Background
Schnitzler syndrome is characterized by an urticarial rash, a monoclonal gammopathy, and clinical, histological, and biological signs of neutrophil‐mediated inflammation. The aim of this ...study was to assess the applicability and validity of the existing diagnostic criteria in real‐life patients.
Methods
This multicentric study was conducted between 2009 and 2014 in 14 hospitals in which patients with Schnitzler syndrome or controls with related disorders were followed up. We compared the sensitivities and specificities and calculated the positive and negative predictive values of the Lipsker and of the Strasbourg criteria for the patients with Schnitzler syndrome and for the controls. We included 42 patients with Schnitzler syndrome, 12 with adult‐onset Still's disease, 7 with cryopyrin‐associated periodic disease, 9 with Waldenström disease, and 10 with chronic spontaneous urticaria.
Results
All patients with Schnitzler syndrome met the Lipsker criteria. According to the Strasbourg criteria, 34 patients had definite Schnitzler syndrome, five had probable Schnitzler syndrome, and three did not meet the criteria. One control met the Lipsker criteria and had probable Schnitzler syndrome according to the Strasbourg criteria. Sensitivity and specificity of the Lipsker criteria were 100% and 97%, respectively. For the Strasbourg criteria, sensitivity for definite and probable diagnosis was 81% and 93%, respectively, with a corresponding specificity of 100% and 97%.
Conclusion
Diagnostic criteria currently in use to diagnose Schnitzler syndrome are reliable. More investigations must be done to attest their efficiency in patients with recent‐onset manifestations.
Objective: To assess the tolerance and efficacy of rituximab in patients with various autoimmune diseases seen in daily rheumatological practice. Methods: 866 rheumatology and internal medicine ...practitioners were contacted by email to obtain the files of patients treated with rituximab for systemic autoimmune diseases. Patients with lymphoma were analysed if the evolution of the autoimmune disease could be evaluated. Results: In all, 43 of 49 cases could be analysed, including 14 with rheumatoid arthritis (RA), 13 with systemic lupus erythematosus (SLE), six with primary Sjögren’s syndrome (pSS), five with systemic vasculitis, and five with other autoimmune diseases. Rituximab was prescribed for lymphoma in two patients with RA and two with pSS. In the 39 other cases, rituximab was given because of the refractory character of the autoimmune disease. The mean follow up period was 8.3 months (range 2 to 26). There were 11 adverse events in 10 patients and treatment had to be discontinued in six. Efficacy was observed in 30 patients (70%): RA 11, SLE 9, pSS 5, vasculitis 2, antisynthetase syndromes 2, sarcoidosis 1. The mean decrease in corticosteroid intake was 9.5 mg/d (range 0 to 50) in responders. Seven patients experienced relapse after mean 8.1 months (5 to 15). Three patients died because of refractory autoimmune disease. Conclusions: Despite absence of marketing authorisation, rituximab is used to treat various refractory autoimmune diseases in daily rheumatological practice. This study showed good tolerance and short term clinical efficacy, with marked corticosteroid reduction in patients with SLE, pSS, vasculitis, and polymyositis.
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by arterial and/or venous thromboses and/or pregnancy-associated morbidity. Some patients develop only obstetric ...complications (obstetric APS), but data on the frequency of thrombotic events during the follow-up of these patients are scarce. This study was undertaken to evaluate the rate of thrombotic events after obstetric APS diagnosis according to the 2006 revised criteria. In total, 32 obstetric APS patients were retrospectively studied, with mean follow-up of 50 ± 37 months. After delivery, aspirin was prescribed to all patients as primary thrombosis prevention. The thrombosis rate was 3.3/100 patient-years and was 4.6, 4.5 and 10/100 patient-years when we considered at least two antiphospholipid antibody positivities (among lupus anticoagulant, anticardiolipin and anti-β2-glycoprotein-I), antinuclear antibody positivity or systemic lupus erythematosus-associated APS patients, respectively. The thrombosis rate was high after obstetric APS diagnosis, even for patients taking aspirin. Larger, prospective studies are needed to confirm this high frequency and determine the associated risk factors.
The association of sarcoidosis and auto-immune thyroid disease has been reported. We report a 29-year-old woman, treated for hypothyroidism caused by thyrotropin-receptor blocking antibodies, who ...developed a sarcoidosis (Löfgren's syndrome). Auto-immune thyroid diseases and sarcoidosis could share common pathogenic mechanisms.
L’association sarcoïdose et pathologie thyroïdienne auto-immune semble être plus que fortuite. Nous rapportons le cas d’une patiente suivie pour maladie de Basedow avec des anticorps de variété ...bloquante entraînant une hypothyroïdie qui développe secondairement un syndrome de Löfgren. Des mécanismes immunitaires communs pourraient être à l’origine de ces deux pathologies rares.
The association of sarcoidosis and auto-immune thyroid disease has been reported. We report a 29-year-old woman, treated for hypothyroidism caused by thyrotropin-receptor blocking antibodies, who developed a sarcoidosis (Löfgren’s syndrome). Auto-immune thyroid diseases and sarcoidosis could share common pathogenic mechanisms.
