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Strauss Syndrome (strauss + syndrome)
Selected AbstractsBrushing, sputum, bronchoalveolar lavage and imprint cytology in the Churg,Strauss SyndromeCYTOPATHOLOGY, Issue 3 2003L'. Babjaková No abstract is available for this article. [source] Epidemiology of primary systemic vasculitis in the Australian Capital Territory and south-eastern New South WalesINTERNAL MEDICINE JOURNAL, Issue 11 2008A. S. Ormerod Abstract Background:, The aim of the study was to determine the epidemiology of primary systemic vasculitis in the Australian Capital Territory and the surrounding rural region between 1995 and 2005. Methods:, Cases were ascertained by a medical record search according to international consensus classification criteria. For antineutrophil cytoplasmic antibody-associated vasculitides, ascertainment was corroborated by a search of all positive antineutrophil cytoplasmic antibody serology during the study period. Denominators were obtained from region-specific census data collected during the study period. Prevalence, incidence and patient characteristics for primary systemic vasculitides were determined for two 5-year periods, 1995,1999 and 2000,2004. Results:, We identified 41 cases of primary systemic vasculitides (Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), Churg,Strauss syndrome or polyarteritis nodosa) between 1995 and 1999 and 67 between 2000 and 2004, giving prevalences of 95/million (95% confidence interval (CI) 76.9,116.1) and 148/million (95%CI 125.1,173.9), respectively. Annual incidence was similar in both periods (approximately 17/year per million adult population). Disease-specific incidences (per million per year) for each of the two periods were 8.8 and 8.4 for WG, 2.3 and 5.0 for MPA, 2.3 and 2.2 for Churg,Strauss syndrome and 2.3 and 1.1 for polyarteritis nodosa. The rural incidence of MPA was 13.9 (95%CI 7.7,23.5) compared with 1.6 (95%CI 0.2,7.2) in the city and there was a trend towards a higher incidence of WG in rural than urban areas. Conclusion:, The overall incidence of primary systemic vasculitides is similar to that reported from other developed countries. WG is more common in south-eastern Australia than in southern Europe, whereas MPA is less common. There was a trend towards higher incidence of antineutrophil cytoplasmic antibody-associated vasculitides in rural than urban areas. [source] Diverse presentation and clinical features of Churg,Strauss syndrome: two cases from a Melbourne teaching hospitalINTERNAL MEDICINE JOURNAL, Issue 6 2004I. Jayasuriya No abstract is available for this article. [source] Collagenolytic (necrobiotic) granulomas: part II , the ,red' granulomasJOURNAL OF CUTANEOUS PATHOLOGY, Issue 6 2004Jane M. Lynch The altered fibers lose their distinct boundaries and exhibit new staining patterns, becoming either more basophilic or eosinophilic. Within the area of altered collagen, there may be deposition of acellular substances such as mucin (blue) or fibrin (red), or there may be neutrophils with nuclear dust (blue), eosinophils (red), or flame figures (red). These color distinctions can be used as a simple algorithm for the diagnosis of collagenolytic granulomas, i.e. ,blue' granulomas vs. ,red' granulomas. Eight diagnoses are included within these two groupings, which are discussed in this two-part article. In the previously published first part, the clinical presentation, pathogenesis and histologic features of the ,blue' collagenolytic granulomas were discussed. These are the lesions of granuloma annulare, Wegener's granulomatosis, and rheumatoid vasculitis. In this second half of the series, the ,red' collagenolytic granulomas are discussed; these are the lesions of necrobiosis lipoidica, necrobiotic xanthogranuloma, rheumatoid nodules, Churg,Strauss syndrome, and eosinophilic cellulitis (Well's Syndrome). [source] Collagenolytic (necrobiotic) granulomas: part 1 , the ,blue' granulomasJOURNAL OF CUTANEOUS PATHOLOGY, Issue 5 2004Jane M. Lynch A collagenolytic or necrobiotic non-infectious granuloma is one in which a granulomatous infiltrate develops around a central area of altered collagen and elastic fibers. The altered fibers lose their distinct boundaries and exhibit new staining patterns, becoming either more basophilic or eosinophilic. Within the area of altered collagen, there may be deposition of acellular substances such as mucin (blue) or fibrin (red), or there may be neutrophils with nuclear dust (blue), eosinophils (red), or flame figures (red). These color distinctions can be used as a simple algorithm for the diagnosis of collagenolytic granulomas, i.e. ,blue' granulomas vs. ,red' granulomas. Eight diagnoses are included within these two groupings, which are discussed in this two-part article. In this first part, the clinical presentation, pathogenesis, and histologic features of the ,blue' collagenolytic granulomas are discussed. These are the lesions of granuloma annulare, Wegener's granulomatosis, and rheumatoid vasculitis. In the subsequent half of this two-part series, the ,red' collagenolytic granulomas will be discussed; these are the lesions of necrobiosis lipoidica, necrobiotic xanthogranuloma, rheumatoid nodules, Churg,Strauss syndrome, and eosinophilic cellulitis (Well's syndrome). [source] Multiple perforating and non perforating pilomatricomas in a patient with Churg,Strauss syndrome and Rubinstein,Taybi syndromeJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 5 2004P Bayle SUMMARY We report an unusual association of multiple perforating and non-perforating pilomatricomas with Churg,Strauss syndrome, and a dysmorphic syndrome evocative of Rubinstein-Taybi syndrome. These syndromes may be independent, but these rare diseases and genetic abnormalities may be linked together. [source] Heart involvement detected by magnetic resonance in a patient with Churg,Strauss syndrome, mimicking severe asthma exacerbationALLERGY, Issue 8 2010W. Szczeklik No abstract is available for this article. [source] IL-5 expression and release from human CD34 cells in vitro; ex vivo evidence from cases of asthma and Churg,Strauss syndromeALLERGY, Issue 7 2010A. Bossios To cite this article: Bossios A, Sjöstrand M, Dahlborn A-K, Samitas K, Malmhäll C, Gaga M, Lötvall J. IL-5 expression and release from human CD34 cells in vitro; ex vivo evidence from cases of asthma and Churg,Strauss syndrome. Allergy 2010; 65: 831,839. Abstract Background:, Eosinophils develop from hematopoietic CD34+ progenitor cells in the bone marrow (BM) under the influence of Interleukin-5 (IL-5). The primary source of IL-5 is T-lymphocytes, although other sources may exist. The aims of this study were to determine whether CD34+ cells from human peripheral blood (PB) and BM have the capacity to produce IL-5 when stimulated in vitro, and secondly, whether an elevated number of IL-5-producing CD34+ cells can be found in situ in ongoing eosinophilic disease. Methods:, CD34+ cells from PB and BM were stimulated in vitro, and IL-5 production and release was assessed by ELISA, ELISPOT, flow cytometry and immunocytochemistry. Blood and BM from a patient with Churg,Strauss syndrome were analyzed by flow cytometry for CD34+/IL-5+ cells, and immunohistochemical staining of CD34+/IL-5+ cells in bronchial biopsies from an asthmatic patient was performed. Results:, Both PB and BM CD34+ cells can produce and release IL-5 when stimulated in vitro. In the Churg,Strauss patient, IL-5-producing CD34+ cells were found in PB and BM. Oral glucocorticoid treatment markedly decreased the number of IL-5-positive CD34 cells in the BM. CD34+/IL-5+ cells were present in a patient with asthma. Conclusion:, CD34+ cells in blood and BM are capable of producing IL-5 both in vitro and in vivo in humans, arguing that these cells may have the capacity to contribute to eosinophilic inflammation. Consequently, targeting CD34+ progenitor cells that produce and release IL-5 may be effective in reducing the mobilization of eosinophil lineage-committed cells in eosinophilic-driven diseases. [source] Persistent airflow obstruction in asthma of patients with Churg,Strauss syndrome and long-term follow-upALLERGY, Issue 4 2009V. Cottin Background:, Little is known about the long-term outcome of airflow obstruction in asthma of patients with Churg,Strauss syndrome (CSS). Methods:, We conducted a retrospective study of 24 consecutive patients (aged 41.1 ± 13.5 years) with CSS in a single center. All had asthma (starting 8.1 ± 9.5 years prior to the diagnosis of CSS), blood eosinophilia (6.1 ± 4.4 × 109/l) and systemic manifestations of CSS. Antineutrophil cytoplasmic antibodies were found in 7 of 22 tested patients. Seven patients had smoked (a mean of 10 pack-years). All patients received oral corticosteroids, 11 cyclophosphamide and 23 inhaled corticosteroids. Results:, Airflow obstruction was found in 14 patients (70%) at diagnosis, and in 11 of 22 patients (50%) at the time of the clinical remission