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Sample records for induced lupus syndrome

  1. Hydralazine Induced Lupus Syndrome Presenting with Recurrent Pericardial Effusion and a Negative Antinuclear Antibody

    OpenAIRE

    Praneet Iyer; Ahmed Dirweesh; Ritika Zijoo

    2017-01-01

    Drug induced lupus erythematosus (DIL or DILE) is an autoimmune disorder caused by chronic use of certain drugs. We report a unique case of hydralazine induced lupus syndrome (HILS) with a negative antinuclear antibody in a female patient who was on hydralazine for a period of 1.5–2 years and developed recurrent pericardial effusion as a result of it. Initially her condition was managed with a pericardial window. The recurrence of a massive pericardial effusion necessitated a right hemiperica...

  2. 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.

  3. Hydralazine Induced Lupus Syndrome Presenting with Recurrent Pericardial Effusion and a Negative Antinuclear Antibody

    Science.gov (United States)

    Iyer, Praneet; Zijoo, Ritika

    2017-01-01

    Drug induced lupus erythematosus (DIL or DILE) is an autoimmune disorder caused by chronic use of certain drugs. We report a unique case of hydralazine induced lupus syndrome (HILS) with a negative antinuclear antibody in a female patient who was on hydralazine for a period of 1.5–2 years and developed recurrent pericardial effusion as a result of it. Initially her condition was managed with a pericardial window. The recurrence of a massive pericardial effusion necessitated a right hemipericardiectomy. After hydralazine was stopped, she never had any further episodes of pericardial effusion or tamponade. PMID:28194293

  4. Hydralazine Induced Lupus Syndrome Presenting with Recurrent Pericardial Effusion and a Negative Antinuclear Antibody

    Directory of Open Access Journals (Sweden)

    Praneet Iyer

    2017-01-01

    Full Text Available Drug induced lupus erythematosus (DIL or DILE is an autoimmune disorder caused by chronic use of certain drugs. We report a unique case of hydralazine induced lupus syndrome (HILS with a negative antinuclear antibody in a female patient who was on hydralazine for a period of 1.5–2 years and developed recurrent pericardial effusion as a result of it. Initially her condition was managed with a pericardial window. The recurrence of a massive pericardial effusion necessitated a right hemipericardiectomy. After hydralazine was stopped, she never had any further episodes of pericardial effusion or tamponade.

  5. Drug-induced lupus.

    Science.gov (United States)

    Rubin, Robert L

    2005-04-15

    Autoantibodies and, less commonly, systemic rheumatic symptoms are associated with treatment with numerous medications and other types of ingested compounds. Distinct syndromes can be distinguished, based on clinical and laboratory features, as well as exposure history. Drug-induced lupus has been reported as a side-effect of long-term therapy with over 40 medications. Its clinical and laboratory features are similar to systemic lupus erythematosus, except that patients fully recover after the offending medication is discontinued. This syndrome differs from typical drug hypersensitivity reactions in that drug-specific T-cells or antibodies are not involved in induction of autoimmunity, it usually requires many months to years of drug exposure, is drug dose-dependent and generally does not result in immune sensitization to the drug. Circumstantial evidence strongly suggests that oxidative metabolites of the parent compound trigger autoimmunity. Several mechanisms for induction of autoimmunity will be discussed, including bystander activation of autoreactive lymphocytes due to drug-specific immunity or to non-specific activation of lymphocytes, direct cytotoxicity with release of autoantigens and disruption of central T-cell tolerance. The latter hypothesis will be supported by a mouse model in which a reactive metabolite of procainamide introduced into the thymus results in lupus-like autoantibody induction. These findings, as well as evidence for thymic function in drug-induced lupus patients, support the concept that abnormalities during T-cell selection in the thymus initiate autoimmunity.

  6. 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.

  7. Yohimbine-induced cutaneous drug eruption, progressive renal failure, and lupus-like syndrome.

    Science.gov (United States)

    Sandler, B; Aronson, P

    1993-04-01

    Yohimbine is an indole alkaloid obtained from the yohimbe tree, a common tree in West Africa. We describe a forty-two-year black man in whom a generalized erythrodermic skin eruption, progressive renal failure, and lupus-like syndrome developed following treatment with the drug, yohimbine. A literature review failed to reveal any reported association of these side effects. We review current information on yohimbine's use in male impotence, reported side effects, and its role as a drug allergen.

  8. Neonatal lupus syndromes.

    Science.gov (United States)

    Buyon, J P; Rupel, A; Clancy, R M

    2004-01-01

    The neonatal lupus syndromes (NLS), while quite rare, carry significant mortality and morbidity in cases of cardiac manifestations. Although anti-SSA/Ro-SSB/La antibodies are detected in > 85% of mothers whose fetuses are identified with congenital heart block (CHB) in a structurally normal heart, when clinicians applied this testing to their pregnant patients, the risk for a woman with the candidate antibodies to have a child with CHB was at or below 1 in 50. While the precise pathogenic mechanism of antibody-mediated injury remains unknown, it is clear that the antibodies alone are insufficient to cause disease and fetal factors are likely contributory. In vivo and in vitro evidence supports a pathologic cascade involving apoptosis of cardiocytes, surface translocation of Ro and La antigens, binding of maternal autoantibodies, secretion of profibrosing factors (e.g., TGFbeta) from the scavenging macrophages and modulation of cardiac fibroblasts to a myofibroflast scarring phenotype. The spectrum of cardiac abnormalities continues to expand, with varying degrees of block identified in utero and reports of late onset cardiomyopathy (some of which display endocardial fibroelastosis). Moreover, there is now clear documentation that incomplete blocks (including those improving in utero with dexamethasone) can progress postnatally, despite the clearance of the maternal antibodies from the neonatal circulation. Better echocardiographic measurements which identify first degree block in utero may be the optimal means of approaching pregnant women at risk. Prophylactic therapies, including treatment with intravenous immunoglobulin, await larger trials. In order to achieve advances at both the bench and bedside, national research registries established in the US and Canada are critical.

  9. 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.

  10. [Hypoprothrombinemia--lupus anticoagulant syndrome].

    Science.gov (United States)

    Campos, Maria Manuel; Reis Santos, Isabel

    2011-12-01

    Diagnosis criteria, pathogenic mechanisms, incidence and prevalence of the Antiphospholipid Syndrome are focused in a brief review. Hypoprothrombinemia (HPT) may be hereditary or acquired; the first is rare and with recessive autossomic transmission. We report the case of a 66-year-old white woman with Systemic Lupus Erythematosus (SLE), autoimmune haemolytic anaemia, periostitis, haematomas, bleeding leg ulcer and rectal haemorrhages; she had decreased levels of the prothrombin. Haemorrhagic episodes were related with the anti-prothrombin specificity of Lupus Anticoagulant (LA) detected. The SLE/LA/HPT association is less frequent than the correlated to SLE/LA/anti- ß2Glycoprotein I antibodies and was first reported in 1960 by Rapaport et al, in an 11-year- -old girl with severe haemorrhagic manifestations.

  11. 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.

  12. [Neonatal lupus syndrome: Literature review].

    Science.gov (United States)

    Morel, N; Georgin-Lavialle, S; Levesque, K; Guettrot-Imbert, G; Le Guern, V; Le Bidois, J; Bessières, B; Brouzes, C; Le Mercier, D; Villain, E; Maltret, A; Costedoat-Chalumeau, N

    2015-03-01

    Neonatal lupus syndrome is associated with transplacental passage of maternal anti-SSA/Ro and anti-SSB/La antibodies. Children display cutaneous, hematological, liver or cardiac features. Cardiac manifestations include congenital heart block (CHB); endocardial fibroelastosis and dilated cardiomyopathy. The prevalence of CHB in newborns of anti-Ro/SSA positive women with known connective tissue disease is between 1 and 2% and the risk of recurrence is around 19%. Skin and systemic lesions are transient, whereas CHB is definitive and associated with significant morbidity and a mortality of 18%. A pacemaker must be implanted in 2/3 of cases. Myocarditis may be associated or appear secondly. Mothers of children with CHB are usually asymptomatic or display Sjogren's syndrome or undifferentiated connective tissue disease. In anti-Ro/SSA positive pregnant women, fetal echocardiography should be performed at least every 2 weeks from the 16th to 24th week gestation. An electrocardiogram should be performed for all newborn babies. The benefit of fluorinated corticosteroid therapy for CHB detected in utero remains unclear. Maternal use of hydroxychloroquine may be associated with a decreased recurrent CHB risk in a subsequent offspring. A prospective study is actually ongoing to confirm these findings.

  13. 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.

  14. 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.

  15. A lupus-like syndrome associated with infliximab therapy.

    Science.gov (United States)

    Klapman, Jason B; Ene-Stroescu, Daniel; Becker, Michael A; Hanauer, Stephen B

    2003-05-01

    Infliximab, a chimeric monoclonal antibody targeting tumor necrosis factor alpha (TNF-alpha), is efficacious in the treatment of rheumatoid arthritis and Crohn's disease. We report in detail an unusual adverse reaction to infliximab therapy, a drug-induced lupus-like clinical syndrome. A 45-year-old woman with steroid-dependent Crohn's colitis, successfully managed with maintenance infliximab infusions and methotrexate, developed a lupus-like syndrome eight months after her initial infusion. This was characterized by inflammatory arthritis and an urticarial and papulosquamous rash and was accompanied by high titers of antinuclear, double-stranded DNA, glomerular-binding, and histone antibodies and by reduced levels of the C4 component of complement. After discontinuance of infliximab infusions and treatment of symptoms with intermittent courses of prednisone, the patient's arthritis progressively improved, with accompanying decrements in autoantibody titers. One year later, she has minimal joint discomfort and no rash or gastrointestinal symptoms despite also discontinuing prednisone and methotrexate. Infliximab therapy may cause a lupus-like syndrome that is reversible upon discontinuing this agent. These findings support recent evidence identifying TNF-alpha as an inhibitor of autoantibody formation.

  16. 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.

  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. 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....

  19. Posterior reversible encephalopathy syndrome in a patient with lupus nephritis

    Directory of Open Access Journals (Sweden)

    Huseyin Kadikoy

    2012-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is characterized by acute onset of headache, nausea, focal neurological deficits or seizures along with radiological findings of white matter defects in the parietal and occipital lobes. Causes of PRES include uremia, hypertensive encephalopathy, eclampsia and immunosuppressive medications. Usually, the treat-ment of choice involves correcting the underlying abnormality. We describe an unusual case of recurrent PRES caused by uremia during a lupus flare in a patient with biopsy-proven Class IV Lupus Nephritis (LN with vasculitis. PRES in systemic lupus erythematosis (SLE is a rare clin-ical phenomenon and, when reported, it is associated with hypertensive encephalopathy. Our patient did not have hypertensive crisis, but had uremic encephalopathy. The patient′s PRES-related symptoms resolved after initiation of hemodialysis. The temporal correlation of the correc-tion of the uremia and the resolution of the symptoms of PRES show the etiology to be uremic encephalopathy, making this the first reported case of uremia-induced PRES in Class IV LN with vasculitis.

  20. Posterior reversible encephalopathy syndrome in a patient with lupus nephritis.

    Science.gov (United States)

    Kadikoy, Huseyin; Haque, Waqar; Hoang, Vu; Maliakkal, Joseph; Nisbet, John; Abdellatif, Abdul

    2012-05-01

    Posterior reversible encephalopathy syndrome (PRES) is characterized by acute onset of headache, nausea, focal neurological deficits or seizures along with radiological findings of white matter defects in the parietal and occipital lobes. Causes of PRES include uremia, hypertensive encephalopathy, eclampsia and immunosuppressive medications. Usually, the treatment of choice involves correcting the underlying abnormality. We describe an unusual case of recurrent PRES caused by uremia during a lupus flare in a patient with biopsy-proven Class IV Lupus Nephritis (LN) with vasculitis. PRES in systemic lupus erythematosis (SLE) is a rare clinical phenomenon and, when reported, it is associated with hypertensive encephalopathy. Our patient did not have hypertensive crisis, but had uremic encephalopathy. The patient's PRES-related symptoms resolved after initiation of hemodialysis. The temporal correlation of the correction of the uremia and the resolution of the symptoms of PRES show the etiology to be uremic encephalopathy, making this the first reported case of uremia-induced PRES in Class IV LN with vasculitis.

  1. Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies.

    Science.gov (United States)

    Galland, Joris; Mohamed, Shirine; Revuz, Sabine; de Maistre, Emmanuel; de Laat, Bas; Marie, Pierre-Yves; Zuily, Stéphane; Lévy, Bruno; Regnault, Véronique; Wahl, Denis

    2016-07-01

    Lupus anticoagulant-hypoprothrombinemia syndrome is a rare condition characterized by the association of acquired factor II deficiency and lupus anticoagulant. Contrary to classical antiphospholipid syndrome, it may cause severe life-threatening bleeding (89% of published cases). We report a patient, positive for antidomain I antibodies, with initially primary lupus anticoagulant-hypoprothrombinemia syndrome without previous clinical manifestation or underlying systemic disease. Five years later, he experienced the first systemic lupus erythematous flare. Within a few days, catastrophic antiphospholipid syndrome was diagnosed with heart, liver and kidney involvement. The patient recovered under pulse steroids, intravenous heparin and intravenous immunoglobulins.

  2. 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...

  3. Isoniazid Induced Lupus Presenting as Oral Mucosal Ulcers with Pancytopenia

    OpenAIRE

    Shah, Ria; Ankale, Padmaraj; Sinha, Kanishk; Iyer, Aparna; Jayalakshmi, T. K.

    2016-01-01

    Drug Induced Lupus Erythematous (DILE) is a rare adverse reaction to a large variety of drugs including Isoniazid (INH), with features resembling idiopathic Systemic Lupus Erythematosus (SLE). Diagnosis require identification of a temporal relationship between drug administered and symptom. It is an idiosyncratic reaction, with no pre-existing lupus. Our case highlights a rare presentation of isoniazid induced lupus with profound pancytopenia and mucosal ulcers, thus posing a diagnostic chall...

  4. 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.

  5. Steroid-dependent nephrotic syndrome in lupus nephritis. Response to chlorambucil.

    Science.gov (United States)

    Abuelo, J G; Esparza, A R; Garella, S

    1984-12-01

    Nephrotic syndrome associated with mesangial lupus nephritis developed in a young woman. The heavy proteinuria exhibited a striking steroid-dependent course during a three-year period of time, with ten relapses occurring whenever attempts were made to withdraw prednisone therapy. A prolonged remission was induced by the administration of chlorambucil.

  6. Tumor necrosis factor-alpha inhibitor-induced lupus-like syndrome presenting as fever of unknown origin in a liver transplant recipient: case report and concise review of the literature.

    Science.gov (United States)

    Page, A V; Liles, W C

    2008-06-01

    A 44-year-old man was admitted to the hospital with fever and myalgias 11 years after deceased donor liver transplantation for primary sclerosing cholangitis associated with ulcerative colitis. During hospitalization, he developed anemia, thrombocytopenia, and serositis. An extensive series of investigations eliminated infectious, malignant, thrombotic, and metabolic causes of fever. Because the patient had received tumor necrosis factor (TNF)-alpha inhibitor therapy for refractory pouchitis, a diagnosis of TNF-alpha inhibitor-induced lupus-like syndrome was considered. Further evaluation revealed an elevated antinuclear antibody titer of 1:640. Following discontinuation of the TNF-alpha inhibitor and a brief course of systemic corticosteroid therapy, the patient's symptoms resolved. TNF-alpha inhibitor therapy is increasingly used for posttransplantation management of inflammatory bowel disease, and drug-induced lupus is an increasingly recognized complication of such therapy. Because TNF-alpha inhibitor-induced lupus may not be recognized due to its nonspecific symptoms and the potential coexisting illnesses present in transplant recipients, a high index of suspicion is required.

  7. New developments in lupus-associated antiphospholipid syndrome

    NARCIS (Netherlands)

    Lockshin, M. D.; Derksen, R. H. W. M.

    2008-01-01

    Systemic lupus erythematosus is the disease in which the antiphospholipid syndrome was first described more than 20 years ago and which is the most frequent underlying disorder in secondary antiphospholipid syndrome. With respect to pathogenic concepts and treatment, the subjects of this review, no

  8. New developments in lupus-associated antiphospholipid syndrome

    NARCIS (Netherlands)

    Lockshin, M. D.; Derksen, R. H. W. M.

    2008-01-01

    Systemic lupus erythematosus is the disease in which the antiphospholipid syndrome was first described more than 20 years ago and which is the most frequent underlying disorder in secondary antiphospholipid syndrome. With respect to pathogenic concepts and treatment, the subjects of this review, no

  9. Type B insulin resistance syndrome induced by systemic lupus erythematosus and successfully treated with intravenous immunoglobulin: case report and systematic review.

    Science.gov (United States)

    Zhang, Sigong; Wang, Guochun; Wang, Jinping

    2013-02-01

    Type B insulin resistance syndrome is characterized by the formation of autoantibodies against insulin receptors, which can cause severe hyperglycemia and insulin resistance. Systemic lupus erythematosus is the most common underlying diseases of the syndrome. This report details our study of a case involving a Chinese female with type B insulin resistance syndrome as well as systemic lupus erythematosus who completely recovered after undergoing immunosuppressive therapy, specifically pulse therapy utilizing intravenous immunoglobulin. We also conducted search in MEDLINE and Chinese BioMedicine database to identify relevant literatures published in the past 46 years. From our searches, six case reports in Chinese, 15 case reports, and a 28-year perspective article in English met our criteria; a total of 67 cases were included in our report. The mean age of subjects at presentation for groups A, B, and C were 42.95, 44.10, and 41.68 years, respectively, yielding no significant difference between these groups. African Americans were the most susceptible group to type B insulin resistance syndrome, followed by Asians representing 20.90 % of all cases. Comparisons between the three main racial groups surveyed indicated that the mean age of subjects at presentation were very contiguous for African Americans and Asians, and mean age of white people was remarkably higher than either of the first two groups. The syndrome appeared most common among Asian males, and white males were relatively less likely to suffer from type B insulin resistance syndrome. Hypoglycemia was most commonly observed in white people than in other racial groups. Hypoalbuminemia, elevated serum immunoglobulin G, and elevated sedimentation rates were more common in African Americans; Asian cases were more likely to show low serum C3 or C4 and nephritis. Two cases received intravenous immunoglobulin therapy, which has a remarkably rapid effect on insulin resistance.

  10. 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 ...

  11. 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.

  12. Episcleritis Related to Drug-Induced Lupus Erythematosus following Infliximab Therapy: A Case Report

    Directory of Open Access Journals (Sweden)

    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.

  13. Lupus-like histopathology in bloom syndrome: reexamining the clinical and histologic implications of photosensitivity.

    Science.gov (United States)

    McGowan, Joseph; Maize, John; Cook, Joel

    2009-12-01

    Bloom syndrome is a rare genodermatosis of autosomal recessive inheritance. Although lupus-like skin lesions characterize this disorder, mechanisms of photosensitivity are poorly understood. In this case presentation, the authors report a patient with Bloom syndrome whose lupus-like facial rash revealed striking histopathologic similarities to cutaneous lupus erythematosus.

  14. Bilateral acute lupus pneumonitis in a case of rhupus syndrome

    Directory of Open Access Journals (Sweden)

    Supriya Sarkar

    2012-01-01

    Full Text Available Rhupus syndrome, the overlap of rheumatoid arthritis (RA and systemic lupus erythematosus (SLE, is an extremely uncommon condition. Organ damages found due to SLE are usually mild in rhupus. Lupus pneumonitis in rhupus syndrome has not been reported worldwide. We are reporting a 23-year-old female with bilateral symmetric erosive arthritis, oral ulcer, alopecia, polyserositis, anemia, leucopenia, positive RA-factor, anti nuclear antibody (ANA and anti ds-DNA. She presented with acute onset dyspnea, high fever, chest pain, tachycardia, tachypnea, hypoxia and respiratory alkalosis. High resolution computed tomography (HRCT-thorax showed bilateral, basal consolidation with air bronchogram. Repeated sputum and single broncho alveolar lavage (BAL fluid examination revealed no organism or Hemosiderin-laden macrophage. The diagnosis of rhupus was confirmed by combined manifestations of RA and SLE, and the diagnosis of acute lupus pneumonitis was established by clinico-radiological picture and by excluding other possibilities.

  15. Bilateral acute lupus pneumonitis in a case of rhupus syndrome.

    Science.gov (United States)

    Sarkar, Supriya; Saha, Kaushik

    2012-07-01

    Rhupus syndrome, the overlap of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), is an extremely uncommon condition. Organ damages found due to SLE are usually mild in rhupus. Lupus pneumonitis in rhupus syndrome has not been reported worldwide. We are reporting a 23-year-old female with bilateral symmetric erosive arthritis, oral ulcer, alopecia, polyserositis, anemia, leucopenia, positive RA-factor, anti nuclear antibody (ANA) and anti ds-DNA. She presented with acute onset dyspnea, high fever, chest pain, tachycardia, tachypnea, hypoxia and respiratory alkalosis. High resolution computed tomography (HRCT)-thorax showed bilateral, basal consolidation with air bronchogram. Repeated sputum and single broncho alveolar lavage (BAL) fluid examination revealed no organism or Hemosiderin-laden macrophage. The diagnosis of rhupus was confirmed by combined manifestations of RA and SLE, and the diagnosis of acute lupus pneumonitis was established by clinico-radiological picture and by excluding other possibilities.

  16. 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.

  17. Shrinking lung syndrome complicating pediatric systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Burns, Natalie S. [University of Washington Medical Center, Department of Radiology, Seattle, WA (United States); Stevens, Anne M. [Seattle Children' s Hospital, Division of Rheumatology, Department of Pediatrics, Seattle, WA (United States); Iyer, Ramesh S. [University of Washington School of Medicine, Seattle Children' s Hospital, Department of Radiology, Seattle, WA (United States)

    2014-10-15

    Systemic lupus erythematosis (SLE) can affect the lungs and pleura, usually manifesting with pleural effusions or diffuse parenchymal disease. A rare manifestation of SLE is shrinking lung syndrome, a severe restrictive respiratory disorder. While pleuropulmonary complications of pediatric SLE are common, shrinking lung syndrome is exceedingly rare in children. We present a case of a 13-year-old girl previously diagnosed with lupus, who developed severe dyspnea on exertion and restrictive pulmonary physiology. Her chest radiographs on presentation demonstrated low lung volumes, and CT showed neither pleural nor parenchymal disease. Fluoroscopy demonstrated poor diaphragmatic excursion. While shrinking lung syndrome is described and studied in adults, there is only sparse reference to shrinking lung syndrome in children. (orig.)

  18. What Do I Need to Know about Sjogren's Syndrome and Lupus?

    Science.gov (United States)

    ... I need to know about Sjögren’s Syndrome and lupus? Sjögren’s syndrome (SS) is a chronic autoimmune disorder in ... establish the diagnosis. Associations of Sjögren's Syndrome with Lupus and Other Disorders The coexistence of Sjögren's syndrome ...

  19. 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.

  20. 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.

  1. 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.

  2. Carcinogenicity, allergenicity, and lupus-inducibility of arylamines.

    Science.gov (United States)

    Chung, King-Thom

    2016-01-01

    Arylamines are widely used in food, drugs, and cosmetics as well as other industries. These chemicals are present ubiquitously in cigarette smoke, smoke emitted from cooking fume hoods as well as are generated by diverse industries. Arylamines can be generated by cleavage of azo dyes by intestinal and skin microbiota. Some arylamines are used as drugs while others are constituents of human metabolism. Many of the arylamines are mutagenic and carcinogenic. They are generally recognized as the major cause of human bladder cancer, but arylamines can induce cancers of other organs in humans and animals. Some arylamines are allergenic, causing lupus like syndrome, or other maladies. In view of their unbiquitious nature and the diseases they cause, arylamines are probably the most important chemicals causing health problems.

  3. Wünderlich syndrome from lupus-associated vasculitis.

    Science.gov (United States)

    Chao, Chia-Ter; Wang, Wei-Jie; Ting, Jui-Tsung

    2013-01-01

    Spontaneous perinephric hemorrhage is a clinically rare life-threatening condition, also known as Wünderlich syndrome. Presentations can be variable, but patients typically display symptoms and signs including flank or abdominal pain, abdominal mass, and hypovolemia. It is important to diagnose the syndrome early because untreated cases carry a high mortality risk, and prompt intervention with an endovascular procedure or surgery is life saving. Causes range from anatomical anomalies, including vascular diseases (vasculitides and aneurysms) and renal tumors, to functional coagulation defect (bleeding tendency). The most common causes of Wünderlich syndrome are renal angiomyolipoma and renal cell carcinoma, constituting 60%~70% of cases. Vascular causes of Wünderlich syndrome are infrequent, and the culprit most frequently is vasculitis resulting from polyarteritis nodosa. Other vasculitides presenting as Wünderlich syndrome are infrequent. We describe a 39-year-old woman with end-stage renal disease from lupus nephritis and spontaneous renal hemorrhage, ascribed to lupus-related vasculitis after serologic testing, computed tomography, and angiographic studies.

  4. Drug-induced subacute cutaneous lupus erythematosus associated with nab-paclitaxel therapy.

    Science.gov (United States)

    Lamond, N W D; Younis, T; Purdy, K; Dorreen, M S

    2013-10-01

    Drug-induced lupus erythematosus (dile) syndromes are documented complications of chemotherapeutic agents, including paclitaxel. Subacute cutaneous lupus erythematosus (scle) is a distinct dile syndrome presenting with characteristic annular or papulosquamous skin lesions in a photosensitive distribution with associated high anti-ssa titres. Previously, dile syndromes complicating paclitaxel therapy have been attributed to polyethoxylated castor oil (Kolliphor EL: BASF, Ludwigshafen, Germany), the biologic solvent included in the drug's original formulation (Taxol: Bristol-Myers Squibb, Montreal, QC), rather than the parent chemotherapy molecule. Here, we report a characteristic case of drug-induced scle complicating treatment with nanoparticle albumin bound (nab)-paclitaxel (Abraxane: Celgene, Summit, NJ, U.S.A.), a solvent-free taxane formulation. The pertinent English-language literature is also discussed. This case report is the first to link solvent-free paclitaxel with scle, and it suggests that the parent molecule is responsible for the reaction.

  5. Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus.

    Science.gov (United States)

    Tincani, A; Rebaioli, C B; Taglietti, M; Shoenfeld, Y

    2006-10-01

    Cardiac involvement is one of the main complications substantially contributing to the morbidity and mortality of patients suffering from systemic autoimmune diseases. All the anatomical heart structures can be affected, and multiple pathogenic mechanisms have been reported. Non-organ-specific autoantibodies have been implicated in immune complex formation and deposition as the initial triggers for inflammatory processes responsible for Libman-Sacks verrucous endocarditis, myocarditis and pericarditis. Anti-phospholipid antibodies have been associated with thrombotic events in coronary arteries, heart valve involvement and intra-myocardial vasculopathy in the context of primary and secondary anti-phospholipid syndrome. Antibodies-SSA/Ro and anti-SSB/La antigens play a major pathogenic role in affecting the heart conduction tissue leading to the electrocardiographic abnormalities of the neonatal lupus syndrome and have been closely associated with endocardial fibroelastosis.

  6. Lupus in a patient with cystinosis: is it drug induced?

    Science.gov (United States)

    Eroglu, F K; Besbas, N; Ozaltin, F; Topaloglu, R; Ozen, S

    2015-11-01

    A 9-year-old girl with a diagnosis of cystinosis since 2 years of age, on cysteamine therapy, presented with complaints of serositis and arthritis, and laboratory tests revealed high antinuclear antibody titers with hypocomplementemia. Kidney biopsy was not consistent with lupus nephritis. With prednisolone treatment her complaints resolved and creatinine level decreased, but on follow-up, serological features of systemic lupus erythematosus (SLE) continued. Six years after cessation of prednisolone, lupus features were reactivated, with positive antihistone antibodies and ANCA. Coincidence of cystinosis and SLE is very rare, and to the best of our knowledge this is the fourth case reported in the literature. Physicians should be aware that cystinosis patients may have some autoimmune manifestations with features of true or drug-induced lupus. In the light of this case, pathophysiology and treatment are discussed.

  7. The features of skin inflammation induced by lupus serum.

    Science.gov (United States)

    Liu, Lena; Xu, Guangqion; Dou, Hui; Deng, Guo-Min

    2016-04-01

    We recently developed a model of lupus serum-induced skin inflammation, which was used to study the pathogenesis of skin injury in systemic lupus erythematosus (SLE). We further characterized the features of lupus serum-induced skin inflammation. This skin inflammation was evident within 3h and lasted for at least two weeks. The skin inflammation was characterized by an influx of monocytic, CD11b+cells and by a scarcity of T and B lymphocytes. Depletion of IgG from the serum abrogated the skin inflammatory response. The skin inflammation was related to lupus patients' skin history but not to SLE disease activity and type of autoantibody. The expression of TNFR1, NF-kB and MCP-1 was increased locally in skin lesions. The TLR9 ligand and lupus serum act synergistically to trigger skin inflammation. These findings suggest that this novel model is valuable for the study of the pathogenesis and therapy of skin injury in SLE.

  8. 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 ...

  9. 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.

  10. 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

  11. Development of Reversible Posterior Leukoencephalopathy Syndrome after Cyclophosphamide Treatment in a Patient with Lupus Nephritis

    Directory of Open Access Journals (Sweden)

    Serkan YILDIZ

    2011-01-01

    Full Text Available Renal involvement in systemic lupus erythematosus is a frequent and serious complication that significantly increases morbidity and mortality. Despite all studies and usage of new drugs, treatment of lupus nephritis continues to be a problem. Diffuse proliferative lupus nephritis has a poor prognosis and aggressive treatment must be undertaken. Cyclophosphamide is commonly used in treatment despite its side effects. Reversible posterior leucoencephalopathy syndrome is a clinico-radiological syndrome manifested by blood pressure elevation, headache, visual disturbances, confusion, seizures and sometimes focal neurological signs that can develop due to usage of cytotoxic drugs. We present a case of lupus nephritis in which reversible posterior leucoencephalopathy syndrome developed after intravenous cyclophosphamide administration and recovered spontaneously by symptomatic treatment in this article.

  12. 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.

  13. 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

  14. [In vitro fertilization and systemic lupus erythematosus or antiphospholipid syndrome: An update].

    Science.gov (United States)

    Orquevaux, P; Masseau, A; Le Guern, V; Gayet, V; Vauthier, D; Boutin, D; Wechsler, B; Morel, N; Guettrot-Imbert, G; Pennaforte, J-L; Piette, J-C; Costedoat-Chalumeau, N

    2015-03-01

    Fertility is not impaired in systemic lupus erythematosus or antiphospholipid syndrome, but, similarly to the general population, these patients may undergo in vitro fertilization. This type of treatment increases the risk of lupus flare, thrombosis, and ovarian hyperstimulation syndrome. This review will focus on in vitro fertilization in systemic lupus erythematosus or antiphospholipid syndrome. Literature data are relatively scant with only 3 reported studies. The first one included 17 patients and 63 cycles of induction ovulation/in vitro fertilization leading to 25 % of lupus flare, no thrombosis, and 3 % of ovarian hyperstimulation syndrome. The second study included 10 patients and 40 cycles of in vitro fertilization showing 31 % of lupus flare, no thrombosis and no ovarian hyperstimulation syndrome. The last one included 34 patients and 83 procedures of in vitro fertilization leading to 8 % of flares, 5 % of thrombosis and no ovarian hyperstimulation syndrome. Interestingly, in this last study, half of the complications were explained by poor adherence to treatment. These data are reassuring but it is important to remember that in vitro fertilization should be scheduled and carefully supervised in the same way as the high-risk pregnancies occurring in these patients.

  15. Headache, Raynaud's syndrome and serotonin receptor agonists in systemic lupus erythematosus.

    Science.gov (United States)

    Bernatsky, S; Pineau, C A; Lee, J L; Clarke, A E

    2006-01-01

    There are potential concerns regarding serotonin receptor agonists in SLE patients with migraine, particularly patients with concomitant Raynaud's syndrome. We estimated the prevalence of lupus-related headache and Raynaud's syndrome in the Montreal General Hospital SLE clinic cohort and evaluated the relationship between these two variables in multivariable logistic regression models, controlling for age, sex, race, SLE duration and the presence of lupus anticoagulant and antibodies to cardiolipin and beta2 glycoprotein I. We also assessed, through chart review in those individuals with both Raynaud's syndrome and migraine, a history of serotonin receptor agonist use, and any associated worsening vasospasm. Based on Systemic Lupus Activity Measure (SLAM) scores, the cumulative incidence of lupus-related headache in our sample (n = 391) was 46.1%; the prevalence of Raynaud's syndrome was 49.4%. The adjusted odds ratio (OR) for lupus-related headache and Raynaud's syndrome was 1.7 (95% CI 1.1, 2.5). In addition, there was a strong independent relationship between headache and anti-beta2 glycoprotein I antibodies (adjusted OR 5.6 [95% CI 1.8, 17.0]). The data from our chart review suggest that careful use of serotonin receptor agonists in patients with both Raynaud's syndrome and migraines may be undertaken, although caution would necessitate that these agents not be used in individuals with very severe Raynaud's (eg, digital ulcerations, and so on).

  16. Induction of systemic lupus erythematosus syndrome in BALB/c mice by immunization with active chromatin

    Institute of Scientific and Technical Information of China (English)

    Hong LI; Yun-yi ZHANG; Ya-nan SUN; Xi-yi HUANG; Yong-feng JIA; Duan LI

    2004-01-01

    AIM: To establish an animal model for systemic lupus erythematosus (SLE)-like syndrome in mice. METHODS:BALB/c mice were immunized with active chromatin isolated from ConA-actived syngeneic spleno-lymphocytes.Plasma samples of mice were tested by enzyme-linked immunosorbent assays (ELISA) for the presence of IgG anti-dsDNA, -ssDNA, and anti-histone antibodies. Tumor necrosis factor-α (TNF-α) in serum was measured by ELISA. Spleno-lymphocyte proliferation assays and the levels of interferon-γ (IFN-γ) in supernatants were tested respectively. Proteinuria was measured. Kidneys were examined by direct immunohistochemical method and light microscopy. RESULTS: Anti-ds DNA, ssDNA, and histone antibodies were induced in active chromatin-immunized mice, the proliferation response of splenocytes to ConA and LPS were reduced, levels of interferon-γ in supernatants and TNF-α in serum were lowered. Lupus nephritis was assessed by the presence of Ig deposits,glomerular pathology and proteinuria. CONCLUSION: The active chromatin-induced SLE-like mouse model was similar to idiopathic SLE in human.

  17. 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

  18. Systemic lupus erythematosus and Wiskott-Aldrich syndrome in an Italian patient

    NARCIS (Netherlands)

    Monteferrante, G.; Giani, M.; van den Heuvel, M. C.

    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

  19. 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

  20. Resveratrol possesses protective effects in a pristane-induced lupus mouse model.

    Directory of Open Access Journals (Sweden)

    Zhuo-Long Wang

    Full Text Available Systemic lupus erythematosus (SLE is a multisystemic autoimmune disease characterized by the production of autoantibodies. To date, no therapy has been found to satisfactorily treat SLE. SIRT1 deficiency results in the development of an autoimmune syndrome in mice, including a high titer of anti-nuclear antibody in serum, immunoglobulin deposition in the kidney, and immune complex glomerulonephritis. Resveratrol is an activator of SIRT1 and possesses anti-inflammation and immune-regulatory properties.To evaluate the preventative effects of resveratrol on a pristane-induced lupus animal model and assess its putative immune modulation effects.BALB/c mice received a single intraperitoneal injection of 0.5 ml of pristane on day 1 and then various doses of resveratrol were given to the mice daily starting on day 2 and continuing for seven months. The autoantibodies in serum and supernatants were measured. Single cells isolated from spleen, isolated CD4+ T cells, and CD19+ B cells were cultured with or without resveratrol in vitro and assessed by flow cytometry.Resveratrol attenuated proteinuria, immunoglobuin depositon in kidney, and glomerulonephritis as well as IgG1 and IgG2a in serum in pristane-induced lupus mice. Resveratrol also suppressed CD69 and CD71 expression on CD4+ T cells as well as CD4+ T cell proliferation, induced CD4+ T cell apoptosis, and decreased CD4 IFNγ+ Th1 cells and the ratio of Th1/Th2 cells in vitro. In vitro antibody production and proliferation of B cells were also inhibited.Resveratrol possesses protective effects in pristane-induced lupus mice and may represent a novel approach for the management of SLE.

  1. Papulonodular mucinosis, Guillain-Barré syndrome and nephrotic syndrome in a patient with systemic lupus erythematosus: a case report

    OpenAIRE

    Su, Xiaole; Qiao, Xi; Li, Jing; Gao, Lifang; Wang, Chen; WANG Lihua

    2017-01-01

    Background Awareness of the spectrum of clinical manifestations of systemic lupus erythematosus (SLE), especially uncommon changes, is essential for diagnosis and effective management of patients. Case presentation A 26-year-old Chinese man with SLE initially manifested cutaneous papulonodular mucinosis and developed acute Guillain-Barré syndrome and class V lupus nephritis 2 years later. His cutaneous nodules had not been idententified for 2 years and were resected by surgical procedures twi...

  2. Minocycline-induced clinical and biological lupus-like disease.

    Science.gov (United States)

    Tournigand, C; Généreau, T; Prudent, M; Diemert, M C; Herson, S; Chosidow, O

    1999-01-01

    A 14-year-old girl developed maculopapular rash, myalgias, arthralgias and myocarditis with elevated anti-nuclear and anti-double-stranded DNA antibodies. She was taking minocycline for acne and all symptoms resolved when this treatment was stopped. The patient has no evidence of disease one year after onset of symptoms. Clinicians should be aware of minocycline's responsibility in inducing lupus-like disease.

  3. 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.

  4. Kallikrein genes are associated with lupus and glomerular basement membrane–specific antibody–induced nephritis in mice and humans

    Science.gov (United States)

    Liu, Kui; Li, Quan-Zhen; Delgado-Vega, Angelica M.; Abelson, Anna-Karin; Sánchez, Elena; Kelly, Jennifer A.; Li, Li; Liu, Yang; Zhou, Jinchun; Yan, Mei; Ye, Qiu; Liu, Shenxi; Xie, Chun; Zhou, Xin J.; Chung, Sharon A.; Pons-Estel, Bernardo; Witte, Torsten; de Ramón, Enrique; Bae, Sang-Cheol; Barizzone, Nadia; Sebastiani, Gian Domenico; Merrill, Joan T.; Gregersen, Peter K.; Gilkeson, Gary G.; Kimberly, Robert P.; Vyse, Timothy J.; Kim, Il; D’Alfonso, Sandra; Martin, Javier; Harley, John B.; Criswell, Lindsey A.; Wakeland, Edward K.; Alarcón-Riquelme, Marta E.; Mohan, Chandra

    2009-01-01

    Immune-mediated nephritis contributes to disease in systemic lupus erythematosus, Goodpasture syndrome (caused by antibodies specific for glomerular basement membrane [anti-GBM antibodies]), and spontaneous lupus nephritis. Inbred mouse strains differ in susceptibility to anti-GBM antibody–induced and spontaneous lupus nephritis. This study sought to clarify the genetic and molecular factors that may be responsible for enhanced immune-mediated renal disease in these models. When the kidneys of 3 mouse strains sensitive to anti-GBM antibody–induced nephritis were compared with those of 2 control strains using microarray analysis, one-fifth of the underexpressed genes belonged to the kallikrein gene family, which encodes serine esterases. Mouse strains that upregulated renal and urinary kallikreins exhibited less evidence of disease. Antagonizing the kallikrein pathway augmented disease, while agonists dampened the severity of anti-GBM antibody–induced nephritis. In addition, nephritis-sensitive mouse strains had kallikrein haplotypes that were distinct from those of control strains, including several regulatory polymorphisms, some of which were associated with functional consequences. Indeed, increased susceptibility to anti-GBM antibody–induced nephritis and spontaneous lupus nephritis was achieved by breeding mice with a genetic interval harboring the kallikrein genes onto a disease-resistant background. Finally, both human SLE and spontaneous lupus nephritis were found to be associated with kallikrein genes, particularly KLK1 and the KLK3 promoter, when DNA SNPs from independent cohorts of SLE patients and controls were compared. Collectively, these studies suggest that kallikreins are protective disease-associated genes in anti-GBM antibody–induced nephritis and lupus. PMID:19307730

  5. 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.

  6. 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.

  7. Lupus anticoagulant-hypoprothrombinemia syndrome: report of two cases and review of the literature.

    Science.gov (United States)

    Mulliez, S M N; De Keyser, F; Verbist, C; Vantilborgh, A; Wijns, W; Beukinga, I; Devreese, K M J

    2015-06-01

    Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare acquired disorder caused by prothrombin antibodies. The disease is most common in the pediatric age group (lupus erythematosus (SLE) and viral infections. The clinical manifestation of LA-HPS varies greatly in severity and it may cause severe life-threatening bleeding diathesis. LA-HPS is to be suspected when a patient presents with bleeding and a prolonged activated partial thromboplastin and prothrombin time, in combination with a lupus anticoagulant. The diagnosis is confirmed in the laboratory by identification of reduced prothrombin levels. There are no standardized recommendations for treatment of the hemorrhage associated with the syndrome; corticosteroids are used as first-line treatment. This review summarizes what is currently known about the pathogenesis, clinical features, diagnosis, treatment and prognosis of LA-HPS, and presents two case reports.

  8. 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.

  9. MR imaging findings suggestive of posterior reversible encephalopathy syndrome in adolescents with systemic lupus erythematosus

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    Muscal, Eyal; De Guzman, Marietta M.; Myones, Barry L. [Texas Children' s Hospital, Baylor College of Medicine and Pediatric Rheumatology Center, Houston, TX (United States); Traipe, Elfrides; Hunter, Jill V. [Texas Children' s Hospital, Baylor College of Medicine and Diagnostic Imaging, Houston, TX (United States); Brey, Robin L. [University of Texas Health Science Center at San Antonio, Department of Neurology, San Antonio, TX (United States)

    2010-07-15

    Endothelial damage, hypertension and cytotoxic medications may serve as risk factors for the posterior reversible encephalopathy syndrome (PRES) in systemic lupus erythematosus. There have been few case reports of these findings in pediatric lupus patients. We describe clinical and neuroimaging findings in children and adolescents with lupus and a PRES diagnosis. We identified all clinically acquired brain MRIs of lupus patients at a tertiary care pediatric hospital (2002-2008). We reviewed clinical features, conventional MRI and diffusion-weighted imaging (DWI) findings of patients with gray- and white-matter changes suggestive of vasogenic edema and PRES. Six pediatric lupus patients presenting with seizures and altered mental status had MRI findings suggestive of PRES. In five children clinical and imaging changes were seen in conjunction with hypertension and active renal disease. MRI abnormalities were diffuse and involved frontal regions in five children. DWI changes reflected increased apparent diffusivity coefficient (unrestricted diffusion in all patients). Clinical and imaging changes significantly improved with antihypertensive and fluid management. MRI changes suggestive of vasogenic edema and PRES may be seen in children with active lupus and hypertension. The differential diagnosis of seizures and altered mental status should include PRES in children, as it does in adults. (orig.)

  10. Post-partum bilateral renal cortical necrosis in antiphospholipid syndrome and systemic lupus erythematosus

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    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.

  11. A lupus-like syndrome develops in mice lacking the Ro 60-kDa protein, a major lupus autoantigen.

    Science.gov (United States)

    Xue, Dahai; Shi, Hong; Smith, James D; Chen, Xinguo; Noe, Dennis A; Cedervall, Tommy; Yang, Derek D; Eynon, Elizabeth; Brash, Douglas E; Kashgarian, Michael; Flavell, Richard A; Wolin, Sandra L

    2003-06-24

    Antibodies against a conserved RNA-binding protein, the Ro 60-kDa autoantigen, occur in 24-60% of all patients with systemic lupus erythematosus. Anti-Ro antibodies are correlated with photosensitivity and cutaneous lesions in these patients and with neonatal lupus, a syndrome in which mothers with anti-Ro antibodies give birth to children with complete congenital heart block and photosensitive skin lesions. In higher eukaryotes, the Ro protein binds small RNAs of unknown function known as Y RNAs. Because the Ro protein also binds misfolded 5S rRNA precursors, it is proposed to function in a quality-control pathway for ribosome biogenesis. Consistent with a role in the recognition or repair of intracellular damage, an orthologue of Ro in the radiation-resistant eubacterium Deinococcus radiodurans contributes to survival of this bacterium after UV irradiation. Here, we show that mice lacking the Ro protein develop an autoimmune syndrome characterized by anti-ribosome antibodies, anti-chromatin antibodies, and glomerulonephritis. Moreover, in one strain background, Ro-/- mice display increased sensitivity to irradiation with UV light. Thus, one function of this major human autoantigen may be to protect against autoantibody development, possibly by sequestering defective ribonucleoproteins from immune surveillance. Furthermore, the finding that mice lacking the Ro protein are photosensitive suggests that loss of Ro function could contribute to the photosensitivity associated with anti-Ro antibodies in humans.

  12. Are Toll-Like Receptors and Decoy Receptors Involved in the Immunopathogenesis of Systemic Lupus Erythematosus and Lupus-Like Syndromes?

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    Giuliana Guggino

    2012-01-01

    Full Text Available In this paper we focus our attention on the role of two families of receptors, Toll-like receptors (TLR and decoy receptors (DcR involved in the generation of systemic lupus erythematosus (SLE and lupus-like syndromes in human and mouse models. To date, these molecules were described in several autoimmune disorders such as rheumatoid arthritis, antiphospholipids syndrome, bowel inflammation, and SLE. Here, we summarize the findings of recent investigations on TLR and DcR and their role in the immunopathogenesis of the SLE.

  13. Epilepsy as part of systemic lupus erythematosus and systemic antiphospholipid syndrome (Hughes syndrome).

    Science.gov (United States)

    Cimaz, R; Meroni, P L; Shoenfeld, Y

    2006-01-01

    The antiphospholipid syndrome (APS) is defined by the presence of antiphospholipid antibodies (aPL), demonstrated by ELISAs for antibodies against phospholipids and associated phospholipid-binding cofactor proteins and/or a circulating lupus anticoagulant (LA) together with diverse systemic clinical manifestations such as thrombosis, and recurrent spontaneous abortions. According to the criteria set out in Sydney the only neurological manifestations that can be suitable as APS classification criteria are ischemic events (stroke and transient ischemic attacks). However, other neurological manifestations, including seizures in particular, have been repeatedly reported in APS patients. The present review will summarize recent research on the association of aPL, as well as other autoantibodies, with seizure disorders, with or without concomitant SLE.

  14. Anesthetic management of patient with systemic lupus erythematosus and antiphospholipid antibodies syndrome for laparoscopic nephrectomy and cholecystectomy

    Science.gov (United States)

    Khokhar, Rashid Saeed; Baaj, Jumana; Al-Saeed, Abdulhamid; Sheraz, Motasim

    2015-01-01

    We report a case of a female having systemic lupus erythematosus and antiphospholipid antibodies syndrome, who was on immunosuppressant therapy. We discussed the preoperative evaluation and perioperative management who underwent nephrectomy and cholecystectomy. PMID:25558207

  15. Anesthetic management of patient with systemic lupus erythematosus and antiphospholipid antibodies syndrome for laparoscopic nephrectomy and cholecystectomy

    Directory of Open Access Journals (Sweden)

    Rashid Saeed Khokhar

    2015-01-01

    Full Text Available We report a case of a female having systemic lupus erythematosus and antiphospholipid antibodies syndrome, who was on immunosuppressant therapy. We discussed the preoperative evaluation and perioperative management who underwent nephrectomy and cholecystectomy.

  16. Pristane-induced lupus: considerations on this experimental model.

    Science.gov (United States)

    Freitas, Eduarda Correa; de Oliveira, Mayara Souza; Monticielo, Odirlei André

    2017-09-06

    Systemic lupus erythematosus (SLE) is a multifactorial, autoimmune inflammatory disease with pleomorphic clinical manifestations involving different organs and tissues. The etiology of this disease has been associated with a dysfunctional response of B and T lymphocytes against environmental stimuli in individuals genetically susceptible to SLE, which determines an immune response against different autoantigens and, consequently, tissue damage. The study of different murine models has provided a better understanding of these autoimmune phenomena. This review primarily focuses on that has been learned from the pristane-induced lupus (PIL) model and how this model can be used to supplement recent advances in understanding the pathogenesis of SLE. We also consider both current and future therapies for this disease. The PubMed, SciELO, and Embase databases were searched for relevant articles published from 1950 to 2016. PIL has been shown to be a useful tool for understanding the multiple mechanisms involved in systemic autoimmunity. In addition, it can be considered an efficient model to evaluate the environmental contributions and interferon signatures present in patients with SLE.

  17. Catastrophic Antiphospholipid Syndrome Presenting as Ischemic Pancreatitis in Systemic Lupus Erythematosus

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    Ajit Vyas

    2009-09-01

    Full Text Available Context Antiphospholipid syndrome is often associated with systemic lupus erythematosus. Both syndromes have different clinical manifestations based on organ involvement. Antiphospholipid syndrome commonly causes spontaneous abortions, cerebral vascular occlusion, and deep venous thrombosis. Catastrophic antiphospholipid syndrome occurs when three or more organ systems are affected by thromboses in less than a week. Case report We report a unique case of a young woman with a history of systemic lupus erythematosus and antiphospholipid syndrome who presented with recurrent ischemic pancreatitis. Pancreatitis was refractory to anticoagulation and low dose steroids. Secondary to recurrence of pancreatitis and other organ involvement, she was treated as a presumed case of catastrophic antiphospholipid syndrome. Aggressive treatment with plasmapheresis, corticosteroids, cyclophosphamide, and anticoagulation eventually led to her recovery. Conclusion Awareness of this rare, rapidly fatal medical condition prompts vital, early intervention to improve patients’ survival. This case report aims to add to the limited therapeutic data available as well as suggest a possible approach to treating this rare syndrome with very high morbidity and mortality.

  18. Severe cognitive dysfunction and shrinking lung syndrome in systemic lupus erythematous

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    Breno José Alencar Pires Barbosa

    2014-12-01

    Full Text Available Systemic lupus erythematosus (SLE is an autoimmune disease that can affect any organ or system. Neuropsychiatric and pulmonary involvement can occur in 40 and 50% of patients respectively, and may occur in several different clinical forms. While the main neuropsychiatric manifestations are represented by cognitive impairment, organic cerebral syndromes, delirium, psychosis, seizures, and peripheral neuropathies, the main forms of pulmonary involvement are pleurisy with or without pleural effusion, pneumonitis, interstitial disease, pulmonary hypertension, and alveolar hemorrhage. The authors report the case of a 49-year-old woman whose first manifestation of SLE was represented by two rare manifestations: rapidly progressive cognitive impairment, which was associated with respiratory failure caused by the shrinking lung syndrome. The authors call attention to the under-diagnosis of lupus pulmonary complications and its association with severe cognitive impairment that often necessitates aggressive treatment.

  19. Therapeutic management of evans syndrome in a pregnancy with maternal systemic lupus erythematosus.

    Science.gov (United States)

    Nause, S L; Spiegler, J; Weichert, J; Hartge, D R

    2015-08-01

    A 31-year-old 2 G 1 P was referred to our unit of prenatal medicine at 35+3 weeks of gestation with a spontaneously conceived singleton pregnancy of a female fetus without detectable anomalies. Maternal hematological evaluation revealed an Evans-syndrome-related thrombocytopenia based on a lupus erythematosus. The former delivery was aggravated by a severe hemorrhage several years before. Anti-autoimmunologic therapy was started and maternal platelets count increased to physiological values. Uneventful ceasarean section was performed at 37 weeks of gestation with favourable outcome for mother and child. This case is the first report of a successful therapy in maternal Evans syndrome in pregnancy combined with a lupus erythematosus.

  20. Calcified right intraventricular thrombus in a patient with systemic lupus erythematous and antiphospholipid syndrome.

    Science.gov (United States)

    Bittencourt, Márcio Sommer; Seltmann, Martin; Muschiol, Gerd; Achenbach, Stephan

    2010-01-01

    A 37-year-old patient with known systemic lupus erythematous, antiphospholipid syndrome and previous pulmonary embolism presented with non-ST elevation myocardial infarction while on adequate anticoagulation therapy. The patient was further evaluated with cardiac computed tomography. A small diagonal branch occlusion was the only coronary lesion present. A partially calcified right ventricular thrombus was incidentally found. Because of the small vessel size, cardiac catheterization was deemed unnecessary, and the patient was discharged with adjustment of immunosuppressive therapy and anticoagulation.

  1. [The clinical characteristics of macrophage activation syndrome secondary to systemic lupus erythematosus].

    Science.gov (United States)

    Jiang, N; Li, M T; Wu, D; Zeng, X F

    2016-11-01

    Objective: To investigate the clinical features of macrophage activation syndrome (MAS) associated with systemic lupus erythematosus (SLE). Method: The clinical data of 15 patients with SLE-induced MAS diagnosed in Peking Union Medical College Hospital from July 2011 to December 2014 were retrospectively analyzed. Results: Fourteen patients were female. The average age was 28.07. When MAS occurred, the average duration of SLE was 20.47 months, and the average SLE disease activity index (SLEDAI) was 18.4. All 15 patients developed fever, hematocytopenia and impaired liver function in the course of MAS, while patients with splenomegaly, coagulation disorders and neuropsychiatric symptoms were 11, 14 and 8, respectively. All 15 patients presented leukocpenia and thrombocytopenia. Hypofibrinogenemia, elevated ferritin and hemophagocytosis in bone marrow were respectively observed in 7, 11 and 12 patients. Glucocorticoids were used in all patients, among whom eight received pulse methylprednisolone therapy. Thirteen patients were treated with immunosuppressants, including cyclosporine A, tacrolimus, cyclophosphamide and mycophemolate mofetil. Complete remission was achieved in 14 patients. One patient died of MAS. Conclusions: In patients with SLE, MAS was most commonly seen in young females with short SLE duration and active disease. Fever, splenomegaly, hematocytopenia, coagulation disorders and liver damage are the most remarkable clinical manifestations. Early diagnosis and intensive therapy are the key parts to improve clinical outcome.

  2. Phospholipid Syndrome and Vasculitis as a presentation of Systemic Lupus Erythematosus. Case report.

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    Sila Castellón Mortera

    2013-09-01

    Full Text Available The systemic Lupus Erythematosus is presented, generally, as a poli articular syndrome, with a long period of fever nephritico or nephrotico; other clinical ways are: neuropsychiatry, vasculitis, etc. They appeared in a progressive manner; but in rare cases as a sickness debutant. It has not being reported in Sancti Spiritus Province patients in which matches the debut of the systemic Lupus Erythematosus with the manifestations of phospholipid syndrome. A Woman with 24 years of age is hospitalized having vasculitis, articular pains, thrombose in her right foot, detecting anticoagulante lupico and possitive Rematoideo factor with periferic pattern diffused in the Inmunoelectroforesis. 5 years later was hospitalized again with poliserositis. She had a positive evolution with a dose in a month of Intacglobin and anticoagulante treatment. Two years later she was hospitalized with articular pains proving she had livedo reticular on her left knee and Raynaud phenomenon on her foot. Beta Prebeta Index and high triglycerides. Lupico anticoagulant positive again. A treatment with Intacglobin and Prednisona was given to the patient with a better clinic without being hospitalized again. There is no evidence (at 17 years of age of a sickness debut of renal dissorder. It is about a Systemic Lupus Eritematoso which debut was a vasculitis and a Phospholipid Syndrome associated.

  3. Devic syndrome associated to systemic lupus erythematosus. Case report.

    OpenAIRE

    Juan Camilo Botero; Alejandro García; Juliana Hidalgo

    2009-01-01

    El síndrome de Devic es una las enfermedades desmielinizantes del sistema nervioso central, de etiología desconocida. Se relaciona mucho con el lupus eritematoso sistémico siendo una complicación rara y grave. Este síndrome se presenta como mielitis transversa de compromiso a nivel cervical y neuritis óptica. Se describe el caso de de una paciente de 32 años, que se presentó al servicio de urgencias por dolor en región torácica, pérdida de la fuerza muscular progresiva en los miembros inferio...

  4. Ticlopidine-induced subacute cutaneous lupus erythematosus: A case report and literature review

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    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.

