus erythematosus

  6. Bilateral sudden sensorineural hearing loss as a presenting feature of systemic lupus erythematosus

    Science.gov (United States)

    Chawki, Sylvain; Aouizerate, Jessie; Trad, Selim; Prinseau, Jacques; Hanslik, Thomas

    2016-01-01

    Abstract Introduction: Sudden sensorineural hearing loss is an unusual presenting clinical feature of systemic lupus erythematosus. Case report: We report the case of a young woman who was admitted to hospital for sudden sensorineural hearing loss and hemophagocytic syndrome which was attributed to systemic lupus erythematosus on the basis of specific renal involvement, thrombocytopenia, and consistent autoantibodies. Favorable outcome was obtained on high-dose corticosteroids, and the hearing fully recovered. Discussion: Sudden sensorineural hearing loss in systemic lupus erythematosus is seemingly more frequently associated with severe systemic involvement and antiphospholipid antibodies may be present. Although management remains empirical, the high risk of permanent hearing impairment seems to justify emergency treatment with high-dose corticosteroids. When the clinical and laboratory criteria of antiphospholipid syndrome are met, antiplatelets agents or anticoagulation therapy shall be considered. PMID:27603334

  7. Influence of smoking on disease severity and antimalarial therapy in cutaneous lupus erythematosus

    DEFF Research Database (Denmark)

    Kuhn, A; Sigges, J; Biazar, C

    2014-01-01

    BACKGROUND: In recent years it has been controversially discussed in the literature if smoking is associated with the activity of cutaneous lupus erythematosus (CLE) and the efficacy of antimalarial agents. OBJECTIVES: To investigate the influence of smoking on disease severity and antimalarial...... treatment in patients with CLE using the Core Set Questionnaire of the European Society of Cutaneous Lupus Erythematosus (EUSCLE). METHODS: A total of 1002 patients (768 female, 234 male) with different CLE subtypes were included in this cross-sectional study, which was performed in 14 different countries....... Smoking behaviour was assessed by the EUSCLE Core Set Questionnaire in 838 patients and statistically analysed using an SPSS database. The results were correlated with the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and the efficacy of antimalarial treatment. RESULTS: A high...

  8. Neglect leads to extremes: maggots and malignancy in a case of discoid lupus erythematosus.

    Science.gov (United States)

    Bhari, N; Khaitan, B K; Gupta, P; Kumar, T; Srivastava, A

    2016-01-01

    Discoid lupus erythematosus (DLE) is a chronic form of cutaneous lupus erythematosus that runs an indolent course. The rare complications of DLE include scarring, mutilation, non-healing ulceration, cicatricial alopecia and malignancy. DLE progresses to systemic lupus erythematosus (SLE) in around 5% of localized cases and 22% of generalized cases. We report a case of DLE, presenting with a six-month history of ulcerated fungating plaques and small crusted nodules superimposed on DLE plaques over both the forearms. Two weeks prior to the presentation, maggots were also noticed on these plaques. Skin biopsies from these lesions were suggestive of squamous cell carcinoma (SCC) and keratoacanthoma. A wide surgical excision of the tumor followed by partial split-thickness skin grafting was performed with complete healing of the lesions. No recurrence has been noted 18 months from follow-up.

  9. Specific cutaneous histologic and immunologic features in a case of early lupus erythematosus scarring alopecia

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    Ana Maria Abreu Velez

    2013-04-01

    Full Text Available Introduction:Immunoreactants detected by direct immunofluorescence (DIF in the skin of patients with lupus erythematosus represent an important tool in the diagnosis of this disorder. Case report: A 46 year old African American female presented complaining of hair loss and scarring in her scalp.Methods: Biopsies for hematoxylin and eosin (H&E examination, as well as for direct immunofluorescence (DIF were performed.Results: The histologic features were representative of early lupus erythematosus. DIF demonstrated immune deposits of several immunoglobulins and complement, primarily around skin appendageal structures(hair follicles and sweat glands. Deposits of immunoglobulin D were seen in several areas of the epidermis.Conclusion: In lupus erythematosus, evaluation of immune reactions against cutaneous appendageal structures may be crucial in differentiating this disorder from other autoimmune and non-autoimmune diseases.

  10. Comparison of estimates of body fat content in childhood-onset systemic lupus erythematosus.

    Science.gov (United States)

    Sinicato, N A; Peres, F A; de Oliveira Peliçari, K; de Oliveira Santos, A; Ramos, C D; Marini, R; Appenzeller, S

    2017-04-01

    Objective We aimed to compare estimates of body fat content with respect to their ability to predict the percentage of body fat, confirmed by dual-energy X-ray absorptiometry scans in childhood-onset systemic lupus erythematosus. Methods We included 64 consecutive childhood-onset systemic lupus erythematosus patients and 64 healthy age and sex-matched controls in a cross-sectional study. Anthropometric data, body mass index and body adiposity index were calculated for all subjects. Childhood-onset systemic lupus erythematosus patients were further assessed for clinical and laboratory childhood-onset systemic lupus erythematosus manifestations and fat mass, lean mass and percentage of body fat evaluated by dual-energy X-ray absorptiometry. Results Elevated waist/hip ratio was observed in childhood-onset systemic lupus erythematosus patients when compared to controls ( p systemic lupus erythematosus patients and controls. Using dual-energy X-ray absorptiometry as gold standard we observed that all indirect estimates of body fat were correlated with whole body fat mass. We observed a correlation between height and cumulative corticosteroid dose adjusted by weight ( r = 0.429, p = 0.005) in childhood-onset systemic lupus erythematosus. On whole body analysis we observed a correlation between lean mass and ACR Damage Index scores ( r = -0.395; p = 0.019); percentage of body fat and adjusted Systemic Lupus Erythematosus Disease Activity Index ( r = 0.402; p = 0.008), disease duration ( r = -0.370; p = 0.012). On trunk analysis we observed a correlation between lean mass and ACR Damage Index ( r = -0.319; p = 0.042); percentage of body fat with adjusted Systemic Lupus Erythematosus Disease Activity Index ( r = 0.402; p = 0.005), disease duration ( r = -0.408; p = 0.005). Conclusions This is the first study analyzing body adiposity index in childhood-onset systemic lupus erythematosus patients. We observed that all

  11. Glial and axonal changes in systemic lupus erythematosus measured with diffusion of intracellular metabolites.

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    Ercan, Ece; Magro-Checa, Cesar; Valabregue, Romain; Branzoli, Francesca; Wood, Emily T; Steup-Beekman, Gerda M; Webb, Andrew G; Huizinga, Tom W J; van Buchem, Mark A; Ronen, Itamar

    2016-05-01

    Systemic lupus erythematosus is an inflammatory autoimmune disease with multi-organ involvement. Central nervous system involvement in systemic lupus erythematosus is common and results in several neurological and psychiatric symptoms that are poorly linked to standard magnetic resonance imaging outcome. Magnetic resonance imaging methods sensitive to tissue microstructural changes, such as diffusion tensor imaging and magnetization transfer imaging, show some correlation with neuropsychiatric systemic lupus erythematosus (NPSLE) symptoms. Histological examination of NPSLE brains reveals presence of cerebral oedema, loss of neurons and myelinated axons, microglial proliferation and reactive astrocytosis, microinfacrts and diffuse ischaemic changes, all of which can affect both diffusion tensor imaging and magnetization transfer imaging in a non-specific manner. Here we investigated the underlying cell-type specific microstructural alterations in the brain of patients with systemic lupus erythematosus with and without a history of central nervous system involvement. We did so combining diffusion tensor imaging with diffusion-weighted magnetic resonance spectroscopy, a powerful tool capable of characterizing cell-specific cytomorphological changes based on diffusion of intracellular metabolites. We used a 7 T magnetic resonance imaging scanner to acquire T1-weighted images, diffusion tensor imaging datasets, and single volume diffusion-weighted magnetic resonance spectroscopy data from the anterior body of the corpus callosum of 13 patients with systemic lupus erythematosus with past NPSLE, 16 patients with systemic lupus erythematosus without past NPSLE, and 19 healthy control subjects. Group comparisons were made between patients with systemic lupus erythematosus with/without past NPSLE and healthy controls on diffusion tensor imaging metrics and on diffusion coefficients of three brain metabolites: the exclusively neuronal/axonal N-acetylaspartate, and the

  12. Current views on etiopathogenesis of systemic lupus erythematosus

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    Agnieszka Klonowska-Szymczyk

    2011-11-01

    Full Text Available The article is a review of information concerning etiopathogenesis of systemic lupus erythematosus (SLE. Due to the risk of serious complications, including death, the clarification of etiology could result in substantial improvement or even complete cure of the disease. Progress in scientific research of observed disorder mechanisms together with implementation of appropriate therapies contributed to a higher detection rate, improved course and decreased mortality in SLE. However, there are still many doubts, which legitimate the need of further research. A significant role in development of the disease and further exacerbations is played by environmental factors. Therefore, decreased exposure to UV light, female sex hormone and microbial antigens is associated with improved course and decreased frequency of exacerbations. Less is known about the genetic basis of SLE, which results from a multigene disease background and complex hereditary mechanisms. It is estimated that the disease may be conditioned by around 100 genes, that only in part are functionally determined. Only part of them is already functionally characterized. The role played by most of them is still unknown. Research currently being conducted is aimed at detecting genetic polymorphism in large and genetically diverse populations. It will allow evaluation of the role of a particular gene in protein biosynthesis, which is responsible for development of regulatory process disturbances, commonly observed in the course of SLE. The article presents current directions of research and the latest advances in epidemiology as well as environmental and genetic risk factors of SLE.

  13. Genetics and novel aspects of therapies in systemic lupus erythematosus.

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    Relle, Manfred; Weinmann-Menke, Julia; Scorletti, Eva; Cavagna, Lorenzo; Schwarting, Andreas

    2015-11-01

    Autoimmune diseases, such as rheumatoid arthritis, multiple sclerosis, autoimmune hepatitis and inflammatory bowel disease, have complex pathogeneses and the factors which cause these disorders are not well understood. But all have in common that they arise from a dysfunction of the immune system, interpreting self components as foreign antigens. Systemic lupus erythematosus (SLE) is one of these complex inflammatory disorders that mainly affects women and can lead to inflammation and severe damage of virtually any tissue and organ. Recently, the application of advanced techniques of genome-wide scanning revealed more genetic information about SLE than previously possible. These case-control or family-based studies have provided evidence that SLE susceptibility is based (with a few exceptions) on an individual accumulation of various risk alleles triggered by environmental factors and also help to explain the discrepancies in SLE susceptibility between different populations or ethnicities. Moreover, during the past years new therapies (autologous stem cell transplantation, B cell depletion) and improved conventional treatment options (corticosteroids, traditional and new immune-suppressants like mycophenolate mofetile) changed the perspective in SLE therapeutic approaches. Thus, this article reviews genetic aspects of this autoimmune disease, summarizes clinical aspects of SLE and provides a general overview of conventional and new therapeutic approaches in SLE. Copyright © 2015. Published by Elsevier B.V.

  14. Competitive endogenous RNA network: potential implication for systemic lupus erythematosus.

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    Li, Lian-Ju; Zhao, Wei; Tao, Sha-Sha; Leng, Rui-Xue; Fan, Yin-Guang; Pan, Hai-Feng; Ye, Dong-Qing

    2017-06-01

    Competitive endogenous RNA (ceRNA) hypothesis proposes that RNA transcripts, both coding and non-coding, crosstalk with and coregulate each other using microRNA response elements (MREs). CeRNA analysis tremendously expands functional information of coding and non-coding RNAs. Mounting evidence have shown that various types of RNAs, including pseudogenes, long non-coding RNAs, circular RNAs, and messenger RNAs, can function as ceRNAs in distinct physiological and pathophysiological states. Many validated ceRNA pairs participate in the initiation and progression of cancers, and systemic ceRNA network analyses revealing potential of ceRNAs in diagnosis, therapy, and prognosis of cancers have also been performed. Areas covered: This review concisely introduces ceRNA hypothesis and characteristics of ceRNA regulations. The major sections focus on representative examples of both protein coding and non-coding RNA transcripts acting as ceRNAs. CeRNA prediction programs and databases and implications of ceRNA network in cancers are then discussed. In the end, we surmise potential implications of ceRNA network for SLE. Expert opinion: The role of ceRNA network in systemic lupus erythematosus (SLE) remains undefined. We speculate that dissecting ceRNA network in SLE may help expand our comprehension of roles of transcriptome, particularly non-coding transcripts, and richen our knowledge of pathogenesis, diagnosis, and therapy of SLE.

  15. Outcomes in hospitalized pediatric patients with systemic lupus erythematosus.

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    Son, Mary Beth F; Johnson, Victor M; Hersh, Aimee O; Lo, Mindy S; Costenbader, Karen H

    2014-01-01

    Disparities in outcomes among adults with systemic lupus erythematosus (SLE) have been documented. We investigated associations between sociodemographic factors and volume of annual inpatient hospital admissions with hospitalization characteristics and poor outcomes among patients with childhood-onset SLE. By using the Pediatric Health Information System, we analyzed admissions for patients aged 3 to <18 years at index admission with ≥ 1 International Classification of Diseases, Ninth Revision code for SLE from January 2006 to September 2011. Summary statistics and univariable analyses were used to examine demographic characteristics of hospital admissions, readmissions, and lengths of stay. We used multivariable logistic regression analyses, controlling for patient gender, age, race, ethnicity, insurance type, hospital volume, US census region, and severity of illness, to examine risk factors for poor outcomes. A total of 10,724 admissions occurred among 2775 patients over the study period. Hispanic patients had longer lengths of stay, more readmissions, and higher in-hospital mortality. In multivariable analysis, African American race was significantly associated with ICU admission. African American race and Hispanic ethnicity were associated with end-stage renal disease and death. Volume of patients with SLE per hospital and hospital location were not significantly associated with outcomes. In this cohort of hospitalized children with SLE, race and ethnicity were associated with outcomes. Further studies are needed to elucidate the relationship between sociodemographic factors and poor outcomes in patients with childhood-onset SLE.