of CSS. The mean postbronchodilator FEV1/FVC and FEV1 were 69 ± 12% and 74 ± 21% of predicted at diagnosis (n = 20); 71 ± 10% and 92 ± 19% of predicted at the clinical remission (n = 22); and 64 ± 13% and 80 ± 21% at last visit (n = 13), respectively. During follow-up, postbronchodilator FEV1 increased by 30 ± 28% in six patients with FEV1/FVC < 70% despite inhaled therapy who received higher dose of oral corticosteroids. At last visit, 5 of 13 patients (38%) with more than 3 years of follow-up had persistent airflow obstruction as defined by postbronchodilator FEV1/FVC < 70% and FEV1 < 80% of predicted. Conclusion:, Airflow obstruction due to uncontrolled asthma is present despite corticosteroids in many patients at diagnosis and at clinical remission of CSS, and during follow-up. It may be still partly reversible with increased oral corticosteroid treatment. [source] Clinicopathological studies of peripheral neuropathy in Churg,Strauss syndromeNEUROPATHOLOGY, Issue 4 2002Toshiko Nagashima Clinicopathological studies were performed on the visceral organs and the sural nerve of a male patient with Churg,Strauss syndrome (CSS) in order to understand the mechanisms of peripheral nervous system damage. A 67-year-old man, with a 2-year history of bronchial asthma, developed acutely painful paraplegia and dyspnea. Laboratory data showed a leukocytosis, an elevated serum creatinine kinase (CK) and marked eosionophilia. Autoantibodies including p- and c-ANCA were negative. Electrophysiological studies revealed a severe sensory-motor neuropathy of multiple mononeuritis type. Steroid pulse therapy performed a day after biopsy of skin, muscle and sural nerve was effective in resolving his respiratory and neurological dysfunction but a perforation of an intestinal ulcer occurred which required surgical intervention. In the biopsied sural nerve and the surgically resected intestine and mesentery there was vasculitis with fibrinoid necrosis accompanied by numerous eosinophils and macrophages containing eosinophil cationic protein (ECP). These findings suggest that in addition to ischemic changes due to vasculitis some neurotoxic substances generated by the eosinophils may be involved in the development of neuropathy in CSS. [source] Identification of patients with Churg,Strauss syndrome (CSS) using automated dataPHARMACOEPIDEMIOLOGY AND DRUG SAFETY, Issue 10 2004Leslie R. Harrold MD Abstract Purpose Our aim was to identify individuals with Churg,Strauss syndrome (CSS) among asthma drug users, based on patterns of diagnostic and procedural codes (termed ,algorithms') contained in automated claims data. Methods A retrospective study was conducted among patients who had been dispensed asthma drugs at three HMOs. Individuals who received ,3 dispensings of an asthma drug during any consecutive 12-month period beginning 1 January 1994 through 20 June 2000 were identified. Information on patient age, gender, enrollment status, asthma drugs dispensed, inpatient and outpatient diagnoses and procedures were obtained from the HMO automated databases. Twelve combinations of diagnostic and billing codes (,algorithms') were developed using the claims data to identify potential cases of CSS. Chart reviews blinded to drug exposure were performed using a standardized abstraction form. A rheumatologist reviewed abstracted information on all subjects, and those who met two or more American College of Rheumatology (ACR) criteria for CSS were further reviewed by two clinical experts. Cases were classified as unlikely, possible, or probable/definite CSS. Each clinical expert independently rated the cases; disagreements were resolved by consensus. Results A total of 185,604 patients who had been dispensed asthma drugs were identified. Three hundred fifty subjects were selected for chart review, and 15 were classified as having ,probable/definite' CSS. The algorithms that were most successful in identifying patients with CSS were as follows: (1) two or more codes for vasculitis (13 confirmed cases from 129 reviewed; positive predictive value 10%); (2) codes for both vasculitis and neurologic symptoms (6 confirmed cases from 15 reviewed; positive predictive value 40%) and (3) codes for both eosinophilia and vasculitis (4 confirmed cases from 5 reviewed; positive predictive value 80%). Conclusion Automated claims data can be used to identify patients with CSS. This approach can facilitate better epidemiologic study of the risk factors for the condition. Copyright © 