  5. Pediatric patient with systemic lupus erythematosus & congenital acquired immunodeficiency syndrome: An unusual case and a review of the literature

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    Rezaee Fariba

    2008-05-01

    Full Text Available Abstract The coexistence of systemic lupus erythematosus (SLE in patients with congenital human immunodeficiency virus (HIV infection is rare. This is a case report of a child diagnosed with SLE at nine years of age. She initially did well on non-steroidal anti-inflammatory agents, hydroxychloroquine, and steroids. She then discontinued her anti-lupus medications and was lost to follow-up. At 13 years of age, her lupus symptoms had resolved and she presented with intermittent fevers, cachexia, myalgias, arthralgias, and respiratory symptoms. Through subsequent investigations, the patient was ultimately diagnosed with congenitally acquired immunodeficiency syndrome (AIDS.

  6. Devic syndrome associated to systemic lupus erythematosus. Case report.

    Directory of Open Access Journals (Sweden)

    Juan Camilo Botero

    2009-11-01

    Full Text Available El síndrome de Devic es una las enfermedades desmielinizantes del sistema nervioso central, de etiología desconocida. Se relaciona mucho con el lupus eritematoso sistémico siendo una complicación rara y grave. Este síndrome se presenta como mielitis transversa de compromiso a nivel cervical y neuritis óptica. Se describe el caso de de una paciente de 32 años, que se presentó al servicio de urgencias por dolor en región torácica, pérdida de la fuerza muscular progresiva en los miembros inferiores, pérdida del control del esfínter urinario y disminución de la agudeza visual. También relató antecedentes de lupus eritematoso sistémico. Al examen físico se encontró atrofia y palidez del disco óptico derecho, disminución en la fuerza muscular 3/5 en miembros superiores e inferiores, alteración en la sensibilidad y propiocepción de extremidades inferiores. La resonancia magnética mostró hallazgos sugestivos de mielitis transversa de C2 a C6. En el laboratorio se encontraron anticuerpos anticardiolipina elevados y anticuerpos antinucleares: 1:320. Se manejó con esteroides, hubo notable mejoría en los síntomas y se le dio de alta al sexto día, con tratamiento ambulatorio. Los hallazgos al examen físico, laboratorio e imagenología permitieron hacer el diagnóstico de síndrome de Devic. Con base en la revisión bibliográfica, se concluye que este síndrome es poco frecuente; sin embargo, se debe tener en cuenta como posible diagnóstico en todo paciente con antecedentes de lupus eritematoso sistémico y síntomas compatibles.

  7. Neuropsychiatric Symptoms as The First Manifestation of Juvenile Systemic Lupus Erythematosus: A Complicated Case with Klinefelter’s Syndrome

    OpenAIRE

    Reza SHIARI; HASSASE YEGANE, Mehrnoush; Farivar, Shirin; JAVADI PARVANEH, Vadood; Seyed Alireza MIRJAVADI

    2014-01-01

    How to Cite This Article: Shiari R, Hassase Yegane M, Farivar S, Javadi Parvaneh V, Mirjavadi SA. Neuropsychiatric symptoms as the first manifestation of Juvenile Systemic Lupus Erythematosus: A complicated case with Klinefelter’s syndrome. Iran J Child Neurol. 2014 Winter; 8(1):62-65.AbstractSystemic Lupus Erythematosus (SLE) is an autoimmune, multisystem disorder with various manifestations. There are limited reports on the neuropsychiatric findings as the first manifestation of SLE in chil...

  8. [Pathogenesis and Laboratory Findings in Antiphospholipid Syndrome, Especially Associated with Lupus Anticoagulant].

    Science.gov (United States)

    Ieko, Masahiro; Naito, Sumiyoshi; Yoshida, Mika; Takahashi, Nobuhiko

    2015-10-01

    Antiphospholipid syndrome (APS), an acquired thrombotic condition, is a complex clinical state characterized by the presence of circulating antiphospholipid antibodies in patients with thrombosis or pregnancy morbidity. Revised APS classification criteria are used for diagnosis, which include at least one clinical criterion (thrombosis or pregnancy loss) and at least one of the laboratory criteria [anticardiolipin antibodies, anti-β2GPI antibodies, lupus anticoagulant (LA)]. LA is also an independent risk factor for developing thrombosis, though some LA-positive cases have been reported to have a bleeding symptom. Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare disorder characterized by a bleeding tendency due to low prothrombin activity in patients with LA, and has recently been reported not only in children but also in adults We have encountered LA cases with bleeding and low coagulation factor activities except for prothrombin. Based on our findings, we propose that LA-positive cases with a bleeding symptom and characterized by low coagulation factor activity including prothrombin be termed lupus anticoagulant-associated coagulopathy (LAAC). Furthermore, coagulation factor autoantibodies are often detected in LAAC patients; thus, correct measurement of LA is important to distinguish LAAC patients from those possessing an inhibitor to coagulation factors such as acquired hemophilia A as well as to select the optimal therapeutic strategy.

  9. Relationship between lupus anticoagulant (LAC) and pregnancy-induced hypertension.

    Science.gov (United States)

    Matsumoto, T; Sagawa, N; Ihara, Y; Kobayashi, F; Itoh, H; Mori, T

    1995-01-01

    Lupus anticoagulant (LAC), a serum antiphospholipid autoantibody, is believed to be one of the causes of infertility or fetal loss. The purpose of the present study was to evaluate the role of LAC in the pathogenesis of hypertension during pregnancy. In this study, 20 pregnant women with hypertension were classified into two groups: 14 patients who did not have hypertension before the pregnancy but developed it during the pregnancy (pregnancy-induced hypertension; Group A) and 6 patients who had hypertensive or renal disease before the pregnancy, and developed further hypertension during the pregnancy (pregnancy-aggravated hypertension; Group B). A LAC coagulation assay was performed, and the presence of LAC in each group was compared. All 14 patients in group A were LAC-negative. In contrast, 3 of the 6 patients in group B were LAC-positive, and had clinical autoimmune diseases. The incidence of pregnancy-induced hypertension was also examined in 15 pregnancies from 9 LAC-positive women who had a history of repeated fetal loss but no systemic autoimmune disease (Group C). None of these 15 pregnancies had hypertensive complications, even when they reached term. In the placentas of LAC-positive women, no characteristic changes other than fibrinoid degeneration and microscopic infarction were observed upon histological examination. These results suggest that LAC does not relate with the onset of hypertension during pregnancy.

  10. The effects of lupus and antiphospholipid antibody syndrome on foetal outcomes.

    Science.gov (United States)

    Nalli, C; Iodice, A; Andreoli, L; Lojacono, A; Motta, M; Fazzi, E; Tincani, A

    2014-05-01

    Systemic lupus erythematosus (SLE) is a multi-organ autoimmune disease that primarily affects women of childbearing-age. Antiphospholipid syndrome (APS) is a systemic autoimmune disorder defined by the occurrence of venous and arterial thrombosis, often multiple, and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). Recently, the long-term outcome of children born to patients with lupus and APS has become a major topic of interest both to patients and physicians. One of the major problems related to maternal disease is preterm delivery with all the consequences that this condition may bring. Prematurity may also be due to the presence of aPL; however, aPL do not generally display any thrombotic potential on neonates. Another complication may be neonatal lupus (NL), mediated by the presence of maternal antibodies (anti-Ro/SSA and anti-La/SSB). In addition, behaviour and neuropsychological outcomes have also been a matter of interest, but there are currently few data available. Beyond the biological influence of both maternal disease and autoimmune background, it is important to focus on the possible influence of maternal chronic illness on the neuropsychological development of her children. Whether aPL exposure could have a direct effect on brain development is still being debated. In children of mothers with APS, language delays have been noted and learning disabilities were described with a higher rate than the general age-school population. Several studies were performed on children born to lupus mothers: even if maternal lupus does not seem to impair intelligence levels, it may increase the occurrence of learning disabilities and particularly dyslexia in male children. To the best of our knowledge, no studies are available on the long-term outcome of children born to mothers with lupus or APS and particularly regarding the development of autoimmune diseases. Nevertheless, common experience of experts in the field is that these children do

  11. 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...

  12. 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.

  13. Antiglutamate Receptor Antibodies and Cognitive Impairment in Primary Antiphospholipid Syndrome and Systemic Lupus Erythematosus

    Science.gov (United States)

    Gerosa, Maria; Poletti, Barbara; Pregnolato, Francesca; Castellino, Gabriella; Lafronza, Annalisa; Silani, Vincenzo; Riboldi, Piersandro; Meroni, Pier Luigi; Merrill, Joan T.

    2016-01-01

    Systemic lupus erythematosus (SLE) and antiphospholipid syndrome have an increased risk to develop cognitive impairment. A possible role for antiphospholipid antibodies (aPL) and antiglutamate receptor (anti-NMDA) antibodies in the pathogenesis of neurological manifestations of these two conditions, have been suggested. In particular, the role of anti-NMDA antibodies in the pathogenesis of neuropsychiatric SLE is supported by several experimental studies in animal models and by the finding of a correlation between anti-NMDA positivity in cerebrospinal fluid and neurological manifestations of SLE. However, data from the literature are controversial, as several studies have reported a correlation of these antibodies with mild cognitive impairment in SLE, but more recent studies have not confirmed this finding. The synergism between anti-NMDA and other concomitant autoantibodies, such as aPL, can be hypothesized to play a role in inducing the tissue damage and eventually the functional abnormalities. In line with this hypothesis, we have found a high incidence of at least one impaired cognitive domain in a small cohort of patients with primary APS (PAPS) and SLE. Interestingly, aPL were associated with low scoring for language ability and attention while anti-NMDA titers and mini-mental state examination scoring were inversely correlated. However, when patients were stratified according to the presence/absence of aPL, the correlation was confirmed in aPL positive patients only. Should those findings be confirmed, the etiology of the prevalent defects found in PAPS patients as well as the synergism between aPL and anti-NMDA antibodies would need to be explored. PMID:26870034

  14. Macrophage activation syndrome as the initial manifestation of severe juvenile onset systemic lupus erythematosus. Favorable response to cyclophosphamide.

    Science.gov (United States)

    Torres Jiménez, Alfonso; Solís Vallejo, Eunice; Zeferino Cruz, Maritza; Céspedes Cruz, Adriana; Sánchez Jara, Berenice

    2014-01-01

    The macrophage activation syndrome is a rare but potentially fatal complication of patients with autoimmune rheumatic diseases. This is a clinicopathological entity characterized by activation of histiocytes with prominent hemophagocytosis in the bone marrow and other reticuloendothelial systems. In patients with lupus it may mimic an exacerbation of the disease or infection. We report the case of a 7-year-old girl in whom the diagnosis of lupus erythematosus and macrophage activation syndrome was simultaneously made with response to the use of cyclophosphamide.

  15. Progressive outer retinal necrosis syndrome in the course of systemic lupus erythematosus.

    Science.gov (United States)

    Turno-Kręcicka, A; Tomczyk-Socha, M; Zimny, A

    2016-12-01

    Progressive outer retinal necrosis syndrome (PORN) is a severe clinical variant of necrotizing herpetic chorioretinitis, which occurs almost exclusively in patients with advanced acquired immunodeficiency syndrome (AIDS). To date, only a few cases of PORN have been reported in patients, mostly among those who were immunocompromised. To our knowledge, only one case of PORN in a patient with systemic lupus erythematosus (SLE) has been described. We report the case of a 44-year old HIV-negative patient with lupus nephritis, whom was being treated by mycophenolate mophetil (MMF), arechin and prednisone. After 14 months of MMF therapy, the patient revealed PORN symptoms; and several months later, the patient developed Type B primary central nervous system lymphoma (PCNSL). PORN is usually compared to acute retinal necrosis (ARN) syndrome, because of having the same causative agent: varicella zoster virus (VZV). There are also some similarities in clinical findings. Our observation supports the hypothesis that PORN symptoms in HIV-negative patients can be an intermediate form between ARN and PORN, and can vary according to the patient's immune status.

  16. Efficacy of plasma exchange and immunoadsorption in systemic lupus erythematosus and antiphospholipid syndrome: A systematic review.

    Science.gov (United States)

    Kronbichler, Andreas; Brezina, Biljana; Quintana, Luis F; Jayne, David R W

    2016-01-01

    Extracorporeal treatments have been used since the 1970s in the management of systemic lupus erythematosus (SLE). A randomised controlled trial comparing the efficacy of standard of care (SOC) combined with plasma exchange against SOC alone in patients with lupus nephritis revealed no difference in terms of renal outcome. Subsequently, initial expectations have been dampened and further experience with plasma exchange is mainly limited to observational studies and single case reports. Beneficial effects have been reported in patients with refractory disease course or in pregnancy with prior complications due to SLE and antiphospholipid syndrome. A more specific form of extracorporeal treatment, immunoadsorption (IAS), has emerged as a valuable option in the treatment of SLE. In line with the plasma exchange experience, IAS seems to have beneficial effects in patients with refractory disease, contraindications to standard immunosuppression or during pregnancy. The mechanism IAS relates to autoantibody removal but for plasma exchange removal of activated complement components, coagulation factors, cytokines and microparticles may also be relevant. Both treatment forms have good safety profiles although reactions to blood product replacement in plasma exchange and procedure related complications such as bleeding or catheter-related infections have occurred. There is a need to more clearly define the clinical utility of plasma exchange and IAS in refractory lupus and APS subgroups.

  17. Atypical hemolytic uremic syndrome secondary to lupus nephritis, responsive to eculizumab

    Directory of Open Access Journals (Sweden)

    Alexander G. Raufi

    2016-09-01

    Full Text Available Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS. Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs – a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage. Although plasma exchange is considered first-line therapy for thrombotic thrombocytopenic purpura – a TMA classically associated with autoimmune depletion of ADAMTS-13 – aHUS demonstrates less reliable responsiveness to this modality. Instead, use of the late complement inhibitor Eculizumab has emerged as an effective modality for the management of such patients. Diagnosis of aHUS, however, is largely clinically based, relying heavily upon a multidisciplinary approach. Herein we present the case of a patient with atypical HUS successfully treated with Eculizumab in the setting of Class IV-G (A lupus nephritis and hypocomplementemia.

  18. Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab

    Science.gov (United States)

    Raufi, Alexander G.; Scott, Shruti; Darwish, Omar; Harley, Kevin; Kahlon, Kanwarpal; Desai, Sheetal; Lu, Yuxin; Tran, Minh-Ha

    2016-01-01

    Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) – a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage. Although plasma exchange is considered first-line therapy for thrombotic thrombocytopenic purpura – a TMA classically associated with autoimmune depletion of ADAMTS-13 – aHUS demonstrates less reliable responsiveness to this modality. Instead, use of the late complement inhibitor Eculizumab has emerged as an effective modality for the management of such patients. Diagnosis of aHUS, however, is largely clinically based, relying heavily upon a multidisciplinary approach. Herein we present the case of a patient with atypical HUS successfully treated with Eculizumab in the setting of Class IV-G (A) lupus nephritis and hypocomplementemia.

  19. Dubin-Johnson syndrome with systemic lupus erythematosus:a case report

    Institute of Scientific and Technical Information of China (English)

    Mamun-Al Mahtab; Md. Fazal Karim; Salimur Rahman; Abul Barkat Muhammad Adnan

    2006-01-01

    BACKGROUND:Dubin-Johnson syndrome (DJS) is a rare clinical entity. We describe a case of DJS complicated by systemic lupus erythematosus (SLE). METHODS:A case of congenital hyperbilirubinemia with SLE was evaluated systematically including review of history, physical examination for the stigmata of chronic liver disease, and other investigations. RESULT:Liver biopsy revealed a black liver with preserved architecture suggestive of DJS. CONCLUSIONS:SLE may develop in DJS. The relationship between DJS and SLE in this case is most likely a chance occurrence.

  20. Kluver–Bucy syndrome in one case with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Hsiu-Fen Lin

    2011-04-01

    Full Text Available Kluver–Bucy syndrome (KBS is a collection of neuropsychiatric symptoms, including visual agnosia (prosopagnosia, hypermetamorphosis, placidity, hypersexuality, and hyperorality. Although neuropsychiatric manifestation is prevalent in cases with systemic lupus erythematosus (SLE, only one literature reported a case with SLE that had KBS previously. In this article, a 37-year-old woman with SLE who developed KBS and other neuropsychiatric symptoms is presented. Brain imaging proved the relevant structural lesion. The possible explanation of pathogenesis of KBS in SLE is discussed.

  1. Autosplenectomy Causing Catastrophic Pneumococcal Meningitis in a Patient with Lupus/Antiphospholipid Antibody Syndrome.

    Science.gov (United States)

    Sheth, Khushboo; Snyder, Aaron; Wu, Ulysses; Lahiri, Bimalin; Grover, Prashant

    2016-01-01

    We present the case ofa26-year-old female who presented to the hospital with pneumococcal meningitis. A review of her records showed atrophic spleen, and a hypercoagulable workup was positive for Systemic Lupus Erythematous (SLE)/Antiphospholipid Antibody Syndrome (APS). An autosplenectomy from thrombotic occlusion of the splenic artery made her susceptible to pneumococcal meningitis. Autoimmune conditions, particularly SLE and APS, are important causes of hypercoagulable states in a young population, and earlier detection of these conditions and appropriate treatment helps to decrease morbidity and mortality among these patients.

  2. Mitral bioprosthetic valve stenosis in a patient with antiphospholipid antibody syndrome and systemic lupus erythematosus.

    Science.gov (United States)

    Morisaki, Akimasa; Hirai, Hidekazu; Sasaki, Yasuyuki; Hosono, Mitsuharu; Sakaguchi, Masanori; Nakahira, Atsushi; Seo, Hiroyuki; Suehiro, Shigefumi

    2012-12-01

    A 45-year-old woman with antiphospholipid antibody syndrome (APS) and systemic lupus erythematosus was admitted because of severe dyspnea. She had undergone mitral valve replacement (MVR) using a Mosaic bioprosthesis for infective endocarditis 9 years previously. She developed congestive heart failure secondary to mitral bioprosthetic valve stenosis resulting from relatively early structural valve deterioration. She underwent a second MVR using a mechanical valve prosthesis. The explanted bioprosthesis showed marked pannus formation and mineralization with fibrin thrombus formation, especially on the outflow surfaces of the leaflets. After the second operation, she was discharged without APS-related thromboembolic events under meticulous anticoagulant and antiplatelet therapies.

  3. Myocardial infarction in a patient with systemic lupus erythematosus and antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    ZHANG Bo; JIANG Da-ming; ZHOU Xu-chen; QI Guo-xian

    2011-01-01

    This case report we presented aims to report a-31-year-old man with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) who developed myocardial infarction (Ml) and also aims to discuss the possible mechanisms. The results showed that traditional risk factors alone do not cause coronary heart disease with SLE, and SLE-related factors influence the atherogenic process. We found that although SLE patients with acute Ml benefit from percutaneous coronary intervention (PCI) therapy, it is very important to choose the reasonable antithrombotic strategies in patients with SLE and APS undergoing PCI who require oral anticoagulant therapy.

  4. Drug-Induced Hematologic Syndromes

    Science.gov (United States)

    Mintzer, David M.; Billet, Shira N.; Chmielewski, Lauren

    2009-01-01

    Objective. Drugs can induce almost the entire spectrum of hematologic disorders, affecting white cells, red cells, platelets, and the coagulation system. This paper aims to emphasize the broad range of drug-induced hematological syndromes and to highlight some of the newer drugs and syndromes. Methods. Medline literature on drug-induced hematologic syndromes was reviewed. Most reports and reviews focus on individual drugs or cytopenias. Results. Drug-induced syndromes include hemolytic anemias, methemoglobinemia, red cell aplasia, sideroblastic anemia, megaloblastic anemia, polycythemia, aplastic anemia, leukocytosis, neutropenia, eosinophilia, immune thrombocytopenia, microangiopathic syndromes, hypercoagulability, hypoprothrombinemia, circulating anticoagulants, myelodysplasia, and acute leukemia. Some of the classic drugs known to cause hematologic abnormalities have been replaced by newer drugs, including biologics, accompanied by their own syndromes and unintended side effects. Conclusions. Drugs can induce toxicities spanning many hematologic syndromes, mediated by a variety of mechanisms. Physicians need to be alert to the potential for iatrogenic drug-induced hematologic complications. PMID:19960059

  5. Drug-Induced Hematologic Syndromes

    Directory of Open Access Journals (Sweden)

    David M. Mintzer

    2009-01-01

    Full Text Available Objective. Drugs can induce almost the entire spectrum of hematologic disorders, affecting white cells, red cells, platelets, and the coagulation system. This paper aims to emphasize the broad range of drug-induced hematological syndromes and to highlight some of the newer drugs and syndromes. Methods. Medline literature on drug-induced hematologic syndromes was reviewed. Most reports and reviews focus on individual drugs or cytopenias. Results. Drug-induced syndromes include hemolytic anemias, methemoglobinemia, red cell aplasia, sideroblastic anemia, megaloblastic anemia, polycythemia, aplastic anemia, leukocytosis, neutropenia, eosinophilia, immune thrombocytopenia, microangiopathic syndromes, hypercoagulability, hypoprothrombinemia, circulating anticoagulants, myelodysplasia, and acute leukemia. Some of the classic drugs known to cause hematologic abnormalities have been replaced by newer drugs, including biologics, accompanied by their own syndromes and unintended side effects. Conclusions. Drugs can induce toxicities spanning many hematologic syndromes, mediated by a variety of mechanisms. Physicians need to be alert to the potential for iatrogenic drug-induced hematologic complications.

  6. Overlap syndrome between Familial Mediterranean fever and tumor necrosis factor receptor-associated periodic syndrome in a lupus patient.

    Science.gov (United States)

    Nonaka, Fumiaki; Migita, Kiyoshi; Iwasaki, Keisuke; Shimizu, Toshimasa; Kawakami, Atsushi; Yasunami, Michio; Eguchi, Katsumi

    2014-06-01

    Autoinflammatory diseases represent an expanding spectrum of genetic and non-genetic inflammatory diseases characterized by recurrent episodes of fever and systemic inflammation, affecting joints, skin and serosal surfaces. Familial Mediterranean fever (FMF) is the most common autosomal recessive hereditary autoinflammatory disease. Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autosomal dominant hereditary autoinflammatory disease. They share some clinical manifestations such as a periodic fever and skin rash. We present here the association of FMF with TRAPS in a systemic lupus erythematosus (SLE) patient. A 54-year-old SLE patient with recurrent attacks of fever, arthritis, and skin rashes was referred to our hospital. She had been diagnosed with lupus nephritis at 19 years old. Her lupus nephritis was controlled by steroid treatments; however, since childhood she has suffered from recurrent episodes of periodic fever, abdominal pain, arthritis, and erythematous skin rashes. An initial diagnosis of FMF was suspected based on the genetic analysis, showing the compound heterozygous L110P/E148Q mutations in the MEFV gene that is responsible for FMF. Her symptoms responded to colchicine, but the febrile attacks were not completely resolved. Therefore, genetic testing for TRAPS was performed. The results revealed a heterozygous T61I mutation in the TNFRSF1A gene that encodes tumor necrosis factor-α receptor and is responsible for TRAPS. The patient was diagnosed with overlapping FMF and TRAPS, in addition to SLE. This is the first report of SLE associated with both FMF and TRAPS.

  7. [A case of antiphospholipid syndrome in course of pregnancy and puerperium with lupus-like disease suspicion].

    Science.gov (United States)

    Lorenc, Anna; Seremak-Mrozikiewicz, Agnieszka; Drews, Krzysztof; Ratajczak, Paweł; Puszczewicz, Mariusz

    2011-04-01

    The antiphospholipid syndrome (APS) is a systemic non-inflammatory and autoimmune connective tissue disease. ASP is characterized by formation of autoantibodies against cell membrane phospholipids which act as procaogulation factors. Association with recurrent pregnancy loss, intrauterine growth retardation, preeclampsia and placenta ablation makes ASP a serious perinatologic problem. The primary form can evaluate into lupus-like disease or full-symptomatic lupus erythematosus, both usually preceded by acute form of APS. In pregnant women this situation may present considerable diagnostic problems. The aim of this work was to present a patient with acute primary antiphospholipid syndrome during pregnancy and puerperium, which subsequently was diagnosed as systemic lupus erythematosus flare. Such progression is rare and, despite several previous reports, requires extensive explanations and research.

  8. [Lupus anticoagulant-hypoprothrombinemia syndrome revealing systemic lupus in an 11-year old girl in a context of clinical and biological emergency].

    Science.gov (United States)

    Favier, Rémi; Kheyar, Tassadit; Renolleau, Sylvain; Tabone, Marie Dominique; Favier, Marie; Ulinski, Tim

    2012-01-01

    We report a case of lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) in an 11 year old girl initially hospitalized for bleeding. The patient presented with petechia, persisting bleeding after tooth extraction performed two days before, nephritic syndrome (renal failure, proteinuria and macroscopic hematuria), severe anemia, thrombocytopenia, lymphopenia. The association of these abnormalities suggested LAHPS secondary to severe systemic lupus. Immediate treatment with fresh frozen plasma and intravenous immunoglobulins (400 mg/kg/5d) was started and followed by steroid (500 mg/d) and cyclophosphamide (800 mg/m(2)) pulse therapy leading to rapid improvement of bleeding, renal involvement and prothrombin levels within 13 days. Lupus diagnosis was confirmed by immunological investigations and renal biopsy. Two early relapses occurred despite adequate treatment. After a follow-up of two years, no further disease activity is noted while the patient is treated only by mycophenolate mofetil (1 200 mg/m(2)/d). LAHPS did not relapse during this follow-up.

  9. First case of mirtazepine-induced Stevens-Johnson syndrome from India

    Directory of Open Access Journals (Sweden)

    Amit Bhasin

    2012-01-01

    Full Text Available A 28-year-old woman, a known case of systemic lupus erythematosus (SLE, was admitted with mucocutaneous ulceroerosive lesions with blisters and thrombocytopenia after taking antidepressant mirtazepine. Exacerbation of SLE and drug-induced eruption was diagnosed. Clinical and laboratory markers were suggestive of Stevens-Johnson syndrome. This is a rare adverse effect of the newer generation antidepressant mirtazepine.

  10. LUPUS ERITEMATOSO SISTÉMICO SÍNDROME DE CUSHING Y PSIQUIATRÍA DE ENLACE Erythematous systemic lupus, Cushing`s syndrome and psychiatric

    Directory of Open Access Journals (Sweden)

    Ludwig Pájaro Silva

    2005-04-01

    Full Text Available Se presenta el caso de una paciente con diagnóstico de lupus eritematoso sistémico y síndrome de Cushing quien presentó síntomas afectivos de características depresivas e insomnio de conciliación, quien recibió múltiples tratamientos farmacológicos con pobre respuesta inicial. La psiquiatría de enlace consolida una alternativa de tratamiento multidisciplinario para una aproximación más eficiente a las patologías médicas con síntomas psiquiátricos.We present a case of systemic erythematosus lupus and Cushing's syndrome who has affective symptoms of depressive characteristic and insomnia and multiple pharmacological treatments with poor response. The intervention by psychiatry liaison is an alternative of multidisciplinary treatment for an efficient approximation of medical pathologies with psychiatric symptoms.

  11. An overlap syndrome involving autoimmune hepatitis and systemic lupus erythematosus in childhood

    Institute of Scientific and Technical Information of China (English)

    Yusuf Usta; Figen Gurakan; Zuhal Akcoren; Seza Ozen

    2007-01-01

    We report a 12 years old female patient with an overlap syndrome involving autoimmune hepatitis (ALM) and systemic lupus erythematosus (SLE). The patient presented with jaundice, hepatosplenomegaly, malAlse, polyarthralgia, arthritis and butterfly rash on the face. Laboratory tests revealed severe liver dysfunction, Coombs positive hemolytic anemia and a positive ANA/ anti-dsDNA test. Renal biopsy showed class IIA kidney disease, while liver biopsy showed chronic hepatitis with severe inflammatory activity. The patient satisfied the international criteria for both SLE and ALM. Clinical symptoms and laboratory findings of SLE improved with high dose treatment with corticosteroids and azathioprine, however, remission of the liver disease could not be achieved. Repeat biopsy of the liver after three years of therapy revealed ongoing chronic hepatitis with high level of inflammatory activity. The present case indicates that children with liver dysfunction and SLE should be investigated for ALM. There is much diagnostic and therapeutic dilemma in patients with ALH-SLE overlap syndrome.

  12. The Significance of Dehydroepiandrosterone for Fatigue in primary Sjögren’s Syndrome and Systemic Lupus Erythematosus

    NARCIS (Netherlands)

    Hartkamp, A.

    2014-01-01

    Fatigue is a prevalent and debilitating symptom in patients with the chronic inflammatory autoimmune diseases primary Sjögren’s syndrome (pSS) and systemic lupus erythematosus (SLE). Both diseases have a female preponderance (women to men ratio 9:1). Doctors recognize the existence of fatigue and

  13. The Significance of Dehydroepiandrosterone for Fatigue in primary Sjögren’s Syndrome and Systemic Lupus Erythematosus

    NARCIS (Netherlands)

    Hartkamp, A.

    2014-01-01

    Fatigue is a prevalent and debilitating symptom in patients with the chronic inflammatory autoimmune diseases primary Sjögren’s syndrome (pSS) and systemic lupus erythematosus (SLE). Both diseases have a female preponderance (women to men ratio 9:1). Doctors recognize the existence of fatigue and it

  14. Sulphasalazine Induced Hypersensitivity Syndrome

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    Hatice Şanlı

    2013-05-01

    Full Text Available Drug-induced hypersensitivity syndrome (DIHS is one of the most dangerous drug reactions. Mortality and morbidity is increased by consequent systemic organ involvement. Maculopapular eruptions are the most common lesions accompanying DIHS, however, the morphology of skin lesions may vary. The most common cause of DIHS is the use of aromatic anticonvulsant drugs. However, one must not forget that other drugs may also cause DIHS. Early recognition of the condition is the most important step in the treatment. Herein, we present a case of DIHS triggered by sulphasalazine and associated with pustular eruption and maculopapular eruption.

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

    OpenAIRE

    2016-01-01

    Abstract The aim of the study was to report the clinical, biological, and pathological characteristics of patients with glomerulonephritis (GN) secondary to systemic lupus erythematosus (SLE)/antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) overlap syndrome. A nationwide survey was conducted to identify cases of SLE/AAV overlap syndrome. Data were collected from SLE and AAV French research groups. Inclusion criteria were diagnosis of both SLE and AAV according to inter...

  16. 46,X,del(X)(q13) Turner's Syndrome Female with Systemic Lupus Erythematosus in a Pedigree Multiplex for SLE

    OpenAIRE

    2009-01-01

    Systemic lupus erythematosus (SLE) disproportionately affects females. Recent work demonstrates that men with Klinefelter's syndrome (47,XXY males) have a similar risk of developing SLE as do genotypic females. We present an unusual case of an African American family with two SLE affected individuals in which one of the SLE patients also has Turner's syndrome [46,X,del(X)(q13)]. While not definitive, this family raises interesting questions regarding the role of genes located on the X chromos...

  17. Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience.

    Science.gov (United States)

    Deeb, M; Tselios, K; Gladman, D D; Su, J; Urowitz, M B

    2017-01-01

    Introduction Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE), characterized by decreased lung volumes and extra-pulmonary restriction. The aim of this study was to describe the characteristics of SLS in our lupus cohort with emphasis on prevalence, presentation, treatment and outcomes. Patients and methods Patients attending the Toronto Lupus Clinic since 1980 ( n = 1439) and who had pulmonary function tests (PFTs) performed during follow-up were enrolled ( n = 278). PFT records were reviewed to characterize the pattern of pulmonary disease. SLS definition was based on a restrictive ventilatory defect with normal or slightly reduced corrected diffusing lung capacity for carbon monoxide (DLCO) in the presence of suggestive clinical (dyspnea, chest pain) and radiological (elevated diaphragm) manifestations. Data on clinical symptoms, functional abnormalities, imaging, treatment and outcomes were extracted in a dedicated data retrieval form. Results Twenty-two patients (20 females) were identified with SLS for a prevalence of 1.53%. Their mean age was 29.5 ± 13.3 years at SLE and 35.7 ± 14.6 years at SLS diagnosis. Main clinical manifestations included dyspnea (21/22, 95.5%) and pleuritic chest pain (20/22, 90.9%). PFTs were available in 20 patients; 16 (80%) had decreased maximal inspiratory (MIP) and/or expiratory pressure (MEP). Elevated hemidiaphragm was demonstrated in 12 patients (60%). Treatment with prednisone and/or immunosuppressives led to clinical improvement in 19/20 cases (95%), while spirometrical improvement was observed in 14/16 patients and was mostly partial. Conclusions SLS prevalence in SLE was 1.53%. Treatment with glucocorticosteroids and immunosuppressives was generally effective. However, a chronic restrictive ventilatory defect usually persisted.

  18. Neonatal lupus syndrome: the heart as a target of the immune system

    Directory of Open Access Journals (Sweden)

    GARCIA SIMONE

    2000-01-01

    Full Text Available Neonatal lupus erythematosus (NLE is an auto-immune disease related to systemic lupus erythematosus (SLE. Unlike SLE it is not a spontaneous syndrome but rather an acquired one. In NLE the most common disease manifestations are a transient cutaneous lesion and cardiac conduction disturbances. The cutaneous lesions and other non-cardiac manifestations of NLE are transient and disappear about six months after birth, at the time when maternal antibodies disappear from the neonatal circulation. This fact suggests that maternal antibodies may cross the placenta leading to an inflamatory reaction in the fetal tissues. NLE is the principal cause of atria-ventricular block, when it is not associated with congenital birth defects. All the clinical studies to date correlate the heart block in NLE with the presence of certain types of circulating maternal antibodies, against the Ro/SSA nuclear proteins, in the serum of the newborn. In this paper we discuss animal models that have been developed by our and others groups to study the participation of the anti-Ro/SSA antibodies in the pathogenesis of the cardiac conduction blockades that occur in NLE.

  19. Acute abdomen in patients with systemic lupus erythematosus and antiphospholipid syndrome: importance of early diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    Alberto Titos-García

    2015-01-01

    Full Text Available Systemic lupus erithematosus (SLE is an autoimmune disease with multiorgan involvement caused principally by vasculitis of small vessels. The gastrointestinal tract is one of the most frequently affected by SLE, with abdominal pain as the most common symptom. An early diagnosis and treatment of lupus enteritis is essential to avoid complications like hemorrhage or perforation, with up to 50 % of mortality rate. However, differential diagnosis sometimes is difficult, especially with other types of gastrointestinal diseases as digestive involvement of antiphospholipid syndrome (APS, moreover when both entities may coexist. We describe the case of a patient with both diseases that was diagnosed with lupus enteritis and treated with steroid therapy; the patient had an excellent response.

  20. Antiphospholipid Syndrome: primary or secondary to Systemic Lupus Erythematosus? Description of a clinical case of avitaminosis D in premenopausal woman with pseudo-Cushing syndrome

    OpenAIRE

    2014-01-01

    Low vitamin D levels have been described in obese individuals and in some autoimmune diseases, such as Systemic Lupus Erythematosus (SLE) and primary antiphospholipid syndrome (pAPS). In particular, more than 50% of premenopausal women with pAPS have hypovitaminosis D. In this issue we report a case of an obese, premenopausal, and hypertensive woman with pseudo-Cushing syndrome, affected by deep venous thrombosis associated with pulmonary embolism after rib fracture who presented hypovitamino...

  1. Reversible cerebral vasoconstriction syndrome in a patient with systemic lupus erythematosus

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    V V Ashraf

    2012-01-01

    Full Text Available A 42-year-old woman, a diagnosed case of systemic lupus erythematosus (SLE, developed severe headache followed by left hemiparesis and cortical blindness. Magnetic resonance imaging (MRI of brain demonstrated right parieto-occipital infarct and the patient was initiated on high-dose steroids and antiplatelet agents with which the patient had clinical and radiological deterioration. Magnetic Resonance angiography showed severe narrowing of bilateral anterior, middle, and posterior cerebral arteries (PCA suggestive of reversible cerebral vasoconstriction syndrome (RCVS. Patient was treated with IV nimodipine and she recovered over a period of 2 weeks. Repeat MR-angiography done on day-30 was normal. The diagnosis of RCVS in patients with SLE is of practical importance, because treatment and prognosis for SLE-associated cerebral vasculitis differ strongly from the treatment of RCVS. Empirical high-dose glucocorticoid therapy should be avoided in patients with typical features of RCVS.

  2. Systemic lupus erythematosis with antiphospholipid antibody syndrome: A mimic of Buerger′s disease

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    Vasugi Zoya

    2006-01-01

    Full Text Available This case report is about a past smoker who presented with history of recurrent ulcers and digital gangrene with claudication pain of the left foot for the past fifteen years. Clinical examination and angiogram showed disease involving the peripheral vessels of lowervlimb. This patient had been labeled as Buerger′s disease 15 years ago based on clinical and demographic profile of the illness. We felt that the progression of the disease despite the patient having stopped smoking 15 years ago along with the presence of elevated inflammatory markers in the blood with proteinuria was not in keeping with the nature of the disease. Furthur evaluation revealed that the patient had systemic lupus erythematosus with antiphospholipid antibody syndrome. This case highlights the need for a careful search for diseases, which can mimic Buerger′s disease in young smokers who present with peripheral vascular disease and who have an atypical clinical presentation or progression.

  3. Tolosa-Hunt syndrome in a patient with systemic lupus erythematosus

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    Calistri, Valentina; Mostardini, Claudio; Pantano, Patrizia; Pierallini, Alberto [Department of Neurological Sciences, University of Rome (Italy); Colonnese, Claudio [IRCCS Neuromed, Pozzilli (Italy); Caramia, Francesca [Department of Neurological Sciences, University of Rome (Italy); IRCCS Neuromed, Pozzilli (Italy)

    2002-02-01

    We report a case of Tolosa-Hunt syndrome (THS) in a patient with systemic lupus erythematosus studied with MRI. Magnetic resonance showed enlargement of the cavernous sinus and compression of the carotid syphon by enhancing tissue. In particular, fat-suppressed T1-weighted images before and after contrast agent injection and MR angiography showed extension of the abnormal tissue to the apex of the orbit and narrowing of the internal carotid artery. A presumptive diagnosis of THS was made and steroid treatment was started with rapid relief of symptoms. Follow-up MR study after steroid therapy demonstrated sub-total resolution of the neuroradiological findings. Neuroradiological findings in THS are quite typical but they may be subtle; furthermore, the presence of a systemic disease may suggest secondary involvement of the cavernous sinus. Utilization of the appropriate MR techniques and follow-up exams may contribute to the diagnosis of THS even in the presence of other systemic diseases. (orig.)

  4. A Case of Systemic Lupus Erythematosus Presenting as Guillain-Barré Syndrome

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    Helen Chioma Okoh

    2015-01-01

    Full Text Available Systemic lupus erythematosus (SLE is an autoimmune systemic disease with multiple organ involvement with high morbidity and mortality rate. Among the severe potential fatal complications are those of the central and peripheral nervous system which usually develop during the course of the disease and very rarely from the outset of the disease. We are reporting a rare case of Miller-Fisher (MFS variant of Guillain-Barré syndrome (GBS as the first manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis with no neurological improvement with initial immunosuppressive therapy, plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG but with remarkable and complete recovery after the second 5-day course of IVIG.

  5. [Hyperhomocystinemia as a thrombotic risk factor in patients suffering from systemic lupus erithematosus and antiphospholipid syndrome].

    Science.gov (United States)

    Onetti, Laura; Villafañe, Susana; Menso, Emilia; Drenkard, Cristina; Gamron, Susana; Barberis, Gloria; Onetti, Carlos M

    2005-01-01

    to detect the prevalence of hyperhcy in SLE patients with and without antiphospholipid syndrom; to compare the Hcy levels between those patients and healthy controls and to determine the correlation between hyperhcy and antiphospholipid antibodies. we studied 44 SLE patients: 17 had antiphospholipid syndrom and 27 didn't have it, and we compared them to 24 healthy controls. All of them where checked clinically and with laboratory tests like anticardiolypin antibodies, lupus anticoagulant and Hcy. Hcy > 9 was considered abnormal. Patient who had hyperhcy were treated with folic acid+vitB6+vitB12 a month along. cualytative variables: chi square or Fischer's; cuantitative variables: Student's T test or Mann-Whitney's test. there were 35 trombotic manifestations in 44 patients. Hyperhcy was present in 27 SLE patients (61,4%), 12 of them had antiphospholipid syndrom. Hcy concentrations patients vs.controls was statisticaly different (p= 0,002). There was also stastisticaly different the hcy concentration from SLE patients with SAF vs controls (p=0,003) and without SAF vs controls (p= 0,015). From 33 SLE patients, 20 (33%) were aCL(+). 15(75%) of them had hiperhcy.

  6. [Hyperhomocysteine like thrombocytic risk factor in patient with systemic lupus erythematous with antiphospholipid syndrome].

    Science.gov (United States)

    Onetti, Laura; Villafañe, Susana; Menso, Emilia; Drenkard, Cristina; Gamron, Susana; Barberis, Gloria; Onetti, Carlos M

    2005-01-01

    to detect the prevalence of hyperhcy in SLE patients with and without antiphospholipid syndrom; to compare the Hcy levels between those patients and healthy controls and to determine the correlation between hyperhcy and antiphospholipid antibodies. we studied 44 SLE patients: 17 had antiphospholipid syndrom and 27 didn't have it, and we compared them to 24 healthy controls. All of them where checked clinically and with laboratory tests like anticardiolypin antibodies, lupus anticoagulant and Hcy. Hcy > 9 was considered abnormal. Patient who had hyperhcy were treated with folic acid+vitB6+vitB12 a month along. cualytative variables: chi square or Fischer's; cuantitative variables: Student's T test or Mann-Whitney's test. there were 35 trombotic manifestations in 44 patients. Hyperhcy was present in 27 SLE patients (61,4%), 12 of them had antiphospholipid syndrom. Hcy concentrations patients vs.controls was statisticaly different (p=0,002). There was also stastisticaly different the hcy concentration from SLE patients with SAF vs controls (p=0,003) and without SAF vs controls (p= 0,015). From 33 SLE patients, 20 (33%) were aCL(+). 15(75%) of them had hiperhcy.

  7. Extremely hypotrophic newborn of mother with systemic lupus erythematosus and antiphospholipid syndrome.

    Science.gov (United States)

    Mockova, A; Dortova, E; Dort, J; Nahlovsky, J; Korecko, V; Ulcova-Gallova, Z

    2014-03-01

    The case presented describes a high-risk pregnancy of a woman with systemic lupus erythematosus (SLE) with multiple lesions of central nervous system (CNS), vasculitis, secondary epilepsy and antiphospholipid syndrome (APS). At gestational age 28 weeks and 3 days the pregnancy was urgently terminated via caesarean section and an extremely hypotrophic immature newborn with a birth weight of 580 g was born. The high disease activity in the mother at the time of conception and the histologically proven chronic placental insufficiency due to APS are presumably the causes for the extensive hypotrophy of the neonate. The significant comorbidity of the newborn, including respiratory distress syndrome, bronchopulmonary dysplasia, necrotizing enterocolitis, osteopathy of prematurity, transient hypothyroidism and hypocortisolism, vesicoureteral reflux, and hypertonic-hyperexcitation syndrome complicated his three-month stay in NICU. A positive titre of transplacentally transferred anticardiolipin and anti-β2 glycoprotein antibody was detected in the child and persisted through the following 30 months. During the three-year follow-up, significantly delayed neuropsychological development with microcephaly (-4 SD) and short stature of the child was observed. Finally, the authors discuss possible causes of neuropsychological consequences in children of mothers with SLE and APS and emphasize the need for long-term monitoring and specialized care to improve development of these children.

  8. Lupus and pulmonary nodules consistent with bronchiolitis obliterans organizing pneumonia induced by carbamazepine in a man

    OpenAIRE

    Awatef Kelati; Salim Gallouj; Mariame Meziane; Fatima Zahra Mernissi

    2016-01-01

    Several drugs have been implicated in the induction of systemic lupus erythematosus (SLE), but there are only some observations of carbamazepine induced SLE since the first case described in 1966, this drug has also been implicated in the induction of other disorders and rarely pulmonary toxicity; but the occurrence of two rare side effects of this drug: the induced SLE and pulmonary nodules consistent with the bronchiolitis obliterans organizing pneumonia in same patient is really unusual an...

  9. Carbamazepine induced anticonvulsant hypersensitivity syndrome

    OpenAIRE

    Shwetha Shivamurthy; Ravishankar Manchukonda

    2015-01-01

    Anticonvulsant hypersensitivity syndrome (AHS) is a potentially fatal drug-induced, multi-organ syndrome. The syndrome has been reported with anticonvulsants such as carbamazepine, phenytoin, phenobarbitone, and lamotrigine. A 17-year-old female who presented with papules and desquamation all over was diagnosed with AHS. She gave a history of fever, earache, peripheral edema, and erythematous papular eruptions 3 days prior. She gave a history of carbamazepine treatment since 15 days for gener...

  10. Drug-induced systemic lupus erythematosus in a child after 3 years of treatment with carbamazepine.

    Science.gov (United States)

    Molina-Ruiz, Ana María; Lasanta, Begoña; Barcia, Ana; Pérez-Vega, Elisa; Requena, Luis

    2017-02-01

    Drug-induced lupus erythematosus (DILE) is a less severe variant of systemic lupus erythematosus (SLE) that generally resolves within weeks or months after the withdrawal of the implicated drug. DILE is unusual during childhood, with the most frequent age of presentation being at 50-70 years of age. Among different drugs, most commonly procainamide and hydralazine have been implicated as a cause of DILE. However carbamazepine (CBZ) is considered a low-risk drug and very few cases have been reported in children. We describe the case of CBZ-induced SLE in a 9-year-old girl following 3 years of CBZ therapy. This case report shows that drug-induced SLE is an important side-effect to be considered, even after long-term treatment with CBZ, and also during childhood.

  11. Interferon-inducible Ifi200-family genes as modifiers of lupus susceptibility.

    Science.gov (United States)

    Choubey, Divaker

    2012-09-01

    Both genetic and environmental factors contribute to the development and progression of systemic lupus erythematosus (SLE), a complex autoimmune disease. The disease exhibits a strong gender bias and develops predominantly in females. Additionally, most SLE patients exhibit increased serum levels of interferon-α (IFN-α) and the "IFN signature". Studies using the mouse models of lupus have identified several lupus susceptibility loci, including the New Zealand Black (NZB)-derived autoimmunity 2 (Nba2) interval on the chromosome 1. The interval, which is syntenic to the human chromosome 1q region, harbors the FcγR family, SLAM/CD2-family, and the IFN-inducible Ifi200-family genes (encoding for the p200-family proteins). Studies involving the B6.Nba2 congenic mice revealed that the development of antinuclear autoantibodies (ANAs) depends on the age, gender, and activation of type I IFN-signaling. Interestingly, recent studies involving the generation of Nba2 subcongenic mouse lines and generation of mice deficient for the Fcgr2b or Aim2 gene within the interval have provided evidence that epistatic interactions among the Nba2 genes contribute to increased lupus susceptibility. Given that the expression of some of the p200-family proteins is differentially regulated by sex hormones and these proteins differentially regulate cytosolic DNA-induced production of type I IFN and proinflammatory cytokines (IL-1β and IL-18), the major known contributors of SLE-associated inflammation, we discuss the recent advancements in our understanding of the role of p200-family proteins in lupus susceptibility modification. An improved understanding of the role of p200-family proteins in the development of autoimmunity is likely to identify new approaches to treat SLE patients.

  12. Lamotrigine induced DRESS syndrome

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    Kikkeri Narayanasetty Naveen

    2012-01-01

    Full Text Available Drug rash with eosinophilia and systemic symptoms (DRESS syndrome is a rare and life-threatening delayed drug hypersensitivity reaction characterized by skin eruption, fever, lymphadenopathies, and visceral involvement. Here, we are presenting a 12 year old boy, who developed rare but life threatening DRESS syndrome due to Lamotrigine. Early detection and treatment led to his rapid recovery. This case is presented to highlight the importance of early detection of rare fatal syndrome.

  13. Trauma induced eagle syndrome.

    Science.gov (United States)

    Koivumäki, A; Marinescu-Gava, M; Järnstedt, J; Sándor, G K; Wolff, J

    2012-03-01

    Eagle syndrome is characterized by secondary calcification and elongation of the styloid process. Eagle syndrome is often associated with sharp, intermittent pain along the path of the glossopharyngeal nerve located in the hypopharynx and at the base of the tongue. In some cases, the stylohyoid apparatus can compress the internal and/or the external carotid arteries and their perivascular sympathetic fibres, resulting in a persistent pain radiating throughout the carotid territory. The pathogenesis of the syndrome is not understood. The authors report the case of a 52-year-old woman with post traumatic Eagle syndrome-like pain and pseudoarthrosis of the stylohyoid ligament.

  14. 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.

  15. The coexistence of antiphospholipid syndrome and systemic lupus erythematosus in Colombians.

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    Juan-Sebastian Franco

    Full Text Available OBJECTIVES: To examine the prevalence and associated factors related to the coexistence of antiphospholipid syndrome (APS and systemic lupus erythematosus (SLE in a cohort of Colombian patients with SLE, and to discuss the coexistence of APS with other autoimmune diseases (ADs. METHOD: A total of 376 patients with SLE were assessed for the presence of the following: 1 confirmed APS; 2 positivity for antiphospholipid (aPL antibodies without a prior thromboembolic nor obstetric event; and 3 SLE patients without APS nor positivity for aPL antibodies. Comparisons between groups 1 and 3 were evaluated by bivariate and multivariate analysis. RESULTS: Although the prevalence of aPL antibodies was 54%, APS was present in just 9.3% of SLE patients. In our series, besides cardiovascular disease (AOR 3.38, 95% CI 1.11-10.96, p = 0.035, pulmonary involvement (AOR 5.06, 95% CI 1.56-16.74, p = 0.007 and positivity for rheumatoid factor (AOR 4.68, 95%IC 1.63-14.98, p = 0.006 were factors significantly associated with APS-SLE. APS also may coexist with rheumatoid arthritis, Sjögren's syndrome, autoimmune thyroid diseases, systemic sclerosis, systemic vasculitis, dermatopolymyositis, primary biliary cirrhosis and autoimmune hepatitis. CONCLUSIONS: APS is a systemic AD that may coexist with other ADs, the most common being SLE. Awareness of this polyautoimmunity should be addressed promptly to establish strategies for controlling modifiable risk factors in those patients.

  16. Spontaneous murine lupus-like syndromes. Clinical and immunopathological manifestations in several strains.

    Science.gov (United States)

    Andrews, B S; Eisenberg, R A; Theofilopoulos, A N; Izui, S; Wilson, C B; McConahey, P J; Murphy, E D; Roths, J B; Dixon, F J

    1978-11-01

    MRL/1 and BXSB male mice have a systemic lupus erythematosus (SLE)-like disease similar to but more acute than that occurring in NZB X W mice. The common elements of lymphoid hyperplasia, B-cell hyperactivity, autoantibodies, circulating immune complex (IC), complement consumption, IC glomerulonephritis with gp70 deposition, and thymic atrophy were found in all three kinds of SLE mice. On the basis of these common elements, SLE seen in these mice can be considered a single disease in the same sense that human SLE is one disease. The differences in the SLE expressed in the different mice are no greater than those found in an unselected series of humans with SLE. However, the significant quantitative and qualitative variations in abnormal immunologic expression suggest that different constellations of factors, genetic and/or pathophysiologic, may operate in the three murine strains and that each constellation is capable of leading, via its particular abnormal immunologic consequences, to the activation of common immunopathologic effector mechanisms that cause quite similar SLE-like syndromes. From an experimental point of view, the availability of several inbred murine strains of commonplace histocompatibility types that express an SLE-like syndrome makes possible innumerable manipulations which should help to elucidate the nature and cause(s) of this disorder.

  17. Successful treatment of Raynaud's syndrome in a lupus patient with continuous bilateral popliteal sciatic nerve blocks: a case report.

    Science.gov (United States)

    Dao, Thuan; Amaro-Driedger, David; Mehta, Jaideep

    2016-01-01

    Raynaud's syndrome has been treated medically and invasively, sometimes with regional anesthesia leading up to sympathectomy. We demonstrate that regional anesthesia was in this case a useful technique that can allow some patients to find temporary but significant relief from symptoms of Raynaud's syndrome exacerbation. We present a 43-year-old woman with Raynaud's syndrome secondary to lupus who was treated with bilateral popliteal nerve block catheters for ischemic pain and necrosis of her feet; this led to almost immediate resolution of her pain and return of color and function of her feet. While medical management should continue to be a front-line treatment for Raynaud's syndrome, regional anesthesia can be useful in providing rapid dissipation of symptoms and may thus serve as a viable option for short-term management of this syndrome.

  18. DRUG REACTION WITH HERBAL SUPPLEMENT: A POSSIBLE CASE OF DRUG INDUCED LUPUS ERYTHEMATOSUS

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    AZIZ NA

    2010-01-01

    Full Text Available A 24-year-old lady presented with four days history of fever, non-pruritic rash, ankle pain and swelling. She had consumed herbal supplement five days before the onset of symptoms. Examinations revealed erythematous maculo-papular lesions of varying sizes on sun exposed areas. Patient was suspected to have Drug Induced Lupus Erythematosus (DILE and subsequently symptoms subsided rapidly on withholding the herbal medication.

  19. [DRUG-INDUCED LUPUS CAUSED BY LONG TERM MINOCYCLINE TREATMENT FOR ACNE VULGARIS].

    Science.gov (United States)

    Hanai, Shunichiro; Sato, Takeo; Takeda, Koichi; Nagatani, Katsuya; Iwamoto, Masahiro; Minota, Seiji

    2015-09-01

    An 18-year-old Japanese girl had received oral minocycline 200mg daily for treatment of acne vulgaris since 16 years old. She had a fever three months before admission, followed by joint pains in her knees, elbows and several proximal interphalangeal joints one month before admission. She was referred to our hospital because of a high serum level of anti-DNA antibody. She had already discontinued oral minocycline five weeks before admission, because she missed her medication refilled. On admission, the arthralgia and fever spontaneously resolved, and there were no laboratory evidence of hypocomplementemia and cytopenia. She had neither erythema nor internal organ involvements. Because her symptoms subsided spontaneously after the cessation of minocycline, she was considered to have drug-induced lupus. Both the arthralgia and fever did not relapse, and anti-ds DNA antibody returned to normal during a follow-up period without treatment. There are few reports of drug-induced lupus caused by minocycline in Japan. This case highlights the importance of considering minocycline-induced lupus.

  20. Rare X chromosome abnormalities in systemic lupus erythematosus and Sjögren's syndrome.

    Science.gov (United States)

    Sharma, Rohan; Harris, Valerie M; Cavett, Joshua; Kurien, Biji T; Liu, Ke; Koelsch, Kristi A; Fayaaz, Anum; Chaudhari, Kaustubh S; Radfar, Lida; Lewis, David; Stone, Donald U; Kaufman, C Erick; Li, Shibo; Segal, Barbara; Wallace, Daniel J; Weisman, Michael H; Venuturupalli, Swamy; Kelly, Jennifer A; Pons-Estel, Bernardo; Jonsson, Roland; Lu, Xianglan; Gottenberg, Jacques-Eric; Anaya, Juan-Manuel; Cunninghame-Graham, Deborah S; Huang, Andrew J W; Brennan, Michael T; Hughes, Pamela; Alevizos, Ilias; Miceli-Richard, Corinne; Keystone, Edward C; Bykerk, Vivian P; Hirschfield, Gideon; Xie, Gang; Nordmark, Gunnel; Bucher, Sara Magnusson; Eriksson, Per; Omdal, Roald; Rhodus, Nelson L; Rischmueller, Maureen; Rohrer, Michael; Wahren-Herlenius, Marie; Witte, Torsten; Alarcon-Riquelme, Marta; Mariette, Xavier; Lessard, Christopher J; Harley, John B; Ng, Wan-Fai; Rasmussen, Astrid; Sivils, Kathy L; Scofield, R Hal

    2017-07-10

    Sjögren's syndrome and systemic lupus erythematosus (SLE) are related by clinical and serological manifestations as well as genetic risks. Both diseases are more commonly found in women compared to men at a ratio of about 10 to 1. Common X chromosome aneuploidies, 47,XXY and 47,XXX, are enriched among men and women, respectively, in either disease suggesting a dose effect on the X chromosome. We examined cohorts of Sjögren's syndrome or SLE patients with intensity plots of X chromosome single nucleotide polymorphism (SNP) alleles along with karyotype of selected subjects. Among ∼2500 women with SLE we found three patients with a triple mosaic consisting of 45,X/46,XX/47,XXX. Among ∼2100 women with Sjögren's syndrome, one patient had 45,X/46,XX/47,XXX with a triplication of the distal p arm of the X chromosome in the 47,XXX cells. Neither the triple mosaic nor the partial triplication were found among controls. In another Sjögren's cohort, we found a mother-daughter pair with partial triplication of this same region of the X chromosome. The triple mosaic occurs in approximately 1 in 25,000 to 50,000 live female births, while partial triplications such are even rarer. Very rare X chromosome abnormalities are present among patients with either Sjögren's or SLE, and may inform the location of a gene(s) that mediate an X dose effect as well as critical cell types in which such effect is operative. This article is protected by copyright. All rights reserved. © 2017, American College of Rheumatology.