  16. The sense of smell in systemic lupus erythematosus.

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    Shoenfeld, Netta; Agmon-Levin, Nancy; Flitman-Katzevman, Iveta; Paran, Daphna; Katz, Bat-sheva Porat; Kivity, Shaye; Langevitz, Pnina; Zandman-Goddard, Gisele; Shoenfeld, Yehuda

    2009-05-01

    To assess the olfactory functions in systemic lupus erythematosus (SLE) patients compared with age- and sex-matched healthy controls, and to examine the association between the sense of smell and disease activity and central nervous system (CNS) involvement. Olfactory functions in 50 SLE patients and 50 age- and sex-matched controls were evaluated using the Sniffin' Sticks test, the 3 stages of which are threshold, discrimination, and identification (TDI) of different odors. TDI scores were analyzed according to SLE disease activity and CNS involvement. In both the SLE and control groups, smell deficit correlated with male sex and older age. A decrease in the sense of smell was observed in SLE patients (46%) and controls (25%) (Psmell (anosmia) was documented only in SLE patients (10%). Total TDI scores and individual stages of smell correlated with SLE Disease Activity Index (Psmell in SLE patients compared with healthy subjects and that the decrease in the sense of smell among SLE patients correlates with disease activity and CNS involvement.

  17. Vitamin D levels in Jamaican patients with systemic lupus erythematosus.

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    McGhie, T K; DeCeulaer, K; Walters, C A; Soyibo, A; Lee, M G

    2014-09-01

    Vitamin D has important effects on the immune system as it has been shown to exert antiproliferative and relative immunosuppressant effects. Low levels of this hormone may contribute to the immune activation in systemic lupus erythematosus (SLE) and other autoimmune diseases. Serum levels of 25-OH vitamin D were measured in 75 patients with SLE in Jamaica, using an enzyme-linked immunoassay. Correlations with clinical data and disease activity as determined by the BILAG index were determined. Of a total of 75 patients, 33 (44%) had vitamin D sufficiency with mean vitamin D level of 39.45 ng/ml (range, 30.35-58.16). Forty-two (56%) patients had either vitamin D deficiency or insufficiency, 30 (40%) had vitamin D insufficiency, mean 26.36 ng/ml (range, 20.26-29.88), and 12 (16%) had vitamin D deficiency, mean 16.07 ng/ml (range, 7.78-19.90). There was an overall negative relationship between the total disease activity score using the BILAG index and vitamin D levels, and this was influenced primarily by the relationship seen among the vitamin D-deficient subgroup. This was also impacted on by a patient population that was significantly skewed toward low disease activity. The negative association trended toward statistical significance. Vitamin D deficiency is prevalent among patients with SLE in Jamaica. A relationship between low serum levels of vitamin D and SLE activity may occur.

  18. Periodontal disease in Chinese patients with systemic lupus erythematosus.

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    Zhang, Qiuxiang; Zhang, Xiaoli; Feng, Guijaun; Fu, Ting; Yin, Rulan; Zhang, Lijuan; Feng, Xingmei; Li, Liren; Gu, Zhifeng

    2017-08-01

    Disease of systemic lupus erythematosus (SLE) and periodontal disease (PD) shares the common multiple characteristics. The aims of the present study were to evaluate the prevalence and severity of periodontal disease in Chinese SLE patients and to determine the association between SLE features and periodontal parameters. A cross-sectional study of 108 SLE patients together with 108 age- and sex-matched healthy controls was made. Periodontal status was conducted by two dentists independently. Sociodemographic characteristics, lifestyle factors, medication use, and clinical parameters were also assessed. The periodontal status was significantly worse in SLE patients compared to controls. In univariate logistic regression, SLE had a significant 2.78-fold [95% confidence interval (CI) 1.60-4.82] increase in odds of periodontitis compared to healthy controls. Adjusted for potential risk factors, patients with SLE had 13.98-fold (95% CI 5.10-38.33) increased odds against controls. In multiple linear regression model, the independent variable negatively and significantly associated with gingival index was education (P = 0.005); conversely, disease activity (P periodontitis of SLE in multivariate logistic regression (OR 1.348; 95% CI: 1.183-1.536, P < 0.001). Chinese SLE patients were likely to suffer from higher odds of PD. These findings confirmed the importance of early interventions in combination with medical therapy. It is necessary for a close collaboration between dentists and clinicians when treating those patients.

  19. The pathophysiology of hypertension in systemic lupus erythematosus.

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    Ryan, Michael J

    2009-04-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disorder that predominantly affects women during their reproductive years. Although SLE can affect any organ system, the kidneys are prominently involved in the form of immune complex glomerulonephritis. In addition, in women with SLE, risk for the development of cardiovascular disease is dramatically increased. Hypertension is a major risk factor for cardiovascular disease and is highly prevalent in women with SLE. Nevertheless, there has been little exploration of the pathophysiological mechanisms that promote SLE hypertension. This review discusses the role of several mechanisms, with an emphasis on the kidney, in SLE hypertension. These mechanisms include the renin-angiotensin system, endothelin, oxidative stress, sex steroids, metabolic changes, peroxisome proliferator-activated receptor-gamma, and, perhaps most importantly, chronic inflammation and cytokines. Growing evidence suggests a link between chronic inflammation and hypertension. Therefore, elucidation of mechanisms that promote SLE hypertension may be of significant value not only for patients with SLE, but also for a better understanding of the basis for essential hypertension.

  20. Degos-Like Lesions Associated with Systemic Lupus Erythematosus

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    Jang, Min Soo; Park, Jong Bin; Yang, Myeong Hyeon; Jang, Ji Yun; Kim, Joon Hee; Lee, Kang Hoon; Kim, Geun Tae; Hwangbo, Hyun

    2017-01-01

    Degos disease, also referred to as malignant atrophic papulosis, was first described in 1941 by Köhlmeier and was independently described by Degos in 1942. Degos disease is characterized by diffuse, papular skin eruptions with porcelain-white centers and slightly raised erythematous telangiectatic rims associated with bowel infarction. Although the etiology of Degos disease is unknown, autoimmune diseases, coagulation disorders, and vasculitis have all been considered as underlying pathogenic mechanisms. Approximately 15% of Degos disease have a benign course limited to the skin and no history of gastrointestinal or central nervous system (CNS) involvement. A 29-year-old female with history of systemic lupus erythematosus (SLE) presented with a 2-year history of asymptomatic lesions on the dorsum of all fingers and both knees. The patient had only skin lesions and no gastrointestinal or CNS vasculitis symptoms. Her skin lesions were umbilicated, atrophic porcelain-white lesions with a rim of erythema. On the basis of clinical, histologic, and laboratory findings, a diagnosis of Degos-like lesions associated with SLE was made. The patient had been treated for SLE for 7 years. Her treatment regimen was maintained over a 2 month follow-up period, and the skin lesions improved slightly with no development of new lesions. PMID:28392651

  1. Dehydroepiandrosterone for the treatment of systemic lupus erythematosus.

    Science.gov (United States)

    van Vollenhoven, Ronald F

    2002-01-01

    The adrenal steroidal hormone dehydroepiandrosterone (DHEA) has been studied as a potential pharmacological agent in the treatment of the autoimmune disease systemic lupus erythematosus (SLE). Both the endocrine effects (the ability to be converted peripherally to androgenic and oestrogenic sex steroids) and the immunomodulatory effects of DHEA (the production of the Th(1) cytokines, such as IL-2) suggest that this hormone could be of benefit for patients with SLE. During the past decade, five controlled clinical trials and a number of additional observational studies have been performed investigating these possibilities. The results from these studies suggest that 200 mg/day of DHEA for 7 - 12 months decreases corticosteroid requirement for the patients, the frequency of disease flares, has an anti-osteoporotic effect and has an overall beneficial effect on SLE disease activity in female patients. A small study suggested benefits for cognitive function in such patients. The side effects acne and hirsutism were seen relatively frequently (30 - 40% and 10 - 12% of patients, respectively) but in most instances were deemed mild. DHEA treatment resulted in changes in lipid profile and may have endocrine effects, the consequences of which will need to be ascertained through longer-term follow-up studies.

  2. MEFV gene variations in patients with systemic lupus erythematosus.

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    Erer, Burak; Cosan, Fulya; Oku, Basar; Ustek, Duran; Inanc, Murat; Aral, Orhan; Gul, Ahmet

    2014-01-01

    The aim of this study was to investigate the frequency of familial Mediterranean fever (FMF)-associated MEFV gene variations in patients with systemic lupus erythematosus (SLE). The study group comprised 190 SLE patients and 101 healthy controls of Turkish origin with no clinical features of FMF. All individuals were genotyped for the four most common MEFV gene variations (M694V, M680I, V726A and E148Q) by PCR-restriction fragment length polymorphism analysis. The frequency of carrying any of the four MEFV gene variations under study was 15 % in patients with SLE and 10 % in the healthy controls (p = 0.23). After the exclusion of the less penetrant E148Q variation, re-analysis for the three penetrant mutations revealed a significant association between exon 10 variations and pericarditis [p = 0.038, odds ratio (OR) 3.5, 95 % confidence interval (CI) 1.0-12.1], and pleural effusion (p = 0.043, OR 5.2, 95 % CI 0.8-30.9). No significant association was detected between the MEFV gene variations and a higher acute phase response. The MEFV gene variations analyzed in our study do not seem to increase the overall susceptibility to SLE and do not have any strong association with its clinical manifestations. The possibility of a modest effect of penetrant exon 10 MEFV variants on the development of serosal effusions needs to be explored in a larger series of patients.

  3. Variants of psychiatric disorders in patients with systemic lupus erythematosus

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    T A Lisitsyna

    2008-01-01

    Full Text Available Objective. To analyze prevalence and structure of psychiatric disorders in pts with systemic lupus erythematosus (SLE examining in the Institute of rheumatology of RAMS. Material and methods. 115 pts with SLE with median age 34 [24; 45] years and median disease duration 8 [4; 17] years were included. SLE activity was assessed with SLEDAI. Psychiatric disorders were diagnosed by a psychiatrist according to ICD-10 using some psychiatric and psychological scales. Results. Psychiatric disorders were revealed in 76 from 115 (66% pts. Anxiety-depressive spectrum disorders prevailed (83%: depressive episode (40%, adjustment disorders (24%, generalized anxiety disorder (10%, dysthymia (9%. Severe cognitive dysfunction was revealed in 7% of pts. Pts with and without psychiatric disorders did not significantly differ in age, sex, duration and activity of the disease, duration of treatment and cumulative dose of prednisolone and cytotoxic drugs. Conclusion. Psychiatric disorders are frequent in pts with SLE (66%. Anxiety-depressive disorders prevail among them (83%. Relationship between SLE and psychiatric disorders requires further examination.

  4. Genital Mycoplasma infection among Mexican women with systemic lupus erythematosus.

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    Méndez-Martínez, Socorro; García-Carrasco, Mario; Cedillo-Ramírez, María L; Mendoza-Pinto, Claudia; Etchegaray-Morales, Ivet; Gil-Juárez, Constantino; Montiel-Jarquín, Álvaro J; Taboada-Cole, Alejandro; Jiménez-Herrera, Erick A; Muñóz-Guarneros, Margarita; Cervera, Ricard

    2017-07-01

    To assess the prevalence of genital Mycoplasma spp. among women with systemic lupus erythematosus (SLE) and to identify factors associated with such infection. A cross-sectional study was conducted among patients with SLE and healthy women who attended a hospital in Puebla, Mexico, between July 29, 2014, and January 4, 2015. All participants were aged 18 years or older and sexually active. A structured interview assessed sociodemographic, obstetric, gynecologic, and clinical characteristics. Disease activity was evaluated using the Mexican SLE Disease Activity Index. Polymerase chain reaction was used to detect the presence of Mycoplasma spp. in genital samples. Ureaplasma urealyticum was the only genital mycoplasma detected; it was present in 32 (24.6%) of 130 patients with SLE and 12 (12.8%) of 94 healthy women. Patients with SLE had increased odds of infection (odds ratio 2.120, 95% confidence interval 1.046-4.296). Among patients with SLE, multiparity was more common in those with U. urealyticum infection (P=0.043). One-quarter of women with SLE had genital infection with U. urealyticum. An association was found between infection and multiparity among women with SLE. © 2017 International Federation of Gynecology and Obstetrics.

  5. Progressive Multifocal Leukoencephalopathy and Systemic Lupus Erythematosus: Focus on Etiology

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    Shala Ghaderi Berntsson

    2016-03-01

    Full Text Available Progressive multifocal leukoencephalopathy (PML caused by reactivation of the JC virus (JCV, a human polyomavirus, occurs in autoimmune disorders, most frequently in systemic lupus erythematosus (SLE. We describe a HIV-negative 34-year-old female with SLE who had been treated with immunosuppressant therapy (IST; steroids and azathioprine since 2004. In 2011, she developed decreased sensation and weakness of the right hand, followed by vertigo and gait instability. The diagnosis of PML was made on the basis of brain MRI findings (posterior fossa lesions and JCV isolation from the cerebrospinal fluid (700 copies/ml. IST was immediately discontinued. Cidofovir, mirtazapine, mefloquine and cycles of cytarabine were sequentially added, but there was progressive deterioration with a fatal outcome 1 year after disease onset. This report discusses current therapeutic choices for PML and the importance of early infection screening when SLE patients present with neurological symptoms. In the light of recent reports of PML in SLE patients treated with rituximab or belimumab, we highlight that other IST may just as well be implicated. We conclude that severe lymphopenia was most likely responsible for JCV reactivation in this patient and discuss how effective management of lymphopenia in SLE and PML therapy remains an unmet need.

