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Churg-Strauss Syndrome (churg-strauss + syndrome)
Selected AbstractsEosinophilic pneumonia in an asthmatic patient treated with omalizumab therapy: forme-fruste of Churg-Strauss syndrome?ALLERGY, Issue 9 2009M. Cazzola No abstract is available for this article. [source] Pediatric Churg-Strauss syndrome in MexicoPEDIATRIC PULMONOLOGY, Issue 4 2006Víctor Manuel Hernández-Bautista MD Abstract Churg-Strauss syndrome (CSS) is one of the rarest forms of vasculitis, and very rarely presents in the pediatric population. We present two cases of childhood CSS, both with hepatic and cardiac involvement. To our knowledge, these are the first two cases of CSS in the pediatric population described in Mexico. Pediatr Pulmonol. © 2006 Wiley-Liss, Inc. [source] Churg-Strauss syndrome revealed by acute abdominal painPEDIATRIC PULMONOLOGY, Issue 1 2001M. Berlioz MD Abstract We describe a 10-year-old girl with Churg-Strauss syndrome, who presented with acute abdominal pain, bloody diarrhea, and pulmonary infiltrates. She had a 6-year history of severe asthma. Bronchoalveolar lavage showed marked eosinophilia. She responded well to high-dose intravenous corticosteroid pulse therapy for 3 consecutive days, followed by oral steroids without developing major side effects. This case should remind pediatricians of the rare existence of this vasculitis in children. Relapse is not uncommon, and long-term careful supervision is necessary. Pediatr Pulmonol. 2001; 32:92,94. © 2001 Wiley-Liss, Inc. [source] Erratum: Hepatitis B vaccination and multiple sclerosis: a data mining perspectivePHARMACOEPIDEMIOLOGY AND DRUG SAFETY, Issue 1 2008Manfred Hauben MD Pharmacoepidemiology and Drug Safety 2007; 16: 943,945 DOI: 10.1002/pds.1420 If has come to our attention that page 945 of this Letter to the Editor contained an error in the text. The published sentence currently reads from line 3: For example12 reported "impressively large" observed/expected ratios for the association of leukotriene receptor modifiers with Churg-Strauss syndrome when they applied a data mining methodology to the US FDA AERS database, while13 failed to find any association using a nested case-control study in US managed care organization databases. It should read: For example DuMouchel et al.12 reported "impressively large" observed/expected ratios for the association of leukotriene receptor modifiers with Churg-Strauss syndrome when they applied a data mining methodology to the US FDA AERS database, while Harrold et al.13 failed to find any association using a nested case-control study in US managed care organization databases. We apologise for this anomaly. [source] Cardiac involvement in Churg-Strauss syndromeARTHRITIS & RHEUMATISM, Issue 2 2010Robert M. Dennert Objective Churg-Strauss syndrome (CSS) is a rare form of systemic vasculitis. Previous studies showing cardiac involvement in CSS patients were limited in the number of patients and were often based solely on clinical manifestations. The aim of the present study was to determine in detail the incidence of cardiac involvement in a large population of ambulatory CSS patients. Methods Thirty-two consecutive patients with CSS in remission (mean ± SD duration of disease between diagnosis and enrollment 6.1 ± 5.8 years, mean ± SD age 61 ± 10 years) who were previously unaware of cardiac involvement were compared with 32 randomly selected age- and sex-matched control subjects, using clinical evaluation, electrocardiography (EKG), echocardiography, and cardiac magnetic resonance imaging (MRI). Results Detailed cardiac evaluation revealed a 62% prevalence of cardiac involvement in CSS patients compared with 3% in controls (P < 0.001), with clinical symptoms in 26% and 3%, respectively (P = 0.009), EKG abnormalities in 66% and 3%, respectively (P < 0.001), and echocardiographic defects in 50% and 3%, respectively (P < 0.001). Cardiac MRI detected cardiac manifestations in 62% of CSS patients. In the presence of cardiac MRI abnormalities, echocardiography could detect cardiac involvement with a sensitivity of 83% and a specificity of 80%. The absence of symptoms or EKG abnormalities did not exclude cardiac involvement, because abnormalities could still be detected in 38% of these patients at the time of echocardiography or cardiac MRI. Conclusion These results demonstrate a high incidence of cardiac involvement in CSS patients. Systematic cardiac evaluation including detailed imaging is required to properly identify CSS patients with cardiac involvement. [source] Classification, presentation, and initial treatment of Wegener's