  1. Effects of 1,25-dihydroxyvitamin D3 on IL-17/IL-23 axis, IFN-γ and IL-4 expression in systemic lupus erythematosus induced mice model

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    Fatemeh Faraji

    2016-04-01

    Conclusion: Our findings showed that vitamin D3 supplementation in lupus induced mice through modulating the expression rate of some inflammatory cytokines diminished the inflammatory conditions in SLE.

  2. Neuropsychiatric Symptoms As The First Manifestation of Juvenile Systemic Lupus Erythematosus: A Complicated Case With Klinefelter's Syndrome

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    Reza SHIARI*

    2014-01-01

    Full Text Available How to Cite This Article: Shiari R, Hassase Yegane M, Farivar S, Javadi Parvaneh V, Mirjavadi SA. Neuropsychiatric symptoms as the first manifestation of Juvenile Systemic Lupus Erythematosus: A complicated case with Klinefelter’s syndrome. Iran J Child Neurol. 2014 Winter; 8(1:62-65.AbstractSystemic Lupus Erythematosus (SLE is an autoimmune, multisystem disorder with various manifestations. There are limited reports on the neuropsychiatric findings as the first manifestation of SLE in children. Herein, we reported a14-year-old Iranian boy with a two-year history of cognitive dysfunction and behavioural problems as well as a recent history of epistaxis. The patient workup ended with a diagnosis of Klinefelter’s syndrome associated with juvenileSLE. Patients with Klinefelter’s syndrome may exhibit behavioural problems and psychological disease. These psychiatric disorders could be complicated with lupus in children. In fact, psychiatric symptoms may occur as the firstmanifestation of juvenile SLE. Specially, if accompanied with Klinefelter’s syndrome. We suggest the diagnosis of SLE must be strongly considered in all children with neuropsychiatric manifestations.References:Brey RL. Neuropsychiatric Lupus, clinical and imaging aspects. Bullet NYU Hosp joint dis 2007;65(3:194-9.Yu CH, Lee MB, Tseng MM, Liao SC. Obsessive-Compulsive symptoms as a manifestation of neuropsychiatric SLE. J Formus Med Assoc 2008;107:68-72.Harel L, Sandborg Ch, Lee T, Scheven E. Neuropsychiatric manifestations in paediatric SLE and association with antiphospholipid antibodies. J Rheumatol 2006;33:1873-7.Bernatsky S, Clarke A, Gladman DD, Urowitz M, Fortin PR, Barr SG, et al. Mortality related to Cerebrovascular disease in SLE. Lupus 2006; 15:835-9.Stern R, Fishman J, Brusman H, Kunkel HG. Systemic lupus erythematosus associated with Klinefelter’s syndrome. Arthritis Rheum 1977; 20:18-22.Paduch DA, Fine RG, Bolyakov AJ. New concepts in Klinefelter

  3. Annexin A1 as a target for managing murine pristane-induced systemic lupus erythematosus.

    Science.gov (United States)

    Mihaylova, Nikolina; Bradyanova, Silviya; Chipinski, Petroslav; Herbáth, Melinda; Chausheva, Stela; Kyurkchiev, Dobroslav; Prechl, József; Tchorbanov, Andrey I

    2017-03-16

    Systemic lupus erythematosus (SLE) is a polygenic pathological disorder which involves multiple organs. Self-specific B cells play a main role in the lupus pathogenesis by generating autoantibodies as well as by serving as important autoantigen-presenting cells. Autoreactive T lymphocytes, on the other hand, are responsible for B cell activation and proliferation, and cytokine production. Therefore, both factors promote the idea that a down-modulation of activated self-reactive T and B cells involved in the pathogenic immune response is a reasonable approach for SLE therapy. Annexin A1 (ANX A1) is expressed by many cell types and binds to phospholipids in a Ca(2+) dependent manner. Abnormal expression of ANX A1 was found on activated B and T cells in both murine and human autoimmunity, suggesting its potential role as a therapeutic target. While its role on T lymphocytes is through formyl peptide receptor-like molecules (FPRL), and the formed ANX A1/FPRL pathway modulates T cell receptor signalling, there is still no fool-proof data available for the role of ANX A1 in B cells. We employed a lupus model of Balb/c mice with pristane-induced SLE which very closely resembles human lupus. In the present study, we investigated the possibility to modulate the autoimmune response in a pristane-induced mouse model of SLE using an anti- ANX A1 antibody. Administration of this monoclonal antibody resulted in the inhibition of T-cell activation and proliferation, suppression of IgG anti-dsDNA antibody-secreting plasma cells and of proteinuria, decreased disease activity and prolonged survival compared to control group.

  4. Intracranial Hypertension Syndrome in Systemic Lupus Erythema-tosus:Clinical Analysis and Review of the Literature

    Institute of Scientific and Technical Information of China (English)

    Zheng XUE; Xuezhen WANG; Fei LIU; Shaoxian HU; Suiqiang ZHU; Suming ZHANG; Bitao BU

    2009-01-01

    In order to better understand the clinical manifestation of systemic lupus erythematosus (SLE) with intracranial hypertension syndrome (IHS),we analyzed the clinical features and treatment of a typical SLE patient with IHS.SLE is one of the most unpredictable autoimmune diseases in-volving multiple organ systems that is defined clinically and associated with antibodies directed against cell nuclei.IHS is an uncommon manifestation of neuropsychiatric SLE (NPSLE) and is characterized by an elevated intracranial pressure,papilledema,and headache with occasional ab-ducens nerve paresis,absence of a space-occupying lesion or ventricular enlargement,and normal cerebrospinal fluid chemical and hematological constituents.IHS has been reported in a few sporadic cases in patients with SLE worldwide,but rarely has been reported in China.In this study,a 34-year-old female SLE patient with IHS was.reported and pertinent literature reviewed.The clinical presentation,image logical features,and investigatory findings were discussed.

  5. Primary antiphospholipid syndrome progressing to systemic lupus erythematosus: a case report

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    Rocco Manganelli

    2013-04-01

    Full Text Available Introduction: Primary antiphospholipid syndrome (APS is a thrombophilic disease that should be suspected in the presence of thrombotic events associated with hematologic abnormalities such as thrombocytopenia and prolongation of the activated partial thromboplastin time. The diagnosis must be confirmed by the demonstration of autoantibodies directed against anionic phospholipids and/or phospholipid-binding proteins. The disease can cause arterial thrombosis in any vascular district, including those of the kidney and central nervous system. Case report: In 2006 a 29-year-old male presented with kidney and brain involvement that was attributed to primary APS. The clinical diagnosis was confirmed by the results of a renal biopsy, which excluded the presence of systemic lupus erythematosus (SLE. The patient remained stable through 32 months of follow-up and then developed a malar rash with deteriorating renal function, decreasing platelet count, and reduced complement levels. Serological studies revealed positivity for ANA (homogeneous pattern, dsDNA, ACA, and beta-2-glycoprotein-1 antibodies. The diagnosis was revised to APS secondary to SLE. Conclusions: A diagnosis of primary APS should not be considered permanent: progression to SLE can occur, in some cases years after the original diagnosis. This case highlights the importance of ongoing follow-up of patients diagnosed with primary APS to detect changes that herald the emergence of SLE.

  6. Metabolic Profiling of Systemic Lupus Erythematosus and Comparison with Primary Sjogren's Syndrome and Systemic Sclerosis.

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    Anders A Bengtsson

    Full Text Available Systemic lupus erythematosus (SLE is a chronic inflammatory autoimmune disease which can affect most organ systems including skin, joints and the kidney. Clinically, SLE is a heterogeneous disease and shares features of several other rheumatic diseases, in particular primary Sjögrens syndrome (pSS and systemic sclerosis (SSc, why it is difficult to diagnose The pathogenesis of SLE is not completely understood, partly due to the heterogeneity of the disease. This study demonstrates that metabolomics can be used as a tool for improved diagnosis of SLE compared to other similar autoimmune diseases. We observed differences in metabolic profiles with a classification specificity above 67% in the comparison of SLE with pSS, SSc and a matched group of healthy individuals. Selected metabolites were also significantly different between studied diseases. Biochemical pathway analysis was conducted to gain understanding of underlying pathways involved in the SLE pathogenesis. We found an increased oxidative activity in SLE, supported by increased xanthine oxidase activity and an increased turnover in the urea cycle. The most discriminatory metabolite observed was tryptophan, with decreased levels in SLE patients compared to control groups. Changes of tryptophan levels were related to changes in the activity of the aromatic amino acid decarboxylase (AADC and/or to activation of the kynurenine pathway.

  7. Clinical and endoscopic assessment of gastric state in systemic lupus erythematosus and antiphospholipid syndrome

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    I. A. Doroshkevich

    2004-01-01

    Full Text Available Objective. To characterize gastric mucosa (GM state in systemic lupus erythematosus (SLE and antiphospholipid syndrome (APS. Material and methods. Gastroscopy (GS with GM biopsy and histological examination was performed in 85 pts (65 with SLE and 20 with primary APS. 26 from 65 pts with SLE had secondary APS. 21 SLE pt before inclusion did not receive glucocorticoids (GC. H. pylory and its cytotoxicity gen CagA, HSV-I, CMV were examined in GM samples with PCR. Results. The most frequent GS-feature in pts with SLE and ARS was antral gastritis (82%. In 25% of pts erosions and in 8% - hemorrhages of GM were present. Erosions localized mostly in stomach (25%, in 7% of cases they were present in duodenum. In APS pts epigastric pain and GM erosions were more frequent than in SLE without APS. H.pylory in GM was revealed in 70-81%. In 42% of pts it was present in combination with HSV-1 and/or CMV. In more than half of pts with antral gastritis and GM erosions revealed H. pylory was CagA-positive. GC therapy did not influence frequency of GM erosions and hemorrhages formation. Conclusion. The most frequent GS-features in pts with SLE and ARS were antral gastritis and GM erosions. Epigastric pain and GM erosions were more frequent in pts with APS.

  8. Autoimmune/Inflammatory Syndrome Induced by Adjuvants and Thyroid Autoimmunity

    Science.gov (United States)

    Watad, Abdulla; David, Paula; Brown, Stav; Shoenfeld, Yehuda

    2017-01-01

    The autoimmune/inflammatory syndrome induced by adjuvants (ASIA), presented by Shoenfeld and Agmon-Levin in 2011, is an entity that incorporates diverse autoimmune conditions induced by the exposure to various adjuvants. Adjuvants are agents that entail the capability to induce immune reactions. Adjuvants are found in many vaccines and used mainly to increase the response to vaccination in the general population. Silicone has also been reported to be able to induce diverse immune reactions. Clinical cases and series of heterogeneous autoimmune conditions including systemic sclerosis, systemic lupus erythematosus, and rheumatoid arthritis have been reported to be induced by several adjuvants. However, only a small number of cases of autoimmune thyroid disorder have been included under the umbrella of ASIA syndrome. Indeed, clinical cases of Hashimoto’s thyroiditis and/or subacute thyroiditis were observed after the exposure to vaccines as well as silicone implantation. In our review, we aimed to summarize the current knowledge on ASIA syndrome presented as endocrinopathies, focusing on autoimmune thyroid disorders associated with the various adjuvants. PMID:28167927

  9. Lupus and pulmonary nodules consistent with bronchiolitis obliterans organizing pneumonia induced by carbamazepine in a man

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    Awatef Kelati

    2016-10-01

    Full Text Available Several drugs have been implicated in the induction of systemic lupus erythematosus (SLE, but there are only some observations of carbamazepine induced SLE since the first case described in 1966, this drug has also been implicated in the induction of other disorders and rarely pulmonary toxicity; but the occurrence of two rare side effects of this drug: the induced SLE and pulmonary nodules consistent with the bronchiolitis obliterans organizing pneumonia in same patient is really unusual and -to our knowledge- the second observation reported in the literature.

  10. An increased frequency of autoantibody-inducing CD4+ T cells in pre-diseased lupus-prone mice.

    Science.gov (United States)

    Busser, Brian W; Cancro, Michael P; Laufer, Terri M

    2004-07-01

    Pathogenic autoantibody production in murine models of lupus is dependent on autoreactive CD4+ helper T cells. However, the mechanisms which permit the selection and maintenance of this autoantibody-inducing CD4+ T-cell repertoire are currently unknown. We hypothesized that the peripheral CD4+ T-cell repertoire of lupus-prone mice was enriched with autoantibody-inducing specificities. To test this, we utilized the splenic focus assay to determine if pre-diseased lupus-prone (NZB x NZW)F(1) mice have an elevated frequency of autoreactive CD4+ T lymphocytes capable of supporting autoantibody production. The splenic focus limiting dilution assay permits anti-nuclear antibodies to be generated from contact-dependent T-B interactions in vitro. We show that young, pre-diseased lupus-prone mice have an elevated frequency of autoantibody-inducing CD4+ T cells. Interestingly, these autoantibody-inducing CD4+ T-cell responses are also present in the thymus. Therefore, an elevated frequency of autoantibody-inducing CD4+ T cells predisposes lupus-prone mice to the development of autoantibodies.

  11. Topical drug-induced subacute cutaneous lupus erythematosus isolated to the hands

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    Ramachandran, Sarika M; Leventhal, Jonathan S; Franco, Loren G; Mir, Adnan; Walters, Ruth F; Franks, Andrew G

    2017-01-01

    Subacute cutaneous lupus erythematosus (SCLE) is a well-defined subtype of lupus erythematosus, characterised by photosensitivity, annular and/or psoriasiform lesions, variable systemic involvement and presence of circulating SSA/anti-Ro antibodies. SCLE may be idiopathic or drug-induced. Both the idiopathic and drug-induced forms of SCLE are analogous in their clinical, serological and histological features. Drug-induced SCLE has been reported with various oral agents, but to our knowledge this is the first reported case due to a topical medication. A 34-year-old female foot masseuse presented with a 2-month history of scaly, erythematous lesions isolated to the dorsal hands and interdigital spaces. She had used topical terbinafine, a topical antifungal cream, to her clients’ feet for a number of years. ANA and anti-SSA/Ro antibodies were positive. Physical examination, serology and histopathology were consistent with SCLE. We propose that our patient's unique presentation of SCLE may be explained by a prolonged occupational exposure to topical terbinafine as a foot masseuse. While oral terbinafine is a drug known to cause drug-induced SCLE, to our knowledge, this is the first topically induced form of the disease. PMID:28331627

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

    Science.gov (United States)

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

    2016-05-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

  14. Clinical Features of Systemic Lupus Erythematosus Patients Complicated With Evans Syndrome: A Case-Control, Single Center Study.

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    Zhang, Lili; Wu, Xiuhua; Wang, Laifang; Li, Jing; Chen, Hua; Zhao, Yan; Zheng, Wenjie

    2016-04-01

    The aim of the study was to investigate the clinical features of systemic lupus erythematous (SLE) complicated with Evans syndrome (ES). We conducted a retrospective case-control study to compare the clinical and laboratory features of age- and gender-matched lupus patients with and without ES in 1:3 ratios. In 5724 hospitalized SLE patients, we identified 27 (0.47%, 22 women and 5 men, average age 34.2 years) SLE patients complicated with ES. Fifteen patients (55.6%) presented with hematologic abnormalities initially, including 6 (22.2%) cases of isolated ITP, 4 (14.8%) cases of isolated AIHA, and 5 (18.5%) cases of classical ES. The median intervals between hematological presentations the diagnosis of SLE was 36 months (range 0-252). ES developed after the SLE diagnosis in 4 patients (14.8%), and concomitantly with SLE diagnosis in 8 patients (29.6%). Systemic involvements are frequently observed in SLE patients with ES, including fever (55.6%), serositis (51.9%), hair loss (40.7%), lupus nephritis (37%), Raynaud phenomenon (33.3%), neuropsychiatric (33.3%) and pulmonary involvement (25.9%), and photosensitivity (25.9%). The incidence of photosensitivity, hypocomplementemia, elevated serum IgG level, and lupus nephritis in patients with ES or without ES was 25.9% vs 6.2% (P = 0.007), 88.9% vs 67.1% (P = 0.029), 48.1% vs 24.4% (P = 0.021), and 37% vs 64.2% (P = 0.013), respectively. Twenty-five (92.6%) patients achieved improvement following treatment of glucocorticoids and immunosuppressants as well as splenectomy, whereas 6 patients experienced the relapse and 1 patient died from renal failure during the follow-up. ES is a relatively rare complication of SLE. Photosensitivity, hypocomplementemia, and elevated serum IgG level were frequently observed in ES patients, but lupus nephritis was less observed. More than half of patients presented with hematological manifestation at onset, and progress to typical lupus over months to years. Therefore

  15. Drug induced pseudolymphoma syndrome

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    Mittal R

    1994-01-01

    Full Text Available Five cases of pseudolymphoma syndrome (PS in children aged 6 to 12 years were observed after anticonvulsant drugs. In 2 cases PS was observed after 10 days and in 3 after 15 days therapy with offending drug. 3 cases of PS were due to carbamazepine and had morbilliform rash and 2 cases due to phenobarbitone had erythroderma. All had fever, generalised lymphadenopathy and 4/5 had hepatosplenomegaly. Therapy with 15 mg prednisolone daily and withdrawal of offending drug led to cure in 4/5 cases and 1 died due to congestive cardiac failure.

  16. Severity of dry eye syndrome is related to anti-dsDNA autoantibody in systemic lupus erythematosus patients without secondary Sjogren syndrome: A cross-sectional analysis.

    Science.gov (United States)

    Chen, Alexander; Chen, Hung-Ta; Hwang, Yih-Hsiou; Chen, Yi-Tsun; Hsiao, Ching-Hsi; Chen, Hung-Chi

    2016-07-01

    There are as many as one-third of the systemic lupus erythematosus (SLE) patients who suffer from dry eye syndrome. To this date, dry eye syndrome in SLE patients is believed to be caused by secondary Sjogren syndrome (sSS). However, there is increasing evidence for possible independency of dry eye syndrome and sSS in patients suffering from autoimmune diseases. The purpose of this retrospective observational case series was to identify SLE patients without sSS who had dry eye syndrome, examine the correlation of different autoantibodies and dry eye severity, and determine the cause of dry eye in these patients.We included 49 consecutive SLE patients with dry eye who visited our dry eye clinic. In order to rule out sSS, these patients were all negative for anti-Sjogren's-syndrome-related antigen A and B (anti-SSA/SSB) and had no oral symptoms. Each patient's lupus activity was determined by serological tests including antidouble-stranded DNA antibody (anti-dsDNA), complement levels (C3, C4), erythrocyte sedimentation rate (ESR), and antinuclear antibody (ANA). Severity of dry eye syndrome was determined by corneal sensation (KSen), superficial punctuate keratopathy (SPK), Schirmer-I test (Schirmer), and tear film break-up time (TBUT). The autoantibodies and the dry eye parameters in each group were tested using the χ test or the Mann-Whitney U test for normally distributed or skewed data, respectively.The anti-dsDNA showed significant correlations with KSen (P syndrome in SLE patients without sSS was strongly correlated with anti-dsDNA and C3 but not with C4, ESR, and ANA.

  17. Severity of dry eye syndrome is related to anti-dsDNA autoantibody in systemic lupus erythematosus patients without secondary Sjogren syndrome

    Science.gov (United States)

    Chen, Alexander; Chen, Hung-Ta; Hwang, Yih-Hsiou; Chen, Yi-Tsun; Hsiao, Ching-Hsi; Chen, Hung-Chi

    2016-01-01

    Abstract There are as many as one-third of the systemic lupus erythematosus (SLE) patients who suffer from dry eye syndrome. To this date, dry eye syndrome in SLE patients is believed to be caused by secondary Sjogren syndrome (sSS). However, there is increasing evidence for possible independency of dry eye syndrome and sSS in patients suffering from autoimmune diseases. The purpose of this retrospective observational case series was to identify SLE patients without sSS who had dry eye syndrome, examine the correlation of different autoantibodies and dry eye severity, and determine the cause of dry eye in these patients. We included 49 consecutive SLE patients with dry eye who visited our dry eye clinic. In order to rule out sSS, these patients were all negative for anti-Sjogren's-syndrome-related antigen A and B (anti-SSA/SSB) and had no oral symptoms. Each patient's lupus activity was determined by serological tests including antidouble-stranded DNA antibody (anti-dsDNA), complement levels (C3, C4), erythrocyte sedimentation rate (ESR), and antinuclear antibody (ANA). Severity of dry eye syndrome was determined by corneal sensation (KSen), superficial punctuate keratopathy (SPK), Schirmer-I test (Schirmer), and tear film break-up time (TBUT). The autoantibodies and the dry eye parameters in each group were tested using the χ2 test or the Mann–Whitney U test for normally distributed or skewed data, respectively. The anti-dsDNA showed significant correlations with KSen (P syndrome in SLE patients without sSS was strongly correlated with anti-dsDNA and C3 but not with C4, ESR, and ANA. PMID:27428227

  18. Lupus erythematosus and lichen planus overlap syndrome: a case report with a rapid response to topical corticosteroid therapy

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    Gulsen Tukenmez Demirci

    2011-11-01

    Full Text Available Lupus erythematosus (LE and lichen planus (LP may occur as an overlap syndrome. We report the clinical characteristics of a young man with lesions diagnosed as LE and LP by histopathological and direct immunoflurosence examinations. We achieved remarkable clinical response from the treatment with topical corticosteroids and no recurrence was seen in a 6 months of follow up time. We found this case interesting because of the rapid improvement with corticosteroid and discussed if there is a real overlap or a coexistence according to the literature.

  19. Atherosclerotic vessel damage in systemic lupus erythematosus and antiphospholipid syndrome in men

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    A. I. Iljina

    2005-01-01

    Full Text Available Objective. To study prevalence of clinical and subclinical atherosclerosis signs in men with systemic lupus erythematosus (SLE and antiphospholipid syndrome, to assess relationship between atherosclerotic vessel damage, risk factors, CRP and anti-cardiolipin antibodies (АСА Material and methods. 62 pts were included. Mean age was 35,7+11,6 years, mean disease duration - 129,3± 102 months. Traditional and related to the disease risk factors were analyzed. To reveal atherosclerotic vessel damage carotid sonographic examination was performed. Serum CRP concentration was evaluated by high sensitivity nephelometric immunoassay. IgG and IgM АСА were assessed by solid-phase immuno-enzyme assay. Results. Sonographic signs of carotid damage was revealed in 58% of pts, clinical signs of atherosclerosis - in 42%. Pts were divided into two groups according to intima-media complex thickness (IMCT. Group I included 36 pts with atherosclerotic vessel damage signs (IMCT?0,9 mm. Group 2-26 pts with IMCT<0,9 mm. Mean age at the examination, age of disease onset, disease duration, smoking frequency damage index in group I pts were higher than in group 2 pts. Mean CRP concentration in atherosclerosis group was significantly higher than in group 2 (p=0,007. 19 pts had APS signs. 43 pts did not. CRP level significantly correlated with IMCT in SLE pts with and without APS (p<0,05. Pts with atherosclerosis had higher IgG АСА level though the differences were not statistically significant. Conclusion. Men with SLE with or without APS have high risk of atherosclerosis development. CRP elevation is associated with IMCT increase.

  20. Acute respiratory distress syndrome in a pregnant woman with systemic lupus erythematosus: a case report.

    Science.gov (United States)

    Chen, Y-J A; Tseng, J-J; Yang, M-J; Tsao, Y-P; Lin, H-Y

    2014-12-01

    When the disease activity of systemic lupus erythematosus (SLE) is controlled appropriately, a pregnant woman who has lupus is able to carry safely to term and deliver a healthy infant. While the physiology of a healthy pregnancy itself influences ventilatory function, acute pulmonary distress may decrease oxygenation and influence both mother and fetus. Though respiratory failure in pregnancy is relatively rare, it remains one of the leading conditions requiring intensive care unit admission in pregnancy and carries a high risk of maternal and fetal morbidity and mortality, not to mention the complexity caused by lupus flare. We report a case of SLE complicated with lupus pneumonitis and followed by acute respiratory distress during pregnancy. Though there is a high risk of maternal and fetal morbidity and mortality, maternal respiratory function improved after cesarean section and treatment of the underlying causes. The newborn had an extremely low birth weight but was well at discharge.

  1. Síndrome do lúpus neonatal Neonatal lupus syndrome

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    Jozélio Freire de Carvalho

    2005-06-01

    Full Text Available A síndrome do lúpus neonatal (SLN é uma doença auto-imune associada à presença de auto-anticorpos na circulação materno-fetal contra complexos ribonucléicos, SSA/Ro e SSB/La, e se caracteriza principalmente por bloqueio cardíaco congênito isolado (BCCI e/ou manifestações cutâneas e hematológicas. A despeito da sua raridade, a SLN é a principal causa de BCCI, sendo responsável pela importante mortalidade (20% a 30% e morbidade desses pacientes. A denominação de lúpus neonatal se baseia na semelhança das lesões cutâneas associadas à SLN nos neonatos com aquelas observadas em pacientes com lúpus eritematoso sistêmico (SLE. Por outro lado, o termo "isolado", para designar o BCC na SLN, é utilizado para especificar a ausência de malformações cardíacas congênitas e a ausência de infecções que causam alterações na condução átrio-ventricular (BAV. A SLN constitui-se num clássico modelo de auto-imunidade adquirida, no qual os anticorpos IgG maternos atravessam a barreira placentária e na circulação fetal podem exercer um papel importante na patogênese da síndrome. A presença quase universal dos anticorpos anti-Ro/SSA e anti-SSB/La no soro materno e fetal os inclui como marcadores para a SLN. Ao contrário da lesão cardíaca que compromete irreversivelmente a condução átrio-ventricular, os acometimentos cutâneos e/ou hematológicos são transitórios e podem regredir após o desaparecimento dos anticorpos maternos da circulação do lactente. Clinicamente, a SLN representa um desafio para profissionais reumatologistas, obstetras, neonatalogistas, dermatologistas e cardiologistas pediátricos que têm como meta identificar o risco gestacional de desenvolvimento da doença fetal, diagnosticar a síndrome precocemente e definir uma estratégia terapêutica adequada quando "in utero" ou pós-natal.Neonatal Lupus Syndrome (NLS is an autoimmune disease associated to the presence of autoantibodies against

  2. Action spectrum and mechanisms of UV radiation-induced injury in lupus erythematosus

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    Kochevar, I.E.

    1985-07-01

    Photosensitivity associated with lupus erythematosus (LE) is well established. The photobiologic basis for this abnormal response to ultraviolet radiation, however, has not been determined. This paper summarizes the criteria for elucidating possible photobiologic mechanisms and reviews the literature relevant to the mechanism of photosensitivity in LE. In patients with LE, photosensitivity to wavelengths shorter than 320 nm has been demonstrated; wavelengths longer than 320 nm have not been adequately evaluated. DNA is a possible chromophore for photosensitivity below 320 nm. UV irradiation of skin produces thymine photodimers in DNA. UV-irradiated DNA is more antigenic than native DNA and the antigenicity of UV-irradiated DNA has been proposed, but not proven, to be involved in the development of clinical lesions. UV irradiation of mice previously injected with anti-UV-DNA antibodies produces Ig deposition and complement fixation that appears to be similar to the changes seen in lupus lesions. Antibodies to UV-irradiated DNA occur in the serum of LE patients although a correlation between antibody titers and photosensitivity was not observed. Defective repair of UV-induced DNA damage does not appear to be a mechanism for the photosensitivity in LE. Other mechanisms must also be considered. The chromophore for photosensitivity induced by wavelengths longer than 320 nm has not been investigated in vivo. In vitro studies indicate that 360-400 nm radiation activates a photosensitizing compound in the lymphocytes and serum of LE patients and causes chromosomal aberrations and cell death. The mechanism appears to involve superoxide anion.

  3. Regulatory Effect of Melatonin on Cytokine Disturbances in the Pristane-Induced Lupus Mice

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    Ling-ling Zhou

    2010-01-01

    Full Text Available Systemic lupus erythematosus (SLE develops in relation to many environmental factors. In our opinion, it is more important to investigate the effect of melatonin on the environmental- related SLE. In the present study, 0.5 ml pristane were used to induce SLE in female BALB/c mice. Melatonin (0.01, 0.1, 1.0 mg/kg was orally administered immediately after pristane-injection for 24 weeks. IgM anti ssDNA and histone antibodies were detected after 0, 1, 2, 4, 8 weeks pristane injection. The levels of IL-2, IL-6 and IL-13 were detected after 24 weeks. Renal lesions were also observed. The results showed that melatonin antagonized the increasing levels of IgM anti ssDNA and histone autoantibodies. Melatonin could also decrease the IL-6 and IL-13 production and increase the IL-2 production. Besides, melatonin could lessen the renal lesions caused by pristane. These results suggested that melatonin has a beneficial effect on pristane-induced lupus through regulating the cytokines disturbances.

  4. Adalimumab (TNFα Inhibitor Therapy Exacerbates IgA Glomerulonephritis Acute Renal Injury and Induces Lupus Autoantibodies in a Psoriasis Patient

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    S. S. Wei

    2013-01-01

    Full Text Available Adalimumab (Humira is a tumour necrosis factor α (TNFα inhibitor that is approved for the treatment of rheumatoid arthritis, psoriasis, psoriatic arthritis, Crohn's disease, ankylosing spondylitis, and juvenile idiopathic arthritis (Sullivan and Preda (2009, Klinkhoff (2004, and Medicare Australia. Use of TNFα inhibitors is associated with the induction of autoimmunity (systemic lupus erythematosus, vasculitis, and sarcoidosis or sarcoid-like granulomas (Ramos-Casals et al. (2010. We report a patient with extensive psoriasis presenting with renal failure and seropositive lupus markers without classical lupus nephritis after 18 months treatment with adalimumab. He has renal biopsy proven IgA nephritis instead. Renal biopsy is the key diagnostic tool in patients presenting with adalimumab induced nephritis and renal failure. He made a remarkable recovery after adalimumab cessation and steroid treatment. To our knowledge, this is a unique case of a psoriasis patient presenting with seropositive lupus markers without classical lupus nephritis renal failure and had renal biopsy proven IgA glomerulonephritis after receiving adalimumab.

  5. Macrophage activation syndrome in children with systemic juvenile idiopathic arthritis and systemic lupus erythematosus.

    Science.gov (United States)

    Aytaç, Selin; Batu, Ezgi Deniz; Ünal, Şule; Bilginer, Yelda; Çetin, Mualla; Tuncer, Murat; Gümrük, Fatma; Özen, Seza

    2016-10-01

    Macrophage activation syndrome (MAS) is a hyper-inflammatory disorder secondary to a rheumatic disease such as systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus (SLE). We aimed to present the characteristics of our pediatric MAS patients. Clinical features, laboratory parameters, treatment, and outcome of 34 patients (28 SJIA; six SLE; 37 MAS episodes) followed at a tertiary health center between 2009 and 2015 were retrospectively reviewed. The median age at MAS onset was 11 years. More SJIA patients had MAS at disease onset than SLE patients (53.6 vs. 16.7 %). Fever, high C-reactive protein and hyperferritinemia were present in all MAS episodes. Rash was less (p = 0.03), and fatigue was more frequent (p = 0.042) in SLE than SJIA patients. All received corticosteroids. Cyclosporine was given in 74.2 % of SJIA-MAS; 66.7 % of SLE-MAS episodes. Intravenous immunoglobulin, anakinra, or etoposide was administered during 67.7; 41.9; 32.3 % of SJIA-MAS and 33.3; 33.3; 50 % of SLE-MAS episodes, respectively. Plasmapheresis was performed during 41.9 % of SJIA-MAS and 33.3 % of SLE-MAS episodes. The mortality rate was 11.8 % (n = 4;3 SJIA, 1 SLE). Hepatosplenomegaly was more frequent (p = 0.005), and plasmapheresis was performed more frequently (p = 0.021) in the patients who died compared to the cured patients. The median duration between symptom onset and admission to our hospital was longer among the patients who died (16.5 vs. 7 days; p = 0.049). Our patients' characteristics were similar to the reported cases, but our mortality rate is slightly higher probably due to late referral to our center. Early diagnosis and effective treatment are crucial to prevent mortality.

  6. Antinuclear antibody-negative, drug-induced lupus caused by lisinopril.

    Science.gov (United States)

    Carter, J D; Valeriano-Marcet, J; Kanik, K S; Vasey, F B

    2001-11-01

    The clinical symptoms of drug-induced lupus (DIL) are similar to those of idiopathic systemic lupus erythematosus. The literature indicates that in patients with DIL, sera generally contain antinuclear antibodies (ANAs); however, ANA-negative DIL has been reported. The list of medications implicated as etiologic agents in DIL continues to grow. This list includes two different types of angiotensin-converting enzyme inhibitors--captopril and enalapril. We report the first case of DIL caused by lisinopril. Our patient had negative results on ANA testing and had histone antibodies (IgG anti-[H2A-H2B]-DNA) mirroring the disease course. Antibodies to the (H2A-H2B)-DNA complex are seen in more than 90% of patients with active DIL, excluding those with DIL due to hydralazine. Thus, it is important to recognize the clinical significance of IgG anti-(H2A-H2B)-DNA antibodies and that negative ANA test results do not preclude the diagnosis of DIL.

  7. Hepatic venous outflow block in a young patient with Systemic Lupus Erythematosus

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    Ali Ghavidel

    2015-08-01

    Full Text Available Introduction: Hepatic venous outflow block or Budd-Chiari syndrome is a severe liver disease with a 3 years survival rate of 50%. Several conditions have been implicated as a cause of Budd-Chiari syndrome, including myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, the presence of lupus anti-coagulant, oral contraceptives, pregnancy, and others. In a small number of cases, Budd-Chiari syndrome is associated with the presence of lupus anticoagulant. Anticardiolipin antibodies (ACA are similar to lupus anti-coagulant antiphospholipid antibodies (APLAs, which have been described in patients with recurrent arterial and venous thrombosis, thrombocytopenia, fetal loss, or miscarriage. Case Report: A 23-year-old woman is reported with Budd-Chiari syndrome in whom lupus anticoagulant and anticardiolipin antibodies were shown; 9 months after diagnosis of systemic lupus erythematosus (SLE treatment with steroids admitted with gastrointestinal problems, abdominal pain and ascites and treated oral anticoagulants induced a considerable improvement. This treatment was continued after 1 year, but interruption was followed by redevelopment of ascites. Further treatment with anticoagulants was continued for 5 years with noticeable improvement. Conclusion: Patients with Budd-Chiari syndrome should be tested for lupus anticoagulants and anticardiolipin antibodies, Budd-Chiari syndrome resulting from this cause may have a good response to treatment with oral anticoagulants; this treatment should be maintained permanently, and pregnancy in such patients may initiate serious difficulties. The condition of the patient at follow-up was good.

  8. An Overlap Syndrome involving systemic lupus erythematosus and autoimmune hepatitis in an adolescent girl.

    Science.gov (United States)

    Battagliotti, Cristina; Rispolo Klubek, Daniela; Karakachoff, Mario; Costaguta, Alejandro

    2016-06-01

    La superposición del lupus eritematoso sistémico y la hepatitis autoinmune se describe ocasionalmente. Aunque ambas enfermedades pueden compartir ciertos hallazgos, como poliartralgias, hipergammaglobulinemia y anticuerpo antinúcleo positivo, son consideradas dos diferentes. Se presenta a una paciente de 14 años con lupus eritematoso sistémico, que, luego de dos años, consultó por ictericia. Sin antecedentes de ingesta de drogas, alcohol o exposición a virus hepatotropos. Tenia un aumento de las enzimas hepáticas con anticuerpos antinúcleo, anti-ADN de doble cadena y LKM 1 positivos. La biopsia hepática mostró una hepatitis de interfase con infiltrado linfoplasmocitario. De esta manera, cumplia con los criterios diagnósticos tanto para lupus eritematoso sistémico como para hepatitis autoinmune. Tratada con corticoides y micofenolato mofetil, mejoró su clinica y laboratorio. Conclusión. La hepatitis autoinmune puede ocurrir en el curso del lupus eritematoso sistémico. Un diagnóstico temprano es importante para prevenir el avance de la enfermedad; es obligatoria la realización de la biopsia hepática.

  9. Clinical analysis of pregnancy-induced systemic lupus erythematosus complicated by multiple organ dysfuntion syndromes:A report of 7 cases%SLE患者妊娠期并发多脏器功能障碍综合征7例临床分析

    Institute of Scientific and Technical Information of China (English)

    申震; 赵卫东; 吴大保; 张秀英

    2006-01-01

    目的:探讨系统性红斑狼疮(systemic lupus erythematosus,SLE)患者妊娠期并发多脏器功能障碍综合征(mutiple organ dysfunctiong syndrome,MODS)的预防与诊治.方法:对7例SLE妊娠期引起的多脏器功能损害临床资料进行回顾性分析.结果:2例妊娠前已确诊为SLE,5例入院后确诊SLE.孕产妇抢救成功6例,1例放弃治疗,围生儿死亡6例.结论:SLE患者妊娠并发多脏器功能障碍对孕产妇及围生儿危害极大,处理的关键是尽早确诊并积极治疗原发病;适时终止妊娠,保护相关脏器功能和全身支持疗法至关重要.

  10. Immunity and early atherosclerosis in the course of systemic lupus erythematosus, mixed connective tissue disease and antiphospholipid syndrome.

    Science.gov (United States)

    Haładyj, Ewa; Paradowska-Gorycka, Agnieszka; Felis-Giemza, Anna; Olesińska, Marzena

    2016-01-01

    Atherosclerosis is a chronic inflammatory disease of the arteries associated with various risk factors that promote lipid abnormalities, development and progression of atherosclerotic lesions, plaque rupture, and vascular thrombosis. Atherosclerosis is accelerated in autoimmune diseases. Non-invasive investigations showed increased intima-media thickness (IMT), carotid plaque, and coronary artery calcifications in patients with antiphospholipid syndrome, systemic lupus erythematosus and mixed connective tissue disease compared to controls. The balance between the proinflammatory and anti-inflammatory cytokines allows the immune equilibrium to be maintained. In autoimmune diseases the prevalence of proinflammatory factors leads to premature atherosclerosis. This review presents complementary knowledge on innate and adaptive immunity, cytokines and the role of inflammasomes in progression of early atherosclerosis.

  11. Immunity and early atherosclerosis in the course of systemic lupus erythematosus, mixed connective tissue disease and antiphospholipid syndrome

    Science.gov (United States)

    Paradowska-Gorycka, Agnieszka; Felis-Giemza, Anna; Olesińska, Marzena

    2016-01-01

    Atherosclerosis is a chronic inflammatory disease of the arteries associated with various risk factors that promote lipid abnormalities, development and progression of atherosclerotic lesions, plaque rupture, and vascular thrombosis. Atherosclerosis is accelerated in autoimmune diseases. Non-invasive investigations showed increased intima-media thickness (IMT), carotid plaque, and coronary artery calcifications in patients with antiphospholipid syndrome, systemic lupus erythematosus and mixed connective tissue disease compared to controls. The balance between the proinflammatory and anti-inflammatory cytokines allows the immune equilibrium to be maintained. In autoimmune diseases the prevalence of proinflammatory factors leads to premature atherosclerosis. This review presents complementary knowledge on innate and adaptive immunity, cytokines and the role of inflammasomes in progression of early atherosclerosis. PMID:27826173

  12. Interferon-inducible Ifi200-family genes as modifiers of lupus susceptibility

    OpenAIRE

    2012-01-01

    Both genetic and environmental factors contribute to the development and progression of systemic lupus erythematosus (SLE), a complex autoimmune disease. The disease exhibits a strong gender bias and develops predominantly in females. Additionally, most SLE patients exhibit increased serum levels of interferon-α (IFN-α) and the “IFN signature”. Studies using the mouse models of lupus have identified several lupus susceptibility loci, including the New Zealand Black (NZB)-derived autoimmunity ...

  13. Successful pregnancy outcome in grade IV lupus nephritis and secondary antiphospholipid antibody syndrome with recurrent pregnancy failures - challenging achievement of motherhood

    Directory of Open Access Journals (Sweden)

    Kaliki Hymavathi Reddy

    2016-12-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic multisystem autoimmune disease that occurs predominantly in women of childbearing age. The risk of complications and adverse fetal outcomes in pregnant women with lupus is high viz., increased risks of preterm birth, hypertensive diseases of pregnancy and lupus flares both during pregnancy and in the postpartum period. An additional association with Antiphospholipid antibody (APLA syndrome is expected to multiply the pregnancy complications. Though improved understanding of the disease nature and greater number of therapeutic options in the treatment of SLE, made the medical community regard these patients with less trepidation, the risk of significant morbidity to both the mother and the fetus still exist. We report an interesting case of grade IV Lupus nephritis (LN with secondary APLA syndrome and h/o recurrent pregnancy failures for twenty times but had a successful pregnancy and delivery in the 21st attempt though pregnancy was absolutely contraindicated in view of her medical illness. Many complications were encountered during her pregnancy which could be successfully tackled and a live male baby was delivered by Caesarean section.

  14. 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...

  15. Ifosfamide-Induced Fanconi's Syndrome

    Directory of Open Access Journals (Sweden)

    Lin-Kun Lee

    2014-06-01

    Full Text Available Ifosfamide is an alkylating antineoplastic prodrug used to treat many solid tumors. The metabolism of ifosfamide is via CYP450 3A4 and 2B6 and produces active ifosfamide mustard, the toxic metabolite acrolein and chloroacetaldehyde (CAA. Additionally, CAA is believed to induce proximal tubular dysfunction which results in Fanconi's syndrome. It is a condition not commonly encountered in adults receiving ifosfamide but relatively common in children. Herein, we have reported a 25-year-old woman with a history of synovial sarcoma with multiple lung metastasis and repetitive locoregional recurrence. She received chemotherapy with high dose ifosfamide as her antineoplastic treatment. Before her 4th cycle of chemotherapy, the patient's pre-chemotherapy evaluation revealed proteinuria, glucosuria, phosphateuria, hypophosphatemia and non-anion gap metabolic acidosis. The above conditions were consistent with Fanconi's syndrome. We treated her with electrolyte supplement and close monitoring of the noted laboratory abnormalities. Fortunately, the laboratory abnormality gradually resolved. Our case highlights the rare potential complication of ifosfamide, especially in patients who had received a high cumulive dose. To avoid this rare but potentially debilitating condition, patients whose cumulative ifosfamide dose reaches threshold should be closely monitored.

  16. Ibuprofen-induced hypersensitivity syndrome.

    Science.gov (United States)

    Nanau, Radu M; Neuman, Manuela G

    2010-06-01

    Ibuprofen is a widely used antipyretic and analgesic nonsteroidal antiinflammatory drug (NSAID). With the aging of the population, there will be a significant increase in the prevalence of painful degenerative and inflammatory rheumatic conditions. This increase likely will lead to a parallel increase in the use of NSAIDs, including ibuprofen. The primary effect of the NSAIDs is to inhibit cyclooxygenase (prostaglandin synthase), thereby impairing the ultimate transformation of arachidonic acid to prostaglandins, prostacyclin, and thromboxanes. Although in the majority of cases it is safe, this NSAID, ibuprofen, can produce an unpredictable, idiosyncratic, type B reaction that may pose a major concern in clinical practice. Type B reactions are known to occur in susceptible individuals. The true hypersensitivity reaction (HSR) is a systemic disease defined by the triad of fever, rash, and internal organ involvement that starts 1 day to 12 weeks after the initiation of therapy. HSR has limited the therapeutic use of many drugs, including ibuprofen. Hypersensitivity syndrome associated with ibuprofen is a host-dependent drug reaction that is idiosyncratic in nature. This reaction likely is caused by a combination of metabolic and immunologic factors. Immune mediated components, such as T-cell and their products cytokines and chemokines, can exacerbate cellular responses and create complex pathways that lead to a variety of clinical manifestations. Our review presents an ibuprofen-induced clinical manifestation of hypersensitivity syndrome and the necessity of wisely monitoring the patients clinically and by laboratory investigations when prescribing this drug.

  17. 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.

  18. Drug-Induced Subacute Cutaneous Lupus Erythematosus Associated with Proton Pump Inhibitors.

    Science.gov (United States)

    Aggarwal, Nitish

    2016-06-01

    Subacute cutaneous lupus erythematosus (SCLE) is an autoimmune disease that may be induced by proton pump inhibitors (PPIs) in at-risk populations. The US FDA does not recognize SCLE as an adverse event associated with PPIs. We queried the FDA Adverse Event Reporting System database, which contains adverse event case reports submitted by the public as well as by industry, and analyzed the data to quantify passive pharmacovigilance signals for SCLE associated with PPIs. A disproportionality analysis of the signals yielded a significant association between SCLE and PPIs. Discontinuation of PPI resulted in remission, with PPI re-challenge causing SCLE to reoccur. A follow-up analysis also yielded a significant association between SCLE and H2 receptor antagonists. We conducted a brief literature survey of published case reports and studies to discern the validity of PPI-induced SCLE signals. Healthcare prescribers and patients should be made aware that SCLE can be induced by PPIs. In such cases, PPIs should be discontinued and alternative clinical treatment sought. Regulatory bodies such as the FDA should incorporate the adverse reaction in PPI prescription labels.

  19. Living With Lupus

    Science.gov (United States)

    ... Information Lupus Find a Clinical Trial Journal Articles Lupus July 2014 Living With Lupus: Health Information Basics for You and Your Family ... If You Have Lupus? Symptom Checklist What Is Lupus? Lupus is an autoimmune (AW-toe-ih-MYOON) ...

  20. Posterior reversible encephalopathy syndrome could be an underestimated variant of “reversible neurological deficits” in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Liu Bin

    2012-12-01

    Full Text Available Abstract Background Posterior reversible encephalopathy syndrome (PRES has been increasingly identified in patients with systemic lupus erythematosus (SLE owing to the advance in neuroimaging techniques. Prompt diagnosis is pivotal to improve its outcome. To analyze the clinical and radiographic profile of PRES in patients with SLE and search for the appropriate treatment strategy PRES in SLE. Methods SLE patients who fulfilled the diagnostic criteria for PRES from August 2008 to January 2011 were evaluated at baseline, and followed to determine clinical outcomes. Data were analysis on clinical characteristics, laboratory abnormalities, treatment details, and outcomes. Results Ten episodes of PRES in patients with SLE were identified. All patients were female, mean age of onset was 22.93 ± 2.48 years, and SLEDAI at the onset of PRES were 25.8 ± 5.7. All cases had acute onset of headache, altered mental status, stupor, vomiting, cortical blindness and seizures. Neurological symptoms were the initial manifestation of SLE in three cases. Head magnetic resonance imaging (MRI demonstrated posterior white matter edema involving the parietal, temporal and occipital lobes, which were more conspicuous on T2 weighted spin echo and diffusion-weighted MR imaging (DWI than on computed tomography (CT scan. Complete clinical and radiographic recovery was observed in 8 patients after prompt treatment with corticosteroids. Conclusions PRES might be due to lupus per se besides other traditional causative factors such as hypertension. PRES might be an underestimated variant of “reversible neurological deficits” in SLE. Prompt recognition and timely management is important to prevent permanent neurological deficits.

  1. Rare Occurrence of Drug Induced Subacute Cutaneous Lupus Erythematosus with Leflunomide Therapy.

    Science.gov (United States)

    Singh, Harpreet; Sukhija, Gagandeep; Tanwar, Vikram; Arora, Sameer; Bhutani, Jaikrit

    2016-10-01

    Leflunomide is an immunomodulatory drug exhibiting anti-inflammatory, anti-proliferative and immunosuppressive effects. It has been widely used for treatment of active rheumatoid arthritis. Despite its good safety profile cutaneous side effects like alopecia, eczema, pruritis and dry skin have been reported with Leflunomide use. Skin ucleration, vasculitis, lichenoid drug rash and Subacute Cutaneous Lupus Erythematosus (SCLE) have been rarely reported with its use. A rare case of Leflunomide induced SCLE is being reported in a female patient with rheumatoid arthritis. The clinical features, histopathological and immunological characteristics were consistent with drug induced SCLE. Withdrawal of Leflunomide along with short course of topical steroids resulted in resolution of symptoms suggesting the drug to be the culprit. As this drug comes into widespread use, it remains to be seen whether more cases of DI-SCLE will occur/be reported. Fortunately, such a condition till times appears rare and is reversible once the drug is discontinued thus avoiding over evaluation and over treatment if the triggering drug is recognized.

  2. Rare Occurrence of Drug Induced Subacute Cutaneous Lupus Erythematosus with Leflunomide Therapy

    Science.gov (United States)

    Singh, Harpreet; Tanwar, Vikram; Arora, Sameer; Bhutani, Jaikrit

    2016-01-01

    Leflunomide is an immunomodulatory drug exhibiting anti-inflammatory, anti-proliferative and immunosuppressive effects. It has been widely used for treatment of active rheumatoid arthritis. Despite its good safety profile cutaneous side effects like alopecia, eczema, pruritis and dry skin have been reported with Leflunomide use. Skin ucleration, vasculitis, lichenoid drug rash and Subacute Cutaneous Lupus Erythematosus (SCLE) have been rarely reported with its use. A rare case of Leflunomide induced SCLE is being reported in a female patient with rheumatoid arthritis. The clinical features, histopathological and immunological characteristics were consistent with drug induced SCLE. Withdrawal of Leflunomide along with short course of topical steroids resulted in resolution of symptoms suggesting the drug to be the culprit. As this drug comes into widespread use, it remains to be seen whether more cases of DI-SCLE will occur/be reported. Fortunately, such a condition till times appears rare and is reversible once the drug is discontinued thus avoiding over evaluation and over treatment if the triggering drug is recognized. PMID:27891379

  3. Successful treatment of Raynaud’s syndrome in a lupus patient with continuous bilateral popliteal sciatic nerve blocks: a case report

    Science.gov (United States)

    Dao, Thuan; Amaro-Driedger, David; Mehta, Jaideep

    2016-01-01

    Raynaud’s syndrome has been treated medically and invasively, sometimes with regional anesthesia leading up to sympathectomy. We demonstrate that regional anesthesia was in this case a useful technique that can allow some patients to find temporary but significant relief from symptoms of Raynaud’s syndrome exacerbation. We present a 43-year-old woman with Raynaud’s syndrome secondary to lupus who was treated with bilateral popliteal nerve block catheters for ischemic pain and necrosis of her feet; this led to almost immediate resolution of her pain and return of color and function of her feet. While medical management should continue to be a front-line treatment for Raynaud’s syndrome, regional anesthesia can be useful in providing rapid dissipation of symptoms and may thus serve as a viable option for short-term management of this syndrome. PMID:27366104

  4. Coexisting Bacillus Calmette-Guérin-Induced Lupus Vulgaris Involving the Vaccination Site and Lichen Scrofulosorum in an Immunocompetent Boy.

    Science.gov (United States)

    Angoori, Gnaneshwar Rao

    2016-09-01

    The coexistence of Bacillus Calmette-Guérin (BCG)-induced lupus vulgaris involving the site of vaccination with lichen scrofulosorum is rare. Herein we report a 3-year-old boy who presented with lupus vulgaris at the vaccination site 3 weeks after neonatal BCG vaccination followed by the development of lichen scrofulosorum approximately 2.5 years later. Characteristic clinical morphology, typical histopathology, and positive DNA polymerase chain reaction for Mycobacterium bovis confirmed the clinical diagnosis.

  5. Salvianolic acid A alleviates renal injury in systemic lupus erythematosus induced by pristane in BALB/c mice

    Directory of Open Access Journals (Sweden)

    Yihuang Lin

    2017-03-01

    Full Text Available The purpose of this study was to investigate the effects of salvianolic acid A (SAA in systemic lupus erythematosus (SLE induced by pristane in BALB/c mice. Lupus mice were established by confirming elevated levels of autoantibodies and IL-6 after intraperitoneal injection of pristane. Mice were then treated with daily oral doses of SAA for 5 months in parallel with mice treated with prednisone and aspirin as positive controls. The levels of autoantibodies were monitored at monthly intervals and nephritic symptoms observed by hematoxylin and eosin (H&E and periodic acid–Schiff (PAS staining. Western blot analysis of renal tissue was also employed. SAA treatment caused a significant reduction in the levels of anti-Sm autoantibodies and reduced renal histopathological changes and pathological effects. SAA treatment also significantly inhibited the phosphorylation of IKK, IκB and NFκB in renal tissues of lupus mice. In conclusion, the results suggest that SAA alleviates renal injury in pristane-induced SLE in BALB/c mice through inhibition of phosphorylation of IKK, IκB and NFκB.

  6. Salvianolic acid A alleviates renal injury in systemic lupus erythematosus induced by pristane in BALB/c mice.

    Science.gov (United States)

    Lin, Yihuang; Yan, Yu; Zhang, Huifang; Chen, Yucai; He, Yangyang; Wang, Shoubao; Fang, Lianhua; Lv, Yang; Du, Guanhua

    2017-03-01

    The purpose of this study was to investigate the effects of salvianolic acid A (SAA) in systemic lupus erythematosus (SLE) induced by pristane in BALB/c mice. Lupus mice were established by confirming elevated levels of autoantibodies and IL-6 after intraperitoneal injection of pristane. Mice were then treated with daily oral doses of SAA for 5 months in parallel with mice treated with prednisone and aspirin as positive controls. The levels of autoantibodies were monitored at monthly intervals and nephritic symptoms observed by hematoxylin and eosin (H&E) and periodic acid-Schiff (PAS) staining. Western blot analysis of renal tissue was also employed. SAA treatment caused a significant reduction in the levels of anti-Sm autoantibodies and reduced renal histopathological changes and pathological effects. SAA treatment also significantly inhibited the phosphorylation of IKK, IκB and NFκB in renal tissues of lupus mice. In conclusion, the results suggest that SAA alleviates renal injury in pristane-induced SLE in BALB/c mice through inhibition of phosphorylation of IKK, IκB and NFκB.

  7. Systematic review of infliximab-induced autoantibodies and systemic lupus erythematosus.

    Science.gov (United States)

    Vaz, João Luiz Pereira; Andrade, Carlos Augusto Ferreira; Pereira, Alessandra Cardoso; Martins, Maria de Fátima M; Levy, Roger Abramino

    2013-08-01

    The present systematic review aims to discuss infliximab-induced autoantibodies and subsequent onset of systemic lupus erythematosus (SLE) through the analyses of primary reports measuring autoantibodies both before and after the administration of infliximab for the treatment of several diseases - e.g., rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, and Crohn's disease. Our literature search was performed in nine databases - PubMed, Science Direct, Scopus, Web of Knowledge, Scirus, Cochrane, EMBASE, Scielo and LILACS, and the search query retrieved 998 primary reports, from which 24 articles were selected and further narrowed down to 14, based on our inclusion criteria. Two independent reviewers performed the article selection and a third reviewer solved discrepancies. Our inclusion criteria comprised primary reports of phase IV clinical trials with duration of at least three months. In total, 760 patients were evaluated and the most prevalent assays performed in the studies were anti-nuclear antibodies (ANA), anti-double stranded DNA antibodies (anti-dsDNA), and antibodies to saline-extracted antigens (ENA panel). Of all patients evaluated, 10 (1.3%) showed clinical signs and laboratorial evidence of infliximabinduced SLE.

  8. Drug-induced lupus: simvastatin or amiodarone? A case report in elderly

    Directory of Open Access Journals (Sweden)

    Mauro Turrin

    2013-03-01

    Full Text Available Reports of systemic lupus erythematosus (SLE seen during treatment with amiodarone are rare in the literature. SLE or immunological abnormalities induced by treatment with statins are more frequent. In this issue we report a case of a 81-year-old male who, after a 2-year therapy with amiodarone, developed a clinical and serologic picture of drug-induced SLE (DILE. He was admitted for congestive heart failure in mechanical aortic valve prosthesis, permanent atrial fibrillation (anticoagulation with warfarin, hypercholesterolaemia, and hypothyroidism. Amiodarone was started two years earlier for polymorphic ventricular tachycardia, statin and L-thyroxine the following year. At admission he presented pleuro-pericardical effusion detected by CT-scan (also indicative of interstitial lung involvement and echocardiography. Serological main indicative findings were: elevation of inflammatory markers, ANA (Anti-Nuclear Antibodies titers = 1:320 (indirect immune-fluorescence – IIF – assay on HEp-2, homogeneous/fine speckled pattern, anti-dsDNA titers = 1:80 (IIF on Crithidia luciliae, negative ENA (Extractable Nuclear Antigens and antibodies anti-citrulline, rheumatoid factor = 253 KU/l, normal C3-C4, negative HbsAg and anti-HCV, negative anticardiolipin antibodies IgG and IgM, negative anti-beta2GPI IgG and IgM. Amiodarone was discontinued and methylprednisolone was started, since the patient was severely ill. At discharge, after a month, the patient was better and pleuro-pericardical effusion was reduced. Readmitted few weeks later for bradyarithmia and worsening of dyspnoea, pericardial effusion was further reduced but he died for refractory congestive heart failure and pneumonia. Clinical picture (sierositis, neither skin nor kidney involvement, other typical side effects of amiodarone (hypothyroidism and lung interstitial pathology and serological findings are suggestive of amiodarone-induced SLE.