  6. Macrophage Polarization Modulates Development of Systemic Lupus Erythematosus

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    Feng Li

    2015-10-01

    Full Text Available Background/Aims: Macrophages have recently been shown to play a critical role in the pathogenesis of systemic lupus erythematosus (SLE. However, the underlying mechanisms remain unclear. Methods: Here, we used an activated lymphocyte-derived DNA (ALD-DNA method to induce SLE in mice. We used a macrophage-specific eliminator clodronate to selectively deplete macrophages in mice. We isolated macrophages from bone marrow of the mice and used cytokines to differentiate M1 and M2 macrophages, respectively. Adoptive transplantation of M1 or M2 macrophages was performed in clodronate-treated mice. The effects on SLE were evaluated by serum anti-dsDNA autoantibody, by renal pathological changes, and by urine protein levels. Results: ALD-DNA induced SLE-like features in mice, manifested by induction of serum anti-dsDNA autoantibody, by renal pathological changes, and by increases in urine protein levels. Clodronate significantly decreased macrophages in mice, which significantly increased SLE severity. Adoptive transplantation of M2, but not M1 macrophages significantly reduced SLE severity in clodronate- and ALD-DNA-treated mice. Conclusion: M1 and M2 macrophages play different roles in development of SLE. M1 macrophages increase the severity of SLE, while M2 macrophages reduce it. Modulation of macrophage polarity may be an attractive therapy for SLE.

  7. Systemic lupus erythematosus and the Crithidia luciliae immunofluorescent test

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    Whitehouse, I.J.; Fehr, K.; Wagenhaeuser, F.J.

    A comparative study of the Crithidia luciliae immunofluorescence (CL-IF) assay and an adapted Farr radioimmunoassay (RIA), for the measurement of antibodies to native deoxyribonucleic acid, was performed using forty-two sera from patients with systematic lupus erythematosus (SLE) and another forty-two from patients with rheumatoid arthritis. Both assays were specific for SLE. The CL-IF assay was statistically significantly more sensitive than the adapted RIA assay. This significant difference was due to greater sensitivity of the CL-IF assay in the cases of sera from patients with SLE of slight activity. Additional advantages of the CL-IF assay were its use to classify the immunoglobulin types of the antibodies (most commonly IgG or IgM) and to measure complement-fixing antibodies to native deoxyribonucleic acid; it affords a simple method of selecting and following SLE patients at risk of developing severe renal disease. These advantages plus the simplicity and inexpensiveness of the CL-IF assay make it a useful tool, especially for use in small laboratories, for the study of antibodies to native deoxyribonucleic acid in patients with SLE.

  8. The Association of the GABRP Polymorphisms with Systemic Lupus Erythematosus

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    Hun-Soo Kim

    2015-01-01

    Full Text Available Gamma-aminobutyric acid receptor subunit pi (GABRP is involved in inhibitory synaptic transmission in the central nervous system. This gene encodes multisubunit chloride channels and is also expressed in numerous nonneuronal tissues such as the uterus and the ovaries. This study was aimed to validate whether the polymorphisms in the GABRP gene are associated with the susceptibility to systematic lupus erythematosus (SLE. The genotype frequencies of the rs929763, rs732157, and rs3805455 of the GABRP gene in SLE patients were significantly different from those of the control group (P<0.0001, P=0.05 and 0.002, resp.. Additional analysis showed that the genotype of the rs929763 and rs3805455 of the GABRP gene were also significantly associated with female SLE patients (P<0.0001, P=0.005, resp.. Two haplotype frequencies including a major haplotype of GABRP SNPs were more significantly different between the SLE patients and the healthy controls (P=0.038 and 4.2E-24, resp.. These results suggest that the polymorphisms in the GABRP gene might be associated with the susceptibility to SLE and the haplotype of GABRP SNPs is useful genetic marker for SLE.

  9. Total Hip Arthroplasty in Systemic Lupus Erythematosus: A Systematic Review

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    John W. Kennedy

    2015-01-01

    Full Text Available The prognosis of systemic lupus erythematosus (SLE has greatly improved in recent years, resulting in an increased number of patients reporting musculoskeletal complications such as osteonecrosis of the femoral head. Total hip arthroplasty (THA can be utilised to alleviate the pain associated with this; however postoperative outcomes in patients with SLE are uncertain. A systematic review of the literature was conducted to identify articles presenting results of THA in SLE, and nine suitable papers were found. All papers were level IV evidence. Pooling the results, a total of 162 patients underwent 214 total hip arthroplasties. Mean follow-up was 72.5 months. The mean Harris Hip Score improved from 45.5 preoperatively to 88.6 and last follow-up. Seventeen percent of patients experienced at least one complication. Superficial wound infection occurred in 3.3%. Revision was required in 2.8% of cases. The mortality rate was 18.5% however no deaths were attributable to undergoing THA. Given the paucity of data present in the literature, more studies are required to adequately assess the postoperative outcomes of THA in patients with SLE, particularly complication rates.

  10. The complement system in systemic lupus erythematosus: an update.

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    Leffler, Jonatan; Bengtsson, Anders A; Blom, Anna M

    2014-09-01

    The complement system plays a major role in the autoimmune disease, systemic lupus erythematosus (SLE). However, the role of complement in SLE is complex since it may both prevent and exacerbate the disease. In this review, we explore the latest findings in complement-focused research in SLE. C1q deficiency is the strongest genetic risk factor for SLE, although such deficiency is very rare. Various recently discovered genetic associations include mutations in the complement receptors 2 and 3 as well as complement inhibitors, the latter related to earlier onset of nephritis. Further, autoantibodies are a distinct feature of SLE that are produced as the result of an adaptive immune response and how complement can affect that response is also being reviewed. SLE generates numerous disease manifestations involving contributions from complement such as glomerulonephritis and the increased risk of thrombosis. Furthermore, since most of the complement system is present in plasma, complement is very accessible and may be suitable as biomarker for diagnosis or monitoring of disease activity. This review highlights the many roles of complement for SLE pathogenesis and how research has progressed during recent years.

  11. Systemic lupus erythematosus activity and beta two microglobulin levels

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    Thelma Larocca Skare

    Full Text Available CONTEXT AND OBJECTIVE: Systemic lupus erythematosus (SLE is an autoimmune disease with a cyclical clinical course. Evaluation of the clinical activity of this disease is important for choosing the correct treatment. The objective of this study was to analyze the value of beta-2 microglobulin (β2M serum levels in determining SLE clinical activity.DESIGN AND SETTING: Cross-sectional analytical study conducted at the rheumatology outpatient clinic of a private university hospital.METHODS: 129 SLE patients were studied regarding disease activity using SLEDAI (SLE Disease Activity Index and cumulative damage using SLICC ACR (SLE International Collaborating Clinics/American College of Rheumatology Damage Index for SLE. At the same time, the β2M serum level, ESR (erythrocyte sedimentation rate, anti-dsDNA (anti-double-stranded DNA and C3 and C4 complement fractions were determined.RESULTS: β2M levels correlated positively with SLEDAI (P = 0.02 and ESR (P = 0.0009 and negatively with C3 (P = 0.007. Patients who were positive for anti-dsDNA had higher β2M serum levels (P = 0.009.CONCLUSION: β2M levels are elevated in SLE patients with active disease.

  12. Prevalence of human herpesvirus 8 infection in systemic lupus erythematosus

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    Sun Shipeng

    2011-05-01

    Full Text Available Abstract Background For decades, scientists have tried to understand the environmental factors involved in the development of systemic lupus erythematosus (SLE, in which viral infections was included. Previous studies have identified Epstein-Barr virus (EBV to incite SLE. Human herpesvirus 8 (HHV-8, another member of the gammaherpesvirus family, shares a lot in common with EBV. The characteristics of HHV-8 make it a well-suited candidate to trigger SLE. Results In the present study, serum samples from patients (n = 108 with diagnosed SLE and matched controls (n = 122 were collected, and the prevalence of HHV-8 was compared by a virus-specific nested PCR and a whole virus enzyme-linked immunoassay (EIA. There was significant difference in the prevalence of HHV-8 DNA between SLE patients and healthy controls (11 of 107 vs 1 of 122, p = 0.001; significant difference was also found in the detection of HHV-8 antibodies (19 of 107 vs 2 of 122, p We also detected the antibodies to Epstein-Barr virus viral capsid antigen (EBV-VCA and Epstein-Barr nuclear antigen-1 (EBNA-1. Both patients and controls showed high seroprevalence with no significant difference (106 of 107 vs 119 of 122, p = 0.625. Conclusion Our finding indicated that there might be an association between HHV-8 and the development of SLE.

  13. Healthcare Utilization and Costs of Systemic Lupus Erythematosus in Medicaid

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    Hong J. Kan

    2013-01-01

    Full Text Available Objective. Healthcare utilization and costs associated with systemic lupus erythematosus (SLE in a US Medicaid population were examined. Methods. Patients ≥ 18 years old with SLE diagnosis (ICD-9-CM 710.0x were extracted from a large Medicaid database 2002–2009. Index date was date of the first SLE diagnosis. Patients with and without SLE were matched. All patients had a variable length of followup with a minimum of 12 months. Annualized healthcare utilization and costs associated with SLE and costs of SLE flares were assessed during the followup period. Multivariate regressions were conducted to estimate incremental healthcare utilization and costs associated with SLE. Results. A total of 14,777 SLE patients met the study criteria, and 14,262 were matched to non-SLE patients. SLE patients had significantly higher healthcare utilization per year than their matched controls. The estimated incremental annual cost associated with SLE was $10,984, with the highest increase in inpatient costs (P<0.001. Cost per flare was $11,716 for severe flares, $562 for moderate flares, and $129 for mild flares. Annual total costs for patients with severe flares were $49,754. Conclusions. SLE patients had significantly higher healthcare resource utilization and costs than non-SLE patients. Patients with severe flares had the highest costs.

  14. Epileptic syndrome in systemic lupus erythematosus and neuronal autoantibody associations.

    Science.gov (United States)

    Kampylafka, E I; Alexopoulos, H; Fouka, P; Moutsopoulos, H M; Dalakas, M C; Tzioufas, A G

    2016-10-01

    We investigated systemic lupus erythematosus (SLE) patients with epilepsy, a major and organic neurological symptom. Our aim was to test patients for the autoimmune epilepsy-associated antibodies anti-GAD, anti-NMDAR, anti-AMPAR1/2, anti-GABABR and anti-VGKC. We tested sera from ten SLE patients with current or previous episodes of epileptic seizures. In addition, sera were tested for staining on primary hippocampal neurons. The patients' clinical and neuroimaging profile, disease activity and accumulated damage scores and therapeutic regimens administered were recorded, and correlations were evaluated. Patients were negative for all anti-neuronal autoantibodies tested, and showed no staining on primary hippocampal cells, which suggests the absence of autoantibodies against neuronal cell surface antigens. Epileptic seizures were all tonic-clonic, and all patients had high disease activity (mean SLE Damage Acticity Index score 19.3 ± 7.3). Six patients had minor or no brain magnetic resonance imaging findings, and three had major findings. 9/10 patients received immunosuppression for 5 ± 4 months, while anti-convulsive treatment was administered to all patients (4.2 ± 3 years). Our results suggest that the majority of SLE-related epileptic seizures cannot be attributed to the action of a single antibody against neuronal antigens. Studies with larger neuropsychiatric SLE populations and stricter inclusion criteria are necessary to verify these findings. © The Author(s) 2016.

  15. Systemic lupus erythematosus and pemphigus vulgaris: association or coincidence.

    Science.gov (United States)

    Calebotta, A; Cirocco, A; Giansante, E; Reyes, O

    2004-01-01

    Few cases have been published relating systemic lupus erythematosus (SLE) and pemphigus vulgaris (PV). We describe a patient with this association. A 35-year old woman who started to develop persistent pain and morning stiffness of proximal inter and metacarpo-phalangeal joints. During the following year, the patient recalled the onset of blisters on both legs, face, arms and thorax, as well as erosions appearing on oral mucous membranes. We observed generalized multiple erosions on her trunk and legs, flaccid bullae located on her right thigh and multiple erosions on oral mucous membranes. A skin biopsy reported PV Direct immunofluorescence on the perilesional skin specimen, showed beehive intercellular IgG deposits in the epidermis (+++), suggesting PV; granular discontinuous IgM and C3 deposits in the dermal-epidermal union (+++), suggesting SLE. Direct immunofluorescence of the healthy unexposed skin specimen, reported granular discontinuous IgG deposits in the dermal-epidermal union and beehive intercellular IgG deposits in the lower levels of the epidermis (+++); granular continuous IgM deposits in the dermal-epidermal union (+++). The results of rheumatic studies were obtained as follows: ANA :3 +, Anti-DNA, Anti-Sm, Anti-Ro and Anti-La :4 + . The definite diagnosis was PVand SLE. Treatment with 50 mg of prednisone daily with good evolution.

  16. A Case of Systemic Lupus Erythematosus Confused with Infective Endocarditis

    Directory of Open Access Journals (Sweden)

    Sibel Serin

    2014-09-01

    Full Text Available Systemic lupus erythematosus (SLE is a multisystemic autoimmune disease resulting from immune system-mediated tissue damage. Clinical findings of SLE can involve skin, kidney, central nervous system, cardiovascular system, serosal membranes, and the hematologic and immune systems. In the differential diagnosis, other connective tissue diseases, infective endocarditis, infections such as viral hepatitis, endocrine disorders such as hypothyroidism, sarcoidosis, and some malignant tumors should be considered. Infective endocarditis can imitate all the symptoms of SLE depending on immune complex accumulation glomerulonephritis. Hemolytic anemia, skin lesions, arthralgia, arthritis, decreased complement levels, and autoantibody positivity, including antinuclear autoantibody (ANA, positivity can be seen. Therefore, high fever, blood cultures, eye examination, and echocardiographic findings are of particular value. Here, we present a case of SLE that was confused with infective endocarditis (IE due to the presence of high fever associated with autoimmune hemolytic anemia (AHA and proteinuria as well as increased erythrocyte sedimentation rate (ESR, cardiac murmur, and Roth’s spots. (The Me­di­cal Bul­le­tin of Ha­se­ki 2014; 52: 212-15

  17. Alveolar hemorrhage in systemic lupus erythematosus: a cohort review.

    Science.gov (United States)

    Andrade, C; Mendonça, T; Farinha, F; Correia, J; Marinho, A; Almeida, I; Vasconcelos, C

    2016-01-01

    Diffuse alveolar hemorrhage (DAH) is a rare but potentially catastrophic manifestation with a high mortality. Among rheumatologic diseases, it occurs most frequently in patients with systemic lupus erythematosus (SLE) and systemic vasculitis. Despite new diagnostic tools and therapies, it remains a diagnostic and therapeutic challenge. The aim of this work was to characterize the SLE patients with an episode of alveolar hemorrhage followed in our Clinical Immunology Unit (CIU). A retrospective chart review was carried out for all patients with SLE followed in CIU between 1984 and the end of 2013. We reviewed the following data: demographic characteristics, clinical and laboratory data, radiologic investigations, histologic studies, treatment, and outcome. We identified 10 episodes of DAH, corresponding to seven patients, all female. These represent 1.6% of SLE patients followed in our Unit. The age at DAH attack was 42.75 ± 18.9 years. The average time between diagnosis of SLE and the onset of DAH was 7.1 years. Three patients had the diagnosis of SLE and the DAH attack at the same time. Disease activity according to SLEDAI was high, ranging from 15 to 41. All patients were treated with methylprednisolone, 37.5% cyclophosphamide and 28.6% plasmapheresis. The overall mortality rate was 28.6%.