granulomatosis in childhoodARTHRITIS & RHEUMATISM, Issue 11 2009David A. Cabral Objective To compare the criteria for Wegener's granulomatosis (WG) of the American College of Rheumatology (ACR) with those of the European League Against Rheumatism/Pediatric Rheumatology European Society (EULAR/PRES) in a cohort of children with WG and other antineutrophil cytoplasmic antibody (ANCA),associated vasculitides (AAVs), and to describe the interval to diagnosis, presenting features, and initial treatment for WG. Methods Eligible patients had been diagnosed by site rheumatologists (termed the "MD diagnosis") since 2004. This diagnosis was used as a reference standard for sensitivity and specificity testing of the 2 WG classification criteria. Descriptive analyses were confined to ACR-classified WG patients. Results MD diagnoses of 117 patients (82 of whom were female) were WG (n = 76), microscopic polyangiitis (n = 17), ANCA-positive pauci-immune glomerulonephritis (n = 5), Churg-Strauss syndrome (n = 2), and unclassified vasculitis (n = 17). The sensitivities of the ACR and EULAR/PRES classification criteria for WG among the spectrum of AAVs were 68.4% and 73.6%, respectively, and the specificities were 68.3% and 73.2%, respectively. Two more children were identified as having WG by the EULAR/PRES criteria than by the ACR criteria. For the 65 ACR-classified WG patients, the median age at diagnosis was 14.2 years (range 4,17 years), and the median interval from symptom onset to diagnosis was 2.7 months (range 0,49 months). The most frequent presenting features by organ system were constitutional (89.2%), pulmonary (80.0%), ear, nose, and throat (80.0%), and renal (75.4%). Fifty-four patients (83.1%) commenced treatment with the combination of corticosteroids and cyclophosphamide, with widely varying regimens; the remainder received methotrexate alone (n = 1), corticosteroids alone (n = 4), or a combination (n = 6). Conclusion The EULAR/PRES criteria minimally improved diagnostic sensitivity and specificity for WG among a narrow spectrum of children with AAVs. Diagnostic delays may result from poor characterization of childhood WG. Initial therapy varied considerably among participating centers. [source] The emergence of antineutrophil cytoplasmic antibodies may precede the clinical onset of Churg-Strauss syndromeARTHRITIS & RHEUMATISM, Issue 2 2009Jochen Zwerina MD No abstract is available for this article. [source] Notice of retraction: Increased expression of TRAIL receptor 3 on eosinophils in Churg-Strauss syndrome (Arthritis Rheum 2007;56:662,73)ARTHRITIS & RHEUMATISM, Issue 9 2008Hideo Mitsuyama MD No abstract is available for this article. [source] HLA,DRB4 as a genetic risk factor for Churg-Strauss syndromeARTHRITIS & RHEUMATISM, Issue 9 2007Augusto Vaglio Objective To explore the association between HLA alleles and Churg-Strauss syndrome (CSS), and to investigate the potential influence of HLA alleles on the clinical spectrum of the disease. Methods Low-resolution genotyping of HLA,A, HLA,B, and HLA,DR loci and genotyping of TNFA ,238A/G and TNFA ,308A/G single-nucleotide polymorphisms were performed in 48 consecutive CSS patients and 350 healthy controls. Results The frequency of the HLA,DRB1*07 allele was higher in the CSS patients than in controls (27.1% versus 13.3%; ,2 = 12.64, P = 0.0003, corrected P [Pcorr] = 0.0042, odds ratio [OR] 2.42, 95% confidence interval [95% CI] 1.47,3.99). The HLA,DRB4 gene, present in subjects carrying either HLA,DRB1*04, HLA,DRB1*07, or HLA,DRB1*09 alleles, was also far more frequent in patients than in controls (38.5% versus 20.1%; ,2 = 16.46, P = 0.000058, Pcorr = 0.000232, OR 2.49, 95% CI 1.58,3.09). Conversely, the frequency of the HLA,DRB3 gene was lower in patients than in controls (35.4% versus 50.4%; ,2 = 7.62, P = 0.0057, Pcorr = 0.0228, OR 0.54, 95% CI 0.35,0.84). CSS has 2 major clinical subsets, antineutrophil cytoplasmic antibody (ANCA),positive, with features of small-vessel vasculitis, and ANCA-negative, in which organ damage is mainly mediated by tissue eosinophilic infiltration; analysis of HLA,DRB4 in patients categorized by different numbers of vasculitic manifestations (purpura, alveolar hemorrhage, mononeuritis multiplex, rapidly progressive glomerulonephritis, and constitutional symptoms) showed that its frequency strongly correlated with the number of vasculitis symptoms (P for trend = 0.001). Conclusion These findings indicate that HLA,DRB4 is a genetic risk factor for the development of CSS and increases the likelihood of development of vasculitic manifestations of the disease. [source] | ||||||||||