  9. Vancomycin induced Red Man Syndrome

    Directory of Open Access Journals (Sweden)

    Drisyamol K.A

    2016-04-01

    Full Text Available Vancomycin is a glycoprotein antibiotic that has been associated with an anaphylactoid reaction termed the Red-man syndrome. It usually consists of erythema, flushing and pruritis of the face and upper torso and occasionally progresses to include dyspnoea, chest pain and hypotension. Red man syndrome (RMS is also known as “red neck syndrome. Discontinuation of the vancomycin infusion and administration of diphenhydramine can abort most of the reactions. Slow intravenous administration of vancomycin should minimize the risk of infusion-related adverse effects. Antibiotics such as ciprofloxacin, amphotericin B, rifampcin and teicoplanin can potentially cause red man syndrome. The effects of red man syndrome can be relieved by antihistamines.

  10. Antiphospholipid Syndrome: primary or secondary to Systemic Lupus Erythematosus? Description of a clinical case of avitaminosis D in premenopausal woman with pseudo-Cushing syndrome

    Directory of Open Access Journals (Sweden)

    Mauro Turrin

    2014-06-01

    Full Text Available Low vitamin D levels have been described in obese individuals and in some autoimmune diseases, such as Systemic Lupus Erythematosus (SLE and primary antiphospholipid syndrome (pAPS. In particular, more than 50% of premenopausal women with pAPS have hypovitaminosis D. In this issue we report a case of an obese, premenopausal, and hypertensive woman with pseudo-Cushing syndrome, affected by deep venous thrombosis associated with pulmonary embolism after rib fracture who presented hypovitaminosis D. 7 years before, diagnosis of pAPS had been made after the detection of thrombocytopenia (present at a young age and arterial ischemia of a lower limb. For seven years she was treated with acetylsalicylic acid without complications. We found positive anti-dsDNA antibodies, a triple antiphospholipid antibodies (aPL positivity and levels of vitamin D < 4 µg/l. The case report arises some questions: is vitamin D deficiency due to obesity or APS? Is the positivity of anti-dsDNA indicative of progression to SLE? Is preventive therapy with hydroxychloroquine indicated? Does the high-risk aPL profile justify a high-intensity and life-long anticoagulation regimen?http://dx.doi.org/10.7175/cmi.v8i2.912

  11. 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.

  12. 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.

  13. Emerging roles for the interferon-inducible p200-family proteins in sex bias in systemic lupus erythematosus.

    Science.gov (United States)

    Choubey, Divaker; Panchanathan, Ravichandran; Duan, Xin; Liu, Hongqi; Liu, Hongzhu

    2011-12-01

    Systemic lupus erythematosus (SLE) is a complex autoimmune disease involving multiple organs. The disease is characterized by the production of pathogenic autoantibodies to DNA and certain nuclear antigens, chronic inflammation, and immune dysregulation. Genetic studies involving SLE patients and mouse models have indicated that multiple lupus susceptible genes contribute to the disease phenotype. Notably, the development of SLE in patients and in certain mouse models exhibits a strong sex bias. In addition, several lines of evidence indicates that activation of interferon-α (IFN-α) signaling in immune cells and alterations in the expression of certain immunomodulatory cytokines contribute to lupus pathogenesis. Studies have implicated factors, such as the X chromosomal gene dosage effect and the sex hormones, in gender bias in SLE. However, the molecular mechanisms remain unclear. Additionally, it remains unclear whether these factors influence the "IFN-signature," which is associated with SLE. In this regard, a mutually positive regulatory feedback loop between IFNs and estrogen receptor-α (ERα) has been identified in immune cells. Moreover, studies indicate that the expression of certain IFN-inducible p200-family proteins that act as innate immune sensors for cytosolic DNA is differentially regulated by sex hormones. In this review, we discuss how the modulation of the expression of the p200-family proteins in immune cells by sex hormones and IFNs contributes to sex bias in SLE. An improved understanding of the regulation and roles of the p200-family proteins in immune cells is critical to understand lupus pathogenesis as well as response (or the lack of it) to various therapies.

  14. Metabolic Profiling of Systemic Lupus Erythematosus and Comparison with Primary Sjögren's Syndrome and Systemic Sclerosis.

    Science.gov (United States)

    Bengtsson, Anders A; Trygg, Johan; Wuttge, Dirk M; Sturfelt, Gunnar; Theander, Elke; Donten, Magdalena; Moritz, Thomas; Sennbro, Carl-Johan; Torell, Frida; Lood, Christian; Surowiec, Izabella; Rännar, Stefan; Lundstedt, Torbjörn

    2016-01-01

    Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease which can affect most organ systems including skin, joints and the kidney. Clinically, SLE is a heterogeneous disease and shares features of several other rheumatic diseases, in particular primary Sjögrens syndrome (pSS) and systemic sclerosis (SSc), why it is difficult to diagnose The pathogenesis of SLE is not completely understood, partly due to the heterogeneity of the disease. This study demonstrates that metabolomics can be used as a tool for improved diagnosis of SLE compared to other similar autoimmune diseases. We observed differences in metabolic profiles with a classification specificity above 67% in the comparison of SLE with pSS, SSc and a matched group of healthy individuals. Selected metabolites were also significantly different between studied diseases. Biochemical pathway analysis was conducted to gain understanding of underlying pathways involved in the SLE pathogenesis. We found an increased oxidative activity in SLE, supported by increased xanthine oxidase activity and an increased turnover in the urea cycle. The most discriminatory metabolite observed was tryptophan, with decreased levels in SLE patients compared to control groups. Changes of tryptophan levels were related to changes in the activity of the aromatic amino acid decarboxylase (AADC) and/or to activation of the kynurenine pathway.

  15. 25-hydroxyvitamin D is associated with metabolic syndrome among premenopausal women with systemic lupus erythematosus in China.

    Science.gov (United States)

    Wang, L-M; Zheng, Z-H; Li, T-F; Han, L-S; He, Y-J; Zhang, Y-L; Zeng, H-L; Liu, S-Y

    2017-04-01

    Objectives This study aimed to investigate the status of 25-hydroxyvitamin D (25(OH)D) and its association with metabolic syndrome (MS) and different MS components among premenopausal women with systemic lupus erythematosus (SLE) in China. Patients and methods Altogether 113 premenopausal women with SLE and the age-matched healthy cohorts were recruited in this cross-sectional study. Clinical manifestations and laboratory data including serum 25(OH)D concentration were collected. A multivariable analysis was performed to analyze the association of 25(OH)D with MS and its components. Results The prevalence of 25(OH)D deficiency (25(OH)D D, MS and its components demonstrated that the lower level of 25(OH)D was associated with increased MS prevalence (OR = 0.920, p = 0.012), a decreased level of high-density lipoprotein (OR = 1.059, p = 0.033) and a higher level of fasting glucose (OR = 0.810, p = 0.004). These associations were still detectible after adjustment for age, body mass index and SLE-related variables. Conclusion The level of 25(OH)D is associated with MS and its components in premenopausal women with SLE.

  16. Fever-Induced Brugada Syndrome

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    Sandhya Manohar MD

    2015-03-01

    Full Text Available Brugada syndrome is increasingly recognized as a cause of sudden cardiac death. Many of these patients do not get diagnosed due its dynamic and often hidden nature. We have come a long way in understanding the disease process, and its electrophysiology appears to be intimately linked with sodium channel mutations or disorders. The cardiac rhythm in these patients can deteriorate into fatal ventricular arrhythmias. This makes it important for the clinician to be aware of the conditions in which arrhythmogenicity of Brugada syndrome is revealed or even potentiated. We present such an instance where our patient’s Brugada syndrome was unmasked by fever.

  17. Lupus miliaris disseminatus faciei: a distinctive rosacea-like syndrome and not a granulomatous form of rosacea.

    NARCIS (Netherlands)

    Scheur, van de M.R.; Waal, van der RI; Starink, T.M.

    2003-01-01

    BACKGROUND: Lupus miliaris disseminatus faciei is an eruption of discrete red-brown, dome-shaped papules, histologically characterized by epithelioid cell granulomas. The pathogenesis of the disorder remains controversial. OBJECTIVE: The authors discuss the place of lupus miliaris disseminatus facie

  18. Different Types of Lupus

    Science.gov (United States)

    ... Twitter Facebook Pinterest Email Print Different types of lupus Lupus Foundation of America February 24, 2017 Resource ... lupus. Learn more about each type below. Systemic Lupus Erythematosus Systemic lupus is the most common form ...

  19. Olanzapine induced neuroleptic malignant syndrome.

    Science.gov (United States)

    Patra, Bichitra Nanda; Khandelwal, Sudhir K; Sood, Mamta

    2013-01-01

    An 18 year old male diagnosed as a case of bipolar affective disorder (BPAD), developed neuroleptic malignant syndrome (NMS) following treatment with olanzapine (20 mg per day), an atypical antipsychotic drug. NMS is usually seen with typical antipsychotic drugs. The patient was diagnosed as a case of NMS, offending agent was immediately withdrawn and prompt treatment with bromocriptine and levodopa produced a good recovery. The various features of the case are discussed in view of the potential mortality of the syndrome.

  20. Cyclophosphamide-induced reversible posterior leukoencephalopathy syndrome.

    Science.gov (United States)

    Abenza-Abildua, Maria Jose; Fuentes, Blanca; Diaz, Domingo; Royo, Aranzazu; Olea, Teresa; Aguilar-Amat, Maria Jose; Diez-Tejedor, Exuperio

    2009-01-01

    Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical radiological syndrome, characterised by acute headache, altered consciousness, seizures and hypertension. The most frequent causes are hypertensive encephalopathy, eclampsia and some immunosuppressive therapies. The pathogenesis remains unclear, but it appears to be related to altered cerebral circulation, producing oedema that can be seen on MRI, and it resolves in 2 or 3 weeks. In the present report, a possible first reported case of cyclophosphamide-induced RPLS in a 27-year-old man with high blood pressure (HBP) and glomerulonephritis caused by Goodpasture syndrome, treated with cyclophosphamide during the last month and prednisone for glomerulonephritis resulting from Goodpasture syndrome without other immunosuppressive drugs, is described.Symptoms appeared during a hypertensive crisis, but when cyclophosphamide was replaced by rituximab and hypertension was controlled, the patient did not have neurological symptoms. Almost all reported cases induced by immunosuppressive therapy or other causes were associated with hypertension as well.

  1. Thrombolytic Therapy for Cerebral Vein Thrombosis in Antiphospholipid Syndrome Secondary to Systemic Lupus Erythematosus

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    Mehrzad Hajialilo

    2012-03-01

    Full Text Available A 20-year-old woman was admitted to a Gynecology Hospital in her 6th month of pregnancy for high blood pressure and tonic-clonic seizure. Primary diagnosis was eclampsia, and for that reason she underwent cesarean section. She also had headache on frontal and parietal areas without nausea or vomiting. There was not a focal neurological sign. Rheumatology consultation was requested. Sys-temic lupus erythematosus and secondary antiphospholipid (APS was confirmed. The patient had headache that continued several days after cesarean section, therefore, brain magnetic resonance im-aging (MRI and magnetic resonance venography (MRV were performed, and cerebral vein thrombosis was documented. Distal segment of right lateral sinus and sigmoid sinus were not ap-peared in brain MRV. Abnormal hypersignal intensity of right lateral sinus/coronal T2 was detected. Thrombolytic therapy with 20 mg tissue plasminogen activator on right sigmoid and transverse sinus was performed by an interventional neurologist. After this procedure, the patient's headache healed and she was discharged in a good condition.

  2. Olanzapine induced neuroleptic malignant syndrome

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    Bichitra Nanda Patra

    2013-01-01

    Full Text Available An 18 year old male diagnosed as a case of bipolar affective disorder (BPAD, developed neuroleptic malignant syndrome (NMS following treatment with olanzapine (20 mg per day, an atypical antipsychotic drug. NMS is usually seen with typical antipsychotic drugs. The patient was diagnosed as a case of NMS, offending agent was immediately withdrawn and prompt treatment with bromocriptine and levodopa produced a good recovery. The various features of the case are discussed in view of the potential mortality of the syndrome.

  3. A case of catastrophic antiphospholipid antibody syndrome complicated with systemic lupus erythematosus, double positive for anti-cardiolipin/β₂ glycoprotein I and anti-phosphatidylserine/prothrombin autoantibodies.

    Science.gov (United States)

    Hirakawa, Eri; Saito, Kazuyoshi; Hirata, Shintaro; Atsumi, Tatsuya; Koike, Takao; Tanaka, Yoshiya

    2012-09-01

    A 16-year-old male with severe thrombocytopenia and progressive multiple organ infarctions was diagnosed as having catastrophic antiphospholipid syndrome (CAPS) complicated with systemic lupus erythematosus, and was successfully treated with combination of anticoagulants, corticosteroids, plasma exchange, and intravenous cyclophosphamide. Antibodies to phosphatidylserine/prothrombin (PS/PT) complex and cardiolipin (CL)/β(2)-glycoprotein I (β(2)GPI) were simultaneously detected, indicating that the different pathways of both PS/PT and CL/β(2)GPI might be associated with the radical manifestation of CAPS.

  4. Clozapine Induced Neuroleptic Malignant Syndrome

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    Yong Suk Jo

    2017-08-01

    Full Text Available Neuroleptic malignant syndrome is a rare, but potentially life-threatening adverse event associated with the use of neuroleptic agents. We describe the case of a 47-year-old schizophrenic woman who was treated with clozapine for years. The patient developed acute renal failure with pulmonary edema, and underwent mechanical ventilation and hemodialysis.

  5. Fluconazole-Induced Type 1 Kounis Syndrome.

    Science.gov (United States)

    Singh Mahal, Hardeep

    2016-01-01

    The administration of fluconazole is commonly used in both inpatient and outpatient settings for the management of candidiasis infection. Although it is associated with a relatively safe side effect profile, some patients experience adverse effects associated with increased morbidity. We describe 1 such patient, a 42-year-old woman with a history of severe eczema who developed fluconazole-induced type 1 Kounis syndrome. Review of literature indicates that this as the first case reported of fluconazole-induced type 1 Kounis syndrome.

  6. Corticosteroid-induced neuropsychiatric disorders: review and contrast with neuropsychiatric lupus.

    Science.gov (United States)

    Bhangle, Samir D; Kramer, Neil; Rosenstein, Elliot D

    2013-08-01

    The aim of this review is to analyze the available literature regarding the neuropsychiatric (NP) disturbances associated with corticosteroid (CS) therapy; to determine the nature, severity, and frequency of these NP symptoms; and to identify the various risk factors involved in the development of CS-induced NP disturbances. We searched the available literature since the advent of corticosteroid therapy (1950) utilizing the PubMed database ( www.pubmed.gov). Primary articles were identified, and they and their pertinent references were reviewed. Due to potential confusion between NP manifestations of CS therapy and central nervous system (CNS) involvement of systemic lupus erythematosus (SLE), a condition often treated with CS, a brief review of NP manifestations of SLE was also performed. The presentation of CS-induced neuropsychiatric disorders (CIPD) can be quite varied with depression, hypomania, and overt psychosis being the most common manifestations. CIPD can also include bipolar affective changes, delirium, panic attacks, agoraphobia, obsessive-compulsive disorder, anxiety, insomnia, restlessness, fatigue, catatonia, reversible dementia-like cognitive changes, impaired memory, and concentration. No factors have been identified that allow for the accurate prediction of development of CIPD. A dose-dependent relationship (increased risk when the daily prednisone-equivalent dose is ≥40 mg) has been observed in most cases of CIPD, although there have been case reports with lower doses, alternate-day therapy, and even inhaled CS. Women are more commonly affected with most symptoms occurring in the first 6 weeks of starting treatment. SLE has been the only specific illness that has been linked to a greater risk of CIPD and the NP manifestations of SLE may mimic those of CIPD, with most occurring in the first year of diagnosis. Antiribosomal P, antineuronal, or antiphospholipid antibodies are frequently seen in patients with SLE developing CIPD. Imaging and EEG

  7. Genetic variants of chemokine receptor CCR7 in patients with systemic lupus erythematosus, Sjogren's syndrome and systemic sclerosis

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    Stanke Frauke

    2007-06-01

    Full Text Available Abstract Background The chemokine receptor CCR7 is a key organizer of the immune system. Gene targeting in mice revealed that Ccr7-deficient animals are severely impaired in the induction of central and peripheral tolerance. Due to these defects, Ccr7-deficient mice spontaneously develop multi-organ autoimmunity showing symptoms similar to those observed in humans suffering from connective tissue autoimmune diseases. However, it is unknown whether mutations of CCR7 are linked to autoimmunity in humans. Results DNA samples were collected from 160 patients suffering from connective tissue autoimmune disease (Sjogren's syndrome, n = 40; systemic lupus erythematosus, SLE, n = 20 and systemic sclerosis, n = 100 and 40 health subjects (n = 40. All participants in this study were of German descent. Samples were screened for single nucleotide polymorphisms (SNP by sequencing the coding region of the CCR7 gene as well asthe exon flaking intron sites and parts of the regions encoding for the 5'- and 3'-UTR. CCR7 variants were rare. We identified six different sequence variants, which occurred in heterozygosis. The identified SNP were observed at position -60 C/T (observed 1x, +6,476 A/G (7x, +6,555 C/T (15x, +6,560 C/T (6x, +10,440 A/G (3x and +11,475 C/A (1x. Four of these variants (+6,476 A/G, +6,555 C/T, +6,560 C/T and +10,440 A/G display allelic frequencies between 1% and 5 % and were present in both patients and control groups. The variants +6,476 A/G, +6,555 C/T, +6,560 C/T are located in the intron 2, while the +10,440 A/G variant corresponds to a silent mutation in exon 3. The variants -60 C/T and +11,475 C/A which are located at the 5'-UTR and 3-UTR respectively, display allelic frequencies below 1%. No correlation between these variants and the autoimmune diseases investigated could be observed. However, reporter gene expression assay demonstrated that the mutation at the -60 C/T position in homozygosis leads to reduced luciferase activity

  8. Pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome. Practical messages from the EULAR guidelines.

    Science.gov (United States)

    Vagelli, Roberta; Tani, Chiara; Mosca, Marta

    2017-01-25

    Over the last few decades, reproductive medicine has observed an improvement in the management and outcome of pregnancy in connective tissue diseases, such as systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). However, pregnancy and related issues remain a challenge in these patients. In routine clinical practice, health professionals dealing with SLE and APS need to consider the numerous aspects of the reproductive life of their patients, such as pregnancy, family planning, fertility, contraception, cancer surveillance, and menopause. The new European League Against Rheumatism recommendations for women's health and family planning reflect the need for a novel approach to communication in the patient-physician relationship. Preconception counseling is essential to ensure optimal pregnancy outcomes through a careful risk stratification involving disease activity, organ involvement, autoantibody profile, use of drugs, and previous pregnancy outcomes, as well as to ensure better preventive and therapeutic strategies to limit complications. In patients with stable/inactive disease and low risk of thrombosis, adequate hormonal contraception and menopausal replacement therapy should be recommended. Assisted reproductive techniques can be safely used in these patients, but anticoagulation or low-dose aspirin (or both) should be added in those with positive antiphospholipid antibody titers. All menstruating women should be counseled on the possibility to preserve fertility with gonadotropin- ‑releasing hormone analogues if receiving alkylating agents. Strict clinical, serological, laboratory, and multidisciplinary monitoring during pregnancy is mandatory to early recognize and effectively treat disease flares or obstetric complications. Doppler ultrasonography and fetal biometry should be regularly performed, especially in the second and third trimesters. Physicians should recommend screening for cervical dysplasia related to human papillomavirus

  9. Cerebrospinal fluid and serum antiphospholipid antibodies in multiple sclerosis, Guillain-Barré syndrome and systemic lupus arythematosus

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    Paulo E. Marchiorji

    1990-12-01

    Full Text Available Immuneglobulins isotypes (IgG and IgM for myelin basic protein (MBP, cerebrosides (CER, gangliosides (GANG and cardiolipin (CARD were detected in the cerebrospinal fluid (CSF from 33 patients with multiple sclerosis (MS, 18 with Guillain-Barré syndrome (GBS and 30 with systemic lupus erythematosus (SLE. In MS patients occurred positive and significant levels of IgG-MBP in 51,5% (p<0.05 and IgM-MBP in only 18.2%, IgG-CARD in 46.2%, as long as CER and GANG were detected in almost 20%. From serum samples of MS patients 20.6% presented IgG-MBP, while 53% showed positive levels foi IgM-MBP. The CSF analysis of patients with GBS showed that 56.3% revealed IgG-MBP (p<0.05, 53% for IgM-MBP. 3&.5% for IgG-CER and 23% for IgM-CER, while 50% of patients had IgG-CARD, as long -as 31% also had IgG-GANG. The serum evaluation from 14 patients showed that 18.8% had positive concentrations of IgG-MBP and 56.3% presented IgM-MBP (p<0.05 Except for 50% of patients with SLE who presented positive CSF levels of IgG-CARD. only 24.1% had positive levels of IgG-MBP. We believe that the presence of antiphosphohoid antibodies in CSF of the above mentioned diseases occurred as immune epiphenomena, but their appearance would permit the maintenance of and perpetuate the immune event.

  10. LUPUS ERITEMATOSO SISTÉMICO SÍNDROME DE CUSHING Y PSIQUIATRÍA DE ENLACE Erythematous systemic lupus, Cushing`s syndrome and psychiatric

    OpenAIRE

    Ludwig Pájaro Silva

    2005-01-01

    Se presenta el caso de una paciente con diagnóstico de lupus eritematoso sistémico y síndrome de Cushing quien presentó síntomas afectivos de características depresivas e insomnio de conciliación, quien recibió múltiples tratamientos farmacológicos con pobre respuesta inicial. La psiquiatría de enlace consolida una alternativa de tratamiento multidisciplinario para una aproximación más eficiente a las patologías médicas con síntomas psiquiátricos.We present a case of systemic erythematosus lu...

  11. Drug-induced pseudolymphoma syndrome

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    Mittal R

    1995-01-01

    Full Text Available Five cases of pseudolymphoma syndrome (PS in children aged six to twelve years were observed after anticonvulsant drugs. In two cases PS was observed after ten days and in three after fifteen days of therapy with the offending drug. Three cases of PS were due to carbamazepine and had morbilliform rash and two cases due to phenobarbitone had erythroderma. All had fever, generalised lymphadenopathy and 4/5 had hepatosplenomegaly. Therapy with 15 mg prednisolone daily and withdrawal of the offending durg led to cure in 4/5 cases and one died due to congestive cardiac failure.

  12. CELEBREX IN TREATMENT OF VERTEBRAL PAIN SYNDROME IN SYSTEMICV LUPUS ERYTHEMATOSUS

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    N. P. Shilkina

    2002-01-01

    Full Text Available Objective. То study of tizol efficacy for local therapy of rheumatoid arthritis (RA. Materials and methods. 90 pts with active RA (2-3 stage of activity age 41-54 (m=18, f—72. The method оГ double blind controlled comparison of 10 - days therapy with pure tizol, tizol with diclophenac (1% and placebo (vaseline was used. In order to evaluate skin permeability of tizol mass-spectrometry of synovial liquid was used. Results. Positive dynamic of joint syndrome under tizol and tizol with diclophenac therapy were showed. There were reduction of arthalgia, decreasing of morning stiffness, increasing of grip strenght. Erythrocyte sedimentation rate didn’t change. Adding of diclophenac to tizol amplified it's analgetic properties. Mass spectrum analysis showed that concentration of titanium in synovial fluid came approximately 10 times higher after 10 days of tizol therapy. Conclusion. The efficacy of local tizol and it’s compounds in inflammatory joint syndrome was showed. Diclophenac intensified analgetic effect of tizol. Efficacy and convenience of treatment, absens of side-effects, low price allow to recommend tizol - gel compounds for local treatment of inflammatory joint diseases.

  13. A Systematic Review of Peripheral and Central Nervous System Involvement of Rheumatoid Arthritis, Systemic Lupus Erythematosus, Primary Sjögren’s Syndrome, and Associated Immunological Profiles

    Directory of Open Access Journals (Sweden)

    Anastasia Bougea

    2015-01-01

    Full Text Available Both central (CNS and peripheral nervous system (PNS complications are frequent and varied in connective tissue diseases. A systematic review was conducted between 1989 and 2014 in the databases Medline, Scopus, and Cochrane Library using the search terms, peripheral and central nervous complications and immunological profiles, to identify studies in specific connective tissue disorders such as rheumatoid arthritis, systemic lupus erythematosus, and primary Sjögren’s syndrome. A total of 675 references were identified, of which 118 were selected for detailed analysis and 22 were included in the final review with a total of 2338 participants. Our search focused only on studies upon connective tissue disorders such as rheumatoid arthritis, systemic lupus erythematosus, and primary Sjögren’s syndrome associated with seroimmunological data. The reported prevalence of CNS involvement ranges from 9 to 92% across the reported studies. However, the association between CNS and PNS manifestations and seroimmunological profiles remains controversial. Τo date, no laboratory test has been shown as pathognomonic neither for CNS nor for PNS involvement.

  14. Lupus mimickers.

    Science.gov (United States)

    Calixto, Omar-Javier; Franco, Juan-Sebastian; Anaya, Juan-Manuel

    2014-08-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by multisystem organ involvement, heterogeneity of clinical features, and variety in degree of severity. The differential diagnosis is a crucial aspect in SLE as many other autoimmune diseases portray clinical similarities and autoantibody positivity. Lupus mimickers refer to a group of conditions that exhibit both clinical features and laboratory characteristics, including autoantibody profiles that resemble those present in patients with SLE, and prompt a diagnostic challenge in everyday clinical practice. Thus, lupus mimickers may present as a lupus-like condition (i.e., 2 or 3 criteria) or as one meeting the classification criteria for SLE. Herein we review and classify the current literature on lupus mimickers based on diverse etiologies which include infections, malign and benign neoplasms, medications, and vaccine-related reactions.

  15. Drug-induced tardive syndromes.

    Science.gov (United States)

    Ortí-Pareja, M; Jiménez-Jiménez, F J; Vázquez, A; Catalán, M J; Zurdo, M; Burguera, J A; Martínez-Martín, P; Molina, J A

    1999-04-01

    We reviewed the database of five Movement Disorders Units to establish drugs responsible for tardive syndromes or TS (tardive dyskinesia, dystonia, akathisia, tremor, tics or tourettism, and myoclonus). The diagnostic criteria for TS included: (1) appearance of persistent dyskinesia, dystonia, akathisia, tremor, tics or tourettism, or myoclonus, related to prolonged drug exposure, (2) exclusion of other possible causes of these movement disorders. One-hundred patients fulfilled the diagnostic criteria for TS (26 males, 74 females, mean age 69.4+/-15.8 years). TS were related to 1, 2, 3, 4 and 5 drugs in 58, 27, 9, 5 and 1 patients, respectively. The most frequently offending drugs were antipsychotic drugs, substituted benzamides, thietylperazine and calcium-channel blockers. Seventy-two patients had buccolinguomasticatory syndrome, 30 had tremor, 22 akathisia and 16 dystonia (35 patients had a combination of at least two of these TS). Forty-two patients had coexistent parkinsonism. The TS disappeared following withdrawal of the offending drug in 40 cases. Old age and being female were more frequently associated with TS, with the exception of tardive dystonia. Substituted benzamides, calcium-channel blockers and thiethylperazine (a neuroleptic used for vertigo) were a frequent cause of TS in our series.

  16. The presentation and evaluation of a case of systemic Lupus erythematosus and anthiphospholipid antibody syndrome with primary clinical manifestation of chorea

    Directory of Open Access Journals (Sweden)

    Asgary S

    1998-06-01

    Full Text Available Manifestation of chorea in patients with systemic lupus erythematosus (SLE and antiphospholipid antibody syndrome (APA synd. is not common. Moreover, primary presentation of the disease with chorea is rare and only few such cases are reported in literature in recent years. We report here the case of a 28 year old woman who was first seen at the age of 10 with clinical manifestations of chorea. Later she developed deep vein thrombosis, thrombocytpenia, stroke, cardiac valve involvement and recurrent abortions. Laboratory investigations confirmed the diagnosis of SLE and the presence of antiphospholipid antibodies. We present this patient as a case of SLE and antiphospholipid antibody syndrome with chorea being her primary clinical presentation

  17. Rare Occurrence of Drug Induced Subacute Cutaneous Lupus Erythematosus with Leflunomide Therapy

    OpenAIRE

    Singh, Harpreet; Sukhija, Gagandeep; Tanwar, Vikram; Arora, Sameer; Bhutani, Jaikrit

    2016-01-01

    Leflunomide is an immunomodulatory drug exhibiting anti-inflammatory, anti-proliferative and immunosuppressive effects. It has been widely used for treatment of active rheumatoid arthritis. Despite its good safety profile cutaneous side effects like alopecia, eczema, pruritis and dry skin have been reported with Leflunomide use. Skin ucleration, vasculitis, lichenoid drug rash and Subacute Cutaneous Lupus Erythematosus (SCLE) have been rarely reported with its use. A rare case of Leflunomide ...

  18. Interferon-inducible Ifi200-family genes in systemic lupus erythematosus

    Science.gov (United States)

    Choubey, Divaker; Panchanathan, Ravichandran

    2008-01-01

    Systemic lupus erythematosus (SLE) is the prototype of complex autoimmune diseases. Studies have suggested that genetic, hormonal, and environmental factors contribute to the development of the disease. Interestingly, several recent studies involving SLE patients and mouse models of the disease have suggested a role for interferon (IFN)-stimulated genes (ISGs) in the development of SLE. One family of ISGs is the Ifi200-family, which includes mouse (Ifi202a, Ifi202b, Ifi203, Ifi204, and Ifi205) and human (IFI16, MNDA, AIM2, and IFIX) genes. The mouse genes cluster between serum amyloid P-component (Apcs) and α-spectrin (Spna-1) genes on chromosome 1 and the human genes cluster in syntenic region 1q23. The Ifi200-family genes encode structurally and functionally-related proteins (the p200-family proteins). Increased expression of certain p200-family proteins in cells is associated with inhibition of cell proliferation, modulation of apoptosis, and cell differentiation. Our studies involving generation of B6.Nba2 congenic mice, coupled with gene expression analyses, identified the Ifi202 as a candidate lupus-susceptibility gene. Importantly, recent studies using different mouse models of SLE have suggested that increased expression of Ifi202 gene (encoding p202 protein) in immune cells contributes to lupus susceptibility. Consistent with a functional role for the p202 protein in lupus susceptibility, increased levels of IFI16 protein in human SLE patients are associated with the diseases. This review summarizes recent findings concerning the regulation and role of p200-family proteins in the development of SLE. PMID:18598717

  19. Murine lupus susceptibility locus Sle1a requires the expression of two sub-loci to induce inflammatory T cells.

    Science.gov (United States)

    Cuda, C M; Zeumer, L; Sobel, E S; Croker, B P; Morel, L

    2010-10-01

    The NZM2410-derived Sle1a lupus susceptibility locus induces activated autoreactive CD4(+) T cells and reduces the number and function of Foxp3(+) regulatory T cells (Tregs). In this study, we first showed that Sle1a contributes to autoimmunity by increasing antinuclear antibody production when expressed on either NZB or NZW heterozygous genomes, and by enhancing the chronic graft versus host disease response indicating an expansion of the autoreactive B-cell pool. Screening two non-overlapping recombinants, the Sle1a.1 and Sle1a.2 intervals that cover the entire Sle1a locus, revealed that both Sle1a.1 and Sle1a.2 were necessary for the full Sle1a phenotype. Sle1a.1, and to a lesser extent Sle1a.2, significantly affected CD4(+) T-cell activation as well as Treg differentiation and function. Sle1a.2 also increased the production of autoreactive B cells. As the Sle1a.1 and Sle1a.2 intervals contain only 1 and 15 known genes, respectively, this study considerably reduces the number of candidate genes responsible for the production of autoreactive T cells. These results also show that the Sle1 locus is an excellent model for the genetic architecture of lupus, in which a major obligate phenotype results from the coexpression of multiple genetic variants with individual weak effects.

  20. Persistence of autoreactive T cell drive is required to elicit anti-chromatin antibodies in a murine model of drug-induced lupus.

    Science.gov (United States)

    Kretz-Rommel, A; Rubin, R L

    1999-01-15

    Long-term treatment with procainamide and numerous other medications is occasionally associated with the development of drug-induced lupus. We recently established a murine model for this syndrome by disrupting central T cell tolerance. Two intrathymic injections of procainamide-hydroxylamine (PAHA), a reactive metabolite of procainamide, into (C57BL/6 x DBA/2)F1 mice resulted in the appearance of chromatin-reactive T cells and anti-chromatin autoantibodies. The current study explores in this model the role of autoreactive T cells in autoantibody production and examines why autoantibodies after a single intrathymic drug injection were much more limited in isotype and specificity. Injection of as few as 5000 chromatin-reactive T cells into naive, syngeneic mice induced a rapid IgM anti-denatured DNA response, while injection of at least 100-fold greater number of activated T cells was required for induction of IgG anti-chromatin Abs, suggesting that small numbers of autoreactive T cells can be homeostatically controlled. Mice subjected to a single intrathymic PAHA injection after receiving splenic B cells from an intrathymic PAHA-injected syngeneic donor also developed anti-chromatin Abs, but adoptive transfer of similarly primed T cells or of B cells without intrathymic PAHA injection of the recipient failed to produce an anti-chromatin response. However, anti-chromatin Abs developed after a single intrathymic PAHA injection in Fas-deficient C57BL/6-lpr/lpr mice, suggesting that activation-induced cell death limited autoimmunity in normal mice. Taken together, these results imply that chromatin-reactive T cells produced by intrathymic PAHA created a B cell population primed to somatically mutate and Ig class switch when subjected to a heavy load or second wave of autoreactive T cells.

  1. Neuroleptic malignant syndrome induced by lamotrigine.

    Science.gov (United States)

    Ishioka, Masamichi; Yasui-Furukori, Norio; Hashimoto, Kojiro; Sugawara, Norio

    2013-01-01

    This case report describes a 54-year-old man with bipolar I disorder who was treated with aripiprazole (ARP) and lithium. The patient was admitted to our hospital because of aggravation of depressive symptoms, and treatment with lamotrigine (LTG) was initiated. Two weeks after admission, we discontinued administration of ARP after the appearance of a tremor. Three weeks after discontinuing ARP, the patient developed a high fever, rigidity of the arms, diarrhea, dysphagia, and diaphoresis. We suspected these symptoms were consistent with neuroleptic malignant syndrome and therefore removed the application of LTG. After 2 days, most of the patient's symptoms and blood results had improved, leading us to conclude that the LTG treatment had induced neuroleptic malignant syndrome. Thus, the purpose of this case report was to warn psychiatrists against therapy with LTG, as it may be conducive to neuroleptic malignant syndrome.

  2. Can Lupus Cause Depression?

    Science.gov (United States)

    ... lupus Living well with lupus Can lupus cause depression? Life with lupus can be challenging. With symptoms ... treatable illness called clinical depression. Symptoms of Clinical Depression People are considered clinically depressed when they have ...

  3. Reversible Posterior Leukoencephalopathy Syndrome Induced by Pazopanib

    Directory of Open Access Journals (Sweden)

    Chelis Leonidas

    2012-10-01

    Full Text Available Abstract Background The reversible posterior leukoencephalopathy syndrome is a clinical/radiological syndrome characterized by headache, seizures, impaired vision, acute hypertension, and typical magnetic resonance imaging findings. There are several reports in the literature that depict its occurrence in cancer patients. The list of common anticancer and supportive care drugs that predispose to reversible posterior leukoencephalopathy syndrome is expanding and includes not only a large number of chemotherapeutic agents but also an increased number of new targeted drugs, particularly angiogenesis inhibitors such as bevacizumab,sorefenib and sunitinib. Pazopanib is an oral tyrosine kinase inhibitor targeting vascular endothelial growth factor receptor, platelet-derived growth factor receptor, and c-Kit which after a positive phase III randomized clinical trial in patients with advanced renal cell cancer received FDA approval for the treatment of advanced renal cell carcinoma. Until now no cases of reversible posterior leukoencephalopathy syndrome induced by pazopanib have been reported. Case report We present the case of a 40 years old female patient with heavily pre-treated metastatic renal cell carcinoma who received pazopanib as salvage treatment. After 21 days of pazopanib therapy the patient referred to the emergency department with epileptic seizure, impaired vision at both eyes and headache. MRI of the brain revealed subcortical oedema at the occipital and parietal lobes bilaterally. She was treated with anticonvulsants, i.v. administration of mannitol and antihypertensives and she recovered completely from her symptoms and was discharged on the tenth hospital day. A brain MRI performed 3 weeks after showed that the subcortical oedema had been subsided. Conclusion In conclusion this is the first case of pazopanib induced reversible posterior leukoencephalopathy syndrome. Although usually reversible, this syndrome is a serious and

  4. Carpal Tunnel Syndrome

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  5. Interferon-induced Raynaud's syndrome.

    Science.gov (United States)

    Schapira, Daniel; Nahir, Abraham Menahem; Hadad, Nuhad

    2002-12-01

    To review the clinical features, diagnosis, treatment, and outcome of interferon-induced Raynaud's phenomenon. The medical literature was reviewed from 1967 to November 2001 with the assistance of a MEDLINE search using the key words: Raynaud, Interferon, ischemia, thrombosis and necrosis. A qualitative review was performed after the articles were abstracted and the relevant information was summarized. Twenty-four cases of interferon-induced Raynaud's phenomenon (including our patient) are described. Interpheron-alpha was the most common causative agent (14 cases). The symptoms appeared weeks to years after beginning treatment and varied from mild vasospasm to occlusion of digital arteries and tissue necrosis (14 cases), sometimes necessitating finger amputation (6 patients). Digital plethysmography, arteriography and capillaroscopy were valuable diagnostic tools. In 4 cases, cardiac, ophthalmic, or central nervous system drug-induced ischemia accompanied the peripheral Raynaud's phenomenon. Of the 15 cases with a documented outcome, withdrawal of the drug alone resulted in complete (6 patients) or partial (1 patient) recovery. In the others, supportive therapy was needed. The recovery period lasted from 2 weeks to 3 months. In 2 patients, continuation of treatment was possible. Raynaud's phenomenon and related complications must be recognized as possible side effects of interferon therapy. Early diagnosis and withdrawal of the drug may prevent unnecessary morbidity and disability. Copyright 2002, Elsevier Science (USA). All rights reserved.

  6. Anti-dsDNA negative and anti-Ro positive lupus nephritis: a report of a rare case.

    Science.gov (United States)

    Jain, D; Aggarwal, H K; Kaverappa, V; Dhayia, S; Jain, P; Yadav, S

    2014-03-17

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, characterized by an autoantibody response to various nuclear and cytoplasmic antigens. Renal disease in SLE occurs in 40-75% of patients, most often within five years of onset of disease, and is one of the strongest predictors of a poor outcome. A hallmark of glomerular involvement in lupus nephritis is the presence of autoantibodies against double-stranded DNA (dsDNA). Its level usually correlates with disease activity. Our patient presented with a rash resembling malar rash and features of nephrotic syndrome. On investigating, patient was found to have pancytopenia, raised erythrocyte sedimentation rate and depressed serum C3 levels with positivity of antinuclear antibodies and anti- Ro antibodies. However, most of the markers of lupus nephritis including anti dsDNA antibody were negative. Renal biopsy showed features of lupus nephritis (class-IV). Differential item functioning studies showed a full house immunoflourescence staining pattern characteristic of lupus nephritis. Association of Anti-Ro antibody alone with lupus nephritis is less known in literature. Negativity of anti-dsDNA antibody, which is usually considered to be diagnostic of lupus nephritis, poses a diagnostic dilemma short of renal biopsy. Till date only very few cases of non-drug induced lupus nephritis with negative dsDNA antibodies have been reported. In this report we wish to highlight a case of lupus nephritis which was negative for its specific anti dsDNA antibodies and with possible role of anti-Ro antibodies in the pathogenesis of lupus nephritis although the underlying mechanism is incompletely understood.

  7. Lupus nephritis

    African Journals Online (AJOL)

    1991-03-02

    Mar 2, 1991 ... guide to treatment. In view of this ... histological features of lupus nephritis in our centre were ... erythematosus (SLE/ - 47 were women (39 coloured,S white .... biopsy only patients with clinical evidence of renal disease and.

  8. 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.

  9. A TRANSIENT DRUG INDUCED LUPUS ERYTHEMATOSUS- LIKE ALLERGIC DRUG REACTION WITH MULTIPLE ANTIBODIES

    Directory of Open Access Journals (Sweden)

    Ana Maria Abreu Velez

    2013-10-01

    Full Text Available Drug reactions may mimic several dermatoses, including lupus erythematosus. We present an 80 year old female patient on multiple medications, who presented with blisters on her hands and arms for two weeks, which then generalized to the rest of her body. The patient was evaluated by a dermatologist, and biopsies for hematoxylin and eosin (H&E examination, as well as for direct immunofluorescence (DIF and immunohistochemistry (IHC were performed. The H&E biopsy examination revealed a mild, superficial, perivascular dermal infiltrate of lymphocytes, histiocytes and abundant eosinophils; neutrophils were rare. No vasculitis was noted. DIF revealed positive basement membrane (BMZ staining, primarily with patchy Complement/C3c and fibrinogen; in addition, strong reactivity to dermal blood vessel was appreciated. Antibodies to cell junction-like structures were also noted in the epidermis and dermis with these two antibodies. IHC using similar immunoglobulins and complement components showed similar patterns. We observed that contrary to lupus erythematosus, neither IgG nor IgM were positive at the BMZ.

  10. 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 ...

  11. Anti-annexin II antibody is associated with thrombosis and/or pregnancy morbidity in antiphospholipid syndrome and systemic lupus erythematosus with thrombosis.

    Science.gov (United States)

    Ao, Wen; Zheng, Hui; Chen, Xiao-Wei; Shen, Yan; Yang, Cheng-De

    2011-07-01

    This study investigated the prevalence of antibody against annexin II (A2) in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). It also explored the role of this antibody in thrombosis. Purified recombinant A2 was used in ELISA to measure the levels of IgG anti-A2 antibody in 101 APS patients, 41 SLE patients with thrombosis, 124 SLE patients without thrombosis, and 120 healthy controls. The positive rates of IgG anti-A2 antibody in APS patients and SLE patients with thrombosis were 21.8 and 26.8%, respectively. Both values are significantly higher than that in SLE patients without thrombosis (6.5%). IgG anti-A2 antibody is associated with thrombosis and/or pregnancy morbidity (P pregnancy morbidity, suggesting that anti-A2 antibody may be helpful in identifying potential APS cases.

  12. [Ruptured aneurysm at the anterior wall of the internal carotid artery in a patient with systemic lupus erythematosus and secondary antiphospholipid syndrome].

    Science.gov (United States)

    Chonan, Masashi; Fujimura, Miki; Inoue, Takashi; Tominaga, Teiji

    2011-07-01

    A 60 year-old woman, who had a 45-year history of systemic lupus erythematosus (SLE) and secondary antiphospholipid syndrome, presented with subarachnoid hemorrhage due to a ruptured aneurysm at the anterior wall of the non-branching site of the right internal carotid artery. She underwent radical surgery on the day of onset. In light of the possibility of arterial dissection, we performed extracranial-intracranial bypass prior to careful exploration of the aneurysm. Based on the finding of saccular aneurysm, she ultimately underwent neck clipping of the aneurysm without complication. Postoperative course was uneventful, and she did not suffer from cerebral vasospasm. We recommend early surgical intervention in patients with aneurysmal SAH associated with SLE, while intrinsic pathologies of SLE such as fragile vascular structure and the risk for ischemic complication should be considered.

  13. The use of angiogenic and antiangiogenic factors in the differential diagnosis of pre-eclampsia, antiphospholipid syndrome nephropathy and lupus nephritis.

    Science.gov (United States)

    de Jesus, G R; de Jesus, N R; Levy, R A; Klumb, E M

    2014-10-01

    Pre-eclampsia (PE) is a major cause of maternal mortality and morbidity, perinatal deaths, preterm birth and intrauterine growth restriction. Differential diagnosis with antiphospholipid syndrome (APS) nephropathy and systemic lupus erythematosus (SLE) nephritis during pregnancy is difficult, if not sometimes impossible, as all three diseases may present hypertension and proteinuria. Improvement in diagnosis of PE has also offered new paths for differential diagnosis with other conditions and the analysis of angiogenic (vascular endothelial growth factor, placental growth factor) and antiangiogenic factors (serum soluble fms-like tyrosine kinase 1, soluble endoglin) is promising for differentiation between PE, APS nephropathy and SLE nephritis. This article reviews published studies about those factors in non-pregnant and pregnant patients with APS and SLE, comparing with patterns described in PE.

  14. Neuroleptic malignant syndrome induced by atypical antipsychotics.

    Science.gov (United States)

    Farver, Debra K

    2003-01-01

    A review of the English literature confirms that neuroleptic malignant syndrome (NMS) occurs with both traditional and atypical antipsychotic medications. Published reports of NMS induced by the traditional antipsychotics have given the practitioner valuable information on the prevention and treatment of this adverse effect. Case reports have also been published concerning NMS and clozapine, risperidone, olanzapine and quetiapine. By evaluating the case reports of atypical antipsychotic-induced NMS, valuable information may be obtained concerning similarities or differences from that induced by the traditional antipsychotics. The case reports of NMS with atypical antipsychotics were evaluated for diagnosis, age/sex of patient, risk factors, antipsychotic doses and duration of use, symptoms of NMS, and clinical course.

  15. Drugs Induced Stevens-Johnson Syndrome

    Directory of Open Access Journals (Sweden)

    Elif ÖNDER

    2010-05-01

    Full Text Available Stevens Johnson Syndrome (SJS is a life threatening mucocutaneous skin disease that mostlydeveloped after using some drug. SJS mostly appear between 2-4th decades. Mucocutaneouslesions were seen between 1-14 days of drug intake. And these lesions spread diffusely all aroundthe body. First treatment choice is the stopping of drug that cause SJS and giving supportingtreatment. After understanding of underlying cytotoxic and immunological mechanism of SJS,new treatment approaches were developed and mortality of disease was reduced. We hereinreport a short review of drug induced SJS and its treatment.

  16. Brain histopathology in patients with systemic lupus erythematosus: identification of lesions associated with clinical neuropsychiatric lupus syndromes and the role of complement.

    Science.gov (United States)

    Cohen, Daniëlle; Rijnink, Emilie C; Nabuurs, Rob J A; Steup-Beekman, Gerda M; Versluis, Maarten J; Emmer, Bart J; Zandbergen, Malu; van Buchem, Mark A; Allaart, Cornelia F; Wolterbeek, Ron; Bruijn, Jan A; van Duinen, Sjoerd G; Huizinga, Tom W J; Bajema, Ingeborg M

    2017-01-01

    Neuropsychiatric (NP) involvement is a poorly understood manifestation of SLE. We studied post-mortem histopathology in relation to clinical NPSLE syndromes and complement deposition in brains of NPSLE and SLE patients and controls. Furthermore, we investigated the correlation between cerebral post-mortem histopathology and ex vivo 7 T MRI findings in SLE and NPSLE. A nationwide search for autopsy material yielded brain tissue from 16 NPSLE and 18 SLE patients. Brains obtained from 24 patients who died of acute cardiac events served as controls. Apart from a histopathological evaluation, paraffin-embedded cortical tissue was stained for components of the classical, lectin and terminal complement pathways. Diffuse vasculopathy, microinfarction, macroinfarction, vasculitis and microthrombi occurred significantly more often in NPSLE than SLE patients and were absent in controls. Focal vasculopathy was found in both SLE patients and controls. Complement deposition was strongly associated with both SLE and NPSLE, but not with controls (P < 0.001). Microthrombi were found uniquely in NPSLE and were associated with C4d and C5b-9 deposits (P < 0.05). A 7 T MRI was unable to detect most small vessel injury that was visible histopathologically. Our study demonstrates that histopathological lesions in NPSLE represent a continuum, ranging from non-specific lesions such as focal vasculopathy, to more specific lesions including C4d- and C5b-9-associated microthrombi and diffuse vasculopathy related to clinical syndromes defining NPSLE. Complement deposition may be a key factor in the interaction between circulating autoantibodies and thromboischaemic lesions observed in NPSLE. Therefore, complement inhibition may have novel therapeutic potential in NPSLE. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  17. Acute Unilateral Blindness from Superior Ophthalmic Vein Thrombosis: A Rare Presentation of Nephrotic Syndrome from Class IV Lupus Nephritis in the Absence of Antiphospholipid or Anticardiolipin Syndrome

    Science.gov (United States)

    Baidoun, Firas; Issa, Rommy; Al-Turk, Bashar

    2015-01-01

    Patients with systemic lupus erythematosus (SLE) are at high risk of arterial and venous thrombosis secondary to anti-phospholipid antibodies. Herein, we are presenting an interesting case of venous thrombosis in a patient with SLE in the absence of anti-phospholipid antibodies. PMID:26858847

  18. Anti-aquaporin-4 autoantibodies in systemic lupus erythematosus persist for years and induce astrocytic cytotoxicity but not CNS disease.

    Science.gov (United States)

    Alexopoulos, Harry; Kampylafka, Eleni I; Fouka, Penelope; Tatouli, Ioanna; Akrivou, Sofia; Politis, Panagiotis K; Moutsopoulos, Haralampos M; Tzioufas, Athanasios G; Dalakas, Marinos C

    2015-12-15

    Anti-aquaporin-4 autoantibodies are specific for the neuromyelitis optica spectrum disorders (NMOSD) and they have also been described in patients with systemic lupus erythematosus (SLE) with neurological signs consistent with NMOSD. Our objective was to test for the presence and pathogenicity of anti-AQP4 antibodies in SLE patients without neurological disease. Sera from 89 non-CNS-SLE patients were screened for anti-AQP4 autoantibodies. Two of the 89 patients were positive. Archived samples dating back 11 years were also positive. A brain and spinal cord MRI did not reveal any NMOSD-compatible lesions. An in vitro cytotoxicity assay showed that either sera or purified IgG from these patients induced a complement-mediated damage in cultured astrocytes comparable to antibodies obtained from typical NMO patients. We conclude that AQP4-antibodies can be present in SLE patients and persist for many years, without concurrent clinical or radiological NMOSD signs. It is unclear why the anti-AQP4 antibodies did not induce CNS disease.

  19. Aim2-deficiency stimulates the expression of interferon-inducible Ifi202, a lupus susceptibility murine gene within the Nba2 autoimmune susceptibility locus

    Science.gov (United States)

    Panchanathan, Ravichandran; Duan, Xin; Shen, Hui; Rathinam, Vijay A. K.; Erickson, Loren D.; Fitzgerald, Katherine A; Choubey, Divaker

    2010-01-01

    Murine Aim2 and p202 proteins (encoded by the Aim2 and Ifi202 genes) are members of the interferon (IFN)-inducible p200-protein family. Both proteins can sense double-stranded DNA (dsDNA) in the cytoplasm. However, upon sensing dsDNA, only the Aim2 protein through its pyrin domain (PYD) can form an inflammasome to activate caspase-1 and induce cell death. Given that the p202 protein has been predicted to inhibit the activation of caspase-1 by the Aim2 protein and that increased levels of the p202 protein in female mice of certain strains are associated with lupus susceptibility, we compared the expression of Aim2 and Ifi202 genes between Aim2-deficient and age-matched wild type mice. We found that the Aim2-deficiency in immune cells stimulated the expression of Ifi202 gene. The increased levels of the p202 protein in cells were associated with increases in the expression of IFN-β, STAT1, and IFN-inducible genes. Moreover, after knockdown of Aim2 expression in the murine macrophage cell line J774.A1, IFN-β treatment of cells robustly increased STAT1 protein levels (as compared to control cells), increased the activating phosphorylation of STAT1 on Tyr-701, and stimulated the activity of an IFN-responsive reporter. Notably, the expression of Aim2 in non lupus-prone (C57BL/6 and B6.Nba2-C) and lupus-prone B6.Nba2-ABC splenic cells and in a murine macrophage cell line that overexpressed p202 protein was found to be inversely correlated with Ifi202. Collectively, our observations demonstrate an inverse correlation between Aim2 and p202 expressions. We predict that defects in Aim2 expression within immune cells contribute to increased susceptibility to lupus. PMID:21057088

  20. Systemic Lupus Erythematosus and Pregnancy.

    Science.gov (United States)

    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.

  1. Sjögren's Syndrome

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  2. Pauci-immune lupus nephritis: possibility or co-incidence?

    Science.gov (United States)

    Cansu, Döndü Üsküdar; Temiz, Gökhan; Açıkalın, Mustafa F.; Korkmaz, Cengiz

    2017-01-01

    Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized with immune complex formation and renal involvement of lupus and may include several kinds of pathological conditions, but mostly, it is associated with immune complex-induced glomerular disease. Pauci-immune lupus nephritis is a very rare condition. We describe a 45-year-old female patient with pauci-immune crescentic necrotizing lupus nephritis and briefly discuss the possible mechanism and pathogenesis.

  3. Lupus Foundation of America

    Science.gov (United States)

    ... and Testing New Treatments Learn More About the Lupus Foundation of America We are devoted to solving ... Spam Control Text: Please leave this field empty Lupus FAQ What is lupus? What are the common ...

  4. Discoid Lupus Erythematosus

    Science.gov (United States)

    ... 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 ...

  5. Síndrome do pulmão encolhido no lúpus eritematoso sistêmico Shrinking lung syndrome in systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Cíntia Andrade Costa

    2004-06-01

    Full Text Available O lúpus eritematoso sistêmico pode envolver o aparelho respiratório de diversas maneiras como com pleurite, pneumonite, doença intersticial ou hipertensão pulmonar. Raramente, o paciente com lúpus eritematoso sistêmico pode apresentar uma síndrome caracterizada por dispnéia, dor torácica, alteração nas provas funcionais pulmonares e ausência de alterações parenquimatosas significativas na avaliação tomográfica de tórax, a qual tem sido denominada síndrome do pulmão encolhido. Descrevemos um caso que preenche os critérios diagnósticos dessa síndrome, e enfatizamos a patogênese que tem sido proposta, assim como as opções terapêuticas disponíveis.Systemic lupus erythematosus (SLE may involve the respiratory tract in several ways, such as through pleuritis, pneumonitis, interstitial disease or pulmonary hypertension. In rare cases, SLE patients present a syndrome characterized by dyspnea, chest pain and abnormalities in pulmonary function testing, although there may be no evidence of major parenchymal lung disease on computerized tomography scans. This condition has come to be known as shrinking lung syndrome. We report a case that meets these diagnostic criteria, emphasizing the pathogenesis proposed, as well as the therapeutic options available.

  6. Gastrointestinal symptomatology as first manifestation of systemic erythematous lupus

    Directory of Open Access Journals (Sweden)

    Kovačević Zoran

    2009-01-01

    Full Text Available Background. Systemic lupus erithematodes (SLE is chronic, often febrile, multisystemic disease unknown origin and relapsing course which affects connective tissue of the skin, joints, kidney and serous membranes. Gastrointestinal manifestations are rarely the first sign of systemic lupus erythematosus. Case report. We presented a female patient, 35 years old, whose first symptoms of SLE were paralitic ileus (chronic intestinal pseudo-obstruction and polyserositis (pleural effusion and ascites. Except for high parameters of inflammation, leucopenia and thrombocytopenia, all immunological and laboratory tests for SLE were negative in the onset of the disease. During next six months the patient had clinical signs of paralitic ileus several times and was twice operated with progressive malabsorptive syndrome. The full picture of SLE was manifested seven months later associated with lupus nephritis. Treatment with cyclophosphamide, corticosteroids and total parenteral nutrition (30 days induced stable remission of the disease. Conclusion. The SLE can be initially manifested with gastroenterological symptoms without any other clinical and immunologic parameters of the disease. If in patients with SLE and gastrointestinal tract involvement malabsorption syndrom is developed, a treatment success depends on both immunosupressive therapy and total parenteral nutrition.

  7. Complement split product C4d deposition in placenta in systemic lupus erythematosus and pregnancy-induced hypertension.