  18. T Cell Transcriptomes Describe Patient Subtypes in Systemic Lupus Erythematosus.

    Directory of Open Access Journals (Sweden)

    Sean J Bradley

    Full Text Available T cells regulate the adaptive immune response and have altered function in autoimmunity. Systemic Lupus Erythematosus (SLE has great diversity of presentation and treatment response. Peripheral blood component gene expression affords an efficient platform to investigate SLE immune dysfunction and help guide diagnostic biomarker development for patient stratification.Gene expression in peripheral blood T cell samples for 14 SLE patients and 4 controls was analyzed by high depth sequencing. Unbiased clustering of genes and samples revealed novel patterns related to disease etiology. Functional annotation of these genes highlights pathways and protein domains involved in SLE manifestation.We found transcripts for hundreds of genes consistently altered in SLE T cell samples, for which DAVID analysis highlights induction of pathways related to mitochondria, nucleotide metabolism and DNA replication. Fewer genes had reduced mRNA expression, and these were linked to signaling, splicing and transcriptional activity. Gene signatures associated with the presence of dsDNA antibodies, low complement levels and nephritis were detected. T cell gene expression also indicates the presence of several patient subtypes, such as having only a minimal expression phenotype, male type, or severe with or without induction of genes related to membrane protein production.Unbiased transcriptome analysis of a peripheral blood component provides insight on autoimmune pathophysiology and patient variability. We present an open source workflow and richly annotated dataset to support investigation of T cell biology, develop biomarkers for patient stratification and perhaps help indicate a source of SLE immune dysfunction.

  19. [Rare cutaneous manifestaions of lupus erythematosus. A clinical overview].

    Science.gov (United States)

    Kuhn, A; Schuppe, H C; Ruzicka, T; Lehmann, P

    2000-11-01

    Lupus erythematosus (LE) is a disease with a wide spectrum of cutaneous and systemic manifestations and has been the subject of many studies over several decades. Clinical features of patients with LE show a great variation, and for this reason it is difficult to develop a unifying concept of this disease. Consequently, this has led to the identification of subsets which have been defined by constellations of clinical and photobiological features, histological changes as well as laboratory abnormalities. Besides the characteristic classical forms such as systemic LE (SLE), subacute cutaneous LE (SCLE), and discoid LE (DLE), there are uncommon variants of LE which often lead to diagnostic difficulties. Bullous LE (BLE) and urticarial vasculitis are listed as characteristic but non-specific manifestations of systemic LE. LE tumidus (LET), LE hypertrophic/verrucous (LEHV), chilblain LE, and LE profundus (LEP) are uncommon subtypes of chronic cutaneous LE. Annular erythema and papulonodular mucinosis are further uncommon cutaneous manifestations of LE. This clinical review summarizes the typical features of the uncommon forms of LE in order to improve clinical diagnostic precision and to achieve a better differentiation of the subtypes.

  20. Cardiac autonomic profile in rheumatoid arthritis and systemic lupus erythematosus.

    Science.gov (United States)

    Aydemir, M; Yazisiz, V; Basarici, I; Avci, A B; Erbasan, F; Belgi, A; Terzioglu, E

    2010-03-01

    Neurological involvement is a well-documented issue in patients with systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). However, little is known about the involvement of the autonomic nervous system. This study was conducted to investigate autonomic nervous system dysfunction in patients with RA and SLE. Twenty-six RA patients, 38 SLE patients and 40 healthy controls were recruited from our in- and out-patient departments. Heart rate variability (HRV) parameters (the power of the high- [HF] and low-frequency [LF] band of haemodynamic time series, the ratio between low- and high-frequency components [LF/HF ratio], the power spectral density), baroreflex sensitivity (BRS) and beat-to-beat blood pressures were assessed by a novel non-invasive haemodynamic monitoring tool (Task Force Monitor [TFM], CNSystems Medizintechnik GmbH, Graz, Austria). Autonomic nervous system dysfunction was determined according to classical Ewing autonomic test battery. Furthermore, we implemented a secondary autonomic test score by modifying the Ewing test battery with additional criteria. Both the classical and modified Ewing test batteries have revealed that the frequencies of autonomic neuropathy were significantly higher in patient groups compared with controls (p disease duration, disease activity and autoantibody positivity. Consequently, we believe that further large-scale studies investigating cardiovascular autonomic neuropathy in rheumatic diseases should be carried out to verify our findings and manifest clinical consequences beyond these results.

  1. Cardiac tamponade as an initial presentation for systemic lupus erythematosus.

    Science.gov (United States)

    Li, William; Frohwein, Thomas; Ong, Kenneth

    2017-08-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease which follows a relapsing and remitting course that can manifest in any organ system. While classic manifestations consist of arthralgia, myalgia, frank arthritis, a malar rash and renal failure to name a few, cardiac tamponade, however, is a far less common and far more dangerous presentation. We highlight the case of a 61year-old male with complaints of acute onset shortness of breath and generalized body aches associated with a fever and chills in the ER. A bedside echocardiogram revealed a significant pericardial effusion concerning for pericardial tamponade. An emergent pericardiocentesis performed drained 800mL of serosanguinous fluid. While denying a history of any rash, photosensitivity, oral ulcers, or seizures, his physical examination did reveal metacarpal phalangeal joint swelling along with noted pulsus paradoxus of 15-200mmHg. Subsequent lab work revealed ANA titer of 1:630 and anti-DS DNA antibody level of 256IU/mL consistent with SLE. This case highlights cardiac tamponade as a rare but life-threatening presentation for SLE and raises the need to keep it in the differential when assessing patients presenting with pertinent exam findings. Published by Elsevier Inc.

  2. Nonenzymatic antioxidants in saliva of patients with systemic lupus erythematosus.

    Science.gov (United States)

    Moori, M; Ghafoori, H; Sariri, R

    2016-03-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by autoantibody-directed self-antigens, immune complex formation and immune deregulation, resulting in damage to essentially all the organs. SLE is associated with the increased production of free radicals. Increase in free radicals or impaired antioxidant defense system in SLE causes oxidative stress. Considering that saliva could be a reflection of the state of health, the purpose of this study was to evaluate some antioxidants in the saliva and serum of patients with SLE and compare these with healthy individuals. This could help us in obtaining a possible marker in saliva in the future. During the course of the practical part of the project, 30 patients with SLE and 30 healthy controls were investigated. After centrifugation of un-stimulated saliva and blood samples, they were examined using spectrophotometric methods and the results were analyzed by statistical software. According to the results, concentrations of malondialdehyde, uric acid and total antioxidants were significantly increased but the level of reduced glutathion was reduced significantly in the saliva and serum of SLE patients as compared to controls. It is therefore suggested that antioxidant power is impaired in saliva and serum of SLE patients. As there was a positive correlation between the antioxidant level of saliva and blood serum, the antioxidant status of saliva could be an indicator of serum antioxidants. © The Author(s) 2015.

  3. Tolerogenic probiotics: potential immunoregulators in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Esmaeili, Seyed-Alireza; Mahmoudi, Mahmoud; Momtazi, Amir Abbas; Sahebkar, Amirhossein; Doulabi, Hassan; Rastin, Maryam

    2017-08-01

    Probiotics are commensal or nonpathogenic microbes that colonize the gastrointestinal tract and confer beneficial effects on the host through several mechanisms such as competitive exclusion, anti-bacterial effects, and modulation of immune responses. There is growing evidence supporting the immunomodulatory ability of some probiotics. Several experimental and clinical studies have been shown beneficial effect of some probiotic bacteria, particularly Lactobacillus and Bifidobacteria strains, on inflammatory and autoimmune diseases. Systemic lupus erythematosus (SLE) is an autoimmune disease that is mainly characterized by immune intolerance towards self-antigens. Some immunomodulatory probiotics have been found to regulate immune responses via tolerogenic mechanisms. Dendritic and T regulatory (Treg) cells, IL-6, IFN-γ, IL-17, and IL-23 can be considered as the most determinant dysregulated mediators in tolerogenic status. As demonstrated by documented experimental and clinical trials on inflammatory and autoimmune diseases, a number of probiotic bacterial strains can restore tolerance in host through modification of such dysregulated mediators. Since there are limited reports regarding to impact of probiotic supplementation in SLE patients, the preset review was aimed to suggest a number of probiotics bacteria, mainly from Bifidobacteria and Lactobacillus strains that are able to ameliorate immune responses. The aim was followed through literature survey on immunoregulatory probiotics that can restore tolerance and also modulate the important dysregulated pro/anti-inflammatory cytokines contributing to the pathogenesis of SLE. © 2016 Wiley Periodicals, Inc.

  4. Alternations of salivary antioxidant enzymes in systemic lupus erythematosus.

    Science.gov (United States)

    Zaieni, S H; Derakhshan, Z; Sariri, R

    2015-11-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with chronic systemic inflammation. Oxidative stress may play a role in the pathogenesis of SLE. An increase in free radicals or an impaired antioxidant defense system in SLE causes oxidative stress. Therefore, oxidative damage plays an important role in the pathogenesis of SLE. Variations in antioxidant activity have been previously studied in serum of patients with this disease. However, salivary factors have not been evaluated. Considering that saliva, the noninvasive biological fluid, could be a reflection of the state of health, the purpose of this study was evaluation of peroxidase (POD), superoxide dismutase (SOD) and catalase (CAT) activity in the saliva of patients with SLE. During the course of the practical part of the project, 30 patients with SLE and 30 healthy controls were selected to donate their saliva samples. After centrifugation of un-stimulated saliva, biological activity of POD, CAT and SOD were evaluated on their appropriate substrates using spectrophotometric methods and the results were statistically analyzed. The results showed that activities of antioxidant enzymes SOD and CAT were significantly reduced in saliva of SLE patients as compared to controls. The results suggest that antioxidant status was impaired in the saliva of SLE patients, and antioxidant status of saliva could be one of the non-invasive markers for SLE.

  5. Senescent profile of angiogenic T cells from systemic lupus erythematosus patients.

    Science.gov (United States)

    López, Patricia; Rodríguez-Carrio, Javier; Martínez-Zapico, Aleida; Caminal-Montero, Luis; Suarez, Ana

    2016-03-01

    The chronic inflammatory environment associated with systemic lupus erythematosus can lead to an accelerated immunosenescence responsible for the endothelial damage and increased cardiovascular risk observed in these patients. The present study analyzed two populations with opposite effects on vascular endothelium, angiogenic T cells and the senescent CD4(+)CD28(null) subset, in 84 systemic lupus erythematosus patients and 46 healthy controls. Also, 48 rheumatoid arthritis patients and 72 individuals with traditional cardiovascular risk factors participated as disease controls. Phenotypic characterization of CD28(+) and CD28(null) cells was performed by analyzing markers of senescence (CCR7, CD27, CD57) and cytotoxicity (CD56, perforin, granzyme B, IFN-γ). IL-1β, IL-6, IL-8, IL-10, IL-12, IL-17A, IFN-α, IFN-γ, TNF-α, B lymphocyte stimulator, and GM-CSF serum levels were analyzed in systemic lupus erythematosus patients and healthy controls. CD4(+)CD28(null) cells were notably increased in the systemic lupus erythematosus patients and disease controls compared with healthy controls. In contrast, angiogenic T cells were only reduced in the disease controls (those with rheumatoid arthritis or traditional cardiovascular risk factors). Nevertheless, an anomalous presence of CD28(null)-angiogenic T cells, with cytotoxic and senescent characteristics, was noted in systemic lupus erythematosus patients in association with anti-dsDNA titer, anti-SSA/Ro antibodies and circulating TNF-α, IL-8, IFN-α, and B lymphocyte stimulator amounts. This subset was also detected in those with traditional cardiovascular risk factors but not in the rheumatoid arthritis patients. In contrast, CD28(+)-angiogenic T cells were reduced in the systemic lupus erythematosus patients with cardiovascular disorders. In conclusion, CD28 expression must be used to redefine the angiogenic T cell population, because in pathologic conditions, a senescent CD28(null)-angiogenic T cell subset with

  6. Lupus erythematosus panniculitis presenting with initial periorbital swelling and positive antiphospholipid antibodies: A rare association

    Directory of Open Access Journals (Sweden)

    Jyotsna Oak

    2013-01-01

    Full Text Available Lupus erythematosus panniculitis (LEP, an unusual form of chronic cutaneous lupus erythematosus, is characterized by chronic inflammation and fibrosis of subcutaneous tissue. Clinically, it presents as subcutaneous nodules on common locations such as forehead, cheeks, proximal limbs and buttocks. Ulceration of the nodules may occur in certain cases. Very few case studies have reported the occurrence of early solitary periorbital involvement, highlighting the need for a high index of suspicion in such cases. We report here a case having generalized extensive LEP with initial manifestation of a solitary periorbital swelling, autoimmune hemolytic anemia, and associated antiphospholipid antibodies positivity.