Ischémie digitale sous bromocriptine Mekinian, A.; Kyndt, X.; Girard-Buttaz, I. ...
La revue de medecine interne,
10/2008, Volume:
29, Issue:
10
Journal Article
Peer reviewed
La prescription de bromocriptine dans le post-partum peut être associée à des accidents vasculaires cérébraux et des infarctus du myocarde. Nous rapportons l’observation d’une patiente présentant une ...ischémie digitale sous bromocriptine.
Mme D, âgée de 28 ans présente un tableau d’ischémie des trois derniers doigts de la main cinq jours après introduction d’un traitement par bromocriptine. L’IRM retrouve également un accident ischémique dans le territoire de l’artère cérébelleuse postéroinférieure droite. Les explorations à la recherche d’une cardiopathie embolique, d’une atteinte des gros troncs artériels ou d’une thrombophilie restent négatives. L’évolution est favorable après l’arrêt de la bromocriptine.
La bromocriptine est un agoniste dopaminergique dérivé de l’ergot de seigle. Une réponse vasoconstrictrice paradoxale peut être rarement à l’origine de complications vasculaires, dont un tableau d’ischémie digitale.
Bromocriptin has been associated with stroke and myocardial infarction in the postpartum period. We report on the case of a patient who developed digital ischemia while receiving this drug.
Mrs D, 28 years old presented with digital ischemia occuring five days after the introduction of bromocriptin. Magnetic resonance imaging also displayed stroke in the area of the right posterior cerebellar artery. The course was favourable after discontinuation of the drug.
Bromocriptin is an ergot derivative with dopaminergic agonist properties. A paradoxical vasoconstriction is rarely associated with vascular ischemic complications, including digital ischemia.
Bromocriptin has been associated with stroke and myocardial infarction in the postpartum period. We report on the case of a patient who developed digital ischemia while receiving this drug.
Mrs D, 28 ...years old presented with digital ischemia occurring five days after the introduction of bromocriptin. Magnetic resonance imaging also displayed stroke in the area of the right posterior cerebellar artery. The course was favourable after discontinuation of the drug.
Bromocriptin is an ergot derivative with dopaminergic agonist properties. A paradoxical vasoconstriction is rarely associated with vascular ischemic complications, including digital ischemia.
Tubulointerstitial nephritis is the most common renal complication in primary Sjögren's syndrome (SS). It is usually associated with symptoms of distal tubular dysfunction, type I (distal) renal ...tubular acidosis (RTA) and nephrogenic diabetes insipidus. Proximal tubular abnormalities are considered to be less frequent, and Fanconi's syndrome has been only exceptionally reported in patients with SS. We describe 2 patients with primary SS, characterized by xerostomia, dry eyes, extensive lymphocytic infiltrate on salivary gland biopsy, positive tests for anti-SSA/SSB antibodies and/or antinuclear antibodies, who presented in renal failure with proteinuria, microscopic hematuria and type I RTA. Further studies revealed proximal tubular dysfunction, including renal glucosuria, generalized aminoaciduria, phosphaturia, uricosuria, together with proximal (type II) RTA in 1 case. Neither of these patients had Bence Jones proteinuria or monoclonal gammopathy. Kidney biopsy showed focal proximal tubulitis, associated with proximal tubular cell atrophy and dedifferentiation, and diffuse interstitial nephritis with fibrosis. No significant glomerular or peritubular deposits of immunoglobulin light or heavy chain were observed. These findings demonstrate that diffuse, distal and proximal, tubular dysfunction may occur in patients with SS and interstitial nephritis. Lymphocytic infiltration of proximal tubular cells is probably involved in the pathogenesis of Fanconi's syndrome in SS. However, the mechanisms involved in the alteration of sodium-dependent apical transports remain to be elucidated.
Adult Still's disease (ASD) is a rare systemic disorder characterized by fever, arthralgia, cutaneous rash, and lymphadenopathy, with high polymorphonuclear leucocytosis and low glycosylated ...ferritinaemia. Kidney involvement has been reported rarely. We present a patient with ASD who developed haemolytic uraemic syndrome (HUS). The 42-year-old patient was admitted for unexplained fever related to ASD according to Yamaguchi's classification criteria. As Still's disease was resistant to prednisone, high-dose intravenous immunoglobulins (IV Ig) were administered. During the follow-up the patient developed acute renal failure and non-immune haemolytic anaemia with high levels of antiphospholipid antibodies (IgG anticardiolipin antibodies and anti- 2 glycoprotein 1 antibodies). Renal biopsy disclosed thrombotic microangiopathy (TMA) with arteriolar and glomerular involvement. Treatment with steroids and intravenous IV Ig was reinitiated but renal function worsened towards end-stage renal failure. In this case, we suggest that antiphospholipid antibodies could have promoted arteriolar and glomerular TMA. HUS may be the cause of acute renal failure in Still's disease.
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