    Science.gov (United States)

    Minamiguchi, Sachiko; Mikami, Yoshiki; Nakajima, Naoki; Salah, Adeeb; Kondoh, Eiji; Tatsumi, Keiji; Konishi, Ikuo; Haga, Hironori

    2013-03-01

    Systemic lupus erythematosus (SLE) and pregnancy-induced hypertension (PIH) are related to premature delivery and intrauterine growth restriction (IUGR), and share histological findings of the placenta. Association with complement dysregulation has been reported in pregnancy for both disorders. The purpose of this study was to investigate the utility of C4d immunohistochemistry for placentas with SLE- and PIH-associated pregnancy. C4d staining was performed on paraffin-embedded tissue of placentas from 26 patients with SLE, 26 with PIH, and 25 control cases. We used the H-score with a range of 0-300 for the evaluation of C4d immunoreactivity. Placentas of SLE and PIH cases showed a higher H-score than control cases (average, SLE, 38.3 (P < 0.05); PIH, 17.8; control, 1.68), with linear staining on the membrane of syncytiotrophoblast. C4d-high groups comprised 50% (12/26) of SLE and 35% (9/26) of PIH cases, with H-scores ranging 14-270 and 15-170. C4d-high groups were significantly associated with low-placental weights and low birth weight in both SLE and PIH (P < 0.05), and lower gestational age (P < 0.05) in PIH cases. These results suggest that C4d might be utilized as a biomarker evaluating the subsequent risk for IUGR and disease control during the gestation period in these patients.

  8. Interleukin-17 expression positively correlates with disease severity of lupus nephritis by increasing anti-double-stranded DNA antibody production in a lupus model induced by activated lymphocyte derived DNA.

    Directory of Open Access Journals (Sweden)

    Zhenke Wen

    Full Text Available Lupus nephritis is one of the most serious manifestations and one of the strongest predictors of a poor outcome in systemic lupus erythematosus (SLE. Recent evidence implicated a potential role of interlukin-17 (IL-17 in the pathogenesis of lupus nephritis. However, the correlation between IL-17 expression level and the severity of lupus nephritis still remains incompletely understood. In this study, we found that serum IL-17 expression level was associated with the severity of lupus nephritis, which was evaluated by histopathology of kidney sections and urine protein. Of note, we showed that enforced expression of IL-17 using adenovirus construct that expresses IL-17 could enhance the severity of lupus nephritis, while blockade of IL-17 using neutralizing antibody resulted in decreased severity of lupus nephritis. Consistently, we observed an impaired induction of lupus nephritis in IL-17-deficient mice. Further, we revealed that IL-17 expression level was associated with immune complex deposition and complement activation in kidney. Of interest, we found that IL-17 was crucial for increasing anti-double-stranded DNA (dsDNA antibody production in SLE. Our results suggested that IL-17 expression level positively correlated with the severity of lupus nephritis, at least in part, because of its contribution to anti-dsDNA antibody production. These findings provided a novel mechanism for how IL-17 expression level correlated with disease pathogenesis and suggested that management of IL-17 expression level was a potential and promising approach for treatment of lupus nephritis.

  9. Tegafur/gimeracil/oteracil (TS-1 induced Stevens–Johnson syndrome: Case report

    Directory of Open Access Journals (Sweden)

    Satoko Minakawa

    2013-09-01

    Full Text Available TS-1 is an oral fluoropyrimidine anticancer drug that contains tegafur, gimeracil, and oteracil. A 78-year-old Japanese male who was diagnosed with carcinoma of the oral floor (rT4aN0M0 was prescribed a standard dose of TS-1 (80 mg/day. On Day 8 after administration of TS-1, an eruption developed. There was erythema, along with vesicles and erosions involving the lip, face, neck, trunk, limbs, and genitals. The drug-induced lymphocyte stimulation test (DLST for TS-1 was negative on the 23rd day, but positive on the 43rd day (20 days after discontinuing prednisolone. The condition was diagnosed as Stevens–Johnson syndrome due to TS-1 because of the clinical course and laboratory results. This case and 24 cases previously reported in the literature were analyzed. The types of drug eruption were drug-related lupus (9 cases, acral erythema (7 cases, scleroderma-like skin lesion (2 cases, Stevens–Johnson syndrome (2 cases, lichenoid eruption (1 case, purpura (1 case, lichen planus (1 case, erythema multiforme (1 case, hypopigmentation (1 case and toxic epidermal necrolysis (1 case, respectively. In view of the increasing usage of TS-1 in several common cancers, clinicians should be aware of drug eruptions due to TS-1.

  10. Neonatal lupus.

    Science.gov (United States)

    Robles, David T; Jaramillo, Lorena; Hornung, Robin L

    2006-12-10

    An otherwise healthy 5-week-old infant with erythematous plaques predominantly on the face and scalp presented to our dermatology clinic. The mother had been diagnosed with lupus erythematosus 2 years earlier but her disease was quiescent. Neonatal lupus is a rare condition associated with transplacental transfer of IgG anti-SSA/Ro and anti-SSB/La antibodies from the mother to the fetus. Active connective tissue disease in the mother does not have to be present and in fact is often absent. Although the cutaneous, hematologic and hepatic manifestations are transient, the potential for permanent heart block makes it necessary for this to be carefully ruled out. As in this case, the dermatologist may be the one to make the diagnosis and should be aware of the clinical presentation, work-up, and management of this important disease.

  11. Postural Orthostatic Tachycardia With Chronic Fatigue After HPV Vaccination as Part of the "Autoimmune/Auto-inflammatory Syndrome Induced by Adjuvants": Case Report and Literature Review.

    Science.gov (United States)

    Tomljenovic, Lucija; Colafrancesco, Serena; Perricone, Carlo; Shoenfeld, Yehuda

    2014-01-01

    We report the case of a 14-year-old girl who developed postural orthostatic tachycardia syndrome (POTS) with chronic fatigue 2 months following Gardasil vaccination. The patient suffered from persistent headaches, dizziness, recurrent syncope, poor motor coordination, weakness, fatigue, myalgias, numbness, tachycardia, dyspnea, visual disturbances, phonophobia, cognitive impairment, insomnia, gastrointestinal disturbances, and a weight loss of 20 pounds. The psychiatric evaluation ruled out the possibility that her symptoms were psychogenic or related to anxiety disorders. Furthermore, the patient tested positive for ANA (1:1280), lupus anticoagulant, and antiphospholipid. On clinical examination she presented livedo reticularis and was diagnosed with Raynaud's syndrome. This case fulfills the criteria for the autoimmune/auto-inflammatory syndrome induced by adjuvants (ASIA). Because human papillomavirus vaccination is universally recommended to teenagers and because POTS frequently results in long-term disabilities (as was the case in our patient), a thorough follow-up of patients who present with relevant complaints after vaccination is strongly recommended.

  12. Unveiling the hidden eagle: Acute parotitis-induced eagle syndrome

    Directory of Open Access Journals (Sweden)

    Nitipong Permpalung

    2014-01-01

    Full Text Available Context: A cervicofacial pain and foreign body sensation in pharynx associated with styloid process elongation is called Eagle syndrome. Typically, this syndrome is provoked by tonsillectomy or trauma. We report the first case of acute parotitis-induced Eagle syndrome. Case Report: A 65-year-old woman presented with right facial pain. CT scan of neck revealed asymmetric enhancement of the right parotid gland compatible with acute parotitis. All inflammation was resolved with antibiotics. However, the patient complained of pain in right mandibular region out of proportion to inflammation. Review CT found to have an asymmetrically long right styloid process measures. The diagnosis of acute parotitis-induced Eagle syndrome was established. Conclusion: Physicians should have a high index of suspicion for Eagle syndrome in patients with atypical neck pain and elongated styloid process since another significant manifestation of Eagle syndrome is carotid artery compression leading to recurrent syncope or stroke.

  13. 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.

  14. Venlafaxine-induced serotonin syndrome with relapse following amitriptyline

    OpenAIRE

    Perry, N

    2000-01-01

    A case of venlafaxine-induced serotonin syndrome is described with relapse following the introduction of amitriptyline, despite a 2-week period between the discontinuation of one drug and the commencement of the other. Electroencephalography may play an important part in diagnosis. With the increasing use of selective serotonin re-uptake inhibitors, greater awareness of the serotonin syndrome is necessary. Furthermore, the potential for drug interactions which may lead to the syndrome needs t...

  15. Unveiling The Hidden Eagle: Acute Parotitis-Induced Eagle Syndrome

    OpenAIRE

    Nitipong Permpalung; Promporn Suksaranjit; Daych Chongnarungsin; Hyman, Charles L.

    2014-01-01

    Context: A cervicofacial pain and foreign body sensation in pharynx associated with styloid process elongation is called Eagle syndrome. Typically, this syndrome is provoked by tonsillectomy or trauma. We report the first case of acute parotitis-induced Eagle syndrome. Case Report: A 65-year-old woman presented with right facial pain. CT scan of neck revealed asymmetric enhancement of the right parotid gland compatible with acute parotitis. All inflammation was resolved with antibiotics. Howe...

  16. What Is Lupus?

    Science.gov (United States)

    ... Lupus? (in Chinese 繁體中文 ) What Is Lupus? (in Korean 한국어 ) What Is Lupus? (in Vietnamese bằng tiếng ... and help you to keep a good outlook. Learning more about lupus is very important. Studies have ...

  17. Cancer treatment induced metabolic syndrome : Improving outcome with lifestyle

    NARCIS (Netherlands)

    Westerink, M. D. N. L.; Nuver, J.; Lefrandt, J. D.; Vrieling, A. H.; Gietema, J. A.; Walenkamp, A. M. E.

    2016-01-01

    Increasing numbers of long-term cancer survivors face important treatment related adverse effects. Cancer treatment induced metabolic syndrome (CTIMetS) is an especially prevalent and harmful condition. The aetiology of CTIMetS likely differs from metabolic syndrome in the general population, but

  18. Cancer treatment induced metabolic syndrome : Improving outcome with lifestyle

    NARCIS (Netherlands)

    Westerink, M. D. N. L.; Nuver, J.; Lefrandt, J. D.; Vrieling, A. H.; Gietema, J. A.; Walenkamp, A. M. E.

    2016-01-01

    Increasing numbers of long-term cancer survivors face important treatment related adverse effects. Cancer treatment induced metabolic syndrome (CTIMetS) is an especially prevalent and harmful condition. The aetiology of CTIMetS likely differs from metabolic syndrome in the general population, but ef

  19. Cancer treatment induced metabolic syndrome : Improving outcome with lifestyle

    NARCIS (Netherlands)

    Westerink, M. D. N. L.; Nuver, J.; Lefrandt, J. D.; Vrieling, A. H.; Gietema, J. A.; Walenkamp, A. M. E.

    2016-01-01

    Increasing numbers of long-term cancer survivors face important treatment related adverse effects. Cancer treatment induced metabolic syndrome (CTIMetS) is an especially prevalent and harmful condition. The aetiology of CTIMetS likely differs from metabolic syndrome in the general population, but ef

  20. Síndrome REM associada a lúpus eritematoso sistêmico e hipotireoidismo REM syndrome associated with systemic lupus erythematosus and hypotiroidism

    Directory of Open Access Journals (Sweden)

    Eleonora Dantas Dias

    2005-12-01

    Full Text Available A mucinose eritematosa reticulada é síndrome crônica e rara de etiologia desconhecida que afeta adultos jovens e de meia idade, principalmente do sexo feminino. Clinicamente é caracterizada por máculas eritematosas reticulares, pápulas e placas localizadas de forma simétrica em área central do tórax e dorso. Em aproximadamente 20% dos casos pode estar associada com várias doenças, especialmente auto-imunes. Os autores apresentam um caso de mucinose eritematosa reticulada associada a lúpus eritematoso sistêmico e hipotireoidismo.Reticular erythematous mucinosis is a chronic and rare syndrome of unknow aetiology that affects young adult and midle-aged women. Clinical presentation is characterized by macular and reticulated erythema, papula and plaques on the central chest and upper back of simmetrical form. In approximately 20% of the cases may be associated with a variety of disorders, especially auto-immune diseases. The authors present a case of reticular erythematous mucinoses associated with systemic lupus erythematosus and hypothiroidism.

  1. Functional impairments in white matter syndrome of neuropsychiatric systemic lupus erythematosus are similar to those observed in patients with multiple sclerosis

    Directory of Open Access Journals (Sweden)

    Denise Sisterolli-Diniz

    2012-10-01

    Full Text Available OBJECTIVE: In order to compare white matter syndrome of neuropsychiatric systemic lupus erythematosus (NPSLE and multiple sclerosis (MS, an assessment on demographic, medical history, and clinical data was proposed. METHODS: Sixty-four patients with NPSLE and 178 with MS answered a questionnaire and were evaluated regarding functional system, expanded disability status scale (EDSS, Beck depression inventory (BDI, and Beck anxiety inventory (BAI. RESULTS: The prevalence of autoimmune diseases and altered consciousness was similar in both groups, however it was higher than in the general population. Systemic signs and symptoms occurred from 2.9 to 61.9% of the MS cases, while neurological signs and symptoms occurred in 9.4 to 76.4% of the NPSLE ones. The motor, visual, and mental systems were the most affected in both diseases. The BDI in NPSLE had higher scores and the BAI in MS. CONCLUSIONS: The functional impairments in NPSLE were similar to those of MS, although greater impairment of the functional systems of cerebellar, sensitivity, and sphincters occurred in MS cases, and greater symptoms of depression, anxiety, and headache also occurred in it.

  2. Effect of Detoxification, Removing Stasis and Nourishing Yin Method on Corticosteroid-induced Hyperlipidemia in Patients with Systemic Lupus Erythematosus

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Objective: To observe the effect of TCM therapy for detoxification, removing stasis, and nourishing yin on corticosteroid-induced hyperlipemia in patients with systemic lupus erythematosus (SLE), and to investigate its mechanism. Methods: One hundred and seventy patients with SLE were randomly assigned to the integrative medicine group (IM group) and the Western medicine group (WM group), 85 in each group. Also, 30 healthy subjects selected from blood donors were enrolled in the normal control (NC) group. All patients were treated mainly with prednisone,while those in the IM group were given TCM therapy additionally, and the therapeutic course for both groups was 6 successive months. The changes of serum total cholesterol (TC), triglyceride (TG), high density lipoprotein cholesterol (HDL-C), Iow density lipoprotein cholesterol (LDL-C), very low density lipoprotein cholesterol (VLDL-C) and apolipoprotein A (ApoA) were determined and observed. A 2-year follow-up study was carried out in 16 patients of the WM group and 25 of the IM group. Results: Before treatment, no significant difference had been found among the three groups in the serum levels of lipids and lipoproteins. After the 6-month treatment, as compared with the WM group, the IM group showed lower levels of TC, TG, LDL-C, and VLDL-C (P<0.05 or P<0.01) and higher levels of HDL-C and ApoA (P<0.05). A similar effect was also shown by the follow-up study in the IM group (P<0.05 or P<0.01).Conclusion: TCM therapy for detoxification, removing stasis, and nourishing yin can effectively regulate the levels of serum lipids and lipoproteins in preventing and treating SLE patients with corticosteroidinduced hyperlipemia.

  3. Ultraviolet radiation (UVR) induces cell-surface Ro/SSA antigen expression by human keratinocytes in vitro: a possible mechanism for the UVR induction of cutaneous lupus lesions

    Energy Technology Data Exchange (ETDEWEB)

    Jones, S.K. (Bristol Royal Infirmary (United Kingdom))

    1992-06-01

    Antinuclear antibodies are useful markers of connective tissue disease. In this study, UVB but not UVA induced the expression of Ro/SSA antigen on keratinocyte surfaces in vitro. This expression was also found with the extractable nuclear antigens RnP and Sm, but not with single or double-stranded DNA. The expression was prevented by blocking protein synthesis, suggesting that it was an active process. The results suggest that UVB exposure may result in the expression of Ro/SSA antigen on the surfaces of basal keratinocytes in vivo. This antigen could then bind circulating antibody leading to the cutaneous lesions in neonatal and subacute cutaneous lupus erythematosus. (Author).

  4. Diet and Nutrition With Lupus

    Science.gov (United States)

    ... on Twitter Facebook Pinterest Email Print Diet and nutrition with lupus Lupus Foundation of America September 26, ... Living with Lupus I Have Lupus Exercise and Nutrition Site Footer Need to talk to someone? Our ...

  5. Diet and Nutrition With Lupus

    Science.gov (United States)

    ... on Twitter Facebook Pinterest Email Print Diet and nutrition with lupus Lupus Foundation of America September 18, ... Living with Lupus I Have Lupus Exercise and Nutrition Site Footer Need to talk to someone? Our ...

  6. S.L.E. Lupus Foundation

    Science.gov (United States)

    ... August 12, 2016 We've Moved! More News › Lupus News Tuesday, August 2, 2016 Congressional Lupus Caucus ... LUPUS RESEARCH ALLIANCE NYS Fall Calendar Living with Lupus Home | About Us | Our Programs | About Lupus | Lupus ...

  7. Ultraviolet light and cutaneous lupus

    NARCIS (Netherlands)

    Bijl, Marc; Kallenberg, Cees G. M.

    2006-01-01

    Exposure to ultraviolet (UV) light is one of the major factors known to trigger cutaneous disease activity in (systemic) lupus erythematosus patients. UV light, UVB in particular, is a potent inducer of apoptosis. Currently, disturbed clearance of apoptotic cells is one of the concepts explaining th

  8. Ultraviolet light and cutaneous lupus

    NARCIS (Netherlands)

    Bijl, Marc; Kallenberg, Cees G. M.

    2006-01-01

    Exposure to ultraviolet (UV) light is one of the major factors known to trigger cutaneous disease activity in (systemic) lupus erythematosus patients. UV light, UVB in particular, is a potent inducer of apoptosis. Currently, disturbed clearance of apoptotic cells is one of the concepts explaining th

  9. Nucleosome-induced neutrophil activation occurs independently of TLR9 and endosomal acidification: implications for systemic lupus erythematosus.

    NARCIS (Netherlands)

    Lindau, D.S.U.; Ronnefarth, V.; Erbacher, A.; Rammensee, H.G.; Decker, P. de

    2011-01-01

    The nucleosome is a major autoantigen known to activate PMN in systemic lupus erythematosus (SLE). TLR9 recognizes bacterial and even mammalian DNA under certain circumstances. Nevertheless, the role of TLR9 in SLE development is still unclear. Since nucleosomes are composed of DNA, we investigated

  10. Flow-Induced Dispersion Analysis for Probing Anti-dsDNA Antibody Binding Heterogeneity in Systemic Lupus Erythematosus Patients

    DEFF Research Database (Denmark)

    Poulsen, Nicklas N; Pedersen, Morten E; Østergaard, Jesper

    2016-01-01

    Detection of immune responses is important in the diagnosis of many diseases. For example, the detection of circulating autoantibodies against double-stranded DNA (dsDNA) is used in the diagnosis of Systemic Lupus Erythematosus (SLE). It is, however, difficult to reach satisfactory sensitivity...

  11. 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.

  12. [Systemic lupus erythematosus and pregnancy].

    Science.gov (United States)

    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.

  13. 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

  14. Increased risk of systemic lupus erythematosus in pregnancy-induced hypertension: A nationwide population-based retrospective cohort study.

    Science.gov (United States)

    Lin, Li-Te; Wang, Peng-Hui; Tsui, Kuan-Hao; Cheng, Jiin-Tsuey; Cheng, Jin-Shiung; Huang, Wei-Chun; Tang, Pei-Ling; Hu, Li-Yu

    2016-07-01

    Dysregulation of the immune system plays a role in the pathogenesis of both, pregnancy-induced hypertension (PIH) and systemic lupus erythematosus (SLE). It is well known that SLE predisposes to be complicated with PIH. However, few studies have attempted to investigate whether PIH increased subsequent SLE risk.The objectives of this study were to assess the association between PIH and subsequent SLE risk and identify predictive risk factors.Patients with newly diagnosed PIH were selected from the Taiwan National Health Insurance Research Database (NHIRD) and compared with a matched cohort without PIH based on age and the year of delivery. The incidence of new-onset SLE was evaluated in both cohorts. The overall observational period was from January 1, 2000 to December 31, 2013.Among the 23.3 million individuals registered in the NHIRD, 29,091 patients with PIH and 116,364 matched controls were identified. The incidence of SLE was higher among patients with PIH than in the matched controls (incidence rate ratio [IRR] = 4.02, 95% confidence interval [CI] 3.98-4.05, P < 0.0001). The IRR for subsequent SLE development remained significantly higher in all stratifications during the follow-up years. The multivariate Cox regression model was performed and the results showed that PIH may be an independent risk factors for the development of subsequent SLE (hazard ratio [HR] = 2.87, 95% CI 2.07-3.98, P < 0.0001). Moreover, multivariate Cox regression model was used again among the PIH cohort only in order to identify the possible risk factors for subsequent SLE in the population with PIH.Patients with PIH may have higher risk of developing newly diagnosed SLE than those without PIH. In addition, among individuals who have experienced PIH, those younger than 30 years, having experienced preeclampsia/eclampsia, single parity, preterm birth, or chronic kidney disease, may display an increased subsequent risk of SLE.

  15. 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

  16. In-hospital mortality in febrile lupus patients based on 2016 EULAR/ACR/PRINTO classification criteria for macrophage activation syndrome.

    Science.gov (United States)

    Ahn, Sung Soo; Yoo, Byung-Woo; Jung, Seung Min; Lee, Sang-Won; Park, Yong-Beom; Song, Jason Jungsik

    2017-10-01

    To evaluate the clinical significance of the 2016 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for macrophage activation syndrome (MAS) in patients with febrile systemic lupus erythematosus (SLE). We performed a retrospective analysis of SLE patients with fever, who were admitted to Severance Hospital between December 2005 and May 2016. Patients were evaluated for MAS using the 2016 classification criteria for MAS. Clinical features and laboratory findings were compared and overall survival rate was analyzed. Forward and backward stepwise logistic regression analysis was used to evaluate the factors associated with in-hospital mortality. Among 157 patients with SLE, 54 (34.3%) were considered to have MAS on admission (n = 42) and during admission (n = 12). For patients who already have MAS on admission, their baseline laboratory findings demonstrated lower CRP, platelets, total protein, albumin, complement C3, fibrinogen and higher AST, ALT, total bilirubin, ferritin, and triglyceride. The overall survival rate was significantly lower in patients with MAS than without MAS (64.8% vs. 97.0%, p < 0.001). Multivariate analysis showed that the presence of MAS was significantly associated with in-hospital mortality in febrile SLE patients (OR = 64.5; 95% CI: 7.6-544.4; p < 0.001). The 2016 classification criteria for MAS is useful to identify febrile SLE patients at high risk for in-hospital mortality. Monitoring febrile SLE patients with the new 2016 classification criteria might aid in the early detection of MAS. Copyright © 2017 Elsevier Inc. All rights reserved.

  17. X Chromosome Dose and Sex Bias in Autoimmune Diseases: Increased Prevalence of 47,XXX in Systemic Lupus Erythematosus and Sjögren's Syndrome.

    Science.gov (United States)

    Liu, Ke; Kurien, Biji T; Zimmerman, Sarah L; Kaufman, Kenneth M; Taft, Diana H; Kottyan, Leah C; Lazaro, Sara; Weaver, Carrie A; Ice, John A; Adler, Adam J; Chodosh, James; Radfar, Lida; Rasmussen, Astrid; Stone, Donald U; Lewis, David M; Li, Shibo; Koelsch, Kristi A; Igoe, Ann; Talsania, Mitali; Kumar, Jay; Maier-Moore, Jacen S; Harris, Valerie M; Gopalakrishnan, Rajaram; Jonsson, Roland; Lessard, James A; Lu, Xianglan; Gottenberg, Jacques-Eric; Anaya, Juan-Manuel; Cunninghame-Graham, Deborah S; Huang, Andrew J W; Brennan, Michael T; Hughes, Pamela; Illei, Gabor G; Miceli-Richard, Corinne; Keystone, Edward C; Bykerk, Vivian P; Hirschfield, Gideon; Xie, Gang; Ng, Wan-Fai; Nordmark, Gunnel; Eriksson, Per; Omdal, Roald; Rhodus, Nelson L; Rischmueller, Maureen; Rohrer, Michael; Segal, Barbara M; Vyse, Timothy J; Wahren-Herlenius, Marie; Witte, Torsten; Pons-Estel, Bernardo; Alarcon-Riquelme, Marta E; Guthridge, Joel M; James, Judith A; Lessard, Christopher J; Kelly, Jennifer A; Thompson, Susan D; Gaffney, Patrick M; Montgomery, Courtney G; Edberg, Jeffrey C; Kimberly, Robert P; Alarcón, Graciela S; Langefeld, Carl L; Gilkeson, Gary S; Kamen, Diane L; Tsao, Betty P; McCune, W Joseph; Salmon, Jane E; Merrill, Joan T; Weisman, Michael H; Wallace, Daniel J; Utset, Tammy O; Bottinger, Erwin P; Amos, Christopher I; Siminovitch, Katherine A; Mariette, Xavier; Sivils, Kathy L; Harley, John B; Scofield, R Hal

    2016-05-01

    More than 80% of autoimmune disease predominantly affects females, but the mechanism for this female bias is poorly understood. We suspected that an X chromosome dose effect accounts for this, and we undertook this study to test our hypothesis that trisomy X (47,XXX; occurring in ∼1 in 1,000 live female births) would be increased in patients with female-predominant diseases (systemic lupus erythematosus [SLE], primary Sjögren's syndrome [SS], primary biliary cirrhosis, and rheumatoid arthritis [RA]) compared to patients with diseases without female predominance (sarcoidosis) and compared to controls. All subjects in this study were female. We identified subjects with 47,XXX using aggregate data from single-nucleotide polymorphism arrays, and, when possible, we confirmed the presence of 47,XXX using fluorescence in situ hybridization or quantitative polymerase chain reaction. We found 47,XXX in 7 of 2,826 SLE patients and in 3 of 1,033 SS patients, but in only 2 of 7,074 controls (odds ratio in the SLE and primary SS groups 8.78 [95% confidence interval 1.67-86.79], P = 0.003 and odds ratio 10.29 [95% confidence interval 1.18-123.47], P = 0.02, respectively). One in 404 women with SLE and 1 in 344 women with SS had 47,XXX. There was an excess of 47,XXX among SLE and SS patients. The estimated prevalence of SLE and SS in women with 47,XXX was ∼2.5 and ∼2.9 times higher, respectively, than that in women with 46,XX and ∼25 and ∼41 times higher, respectively, than that in men with 46,XY. No statistically significant increase of 47,XXX was observed in other female-biased diseases (primary biliary cirrhosis or RA), supporting the idea of multiple pathways to sex bias in autoimmunity. © 2016, American College of Rheumatology.

  18. X Chromosome Dose and Sex Bias in Autoimmune Diseases: Increased 47,XXX in Systemic Lupus Erythematosus and Sjögren’s Syndrome

    Science.gov (United States)

    Liu, Ke; Kurien, Biji T.; Zimmerman, Sarah L.; Kaufman, Kenneth M.; Taft, Diana H.; Kottyan, Leah C.; Lazaro, Sara; Weaver, Carrie A.; Ice, John A.; Adler, Adam J.; Chodosh, James; Radfar, Lida; Rasmussen, Astrid; Stone, Donald U.; Lewis, David M.; Li, Shibo; Koelsch, Kristi A.; Igoe, Ann; Talsania, Mitali; Kumar, Jay; Maier-Moore, Jacen S.; Harris, Valerie M.; Gopalakrishnan, Rajaram; Jonsson, Roland; Lessard, James A.; Lu, Xianglan; Gottenberg, Jacques-Eric; Anaya, Juan-Manuel; Cunninghame-Graham, Deborah S.; Huang, Andrew J. W.; Brennan, Michael T.; Hughes, Pamela; Illei, Gabor G.; Miceli-Richard, Corinne; Keystone, Edward C.; Bykerk, Vivian P.; Hirschfield, Gideon; Xie, Gang; Ng, Wan-Fai; Nordmark, Gunnel; Eriksson, Per; Omdal, Roald; Rhodus, Nelson L.; Rischmueller, Maureen; Rohrer, Michael; Segal, Barbara M.; Vyse, Timothy J.; Wahren-Herlenius, Marie; Witte, Torsten; Pons-Estel, Bernardo; Alarcon-Riquelme, Marta E.; Guthridge, Joel M.; James, Judith A.; Lessard, Christopher J.; Kelly, Jennifer A.; Thompson, Susan D.; Gaffney, Patrick M.; Montgomery, Courtney G.; Edberg, Jeffrey C; Kimberly, Robert P; Alarcón, Graciela S.; Langefeld, Carl L.; Gilkeson, Gary S.; Kamen, Diane L.; Tsao, Betty P.; McCune, W. Joseph; Salmon, Jane E.; Merrill, Joan T.; Weisman, Michael H; Wallace, Daniel J; Utset, Tammy O; Bottinger, Erwin P.; Amos, Christopher I.; Siminovitch, Katherine A.; Mariette, Xavier; Sivils, Kathy L.

    2016-01-01

    Objective More than 80% of autoimmune disease is female dominant, but the mechanism for this female bias is poorly understood. We suspected an X chromosome dose effect and hypothesized that trisomy X (47,XXX , 1 in ~1,000 live female births) would be increased in female predominant diseases (e.g. systemic lupus erythematosus [SLE], primary Sjögren’s syndrome [SS], primary biliary cirrhosis [PBC] and rheumatoid arthritis [RA]) compared to diseases without female predominance (sarcoidosis) and controls. Methods We identified 47,XXX subjects using aggregate data from single nucleotide polymorphism (SNP) arrays and confirmed, when possible, by fluorescent in situ hybridization (FISH) or quantitative polymerase chain reaction (q-PCR). Results We found 47,XXX in seven of 2,826 SLE and three of 1,033 SS female patients, but only in two of the 7,074 female controls (p=0.003, OR=8.78, 95% CI: 1.67-86.79 and p=0.02, OR=10.29, 95% CI: 1.18-123.47; respectively). One 47,XXX subject was present for ~404 SLE women and ~344 SS women. 47,XXX was present in excess among SLE and SS subjects. Conclusion The estimated prevalence of SLE and SS in women with 47,XXX was respectively ~2.5 and ~2.9 times higher than in 46,XX women and ~25 and ~41 times higher than in 46,XY men. No statistically significant increase of 47,XXX was observed in other female-biased diseases (PBC or RA), supporting the idea of multiple pathways to sex bias in autoimmunity. PMID:26713507

  19. Clinical significance and correlations between anti-β2 glycoprotein I IgA assays in antiphospholipid syndrome and/or systemic lupus erythematosus.

    Science.gov (United States)

    Tebo, Anne E; Willis, Rohan; Jaskowski, Troy D; Guerra, Marta; Pierangeli, Silvia S; Salmon, Jane; Petri, Michelle; Branch, D Ware

    2016-09-01

    The objective of this investigation was to examine the clinical significance of IgA anti-β2 glycoprotein I (anti-β2GPI) antibodies and the inter-assay relationships between kits for their determination. Serum samples from 269 patients with clinical diagnoses of systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS), individuals positive for antiphospholipid antibodies (aPL) with or without APS or SLE, and 182 controls were tested for anti-β2GPI IgA antibodies using kits from four manufacturers. The positivity rates for the different IgA anti-β2GPI antibody kits varied in the disease groups; 7.8-14.7% (SLE only), 12.0-15.7% (SLE and APS/aPL), 14.7-58.8% (APS only), and 17.4-52.2% (aPL only). Kappa agreements between any 2 kits within disease groups were also variable and ranged from 0.25-1.00 (SLE), 0.18-1.00 (SLE and APS/aPL), 0.22-0.94 (APS only), and 0.32-0.91 (aPL only). Univariate analyses also showed variable relative risks for specific APS clinical manifestations with the different kits evaluated. Overall, diagnostic and predictive values for IgA anti-β2GPI antibodies are kit-dependent; therefore results are not interchangeable. While all 4 kits seem able to predict venous thrombosis tolerably well, there was a variable performance in predicting pregnancy related morbidity. Efforts to standardize these assays are highly needed prior to their formal adoption in routine clinical evaluation. Copyright © 2016. Published by Elsevier B.V.

  20. EULAR recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome.

    Science.gov (United States)

    Andreoli, L; Bertsias, G K; Agmon-Levin, N; Brown, S; Cervera, R; Costedoat-Chalumeau, N; Doria, A; Fischer-Betz, R; Forger, F; Moraes-Fontes, M F; Khamashta, M; King, J; Lojacono, A; Marchiori, F; Meroni, P L; Mosca, M; Motta, M; Ostensen, M; Pamfil, C; Raio, L; Schneider, M; Svenungsson, E; Tektonidou, M; Yavuz, S; Boumpas, D; Tincani, A

    2017-03-01

    Develop recommendations for women's health issues and family planning in systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS). Systematic review of evidence followed by modified Delphi method to compile questions, elicit expert opinions and reach consensus. Family planning should be discussed as early as possible after diagnosis. Most women can have successful pregnancies and measures can be taken to reduce the risks of adverse maternal or fetal outcomes. Risk stratification includes disease activity, autoantibody profile, previous vascular and pregnancy morbidity, hypertension and the use of drugs (emphasis on benefits from hydroxychloroquine and antiplatelets/anticoagulants). Hormonal contraception and menopause replacement therapy can be used in patients with stable/inactive disease and low risk of thrombosis. Fertility preservation with gonadotropin-releasing hormone analogues should be considered prior to the use of alkylating agents. Assisted reproduction techniques can be safely used in patients with stable/inactive disease; patients with positive antiphospholipid antibodies/APS should receive anticoagulation and/or low-dose aspirin. Assessment of disease activity, renal function and serological markers is important for diagnosing disease flares and monitoring for obstetrical adverse outcomes. Fetal monitoring includes Doppler ultrasonography and fetal biometry, particularly in the third trimester, to screen for placental insufficiency and small for gestational age fetuses. Screening for gynaecological malignancies is similar to the general population, with increased vigilance for cervical premalignant lesions if exposed to immunosuppressive drugs. Human papillomavirus immunisation can be used in women with stable/inactive disease. Recommendations for women's health issues in SLE and/or APS were developed using an evidence-based approach followed by expert consensus. Published by the BMJ Publishing Group Limited. For permission to use

  1. Neuroleptic-induced acute respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    Francisco Garcia Soriano

    Full Text Available CONTEXT: A case of neuroleptic malignant syndrome and acute respiratory distress syndrome is presented and discussed with emphasis on the role of muscle relaxation, creatine kinase, and respiratory function tests. CASE REPORT: A 41-year-old man presented right otalgia and peripheral facial paralysis. A computed tomography scan of the skull showed a hyperdense area, 2 cm in diameter, in the pathway of the anterior intercommunicating cerebral artery. Preoperative examination revealed: pH 7.4, PaCO2 40 torr, PaO2 80 torr (room air, Hb 13.8 g/dl, blood urea nitrogen 3.2 mmol/l, and creatinine 90 mmol/l. The chest x-ray was normal. The patient had not eaten during the 12-hour period prior to anesthesia induction. Intravenous halothane, fentanyl 0.5 mg and droperidol 25 mg were used for anesthesia. After the first six hours, the PaO2 was 65 torr (normal PaCO2 with FiO2 50% (PaO2/FiO2 130, and remained at this level until the end of the operation 4 hours later, maintaining PaCO2 at 35 torr. A thrombosed aneurysm was detected and resected, and the ends of the artery were closed with clips. No vasospasm was present. This case illustrates that neuroleptic drugs can cause neuroleptic malignant syndrome associated with acute respiratory distress syndrome. Neuroleptic malignant syndrome is a disease that is difficult to diagnose. Acute respiratory distress syndrome is another manifestation of neuroleptic malignant syndrome that has not been recognized in previous reports: it may be produced by neuroleptic drugs independent of the manifestation of neuroleptic malignant syndrome. Some considerations regarding the cause and effect relationship between acute respiratory distress syndrome and neuroleptic drugs are discussed. Intensive care unit physicians should consider the possibility that patients receiving neuroleptic drugs could develop respiratory failure in the absence of other factors that might explain the syndrome.

  2. Disruption of mutually negative regulatory feedback loop between interferon-inducible p202 protein and the E2F family of transcription factors in lupus-prone mice

    Science.gov (United States)

    Panchanathan, Ravichandran; Xin, Hong; Choubey, Divaker

    2010-01-01

    Summary Studies have identified interferon-inducible Ifi202 gene as a lupus susceptibility gene (encoding p202 protein) in mouse models of lupus disease. However, signaling pathways that regulate the Ifi202 expression in cells remain to be elucidated. We found that steady-state levels of Ifi202 mRNA and protein were high in mouse embryonic fibroblasts (MEFs) from E2F1-knockout (E2F1-/-) and E2F1 and E2F2 double knockout (E2F1-/- E2F2-/-) mice than isogenic wild type MEFs. Moreover, overexpression of E2F1 in mouse fibroblasts decreased expression of p202. Furthermore, expression of E2F1, but not E2F4, transcription factor in mouse fibroblasts repressed the activity of 202-luc-reporter in promoter-reporter assays. Interestingly, the E2F1-mediated transcriptional repression of the 202-luc-reporter was independent of p53 and pRb expression. However, the repression was dependent on the ability of E2F1 to bind DNA. We have identified a potential E2F DNA-binding site in the 5′-regulatory region of the Ifi202 gene and mutations in this E2F DNA-binding site reduced the E2F1-mediated transcriptional repression of 202-luc-reporter. Because p202 inhibits the E2F1-mediated transcriptional activation of genes, we compared the expression of E2F1 and its target genes in splenic cells from lupus-prone B6.Nba2 congenic mice, which express increased levels of p202, with age-matched C57BL/6 mice. We found that increased expression of Ifi202 in the congenic mice was associated with inhibition of E2F1-mediated transcription and decreased expression of E2F1 and its target genes that encode pro-apoptotic proteins. Our observations support for the idea that increased Ifi202 expression in certain strain of mice contributes to lupus susceptibility in part by inhibiting E2F1-mediated functions. PMID:18424712

  3. Systemic Lupus Erythematosus

    Science.gov (United States)

    ... 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 ...

  4. Treatment of lupus nephritis.

    NARCIS (Netherlands)

    Dolff, S.; Berden, J.H.M.; Bijl, M. van der

    2010-01-01

    Renal involvement in systemic lupus erythematosus patients is a severe disease manifestation characterized by various clinical and histopathological alterations. The revised International Society of Nephrology/Renal Pathology Society 2003 classification defines the subclasses of lupus nephritis (LN)

  5. Treatment of lupus nephritis

    NARCIS (Netherlands)

    Dolff, Sebastian; Berden, Jo H. M.; Bijl, Marc

    2010-01-01

    Renal involvement in systemic lupus erythematosus patients is a severe disease manifestation characterized by various clinical and histopathological alterations The revised International Society of Nephrology/Renal Pathology Society 2003 classification defines the subclasses of lupus nephritis (LN)

  6. Stevens-Johnson syndrome induced by phenytoin: a case report

    Directory of Open Access Journals (Sweden)

    Lalkota Prakash Bhanu

    2016-12-01

    Full Text Available Stevens-Johnson syndrome (SJS and Toxic epidermal necrolysis (TEN are rare (one to two per 10,00,00 population per year but life threatening adverse drug reactions. Antiepileptic drugs-induced Stevens-Johnson syndrome (SJS is a life-threatening severe cutaneous adverse reaction, amongst anti-epileptics; carbamazepine and phenytoin are the major culprits. We report here a case of SJS due to phenytoin (CTC vs 2 Grade 3.

  7. 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

  8. Lupus Activity in Pregnancy

    OpenAIRE

    Clowse, Megan E. B.

    2007-01-01

    Pregnancy in a woman with Systemic Lupus Erythematosus (SLE) can be complicated by both lupus activity and pregnancy mishaps. The majority of recent studies demonstrate an increase in lupus activity during pregnancy, perhaps exacerbated by hormonal shifts required to maintain pregnancy. Increased lupus activity, in turn, prompts an elevated risk for poor pregnancy outcomes, including stillbirth, preterm birth, low birth weight, and preeclamspsia. Fortunately, the majority of pregnancies in wo...

  9. Mycophenolate mofetil in the treatment of lupus nephritis

    Directory of Open Access Journals (Sweden)

    Patrick FK Yong

    2008-06-01

    Full Text Available Patrick FK Yong1,2, David P D’Cruz21Department of Clinical Immunology, Kings College Hospital; 2The Lupus Research Unit, St Thomas’ Hospital, London, UKAbstract: Lupus nephritis is a complication of systemic lupus erythematosus, which has significant morbidity and mortality. The accepted standard of treatment for severe lupus nephritis is cyclophosphamide for induction of remission. This has significant adverse effects including severe infection and amenorrhea. In addition, although cyclophosphamide induces remission, long-term mortality does not seem to be altered. Mycophenolate mofetil (MMF is an immunosuppressive agent originally used in solid organ transplantation, which has been compared with cyclophosphamide in trials for lupus nephritis. Randomized trials with MMF have been relatively small, although pooled data seem to suggest that it is at least as effective as cyclophosphamide in inducing remission. In addition, MMF has also been associated with a reduced risk of infection and amenorrhea, although this finding is not universal. MMF appears to be associated with more diarrhea compared with cyclophosphamide. MMF is likely to be a useful treatment for lupus nephritis, although available trial data are limited due to the small size of previous studies. A large trial (the Aspreva Lupus Management Study is currently underway to attempt to establish the place of MMF in treatment of lupus nephritis.Keywords: mycophenolate mofetil, lupus nephritis, systemic lupus erythematosus

  10. IL-17 induces autoantibody overproduction and peripheral blood mononuclear cell overexpression of IL-6 in lupus nephritis patients

    Institute of Scientific and Technical Information of China (English)

    董光富; 叶任高; 史伟; 刘双信; 汪涛; 阳晓; 杨念生; 余学清

    2003-01-01

    Objective To investigate the role of IL-17 in the overproduction of autoantibodies and IL-6 overexpression by peripheral blood mononuclear cells (PBMC) of lupus nephritis (LN) patients.Methods Fifteen consecutively hospitalized LN patients were selected as subjects and 15 healthy adults as normal controls. PBMC were obtained by Ficoll density gradient centrifugation. IgG, anti-dsDNA antibody and IL-6 protein levels were assessed using enzyme-linked immunosorbent assays (ELISA) on the supernatant of cultured PBMC of LN patients or normal controls. IL-6 mRNA levels in PBMC were measured using reverse transcription-polymerase chain reaction (RT-PCR).Results In medium culture, IgG, anti-dsDNA and IL-6 protein levels of the supernatant of PBMC from LN patients were significantly higher than those from normal controls (1492.1±73.2 ng/ml vs 636.7±51.9 ng/ml for IgG, 306.6±53.7 IU/ml vs 95.8±11.6 IU/ml for anti-dsDNA and 50.92±15.92 ng/ml vs 1.77±0.73 ng/ml for IL-6, all P<0.001). In LN patients, IgG, anti-dsDNA and IL-6 protein levels were higher in the supernatants of PBMC in the IL-17-stimulated culture than the medium culture, but in normal controls, only the IL-6 protein levels were significantly higher. The increase in IgG, anti-dsDNA and IL-6 protein levels induced by IL-17 was dose-dependent and could be completely blocked by IL-17 monoclonal antibody mIgG28 and partially blocked by dexamethasone. Similarly, IL-6 mRNA overexpression of PBMC in LN patients or normal controls induced by IL-17 was both dose- and time-dependent. During medium culture, IL-6 mRNA levels in LN patients were significantly higher than those in normal controls (1.80±0.11 vs 0.36±0.07). During stimulation with IL-17, IL-6 mRNA levels in LN patients were higher than those in normal controls (3.21±0.24 vs 1.30±0.14, P<0.05) and also significantly higher when comparing the stimulated culture with the medium culture either in LN patients or normal control.Conclusions IL-17 may play an

  11. Lupus nephritis: the central role of nucleosomes revealed.

    Science.gov (United States)

    Mortensen, Elin S; Fenton, Kristin A; Rekvig, Ole P

    2008-02-01

    Systemic lupus erythematosus (SLE) is an autoimmune syndrome characterized by autoantibodies to nuclear constituents. Some of these antibodies are diagnostically important, whereas others act as disease-modifying factors. One clinically important factor is autoantibodies against dsDNA and nucleosomes, which have overlapping diagnostic and nephritogenic impact in SLE. Although a scientific focus for 5 decades, the molecular and cellular origin of these antibodies, and why they are associated with lupus nephritis, is still not fully understood. A consensus has, however, evolved that antibodies to dsDNA and nucleosomes are central pathogenic factors in the development of lupus nephritis. In contrast, no agreement has been reached as to which glomerular structures are bound by nephritogenic anti-nucleosome antibodies in vivo. Mutually contradictory paradigms and models have evolved simply because we still lack precise and conclusive data to provide definitive insight into how autoantibodies induce lupus nephritis and which specificity is critical in the nephritic process(es). In this review, data demonstrating the central role of nucleosomes in inducing and binding potentially nephritogenic antibodies to DNA and nucleosomes are presented and discussed. These autoimmune-inducing processes are discussed in the context of Matzinger's danger model (Matzinger P: Friendly and dangerous signals: is the tissue in control? Nat Immunol 2007, 8:11-13; Matzinger P: The danger model: a renewed sense of self. Science 2002, 296:301-305; Matzinger P: Tolerance, danger, and the extended family. Annu Rev Immunol 1994, 12:991-1045) and Medzhitov's and Janeway's (Medzhitov R, Janeway CA Jr: Decoding the patterns of self and nonself by the innate immune system. Science 2002, 296:298-300; Medzhitov R, Janeway CA Jr: How does the immune system distinguish self from nonself? Semin Immunol 2000, 12:185-188; Janeway CA Jr, Medzhitov R: Innate immune recognition. Annu Rev Immunol 2002, 20

  12. Interferon-alpha induced Raynaud's syndrome.

    Science.gov (United States)

    Kruit, W H; Eggermont, A M; Stoter, G

    2000-11-01

    The cytokine interferon-alpha (IFN-alpha) is increasingly prescribed for a number of indications, especially viral hepatitis and several malignancies. Two patients are described who developed Raynaud's syndrome during treatment with IFN-alpha as adjuvant therapy for high-risk melanoma. With a review of the available literature the symptomatology, possible pathophysiologic mechanisms and treatment options are discussed.

  13. Rowell syndrome

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    Ramesh Y Bhat

    2014-01-01

    Full Text Available Rowell syndrome is a rare disease consisting of erythema multiforme-like lesions associated with lupus erythematosus. The syndrome occurs mostly in middle-aged women. The authors describe the syndrome in a 15-year-old boy who responded well to systemic steroids and hydroxychloroquine.

  14. Olanzapine-Induced Neuroleptic Malignant Syndrome

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    Seyedhamze Hosseini

    2017-05-01

    Full Text Available Neuroleptic malignant syndrome (NMS is a rare but life-threatening idiosyncratic side effect resulting from neuroleptic drugs. NMS mainly occurs in patients treated with high-potency typical antipsychotics, but rarely caused by atypical antipsychotics. Although NMS is less common with atypical antipsychotic, but it seems that its incidence is rising due to increased administration of such drugs. We present the case of a 27-year-old man with a history of paranoid schizophrenia that showed signs consistent with NMS that occurred after treatment with olanzapine. The patient was adherent to treatment. He had decreased level of consciousness, muscle rigidity, diaphoresis, fever, drooling, urinary incontinence, and high blood pressure. This patient illustrates that NMS can occur due to treatment with atypical antipsychotic drugs like olanzapine, particularly in the presence of risk factors. This phenomenon is often unrecognized, underdiagnosed, or not treated properly. Physicians should be aware that NMS with extrapyramidal syndrome could occur with olanzapine at steady state doses without recent dosage adjustments or titration. It is essential that adequate and safe dose of medication is chosen and the patient is monitored by the signs and symptoms of this lethal syndrome.

  15. Phenobarbital induced Stevens–Johnson syndrome in a child

    OpenAIRE

    Sumit Gaur; Rupali Agnihotri

    2012-01-01

    Phenobarbital, an antiepileptic agent has numerous adverse reactions including Stevens- Johnson syndrome (SJS), a rare medical emergency. A 12-year-old male epileptic child with phenobarbital-induced SJS was referred for the management of severe pain in relation to extensively decayed molar tooth and oral mucosal ulcerations. The patient was managed by withdrawal of phenobarbital and palliative treatment of the lesions.

  16. Phenobarbital induced Stevens-Johnson syndrome in a child.

    Science.gov (United States)

    Gaur, Sumit; Agnihotri, Rupali

    2012-01-01

    Phenobarbital, an antiepileptic agent has numerous adverse reactions including Stevens- Johnson syndrome (SJS), a rare medical emergency. A 12-year-old male epileptic child with phenobarbital-induced SJS was referred for the management of severe pain in relation to extensively decayed molar tooth and oral mucosal ulcerations. The patient was managed by withdrawal of phenobarbital and palliative treatment of the lesions.

  17. Haematological manifestations of lupus

    Science.gov (United States)

    Fayyaz, Anum; Igoe, Ann; Kurien, Biji T; Danda, Debashish; James, Judith A; Stafford, Haraldine A; Scofield, R Hal

    2015-01-01

    Our purpose was to compile information on the haematological manifestations of systemic lupus erythematosus (SLE), namely leucopenia, lymphopenia, thrombocytopenia, autoimmune haemolytic anaemia (AIHA), thrombotic thrombocytopenic purpura (TTP) and myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manifestation. Neutropenia can lead to morbidity and mortality from increased susceptibility to infection. Severe neutropenia can be successfully treated with granulocyte colony-stimulating factor. While related to disease activity, there is no specific therapy for lymphopenia. Severe lymphopenia may require the use of prophylactic therapy to prevent select opportunistic infections. Isolated idiopathic thrombocytopenic purpura maybe the first manifestation of SLE by months or even years. Some manifestations of lupus occur more frequently in association with low platelet count in these patients, for example, neuropsychiatric manifestation, haemolytic anaemia, the antiphospholipid syndrome and renal disease. Thrombocytopenia can be regarded as an important prognostic indicator of survival in patients with SLE. Medical, surgical and biological treatment modalities are reviewed for this manifestation. First-line therapy remains glucocorticoids. Through our review, we conclude glucocorticoids do produce a response in majority of patients initially, but sustained response to therapy is unlikely. Glucocorticoids are used as first-line therapy in patients with SLE with AIHA, but there is no conclusive evidence to guide second-line therapy. Rituximab is promising in refractory and non-responding AIHA. TTP is not recognised as a criteria for classification of SLE, but there is a considerable overlap between the presenting features of TTP and SLE, and a few patients with SLE have concurrent

  18. Haematological manifestations of lupus.

    Science.gov (United States)

    Fayyaz, Anum; Igoe, Ann; Kurien, Biji T; Danda, Debashish; James, Judith A; Stafford, Haraldine A; Scofield, R Hal

    2015-01-01

    Our purpose was to compile information on the haematological manifestations of systemic lupus erythematosus (SLE), namely leucopenia, lymphopenia, thrombocytopenia, autoimmune haemolytic anaemia (AIHA), thrombotic thrombocytopenic purpura (TTP) and myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manifestation. Neutropenia can lead to morbidity and mortality from increased susceptibility to infection. Severe neutropenia can be successfully treated with granulocyte colony-stimulating factor. While related to disease activity, there is no specific therapy for lymphopenia. Severe lymphopenia may require the use of prophylactic therapy to prevent select opportunistic infections. Isolated idiopathic thrombocytopenic purpura maybe the first manifestation of SLE by months or even years. Some manifestations of lupus occur more frequently in association with low platelet count in these patients, for example, neuropsychiatric manifestation, haemolytic anaemia, the antiphospholipid syndrome and renal disease. Thrombocytopenia can be regarded as an important prognostic indicator of survival in patients with SLE. Medical, surgical and biological treatment modalities are reviewed for this manifestation. First-line therapy remains glucocorticoids. Through our review, we conclude glucocorticoids do produce a response in majority of patients initially, but sustained response to therapy is unlikely. Glucocorticoids are used as first-line therapy in patients with SLE with AIHA, but there is no conclusive evidence to guide second-line therapy. Rituximab is promising in refractory and non-responding AIHA. TTP is not recognised as a criteria for classification of SLE, but there is a considerable overlap between the presenting features of TTP and SLE, and a few patients with SLE have concurrent

  19. MicroRNA expression profiles identify disease-specific alterations in systemic lupus erythematosus and primary Sjögren's syndrome

    Science.gov (United States)

    Póliska, Szilárd; Szabó, Krisztina; Tarr, Tünde; Bálint, Bálint László; Szodoray, Péter

    2017-01-01

    The discovery of microRNAs (miRNAs) and their critical role in genetic control opened new avenues in understanding of various biological processes including immune cell lineage commitment, differentiation, proliferation and apoptosis. However, a given miRNA may have hundreds of different mRNA targets and a target might be regulated by multiple miRNAs, thus the characterisation of dysregulated miRNA expression profiles could give a better insight into the development of immunological disturbances in autoimmune diseases. The aim of our study was to examine the changes in miRNA expression profiles in patients with systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS). Eight SLE patients, 8 pSS patients and 7 healthy subjects were enrolled in the investigation. MiRNAs were isolated from peripheral blood mononuclear cells, and expression patterns were determined with Illumina next-generation sequencing technology. Since the immunopathogenesis of pSS and SLE encompasses pronounced B cell hyperactivity along with specific autoantibody production, we paid a special attention on the association between miRNA expression levels and altered peripheral B cell distribution. In SLE patients 135, while in pSS patients 26 miRNAs showed altered expression. Interestingly, the 25 miRNAs including miR-146a, miR-16 and miR-21, which were over-expressed in pSS patients, were found to be elevated in SLE group, as well. On the contrary, we observed the down-regulation of miR-150-5p, which is a novel and unique finding in pSS. Levels of several miRNAs over-expressed in SLE, were not changed in pSS, such as miR-148a-3p, miR-152, miR-155, miR-223, miR-224, miR-326 and miR-342. Expression levels of miR-223-5p, miR-150-5p, miR-155-5p and miR-342-3p, which miRNAs are potentially linked to B cell functions, showed associations with the B cell proportions within peripheral blood mononuclear cells. The observed differences in miRNA expression profiles and the better understanding

  20. Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA)

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  1. Anti-Lipid IgG Antibodies Are Produced via Germinal Centers in a Murine Model Resembling Human Lupus

    Science.gov (United States)

    Wong-Baeza, Carlos; Reséndiz-Mora, Albany; Donis-Maturano, Luis; Wong-Baeza, Isabel; Zárate-Neira, Luz; Yam-Puc, Juan Carlos; Calderón-Amador, Juana; Medina, Yolanda; Wong, Carlos; Baeza, Isabel; Flores-Romo, Leopoldo

    2016-01-01

    Anti-lipid IgG antibodies are produced in some mycobacterial infections and in certain autoimmune diseases [such as anti-phospholipid syndrome, systemic lupus erythematosus (SLE)]. However, few studies have addressed the B cell responses underlying the production of these immunoglobulins. Anti-lipid IgG antibodies are consistently found in a murine model resembling human lupus induced by chlorpromazine-stabilized non-bilayer phospholipid arrangements (NPA). NPA are transitory lipid associations found in the membranes of most cells; when NPA are stabilized they can become immunogenic and induce specific IgG antibodies, which appear to be involved in the development of the mouse model of lupus. Of note, anti-NPA antibodies are also detected in patients with SLE and leprosy. We used this model of lupus to investigate in vivo the cellular mechanisms that lead to the production of anti-lipid, class-switched IgG antibodies. In this murine lupus model, we found plasma cells (Gr1−, CD19−, CD138+) producing NPA-specific IgGs in the draining lymph nodes, the spleen, and the bone marrow. We also found a significant number of germinal center B cells (IgD−, CD19+, PNA+) specific for NPA in the draining lymph nodes and the spleen, and we identified in situ the presence of NPA in these germinal centers. By contrast, very few NPA-specific, extrafollicular reaction B cells (B220+, Blimp1+) were found. Moreover, when assessing the anti-NPA IgG antibodies produced during the experimental protocol, we found that the affinity of these antibodies progressively increased over time. Altogether, our data indicate that, in this murine model resembling human lupus, B cells produce anti-NPA IgG antibodies mainly via germinal centers. PMID:27746783

  2. Clinical utility of circulating anti-N-methyl-d-aspartate receptor subunits NR2A/B antibody for the diagnosis of neuropsychiatric syndromes in systemic lupus erythematosus and Sjögren's syndrome: An updated meta-analysis.