  7. Mortality and causes of death of 513 Danish patients with systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Jacobsen, Søren; Petersen, Jørgen; Ullman, S;

    1999-01-01

    A multicentre cohort of 513 clinic attenders with systemic lupus erythematosus (SLE) was retrospectively identified, representing 4185 patient-years of follow-up. Expected numbers of death were calculated by means of age- and sex-specific mortality rates of the general Danish population. The obse......A multicentre cohort of 513 clinic attenders with systemic lupus erythematosus (SLE) was retrospectively identified, representing 4185 patient-years of follow-up. Expected numbers of death were calculated by means of age- and sex-specific mortality rates of the general Danish population...

  8. [The involvement of pulmonary interstitial tissue in multisystemic lupus erythematosus: interdisciplinarity and role of the pneumologists].

    Science.gov (United States)

    Salvati, F

    2015-01-01

    The Author remarks the interstitial lung involvement in systemic lupus erythematosus. This secondary respiratory manifestation is infrequent as well as the consequent pulmonary hypertension making it possible to miss or delay the diagnosis. Therefore the interdisciplinary evaluation of the multisystemic disease lupus erythematosus needs. In this context the role of the pneumologists is relevant for the global treatment of the patients with LES in particular as concerns the early detection of the clinical and functional respiratory symptoms as well as the appropriate treatment plan within their specialistic competence.

  9. A Rare Case of Juvenile Systemic Lupus Erythematosus with Disseminated Histoplasmosis

    Science.gov (United States)

    Sarkar, Piyabi; Basu, Keya; Mallick (Sinha), Mamata Guha

    2016-01-01

    Histoplasmosis is a systemic fungal infection caused by dimorphic fungus, Histoplasma capsulatum. Immunocompetent individuals usually have self-limiting or localized disease whereas immunocompromised individuals develop disseminated disease. The occurrence of progressive disseminated histoplasmosis in juvenile systemic lupus erythematosus is extremely rare with only one reported case in literature showing such association. Therefore, we report a case of severe opportunistic fungal infection caused by Histoplasma in a 13-year-old girl who was diagnosed with juvenile lupus erythematosus, subsequently developed septic shock and died of the disease despite of aggressive antifungal therapy. PMID:27904204

  10. Ticlopidine-induced subacute cutaneous lupus erythematosus: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Yi-Ching Chen

    2014-09-01

    Full Text Available Many drugs have been reported to induce lupus in a minority of patients. Ticlopidine hydrochloride inhibits platelet aggregation and is widely used for the prevention of thrombosis. There have been only a few reports of ticlopidine-induced lupus. Here, we review 13 previously reported cases and describe the case of a 71-year-old man with ticlopidine-induced subacute cutaneous lupus erythematosus. His diagnosis was supported by the appearance of papulosquamous skin lesions on sun-exposed areas and detectable anti-Ro/SS-A antibodies, shortly after drug initiation as well as the gradual resolution of these symptoms after the discontinuation of ticlopidine. Our case highlights that when a patient presents with subacute cutaneous lupus erythematosus-like skin lesions, ticlopidine should be considered as a potential causative agent.

  11. Management of autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus.

    Science.gov (United States)

    Newman, Kam A; Akhtari, Mojtaba

    2011-05-01

    Autoimmune neutropenia, caused by neutrophil-specific autoantibodies is a common phenomenon in autoimmune disorders such as Felty's syndrome and systemic lupus erythematosus. Felty's syndrome is associated with neutropenia and splenomegaly in seropositive rheumatoid arthritis which can be severe and with recurrent bacterial infections. Neutropenia is also common in systemic lupus erythematosus and it is included in the current systemic lupus classification criteria. The pathobiology of the autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus is complex, and it could be a major cause of morbidity and mortality due to increased risk of sepsis. Treatment should be individualized on the basis of patient's clinical situation, and prevention or treatment of the infection. Recombinant human granulocyte colony-stimulating factor is a safe and effective therapeutic modality in management of autoimmune neutropenia associated with Felty's syndrome and systemic lupus erythematosus, which stimulates neutrophil production. There is a slight increased risk of exacerbation of the underlying autoimmune disorder, and recombinant human granulocyte colony-stimulating factor dose and frequency should be adjusted at the lowest effective dose.

  12. Polarization of TH2 response is decreased during pregnancy in systemic lupus erythematosus.

    Science.gov (United States)

    Iaccarino, L; Ghirardello, A; Zen, M; Villalta, D; Tincani, A; Punzi, L; Doria, A

    2012-12-11

    This study evaluated some cytokines involved in the Th1-Th2 shift during pregnancy in patients with systemic lupus erythematosus (SLE) and healthy women. Twenty-seven consecutive successful pregnancies in 26 SLE patients and 28 pregnancies in 28 matched healthy subjects, as controls, were enrolled and prospectively studied. Sera obtained at first and third trimesters of pregnancy were tested for IL-1α, IL-1β, IL-2, IL-6, IL-8, IL-10, IL-12p70, INF-γ, and TNF-α with a highly sensitive, multiplexed sandwich ELISA (SearchLight Human Inflammatory Cytokine Array). Statistics were performed by SPSS package. IL-8 serum levels were higher in the first (Ppregnancy in SLE patients compared with controls, INF-γ serum levels in the third trimester (P=0.009), and IL-10 serum levels in the first and third trimesters (P=0.055 and Ppregnancy. Cytokine profile was similar in patients with and without lupus nephritis both in the first and in the third trimesters of pregnancy. IL-8 serum levels were lower in patients with a previous diagnosis of antiphospholipid antibody syndrome compared with those without, both in the first and in the third trimesters of pregnancy. In SLE patients, a lower than expected decrease in Th1 cytokine serum levels was observed in the third trimester of gestation which could contribute to a lower Th2 cytokine polarization during pregnancy.

  13. Different end-points to assess effects in systemic lupus erythematosus patients exposed to pesticide mixtures.

    Science.gov (United States)

    Simoniello, M F; Contini, L; Benavente, E; Mastandrea, C; Roverano, S; Paira, S

    2017-02-01

    Systemic Lupus Erythematosus (SLE) is an autoimmune disease with high female predominance in reproductive years. It is characterized by a pronounced inflammation and production of a variety of autoantibodies. SLE pathogenesis is influenced by genes, hormones and environmental agents. The aim of this study was assess the possible effect of environmental pesticide mixtures in SLE patients. Oxidative DNA damage was measured using the comet assay modified by enzyme Endo III for detection of oxidized bases (Endo Sites), and oxidative stress by the measurement of the activity of catalase (CAT), superoxide dismutase (SOD) and lipid peroxidation (TBARS). Eighty-nine patients with diagnosis of SLE were included, 46% of them came from areas highly sprayed with pesticides and were compared with patients from urban areas with the same clinical and socio-demographic characteristics (p≥0.155). In order to identify factors that could predict DNA damage and oxidative stress, a binary logistic regression model with independent variables was developed: place of residence (p=0.007) have 75% of positive predictive value while smoking habit (p=0.186) have a 56% negative predictive value. The Odd Ratio (OR) obtained indicate that lupus patients living in rural areas presented 3.52 times more oxidative DNA damage compared to those living in the city. The prospects of applying biomarkers to assess exposure and biological effects, such as DNA damage and oxidative stress in autoimmune diseases, allow improving the characterization of individual risk. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  14. Complement-fixing properties of antinuclear antibodies distinguish drug-induced lupus from systemic lupus erythematosus.

    Science.gov (United States)

    Rubin, R L; Teodorescu, M; Beutner, E H; Plunkett, R W

    2004-01-01

    The immunofluorescence antinuclear antibody (ANA) test has been widely used to monitor autoimmune disease, but its value for diagnostic purposes is compromised by low specificity and high prevalence in disease-free individuals. The capacity of autoantibodies to fix serum complement proteins when bound to antigen is an important effector function because this property is associated with acute and chronic inflammatory processes. The current study evaluates the complement-fixing properties of antinuclear antibodies (CANA) in three well-defined and clinically-related patient groups: systemic lupus erythematosus (SLE), drug-induced lupus (DIL) and drug-induced autoimmunity (DIA). Of 20 patients diagnosed with SLE, 90% displayed complement-fixing ANA while this feature was present in only two of 18 patients with DIL and no patients with DIA without associated disease even though the mean ANA titres were similar among these patient groups. CANA was significantly correlated with anti-Sm activity. Because SLE but not DIL or DIA can be a life-threatening disease associated with complement consumption in vivo, these results demonstrate that measurement of CANA is a diagnostically useful tool and may have immunopathologic implications.

  15. A patient with systemic lupus erythematosus and lupus nephritis: A 12-year follow-up

    Directory of Open Access Journals (Sweden)

    Jovanović Nataša

    2011-01-01

    Full Text Available Introduction. Systemic lupus erythematosus (SLE is a chronic immunological disease causing a significant morbidity and mortality in younger women and involving several organs and systems, most often the kidneys, being consequently the incidence of lupus nephritis (LN about 60%. Case report. We reported a 57 year-old patient with the diagnosed SLE in 1995. Pathohistological analysis of kidney biopsy revealed LN type V. The patient was treated with corticosteroid pulses and azathioprine during one year. A remission was achieved and maintained with prednisone, 15 mg daily. Nephrotic relapse was diagnosed in 2006 and the second kidney biopsy revealed recent kidney infarction due to extensive vasculitis. Soon, a cerebrovascul insult developed and CT-scan revealed endocranial infarctus. The patient was treated with corticosteroids and cyclophosphamide pulses (totally VI monthly pulses, and also with low-molecular heparine, anticoagulants and salicylates because of the right leg phlebothrombosis. After the pulses, the patient was adviced to take prednisone 20 mg daily and azothioprine 100 mg daily, and 6 months later mycophenolate mofetil because of persistent active serological immunological findings (ANA 1 : 320 and nephrotic syndrome. Mycophenolate mofetil was efficient in inducing and maintaining remission of nephrotic syndrome. Conclusion. The aim of LN treatment is to achieve and maintain remission, improve patients’ outcome, reduce the toxicity of immunosuppressive drugs and the incidence of relapses. Mycophenolate mofetil was shown to be efficient in inducing and maintaining remission of nephrotic syndrome in the frame of LN.

  16. Interleukin-27 and interleukin-23 in patients with systemic lupus erythematosus: possible role in lupus nephritis.

    Science.gov (United States)

    Xia, L P; Li, B F; Shen, H; Lu, J

    2015-05-01

    To analyse the concentration of interleukin (IL)-27 and IL-23 in serum and urine of patients with systemic lupus erythematosus (SLE) compared with healthy controls (HC). An enzyme-linked immunosorbent assay (ELISA) was used to analyse the serum and urine concentration of IL-27 and IL-23 from 50 patients with lupus nephritis (LN), 55 patients without LN, and 30 HC. The correlations between the levels of IL-27, IL-23, and disease activity, clinical parameters in SLE patients were analysed. The levels of IL-27 and IL-23 increased significantly in the serum and urine of SLE patients with and without LN compared with HC. Moreover, urine levels of IL-27 and IL-23 were correlated with the renal SLE Disease Activity Index (rSLEDAI) score and 24-h urinary protein levels. After 6 months of immunosuppressive treatment, urine IL-27 expression rose significantly in SLE patients with LN. IL-27 and IL-23 may be involved in the pathogenesis of LN.

  17. MRI changes in the central nervous system in a child with lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Gieron, M.A. [Dept. of Pediatrics, Univ. of South Florida, Coll. of Medicine, Tampa, FL (United States); Khoromi, S. [Dept. of Neurology, Univ. of South Florida, Coll. of Medicine, Tampa, FL (United States); Campos, A. [Dept. of Pediatrics, Univ. of South Florida, Coll. of Medicine, Tampa, FL (United States)

    1995-05-01

    We report on a 10-year-old girl with systemic lupus erythematosus who presented in status epilepticus as the only manifestation of central nervous system involvement. MRI of the brain showed diffuse gray and white matter lesions which almost completely resolved after treatment with methylprednisolone. MRI findings in this child are similar to those in adults with diffuse clinical manifestations. The study is essential in the initial evaluation of patients suspected of central nervous system lupus. (orig.)

  18. Netting Neutrophils Are Major Inducers of Type I IFN Production in Pediatric Systemic Lupus Erythematosus

    OpenAIRE

    Garcia-Romo, Gina S; Caielli, Simone; Vega, Barbara; Connolly, John; Allantaz, Florence; Xu, Zhaohui; PUNARO, MARILYNN; Baisch, Jeanine; Guiducci, Cristiana; Coffman, Robert L.; Barrat, Franck J.; Banchereau, Jacques; Pascual, Virginia

    2011-01-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by a breakdown of tolerance to nuclear antigens and the development of immune complexes. Genomic approaches have shown that human SLE leukocytes homogeneously express type I interferon (IFN)–induced and neutrophil-related transcripts. Increased production and/or bioavailability of IFN-α and associated alterations in dendritic cell (DC) homeostasis have been linked to lupus pathogenesis. Although neutrophils have...

  19. Appearance of Systemic Lupus Erythematosus in Patients with Myasthenia Gravis following Thymectomy: Two Case Reports

    OpenAIRE

    2004-01-01

    We report two cases of systemic lupus erythematosus (SLE) in myasthenia gravis (MG) patients who had undergone thymectomy. SLE developed in the patients 3 months or 13 yr after thymectomy, and polyarthritis was the main clinical manifestation of SLE. Both patients fulfilled at least four of the revised criteria for the classification of SLE. In this report, we describe two postthymectomy lupus patients and perform a comparative review of previous cases.

  20. Appearance of systemic lupus erythematosus in patients with myasthenia gravis following thymectomy: two case reports.