    Science.gov (United States)

    Tay, Sen Hee; Fairhurst, Anna-Marie; Mak, Anselm

    2017-02-01

    Neuropsychiatric (NP) events are found in patients with rheumatic diseases, commonly in systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS). The standard nomenclature and case definitions for 19 NPSLE syndromes by the American College of Rheumatology (ACR) Committee on Research cover a wide range of NP events seen in both SLE and SS. Despite advances in the understanding of SLE and SS, NP syndromes continue to pose diagnostic challenges. Correct attribution of NP events is critical in determining the correct treatment and prognosis. Anti-N-methyl-d-aspartate receptor subunits NR2A/B (anti-NR2A/B) antibodies have been demonstrated in the sera of SLE and SS patients and have been associated with collective or specific NP syndromes, though not consistently. Interpretation of anti-NR2A/B antibody data in the medical literature is rendered difficult by small sample size of patient groups. By combining different studies to generate a pooled effect size, a meta-analysis can increase the power to detect differences in the presence or absence of NP syndromes. Hence, we set out to perform a meta-analysis to assess the association between anti-NR2A/B antibodies and NP syndromes in SLE and SS. A literature search was conducted using PubMed and other databases from inception to June 2016. We abstracted data relating to anti-NR2A/B antibodies from the identified studies. The random effects model was used to calculate overall combined odds ratio (OD) with its corresponding 95% confidence interval (CI) to evaluate the relationship between anti-NR2A/B antibodies and NP syndromes in SLE and SS patients with and without NP events. We also included our own cohort of 57 SLE patients fulfilling the ACR 1997 revised classification criteria and 58 healthy controls (HCs). In total, 17 studies with data on anti-NR2A/B antibodies in 2212 SLE patients, 66 SS patients, 99 disease controls (DCs) (e.g. antiphospholipid syndrome, myasthenia gravis and autoimmune polyendocrine syndrome

  3. Living with Lupus (For Parents)

    Science.gov (United States)

    ... Your 1- to 2-Year-Old Living With Lupus KidsHealth > For Parents > Living With Lupus A A ... disease for both doctors and their patients. About Lupus A healthy immune system produces proteins called antibodies ...

  4. A Unique Cause of Proteinuria in Pregnancy: Class II Lupus Nephritis with Concomitant Minimal Change Disease

    Science.gov (United States)

    Kunjal, Ryan; Adam-Eldien, Rabie; Makary, Raafat; Jo-Hoy, Francois; Heilig, Charles W.

    2016-01-01

    We report the case of a 22-year-old African American female who presented to another facility for routine follow-up in the 34th week of pregnancy with lower extremity swelling and nephrotic-range proteinuria. Although she was normotensive, it was initially thought that she had preeclampsia. She was monitored carefully and delivery was induced at 37 weeks of gestation. She was transferred to our hospital, where she was diagnosed with systemic lupus erythematosus (SLE) based on clinical and laboratory criteria. Renal biopsy revealed a surprising finding of minimal change disease (MCD) concomitant with class II lupus nephritis (LN). She was managed with pulses and then tapering doses of steroid therapy with dramatic resolution of the nephrotic syndrome. This case demonstrates not only the rare de novo occurrence of SLE in pregnancy, but the unique finding of MCD coexisting with class II LN. We propose that altered T cell activity may be the link between these seemingly distinct entities.

  5. Neuropsychiatric Lupus in clinical practice

    Directory of Open Access Journals (Sweden)

    Helena Alessi

    Full Text Available ABSTRACT Systemic lupus erythematosus (SLE is a chronic autoimmune disease involving multiple organs, characterized by the production of autoantibodies and the development of tissue injury. The etiology of SLE is partially known, involving multiple genetic and environmental factors. As many as 50% of patients with SLE have neurological involvement during the course of their disease. Neurological manifestations are associated with impaired quality of life, and high morbidity and mortality rates. Nineteen neuropsychiatric syndromes have been identified associated with SLE, and can be divided into central and peripheral manifestations. This article reviews major neuropsychiatric manifestations in patients with SLE and discusses their clinical features, radiological findings and treatment options.

  6. Moderate Sle With Lupus Nefritis

    OpenAIRE

    Marpaung, Blondina; Sinurat, Faisal

    2016-01-01

    Lupus nephritis is one of the most serious manifestations of systemic lupus erythematosus (LES) and usually appear within 5 years after diagnosis. Lupus nephritis is histologically evident in most patients with SLE, even those who do not show clinical manifestations of kidney disease. Symptoms of lupus nephritis is generally associated with hypertension, proteinuria and renal failure. Reported a case of systemic lupus erythematosus (SLE) with severe lupus nephritis in a man 23-years old bo...

  7. Systemic lupus erythematosus: 2 case reports from Eritrea

    African Journals Online (AJOL)

    and cells are damaged by pathogenic antibodies ... reproductive age and characterized by the presence of antibodies in the serum against the nuclear components. (ANA) ... Case B. Systemic Lupus Erythematosis and Vasculitis. A 23 year young lady admitted to the Sembel .... to be associated with drug- induced lupus.

  8. Methyl salicylate 2-O-β-d-lactoside alleviates the pathological progression of pristane-induced systemic lupus erythematosus-like disease in mice via suppression of inflammatory response and signal transduction

    Science.gov (United States)

    He, Yang-Yang; Yan, Yu; Zhang, Hui-Fang; Lin, Yi-Huang; Chen, Yu-Cai; Yan, Yi; Wu, Ping; Fang, Jian-Song; Yang, Shu-Hui; Du, Guan-Hua

    2016-01-01

    Systemic lupus erythematosus (SLE), with a high incidence rate and insufficient therapy worldwide, is a complex disease involving multiple organs characterized primarily by inflammation due to deposition of immunocomplexes formed by production of autoantibodies. The mechanism of SLE remains unclear, and the disease still cannot be cured. We used pristane to induce SLE in female BALB/c mice. Methyl salicylate 2-O-β-d-lactoside (MSL; 200, 400, and 800 mg/kg) was orally administered 45 days after pristane injection for 4.5 months. The results showed that MSL antagonized the increasing levels of multiple types of antibodies and cytokines in lupus mice. MSL was found to suppress joint swelling and have potent inhibitory effect on arthritis-like symptoms. MSL also significantly decreased the spleen index and expression of inflammatory markers in the lupus mice. MSL protected the kidneys of lupus mice from injury through inhibiting the expression of inflammatory cytokines and reducing the IgG and C3 immunocomplex deposits. Further Western blot assays revealed that the downregulation of the intracellular inflammatory signals of NFκB and JAK/STAT3 might be the potential molecular mechanisms of the pharmacological activity of MSL against SLE in vivo. These findings may demonstrate that MSL has the potential to be a useful and highly effective treatment for SLE. PMID:27729775

  9. Schwannosis induced medullary compression in VACTERL syndrome.

    LENUS (Irish Health Repository)

    Treacy, A

    2011-10-21

    A 7-year-old boy with a history of VACTERL syndrome was found collapsed in bed. MRI had shown basilar invagination of the skull base and narrowing of the foramen magnum. Angulation, swelling and abnormal high signal at the cervicomedullary junction were felt to be secondary to compression of the medulla. Neuropathologic examination showed bilateral replacement of the medullary tegmentum by an irregularly circumscribed cellular lesion which was composed of elongated GFAP\\/S 100-positive cells with spindled nuclei and minimal atypia. The pathologic findings were interpreted as intramedullary schwannosis with mass effect. Schwannosis, is observed in traumatized spinal cords where its presence may represent attempted, albeit aberrant, repair by inwardly migrating Schwann cells ofperipheral origin. In our view the compressive effect of the basilar invagination on this boy\\'s medulla was of sufficient magnitude to have caused tumoral medullary schwannosis with resultant intermittent respiratory compromise leading to reflex anoxic seizures.

  10. Association Study of a Proliferation-inducing Ligand, Spermatogenesis Associated 8, Platelet-derived Growth Factor Receptor-alpha, and POLB Polymorphisms with Systemic Lupus Erythematosus in Chinese Han Population

    OpenAIRE

    Ping Li; Yuan Li; Ai-Hong Zhou; Si Chen; Jing Li; Xiao-Ting Wen; Zi-Yan Wu; Liu-Bing Li; Feng-Chun Zhang; Yong-Zhe Li

    2016-01-01

    Background: Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease with complex genetic inheritance. This study was conducted to examine whether the association of a proliferation-inducing ligand (APRIL), spermatogenesis associated 8 (SPATA8), platelet-derived growth factor receptor-alpha (PDGFRA), and DNA polymerase beta (POLB) with SLE can be replicated in a Chinese Han population. Methods: Chinese SLE patients (n = 1247) and ethnically and geographically matched healthy cont...

  11. Radiotherapy- and Chemotherapy-Induced Myelodysplasia Syndrome

    Science.gov (United States)

    Sun, Li-Min; Lin, Cheng-Li; Lin, Ming-Chia; Liang, Ji-An; Kao, Chia-Hung

    2015-01-01

    Abstract This study explored which kinds of cancer are related to a higher incidence of subsequent myelodysplastic syndrome (MDS) after radiotherapy (RT) and chemotherapy (CT). We performed a nested case–control study by using data from the Taiwanese National Health Insurance (NHI) system. The case group included cancer patients who developed MDS. For the control group, 4 cancer patients without MDS were frequency-matched with each MDS case by age, sex, year of cancer diagnosis, and MDS index year. A multivariable logistic regression analysis was conducted, and odds ratios (ORs) and 95% confidence intervals (CIs) were estimated. Overall, cancer patients who received RT or CT exhibited secondary MDS more frequently than did those who did not (RT: OR = 1.53; 95% CI = 1.33–1.77; CT: OR = 1.51; 95% CI = 1.25–1.82). Analysis by cancer site showed that RT increased the risk of MDS for patients with stomach, colorectal, liver, breast, endometrial, prostate, and kidney cancers. By contrast, CT was more likely to increase the risk of MDS for patients with lung, endometrial, and cervical cancers. Further analysis revealed that RT and CT seemed to have a positive interaction. The major limitation of this study was the lack of certain essential data in the NHI Research Database, such as data regarding cancer stage and treatment dose details. This population-based nested case–control study determined that RT and CT predisposed patients in Taiwan to the development of MDS. This effect was more prominent when both modalities were used. PMID:25929909

  12. Treatment of lichen planus-lupus erythematosus overlap syndrome with acitretin and tacrolimus%阿维A联合他克莫司治疗扁平苔藓——红斑狼疮重叠综合征

    Institute of Scientific and Technical Information of China (English)

    刘佳玮; 刘洁; 曾跃平; 刘跃华; 孙秋宁

    2013-01-01

    报告阿维A联合他克莫司软膏封包治疗扁平苔藓-红斑狼疮重叠综合征1例.患者女,72岁.双足跟反复糜烂34年.皮肤科检查:双足跟水肿性红斑散在糜烂,皮损组织病理符合扁平苔藓组织病理表现.实验室检查:抗核抗体(ANA)(+),滴度1∶80,均质型,抗双链DNA抗体(+),抗SS-A抗体(+),滴度1∶4.诊断:扁平苔藓-红斑狼疮重叠综合征.给予口服阿维A30 mg/d,0.1%他克莫司软膏局部封包治疗,皮损好转明显.%A case of lichen planus-lupus erythematosus overlap syndrome treated with oral acitretin and tacrolimus occlusion is reported. A 72-year-old woman presented with recurrent erosions on her soles for 34 years. Skin examination showed erosions and edematous erythemas on both heels. Histological findings were consistent with lichen planus. Laboratory tests revealed a homogeneous pattern of ANA, with titer of 1 : 80. Anti-dsDNA antibody was positive. Anti-SS-A antibody was positive, with titer of 1 : 4. The patient was diagnosed as lichen planus-lupus erythematosus overlap syndrome. Treatment with o-ral acitretin (30 mg/d) and 0.1% tacrolimus occlusion was successful.

  13. Neuroleptic malignant syndrome in cycloserine-induced psychosis

    Science.gov (United States)

    Sawant, Neena S.; Kate, Natasha S.; Bhatankar, Swaroop S.; Kulkarni, Prathamesh S.

    2015-01-01

    A 33-year-old multidrug-resistant tuberculosis female patient diagnosed as cycloserine-induced psychosis developed several neuroleptic side effects such as extrapyramidal reaction, neuroleptic malignant syndrome, and drug-induced parkinsonism while she was being treated with initially haloperidol and then olanzapine over a period of 2 months. Patient's antipsychotic medications were withdrawn, and treatment with bromocriptine showed prompt recovery. The multiple neurological adverse effects which the patient developed had implications on the management of the complications as well as her illness. PMID:26069374

  14. Neuroleptic malignant syndrome in cycloserine-induced psychosis

    Directory of Open Access Journals (Sweden)

    Neena S Sawant

    2015-01-01

    Full Text Available A 33-year-old multidrug-resistant tuberculosis female patient diagnosed as cycloserine-induced psychosis developed several neuroleptic side effects such as extrapyramidal reaction, neuroleptic malignant syndrome, and drug-induced parkinsonism while she was being treated with initially haloperidol and then olanzapine over a period of 2 months . Patient′s antipsychotic medications were withdrawn, and treatment with bromocriptine showed prompt recovery. The multiple neurological adverse effects which the patient developed had implications on the management of the complications as well as her illness.

  15. Neuroleptic malignant syndrome in cycloserine-induced psychosis.

    Science.gov (United States)

    Sawant, Neena S; Kate, Natasha S; Bhatankar, Swaroop S; Kulkarni, Prathamesh S

    2015-01-01

    A 33-year-old multidrug-resistant tuberculosis female patient diagnosed as cycloserine-induced psychosis developed several neuroleptic side effects such as extrapyramidal reaction, neuroleptic malignant syndrome, and drug-induced parkinsonism while she was being treated with initially haloperidol and then olanzapine over a period of 2 months. Patient's antipsychotic medications were withdrawn, and treatment with bromocriptine showed prompt recovery. The multiple neurological adverse effects which the patient developed had implications on the management of the complications as well as her illness.

  16. [Lupus nephritis treatment].

    Science.gov (United States)

    Santos-Araújo, Carla; Pestana, Manuel

    2008-01-01

    Systemic lupus erithematosus (SLE) is a multiorganic inflammatory disease characterized by a significant morbidity and mortality related not just to disease evolution but also to therapeutic side effects. Sixty percent of SLE patients develop renal disease related to lupus. Moreover, several studies report that lupus nephritis is an important predictor of both renal impairment and global mortality in these patients. In lupus nephritis, the renal biopsy still represents a cornerstone for both histological grading and therapeutical management. Several classification schemes for lupus nephritis based mainly on morphological parameters have been proposed so far. In the WHO grading system the most severe form of lupus nephritis is the diffuse proliferative lupus nephritis or lupus nephritis class IV. In fact, several authors have documented an invariable course to end stage renal failure in these patients, in the absence of specific therapy. Despite the considerable improvement observed since the introduction of corticosteroid and cyclophosphamide treatment, a significant number of patients still present an incomplete response to therapy. Moreover, even in the cases of good response to therapy adverse events related to the treatment such as infertility, hemorrhagic cystitis or increased susceptibility to infection frequently supervenes.

  17. Acute coronary syndrome in diclofenac sodium-induced type I hypersensitivity reaction : Kounis syndrome

    OpenAIRE

    Gluvic, Zoran M.; Putnikovic, Biljana; Panic, Milos; Stojkovic, Aleksandra; Rasic-Milutinovic, Zorica; Jankovic-Gavrilovic, Jelena

    2007-01-01

    Drug-induced type I hypersensitivity reactions are frequent. Sometimes, acute coronary syndrome (ACS) can be registered in such patients, which may have a serious impact on the course and management of the allergic reaction. Because of potentially atypical ACS clinical presentations, the ECG is an obligatory diagnostic tool in any allergic reaction. Coronary artery spasm is the pathophysiological basis of ACS, triggered by the action of potent vasoactive mediators (histamine, neutral protease...

  18. Lupus erythematosus cell phenomenon in pediatric bronchoalveolar lavages: possible manifestation of early radioadaptive response in radiation induced alveolitis.

    Science.gov (United States)

    Zunic, S

    2013-01-01

    A ten-year (December 1992 - December 2002) evaluation of 225 pediatric bronchoalveolar lavage (BAL) differential cell counts showed appearance of the cells corresponding to the cytological entity - lupus erythematosus cell (LEC) in 47 specimens of which not a single case was associated with the coexistent autoimmune disease. There was a significant increase in the percentage of LEC in BAL samples of the examinees during the first 6 months after the bombing of targets in Serbia (July-December 1999) in comparison to the period 1992 to March 24, 1999, and after the bombing of targets in Serbia (2000-2002). Maintaining the character of occurrence of LEC in BAL as nonspecific (Zunic et al. 1996), the devastating power of alpha particles (originated from uranium decay) gives an opportunity to discuss this phenomenon more comprehensibly and perceive a new vista related to the pathogenesis of LEC phenomenon in BAL. Since the period after 1991 corresponds to the time after the first Gulf War, and later the bombing of targets in Bosnia, the possibility of occurrence of LEC in BAL as a manifestation of radiation alveolitis due to contamination by air transferred depleted uranium (DU) particles could not be excluded.

  19. Autoimmune/autoinflammatory syndrome induced by adjuvants (ASIA syndrome) in commercial sheep.

    Science.gov (United States)

    Luján, Lluís; Pérez, Marta; Salazar, Eider; Álvarez, Neila; Gimeno, Marina; Pinczowski, Pedro; Irusta, Silvia; Santamaría, Jesús; Insausti, Nerea; Cortés, Yerzol; Figueras, Luis; Cuartielles, Isabel; Vila, Miguel; Fantova, Enrique; Chapullé, José Luis Gracia

    2013-07-01

    We describe a form of the autoimmune/autoinflammatory syndrome induced by adjuvants (ASIA syndrome) in commercial sheep, linked to the repetitive inoculation of aluminum-containing adjuvants through vaccination. The syndrome shows an acute phase that affects less than 0.5% of animals in a given herd, it appears 2-6 days after an adjuvant-containing inoculation and it is characterized by an acute neurological episode with low response to external stimuli and acute meningoencephalitis, most animals apparently recovering afterward. The chronic phase is seen in a higher proportion of flocks, it can follow the acute phase, and it is triggered by external stimuli, mostly low temperatures. The chronic phase begins with an excitatory phase, followed by weakness, extreme cachexia, tetraplegia and death. Gross lesions are related to a cachectic process with muscular atrophy, and microscopic lesions are mostly linked to a neurodegenerative process in both dorsal and ventral column of the gray matter of the spinal cord. Experimental reproduction of ovine ASIA in a small group of repeatedly vaccinated animals was successful. Detection of Al(III) in tissues indicated the presence of aluminum in the nervous tissue of experimental animals. The present report is the first description of a new sheep syndrome (ovine ASIA syndrome) linked to multiple, repetitive vaccination and that can have devastating consequences as it happened after the compulsory vaccination against bluetongue in 2008. The ovine ASIA syndrome can be used as a model of other similar diseases affecting both human and animals. A major research effort is needed in order to understand its complex pathogenesis.

  20. Bioactivation of minocycline to reactive intermediates by myeloperoxidase, horseradish peroxidase, and hepatic microsomes: implications for minocycline-induced lupus and hepatitis.

    Science.gov (United States)

    Mannargudi, Baskar; McNally, David; Reynolds, William; Uetrecht, Jack

    2009-09-01

    Of the tetracyclines, minocycline is unique in causing a significant incidence of a lupus-like syndrome and autoimmune hepatitis. It is also unique among the tetracyclines in having a para-N,N-dimethylaminophenol ring. Many drugs that cause autoimmune reactions are oxidized to reactive metabolites by the myeloperoxidase (MPO) system of macrophages. In this study, we showed that minocycline is oxidized to reactive intermediates by MPO/H(2)O(2)/Cl(-), HOCl, horseradish peroxidase/H(2)O(2), or hepatic microsomes. When trapped with N-acetylcysteine (NAC), two adducts with protonated molecular ions at m/z 619 were isolated and analyzed by NMR. One represents attack of the aromatic D ring by NAC meta to the N,N-dimethylamino group, which implies that the reactive intermediate was a quinone iminium ion. The NMR of the other adduct, which was not observed when minocycline was oxidized by hepatic microsomes, indicates that the NAC is attached at the junction of the B and C rings. In the oxidation by HOCl, we found an intermediate with a protonated molecular ion of m/z 510 that represents the addition of HOCl to minocycline. The HOCl presumably adds across the double bond of the B ring, and reaction of this intermediate with NAC led to the second NAC adduct. We were surprised to find that the same NAC adduct was not observed after oxidation of tetracycline with HOCl, even though this part of the tetracycline structure is the same as for minocycline. We propose that one or more of these reactive metabolites are responsible for the idiosyncratic drug reactions that are specific to this tetracycline.

  1. Recurrent congenital heart block in neonatal lupus.

    Science.gov (United States)

    Escobar, Maria C; Gómez-Puerta, José A; Albert, Dimpna; Ferrer, Queralt; Girona, Josep

    2007-07-01

    Congenital heart block (CHB) is the main complication of neonatal lupus (NL) and is strongly associated with the presence of anti-SSA/Ro and anti-SSB/La antibodies. The recurrence of CHB in subsequent pregnancies in mothers with these antibodies is uncommon, occurring in approximately 15% of cases. We describe here a case of recurrent CHB in a previously asymptomatic mother with Sjögren syndrome and discuss the current strategies for the prevention and treatment of CHB in NL.

  2. Alcohol-induced Cushing syndrome. Hypercortisolism caused by alcohol abuse.

    Science.gov (United States)

    Besemer, F; Pereira, A M; Smit, J W A

    2011-01-01

    Cushing's syndrome (CS), a rare syndrome caused by overexposure to glucocorticoids, is difficult to diagnose. The underlying causes of CS include pituitary and ectopic adrenocorticotropic hormone (ACTH) producing tumours and adrenal adenomas or hyperplasia. Alcoholism, however, can cause similar symptoms, giving rise to a so-called pseudo-Cushing state, which aggravates the differential diagnostic dilemmas of CS. To document any specific clinical or biochemical features of alcohol-induced CS. A Medline computer-aided search was performed to identify studies that have attempted to differentiate between alcohol-induced pseudo-Cushing and CS. Only original articles, not reviews, written in English were included. A total of 62 articles were included. Clinical and biochemical abnormalities mimicking increased hypothalamus-pituitary-adrenal (HPA) axis activity were found in the majority of the patients, although the severity of the changes varied widely. The most frequently occurring abnormalities were: insufficient suppression after low-dose dexamethasone or increased 24-hour urinary free cortisol (UFC). After alcohol withdrawal, cortisol decreased and dexamethasone-induced suppression of cortisol increased. No differences were noted between alcoholic and control subjects after an ACTH stimulation test, insulin tolerance test or metyrapone test. Differences were found after a naloxone test and hexarelin test. Studies using corticotropin-releasing hormone stimulation and tests after ethanol ingestion revealed inconclusive results. There is no clear definition for the alcohol-induced pseudo-Cushing state, and hitherto studies fail to provide clues to differentiate between pseudo-Cushing and Cushing's syndrome. Only cessation of alcohol can normalise biochemical abnormalities and regress hypercortisolic symptoms.

  3. Sepsis-Induced Myocardial Depression and Takotsubo Syndrome

    Directory of Open Access Journals (Sweden)

    Mustafa Kemal Arslantaş

    2015-08-01

    Full Text Available Sepsis induced temporary myocardial dysfunction characterized as impairment of myocardial contraction is an important cause of mortality and morbidity in intensive care units. Takotsubo syndrome (TS is temporary ballooning and dysfunction of the apical part of left ventricle without significant stenosis of coronary arteries. Recently, it was suggested that impairment in regional catecholamine distribution caused by stress factors and excessive cardiac sympathetic activity mechanism play role in sepsis such as other causes of TS. Additionally, vasopressor agents (as noradrenaline which are widely used in sepsis treatment may be triggering factor. Serial case reports of sepsis associated TS are reported, however pathophysiology, diagnosis and treatment strategies of these two different syndromes is not obvious.

  4. 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.

  5. Con: Cyclophosphamide for the treatment of lupus nephritis.

    Science.gov (United States)

    Mok, Chi Chiu

    2016-07-01

    Kidney involvement is a major determinant for morbidity and mortality in patients with systemic lupus erythematosus. The treatment target of lupus renal disease is to induce and maintain remission and to minimize disease or treatment-related comorbidities. Cyclophosphamide (CYC), in conjunction with glucocorticoids, has conventionally been used for the initial treatment of lupus nephritis. However, the major concerns of CYC are its toxicities, such as infertility, urotoxicity and oncogenicity, which are particularly relevant in women of childbearing age. As a result, maintenance therapy of lupus nephritis with an extended course of CYC pulses has largely been replaced by other immunosuppressive agents such as mycophenolate mofetil (MMF) and azathioprine. Recent randomized controlled trials have demonstrated non-inferiority of MMF to pulse CYC as induction therapy of lupus nephritis. Although MMF as induction-maintenance therapy has been increasingly used in lupus nephritis, its efficacy in the long-term preservation of renal function remains to be elucidated. MMF is not necessarily less toxic than CYC. Meta-analyses of clinical trials show similar incidence of infective complications and gastrointestinal adverse events in both MMF- and CYC-based regimens. However, considering the reduction in gonadal toxicity and the risk of oncogenicity, MMF may be used as first-line therapy of lupus nephritis. Tacrolimus (TAC) has recently been shown to be equivalent to either MMF or CYC for inducing remission of lupus nephritis and may be considered as another non-CYC alternative. Combined low-dose MMF and TAC appears to be more effective than CYC pulses in Chinese patients with lupus nephritis and has the potential to replace the more toxic CYC regimens in high-risk patients. Currently, CYC still plays an important role in the management of lupus nephritis patients with impaired or rapidly deteriorating renal function, crescentic glomerulonephritis or as salvage therapy for

  6. Hypertrophic discoid lupus erythematosus.

    Science.gov (United States)

    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.

  7. Cutaneous lupus erythematosus

    DEFF Research Database (Denmark)

    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...

  8. Antiphospholipid antibody syndrome presenting as transverse myelitis

    Directory of Open Access Journals (Sweden)

    Javvid M Dandroo

    2015-01-01

    Full Text Available The antiphospholipid syndrome (APS is characterized by arterial and/or venous thrombosis and pregnancy morbidity in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disorder or secondary to a connective tissue disease, most frequently systemic lupus erythematosus. Central nervous system involvement is one of the most prominent clinical manifestations of APS, and includes arterial and venous thrombotic events, psychiatric features, and a variety of other nonthrombotic neurological syndromes. Although the mechanism of neurological involvement in patients with APS is thought to be thrombotic in origin and endothelial dysfunction associated with antiphospholipid antibodies. APS presenting as acute transverse myelitis is very rarely seen with a prevalence rate of 1%. We are describing a foreigner female presenting as acute transverse myelitis which on evaluation proved to be APS induced. So far, very few cases have been reported in literature with APS as etiology.

  9. 吉兰-巴雷综合征为首发表现的系统性红斑狼疮合并原发性胆汁性肝硬化一例并文献复习%Systemic Lupus Erythematosus Combined With Primary Biliary Cirrhosis With Guillain - barre Syndrome as the Primary Manifestation:A Case Study and Literature Review

    Institute of Scientific and Technical Information of China (English)

    刘伟; 宋慧; 王丽芳; 颜淑敏

    2016-01-01

    Guillain - barre syndrome is a rare manifestation of nervous system damage caused by systemic lupus erythematosus. Systemic lupus erythematosusis is easily ignored when Guillain - barre syndrome is the primary manifestation. The paper reported the treatment process of a case of systemic lupus erythematosus combined with primary biliary cirrhosis with Guillain - barre syndrome as the primary manifestation. Literature was combined to make a review together in order to improve clinical doctors' understanding about the disease.%吉兰-巴雷综合征是系统性红斑狼疮神经系统损害的少见表现之一,特别是以吉兰-巴雷综合征为首发表现时更容易忽视系统性红斑狼疮的存在。本文报道1例以吉兰-巴雷综合征为首发表现的系统性红斑狼疮合并原发性胆汁性肝硬化患者的诊治经过,并结合文献进行复习,以提高临床医生对该病的认识。

  10. Lúpus eritematoso sistêmico juvenil em adolescente com síndrome da imunodeficiência adquirida Juvenile systemic lupus erythematosus in a adolescent with acquired immunodeficiency syndrome

    Directory of Open Access Journals (Sweden)

    Nathália de Carvalho Sacilotto

    2010-08-01

    Full Text Available O lúpus eritematoso sistêmico juvenil (LESJ é uma doença inflamatória crônica, multissistêmica e autoimune. Algumas manifestações clínicas dessa condição são semelhantes às encontradas na síndrome da imunodeficiência adquirida (SIDA. A coexistência da SIDA com o LESJ é rara, especialmente na população pediátrica, sendo descritos na literatura pesquisada apenas cinco casos de pacientes com infecção congênita por HIV que desenvolveram essa enfermidade reumatológica, tendo como manifestação inicial a nefrite lúpica. Relata-se o caso de uma paciente de 14 anos, com diagnóstico de infecção pelo HIV aos 8 meses de idade, na qual se realizou diagnóstico de LESJ com base nos critérios diagnósticos do Colégio Americano de Reumatologia. Este relato tem a finalidade de descrever uma paciente com SIDA que, embora tenha desenvolvido LESJ em sua forma clássica e grave, evoluiu satisfatoriamenteSystemic lupus erythematosus juvenile (SLEJ is a multi-systemic, chronic inflammatory disease, and with autoimmune features. Some clinical manifestations of this disease are similar to those found inAcquired Immunodeficiency Syndrome (AIDS. Coexistence of AIDS with SLEJ is rare, especially in the pediatric population, being described in the literature just 5 patients with congenital HIV infection who developed this rheumatological condition, presenting lupus nephritis as the initial manifestation. We report the case of a 14 year old patient, diagnosed with HIV infection at 8 months of age, with signs and symptoms of SLEJ. This report aims to describe a female patient with AIDS who developed SLE in its classic and forms, but has evolved satisfactorily

  11. Postural Orthostatic Tachycardia With Chronic Fatigue After HPV Vaccination as Part of the “Autoimmune/Auto-inflammatory Syndrome Induced by Adjuvants”

    Directory of Open Access Journals (Sweden)

    Lucija Tomljenovic PhD

    2014-03-01

    Full Text Available We report the case of a 14-year-old girl who developed postural orthostatic tachycardia syndrome (POTS with chronic fatigue 2 months following Gardasil vaccination. The patient suffered from persistent headaches, dizziness, recurrent syncope, poor motor coordination, weakness, fatigue, myalgias, numbness, tachycardia, dyspnea, visual disturbances, phonophobia, cognitive impairment, insomnia, gastrointestinal disturbances, and a weight loss of 20 pounds. The psychiatric evaluation ruled out the possibility that her symptoms were psychogenic or related to anxiety disorders. Furthermore, the patient tested positive for ANA (1:1280, lupus anticoagulant, and antiphospholipid. On clinical examination she presented livedo reticularis and was diagnosed with Raynaud’s syndrome. This case fulfills the criteria for the autoimmune/auto-inflammatory syndrome induced by adjuvants (ASIA. Because human papillomavirus vaccination is universally recommended to teenagers and because POTS frequently results in long-term disabilities (as was the case in our patient, a thorough follow-up of patients who present with relevant complaints after vaccination is strongly recommended.

  12. Thrombotic microangiopathy involving the gallbladder as an unusual manifestation of systemic lupus erythematosus and antiphospholipid syndrome: Case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    Beatriz De-Leon-Bojorge; Samuel Zaltzman-Girsevich; Arturo Ortega-Salgado; Adelina Prieto-Patron; Ruth Córdoba-Córdoba; Marco Yamazaki-Nakashimada

    2006-01-01

    Gallbladder disease is no more common in patients with systemic lupus erythematosus (SLE) than in the general population. We describe a 17-year-old patient with SLE, who developed nephritis that was well controlled with medications. Initial treatment consisted of azathioprine, aspirin and prednisone with stable control of her symptoms. Two years later she developed a right quadrant abdominal pain,and an abdominal ultrasound revealed microlithiasic cholecystitis. Open cholecystectomy was performed and the histopathological findings revealed vasculitis with thrombotic microangiopathy in the gallbladder. This case presentation illustrates that calculous or acalculous cholecystitis should be considered as a manifestation of active SLE and APS.

  13. Recurrent Hemolytic and Uremic Syndrome Induced by Escherichia Coli

    Science.gov (United States)

    Commereuc, Morgane; Weill, Francois-Xavier; Loukiadis, Estelle; Gouali, Malika; Gleizal, Audrey; Kormann, Raphaël; Ridel, Christophe; Frémeaux-Bacchi, Véronique; Rondeau, Eric; Hertig, Alexandre

    2016-01-01

    Abstract A widespread belief is that typical hemolytic and uremic syndrome (HUS) does not recur. We report the case of a patient infected twice with raw milk taken from his own cow and containing a Shiga toxin–producing Escherichia coli O174:H21 that induced recurrent HUS causing severe renal and cerebral disorders. A genomic comparison of the human and bovine Shiga toxin–producing Escherichia coli O174:H21 isolates revealed that they were identical. Typical HUS may recur. Since milk from this animal was occasionally distributed locally, thereby posing a serious threat for the whole village, this particular cow was destroyed. PMID:26735524

  14. Antipsychotic-Induced Neuroleptic Malignant Syndrome After Cardiac Surgery.

    Science.gov (United States)

    Moll, Vanessa; Ward, Ceressa T; Zivot, Joel B

    2016-07-01

    We report a case of neuroleptic malignant syndrome (NMS) in a postoperative cardiac surgery patient after the administration of typical and atypical antipsychotics for the treatment of delirium. On postoperative day 8, the patient's temperature peaked at 40.6°C. Agitation, rigidity, elevation in creatine kinase, and leukocytosis were associated findings. NMS was suspected on postoperative day 10. All antipsychotics were discontinued; dantrolene infusions and fluid therapy were initiated. After 2 days of NMS treatment, the patient's symptoms resolved. The temporal relationship between discontinuation of all antipsychotics, initiation of dantrolene, and clinical improvement supports the diagnosis of antipsychotic-induced NMS.

  15. Subacute lupus erythematosus during treatment with golimumab for seronegative rheumatoid arthritis.

    Science.gov (United States)

    Brunasso, A M G; Aberer, W; Massone, C

    2014-02-01

    We report on a 52-year-old woman with a history of severe seronegative rheumatoid arthritis. Several conventional therapies and biological therapy with etanercept and infliximab had been unsuccessful. In 2010 she was given golimumab subcutaneously at a monthly dose of 50 mg. She had a negative ANA titre. After 16 months of uninterrupted therapy and sustained response, she developed skin lesions on the upper trunk, back and upper extremities, which worsened on exposure to the sun. The skin biopsy was compatible with subacute lupus erythematosus. Laboratory findings included an ANA titre 1:640, negative anti-Ro/SSA and anti-DNA antibodies. Topical corticosteroid therapy proved inadequate. The patient's condition improved only after discontinuation of golimumab. The causal relationship between subacute cutaneous lupus erythematosus and golimumab is not dose-related and occurs with some delay (a typical feature of immunological adverse reactions). The association is likely, but not confirmed (because re-challenge was not performed). However, a clear improvement was noted after withdrawal. Based on this case, we hypothesized the aetiological role of golimumab-associated immunogenicity. TNF-α antagonist-induced lupus-like syndrome (TAILS) is a well-known side effect of this class of substances. The British Society of Rheumatology recommends discontinuation of the causal anti-TNF-α treatment in patients with TAILS.

  16. Nephrotic Syndrome and Acute Renal Failure Apparently Induced by Sunitinib

    Directory of Open Access Journals (Sweden)

    Ying-Shou Chen

    2009-10-01

    Full Text Available We report a case of nephrotic syndrome and acute renal failure apparently induced by sunitinib. A 67-year-old man with a history of metastatic renal cell carcinoma presented with progressive kidney dysfunction with proteinuria, general edema, and body weight gain of 21 kg after undergoing 3 weeks of sunitinib therapy. The patient had taken no other over-the-counter medications, and all other possible causes of nephrotic syndrome were excluded. The Naranjo Adverse Drug Reaction Probability Scale score for this event was 6, indicating a high probability that the observed presentations were associated with use of the drug. However, despite the discontinuation of sunitinib, his condition deteriorated, and hemodialysis was initiated for respiratory distress. A renal biopsy was performed, which revealed ischemic acute tubular necrosis with minimal change nephropathy. In conclusion, nephrologists and oncologists should be aware that nephrotic syndrome with ischemic acute tubular necrosis is a possible adverse effect of sunitinib. For early diagnosis of this condition and to avoid renal damage, we recommend differential diagnosis of serum creatinine and proteinuria in patients undergoing sunitinib therapy.

  17. Voriconazole-induced periostitis in a patient with overlap syndromes.

    Science.gov (United States)

    Hirota, Keisho; Yasoda, Akihiro; Fujii, Toshihito; Inagaki, Nobuya

    2014-03-05

    A 52-year-old woman with overlap syndrome and interstitial pneumonia underwent immunosuppressive therapy and she was suspected to suffer from pulmonary aspergillosis. Oral voriconazole was initiated, and a rapid elevation of alkaline phosphatase (ALP) occurred after 4 weeks. After 2 months, the patient presented diffuse pain in bilateral skeletal regions, and bone scintigraphy revealed bilateral multiple areas of increased radiotracer uptake. We suspected the skeletal involvement as voriconazole-induced periostitis. Actually, the plasma fluoride level was increased. Voriconazole was replaced with itraconazole, and after 3 weeks, the patient stopped complaining of bone pain concomitant with the decrease in ALP. Voriconazole-induced periostitis is a rare condition but had previously been reported in solid organ or patients with bone marrow transplant who received a long-term voriconazole therapy. Our present case is distinctive of previous ones, because it occurred in a patient with connective tissue disease which had its rapid progression.

  18. Successful capecitabine rechallenge following 5-fluorouracil-induced Takotsubo syndrome

    Science.gov (United States)

    Abdelrahman, Mohamed; McCarthy, Michael T.; Yusof, Haliana; Osman, Nemer

    2016-01-01

    Cardiac toxicity is a widely reported complication of fluoropyrimidine chemotherapies (5-fluorouracil and capecitabine); however, Takotsubo syndrome (TS) is less widely reported. There is little data available describing the viability of fluoropyrimidine rechallenge after fluoropyrimidine-induced TS. We report the case of Ms X, a 41-year-old woman with metastatic oesophageal cancer, who developed acute onset left ventricular dysfunction, with a measured left ventricular ejection fraction of 15% on cycle 1 day 3 of FOLFOX chemotherapy, after disconnection of the fluorouracil infusion pump. Her symptoms resolved over 2 days, and an echocardiogram returned to normal within 2 weeks. 5-Fluorouracil was discontinued, and replaced with capecitabine, without recurrence of symptoms. The remainder of her treatment was uneventful. This is the second case to describe successful capecitabine retreatment following 5-fluorouracil-induced TS. PMID:26989494

  19. A case of neuroleptic malignant syndrome induced by perospirone

    Institute of Scientific and Technical Information of China (English)

    Jing CHEN; Shengli ZHI

    2013-01-01

    Neuroleptic malignant syndrome (NMS) is a rare but life-threatening condition induced by neuroleptic medications. Its main symptoms include the rapid onset of fever, severe extrapyramidal symptoms, autonomic nervous system dysfunction, and impaired consciousness. In severe cases, acute renal failure and circulatory failure can develop, which can rapidly lead to death. In this case report, we discuss the etiology, pathophysiology and management of this condition in a female patient with NMS induced by perospirone. The case highlights the need for clinicians to be vigilant:rapid identification of NMS and vigorous symptomatic treatment of NMS symptoms is the key to decreasing the case-fatality of this rare but serious adverse reaction to antipsychotic medications.

  20. Clinical Features of Systemic Lupus Erythematosus Complicated with Evans Syndrome%系统性红斑狼疮合并 Evans 综合征临床特点

    Institute of Scientific and Technical Information of China (English)

    王来芳; 赵清; 王立; 郑文洁; 赵岩; 曾小峰; 张奉春

    2014-01-01

    目的:总结系统性红斑狼疮(systemic lupus erythematosus,SLE)合并 Evans 综合征患者的临床特点。方法回顾性分析北京协和医院2004年1月至2012年7月 SLE 合并 Evans 患者的临床表现及实验室特点及治疗和预后。结果 SLE 并发 Evans 综合征患者22例,占同期 SLE 住院患者3400例的0.65%。其中男3例,女19例,平均35.1岁(16~53岁)。22例患者中以血液系统受累[特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)或自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)]为首发表现的11例(50%),确诊 SLE 后诊断 Evans 综合征者6例,二者同时诊断的5例。SLE 并发 Evans 综合征时,患者往往有多系统受累,表现为肾脏受累13例(59.1%),皮肤黏膜受累、关节炎各9例(40.9%),神经系统受累4例(18.2%),胃肠道、肺部受累各2例等。Evans 综合征多发生于 SLE 活动期,患者平均狼疮活动指数评分(11.45±7.6)分(3~30分)。伴发其他结缔组织病5例(22.7%)。经激素联合免疫抑制剂治疗后,20例好转,2例无效者应用利妥昔单抗后好转。结论 SLE 合并 Evans 综合征罕见,发生于 SLE 多系统受累及活动期。部分患者以ITP 或 AIHA 为 SLE 首发表现,应及时筛查多种自身抗体,并定期随访密切观察,以期早期诊治。%Objective To analyze the clinical features of Evans syndrome in systemic lupus erythematosus (SLE) patients.Methods Clinical and laboratory manifestations occurring during the disease course,as well as concomitant diseases,treatment and outcomes was carefully reviewed.Results There were 22 SLE patients with Evans Syndrome accounted for 0.65% of the 3 400 hospitalized SLE patients.Nineteen were female and 3 were male,with the mean age of 35.1 year.Evans syndrome was diagnosed prior to SLE in 11 patients,after SLE in 6 patients

  1. Renal cell apoptosis in human lupus nephritis: a histological study

    DEFF Research Database (Denmark)

    Faurschou, M; Penkowa, Milena; Andersen, C B

    2009-01-01

    Nuclear autoantigens from apoptotic cells are believed to drive the immunological response in systemic lupus erythematosus (SLE). Conflicting data exist as to the possible renal origin of apoptotic cells in SLE patients with nephritis. We assessed the level of renal cell apoptosis in kidney...... biopsies from 35 patients with lupus nephritis by means of terminal deoxynucleotidyl-transferase (TdT)-mediated deoxyuridine triphosphate (dUTP)-digoxigenin nick end labeling (TUNEL). Five samples of normal kidney tissue served as control specimens. We did not observe apoptotic glomerular cells in any...... cells constitute a quantitatively important source of auto-antibody-inducing nuclear auto-antigens in human lupus nephritis....

  2. Lupus nephritis: induction therapy in severe lupus nephritis--should MMF be considered the drug of choice?

    Science.gov (United States)

    Rovin, Brad H; Parikh, Samir V; Hebert, Lee A; Chan, Tak Mao; Mok, Chi Chiu; Ginzler, Ellen M; Hooi, Lai Seong; Brunetta, Paul; Maciuca, Romeo; Solomons, Neil

    2013-01-01

    Severe lupus nephritis is an aggressive disease that requires an aggressive approach to treatment. Recent randomized clinical trials showed that mycophenolate mofetil compared favorably with cyclophosphamide (traditional approach) for remission induction. Consequently, mycophenolate mofetil is now commonly recommended as first-line therapy. Nevertheless, the role of mycophenolate mofetil in treating severe lupus nephritis is unclear, because such patients were excluded from these trials. With this limitation as background, this work addresses the question of mycophenolate mofetil for induction therapy for severe lupus nephritis. We performed a systematic review of the outcomes of treating severe lupus nephritis with mycophenolate mofetil or cyclophosphamide. Because no studies directly addressed this question, these data were extracted from the published literature or obtained by personal communications from investigators. There is no universally accepted definition, and therefore, severe lupus nephritis was arbitrarily defined by renal histology, resistance to therapy, or level of kidney function at presentation. For each trial analyzed, we determined the partial and complete remission rates. Long-term outcomes were compared when available. The pooled results suggest that mycophenolate mofetil and cyclophosphamide are equally effective in inducing remission of severe lupus nephritis. However, relapse rates and risk of developing ESRD were higher for mycophenolate mofetil compared with cyclophosphamide. In conclusion, in the short term, mycophenolate mofetil and cyclophosphamide are about equal in inducing remission. However, long-term outcomes suggest better preservation of kidney function and fewer relapses with cyclophosphamide therapy. Therefore, mycophenolate mofetil should not yet be considered the induction drug of choice for severe lupus nephritis.

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

    Science.gov (United States)

    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

  4. Taurine inhibits ischemia/reperfusion-induced compartment syndrome in rabbits

    Institute of Scientific and Technical Information of China (English)

    Ji-xian WANG; Yan LI; Li-ke ZHANG; Jing ZHAO; Yong-zheng PANG; Chao-shu TANG; Jing ZHANG

    2005-01-01

    Aim: To investigate effects of taurine on ischemia/reperfusion (I/R)-induced compartment syndrome in rabbit hind limbs.Methods: Rabbits underwent femoral artery occ lusion after ligation of branches from terminal aorta to femoral artery.After a 7-h ischemia, reperfusion was established with the use of heparinized by iv infusion 10 min before shunt placement.During reperfusion, anterior compartment pressure (ACP) was monitored continuously in the left lower extremity.Gastrocnemius muscle triphenyltetrazolium chloride (TTC) level, taurine content and myeloperoxidase activity were assayed.Oxidative stress was induced in the in vitro gastrocnemius muscle slices by free radical generating systems (FRGS),and the malondialdehyde content was measured in presence or absence of taurine.Results: After 7 h of ischemia, none of the parameters that we measured were different from those before ischemia, except that TTC reduction decreased by 80%.In the control group, after 2 h of reperfusion, ACP increased 4.5-fold, and gastrocnemius muscle taurine content was reduced by 33%.In taurine-treated animals, at 2 h reperfusion, the mean arterial blood pressure and heart rate were increased, by 6% and 10%.ACP decreased by 39%, muscle edema decreased by 16%, TTC reduction increased by 150%, and lactate dehydrogenase decreased by 36% compared to control group.Plasma and muscle taurine content increased by 70% and 88%, respectively.In the taurine-treated group, at 2 h reperfusion, plasma malondialdehyde and conjugated diene content were decreased by 38% and 23%,respectively, and muscle malondialdehyde and conjugated diene content decreased by 22% and 30%, respectively compared to the control group.At 2 h reperfusion,myeloperoxidase activity was increased 3.5-fold in control animals.In the in vitro study, taurine decreased malondialdehyde content in muscle slices incubated with hypochlorous acid in a dose-dependent manner, but there was no change when incubated with hydrogen peroxide and

  5. Síndrome de ativação macrofágica em paciente com lúpus eritematoso sistêmico juvenil Macrophage activation syndrome in a patient with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Simone Manso de Carvalho

    2008-08-01

    Full Text Available A hemofagocitose reativa ou síndrome de ativação macrofágica (SAM é uma complicação das doenças inflamatórias sistêmicas, causada por expansão de células T e macrófagos, com produção maciça de citocinas pró-inflamatórias, ocorrendo mais freqüentemente na artrite idiopática juvenil sistêmica e raramente no lúpus eritematoso sistêmico juvenil (LESJ. OBJETIVO: Relatar um caso de LESJ que evoluiu com SAM precipitada por infecção e infarto esplênico, com desfecho fatal. RELATO DE CASO: Uma menina de 7 anos, com diagnóstico de LESJ desde os 5 anos, evoluiu com artrite em atividade, alopecia intensa, citopenias, cefaléia, infecções respiratórias recorrentes e elevação intermitente de transaminases. Os anticorpos anti-DNA e anticardiolipina IgG e IgM foram identificados e a biópsia renal evidenciou glomerulonefrite lúpica de classe III. A paciente foi tratada com pulso de metilprednisolona, prednisona, azatioprina e hidroxicloroquina. Após dois anos, na vigência de pneumonia apresentou abdome agudo e convulsões, evoluindo para o choque hemorrágico fatal após esplenectomia, que evidenciou infarto esplênico e infiltração maciça por macrófagos hemofagocíticos CD163+. CONCLUSÃO: A revisão do desfecho sugere a SAM precipitada por infecção e sobreposta a atividade inflamatória do lúpus com febre persistente, citopenias, disfunção hepática, hepatomegalia e esplenomegalia, como efeitos do excesso de produção de citocinas. Os anticorpos anticardiolipina podem ter tido papel precipitante na coagulopatia, que resultou infarto esplênico e choque hemorrágico.Reactive haemophagocytosis or macrophage activation syndrome (MAS is a complication of systemic inflammatory disorders, caused by expansion of T cells and haemophagocytic macrophages, with cytokine overproduction. It has been described most often in systemic juvenile idiopathic arthritis and rarely in juvenile systemic lupus erythematosus (JSLE

  6. Involvement of central serotonergic systems in dextromethorphan-induced behavioural syndrome in rats.

    Science.gov (United States)

    Gaikwad, R V; Gaonkar, R K; Jadhav, S A; Thorat, V M; Jadhav, J H; Balsara, J J

    2005-07-01

    Dextromethorphan, a noncompetitive blocker of the N-methyl-D-aspartate (NMDA) type of glutamate receptor, at 45, 60 and 75 mg/kg, ip doses induced a behavioural syndrome characterised by reciprocal forepaw treading, lateral head-weaving, hind-limb abduction and flat body posture. Such type of behavioural syndrome is induced by 8-hydroxy-2- (di-n-propylamino) tetralin (8-OH-DPAT) by directly stimulating the central postsynaptic 5-hydroxytryptamine (5-HT, serotonin) receptors of the 5-HT1A type. Pretreatment with buspirone (5, 10 mg/kg, ip) and l-propranolol (10, 20 mg/kg, ip) antagonised the behavioural syndrome induced by 8-OH-DPAT and dextromethorphan. Pretreatment with p-chlorophenylalanine (100 mg/kg/day x 4 days) antagonised the behavioural syndrome induced by dextromethorphan and dexfenfluramine but had no significant effect on 8-OH-DPAT induced behavioural syndrome. This indicates that dextromethorphan induces the behavioural syndrome by releasing 5-HT from serotonergic neurons with resultant activation of the postsynaptic 5-HT1A receptors by the released 5-HT. Pretreatment with fluoxetine (10 mg/kg, ip) significantly potentiated the behavioural syndrome induced by dextromethorphan and 5-hydroxytryptophan but significantly antagonised dexfenfluramine induced behavioural syndrome. This indicates that dextromethorphan releases 5-HT by a mechanism which differs from that of dexfenfluramine. Dextromethorphan may be releasing 5-HT by blocking the NMDA receptors and thereby counteracting the inhibitory influence of l-glutamate on 5-HT release.

  7. Lupus anticoagulants and antiphospholipid antibodies

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000547.htm Lupus anticoagulants and antiphospholipid antibodies To use the sharing features on this page, please enable JavaScript. Lupus anticoagulants are antibodies against substances in the lining ...

  8. Diet and Nutrition With Lupus

    Science.gov (United States)

    ... on Twitter Facebook Pinterest Email Print Diet and nutrition with lupus Lupus Foundation of America March 16, ... Recipes Get email updates Related Resources ABCs of nutrition U.S. English español Medically reviewed on May 28, ...

  9. Lúpus induzido por drogas: da imunologia básica à aplicada Drug-induced lupus: from basic to spplied immunology

    Directory of Open Access Journals (Sweden)

    Licia Maria Henrique da Mota

    2007-12-01

    Full Text Available O lúpus induzido por drogas (LID é descrito como o desenvolvimento de sintomas semelhantes ao do lúpus eritematoso sistêmico idiopático, temporalmente relacionado à exposição a drogas, havendo, comumente, a resolução do quadro com a suspensão do medicamento desencadeante. A associação mais clássica é feita com a procainamida e a hidralazina. Recentemente, com a introdução de novas drogas na prática clínica, tem sido relatado um aumento no número de medicamentos implicados como causadores da doença, e a lista atual inclui quase uma centena de drogas relacionadas à ocorrência de LID. Embora descrito há mais de 60 anos, o mecanismo imunológico básico do LID ainda não está totalmente compreendido. Há várias hipóteses para o processo de indução de auto-imunidade pelas drogas, e o fenômeno geralmente é interpretado como uma inapropriada ativação do sistema imunitário. Entre as diversas teorias propostas, as mais aceitas são: a inibição da metilação do ácido desoxirribonucléico (DNA por algumas drogas, o que permitiria a ativação das células T; a oxidação de certas substâncias pelos monócitos, gerando metabólitos ativos que ocasionariam ativação das células apresentadoras de antígenos e/ou a interferência dos metabólitos de determinadas drogas com a tolerância do sistema imune. Novos estudos são necessários para a melhor compreensão da imunopatogenia do LID, objetivando desenvolver tratamentos específicos com base no melhor conhecimento dos mecanismos patogênicos.Drug-induced lupus (DIL has been described as the development of idiopathic systemic lupus erythematous-like symptoms, temporarily associated to the exposition to drugs, and as a rule, the condition is improved with the suspension of the triggering medication. The most classical association is with procainamide and hydralazine. Recently, with the introduction of new drugs in the clinical practice, an increase on the number of

  10. Stimulation of T cells up-regulates expression of Ifi202, an interferon-inducible lupus susceptibility gene, through activation of JNK/c-Jun pathway

    Science.gov (United States)

    Chen, Jianming; Panchanathan, Ravichandran; Choubey, Divaker

    2008-01-01

    Studies have revealed that increased expression of interferon (IFN)-inducible Ifi202 gene (encoding p202 protein) in splenic B and T cells from B6.Nba2 congenic (congenic for Nb2 locus derived from NZB mice) female mice is associated with lupus susceptibility. However, signaling pathways that regulate Ifi202 expression in immune cells remain to be elucidated. Here we report that stimulation of T cells up-regulates the Ifi202 expression. We found that steady-state levels of Ifi202 mRNA and protein were detectable in splenic T cells from NZB mice and stimulation of T cells with anti-CD3 and anti-CD28 up-regulated expression of the Ifi202 gene. Similarly, stimulation of cells of a mouse T-cell hybridoma cell line (2B4.11) also activated transcription of the Ifi202 gene. Significantly, up-regulation of Ifi202 expression in stimulated T cells was inhibited by treatment of cells with SP600125, a specific inhibitor of c-Jun N-terminal kinase (JNK). Conversely, treatment of cells with anisomycin, a potent activator of the JNK and c-Jun, up-regulated Ifi202 expression. Consistent with the activation of JNK/c-Jun pathway by T cell stimulation, forced expression of c-Jun in 2B4 T-cells and in mouse embryonic fibroblasts (MEFs) also up-regulated the Ifi202 expression. Furthermore, we found that stimulation of T cells increased association of the activated c-Jun to the 5′-regulatory region of the Ifi202 gene in chromatin immunoprecipitation assays (ChIPs). Together, our observations demonstrate that stimulation of T cells up-regulates the Ifi202 expression in part through the JNK/c-Jun pathway. PMID:18374989

  11. Neuroleptic-induced deficit syndrome in bipolar disorder with psychosis

    Directory of Open Access Journals (Sweden)

    Ueda S

    2016-02-01

    Full Text Available Satoshi Ueda,1 Takeshi Sakayori,1 Ataru Omori,2 Hajime Fukuta,3 Takashi Kobayashi,3 Kousuke Ishizaka,1 Tomoyuki Saijo,4 Yoshiro Okubo1 1Department of Neuropsychiatry, Nippon Medical School, Tokyo, Japan; 2Tamachuo Hospital, Tokyo, Japan; 3Kurumegaoka Hospital, Tokyo, Japan; 4Saijo Clinic, Tokyo, Japan Abstract: Neuroleptics can induce not only physical adverse effects but also mental effects that produce deficit status in thought, affect, cognition, and behavior. This condition is known as neuroleptic-induced deficit syndrome (NIDS, which includes apathy, lack of initiative, anhedonia, indifference, blunted affect, and reduced insight into disease. Although this old concept now appears almost forgotten, neuroleptics, whether typical or atypical, can make depression or bipolar disorder resemble other more refractory conditions, readily leading to mistaken diagnosis and inappropriate treatment. The authors describe three cases of NIDS superimposed on depressive phase in bipolar disorder with psychosis, where the attending psychiatrist’s failure to recognize NIDS prevented patients from receiving effective treatment and achieving remission. All cases achieved remission after reduction of neuroleptics and intensive therapy, including electroconvulsive therapy, for bipolar depression. The concept of NIDS was originally introduced for schizophrenia, and it has rarely been highlighted in other diseases. In recent years, however, atypical antipsychotics are being more often administered to patients with bipolar disorder. Psychiatrists, therefore, should also remember and exercise caution regarding NIDS in the pharmacotherapy of bipolar disorder with and without psychosis. The authors believe that the concept of NIDS needs to be reappraised in current psychiatry. Keywords: neuroleptic-induced deficit syndrome (NIDS, bipolar disorder, psychosis, atypical antipsychotics, electroconvulsive therapy

  12. Lupus vulgaris of external nose.

    Science.gov (United States)

    Bhandary, Satheesh Kumar; Ranganna, B Usha

    2008-12-01

    Lupus vulgaris is the commonest form of cutaneous tuberculosis which commonly involve trunk and buttocks. Lupus vulgaris affecting nose and face, are rarely reported in India. This study reports an unusual case of lupus vulgaris involving the external nose that showed dramatic outcome after six months of anti- tubercular treatment.

  13. Lupus vulgaris: difficulties in diagnosis.

    Science.gov (United States)

    Rhodes, Julia; Caccetta, Tony Philip; Tait, Clare

    2013-05-01

    Lupus vulgaris is one of the most common forms of cutaneous tuberculosis. It presents a diagnostic challenge due to its paucibacillary nature. This is a report of a case of a delayed diagnosis of lupus vulgaris, presenting as perianal and peristomal plaques, followed by a review of the diagnostic tools for lupus vulgaris and their limitations.

  14. Guillain-Barré syndrome- and Miller Fisher syndrome-associated Campylobacter jejuni lipopolysaccharides induce anti-GM1 and anti-GQ1b Antibodies in rabbits.

    NARCIS (Netherlands)

    M.A. de Klerk; H.P. Endtz (Hubert); B.C. Jacobs (Bart); J.D. Laman (Jon); F.G.A. van der Meché (Frans); P.A. van Doorn (Pieter); C.W. Ang (Wim)

    2001-01-01

    textabstractCampylobacter jejuni infections are thought to induce antiganglioside antibodies in patients with Guillain-Barre syndrome (GBS) and Miller Fisher syndrome (MFS) by molecular mimicry between C. jejuni lipopolysaccharides (LPS) and gangliosides. We used purifi

  15. Guillain-Barré syndrome- and Miller Fisher syndrome-associated Campylobacter jejuni lipopolysaccharides induce anti-GM1 and anti-GQ1b Antibodies in rabbits.

    NARCIS (Netherlands)

    M.A. de Klerk; H.P. Endtz (Hubert); B.C. Jacobs (Bart); J.D. Laman (Jon); F.G.A. van der Meché (Frans); P.A. van Doorn (Pieter); C.W. Ang (Wim)

    2001-01-01

    textabstractCampylobacter jejuni infections are thought to induce antiganglioside antibodies in patients with Guillain-Barre syndrome (GBS) and Miller Fisher syndrome (MFS) by molecular mimicry between C. jejuni lipopolysaccharides (LPS) and gangliosides. We used

  16. Methylglyoxal induces systemic symptoms of irritable bowel syndrome.

    Science.gov (United States)

    Zhang, Shuang; Jiao, Taiwei; Chen, Yushuai; Gao, Nan; Zhang, Lili; Jiang, Min

    2014-01-01

    Patients with irritable bowel syndrome (IBS) show a wide range of symptoms including diarrhea, abdominal pain, changes in bowel habits, nausea, vomiting, headache, anxiety, depression and cognitive impairment. Methylglyoxal has been proved to be a potential toxic metabolite produced by intestinal bacteria. The present study was aimed at investigating the correlation between methylglyoxal and irritable bowel syndrome. Rats were treated with an enema infusion of methylglyoxal. Fecal water content, visceral sensitivity, behavioral tests and serum 5-hydroxytryptamine (5-HT) were assessed after methylglyoxal exposure. Our data showed that fecal water content was significantly higher than controls after methylglyoxal exposure except that of 30 mM group. Threshold volumes on balloon distension decreased in the treatment groups. All exposed rats showed obvious head scratching and grooming behavior and a decrease in sucrose preference. The serum 5-HT values were increased in 30, 60, 90 mM groups and decreased in 150 mM group. Our findings suggested that methylglyoxal could induce diarrhea, visceral hypersensitivity, headache as well as depression-like behaviors in rats, and might be the key role in triggering systemic symptoms of IBS.

  17. Serotonin Syndrome Induced by Fentanyl in a Child: Case Report.

    Science.gov (United States)

    Robles, Luis A

    2015-01-01

    Serotonin syndrome (SS) is a potentially fatal condition associated with increased serotonergic activity in the central nervous system that can be attributed to certain drugs or interactions between drugs. There are some published articles reporting this syndrome caused by the combination of fentanyl and selective serotonin reuptake inhibitors antidepressants in adult patients; however, there are no reports of SS associated to the use of fentanyl as a single causative agent. The author reports a case of a 7-year-old boy who was admitted to the emergency department with neurological deterioration secondary to an intracerebral hemorrhage. The patient was operated to remove the bleeding. Postoperatively, he experienced a diversity of progressive neurological signs (shivering, tremor, hypertonia, hyperreflexia, clonus, bilateral mydriasis, and intracranial hypertension), which were initially considered to be signs of neurological deterioration, but finally, it was proved that they were part of a SS caused by fentanyl.The absence of concomitant use of another medications known to induce SS and the dramatic improving observed after stopping fentanyl strongly indicates that fentanyl was the causative agent in this case of SS.Fentanyl is a medication used frequently, and therefore, clinicians should be aware of this potential adverse effect when this drug is administered.

  18. Radiation-induced neuroinflammation and radiation somnolence syndrome.

    Science.gov (United States)

    Ballesteros-Zebadúa, Paola; Chavarria, Anahi; Celis, Miguel Angel; Paz, Carlos; Franco-Pérez, Javier

    2012-11-01

    Cranial irradiation remains a standard treatment for malignant and benign brain diseases. Although this procedure helps to lengthen the life expectancy of the patient, the appearance of adverse effects related to radiation-induced injury is inevitable. Radiation somnolence syndrome (RSS) has been described as a delayed effect observed mainly after whole-brain radiotherapy in children. The RSS was first linked to demyelination, but more recently it has been proposed that the inflammatory response plays a primary role in the aforementioned syndrome. To evaluate the feasibility of this hypothesis, we explored previous work about RSS and reviewed published research that included measurements of the inflammatory response in models of brain exposure to ionizing radiation. Pro-inflammatory cytokines such as interleukin-1β, tumor necrosis factor-α, interleukin-6 and interleukin-18 as well as other inflammatory markers such as cyclooxygenase-2, prostaglandin E₂, glial fibrillary acid protein, intercellular adhesion molecule-1 and nuclear factor-κB appear to be involved in the brain's response to radiation. However, certain publications have described the somnogenic effects of these cytokines and inflammatory markers. Although the radiation response is a complex phenomenon that involves several molecular and cellular processes, we propose that inflammation may be closely related to the adverse effects of brain irradiation and therefore to the etiology of RSS.

  19. Food protein-induced enterocolitis syndrome, from practice to theory.

    Science.gov (United States)

    Miceli Sopo, Stefano; Greco, Monica; Monaco, Serena; Tripodi, Salvatore; Calvani, Mauro

    2013-08-01

    Food protein-induced enterocolitis syndrome (FPIES) is an allergic disease, probably non-IgE-mediated, with expression predominantly in the GI tract. The most characteristic symptom is repeated, debilitating vomiting. It occurs 2-6 h after ingestion of culprit food and is usually accompanied by pallor and lethargy. There may be diarrhea, and in 10-20% of cases, severe hypotension. These symptoms resolve completely within a few hours. The food most frequently involved is cow's milk, followed by rice, but many other foods may be involved. The prognosis is generally good in a few years. In this review the authors try to cope, with the help of some case histories, with the practical clinical aspects of FPIES. The authors also try to provide a management approach based on current knowledge, and finally, to point out the aspects of FPIES that are still controversial.

  20. Aceclofenac induced Stevens-Johnson/toxic epidermal necrolysis overlap syndrome

    Directory of Open Access Journals (Sweden)

    Kaderthambi Hajamohideen Nooru Ameen

    2013-01-01

    Full Text Available The purpose of this paper is to report a rare occurrence of Stevens-Johnson/Toxic epidermal necrolysis (SJS/TEN overlap syndrome after the use of aceclofenac. A 38 year old healthy adult male presented with rapidly evolving rash over face and upper body with ulceration of buccal mucosa and breathlessness after taking aceclofenac tablet. Naranjo score for this adverse drug event was six, thereby making it a probable adverse drug reaction. Despite aggressive fluid resuscitation and use of antihistamines and systemic steroids, patient′s health rapidly worsened and died within six hours of presentation. Aceclofenac induced SJS/TEN overlap is an extremely rare clinical association previously reported only once in medical literature. To the best of our knowledge, this is the first case report of such an association in the Indian population. We are presenting this case to highlight the serious adverse reactions possible from a routinely prescribed drug.

  1. Nasogastric tube syndrome induced by an indwelling long intestinal tube.

    Science.gov (United States)

    Sano, Naoki; Yamamoto, Masayoshi; Nagai, Kentaro; Yamada, Keiichi; Ohkohchi, Nobuhiro

    2016-04-21

    The nasogastric tube (NGT) has become a frequently used device to alleviate gastrointestinal symptoms. Nasogastric tube syndrome (NTS) is an uncommon but potentially life-threatening complication of an indwelling NGT. NTS is characterized by acute upper airway obstruction due to bilateral vocal cord paralysis. We report a case of a 76-year-old man with NTS, induced by an indwelling long intestinal tube. He was admitted to our hospital for treatment of sigmoid colon cancer. He underwent sigmoidectomy to release a bowel obstruction, and had a long intestinal tube inserted to decompress the intestinal tract. He presented acute dyspnea following prolonged intestinal intubation, and bronchoscopy showed bilateral vocal cord paralysis. The NGT was removed immediately, and tracheotomy was performed. The patient was finally discharged in a fully recovered state. NTS be considered in patients complaining of acute upper airway obstruction, not only with a NGT inserted but also with a long intestinal tube.

  2. Tenofovir induced Fanconi syndrome: A possible pharmacokinetic interaction

    Directory of Open Access Journals (Sweden)

    Jigar Kapadia

    2013-01-01

    Full Text Available Tenofovir was introduced as a second line drug for the treatment of human immunodeficiency virus (HIV infection in India in December 2009. Although rare, renal toxicity is a recognized adverse drug reaction (ADR of this drug, especially when administered with boosted lopinavir-ritonavir. In this case, an HIV positive patient receiving tenofovir based antiretroviral therapy (ART for last 1 year developed albuminuria, glycosuria and hypophosphatemia. Renal function tests and random blood sugar were within normal limits. He was diagnosed as a case of tenofovir induced Fanconi syndrome. Tenofovir was discontinued and patient was prescribed an alternate regimen. Five months later clinical symptoms and renal functions returned to normal. A pharmacokinetic interaction between tenofovir and ritonavir may have resulted in the toxicity. A periodic monitoring of renal functions is desirable in patients on tenofovir based ART.

  3. Paracetamol induced Steven-Johnson syndrome: A rare case report

    Directory of Open Access Journals (Sweden)

    Rajan Rajput

    2015-01-01

    Full Text Available In the contemporary era, use of drugs is the dominant paradigm of health care. The most quotidian drug used for fever and pain is paracetamol. Although adverse reactions to paracetamol in India are rare, at times they can cause life-threatening situations. Stevens-Johnson syndrome (SJS is one such potentially lethal adverse drug reaction. The most reported cases of analgesic-induced SJS were due to oxicams or propionic acid derivatives. There are very few detailed reports of SJS due to the use of paracetamol. We report a case of SJS, which occurred due to the use of paracetamol. The clinical features of this condition and multidisciplinary management of the patient are described in brief.

  4. Abacavir-induced reversible Fanconi syndrome with nephrogenic diabetes insipidus in a patient with acquired immunodeficiency syndrome

    Directory of Open Access Journals (Sweden)

    Ahmad M

    2006-01-01

    Full Text Available There are several reports of Fanconi syndrome (FS with or without nephrogenic diabetes insipidus (NDI in patients with human immunodeficiency virus (HIV infection, treated with various antiretroviral medications like cidofovir, adefovir, didenosine and tenofovir. But neither FS nor NDI has been documented with abacavir therapy. We are reporting the first case of abacavir-induced reversible FS with NDI in a patient with acquired immunodeficiency syndrome, who recovered completely with supportive treatment and discontinuation of abacavir.

  5. Evidence Refuting the Existence of Autoimmune/Autoinflammatory Syndrome Induced by Adjuvants (ASIA)

    DEFF Research Database (Denmark)

    Ameratunga, Rohan; Gillis, David; Gold, Michael

    2017-01-01

    Autoimmune/autoinflammatory syndrome induced by adjuvants (ASIA) was described in 2011. Over time the condition and its triggers have broadened to include several autoimmune disorders, the macrophagic myofasciitis syndrome, the Gulf war syndrome, the sick building syndrome, siliconosis......, and the chronic fatigue syndrome. The aluminum-containing adjuvants in the hepatitis B vaccine and the human papillomavirus vaccine in particular have been stated to be the major causes of the disorder. Here, we review the specificity of the diagnostic criteria for ASIA. We also examine relevant human data...

  6. Oxcarbazepine-induced drug rash with eosinophilia and systemic symptoms syndrome presenting as exfoliative dermatitis

    Directory of Open Access Journals (Sweden)

    Mahimanjan Saha

    2016-01-01

    Full Text Available Drug rash with eosinophilia and systemic symptoms (DRESS syndrome is a type of severe adverse cutaneous drug reaction characterized by fever, skin eruption, hematological abnormalities, and internal organ involvement. Although anticonvulsant drugs are mainly implicated in DRESS, newer anticonvulsants such as oxcarbazepine-induced definite cases of DRESS syndrome are rare and oxcarbazepine-induced DRESS syndrome presenting as exfoliative dermatitis is even rarer. We report a case of a 35-year-old male who developed DRESS syndrome presenting as exfoliative dermatitis after taking oxcarbazepine for 3 weeks.

  7. Oxcarbazepine-induced drug rash with eosinophilia and systemic symptoms syndrome presenting as exfoliative dermatitis.

    Science.gov (United States)

    Saha, Mahimanjan; Gorai, Surajit; Madhab, Vaswatee

    2016-01-01

    Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a type of severe adverse cutaneous drug reaction characterized by fever, skin eruption, hematological abnormalities, and internal organ involvement. Although anticonvulsant drugs are mainly implicated in DRESS, newer anticonvulsants such as oxcarbazepine-induced definite cases of DRESS syndrome are rare and oxcarbazepine-induced DRESS syndrome presenting as exfoliative dermatitis is even rarer. We report a case of a 35-year-old male who developed DRESS syndrome presenting as exfoliative dermatitis after taking oxcarbazepine for 3 weeks.

  8. High incidence of potentially virus-induced malignancies in systemic lupus erythematosus: a long-term followup study in a Danish cohort

    DEFF Research Database (Denmark)

    Dreyer, Lene; Faurschou, Mikkel; Mogensen, Mette

    2011-01-01

    Patients with systemic lupus erythematosus (SLE) seem to experience an increased prevalence of oncogenic virus infections. The aim of the present study was to investigate whether SLE patients have an increased risk of virus-associated malignancies, defined as malignancies potentially caused...

  9. Neuropsychiatric Systemic Lupus Erythematosus

    Science.gov (United States)

    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

  10. RHEUMATIC MASKS OF PARANEOPLASTIC SYNDROME

    Directory of Open Access Journals (Sweden)

    A. P. Rebrov

    2001-01-01

    Full Text Available Summary Analysis of the prevalence and clinical manifestations of paraneoplastic syndrome in 173 patients with malignant tumors admitted in departments internal medicine of Regional Clinical hospital was done. Paraneoplastic syndromes was found in 13 patients (7% and was characterized by the following rheumatic manifestations: articular syndrome, dermato- and polymyositis, lupus-like syndrome.

  11. Food protein induced enterocolitis syndrome caused by rice beverage.

    Science.gov (United States)

    Caminiti, Lucia; Salzano, Giuseppina; Crisafulli, Giuseppe; Porcaro, Federica; Pajno, Giovanni Battista

    2013-05-14

    Food protein-induced enterocolitis syndrome (FPIES) is an uncommon and potentially severe non IgE-mediated gastrointestinal food allergy. It is usually caused by cow's milk or soy proteins, but may also be triggered by ingestion of solid foods. The diagnosis is made on the basis of clinical history and symptoms. Management of acute phase requires fluid resuscitation and intravenous steroids administration, but avoidance of offending foods is the only effective therapeutic option.Infant with FPIES presented to our emergency department with vomiting, watery stools, hypothension and metabolic acidosis after ingestion of rice beverage. Intravenous fluids and steroids were administered with good clinical response. Subsequently, a double blind placebo control food challenge (DBPCFC) was performed using rice beverage and hydrolyzed formula (eHF) as placebo. The "rice based formula" induced emesis, diarrhoea and lethargy. Laboratory investigations reveal an increase of absolute count of neutrophils and the presence of faecal eosinophils. The patient was treated with both intravenous hydration and steroids. According to Powell criteria, oral food challenge was considered positive and diagnosis of FPIES induced by rice beverage was made. Patient was discharged at home with the indication to avoid rice and any rice beverage as well as to reintroduce hydrolyzed formula. A case of FPIES induced by rice beverage has never been reported. The present case clearly shows that also beverage containing rice proteins can be responsible of FPIES. For this reason, the use of rice beverage as cow's milk substitute for the treatment of non IgE-mediated food allergy should be avoided.

  12. Psoriasiform lupus vulgaris.

    Science.gov (United States)

    Padmavathy, L; Rao, L Lakshmana; Ethirajan, N; Dhanlaklshmi, M

    2008-04-01

    Tuberculosis is a major public health problem in both developing and developed countries. Cutaneous Tuberculosis constitutes a minor proportion of extra-pulmonary manifestations of Tuberculosis. Lupus Vulgaris (LV) is one of the clinical variants of Cutaneous Tuberculosis. A case of a large plaque type psoriasiform lesion of lupus vulgaris on the thigh, of 15 years' duration, in an 18-year-old girl is reported. This case highlights the ignorance level among the patients and consequent failure to avail proper anti-tuberculous treatment despite campaign in print and audio visual media.

  13. Histopathological changes in exocrine glands of murine transplantation chimeras. II: Sjögren's syndrome-like exocrinopathy in mice without lupus nephritis. A model of primary Sjögren's syndrome

    DEFF Research Database (Denmark)

    Ussing, Anne Phaff; Prause, J.U.; Sørensen, Inger

    1992-01-01

    Autoimmune disease, primary Sjögren's syndrome, transplantation chimeras, experimental model, exocrinopathy, inbred mouse strains......Autoimmune disease, primary Sjögren's syndrome, transplantation chimeras, experimental model, exocrinopathy, inbred mouse strains...

  14. Glutamate receptor antibodies in neurological diseases: anti-AMPA-GluR3 antibodies, anti-NMDA-NR1 antibodies, anti-NMDA-NR2A/B antibodies, anti-mGluR1 antibodies or anti-mGluR5 antibodies are present in subpopulations of patients with either: epilepsy, encephalitis, cerebellar ataxia, systemic lupus erythematosus (SLE) and neuropsychiatric SLE, Sjogren's syndrome, schizophrenia, mania or stroke. These autoimmune anti-glutamate receptor antibodies can bind neurons in few brain regions, activate glutamate receptors, decrease glutamate receptor's expression, impair glutamate-induced signaling and function, activate blood brain barrier endothelial cells, kill neurons, damage the brain, induce behavioral/psychiatric/cognitive abnormalities and ataxia in animal models, and can be removed or silenced in some patients by immunotherapy.

    Science.gov (United States)

    Levite, Mia

    2014-08-01

    pathological effects: they activate glutamate/AMPA receptors, kill neurons by 'Excitotoxicity', and/or by complement activation modulated by complement regulatory proteins, cause multiple brain damage, aggravate chemoconvulsant-induced seizures, and also induce behavioral/motor impairments. Some patients with 'Autoimmune Epilepsy' that have anti-AMPA-GluR3B antibodies respond well (although sometimes transiently) to immunotherapy, and thanks to that have reduced seizures and overall improved neurological functions. (2) Anti-NMDA-NR1 antibodies are present in patients with autoimmune 'Anti-NMDA-receptor Encephalitis'. In humans, in animal models and in vitro the anti-NMDA-NR1 antibodies can be very pathogenic since they can cause a pronounced decrease of surface NMDA receptors expressed in hippocampal neurons, and also decrease the cluster density and synaptic localization of the NMDA receptors. The anti-NMDA-NR1 antibodies induce these effects by crosslinking and internalization of the NMDA receptors. Such changes can impair glutamate signaling via the NMDA receptors and lead to various neuronal/behavior/cognitive/psychiatric abnormalities. Anti-NMDA-NR1 antibodies are frequently present in high levels in the CSF of the patients with 'Anti-NMDA-receptor encephalitis' due to their intrathecal production. Many patients with 'Anti-NMDA receptor Encephalitis' respond well to several modes of immunotherapy. (3) Anti-NMDA-NR2A/B antibodies are present in a substantial number of patients with Systemic Lupus Erythematosus (SLE) with or without neuropsychiatric problems. The exact percentage of SLE patients having anti-NMDA-NR2A/B antibodies varies in different studies from 14 to 35%, and in one study such antibodies were found in 81% of patients with diffuse 'Neuropshychiatric SLE', and in 44% of patients with focal 'Neuropshychiatric SLE'. Anti-NMDA-NR2A/B antibodies are also present in subpopulations of patients with Epilepsy of several types, Encephalitis of several types (e

  15. Ectopic Axillary Breast during Systemic Lupus

    Directory of Open Access Journals (Sweden)

    Besma Ben Dhaou

    2012-01-01

    Full Text Available Many breast changes may occur in systemic lupus erythematosus. We report a 41-year-old woman with lupus who presented three years after the onset of lupus an ectopic mammary gland confirmed by histological study.

  16. Genetics Home Reference: systemic lupus erythematosus

    Science.gov (United States)

    ... 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 ...

  17. 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...

  18. Aim2-deficiency in mice suppresses the expression of the inhibitory Fcγ receptor (FcγRIIB) through the induction of the interferon-inducible p202, a lupus susceptibility protein

    Science.gov (United States)

    Panchanathan, Ravichandran; Shen, Hui; Duan, Xin; Rathinam, Vijay A. K.; Erickson, Loren D.; Fitzgerald, Katherine A.; Choubey, Divaker

    2011-01-01

    Murine Aim2 and Ifi202 genes (encoding for the Aim2 and p202 proteins) are members of the interferon (IFN)-inducible Ifi200-gene family. The Aim2-deficiency in mice activates IFN-signaling and stimulates the expression of lupus susceptibility gene, the Ifi202, located within the Nba2 interval. Given that the deficiency in the expression of the Fcgr2b gene (encoding for the inhibitory FcγRIIB receptor) is associated with increased lupus susceptibility in mice, we investigated whether the Aim2 protein could regulate the expression of Fcgr2b gene. Here we report that Aim2-deficiency in mice suppresses the expression of the FcγRIIB receptor. Interestingly, the Fcgr2b-deficient cells expressed increased levels of the IFN-β, activated IFN-signaling, and expressed reduced levels of the Aim2 protein. Treatment of splenic cells with IFN-α or γ reduced levels of the FcγRIIB mRNA and protein, and also decreased the activity of the FcγRIIB p(−729/+ 585) Luc reporter. Moreover, levels of the FcγRIIB receptor were significantly higher in the Stat1-deficient splenic cells than the wild type cells. Accordingly, increased expression of IFN-β in lupus-prone B6.Nba2-ABC mice, as compared with non lupus-prone B6 or B6.Nba2-C mice, was associated with reduced expression of the FcγRIIB receptor. Notably, overexpression of the p202 protein in cells decreased the expression of the Aim2 gene, activated the IFN-response, and suppressed the expression of the Fcgr2b gene. These observations demonstrate that the expression of Aim2 protein is required to maintain the expression of the Fcgr2b gene and also predict epistatic interactions between the Ifi200-genes and the Fcgr2b gene within the Nba2 interval. PMID:21551362

  19. Aim2 deficiency in mice suppresses the expression of the inhibitory Fcgamma receptor (FcgammaRIIB) through the induction of the IFN-inducible p202, a lupus susceptibility protein.

    Science.gov (United States)

    Panchanathan, Ravichandran; Shen, Hui; Duan, Xin; Rathinam, Vijay A K; Erickson, Loren D; Fitzgerald, Katherine A; Choubey, Divaker

    2011-06-15

    Murine Aim2 and Ifi202 genes (encoding for the Aim2 and p202 proteins) are members of the IFN-inducible Ifi200 gene family. The Aim2 deficiency in mice activates IFN signaling and stimulates the expression of the lupus susceptibility gene, the Ifi202, located within the NZB autoimmunity 2 (Nba2) interval. Given that the deficiency in the expression of the Fcgr2b gene (encoding for the inhibitory FcγRIIB receptor) is associated with increased lupus susceptibility in mice, we investigated whether the Aim2 protein could regulate the expression of Fcgr2b gene. In this article, we report that Aim2 deficiency in mice suppresses the expression of the FcγRIIB receptor. Interestingly, the Fcgr2b-deficient cells expressed increased levels of the IFN-β, activated IFN signaling, and expressed reduced levels of the Aim2 protein. Treatment of splenic cells with IFN-α or -γ reduced levels of the FcγRIIB mRNA and protein and also decreased the activity of the FcγRIIB p(-729/+585) Luc reporter. Moreover, levels of the FcγRIIB receptor were significantly higher in the Stat1-deficient splenic cells than in the wild-type cells. Accordingly, increased expression of IFN-β in lupus-prone B6.Nba2-ABC mice, as compared with non-lupus-prone C57BL/6 (B6) or B6.Nba2-C mice, was associated with reduced expression of the FcγRIIB receptor. Notably, overexpression of the p202 protein in cells decreased the expression of the Aim2 gene, activated the IFN response, and suppressed the expression of the Fcgr2b gene. These observations demonstrate that the expression of Aim2 protein is required to maintain the expression of the Fcgr2b gene and also predict epistatic interactions between the Ifi200 genes and the Fcgr2b gene within the Nba2 interval.

  20. Renal expression of polyomavirus large T antigen is associated with nephritis in human systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Fenton, Kristin Andreassen; Mjelle, Janne Erikke; Jacobsen, Søren

    2008-01-01

    ) that these complexes bound induced anti-nucleosome antibodies and finally (iv) that they associated with glomerular membranes as immune complexes. This process may be relevant for human lupus nephritis, since productive polyomavirus infection is associated with this organ manifestation. Here, we compare nephritis...... to the evolution of lupus nephritis in human SLE....

  1. Rapidly progressive lupus nephritis and concomitant thrombotic microangiopathy.

    Science.gov (United States)

    Gharbi, Chems; Bourry, Edward; Rouvier, Philippe; Hacini, Sabria; Letaief, Ahmed; Baumelou, Alain; Izzedine, Hassane

    2010-10-01

    Although uncommon, thrombotic microangiopathy (TMA) is one of the most serious complications in patients with systemic lupus erythematosus. A 30-year-old black woman admitted to our hospital because of fever, fatigue, 'dark' urine and rapidly progressive renal failure was found to have systemic lupus erythematous and atypical hemolytic uremic syndrome. Kidney biopsy showed WHO class IV lupus nephritis with crescents and TMA. Hemodialysis was initiated for worsening renal failure. The patient was treated with corticosteroids, monthly pulse intravenous Cyclophosphamide, plasmapheresis and Rituximab on a weekly basis for 4 weeks. The patient's blood pressure was aggressively controlled using antihypertensive agents. Despite this extensive therapy, she remained dialysis dependent although hematological parameters returned to normal values.

  2. 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.

  3. Pathological characteristics of lupus-like renal damages induced by exogenous double stranded DNA%外源性双链DNA诱导小鼠狼疮样肾损害的病理学特征

    Institute of Scientific and Technical Information of China (English)

    夏育民; 江珊; 杨红霞; 熊腊元

    2009-01-01

    目的 观察马疫锥虫双链DNA(dsDNA)诱导的小鼠狼疮样肾脏损害的病理学特征.方法 将分离纯化的马疫锥虫动基体DNA(kDNA)与不完全弗氏佐剂乳化混合,以皮下途径对健康BALB/c小鼠进行免疫.8周后,检测相关生化与免疫学指标并观察其肾脏病理学表现;同时与BXSB小鼠和抗dsDNA抗体阳性狼疮肾炎(LN)患者进行比较.结果 经马疫锥虫kDNA免疫后小鼠的免疫学指标符合LN的特征,肾脏损害以肾病综合征为主要表现;与BXSB小鼠和LN患者相比较,其肾脏病理改变有一定相似性,但以Ⅱ型(系膜细胞增殖)与Ⅳ型(弥漫性增殖)等类型为主.结论 马疫锥虫dsDNA诱导的小鼠肾脏损害与抗dsDNA抗体阳性的LN损害相似,可以作为研究该类型LN的有效工具.%Objective To investigate the pathological characteristics of lupus-like renal damages induced by double stranded DNA (dsDNA) derived from Trypanosoma Equiperdum (TE). Methods The TEs were propagated in normal rats and isolated from fresh rat blood by DEAE cellulose-chromatography. Their kinetoplast dsDNA (kDNA) was purified with Gibson's method. The emulsive mixture of kDNA and incomplete Freund's adjuvant (IFA) was injected into normal BALB/c mice subcutaneously. Eight weeks Later some parameters were examined, including sera titers of ANA and anti-dsDNA antibodies, 24h urine protein concentration, ESR, BUN, Scr and renal histological active index (AI). The pathological characteristics of renal tissues were observed under optical and electron microscopes, and then compared with that of BXSB mice and lupfis nephritis (LN) patients with positive anti-dsDNA antibodies in the sera. Results The results of all immunological parameters of TE kDNA-immunized mice corresponded with that of LN. Their renal damages mainly represented nephropathy syndrome. The pathological characteristics of these mice were similar to that of BXSB mice and LN patients, but Ⅱ (mesangial proliferative) and

  4. Challenge of liver disease in systemic lupus erythematosus: Clues for diagnosis and hints for pathogenesis

    Science.gov (United States)

    Bessone, Fernando; Poles, Natalia; Roma, Marcelo G

    2014-01-01

    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 of lupus (e.g., autoimmune hepatitis, primary biliary cirrhosis). The second group includes non-autoimmune liver diseases, such as esteatosis, hepatitis C, hypercoagulation state-related liver lesions, hyperplasic parenchymal and vascular lesions, porphyria cutanea tarda, and drug-induced hepatotoxicity. Finally, the data in the literature to support the existence of a hepatic disease produced by SLE itself, or the occurrence of a SLE-associated prone condition that increases susceptibility to acquire other liver diseases, is critically discussed. The pathological mechanisms underlying each of these liver disorders are also reviewed. Despite the high heterogeneity in the literature regarding the prevalence of SLE-associated liver diseases and, in most cases, lack of histopathological evidence or clinical studies large enough to support their existence, it is becoming increasingly apparent that liver is an important target of SLE. Consequently, biochemical liver tests should be routinely carried out in SLE patients to discard liver disorders, particularly in those patients chronically exposed to potentially hepatotoxic drugs. Diagnosing liver disease in SLE patients is always challenging, and the systematization of the current information carried out in this review is expected to be of help both to attain a better understanding of pathogenesis and to build an appropriate work-up for diagnosis. PMID:25018850

  5. Cardiac dysfunction in HgCl2-induced nephrotic syndrome.

    Science.gov (United States)

    Moreira-Rodrigues, Mónica; Henriques-Coelho, Tiago; Moura, Cláudia; Vasques-Nóvoa, Francisco; Sampaio-Maia, Benedita; Pestana, Manuel; Leite-Moreira, Adelino F

    2010-03-01

    The experimental model of HgCl(2) injection is characterized by a systemic autoimmune disease which leads to the development of nephrotic syndrome (NS). NS seems to be accompanied by cardiovascular alterations, since patients with NS present an increased incidence in cardiac disease. The aim of our work was to study the effects of HgCl(2)-induced NS on myocardial function and morphometry. Normotensive Brown-Norway rats were injected with HgCl(2) (1 mg/kg, HgCl(2) group; n = 6, subcutaneous) or the vehicle (control group; n = 6, subcutaneous) on days 0, 2, 4, 7, 9 and 11. The animals were placed in metabolic cages for evaluation of urinary excretion of noradrenaline, sodium, total proteins, albumin and creatinine. Fourteen and 21 days after the first HgCl(2) injection, left ventricle (LV) hemodynamics was evaluated through pressure micromanometers in basal and isovolumetric heartbeats. The heart and gastrocnemius muscle weights and tibial length were also examined. In an additional group of animals cardiac dimensions and ejection fraction were assessed by echocardiography and LV apoptosis and fibrosis were studied. HgCl(2)-injected rats presented proteinuria, albuminuria, hyperlipidemia, anemia, sodium retention and ascites at day 14. These alterations were accompanied by LV hemodynamic changes only in isovolumetric heartbeats. Similarly, on day 21, HgCl(2)-injected rats presented proteinuria, albuminuria, hyperlipidemia, anemia, but no sodium retention or ascites. These animals presented LV systolic and diastolic dysfunction in both basal and isovolumetric heartbeats, as well as cardiac atrophy, LV fibrosis and an increase in myocyte apoptosis. In conclusion, HgCl(2)-induced NS is accompanied by LV dysfunction and can be a promising model for studying the link between NS and cardiac disease.

  6. Contraception for adolescents with lupus

    Directory of Open Access Journals (Sweden)

    Wagner-Weiner Linda

    2010-03-01

    Full Text Available Abstract Sexually active adolescents, including young women with lupus, are at high risk for unplanned pregnancy. Unplanned pregnancy among teens with lupus is associated with an elevated risk of poor maternal and fetal outcomes. The provision of effective contraception is a crucial element of care for a sexually-active young woman with lupus. Unfortunately, providers may be hesitant to prescribe contraception to this group due to concerns about increasing the risk of lupus complications. This article reviews the risks and benefits of currently-available contraceptives for young women with lupus. Providers are encouraged to consider long-term, highly-effective contraception, such as implantables and intrauterine devices, for appropriately selected adolescents with lupus.

  7. 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.

  8. The clinical syndrome of bilirubin-induced neurologic dysfunction.

    Science.gov (United States)

    Bhutani, Vinod K; Johnson-Hamerman, Lois

    2015-02-01

    Clinicians have hypothesized a spectrum of minor neurologic manifestations, consistent with neuroanatomical reports and collectively termed as a "syndrome of bilirubin-induced neurologic dysfunction (BIND)," which can occur in the absence of classical kernicterus. The current review builds on these initial reports with a focus on clinical signs and symptoms that are assessed by standardized tools and manifest from neonatal age to childhood. These clinical manifestations are characterized by the following domains: (i) neuromotor signs; (ii) muscle tone abnormalities; (iii) hyperexcitable neonatal reflexes; (iv) variety of neurobehavior manifestations; (v) speech and language abnormalities; and (vi) evolving array of central processing abnormalities, such as sensorineural audiology and visuomotor dysfunctions. Concerns remain that the most vulnerable infants are likely to acquire BIND, either because their exposure to bilirubin is not identified as severe enough to need treatment or is prolonged but slightly below current threshold levels for intervention. Knowing that a total serum/plasma bilirubin (TB) level is not the most precise indicator of neurotoxicity, the role of expanded biomarkers or a "bilirubin panel" has yet to be validated in prospective studies. Future studies that correlate early "toxic" bilirubin exposure to long-term academic potential of children are needed to explore new insights into bilirubin's effect on the structural and functional maturation of an infant's neural network topology.

  9. Copper and zinc metabolism in aminonucleoside-induced nephrotic syndrome.

    Science.gov (United States)

    Pedraza-Chaverrí, J; Torres-Rodríguez, G A; Cruz, C; Mainero, A; Tapia, E; Ibarra-Rubio, M E; Silencio, J L

    1994-01-01

    Copper (Cu) and zinc (Zn) were measured in urine, serum and tissues from rats with nephrotic syndrome (NS) induced with a single subcutaneous dose of puromycin aminonucleoside (PAN; 15 mg/100 g BW). Control animals were pair-fed. Urine was collected daily, and the rats were sacrificed on day 10. PAN-nephrotic rats had proteinuria (days 3-10), high urinary Cu (days 1, 2, 4-10) and Zn (days 3-10) excretion. On day 10, nephrotic rats had: (a) albuminuria, hypoalbuminemia, hypoproteinemia, high urine and low serum levels of ceruloplasmin; (b) low Cu and Zn serum levels; (c) high clearance and fractional excretion of Cu and Zn, and (d) low kidney and liver Cu content and essentially normal tissue Zn levels. The alterations in Cu metabolism were more intense than those in Zn metabolism. Urine Cu and Zn showed a positive correlation with urine total protein on days 3-10 which suggests that high urinary excretion of Cu and Zn may be due to the excretion of its carrier proteins. In conclusion, these rats did not show a typical Zn deficiency but a clear decrease in Cu in the liver and kidney.

  10. 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

  11. [Rare cutaneous tuberculosis in sub-Saharan Africa developed on discoid lupus erythematous lesion].

    Science.gov (United States)

    Diop, A; Ndiaye, M T; Ndiaye, M; Seck, B; Diouf, A; Diatta, B H; Diallo, M; Ly, F

    2017-02-28

    Lupus vulgaris is a common presentation of cutaneous tuberculosis (TB), but its ulcerative or vegetating form also called vorax is rare.We report a case of lupus vulgaris in its vorax form, which occurred in a patient with discoid lupus erythematous. A 42-year-old patient monitored for chronic lupus erythematosus consulted again for a facial tumor and fever. Physical examination revealed painful ulcerative and crusted lesions on an old discoid lupus lesion and covered the entire upper lip. Similar lesions were noted on cheeks. Moreover, there were cervical lymphadenopathy, a bilateral pulmonary condensation syndrome, and dysphonia. Cutaneous histopathology had revealed a tuberculoid granuloma without caseating, and nucleic acid amplification tests (GeneXpert(®)) performed on sputum were positive. Thus, the diagnosis of multifocal TB with skin involvement of vorax type was confirmed. The outcome was favorable following TB treatment. Our observation is distinctive as it is a granulomatosis and ulcerative form of lupus vulgaris, which occurred on a discoid lupus erythematous lesion. This is a rare form of lupus vulgaris. This scarcity can be explained by diagnostic difficulties as there are numerous differential diagnoses and histopathology is not often helpful.

  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. Aspirin for Prevention of Preeclampsia in Lupus Pregnancy

    Directory of Open Access Journals (Sweden)

    Amelie M. Schramm

    2014-01-01

    Full Text Available Preeclampsia, the onset of hypertension and proteinuria during pregnancy, is a common medical disorder with high maternal and fetal mortality and morbidity. The underlying pathology remains poorly understood and includes inflammation, endothelial dysfunction, and an unbalanced thromboxane A2/prostacyclin ratio. For women with systemic lupus erythematosus (SLE, particularly those with preexisting renal disease or with active lupus, the risk of developing preeclampsia is up to 14% higher than it is among healthy individuals. The mechanism is still unknown and the data for preventing preeclampsia in lupus pregnancies are rare. Modulating the impaired thromboxane A2/prostacyclin ratio by administration of low-dose aspirin appears to be the current best option for the prevention of preeclampsia. After providing an overview of the pathogenesis of preeclampsia, preeclampsia in lupus pregnancies, and previous trials for prevention of preeclampsia with aspirin treatment, we recommend low-dose aspirin administration for all lupus patients starting prior to 16 weeks of gestation. Patients with SLE and antiphospholipid syndrome should receive treatment with heparin and low-dose aspirin during pregnancy.

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

    Science.gov (United States)

    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.

  15. 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.

  16. 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

  17. Clinical features and associated factors of abdominal pain in systemic lupus erythematosus.

    Science.gov (United States)

    Yuan, Shiwen; Lian, Fan; Chen, Dongying; Li, Hao; Qiu, Qian; Zhan, Zhongping; Ye, Yujin; Xu, Hanshi; Liang, Liuqin; Yang, Xiuyan

    2013-12-01

    To evaluate the clinical characteristics of systemic lupus erythematosus (SLE)-induced abdominal pain in a cohort in South China and identify the risk factors for SLE-induced abdominal pain. This is a retrospective cohort study of SLE patients with complaint of abdominal pain admitted to the first affiliated university hospital of Sun Yat-sen University between 2002 and 2011. Demographic information, clinical features, laboratory findings, SLE Disease Activity Index, and imaging characteristics were documented. Of the 3823 SLE patients reviewed, 213 patients complained of abdominal pain and 132 cases were considered SLE-induced. The most common causes were lupus mesenteric vasculitis (LMV; 73.5%, 97/132) and lupus pancreatitis (LP; 17.4%, 23/132). Other causes included appendicitis, acute gastroenteritis, and peritonitis. Univariate and multivariate logistic regression analysis indicated the European Consensus Lupus Activity Measurement (ECLAM) score was significantly associated with lupus-induced abdominal pain (OR = 1.858, 95% CI: 1.441-2.394, p lupus-induced abdominal pain. Moderate and large amounts of ascetic fluid was significantly associated with lupus-induced abdominal pain and LMV. Elevated liver enzymes was a risk factor for LP (OR = 34.605, 95% CI: 3.591-333.472, p = 0.002). LMV and LP were the leading causes of SLE-induced abdominal pain. The serum D-dimer was a strongly associated factor for lupus-induced abdominal pain. ECLAM score was a reliable index in assessment of SLE-associated abdominal pain. Elevated liver enzymes, and moderate or large amounts of ascites, were positively associated with lupus-induced abdominal pain.

  18. 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....

  19. Drug-induced lupus erythematosus

    Science.gov (United States)

    ... A chest x-ray may show signs of pleuritis or pericarditis (inflammation around the lining of the ... anti-inflammatory drugs (NSAIDs) to treat arthritis and pleurisy Corticosteroid creams to treat skin rashes Antimalarial drugs ( ...

  20. 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.

  1. 系统性红斑狼疮继发抗磷脂抗体综合征临床分析%Clinical analysis of systemic lupus erythematosus complicated with antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    金莉; 马艳; 李向培; 厉小梅; 吴竞生; 汪国生; 陶金辉; 钱龙

    2014-01-01

    目的:探讨系统性红斑狼疮(system ic lupus erythematosus,SLE)继发抗磷脂抗体综合征(antiphospholipid syndrome,APS)的临床特点、实验室指标及相关因素,以早期发现继发性APS.方法:回顾性分析2005年1月至2014年1月收集的129例SLE和APS患者的临床资料,包括临床表现、妊娠情况、抗核抗体、抗心磷脂抗体等.结果:129例患者中,原发性APS (primary antiphospholipid syndrome,PAPS)8例;SLE者121例,其中SLE合并APS患者(SLE-APS)41例,仅诊断为SLE患者(SLE-APA-)40例及出现抗磷脂抗体(antiphospholipid antibody,APA)异常但APS诊断依据不足(SLE-APA+)的SLE患者40例.8例原发性APS患者中,3例男性均表现为下肢深静脉血栓形成及手微动脉血栓形成,有婚育史的女性患者中5例均有病态妊娠表现,自发性流产和(或)死胎,其中2例有肺栓塞、门(脾)静脉栓塞.41例SLE-APS患者中,表现为下肢深静脉血栓有15例,肺栓塞患者3例,脑梗死10例;SLE-APS组抗β2-糖蛋白1(抗β2-GP-1)浓度明显高于SLE-APA+组及SLE-APA-组,而血小板计数明显减低.结论:SLE合并APS患者的血清中存在高浓度抗β2-GP-1,提示高浓度抗β2-GP-1是SLE继发APS的独立危险因素.

  2. Lung and lupus vulgaris.

    Science.gov (United States)

    Mukta, V; Jayachandran, K

    2011-04-01

    Lupus vulgaris is chronic, postprimary, paucibacillary cutaneous tuberculosis found in individuals with moderate immunity and high degree of tuberculin sensitivity. Eighty percent of the lesions are on the head and neck. We present the case of a 38 year old lady who was admitted with complaints of worsening breathlessness and low grade fever of one month duration. Examination showed multiple, nontender skin ulcers on bilateral lumbar areas, two oozing serosanguinous discharge and others scarred in the centre. Respiratory system examination and chest X-ray revealed right sided pleural effusion. On investigation, pleural fluid was tuberculous in nature. Skin biopsy from the edge of ulcer was also suggestive of tuberculosis. Patient is doing well on antituberculous drugs. This case highlights the importance of cutaneous manifestations of systemic disease and is an example of the unusual presentation of lupus vulgaris in a case of pleural effusion.

  3. What Causes Lupus Flares?

    Science.gov (United States)

    Fernandez, David; Kirou, Kyriakos A

    2016-03-01

    Systemic lupus erythematosus (SLE), the prototypic systemic autoimmune disease, follows a chronic disease course, punctuated by flares. Disease flares often occur without apparent cause, perhaps from progressive inherent buildup of autoimmunity. However, there is evidence that certain environmental factors may trigger the disease. These include exposure to UV light, infections, certain hormones, and drugs which may activate the innate and adaptive immune system, resulting in inflammation, cytotoxic effects, and clinical symptoms. Uncontrolled disease flares, as well as their treatment, especially with glucocorticoids, can cause significant organ damage. Tight surveillance and timely control of lupus flares with judicial use of effective treatments to adequately suppress the excessive immune system activation are required to bring about long term remission of the disease. We hope that new clinical trials will soon offer additional effective and target-specific biologic treatments for SLE.

  4. Lupus miliaris disseminatus faciei

    Directory of Open Access Journals (Sweden)

    Gupta Dinesh

    1996-01-01

    Full Text Available Lupus miliaris disseminatus faciei also known as acne agminata is a rare disease affecting face in adults. Previously, it was thought to be a tuberculid; and its relation with rosacea is undefined. We report a case who had multiple yellowish brown to erythematous small papular lesions and many pitted atrophic scars on the face of 8 months duration. Investigations for tuberculosis were negative. Histopathology revealed tuberculoid granuloma.

  5. Haematological manifestations of lupus

    OpenAIRE

    2015-01-01

    Our purpose was to compile information on the haematological manifestations of systemic lupus erythematosus (SLE), namely leucopenia, lymphopenia, thrombocytopenia, autoimmune haemolytic anaemia (AIHA), thrombotic thrombocytopenic purpura (TTP) and myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manif...

  6. Kidney disease in lupus is not always 'lupus nephritis'

    NARCIS (Netherlands)

    H.J. Anders (Hans-Joachim); J.J. Weening (Jan)

    2013-01-01

    textabstractIn lupus erythematosus, elevated serum creatinine levels and urinary abnormalities implicate a kidney disorder, which may not always be lupus nephritis as defined by the current classification of the International Society of Nephrology/Renal Pathology Society. The signs of renal dysfunct

  7. Kidney disease in lupus is not always 'lupus nephritis'

    NARCIS (Netherlands)

    H.J. Anders (Hans-Joachim); J.J. Weening (Jan)

    2013-01-01

    textabstractIn lupus erythematosus, elevated serum creatinine levels and urinary abnormalities implicate a kidney disorder, which may not always be lupus nephritis as defined by the current classification of the International Society of Nephrology/Renal Pathology Society. The signs of renal dysfunct

  8. 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.

  9. 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...

  10. Neuropsychiatric lupus: classification criteria in neuroimaging studies.

    Science.gov (United States)

    Netto, Tania M; Zimmermann, Nicolle; Rueda-Lopes, Fernanda; Bizzo, Bernardo C; Fonseca, Rochele P; Gasparetto, Emerson L

    2013-05-01

    This systematic review described the criteria and main evaluations methods procedures used to classify neuropsychiatric systemic lupus erythematosus (NPSLE) patients. Also, within the evaluations methods, this review aimed to identify the main contributions of neuropsychological measurements in neuroimaging studies. A search was conducted in PubMed, EMBASE and SCOPUS databases with the terms related to neuropsychiatric syndromes, systemic lupus erythematosus, and neuroimaging techniques. Sixty-six abstracts were found; only 20 were completely analyzed and included. Results indicated that the 1999 American College of Rheumatology (ACR) criteria is the most used to classify NPSLE samples together with laboratorial, cognitive, neurological and psychiatric assessment procedures. However, the recommended ACR assessment procedures to classify NPSLE patients are being used incompletely, especially the neuropsychological batteries. Neuropsychological instruments and neuroimaging techniques have been used mostly to characterize NPSLE samples, instead of contributing to their classifications. The most described syndromes in neuroimaging studies have been seizure/cerebrovascular disease followed by cognitive dysfunctions as well as headache disorder.

  11. Exercise-induced albuminuria is related to metabolic syndrome.

    Science.gov (United States)

    Greenberg, Sharon; Shenhar-Tsarfaty, Shani; Rogowski, Ori; Shapira, Itzhak; Zeltser, David; Weinstein, Talia; Lahav, Dror; Vered, Jaffa; Tovia-Brodie, Oholi; Arbel, Yaron; Berliner, Shlomo; Milwidsky, Assi

    2016-06-01

    Microalbuminuria (MA) is a known marker for endothelial dysfunction and future cardiovascular events. Exercise-induced albuminuria (EiA) may precede the appearance of MA. Associations between EiA and metabolic syndrome (MS) have not been assessed so far. Our aim was to investigate this association in a large sample of apparently healthy individuals with no baseline albuminuria. This was a cross-sectional study of 2,027 adults with no overt cardiovascular diseases who took part in a health survey program and had no baseline MA. Diagnosis of MS was based on harmonized criteria. All patients underwent an exercise test (Bruce protocol), and urinary albumin was measured before and after the examination. Urinary albumin-to-creatinine ratio (ACR) values before and after exercise were 0.40 (0.21-0.89) and 1.06 (0.43-2.69) mg/g for median (interquartile range) respectively. A total of 394 (20%) subjects had EiA; ACR rose from normal rest values (0.79 mg/g) to 52.28 mg/g after exercise (P metabolic equivalents (P < 0.001), higher baseline blood pressure (P < 0.001), and higher levels of fasting plasma glucose, triglycerides, and body mass index (P < 0.001). Multivariate binary logistic regression model showed that subjects with MS were 98% more likely to have EiA (95% confidence interval: 1.13-3.46, P = 0.016). In conclusion, EiA in the absence of baseline MA is independently related to MS.

  12. 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.

  13. Pro: Cyclophosphamide in lupus nephritis

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    2016-01-01

    Based on efficacy and toxicity considerations, both low-dose pulse cyclophosphamide as part of the Euro-Lupus Nephritis protocol and mycophenolate mofetil (MMF) with corticosteroids may be considered for induction of remission in patients with proliferative lupus nephritis. The long-term follow-up d

  14. Prognostic factors in lupus nephritis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Starklint, Henrik; Halberg, Poul

    2006-01-01

    To evaluate the prognostic significance of clinical and renal biopsy findings in an unselected cohort of patients with systemic lupus erythematosus (SLE) and nephritis.......To evaluate the prognostic significance of clinical and renal biopsy findings in an unselected cohort of patients with systemic lupus erythematosus (SLE) and nephritis....

  15. Pro: Cyclophosphamide in lupus nephritis

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    2016-01-01

    Based on efficacy and toxicity considerations, both low-dose pulse cyclophosphamide as part of the Euro-Lupus Nephritis protocol and mycophenolate mofetil (MMF) with corticosteroids may be considered for induction of remission in patients with proliferative lupus nephritis. The long-term follow-up d

  16. [Systemic lupus erythematosus and weakness].

    Science.gov (United States)

    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.

  17. 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)....

  18. Clinical analysis for euthyroid sick syndrome in patients with systemic lupus erythematosus%系统性红斑狼疮合并正常甲状腺病态综合征的临床分析

    Institute of Scientific and Technical Information of China (English)

    邓慧; 王春

    2012-01-01

    Objective To investigate the clinical features and related factors of euthyroid sick syndrome (ESS) in patients with systemic lupus erythematosus ( SLE). Methods Retrospective analysis was carried out in 43 SLE with ESS and 50 SLE with normal thyroid function. Results The incidence of ESS in SLE patients was positively correlated with the level of systemic lupus erythematosus disease activity index ( SLEDAI). In 43 SLE patients with ESS, the rates of Raynauds phenomenon(RP) , pulmonary artery hypertension (PAH) and serous cavity effusion were significantly higher than in normal thyroid function group. The levels of erythrocyte sedimentation rate ( ESR) , 24 hour urine protein , immu-noglobulin A(IgA) , immunoglobulin G(IgG) , immunoglobulin M(IgM) and the rate of positive antibodies to double -stranded DNA ( ds - DNA) were significantly higher, the levels of serum albumin and cardiac ejection fraction ( EF) were significantly lower than in normal thyroid function group . Conclusion There is a high risk of ESS in patients with SLE. The prevalence of ESS in SLE with SLEDAI score > 10, RP, PAH, serous cavity effsuion, higher levels of ESR, 24 hour urine protein, IgG, IgM, higher rates of positive antibodies to ds -DNA and lower levels of serum albumin and cardiac EF is higher than that of SLE patients without these features .%目的 分析系统性红斑狼疮(SLE)合并正常甲状腺病态综合征(ESS)的临床特点.方法 回顾性分析比较43例合并ESS的SLE患者和50例甲状腺功能正常SLE患者的临床资料.结果 在SLE患者中,ESS的发病率随着SLE疾病活动指数(SLEDAI)评分的增加而升高;与甲功正常组比较,ESS组患者雷诺征(RP)、肺动脉高压(PAH)、浆膜腔积液发生率高(P<0.05),ESS组患者血沉(ESR)、24 h尿蛋白、免疫球蛋白G(IgG)、免疫球蛋白M(IgM)、抗双链DNA(ds-DNA)抗体阳性率增高(P<0.05),而血清白蛋白(ALB)、心脏射血分数(EF)降低(P<0.05).结论 SLE患者易合并ESS,当SLE

  19. Fever of unknown origin as the initial manifestation of valproate-induced Fanconi syndrome.

    Science.gov (United States)

    Nozaki, Fumihito; Kumada, Tomohiro; Kusunoki, Takashi; Fujii, Tatsuya; Murayama, Kei; Ohtake, Akira

    2014-12-01

    Valproate-induced Fanconi syndrome is a rare adverse effect of valproate. Severely disabled patients who require tube feeding are reported to be susceptible to valproate-induced Fanconi syndrome. Although most patients with valproate-induced Fanconi syndrome are asymptomatic and detected incidentally with findings such as hypophosphatemia, hypouricemia, increased urinary β2-microglobulin, and generalized hyperaminoaciduria, clinical symptoms such as bone fracture, fever, tachypnea, and edema have been reported. This 15-year-old, severely disabled, tube-fed, male patient with cytochrome oxidase deficiency had taken valproate for 3 years when he developed fever for 3 weeks. Hypophosphatemia, hypouricemia, hypokalemia, increased urinary β2-microglobulin, and generalized hyperaminoaciduria, as well as hypocarnitinemia, were found, indicating that he had Fanconi syndrome. Valproate was the most likely cause of Fanconi syndrome in this patient. After discontinuation of valproate, the fever resolved immediately, and the laboratory findings normalized. Valproate-induced Fanconi syndrome should be considered when individuals taking valproate develop fever of unknown origin. Copyright © 2014 Elsevier Inc. All rights reserved.

  20. Drug-induced Fanconi syndrome associated with fumaric acid esters treatment for psoriasis: A case series

    NARCIS (Netherlands)

    D.M.W. Balak (Deepak); J.N.B. Bavinck (Jan Nico Bouwes); De Vries, A.P.J. (Aiko P. J.); Hartman, J. (Jenny); Martino Neumann, H.A. (Hendrik A.); R. Zietse (Bob); H.B. Thio (Bing)

    2016-01-01

    textabstractBackground: Fumaric acid esters (FAEs), an oral immunomodulating treatment for psoriasis and multiple sclerosis, have been anecdotally associated with proximal renal tubular dysfunction due to a drug-induced Fanconi syndrome. Few data are available on clinical outcomes of FAE-induced Fan

  1. Genetics Home Reference: cold-induced sweating syndrome

    Science.gov (United States)

    ... affected individuals begin having episodes of profuse sweating (hyperhidrosis) and shivering involving the face, torso, and arms. ... syndrome Patient Support and Advocacy Resources (3 links) Hyperhidrosis Support Group (UK) International Hyperhidrosis Society Resource list ...

  2. Methylglyoxal Induces Systemic Symptoms of Irritable Bowel Syndrome

    OpenAIRE

    Shuang Zhang; Taiwei Jiao; Yushuai Chen; Nan Gao; Lili Zhang; Min Jiang

    2014-01-01

    Patients with irritable bowel syndrome (IBS) show a wide range of symptoms including diarrhea, abdominal pain, changes in bowel habits, nausea, vomiting, headache, anxiety, depression and cognitive impairment. Methylglyoxal has been proved to be a potential toxic metabolite produced by intestinal bacteria. The present study was aimed at investigating the correlation between methylglyoxal and irritable bowel syndrome. Rats were treated with an enema infusion of methylglyoxal. Fecal water conte...