    Science.gov (United States)

    Park, Mi Jeong; Kim, Yun A; Lee, Shin Seok; Kim, Byeong Chae; Kim, Myeong Kyu; Cho, Ki Hyun

    2004-02-01

    We report two cases of systemic lupus erythematosus (SLE) in myasthenia gravis (MG) patients who had undergone thymectomy. SLE developed in the patients 3 months or 13 yr after thymectomy, and polyarthritis was the main clinical manifestation of SLE. Both patients fulfilled at least four of the revised criteria for the classification of SLE. In this report, we describe two postthymectomy lupus patients and perform a comparative review of previous cases.

  1. Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

    OpenAIRE

    Tselios K; Gladman DD; Urowitz MB

    2016-01-01

    Konstantinos Tselios, Dafna D Gladman, Murray B Urowitz, University of Toronto Lupus Clinic, Centre for Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, ON, Canada Abstract: Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (&...

  2. Challenge of liver disease in systemic lupus erythematosus: Clues for diagnosis and hints for pathogenesis

    OpenAIRE

    Bessone, Fernando; Poles, Natalia; Roma, Marcelo Gabriel

    2016-01-01

    Systemic lupus erythematosus (SLE) encompass a broad spectrum of liver diseases. We propose here to classify them as follows: (1) immunological comorbilities (overlap syndromes); (2) non-immunological comorbilities associated to SLE; and (3) a putative liver damage induced by SLE itself, referred to as “lupus hepatitis”. In the first group, liver injury can be ascribed to overlapping hepatopathies triggered by autoimmune mechanisms other than SLE occurring with higher incidence in the context...

  3. Stroke of the substance nigra and parkinsonism as first manifestation of systemic lupus erythematosus.

    Science.gov (United States)

    Orta Daniel, San-Juan; Ulises, Rodríguez-Ortiz

    2008-01-01

    The authors report one case of parkinsonism associated with systemic lupus erythematosus (SLE): a 45-year-old woman, who presented with a strategic infarct of the right substance nigra secondary to vasculitis, and contralateral symptoms. Other causes of parkinsonism were excluded. She had a partial response to therapy with levodopa. Parkinsonism as the first manifestation of lupus is extremely rare. This is the first reported case with strategic infarction in the substance nigra secondary to vasculitis in a patient with SLE.

  4. Gastrointestinal involvement in systemic lupus erythematosus:Insight into pathogenesis, diagnosis and treatment

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by the presence of a plethora of autoantibodies and immune complex formation. Virtually every system and organ can be affected by SLE. Gastrointestinal symptoms are com-mon in SLE patients, and more than half of them are caused by adverse reactions to medications and viral or bacterial infections. Though not as common as lu-pus nephritis, SLE-related gastrointestinal involvement is clinically important because most cases ...

  5. Clinical and immunological characteristics of 150 systemic lupus erythematosus patients in Jamaica: a comparative analysis.

    Science.gov (United States)

    Maloney, K C; Ferguson, T S; Stewart, H D; Myers, A A; De Ceulaer, K

    2017-01-01

    Background Epidemiological studies in systemic lupus erythematosus have been reported in the literature in many countries and ethnic groups. Although systemic lupus erythematosus in Jamaica has been described in the past, there has not been a detailed evaluation of systemic lupus erythematosus patients in urban Jamaica, a largely Afro-Caribbean population. The goal of this study was to describe the clinical features, particularly disease activity, damage index and immunological features, of 150 systemic lupus erythematosus subjects. Methods 150 adult patients (≥18 years) followed in rheumatology clinic at a tertiary rheumatology hospital centre (one of two of the major public referral centres in Jamaica) and the private rheumatology offices in urban Jamaica who fulfilled Systemic Lupus International Collaborating Clinics (SLICC) criteria were included. Data were collected by detailed clinical interview and examination and laboratory investigations. Hence demographics, SLICC criteria, immunological profile, systemic lupus erythematosus disease activity index 2000 (SLEDAI-2K) and SLICC/American College of Rheumatology (ACR) damage index (SDI) were documented. Results Of the 150 patients, 145 (96.7%) were female and five (3.3%) were male. The mean age at systemic lupus erythematosus onset was 33.2 ± 10.9. Mean disease duration was 11.3 ± 8.6 years. The most prevalent clinical SLICC criteria were musculoskeletal, with 141 (94%) of subjects experiencing arthralgia/arthritis, followed by mucocutaneous manifestations of alopecia 103 (68.7%) and malar rash 46 (30.7%), discoid rash 45 (30%) and photosensitivity 40 (26.7%). Lupus nephritis (biopsy proven) occurred in 42 (28%) subjects and 25 (16.7%) met SLICC diagnostic criteria with only positive antinuclear antibodies/dsDNA antibodies and lupus nephritis on renal biopsy. The most common laboratory SLICC criteria were positive antinuclear antibodies 136 (90.7%) followed by anti-dsDNA antibodies 95 (63.3%) and

  6. [A case report of childhood systemic lupus erythematosus complicated with lupus cystitis].

    Science.gov (United States)

    Kurosawa, Rumiko; Miyamae, Takako; Imagawa, Tomoyuki; Katakura, Shigeki; Mori, Masaaki; Aihara, Yuhkoh; Yokota, Shumpei

    2006-06-01

    The patient was a 13-year-old girl. In August 2000, she presented with a fever, together with diarrhea, vomiting, arthralgia, nasal bleeding and malaise, and was examined by another physician. Because her platelet count was low, and there were positive reactions for anti-nuclear antibodies, anti-DNA antibodies and platelet-associated IgG, idiopathic thrombopenic purpura, and systemic lupus erythematosus (SLE) was suspected. From January 2001, when she caught measles, she reported abdominal pain, and urinalysis indicated urinary protein and occult blood, and the left kidney was found hydronephrotic. At the same time left ureter stenosis and dilatation were demonstrated. Symptoms were disappeared by hydration and treatment with NSAIDs, but 2 months later fever and erythematous patches seen on both cheeks led to the proper diagnosis of SLE, and she was admitted to our hospital. Intravenous pyelography revealed hydronephrosis on left kidney, constriction and dilatation of the left ureter, and intracystic endoscopy showed erythema at the orifice of the left ureter. The pathological examination indicated the presence of vasculitis, and finally lupus cystitis was diagnosed. Intravenous cyclophosphamide (IVCY)-pulse therapy was introduced to a total of 8 times over the period of a year, and maintenance therapy with predonisolone and azathioprin was also used. After completion of the IVCY-pulse therapy, the hydronephrosis and constriction of the ureter were disappeared. No side effects of IVCY-pulses were observed, and the patient is now in remission. We reported a case of childhood SLE complicated with lupus cystitis and successfully treated by IVCY-pulse therapy and maintenance predonisolone and azathioprin.

  7. [Neuropsychiatric systemic lupus erythematosus (1st part). Cases definitions and diagnosis and treatment of central nervous system and psychiatric manifestations of systemic lupus erythematosus].

    Science.gov (United States)

    Lefèvre, G; Zéphir, H; Warembourg, F; Michelin, E; Pruvo, J-P; Hachulla, E; Semah, F; Dubucquoi, S; Lenfant, P; Vermersch, P; Hatron, P-Y; Prin, L; Launay, D

    2012-09-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease, which primarily affects skin and joints. Peripheral neurologic syndrome and central nervous system (CNS) manifestations are common in lupus patients but are not always attributable to lupus itself. A classification, published in 1999 by the American College of Rheumatology (ACR) research committee, described 12 CNS syndromes and seven peripheral neurologic syndromes compatible with "neuropsychiatric systemic lupus erythematosus" (NPSLE). Despite this consensus, studies which have been published since 1999 have reported a prevalence of NPSLE varying from 20 to 97 %, which shows the diagnosis difficulty and the heterogeneity of neuropsychiatric manifestations in SLE. In order to understand the limits of this classification, we propose in this first part an exhaustive review of publications describing neuropsychiatric manifestations according to the ACR 1999 classification. We also detail case definitions, prevalence and risk factors, clinical characteristics and diagnosis of each lupus-related psychiatric and CNS manifestation. Copyright © 2012 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  8. Acute necrotizing retinal vasculitis as onset of systemic lupus erythematosus

    Science.gov (United States)

    Monov, Simeon; Hristova, Ruska; Dacheva, Rositza; Toncheva, Reni; Shumnalieva, Russka; Shoumnalieva-Ivanova, Viara; Monova, Daniela

    2017-01-01

    Abstract Rationale: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by autoantibody production, complement activation, and deposition of immune complexes in tissues and organs. SLE can involve any region of the visual system. Although ocular manifestations are not part of the classification criteria for SLE, they can be observed in up to one-third of the patients with SLE. They are rarely reported at the time of disease onset. Retinal vasculitis is usually associated with active generalized disease. Due to its low frequency, we report a case of acute necrotizing retinal vasculitis as onset of SLE. Patient concerns and diagnosis: A 25-year-old white female was referred to the rheumatology clinic with gradually and rapid deterioration of the vision due to abnormal vessel permeability in the right fundus with edema along the vessels, occlusion of arterial branches in the middle periphery with leakage of the dye in these areas and indentical but less prominent changes with cotton wool spots in the papillomacular area and extensive hemorrhages in the left eye. The onset of malar rash, arthralgias and positive antinuclear, anti-double stranded DNA, anti-ribosomal P and anti-β2 glycoprotein I antibodies with decreased C4 complement levels, as well as the positive lupus-band test confirmed the diagnosis of SLE. Interventions: Aggressive immunomodulating therapy with high-dose methylprednisolone, intravenous immunoglobulin, and cyclophosphamide was used for suppression of the disease activity followed by azathioprine as maintaince therapy. Outcomes: Substantial improvement and partial resorption of the vasculitic changes, including central retinal artery and vein, was achieved prominently in the left eye. The study was conducted in accordance with the Declaration of Helsinki and written informed consent was obtained from the patient. Because of this, there is no need to conduct special ethic review and the ethical approval is not necessary

  9. Distinct proteome pathology of circulating microparticles in systemic lupus erythematosus.

    Science.gov (United States)

    Østergaard, Ole; Nielsen, Christoffer Tandrup; Tanassi, Julia T; Iversen, Line V; Jacobsen, Søren; Heegaard, Niels H H

    2017-01-01

    The pathogenesis of systemic lupus erythematosus (SLE) is poorly understood but has been linked to defective clearance of subcellular particulate material from the circulation. This study investigates the origin, formation, and specificity of circulating microparticles (MPs) in patients with SLE based on comprehensive MP proteome profiling using patients with systemic sclerosis (SSc) and healthy donors (HC) as controls. We purified MPs from platelet-poor plasma using differential centrifugation of samples from SLE (n = 45), SSc (n = 38), and two sets of HC (n = 35, n = 25). MP proteins were identified and quantitated after trypsin digestion by liquid chromatography-tandem mass spectrometry. The abundance of specific proteins was compared between the groups using univariate statistics and false discovery rate correction for multiple comparisons. Specific proteins and protein ratios were explored for diagnostic and disease activity information using receiver-operating characteristic curves and by analysis of correlations of protein abundance with disease activity scores. We identify and quantitate more than 1000 MP proteins and show that a subpopulation of SLE-MPs (which we propose to call luposomes) are highly specific for SLE, i.e. not found in MP preparations from HC or patients with another autoimmune, systemic disease, SSc. In SLE-MPs platelet proteins and mitochondrial proteins are significantly diminished, cytoskeletal proteins deranged, and glycolytic enzymes and apoptotic proteins significantly increased. Normal MPs are efficiently removed in SLE, but aberrant MPs, derived from non-lymphoid leukocytes, are less efficiently removed and abundantly produced leading to an altered MP proteome in SLE. The data suggest that an abnormal generation of MPs may partake in the pathology of SLE and that new diagnostic, monitoring, and treatment strategies targeting these processes may be advantageous.

  10. CTLA-4 polymorphism in Iranian patients with systemic lupus erythematosus

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    Mahdieh Shojaa

    2014-09-01

    Full Text Available Cytotoxic T lymphocyte-associated antigen-4 (CTLA-4 is an important negative regulator of T-cell responses. CTLA-4 polymorphisms have been confirmed to be associated with several autoimmune diseases such as systemic lupus erythematosus (SLE. We analyzed the role of +49AG polymorphism in exon1 of the CTLA-4 gene in Iranian patients suffering from SLE. A cohort of 180 SLE patients and 304 ethnically and age matched healthy controls were studied. Polymerase chain reaction restriction fragments length polymorphism (PCR-RFLP was used to analyze the genotype and allele frequencies of these polymorphisms. We found that the AA genotype was significantly higher in SLE patients (67.2% vs. 41.1%, p=0.0001. The AG genotype frequency, on the other hand, was more frequently reported in the controls (49.7% vs. 27.8%, p=0.0001. The GG genotype was also more common in the control group than SLE patients but the difference was not significant (p=0.06. The frequency of G allele was significantly higher in SLE patients: 34% versus 18.9% than in control (p=0.0001. There was no significant correlation between the risk of developing SLE and the individual’s age, parental consanguinity, and family history of SLE. We didn’t observe any significant association between genotype and the clinical features of SLE. We conclude that the +49AG polymorphism of CTLA-4 gene appear to play a significant role in the development SLE in the Iranian patients, but not to be associated with clinical features of SLE.

  11. Burden of Serious Infections in Adults with Systemic Lupus Erythematosus

    Science.gov (United States)

    Tektonidou, Maria G.; Wang, Zhong; Dasgupta, Abhijit; Ward, Michael M.