  3. [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.

  4. ‘ASIA’ - Autoimmune/inflammatory syndrome induced by adjuvants: even and odd

    Directory of Open Access Journals (Sweden)

    G. Valesini

    2011-09-01

    Full Text Available Recently, Shoenfeld and Agmon-Levin described a potential new syndrome, namely ASIA - autoimmune/inflammatory syndrome induced by adjuvants, that comprises four medical conditions: siliconosis, the Gulf war syndrome, the macrophagic myofasciitis syndrome and post-vaccination phenomena, characterized by hyperactive immune responses accompanied by a similar complex of signs and symptoms. Most relevantly, these conditions share a linkage represented by adjuvants. This common soil may possibly induce autoimmune or auto-inflammatory diseases in humans as it was demonstrated in different animal models. Reconsidering under a unified umbrella this apparently detached condition is not only intriguing, but also provocative, and may help in unraveling novel pathogenetic mechanisms, preventive measures, and therapeutic targets.

  5. Minimal change disease: a variant of lupus nephritis.

    Science.gov (United States)

    Moysés-Neto, Miguel; Costa, Roberto S; Rodrigues, Fernanda F; Vieira Neto, Osvaldo M; Reis, Marlene A; Louzada Júnior, Paulo; Romão, Elen A; Dantas, Márcio

    2011-02-01

    Some patients with systemic lupus erythematosus (SLE) present with nephrotic syndrome due to minimal change disease (MCD). Histopathological diagnosis of patients with SLE and nephrotic-range proteinuria has shown that these patients present with diffuse proliferative glomerulonephritis and membranous glomerulonephritis, World Health Organization (WHO) classes IV and V, respectively, more frequently than the other classes. In the present study, we reported a case of nephrotic syndrome and renal biopsy-proven MCD associated with SLE. A complete remission occurred after steroid treatment, which was followed by a relapse 15 months later with a concomitant reactivation of SLE. A second biopsy showed WHO class IIb lupus nephritis. Prednisone treatment was restarted, and the patient went into complete remission again. The association of MCD and SLE may not be a coincidence, and MCD should be considered as an associated SLE nephropathy.

  6. 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.

  7. Paroxysmal Exercise-induced Dyskinesias Caused by GLUT1 Deficiency Syndrome

    Science.gov (United States)

    Mongin, Marie; Mezouar, Nicolas; Dodet, Pauline; Vidailhet, Marie; Roze, Emmanuel

    2016-01-01

    Background Glucose transporter type 1 deficiency syndrome is due to de novo mutations in the SLC2A1 gene encoding the glucose transporter type 1. Phenomenology Shown Paroxysmal motor manifestations induced by exercise or fasting may be the main manifestations of glucose transporter type 1 deficiency syndrome. Educational Value Proper identification of the paroxysmal events and early diagnosis is important since the disease is potentially treatable. PMID:27351150

  8. Mitochondrial translocation of Nur77 induced by ROS contributed to cardiomyocyte apoptosis in metabolic syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Xu, Aibin; Liu, Jingyi [Department of Cardiology, Xijing Hospital, Fourth Military Medical University, Xi’an (China); Institute of Cardiovascular Disease, General Hospital of Beijing Command, PLA, Beijing (China); Liu, Peilin; Jia, Min; Wang, Han [Department of Cardiology, Xijing Hospital, Fourth Military Medical University, Xi’an (China); Tao, Ling, E-mail: lingtao2006@gmail.com [Department of Cardiology, Xijing Hospital, Fourth Military Medical University, Xi’an (China)

    2014-04-18

    Highlights: • Metabolic syndrome exacerbated MI/R induced injury accompanied by decreased Nur77. • ROS led to Nur77 translocation in metabolic syndrome. • Inhibiting relocation of Nur77 to mitochondria reduced ROS-induced cardiomyocyte injury in metabolic syndrome. - Abstract: Metabolic syndrome is a major risk factor for cardiovascular diseases, and increased cardiomyocyte apoptosis which contributes to cardiac dysfunction after myocardial ischemia/reperfusion (MI/R) injury. Nur77, a nuclear orphan receptor, is involved in such various cellular events as apoptosis, proliferation, and glucose and lipid metabolism in several cell types. Apoptosis is positively correlated with mitochondrial translocation of Nur77 in the cancer cells. However, the roles of Nur77 on cardiac myocytes in patients with metabolic syndrome remain unclear. The objective of this study was to determine whether Nur77 may contribute to cardiac apoptosis in patients with metabolic syndrome after I/R injury, and, if so, to identify the underlying molecular mechanisms responsible. We used leptin-deficient (ob/ob) mice to make metabolic syndrome models. In this report, we observed that, accompanied by the substantial decline in apoptosis inducer Nur77, MI/R induced cardiac dysfunction was manifested as cardiomyopathy and increased ROS. Using the neonatal rat cardiac myocytes cultured in a high-glucose and high-fat medium, we found that excessive H{sub 2}O{sub 2} led to the significant alteration in mitochondrial membrane potential and translocation of Nur77 from the nucleus to the mitochondria. However, inhibition of the relocation of Nur77 to mitochondria via Cyclosporin A reversed the changes in membrane potential mediated by H{sub 2}O{sub 2} and reduced myocardial cell injury. Therefore, these data provide a potential underlying mechanism for cardiac dysfunction in metabolic syndrome and the suppression of Nur77 translocation may provide an effective approach to reduce cardiac injury in the

  9. 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.

  10. Neurological Sequelae of Lupus

    Science.gov (United States)

    ... Craniosynostosis Information Page Creutzfeldt-Jakob Disease Information Page Cushing's Syndrome Information Page Dandy-Walker Syndrome Information Page Deep Brain Stimulation for Parkinson's Disease Information Page Dementia Information ...

  11. Combination of Captopril and Allopurinol Retards Fructose-Induced Metabolic Syndrome

    Science.gov (United States)

    Roncal, Carlos A.; Reungjui, Sirirat; Sánchez-Lozada, Laura Gabriela; Mu, Wei; Sautin, Yuri Y.; Nakagawa, Takahiko; Johnson, Richard J.

    2009-01-01

    Background Both ACE inhibitors and allopurinol have been shown to partially prevent metabolic syndrome induced by fructose. We tested the hypothesis that combined therapy might be more effective at blocking the metabolic syndrome induced with fructose. Methods Male Sprague-Dawley rats were fed a high fructose diet with or without allopurinol, captopril, or the combination for 20 weeks. A control group received a normal diet. All groups were pair-fed to assure equivalent caloric intake. Results Despite reduced energy intake, the fructose-fed rats developed features of metabolic syndrome including elevated blood pressure, abdominal obesity, hypertriglyceridemia, hyperuricemia and hyperinsulinemia. While both allopurinol and captopril alone tended to reduce features of the metabolic syndrome, the combined therapy was synergistic, with significant reduction in blood pressure, less accumulation of abdominal fat, an improvement in the dyslipidemia and a complete prevention of insulin resistance. Conclusion A high fructose diet can induce metabolic syndrome even in the setting of caloric restriction. Captopril and allopurinol synergistically reduce features of the metabolic syndrome, especially hypertension, insulin resistance and dyslipidemia. Combination allopurinol and ACE inhibitor therapy might provide a superior means to prevent diabetes and cardiovascular disease. PMID:19696478

  12. 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.

  13. 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.

  14. Thiazide diuretics exacerbate fructose-induced metabolic syndrome.

    Science.gov (United States)

    Reungjui, Sirirat; Roncal, Carlos A; Mu, Wei; Srinivas, Titte R; Sirivongs, Dhavee; Johnson, Richard J; Nakagawa, Takahiko

    2007-10-01

    Fructose is a commonly used sweetener associated with diets that increase the prevalence of metabolic syndrome. Thiazide diuretics are frequently used in these patients for treatment of hypertension, but they also exacerbate metabolic syndrome. Rats on high-fructose diets that are given thiazides exhibit potassium depletion and hyperuricemia. Potassium supplementation improves their insulin resistance and hypertension, whereas allopurinol reduces serum levels of uric acid and ameliorates hypertension, hypertriglyceridemia, hyperglycemia, and insulin resistance. Both potassium supplementation and treatment with allopurinol also increase urinary nitric oxide excretion. We suggest that potassium depletion and hyperuricemia in rats exacerbates endothelial dysfunction and lowers the bioavailability of nitric oxide, which blocks insulin activity and causes insulin resistance during thiazide usage. Addition of potassium supplements and allopurinol with thiazides might be helpful in the management of metabolic syndrome.

  15. Pregnancy and Lupus Nephritis.

    Science.gov (United States)

    Kattah, Andrea G; Garovic, Vesna D

    2015-09-01

    The management of lupus nephritis in pregnancy presents a diagnostic and therapeutic challenge for providers. Pregnancy creates a series of physiologic changes in the immune system and kidney that may result in an increased risk of disease flare and adverse maternal and fetal outcomes, such as preeclampsia, fetal loss, and preterm delivery. Conception should be delayed until disease is in remission to ensure the best pregnancy outcomes. Maternal disease activity and fetal well-being should be monitored closely by an interdisciplinary team, including obstetricians, rheumatologists, and nephrologists throughout pregnancy. Careful attention must be paid to the dosing and potential teratogenicity of medications.

  16. Systemic lupus erythematosus serositis

    Energy Technology Data Exchange (ETDEWEB)

    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.

  17. Lupus Alma Disk Survey

    Science.gov (United States)

    Ansdell, Megan

    2016-07-01

    We present the first unbiased ALMA survey of both dust and gas in a large sample of protoplanetary disks. We surveyed 100 sources in the nearby (150-200 pc), young (1-2 Myr) Lupus region to constrain M_dust to 2 M_Mars and M_gas to 1 M_Jup. Most disks have masses < MMSN and gas-to-dust ratios < ISM. Such rapid gas depletion may explain the prevalence of super-Earths in the exoplanet population.

  18. Filaments in Lupus I

    Science.gov (United States)

    Takahashi, Satoko; Rodon, J.; De Gregorio-Monsalvo, I.; Plunkett, A.

    2017-06-01

    The mechanisms behind the formation of sub-stellar mass sources are key to determine the populations at the low-mass end of the stellar distribution. Here, we present mapping observations toward the Lupus I cloud in C18O(2-1) and 13CO(2-1) obtained with APEX. We have identified a few velocity-coherent filaments. Each contains several substellar mass sources that are also identified in the 1.1mm continuum data (see also SOLA catalogue presentation). We will discuss the velocity structure, fragmentation properties of the identified filaments, and the nature of the detected sources.

  19. Systemic lupus erythematosus.

    Science.gov (United States)

    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.

  20. Unusual case of adult hemophagocytic syndrome

    Directory of Open Access Journals (Sweden)

    Babak Tamizifar

    2014-01-01

    Full Text Available Hemophagocytic syndrome (HPS is an uncommon manifestation in systemic lupus erythematosus (SLE. Clinical features of HPS include fever, pancytopenia, abnormal liver enzyme, hepatosplenomegaly, lymphadenopathy, and coagulation disorder. HPS comprises primary and reactive forms. Herein, we describe a case of untreated SLE with HPS as one of the first manifestations of systemic Lupus.

  1. Organ damage in zymosan-induced multiple organ dysfunction syndrome in mice is not mediated by inducible nitric oxide synthase.

    NARCIS (Netherlands)

    Volman, T.J.H.; Goris, R.J.A.; Jagt, M. van der; Loo, F.A.J. van de; Hendriks, T.

    2002-01-01

    OBJECTIVE: To examine the role of inducible nitric oxide synthase (iNOS) in the development of the multiple organ dysfunction syndrome (MODS) in a murine model by using either a selective iNOS inhibitor or iNOS knockout mice. DESIGN: Prospective randomized laboratory study. SETTING: Central animal

  2. Lupus erítematoso sistêmico juvenil: comprometimento neurológico

    OpenAIRE

    Schmutzler,Katia M.R.S.; Luiz Celso P Vilanova; José Geraldo C. Lima; Maria Odete Hilário; Charles K. Naspitz

    1997-01-01

    With the purpose of analyzing the neurological involvement due to systemic lupus erythematosus (SLE), we evaluated 17 female patients who were seen regularly at the hospital and had been diagnosed as having SLE according to classification criteria proposed by the American College of Rheumatology revised in 1982, before the age of 16. Neurological involvement was detected in 12 patients (71%): headache (35%), extrapyramidal syndrome (35%), epileptic syndrome (24%) pyramidal syndrome (24%), per...

  3. Olmesartan-induced Enteropathy Manifesting as Wernicke-Korsakoff Syndrome.

    Science.gov (United States)

    Uehara, Takanori; Ikusaka, Masatomi; Ohira, Yoshiyuki; Noda, Kazutaka; Suzuki, Shingo; Shikino, Kiyoshi; Kondo, Takeshi; Kajiwara, Hideki; Ikegami, Akiko; Hirota, Yusuke

    Cases of sprue-like enteropathy associated with olmesartan have sporadically been encountered since it was first reported in 2012, and their most characteristic manifestation is severe diarrhea. We herein report the first case of sprue-like enteropathy manifesting as Wernicke-Korsakoff syndrome due to vitamin B1 malabsorption with only minimally increased bowel movements. When patients are receiving olmesartan and they complain of nonspecific chronic gastrointestinal symptoms, it is important to consider changing the drugs before any serious malabsorption syndrome develops.

  4. Blackcurrant Suppresses Metabolic Syndrome Induced by High-Fructose Diet in Rats

    Directory of Open Access Journals (Sweden)

    Ji Hun Park

    2015-01-01

    Full Text Available Increased fructose ingestion has been linked to obesity, hyperglycemia, dyslipidemia, and hypertension associated with metabolic syndrome. Blackcurrant (Ribes nigrum; BC is a horticultural crop in Europe. To induce metabolic syndrome, Sprague-Dawley rats were fed 60% high-fructose diet. Treatment with BC (100 or 300 mg/kg/day for 8 weeks significantly suppressed increased liver weight, epididymal fat weight, C-reactive protein (CRP, total bilirubin, leptin, and insulin in rats with induced metabolic syndrome. BC markedly prevented increased adipocyte size and hepatic triglyceride accumulation in rats with induced metabolic syndrome. BC suppressed oral glucose tolerance and protein expression of insulin receptor substrate-1 (IRS-1 and phosphorylated AMP-activated protein kinase (p-AMPK in muscle. BC significantly suppressed plasma total cholesterol, triglyceride, and LDL content. BC suppressed endothelial dysfunction by inducing downregulation of endothelin-1 and adhesion molecules in the aorta. Vascular relaxation of thoracic aortic rings by sodium nitroprusside and acetylcholine was improved by BC. The present study provides evidence of the potential protective effect of BC against metabolic syndrome by demonstrating improvements in dyslipidemia, hypertension, insulin resistance, and obesity in vivo.

  5. 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

  6. 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.

  7. A prospective naturalistic study of antidepressant-induced jitteriness/anxiety syndrome

    Directory of Open Access Journals (Sweden)

    Harada T

    2014-11-01

    Full Text Available Tsuyoto Harada, Ken Inada, Kazuo Yamada, Kaoru Sakamoto, Jun Ishigooka Department of Psychiatry, Tokyo Women’s Medical University School of Medicine, Tokyo, Japan Objective: Patients often develop neuropsychiatric symptoms such as anxiety and agitation after they have started taking an antidepressant, and this is thought to be associated with a potentially increased risk of suicide. However, the incidence of antidepressant-induced jitteriness/anxiety syndrome has not been fully investigated, and little has been reported on its predictors. The aim of this study was to survey the incidence of antidepressant-induced jitteriness/anxiety syndrome and clarify its predictors in a natural clinical setting.Materials and methods: Between January 2009 and July 2012, we prospectively surveyed 301 patients who had not taken any antidepressants for 1 month before presentation, and who were prescribed antidepressants for 1 month after their initial visit. Patients were classified as developing antidepressant-induced jitteriness/anxiety syndrome if they experienced any symptoms of anxiety, agitation, panic attacks, insomnia, irritability, hostility, aggressiveness, impulsivity, akathisia, hypomania, or mania during the first month.Results: Among the 301 patients, 21 (7.0% developed antidepressant-induced jitteriness/anxiety syndrome. Major depressive disorder and a diagnosis of mood disorder in first-degree relatives of patients were significantly associated with induction of antidepressant-induced jitteriness/anxiety syndrome (odds ratio 10.2, P=0.001; odds ratio 4.65, P=0.02; respectively. However, there was no such relationship for sex, age, class of antidepressant, combined use of benzodiazepines, or diagnosis of anxiety disorder.Conclusion: The findings of this study suggest that major depressive disorder and a diagnosis of mood disorder in first-degree relatives may be clinical predictors of antidepressant-induced jitteriness/anxiety syndrome

  8. Apoptosis and the thymic microenvironment in murine lupus.

    Science.gov (United States)

    Takeoka, Y; Taguchi, N; Shultz, L; Boyd, R L; Naiki, M; Ansari, A A; Gershwin, M E

    1999-11-01

    The thymus of New Zealand black (NZB) mice undergoes premature involution. In addition, cultured thymic epithelial cells from NZB mice undergo accelerated preprogrammed degeneration. NZB mice also have distinctive and well-defined abnormalities of thymic architecture involving stromal cells, defined by staining with monoclonal antibodies specific for the thymic microenvironment. We took advantage of these findings, as well as our large panel of monoclonal antibodies which recognize thymic stroma, to study the induction of apoptosis in the thymus of murine lupus and including changes of epithelial architecture. We studied NZB, MRL/lpr, BXSB/Yaa, C3H/gld mice and BALB/c and C57BL/6 as control mice. Apoptosis was studied both at basal levels and following induction with either dexamethasone or lipopolysaccharide (LPS). The apoptotic cells were primarily found in the thymic cortex, and the frequency of apoptosis in murine lupus was less than 20% of controls. Moreover, all strains of murine lupus had severe abnormalities of the cortical network. These changes were not accentuated by dexamethasone treatment in cultured thymocytes. However, the thymus in murine lupus was less susceptible to LPS-induced apoptosis than control mice. Finally we note that the number of thymic nurse cells (TNC) was lowest in NZB mice. Our findings demonstrate significant abnormalities in the induction of apoptosis and the formation of TNC-like epithelial cells in SLE mice, and suggest that the abnormalities of the thymic microenvironment have an important role in the pathogenesis of murine lupus.

  9. Sunlight triggers cutaneous lupus through a CSF-1-dependent mechanism in MRL-Fas(lpr) mice.

    Science.gov (United States)

    Menke, Julia; Hsu, Mei-Yu; Byrne, Katelyn T; Lucas, Julie A; Rabacal, Whitney A; Croker, Byron P; Zong, Xiao-Hua; Stanley, E Richard; Kelley, Vicki R

    2008-11-15

    Sunlight (UVB) triggers cutaneous lupus erythematosus (CLE) and systemic lupus through an unknown mechanism. We tested the hypothesis that UVB triggers CLE through a CSF-1-dependent, macrophage (Mø)-mediated mechanism in MRL-Fas(lpr) mice. By constructing mutant MRL-Fas(lpr) strains expressing varying levels of CSF-1 (high, intermediate, none), and use of an ex vivo gene transfer to deliver CSF-1 intradermally, we determined that CSF-1 induces CLE in lupus-susceptible MRL-Fas(lpr) mice, but not in lupus-resistant BALB/c mice. UVB incites an increase in Møs, apoptosis in the skin, and CLE in MRL-Fas(lpr), but not in CSF-1-deficient MRL-Fas(lpr) mice. Furthermore, UVB did not induce CLE in BALB/c mice. Probing further, UVB stimulates CSF-1 expression by keratinocytes leading to recruitment and activation of Møs that, in turn, release mediators, which induce apoptosis in keratinocytes. Thus, sunlight triggers a CSF-1-dependent, Mø-mediated destructive inflammation in the skin leading to CLE in lupus-susceptible MRL-Fas(lpr) but not lupus-resistant BALB/c mice. Taken together, CSF-1 is envisioned as the match and lupus susceptibility as the tinder leading to CLE.

  10. B6.MRL-Faslpr/J狼疮小鼠脾脏调节性 T细胞与狼疮样病征的相关性研究%Correlation between Splenic Regulatory T Cells and Lupus-Like Syndrome in B6 .MRL-Faslpr/J Mice

    Institute of Scientific and Technical Information of China (English)

    廖烈兰; 毛胜富; 丁永雄; 张秀娟; 梁春业

    2014-01-01

    Objective To investigate the correlation between splenic regulatory T cells (Treg cells) and lupus-like syndrome in lupus mice,and to provide the basis for the immune therapy of systemic lupus erythematosus (SLE).Methods Thirty 22-week-old female B6.MRL-Faslpr/J mice were selected ,they were killed after their 24-hour urine and peripheral blood had been collected ,the spleen tissue sample was made into homogenate and then was filtrated ,the levels of 24-hour urinary protein,serum antinuclear antibody(ANA) and anti-ds-DNA antibody of the mice were detected,flow cytometry was used to detect splenic Treg cells and lymphocytes of the mice .The correlation of the proportion of Treg cells with the levels of 24-hour urinary protein,serum ANA and anti-ds-DNA antibody was analyzed by Pearson correlation .Results The proportion of splenic Treg cells highly negatively correlated with the levels of 24-hour urinary protein and serum ds-DNA antibody in lupus mice(all P<0.01),and negatively correlated with the level of serum ANA (P<0.05).Conclusion The proportion of splenic Treg cells might negatively correlate with the activity of lupus -like syndrome in lupus mice .%目的:探讨狼疮小鼠脾脏调节性T细胞( Treg细胞)与狼疮样病征的相关性,为系统性红斑狼疮(SLE)的免疫疗法提供依据。方法选取22周龄雌性B6.MRL-Faslpr/J狼疮小鼠30只,留取24 h尿液及外周血后处死,取脾脏组织进行匀浆过滤,分别检测各小鼠24 h尿蛋白量、血清中抗核抗体( ANA )和抗ds-DNA抗体水平,流式细胞仪检测小鼠脾脏Treg细胞及淋巴细胞。应用Pearson对Treg细胞比例与24 h尿蛋白量、血清ANA和抗ds-DNA抗体水平进行相关分析。结果狼疮小鼠脾脏Treg 细胞比例与24 h 尿蛋白量及血清ds-DNA抗体水平均呈高度负相关关系( P均<0.01),与血清ANA水平呈负相关关系( P<0.05)。结论狼疮小鼠脾脏Treg细胞比例可能与狼疮样病

  11. LUPUS ERITEMATOSO SISTEMICO Y EMBARAZO

    OpenAIRE

    Gutiérrez C.,Ricardo; Caro M,José; Pérez,Loreno; Ramírez, Javier

    2002-01-01

    Se presenta una revisión de los casos de embarazos asociados a lupus eritematoso sistémico (LES) atendidos en el Hospital base de Valdivia, en un período de 9 años. Se observó que 13 mujeres portadoras de lupus tuvieron un total de 35 embarazos en los que no hubo abortos o patologías obstétricas asociadas. La frecuencia de exacerbación del lupus fue similar a la observada en la literatura. Hubo un caso de bloqueo aurículo ventricular fetal, con resultado en muerte fetal tardía. Se destaca la ...

  12. Takotsubo cardiomyopathy is an acute cardiac syndrome induced by stress

    DEFF Research Database (Denmark)

    Fuchs, Annette Maria; Bang, Lia E; Holmvang, Lene

    2016-01-01

    Takotsubo cardiomyopathy (TTC) is an acute cardiac syndrome, characterized by transient left ventricular dysfunction often following a stressful event in post-menopausal women. Symptoms are indistinguishable from myocardial infarction. However, TTC patients do not have a culprit lesion on acute...

  13. Btk inhibition treats TLR7/IFN driven murine lupus.

    Science.gov (United States)

    Bender, Andrew T; Pereira, Albertina; Fu, Kai; Samy, Eileen; Wu, Yin; Liu-Bujalski, Lesley; Caldwell, Richard; Chen, Yi-Ying; Tian, Hui; Morandi, Federica; Head, Jared; Koehler, Ursula; Genest, Melinda; Okitsu, Shinji L; Xu, Daigen; Grenningloh, Roland

    2016-03-01

    Bruton's tyrosine kinase (Btk) is expressed in a variety of immune cells and previous work has demonstrated that blocking Btk is a promising strategy for treating autoimmune diseases. Herein, we utilized a tool Btk inhibitor, M7583, to determine the therapeutic efficacy of Btk inhibition in two mouse lupus models driven by TLR7 activation and type I interferon. In BXSB-Yaa lupus mice, Btk inhibition reduced autoantibodies, nephritis, and mortality. In the pristane-induced DBA/1 lupus model, Btk inhibition suppressed arthritis, but autoantibodies and the IFN gene signature were not significantly affected; suggesting efficacy was mediated through inhibition of Fc receptors. In vitro studies using primary human macrophages revealed that Btk inhibition can block activation by immune complexes and TLR7 which contributes to tissue damage in SLE. Overall, our results provide translational insight into how Btk inhibition may provide benefit to a variety of SLE patients by affecting both BCR and FcR signaling.

  14. Class Ⅱ lupus nephritis with nephrotic syndrome%伴肾病综合征Ⅱ型狼疮性肾炎的临床病理特征

    Institute of Scientific and Technical Information of China (English)

    刘瑾; 陈惠萍; 陈浩; 曾彩虹; 刘正钊; 章海涛; 刘志红; 胡伟新

    2012-01-01

    目的:比较临床表现肾病综合征(NS)和非NS的Ⅱ型狼疮性肾炎(LN)患者的临床、病理特征和预后. 方法:回顾性分析经肾活检病理确诊为Ⅱ型LN患者124例,其中女性112例,男性12例,平均年龄29.2±11.4岁,根据尿蛋白定量分为NS组(尿蛋白定量≥3 g/d,血清白蛋白<30 g/L)和非NS组(尿蛋白定量<3g/d,血清白蛋白≥30 g/L,伴或不伴镜下血尿).比较两组患者的临床、免疫学指标、肾脏病理特征及预后. 结果:124例Ⅱ型LN中NS组27例(21.8%),非NS组97例(78.2%),两组患者性别、发病年龄和病程无差异.NS组以肾损害为首发症状(77.8%vs15.5%,P<0.01)及病程中出现急性肾损伤的比例(29.6%vs0,P<0.01)均显著高于非NS组,而皮疹(40.7% vs 69.1%,P<0.01)、发热(14.8%vs62.9%,P<0.01)和关节炎(29.6%vs75.3%,P<0.01)的发生率及血清抗-dsDNA阳性率(29.6%vs52.6%,P<0.05)明显低于非NS组.肾活检病理NS组患者肾小球中重度系膜增生的比例显著低于非NS组(7.4%vs59.8%,P<0.01),电镜下肾小球足细胞足突广泛融合的比例显著高于非NS组(88.9%vs0,P<0.01).NS组和非NS组激素治疗均获得高缓解率(100%vs98.4%,P=0.882),但NS组复发率显著高于非NS组(69.9%vs33.3%,P<0.01),两组分别随访8~125月(中位时间55月)和6~274月(中位时间57月),均未发生终末期肾病.7例复发者重复肾活检,NS组2例病理类型均未转型,非NS组5例均发生转型. 结论:表现为NS的Ⅱ型LN其本质为足细胞病,非NS的Ⅱ型LN为系膜增生性病变,两者临床表现、免疫学异常及病理转型的显著差异表明两者的发生机制不同,狼疮足细胞损伤的机制有待进一步研究.%Objective: To investigate the clinical characteristics, renal histological features and the prognosis in patients of Class Ⅱ LN with nephrotic syndrome. Methodology;One hundred twenty four cases(112 females, 12 males, mean age 29. 22 ± 11

  15. 系统性红斑狼疮继发干燥综合症患者临床分析%Clinical Analysis of Sjogren's Syndrome Secondary to Systemic Lupus Erythernatosus

    Institute of Scientific and Technical Information of China (English)

    冯艳; 李志军

    2011-01-01

    Objective To investigate the clinical and serologic features of patients with Sjogren' s syndrome(SS) secondary to systemic lupus erythematosus(SLE), and compare to the features of patients with primary Sjogren' s syndrome(pSS) or with SLE. Methods The patients met both SLE and SS diagnostic criteria( SS secondary to SLE,SLE-SS) were selected from 208 patients with SLE, and the clinical and serologic features were compared to those of patients only with SLE or pSS. Results Twenty six patients satisfied SS diagnostic criteria and are all secondary to SLE. Compared with the SLE patients without SS group, the patients with SLE-SS were older, with a higher frequency of arthritis and pulmonary diseases, hypergtobulinemia, CRP, anti-Ul RNP,anti-SSA52 and anti-SSA60. Compared with the pSS patients ,SLE-SS patients were younger,with a higher frequency of arthritis,Raynaud's phenomenon, mucocutaneous diseases, hypocomplementemia; high positive rate of SmD1, dsDNA and SSA60; low leucocyte amount,low neutrophil amount and low platelet amount. Conclusion SLE-SS patients have distinct clinical and laboratory features that differ from SLE or pSS patients.%目的 探讨系统性红斑狼疮(SLE)继发干燥综合征(SS)患者的临床及实验特征,并与SLE及原发性干燥综合征(pSS)患者进行比较,了解SLE继发SS患者的临床及实验室特点.方法 入选SLE患者208例,参照SS诊断标准,将满足SS及SLE诊断标准的患者(SLE-SS)与不伴SS的SLE患者(单纯SLE)及pSS患者进行比较.结果 在208例SLE患者中,有26例(12.5%)满足SS的诊断标准且均为继发.与单纯SLE患者比较,SLE-SS患者年龄大,关节炎、肺部受累发生率高,高球蛋白血症、CRP、抗U1snRNP、抗SSA52、抗SSA60抗体阳性率高.与pSS患者相比,SLE-SS患者年龄小,关节炎、雷诺现象、皮肤黏膜病变发生率高.白细胞降低、中性粒细胞降低、血小板降低、低补体血症阳性率高,SmD1、dsDNA、SSA60阳性率高.结论 SLE

  16. 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.

  17. 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

  18. [Psychotic disorder induced by Fahr's syndrome: a case report].

    Science.gov (United States)

    El Hechmi, S; Bouhlel, S; Melki, W; El Hechmi, Z

    2014-06-01

    Fahr's syndrome is a rare disorder characterized by abnormal deposits of calcium in areas of the brain that control movement, including the basal ganglia and the cerebral cortex associated with many neurological and psychiatric abnormalities such as a rigid hypokinetic syndrome, mood disorders and cognitive impairment. Fahr's syndrome is secondary to some disorders, such as hypoparathyroidism. We report the case of a 56 year-old man, with a history of cataract, who was admitted to our psychiatric hospital for the first time in his life because of psychotic symptoms associated with irritability and aggressiveness. Since the age of 38 the patient had become nervous, 10 years later he developed tonic-clonic seizures. Two months ago, he began expressing delusions of persecution against his wife and sons and making fugues. According to his family during this period, he was agitated, aggressive, and suffered from insomnia and anorexia. The general and psychiatric examination showed an upright and bronzed patient with neglected hygiene. He was indifferent to his environment and expressed poor mimics and gestures. He was anxious, suspicious and not very talkative. He was conscious but his attention was slightly decreased. Moreover, he was not aware of his problems. The neurological examination showed extrapyramidal syndrome with postural tremor and cerebellar ataxia. A cranial computed tomography brain scan found bilateral, symmetric basal ganglia calcifications, in favour of Fahr's syndrome. Phosphocalcic investigations revealed low concentration of serum calcium at 1.01mmol/L (normal 2.15 to 2.57mmol/L) and hyperphosphoremia at 2.69mmol/L (normal 0.81 to 1.55mmol/L). He also had low concentrations of 25-OH vitamin as well as decreased urinary levels of phosphate and calcium. The blood level of parathyroid hormone was 0ng/L. The diagnosis of Fahr's syndrome, revealing a hypoparathyroidism was posed. He was supplemented with calcium and alpha cholecalciferol and treated

  19. Herpes Zoster-Induced Ogilvie’s Syndrome

    Directory of Open Access Journals (Sweden)

    Irfan Masood

    2015-01-01

    Full Text Available Ogilvie’s syndrome due to herpes zoster infection is a rare manifestation of VZV reactivation. The onset of rash of herpes zoster and the symptoms of intestinal obstruction can occur at different time intervals posing a significant diagnostic challenge resulting in avoidable surgical interventions. Herein, we describe a case of 35-year-old male who presented with 6-day history of constipation and colicky abdominal pain along with an exquisitely tender and vesicular skin eruption involving the T8–T11 dermatome. Abdominal X-ray and ultrasound revealed generalized gaseous distention of the large intestine with air up to the rectum consistent with paralytic ileus. Colonoscopy did not show any obstructing lesion. A diagnosis of Ogilvie’s syndrome associated with herpes zoster was made. He was conservatively managed with nasogastric decompression, IV fluids, and acyclovir. The patient had an uneventful recovery and was later discharged.

  20. Hypersensitivity to proton pump inhibitors: lansoprazole-induced Kounis syndrome.

    Science.gov (United States)

    Vlahos, Nicholas P; Vavilis, George K; Giannelou, Ageliki G; Georgopoulou, Christina N; Kommata, Varvara J; Kougias, Constantinos T; Tsartsalis, Dimitrios N; Kounis, George N; Mazarakis, Andreas; Batsolaki, Maria; Gouvelou-Deligianni, Geogia V; Hahalis, George; Kounis, Nicholas G

    2009-05-29

    Proton pump inhibitors are commonly used in clinical practice for the treatment of peptic ulcer and gastroesophageal reflux and are well tolerated by the patients. Their use is rarely associated with hypersensitivity and anaphylactic reactions. According to the reports in the Uppsala Monitoring Center database the frequency of hypersensitivity reactions out of all reported adverse reactions for proton pump inhibitors and H2-histamine receptor antagonists was between 0.2% and 0.7%. A few cases of hypersensitivity to lansoprazole have been reported. We report a patient who developed Kounis syndrome after taking 30 mg of lansoprazole. This is the first report of Kounis syndrome associated with lansoprazole administration in the world literature.

  1. Cyclosporin-A induced Posterior Reversible Encephalopathy Syndrome

    Directory of Open Access Journals (Sweden)

    Saeed Bassam

    2008-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a recently proposed clinico-neuroradiological entity observed in a variety of clinical settings such as cyclosporin A (CsA neurotoxicity. We report a 3.5-year-old Syrian boy in whom steroid-resistant focal segmental glomerulosclerosis (FSGS was recently diagnosed. The patient remitted his nephrotic syndrome after 10 days of CsA administration. However, he shortly developed altered mental status, visual impairment, focal neurological deficits and seizures. We discontinued CsA that resulted in complete reversal of the patient′s encephalopathical condition over a period of 4 months. We conclude that PRES should be suspected in immunosuppresed patients with kidney disease if they have a sudden episode of neurological symptoms.

  2. Neuromodulation of neuropathic pain syndrome induced by elapidae (cobra) envenomation.

    Science.gov (United States)

    Stretanski, Michael F

    2009-01-01

    Objectives.  This study aims to demonstrate the utility of spinal cord stimulation in a neuropathic pain syndrome and overall decline in health and functional independence following elapid envenomation in a morbidly obese, insulin-dependent diabetic. Materials and Methods.  A two-lead, 16-electrode constant-current, independently controlled system is placed in the mid-cervical spine. Results.  Noted were a improvement in overall health status with better glycemic control and return to work status in response to adequate pain control. Conclusions.  The case serves as a model for other orphan pain cases with a seemingly esoteric etiology and adds to the existing body of literature that spinal cord stimulation and neuromodulation, in general, has a wide-ranging applicability peripheral neuropathic pain syndromes.

  3. 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.

  4. 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.

  5. 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.

  6. "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

  7. "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

  8. 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...

  9. 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...

  10. "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

  11. Self-induced vomiting in X-linked {alpha}-thalassemia/mental retardation syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kurosawa, Kenji; Akatsuka, Akira; Ochiai, Yukikatsu [Jikei Univ. School of Medicine, Tokyo (Japan)] [and others

    1996-06-14

    This report poses the question of whether the vomiting observed in X-linked {alpha}-thalassemia/mental retardation syndrome could be self-induced. The authors present a case history which seems to support this hypothesis. 5 refs., 1 fig.

  12. Improved survival of TNF-deficient mice during the zymosan-induced multiple organ dysfunction syndrome.

    NARCIS (Netherlands)

    Volman, T.J.H.; Hendriks, T.; Verhofstad, A.A.J.; Kullberg, B.J.; Goris, R.J.A.

    2002-01-01

    The purpose of the study was to investigate the course of the zymosan-induced multiple organ dysfunction syndrome (MODS) in the absence of tumor necrosis factor (TNF) in a murine model. Tumor Necrosis Factor-alpha-lymphotoxin-a knockout (TNF/LT-/-) mice (n = 36) and wild-type (TNF/LT+/+) mice (n =

  13. SUNCT syndrome in a patient with prolactinoma and cabergoline-induced attacks.

    Science.gov (United States)

    Jiménez Caballero, P E

    2007-01-01

    Short-lasting unilateral neuralgiform headache with conjuntival injection and tearing (SUNCT) syndrome is a rare trigeminal autonomic cephalalgia. We report a patient with prolactinoma and cabergoline-induced SUNCT attacks and the literature is reviewed for a better understanding of the pathophysiology.

  14. Antiphospholipid antibody syndrome and autoimmune diseases.

    Science.gov (United States)

    Ostrowski, Rochella A; Robinson, John A

    2008-02-01

    The arbitrary division between antiphospholipid antibody syndrome and secondary antiphospholipid antibody syndrome has not proven useful. Antiphospholipid antibodies in the absence of antiphospholipid antibody syndrome often occur as epiphenomena in many autoimmune diseases. They are very common in systemic lupus erythematosus. Antiphospholipid antibody syndrome is a significant comorbidity in lupus but is uncommon in Sjögren's syndrome, rheumatoid arthritis, scleroderma, and systemic vasculitis. Evidence is growing that antiphospholipid antibodies may have a pathogenic role in pulmonary hypertension and accelerated atherosclerosis of autoimmune diseases.

  15. Historia del Lupus

    Directory of Open Access Journals (Sweden)

    Alvaro Rodríguez Gama

    2004-09-01

    Se encontrarán los lectores con una gran cantidad y calidad de biografías sobre investigaciones, médicos y científicos que han enfrentado el reto de tratar de entender y tratar el lupus, desde los médicos clásicos como Hipócrates, Galeno y Celso, los protodermatólogos Daniel Turner, Jean Astruc, Antoine Lorry y Josef von Plenk; el primer dematológo Robert Willam y médicos de los siglos XVIII y XIX como Thomas Bateman, Jean Lous Alibert, Theódore Biett, Olive Rayer, Pierre Cazenave, Antoine Bazin, Ferdinand von Hebra, Moriz Kaposi, Ernest Besnier, Alfred Fournier, Louis Brocq, Jean Darier, el clasificador Jonathan Hutchinson, Paul Unna, William Osler, Emanuel Libman, George Baehr y así decenas de colosos de la ciencia médica, cuyas historias son descritas minuciosamente por el Dr. Iglesias...

  16. [Systemic lupus erythematodes].

    Science.gov (United States)

    Lukác, J; Rovenský, J; Lukácová, O; Kozáková, D

    2006-01-01

    Systemic lupus erythematodes (SLE) is chronic autoimmune disease, characteristic by production of autoantibodies against different autoantigens. Etiopathogenesis in not precise determinated, but genetic, immunologic, hormonal factors or influence of environment are assumed. It manifests by various symptoms and it can affect whichever organ or system in the body. Clinical manifestation are due chronic inflammation in the tissues, which is caused first of all by deposit of immunocomplex and by cytotoxic damage. At the last decades the mortality of patients with SLE is markly lower and their live is prolong. In spite of this diagnostic, to follow up and therapy of this disease is complicated and it requires the colaboration of more branches of medicine.

  17. Exogenous Cushing syndrome

    Science.gov (United States)

    Cushing syndrome - corticosteroid induced; Corticosteroid-induced Cushing syndrome; Iatrogenic Cushing syndrome ... Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the ...

  18. 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

  19. Anterior opercular syndrome induced by Epstein-Barr virus encephalitis.

    Science.gov (United States)

    Matsushima, Takashi; Nishioka, Kenya; Tanaka, Ryota; Yokoyama, Kazumasa; Hattori, Nobutaka

    2016-01-01

    We report a 19-year-old female presenting with fever, drooling, anarthria, and voluntary facial movement disruption, characteristic of anterior opercular syndrome (AOS). Serological examination revealed Epstein-Barr virus (EBV) infection following acute encephalitis with severe ataxia. A single-photon emission computerized tomography (SPECT) examination indicated hypoperfusion in the left perisylvian region, bilateral thalamus, occipital lobe, and cerebellum. This is the first report of AOS related to EBV encephalitis. SPECT was a useful method for detecting the damaged region of the operculum. In addition, AOS is a clinically distinct entity that may help us understand the mechanisms of language circuits within the operculum.

  20. Lupus, discoid on a child's face (image)

    Science.gov (United States)

    The round or disk shaped (discoid) rash of lupus produces red, raised patches with scales. The pores ( ... The majority (approximately 90%) of individuals with discoid lupus have only skin involvement as compared to more ...

  1. 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

  2. Blunting Autoantigen-induced FOXO3a Protein Phosphorylation and Degradation Is a Novel Pathway of Glucocorticoids for the Treatment of Systemic Lupus Erythematosus.

    Science.gov (United States)

    Lu, Mudan; Xu, Wei; Gao, Bo; Xiong, Sidong

    2016-09-16

    Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease affecting multiple organs. Glucocorticoids (GCs), the potent anti-inflammatory drugs, remain as a cornerstone in the treatment for SLE; nevertheless, their clinical efficacy is compromised by the side effects of long term treatment and resistance. To improve the therapeutic efficacy of GCs in SLE, it is important to further decipher the molecular mechanisms of how GCs exert their anti-inflammatory effects. In this investigation, FOXO3a was identified as a molecule that was down-regulated in the course of SLE. Of interest, GC treatment was found to rescue FOXO3a expression both in SLE mice and in SLE patients. Gain- and loss-of-function studies demonstrated that FOXO3a played a crucial role in GC treatment of SLE via inhibiting inflammatory responses. Further studies showed that the up-regulation of FOXO3a by GCs relied on the suppression of pI3K/AKT-mediated FOXO3a phosphorylation and the arrest of FOXO3a in the nucleus. Finally, our data revealed that FOXO3a was critical for GC-mediated inhibition of NF-κB activity, which might involve its interaction with NF-κB p65 protein. Collectively, these data indicated that FOXO3a played an important role in GC treatment of SLE by suppressing pro-inflammatory response, and targeting FOXO3a might provide a novel therapeutic strategy against SLE.

  3. 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...

  4. Exercise Induced Adipokine Changes and the Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Saeid Golbidi

    2014-01-01

    Full Text Available The lack of adequate physical activity and obesity created a worldwide pandemic. Obesity is characterized by the deposition of adipose tissue in various parts of the body; it is now evident that adipose tissue also acts as an endocrine organ capable of secreting many cytokines that are though to be involved in the pathophysiology of obesity, insulin resistance, and metabolic syndrome. Adipokines, or adipose tissue-derived proteins, play a pivotal role in this scenario. Increased secretion of proinflammatory adipokines leads to a chronic inflammatory state that is accompanied by insulin resistance and glucose intolerance. Lifestyle change in terms of increased physical activity and exercise is the best nonpharmacological treatment for obesity since these can reduce insulin resistance, counteract the inflammatory state, and improve the lipid profile. There is growing evidence that exercise exerts its beneficial effects partly through alterations in the adipokine profile; that is, exercise increases secretion of anti-inflammatory adipokines and reduces proinflammatory cytokines. In this paper we briefly describe the pathophysiologic role of four important adipokines (adiponectin, leptin, TNF-α, and IL-6 in the metabolic syndrome and review some of the clinical trials that monitored these adipokines as a clinical outcome before and after exercise.

  5. Quetiapine and clarithromycin-induced neuroleptic malignant syndrome.

    Science.gov (United States)

    Christodoulou, Christos; Margaritis, Dimitris; Makris, Gerasimos; Kavatha, Dimitra; Efstathiou, Vasiliki; Papageorgiou, Charalabos; Douzenis, Athanasios

    2015-01-01

    We report a case of neuroleptic malignant syndrome possibly caused by the combined administration of quetiapine and clarithromycin in a 75-year-old male patient. He was receiving quetiapine regularly. Two days before his admission to the hospital, he had been feverish and started receiving clarithromycin without consulting a doctor. Clarithromycin administration was interrupted 3 days after his admission because it was ineffective and because his clinical state was deteriorating. The patient presented altered level of consciousness and excessive muscular rigidity on his limbs, while he remained feverish (38.7 °C). Laboratory abnormalities included elevated serum creatine phosphokinase level (5.387 U/L), leukocytosis, and low serum iron. The patient was diagnosed with neuroleptic malignant syndrome, and quetiapine was immediately discontinued. After the following days, his muscle rigidity and mental status ameliorated, his fever withdrew, and his laboratory findings improved. The various features of the case are discussed in view of the fact that the concomitant administration of cytochrome 3A4 inhibitors, such as clarithromycin, is suggested to cause an increase of plasma concentrations of quetiapine. Thus, physicians should have a high index of suspicion of the interactions of commonly administered medications.

  6. Beneficial effect of Curcumin in Letrozole induced polycystic ovary syndrome

    Directory of Open Access Journals (Sweden)

    P. Sushma Reddy

    2016-04-01

    Conclusion: Curcumin showed beneficial effects in Letrozole induced PCOS in female Wistar rats. Its effect was comparable to that of Clomiphene citrate, most widely used treatment for ovulation induction in PCOS condition.

  7. Lamotrigine-induced hypersensitivity syndrome with histologic features of cd30+ lymphoma

    Directory of Open Access Journals (Sweden)

    Farid Stephan

    2016-01-01

    Full Text Available Drug rash with eosinophilia and systemic symptoms (DRESS syndrome or drug-induced hypersensitivity syndrome (DIHS is a severe adverse drug reaction. It can present with clinical, paraclinical, and histological findings mimicking skin and/or systemic lymphomas. We report the first case of a lamotrigine-induced DRESS with histologic features of a cutaneous CD30+ lymphoma. The patient responded well to a tapering course of oral steroids. This case highlights the atypical presentation of a lamotrigine-induced DRESS/DIHS in the presence of a cutaneous and a lymph node CD30 + lymphocytic infiltrate mimicking systemic lymphoma. Pathologists and clinicians must be aware of this “lymphomatous” presentation of drug reactions.

  8. Lupus vasculopathy: Diagnostic, pathogenetic and therapeutic considerations.

    Science.gov (United States)

    Gonzalez-Suarez, M L; Waheed, A A; Andrews, D M; Ascherman, D P; Zeng, X; Nayer, A

    2014-04-01

    A rare form of vascular disease in systemic lupus erythematosus (SLE), lupus vasculopathy is characterized by necrosis and accumulation of immunoglobulins (IGs) and complements in the wall of arterioles and small arteries resulting in luminal narrowing. Lupus vasculopathy often accompanies lupus nephritis and portends a poor prognosis. Although there is general agreement on the treatment of lupus nephritis, effective treatment strategies for lupus vasculopathy remain to be defined. We report a 20-year-old woman with SLE who presented with generalized tonic-clonic seizure. Her immunosuppressive regimen consisted of mycophenolate mofetil, prednisone and hydroxychloroquine. On physical examination, she was Cushingoid in appearance and hypertensive. Laboratory tests indicated renal disease. Coagulation studies disclosed de novo lupus anticoagulant. Magnetic resonance imaging of the brain demonstrated acute focal cerebral hemorrhage. Echocardiography revealed reduced ejection fraction and severe mitral regurgitation. Despite high-dose glucocorticoids and mycophenolate mofetil, renal function remained poor. Kidney biopsy demonstrated lupus vasculopathy and glomerulonephritis. Plasma exchange therapy and intravenous cyclophosphamide were administered. Over the ensuing four weeks, renal function improved, complement levels increased, autoantibody titers decreased and lupus anticoagulant disappeared. In conclusion, lupus vasculopathy can occur in SLE despite a heavy immunosuppressive regimen. Antiphospholipid antibodies might be involved in the pathogenesis of lupus vasculopathy. Plasma exchange therapy in conjunction with intravenous cyclophosphamide may represent an effective treatment strategy for lupus vasculopathy.

  9. 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.

  10. 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...

  11. Thrombotic and hemorrhagic complications in children with the lupus anticoagulant.

    Science.gov (United States)

    Bernstein, M L; Salusinsky-Sternbach, M; Bellefleur, M; Esseltine, D W

    1984-12-01

    Endogenous circulating anticoagulants are unusual in children without a congenital factor deficiency. In particular, the lupus anticoagulant has only rarely been reported in children. Despite its functioning in vitro to prolong the partial thromboplastin time, patients more frequently have problems with thrombosis than bleeding, unless there is a coexistent prothrombin deficiency or thrombocytopenia. We report the cases of three children with the lupus anticoagulant. Two children had associated thromboses. One had a thrombosis of the iliofemoral system and the other had a partial Budd-Chiari syndrome, a thrombosis of the deep calf veins and ureteric obstruction. The third child had a concomitant prothrombin deficiency and bleeding after tooth extraction. Associated findings in these patients included a positive antinuclear antibody test in two, a positive anti-DNA antibody test in two, a false-positive VDRL test in two, and an antiphospholipid antibody test in two.

  12. 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.

  13. 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.

  14. Pregnancy Outcomes in Chinese Patients with Systemic Lupus Erythematosus (SLE: A Retrospective Study of 109 Pregnancies.

    Directory of Open Access Journals (Sweden)

    Ming Ku

    Full Text Available Systemic lupus erythematosus (SLE is a multisystem autoimmune disease that primarily affects women during their reproductive years. The interaction between SLE and pregnancy remains debated. The objective of this study was to analyze the fetal and maternal outcomes of Chinese women with SLE. A total of 109 pregnancies in 83 SLE patients from June 2004 to June 2014 at a tertiary university hospital were reviewed retrospectively. Patients' characteristics, clinical and laboratory data during pregnancy were obtained from electronic medical records. After exclusion of elective abortions, the live birth rate was 61.5%. Significantly, APS (antiphospholipid syndrome, disease activity, hypertension, hypocomplementemia, thrombocytopenia, and anemia during pregnancy were more commonly observed in fetal loss pregnancies than in live birth pregnancies. Compared to the 64 women with a history of SLE, 19 women with new-onset lupus during pregnancy had worse pregnancy outcome. Furthermore, the 64 patients with a history of SLE were divided into lupus nephritis group and SLE group (non-renal involvement. We found that the lupus nephritis group had worse maternal outcome than the SLE group. We conclude that new-onset lupus during pregnancy predicts both adverse maternal and fetal outcomes, while a history of lupus nephritis predicts adverse maternal outcomes. It is essential to provide SLE women with progestational counseling and regular multispecialty care during pregnancy.

  15. Pregnancy Outcomes in Chinese Patients with Systemic Lupus Erythematosus (SLE): A Retrospective Study of 109 Pregnancies.

    Science.gov (United States)

    Ku, Ming; Guo, Shuiming; Shang, Weifeng; Li, Qing; Zeng, Rui; Han, Min; Ge, Shuwang; Xu, Gang

    2016-01-01

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that primarily affects women during their reproductive years. The interaction between SLE and pregnancy remains debated. The objective of this study was to analyze the fetal and maternal outcomes of Chinese women with SLE. A total of 109 pregnancies in 83 SLE patients from June 2004 to June 2014 at a tertiary university hospital were reviewed retrospectively. Patients' characteristics, clinical and laboratory data during pregnancy were obtained from electronic medical records. After exclusion of elective abortions, the live birth rate was 61.5%. Significantly, APS (antiphospholipid syndrome), disease activity, hypertension, hypocomplementemia, thrombocytopenia, and anemia during pregnancy were more commonly observed in fetal loss pregnancies than in live birth pregnancies. Compared to the 64 women with a history of SLE, 19 women with new-onset lupus during pregnancy had worse pregnancy outcome. Furthermore, the 64 patients with a history of SLE were divided into lupus nephritis group and SLE group (non-renal involvement). We found that the lupus nephritis group had worse maternal outcome than the SLE group. We conclude that new-onset lupus during pregnancy predicts both adverse maternal and fetal outcomes, while a history of lupus nephritis predicts adverse maternal outcomes. It is essential to provide SLE women with progestational counseling and regular multispecialty care during pregnancy.

  16. Mycophenolate mofetil for drug-induced vanishing bile duct syndrome

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Amoxicillin/clavulanate is associated with liver injury,mostly of a cholestatic pattern. While outcomes are usually benign, progression to cirrhosis and death has been reported. The role of immunosuppressive therapy for patients with a protracted course is unclear. We report the case of an elderly patient who developed prolonged cholestasis secondary to amoxicillin/clavulanate. Vanishing bile duct syndrome was confirmed by sequential liver biopsies. The patient responded to prednisone treatment,but could not be weaned off corticosteroids, even when azathioprine was added. Complete withdrawal of both prednisone and azathioprine was possible by using mycophenolate mofetil, an inosine monophosphate dehydrogenase inhibitor. Sustained remission has been maintained for more than 3 years with low-dose mycophenolate mofetil.

  17. Clozapine-induced rabbit syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Cicek Hocaoglu

    2009-10-01

    Full Text Available Rabbit syndrome (RS is an antipsychoticinduced rhythmic motion of the mouth/lips resembling the chewing movements of a rabbit. The movement consists of a vertical-only motion, at about 5 Hz, with no involvement of the tongue. Long-term exposure to typical antipsychotics has clearly been associated with RS, but little is known of the risk of RS due to exposure to newer atypical antipsychotics. There have been isolated reports of RS in patients treated with the atypical agents risperidone, aripiprazole, olanzapine, and clozapine. We present the case history of a 44-year old female patient treated for paranoid schizophrenia for 22 years and RS during her last 10-month clozapine treatment. Background information from the literature is also discussed.

  18. Stress-induced Cushing's syndrome in fur-chewing chinchillas.

    Science.gov (United States)

    Tisljar, Marina; Janić, D; Grabarević, Z; Simpraga, Borka; Marinculić, A; Pinter, Ljiljana; Janicki, Z; Nemanic, Ankica

    2002-01-01

    One of the most serious problems in the chinchilla industry is 'fur-chewing', when the chinchilla bites off areas of its own or some other animal's fur. The condition generally develops in both genders at the age of 6-8 months. In chinchilla farms in Croatia an incidence of 15-20% has been observed. A pathomorphological, microbiological and parasitological investigation was conducted on eleven 6- to 11-month-old chinchillas of both sexes with clinical symptoms of 'fur-chewing' and three chinchillas without such signs. Histopathology of the adrenal glands and of the chewed skin revealed changes typical of Cushing's syndrome in 'fur-chewed' chinchillas, such as hyperkeratinisation of the epidermis, epidermal atrophy, pronounced follicular and sebaceous gland atrophy, hyperkeratinisation of the follicles with comedo formations and the presence of calcium salts in subcutis.

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

    Directory of Open Access Journals (Sweden)

    Nigel P. Murray

    2012-01-01

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

  20. [2016 review on catastrophic antiphospholipid syndrome].

    Science.gov (United States)

    Costedoat-Chalumeau, Nathalie; Coutte, Laetitia; Le Guern, Véronique; Morel, Nathalie; Leroux, Gaelle; Paule, Romain; Mouthon, Luc; Piette, Jean-Charles

    2016-12-01

    The catastrophic antiphospholipid syndrome (CAPS) develops in at least 1% of patients with antiphospholipid syndrome, either primary or associated with systemic lupus erythematosus. CAPS reveals the antiphospholipid syndrome in about 50% of cases. The CAPS is characterized by rapidly-progressive widespread thromboses mainly affecting the microvasculature in the presence of antiphospholipid antibodies. In a few days, the patients develop multiorgan failure with renal insufficiency with severe hypertension, pulmonary, cerebral, cardiac, digestive and/or cutaneous involvement. The vital prognosis is frequently engaged. CAPS is often precipitated by infectious diseases, surgical procedures and/or withdrawal or modification of the anticoagulation. CAPS overall mortality rate has decreased and is currently below 30%. The main differential diagnoses are other thrombotic microangiopathies, and heparin-induced thrombocytopenia. The treatment of CAPS consists of the association of anticoagulation and steroids, plus plasma exchange and/or intravenous immunoglobulins. Cyclophosphamide is added only in patients with active systemic lupus erythematosus. The potential contribution of some additional therapies (rituximab, eculizumab or sirolimus) needs to be assessed. The prevention of CAPS is essential and is based upon the adequate management of the perioperative period when surgery cannot be avoided, the prompt treatment and the prevention with immunization of infections and the education of patients with antiphospholipid syndrome, especially for the management of oral anticoagulants.