    2015-01-01

    Objective To compare rates of hospitalizations for serious infections, trends in rates from 1996 to 2011, and in-hospital mortality between patients with systemic lupus erythematosus (SLE) and those without SLE in a national sample. Methods We analyzed hospitalizations for pneumonia, bacteremia/sepsis, urinary tract infections, skin infections, and opportunistic infections among adults in the Nationwide Inpatient Sample. We compared rates of hospitalizations yearly among patients with SLE and the general population. We also computed odds ratios for in-hospital mortality. Results In 1996, the estimated number of hospitalizations for pneumonia in patients with SLE was 4382, followed by sepsis (2305), skin infections (1422), urinary tract infections (643), and opportunistic infections (370). Rates were much higher in SLE than those without SLE, with age-adjusted relative risks ranging from 5.7 (95% confidence interval (CI) 5.5, 6.0) for pneumonia to 9.8 (95% CI 9.1, 10.7) for urinary tract infection in 1996. Risks increased over time, so that by 2011, all relative risks exceeded 12.0. Overall risk of in-hospital mortality was higher in SLE only for opportunistic infections (adjusted odds ratio 1.52; 95% CI 1.12, 2.07). However, in pneumonia and sepsis, mortality risks were higher in SLE among those that required mechanical ventilation. Conclusion Hospitalization rates for serious infections in SLE increased substantially between 1996 and 2011, reaching over 12 times higher than in patients without SLE in 2011. Reasons for this acceleration are unclear. In-hospital mortality was higher among patients with SLE and opportunistic infections, and those with pneumonia or sepsis who required mechanical ventilation. PMID:25732901

  12. Fibromyalgia, systemic lupus erythematosus (SLE), and evaluation of SLE activity.

    Science.gov (United States)

    Wolfe, Frederick; Petri, Michelle; Alarcón, Graciela S; Goldman, John; Chakravarty, Eliza F; Katz, Robert S; Karlson, Elizabeth W

    2009-01-01

    To determine if fibromyalgia (FM) or fibromyalgia-ness (the tendency to respond to illness and psychosocial stress with fatigue, widespread pain, general increase in symptoms, and similar factors) is increased in patients with compared to those without systemic lupus erythematosus (SLE); to determine whether FM or fibromyalgia-ness biases the SLE Activity Questionnaire (SLAQ); and to determine if the SLAQ is overly sensitive to FM symptoms. We developed a 16-item SLE Symptom Scale (SLESS) modeled on the SLAQ and used that scale to investigate the relation between SLE symptoms and fibromyalgia-ness in 23,321 patients with rheumatic disease. FM was diagnosed by survey FM criteria, and fibromyalgia-ness was measured using the Symptom Intensity (SI) Scale. As comparison groups, we combined patients with rheumatoid arthritis and noninflammatory rheumatic disorders into an "arthritis" group and also utilized a physician-diagnosed group of patients with FM. FM was identified in 22.1% of SLE and 17.0% of those with arthritis. The SI scale was minimally increased in SLE. The correlation between SLAQ and SLESS was 0.738. SLESS/SLAQ scale items (Raynaud's phenomenon, rash, fever, easy bruising, hair loss) were significantly more associated with SLE than FM, while the reverse was true for headache, abdominal pain, paresthesias/stroke, fatigue, cognitive problems, and muscle pain or weakness. There was no evidence of disproportionate symptom-reporting associated with fibromyalgia-ness. Self-reported SLE was associated with an increased prevalence of FM that was unconfirmed by physicians, compared to SLE confirmed by physicians. The prevalence of FM in SLE is minimally increased compared with its prevalence in patients with arthritis. Fibromyalgia-ness does not bias the SLESS and should not bias SLE assessments, including the SLAQ.

  13. Selective involvement of the amygdala in systemic lupus erythematosus.

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    Bart J Emmer

    2006-12-01

    Full Text Available BACKGROUND: Antibodies specifically affect the amygdala in a mouse model of systemic lupus erythematosus (SLE. The aim of our study was to investigate whether there is also specific involvement of the amygdala in human SLE. METHODS AND FINDINGS: We analyzed a group of 37 patients with neuropsychiatric SLE (NP-SLE, 21 patients with SLE, and a group of 12 healthy control participants with diffusion weighted imaging (DWI. In addition, in a subset of eight patients, plasma was available to determine their anti-NMDAR antibody status. From the structural magnetic resonance imaging data, the amygdala and the hippocampus were segmented, as well as the white and gray matter, and the apparent diffusion coefficient (ADC was retrieved. ADC values between controls, patients with SLE, and patients with NP-SLE were tested using analysis of variance with post-hoc Bonferroni correction. No differences were found in the gray or white matter segments. The average ADC in the amygdala of patients with NP-SLE and SLE (940 x 10(-6 mm2/s; p = 0.006 and 949 x 10(-6 mm2/s; p = 0.019, respectively was lower than in healthy control participants (1152 x 10(-6 mm2/s. Mann-Whitney analysis revealed that the average ADC in the amygdala of patients with anti-NMDAR antibodies (n = 4; 802 x 10(-6 mm2/s was lower (p = 0.029 than the average ADC of patients without anti-NMDAR antibodies (n = 4; 979 x 10(-6 mm2/s and also lower (p = 0.001 than in healthy control participants. CONCLUSIONS: This is the first study to our knowledge to observe damage in the amygdala in patients with SLE. Patients with SLE with anti-NMDAR antibodies had more severe damage in the amygdala compared to SLE patients without anti-NMDAR antibodies.

  14. Neuropsychiatric involvement in systemic lupus erythematosus: current therapeutic approach.

    Science.gov (United States)

    Sanna, Giovanni; Bertolaccini, Maria Laura; Khamashta, Munther A

    2008-01-01

    The involvement of the central nervous system (CNS) is one of the major causes of morbidity and mortality in systemic lupus erythematosus (SLE) patients and the less understood aspect of the disease. Its recognition and treatment continue to represent a major diagnostic and therapeutic challenge. Due to the lack of controlled randomized trials, current therapeutic approach is still empirical and based on clinical experience. The therapeutic choice depends on accurate diagnosis, identification of underlying pathogenic mechanism, severity of the presenting neuropsychiatric symptoms, and on prompt identification and management of contributing causes of CNS disease. Mild neuropsychiatric manifestations may need symptomatic treatment only. In more severe CNS disease it is important to distinguish between thrombotic and non-thrombotic mechanisms. Focal CNS manifestations, particularly TIA and stroke, are associated with the presence of antiphospholipid antibodies (aPL). Anticoagulation is warranted in patients with thrombotic disease, particularly in those with the antiphospholipid (Hughes) syndrome (APS). Other CNS manifestations, such as demyelinating syndrome, transverse myelitis, chorea, seizures, migraine and/or cognitive dysfunction, when associated with persistent positivity for aPL, may also benefit from anticoagulation in selected patients. Severe diffuse CNS manifestations, such as acute confusional state, generalised seizures, mood disorders and psychosis, generally require corticosteroids in the first instance. Pulse intravenous cyclophosphamide therapy may help when more severe manifestations are refractory to corticosteroids and other immunosuppressive agents, generally when response is not seen in 3-5 days. Plasmapheresis may also be added in severe cases of symptoms refractory to conventional treatment. Intravenous immunoglobulins, mycophenolate mofetil, rituximab, intratecal methotrexate and dexametasone deserve further studies to confirm their

  15. Serum cryoglobulins and disease activity in systematic lupus erythematosus

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    Mansoor Karimifar

    2013-01-01

    Full Text Available Background: To determine the prevalence of cryoglobulins in Iranian patients with systemic lupus erythematosus (SLE and evaluate the correlation of cryoglobulins with disease activity in these patients. Materials and Methods: In a cross-sectional study, we investigated 80 consecutive women who fulfilled the 1982 revised criteria of the American College of Rheumatology for the classification of SLE. All the patients had undergone a medical interview and general physical examination by a rheumatologist for clinical and serologic characteristics of SLE. For the determination of cryoglobulins, sera were collected by a standard protocol at 37°C, and after incubation at 4°C for seven days, the level of cryoglobulins was estimated for each patient. Results: Cryoglobulins were detected in the sera of 39 (48.8% patients. All of these patients had cryocrit over 5%. Disease was active in 30 patients [SLEDAI ≥6 (DAI: disease activity index] and inactive in 50 (SLEDAI <6. There was no significant difference between active and inactive patients for the presence of serum cryoglobulins (r = 0.086, P = 0.56. A significant positive correlation was observed between antinuclear antibody (ANA, anti-dsDNA (dsDNA: Double-stranded deoxyribonucleic acid, CH50 (CH50: total hemolytic complement assay, and C-reactive protein (CRP (r = 0.21, P = 0.004, r = 0.65, P = 0.001, r = 0.45, P = 0.023, r = 0.38, P = 0.036, respectively. Hepatitis C virus (HCV infection was not detected in any of the SLE patients. Conclusion: Although the presence of cryoglobulins in the SLE patients correlated with positive anti-ds DNA and low CH50, it could not be predict activity of the disease.

  16. Metabolic syndrome in patients with systemic lupus erythematosus

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    T Y Popkova

    2008-01-01

    Full Text Available Objective. To characterize metabolic syndrome (M S in pts wit h systemic lupus erythematosus (SLE and determine contribution of immune inflammation to the development of MS. Material and methods. 156 females with SLE (mean age 35 years, mean disease duration 99 months were included. Control group consisted of 69 people of comparable age without rheumatic diseases. MS was diagnosed according to ATP III criteria, \\fascular atherosclerotic damage was assessed by carotid sonographic evaluation. Serum cholesterol (CS, triglycerides (TG and high-density lipoprotein (HDLP CS concentration was assessed with colorimetric and photometric methods, hs CRP level — with nephelometric immunoassay. Results. MS was revealed in 29 from 154 (19% pts with SLE and in 5 from 69 (7% controls (p=0,02. MS components (hypertension, TG elevation and a lipoprotein decrease in SLE were significantly more frequent than in control group. TG, HDLP CS and CRP levels in SLE were higher than in control. Thickness of carotid intima-media complex did not differ in SLE and control. Frequency of atherosclerotic plaques (15% and coronary heart disease (14% in SLE was higher than in control (4% and 2% respectively, p=0,01. Pts with SLE and MS were older, had higher disease activity and maximal glucocorticoid dose during disease period (p<0,05. CRP concentration in SLE with MS was significantly higher. Subclinical signs of atherosclerosis in SLE with MS were more frequent than in SLE without MS (p<0,05. Frequency of clinical signs of atherosclerosis did not differ in these groups. Conclusion. Autoimmune inflammation in SLE plays an important role in the development of MS.

  17. Disease characterization of systemic lupus erythematosus (SLE) patients in Quebec.

    Science.gov (United States)

    Ng, R; Bernatsky, S; Rahme, E

    2017-01-01

    Objective Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by an array of organ manifestations that can appear during flares and disappear during remissions. The objectives of this study were: (i) to examine SLE manifestation groups longitudinally in an SLE cohort; and (ii) to assess the association between early antimalarial treatment and renal manifestations. Methods Seven SLE manifestation groups-cutaneous, hematologic, lung, musculoskeletal, neuropsychiatric, serositis, renal-were tracked using Kaplan-Meier survival curves in an incident SLE cohort from Quebec health administrative data ( n = 2010). A subgroup with provincial drug insurance coverage was followed over time to examine the association between early antimalarial treatment (within three months after SLE diagnosis) and renal manifestations using a Cox proportional hazards survival model. Results Cutaneous manifestations was the most common manifestation at SLE diagnosis (30.0%, 95% CI: 27.7-32.2%). About two-thirds (66.2%, 95% CI: 63.4-68.9%) of patients had evidence of at least one SLE manifestation at diagnosis, which increased to 87.2% (95% CI: 84.2-90.3%) by the end of follow-up. After adjusting for age, sex, early concomitant systemic steroid therapy, Charlson comorbidity index, primary care visits in the year prior and other SLE manifestations at baseline, no statistically significant association was established between antimalarial therapy and renal manifestations. Conclusion This study provides insight regarding organ manifestations within a population-based sample. Most patients identified with SLE had other diagnostic evidence that supports an underlying diagnosis of SLE. No protective effects for antimalarial agents against renal manifestations could be established in this population-based cohort.

  18. Neuropsychiatric systemic lupus erythematosus: where are we now?

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    M. Padovan

    2011-09-01

    Full Text Available When dealing with neuropsychiatric Systemic Lupus Erythematosus (NPSLE there are still many controversial topics. In 1999 the American College of Rheumatology gave classification criteria for 19 clinical syndromes. However major problems are still related to low specificity of some of them such as headache, cognitive impairment or mood disorders. Even though a frequency of CNS involvement from 14 to 75% has been described, depending on both the population studied and the methodology of assessment, a lower frequency ranging from 21 to 28 % derived by larger case series seems more realistic. The introduction of the concept of “borderline cases”, proposed by Italian Study Group for NP-SLE, is based both on clinical and instrumental evaluation and could represent a useful tool when dealing with conditions which do not fulfil ACR classification. Also the relationship between SLE activity and NP involvement is a debated issue. Concerning pathogenesis, it seems reasonable to consider multifactorial mechanisms related to antibody-mediated damage, antiphospholipid pro-thrombotic effect, non-inflammatory vasculopathy and cytokines mediated cytotoxycity. However, direct and unequivocal evidence for the implication of any of the above-mentioned mechanisms is still lacking. Although a wide range of neuroimaging tools have been used to evaluate CNS involvement, no single technique has proven to be definitive and, when dealing with a patient with suspected NPSLE, it is important to combine different diagnostic techniques. Due to the lack of effective imaging along with limitation in knowledge of underlying pathogenetic mechanisms and paucity of histopathologic findings, therapeutic approach in NPSLE remains a difficult issue and is currently based on personal experience. Italian Study Group for NP-SLE proposes the creation of a national registry on NPSLE to validate ACR classification criteria. Furthermore, the possibility to collect large series and

  19. Rapid onset of massive ascites as the initial presentation of systemic lupus erythematosus.

    Science.gov (United States)

    Weinstein, P J; Noyer, C M

    2000-01-01

    Ascites in systemic lupus erythematosus (SLE) is rarely massive, and either accompanies the typical manifestations of active disease or results from nephrotic syndrome, protein-losing enteropathy, constrictive pericarditis, and conditions unrelated to lupus. Marked ascites has been attributed to chronic lupus peritonitis, characterized by the insidious onset of massive, painless ascites and unrelated to disease activity. Regardless of the etiology, ascites typically has a gradual onset and occurs after a diagnosis of SLE has been made. We describe a young woman presenting with the rapid development of massive ascites as the initial manifestation of SLE.

  20. Episcleritis Related to Drug-Induced Lupus Erythematosus following Infliximab Therapy: A Case Report

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    Irini P. Chatziralli

    2011-01-01

    Full Text Available Drug-induced lupus erythematosus is defined as a lupus-like syndrome temporally related to continuous drug exposure which resolves after discontinuation of the offending drug. Herein, we describe a patient with distinct clinical manifestations of anti-TNF-associated DILE related to infliximab therapy. The patient exhibited clinical and laboratory findings of lupus-like illnesses as well as ocular disorders, such as episcleritis. The main message is that the symptoms of DILE should not be overlooked, although sometimes other systematic conditions may underlie them. As a result, it is very important for the clinicians to evaluate the symptoms of DILE and manage appropriately these cases.

  1. One Case of Systemic Lupus Erythematosus treated with traditional Korean Medicine

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    An, Chang-Suk

    2000-12-01

    Full Text Available After observing a patient diagnosed with Systemic Lupus Erythematosus from the September 21, 2000 to the January 13, 2001, the following results are obtained. Method and Result: We treated one case of Systemic Lupus Erythematosus with the Korean Bee Venom Therapy and the other Oriental Medical treatments. In acupuncture therapy, Korean Bee-venom Therapy is believed to be outstanding way to treat Systemic Lupus Erythematosus. Korean Bee Venom Therapy is treated on the following acupuncture points: ST36(B23(Shinsu:腎兪, BL26(Guanyuanshu:關元兪, ST36(Chok-Samni:足三里, LI4(Hapkok:合谷, LV3(Taechung, SP10(Hyolhae:血海 SP6 (Samumgyo;三陰交. In addition, CFC(Carthami Flos;紅花 and Cervi Pantotrichum Cornu; Herbal-Acupuncture was applied on the acupucture points of GB20(Pungji: 風池, GB21(Kyonjong:, as well as BUM(It was made of Boviscalculus(牛黃, Moschus and Fel ursi Herbal-Acupuncture was given on CV17(Chonjung. In herbal medication, based on the Sasang Constitution, Taeyumin Chungsimyunja-Tang was given to the patient. As the results of these treatments, general conditions of this patient improved drastically. Conclusion: Based on the clinical results, traditional Korean Medical treatment is believed to be effective for treating Systemic Lupus Erythematosus, and futher studies should be carried out to provide more valuable information.

  2. Treatment of chronic discoid lupus erythematosus with an oral gold compound (auranofin).

    Science.gov (United States)

    Dalziel, K; Going, G; Cartwright, P H; Marks, R; Beveridge, G W; Rowell, N R

    1986-08-01

    Twenty-three patients with severe longstanding discoid lupus erythematosus, unresponsive to conventional treatments, were treated with oral gold in a multicentre open study. Nineteen patients showed clinical improvement and in four of these there was complete resolution of lesions. Adverse reactions were generally mild and self limiting.

  3. Post-partum bilateral renal cortical necrosis in antiphospholipid syndrome and systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Venkat Sainaresh Vellanki

    2013-01-01

    Full Text Available In the presence of systemic lupus erythematosus or related autoimmune disorders, antiphospholipid syndrome (APS is termed secondary APS. Pregnancy-related renal failure due to SAPS is rarely reported in the literature. We present the case of a young primgravida woman with bilateral renal cortical necrosis due to secondary APS in late pregnancy.

  4. Immune Response Modulation by Vitamin D: Role in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Iruretagoyena, Mirentxu; Hirigoyen, Daniela; Naves, Rodrigo; Burgos, Paula Isabel

    2015-01-01

    Vitamin D plays key roles as a natural immune modulator and has been implicated in the pathophysiology of autoimmune diseases, including systemic lupus erythematosus (SLE). This review presents a summary and analysis of the recent literature regarding immunoregulatory effects of vitamin D as well as its importance in SLE development, clinical severity, and possible effects of supplementation in disease treatment.

  5. Bone metabolism in patients with systemic lupus erythematosus. Effect of disease activity and glucocorticoid treatment

    DEFF Research Database (Denmark)

    Hansen, M; Halberg, P; Kollerup, G

    1998-01-01

    The bone metabolism in patients with systemic lupus erythematosus (SLE) has previously been examined, but the results are conflicting. In the present study the bone mineral density (BMD) of the axial and the appendicular skeleton was examined by means of dual energy x-ray absorptiometry. The bone...

  6. Toxoplasma pericarditis mimicking systemic lupus erythematosus. Diagnostic and treatment difficulties in one patient

    DEFF Research Database (Denmark)

    Lyngberg, K K; Vennervald, B J; Bygbjerg, I C

    1992-01-01

    A life-threatening T. gondii pericarditis developed in a patient with symptoms corresponding to systemic lupus erythematosus (SLE) with high concentrations of antinuclear antibodies and lymphadenopathy. The diagnosis would have been SLE-associated serositis, had not pericardial fluid been...

  7. Off-label use of rituximab for systemic lupus erythematosus in Europe

    DEFF Research Database (Denmark)

    Ryden-Aulin, Monica; Boumpas, Dimitrios T; Bultink, Irene

    2016-01-01

    Objectives: Rituximab (RTX) is a biological treatment used off-label in patients with systemic lupus erythematosus (SLE). This survey aimed to investigate the off-label use of RTX in Europe and compare the characteristics of patients receiving RTX with those receiving conventional therapy. Method...

  8. Replication of recently identified systemic lupus erythematosus genetic associations : a case-control study

    NARCIS (Netherlands)

    Suarez-Gestal, Marian; Calaza, Manuel; Endreffy, Emoeke; Pullmann, Rudolf; Ordi-Ros, Josep; Sebastiani, Gian Domenico; Ruzickova, Sarka; Santos, Maria Jose; Papasteriades, Chryssa; Marchini, Maurizio; Skopouli, Fotini N.; Suarez, Ana; Blanco, Francisco J.; D'Alfonso, Sandra; Bijl, Marc; Carreira, Patricia; Witte, Torsten; Migliaresi, Sergio; Gomez-Reino, Juan J.; Gonzalez, Antonio

    2009-01-01

    Introduction We aimed to replicate association of newly identified systemic lupus erythematosus (SLE) loci. Methods We selected the most associated SNP in 10 SLE loci. These 10 SNPs were analysed in 1,579 patients with SLE and 1,726 controls of European origin by single-base extension. Comparison of

  9. Acquired generalised neuromyotonia, cutaneous lupus erythematosus and alopecia areata in a patient with myasthenia gravis.

    LENUS (Irish Health Repository)

    O'Sullivan, S S

    2012-02-03

    We describe a patient with the diagnoses of acquired neuromyotonia, cutaneous lupus erythematosus and alopecia areata, occurring many years after a thymectomy for myasthenia gravis associated with a thymoma. We review the current literature on autoimmune conditions associated with myasthenia gravis and thymectomy. To our knowledge, this combination of multiple autoimmune conditions has not been reported previously.

  10. Bone metabolism in patients with systemic lupus erythematosus. Effect of disease activity and glucocorticoid treatment

    DEFF Research Database (Denmark)

    Hansen, M; Halberg, P; Kollerup, G

    1998-01-01

    The bone metabolism in patients with systemic lupus erythematosus (SLE) has previously been examined, but the results are conflicting. In the present study the bone mineral density (BMD) of the axial and the appendicular skeleton was examined by means of dual energy x-ray absorptiometry. The bone...

  11. Systemic lupus erythematosus and Wiskott-Aldrich syndrome in an Italian patient

    NARCIS (Netherlands)

    Monteferrante, G.; Giani, M.; van den Heuvel, M. C.

    2009-01-01

    Systemic lupus erythematosus has not yet been associated with mutations in the Wiskott-Aldrich syndrome gene; moreover, the time courses of platelet number and size in patients with Wiskott-Aldrich syndrome are unknown. In this case, we present the time trends of platelet count and volume and the hi

  12. Cutaneous lupus erythematosus and the risk of deep venous thrombosis and pulmonary embolism

    DEFF Research Database (Denmark)

    Ahlehoff, O; Wu, Jashin J; Raunsø, Jakob

    2017-01-01

    Background Venous thromboembolism (VTE) is a major public health concern. Lupus erythematosus (LE) is a chronic autoimmune disease ranging from localized cutaneous disease (CLE) to systemic involvement (SLE). Patients with SLE have an increased risk of venous thromboembolism (VTE), but little...

  13. 21 CFR 866.5820 - Systemic lupus erythema-tosus immunological test system.

    Science.gov (United States)

    2010-04-01

    ... diagnosis of SLE (a multisystem autoimmune disease in which tissues are attacked by the person's own... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Systemic lupus erythema-tosus immunological test system. 866.5820 Section 866.5820 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH...

  14. Safety and efficacy of influenza vaccination in systemic lupus erythematosus patients with quiescent disease

    NARCIS (Netherlands)

    Holvast, A; Huckriede, A; Wilschut, J; Horst, G; De Vries, JJC; Benne, CA; Kallenberg, CGM; Bijl, M

    2006-01-01

    Objective: to assess the safety and efficacy of influenza vaccination in patients with systemic lupus erythematosus (SLE), and to evaluate the influence of immunosuppressive drugs on the immune response. Methods: SLE patients (n = 56) and healthy controls (n = 18) were studied. All patients had quie

  15. Studies of cell-mediated immune responses to influenza vaccination in systemic lupus erythematosus

    NARCIS (Netherlands)

    Holvast, Albert; Van Assen, Sander; De Haan, Aalzen; Huckriede, Anke; Benne, Cornelis A.; Westra, Johanna; Palache, Abraham; Wilschut, Jan; Kallenberg, Cornelis; Bijl, Marc

    2009-01-01

    Objective. Both antibody and cell-mediated responses are involved in the defense against influenza. In patients with systemic lupus erythematosus (SLE), a decreased antibody response to subunit influenza vaccine has been demonstrated, but cell-mediated responses have not yet been assessed. This stud

  16. HPV infection and vaccination in Systemic Lupus Erythematosus patients : what we really should know

    NARCIS (Netherlands)

    Rotstein Grein, Ingrid Herta; Groot, Noortje; Lacerda, Marcela Ignacchiti; Wulffraat, Nico; Pileggi, Gecilmara

    2016-01-01

    Patients with Systemic Lupus Erythematosus (SLE) are at increased risk for infections. Vaccination is a powerful tool to prevent infections, even in immunocompromised patients. Most non-live vaccines are immunogenic and safe in patients with SLE, even if antibody titres are frequently lower than tho

  17. Longitudinal study on premature atherosclerosis in patients with systemic lupus erythematosus

    NARCIS (Netherlands)

    de Leeuw, Karina; Smit, Andries J.; de Groot, Eric; van Roon, Arie M.; Kallenberg, Cees G.; Bijl, Marc

    2009-01-01

    Objectives: To determine risk factors of accelerated atherosclerosis and progression of intima-media thickness (IMT) in patients with systemic lupus erythematosus (SLE). Methods: 74 SLE patients, age ranging from 13 to 69 years, and 74 age- and sex-matched controls were included. IMT of the common c

  18. Hormone profile in juvenile systemic lupus erythematosus with previous or current amenorrhea

    NARCIS (Netherlands)

    Silva, Clovis A.; Deen, Maria E. J.; Febronio, Marilia V.; Oliveira, Sheila K.; Terreri, Maria T.; Sacchetti, Silvana B.; Sztajnbok, Flavio R.; Marini, Roberto; Quintero, Maria V.; Bica, Blanca E.; Pereira, Rosa M.; Bonfa, Eloisa; Ferriani, Virginia P.; Robazzi, Teresa C.; Magalhaes, Claudia S.; Hilario, Maria O.

    2011-01-01

    To identify the underlying mechanism of amenorrhea in juvenile systemic lupus erythematosus (JSLE) patients, thirty-five (11.7%) JSLE patients with current or previous amenorrhea were consecutively selected among the 298 post-menarche patients followed in 12 Brazilian pediatric rheumatology centers.

  19. Concordance of autoimmune disease in a nationwide Danish systemic lupus erythematosus twin cohort

    DEFF Research Database (Denmark)

    Ulff-Møller, Constance Jensina; Svendsen, Anders Jørgen; Viemose, Louise Nørgaard

    2017-01-01

    OBJECTIVE: To determine the concordance of systemic lupus erythematosus (SLE) and co-aggregating autoimmune diseases among Danish twins. METHODS: SLE-affected twins were ascertained by record linkage between the National Patient Register (NPR) and the Danish Twin Registry (DTR). Registered SLE co...

  20. A systemic sclerosis and systemic lupus erythematosus pan-meta-GWAS reveals new shared susceptibility loci

    NARCIS (Netherlands)

    Martin, J.E.; Assassi, S.; Diaz-Gallo, L.M.; Broen, J.C.A.; Simeon, C.P.; Castellvi, I.; Vicente-Rabaneda, E.; Fonollosa, V.; Ortego-Centeno, N.; Gonzalez-Gay, M.A.; Espinosa, G.; Carreira, P.; Scleroderma, G. Spanish; consortium, S.; group, U.S.S.G.; Biolupus, .; Camps, M.; Sabio, J.M.; D'Alfonso, S.; Vonk, M.C.; Voskuyl, A.E.; Schuerwegh, A.J.; Kreuter, A.; Witte, T. de; Riemekasten, G.; Hunzelmann, N.; Airo, P.; Beretta, L.; Scorza, R.; Lunardi, C.; Laar, J. van; Chee, M.M.; Worthington, J.; Herrick, A.; Denton, C.; Fonseca, C.; Tan, F.K.; Arnett, F.; Zhou, X.; Reveille, J.D.; Gorlova, O.; Koeleman, B.P.; Radstake, T.R.D.J.; Vyse, T.; Mayes, M.D.; Alarcon-Riquelme, M.E.; Martin, J.

    2013-01-01

    Systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) are two archetypal systemic autoimmune diseases which have been shown to share multiple genetic susceptibility loci. In order to gain insight into the genetic basis of these diseases, we performed a pan-meta-analysis of two genome-wide