2003 John Wiley & Sons, Ltd. [source] Multiple tracheobronchial mucosal lesions in two cases of Churg,Strauss syndromeRESPIROLOGY, Issue 1 2006Hidekazu MATSUSHIMA Abstract: Churg,Strauss syndrome (CSS) is characterized by hypereosinophilia and a systemic necrotizing vasculitis seen almost exclusively in patients with asthma. The most common pathological findings in the chest in CSS are eosinophilic pneumonia, necrotizing vasculitis and granulomatous inflammation (extravascular granuloma). However, tracheobronchial mucosal lesions have rarely been reported in CSS. The authors report two patients with CSS who had multiple tracheobronchial mucosal lesions that were found by fibreoptic bronchoscopy. They were tiny nodular lesions and necrotizing bronchial inflammation with many eosinophils was observed upon pathological examination. The authors concluded that tracheobronchial mucosal lesions may be one of the manifestations of vasculitis seen in CSS. [source] New algorithm (KAWAKAMI algorithm) to diagnose primary cutaneous vasculitisTHE JOURNAL OF DERMATOLOGY, Issue 2 2010Tamihiro KAWAKAMI Abstract Palpable purpura tends to indicate involvement of small vessel vasculitis in the upper dermis. Livedo racemosa, nodular lesion and skin ulceration are indicative of involvement of small to medium-sized vessel vasculitis in the lower dermis to subcutaneous fat. We set out to establish a new algorithm (KAWAKAMI algorithm) for primary cutaneous vasculitis based on the Chapel Hill Consensus Conference classification and our research results, and apply to the diagnosis. The first step is to measure serum antineutrophil cytoplasmic antibodies (ANCA) levels. If myeloperoxidase-ANCA is positive, Churg,Strauss syndrome or microscopic polyangiitis can be suspected, and if the patient is positive for proteinase 3-ANCA, Wegener's granulomatosis is most likely. Next, if cryoglobulin is positive, cryoglobulinemic vasculitis should be suspected. Third, if direct immunofluorescence of the skin biopsy specimen reveals immunoglobulin A deposition within the affected vessels, Henoch,Schönlein purpura is indicated. Finally, the presence of anti-phosphatidylserine,prothrombin complex antibodies and/or lupus anticoagulant and histopathological necrotizing vasculitis in the upper to middle dermis (leukocytoclastic vasculitis) indicates cutaneous leukocytoclastic angiitis, whereas if necrotizing vasculitis exists in the lower dermis and/or is associated with the subcutaneous fat, cutaneous polyarteritis nodosa is indicated. The KAWAKAMI algorithm may allow us to refine our earlier diagnostic strategies and allow for efficacious treatment of primary cutaneous vasculitis. In cutaneous polyarteritis nodosa, warfarin or clopidogrel therapies should be administrated, and in cases that have associated active inflammatory lesions, corticosteroids or mizoribine (mycophenolate mofetil) therapy should be added. We further propose prophylactic treatment of renal complications in patients with Henoch,Schönlein purpura. [source] Granuloma formation in ANCA-associated vasculitidesAPMIS, Issue 2009PETER LAMPRECHT Granuloma formation is a key pathologic finding in two of the anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides: Wegener's granulomatosis (WG) and Churg,Strauss syndrome (CSS). So far, no animal models have been established convincingly reproducing both vasculitic and granulomatous features typical of WG and CSS. In biopsies, granulomatous lesions are found both at distant extravascular sites and in the vicinity of inflamed vessels, e.g. in the lung. Intriguingly, WG-granulomata appear to display features of tertiary lymphoid tissue. Cartilaginous and osseous destruction is caused by granulomatous inflammation invading adjacent tissues. Rhinosinusitis is regularly encountered in WG and CSS. Septal perforation, saddle nose deformity, middle and inner ear symptoms, and granulomatous invasion of the palate, orbita, meninges, or the pituitary gland may complicate WG. Both common (e.g. FCGR3B copy number) and distinct (e.g. HLA-DP, IL-10.2) genetic factors have been identified in AAV potentially favouring inflammation and autoimmunity. The HLA-DPB1/RING1/RXRB region constitutes a quantitative trait locus for ANCA-positive WG with the strongest association to be reported up to now. A profound alteration of the T-cell response including Th1 and Th17 responses, anomalously NK-receptor-expressing ,NK-like' T cells, and dysfunctional regulatory T cells could facilitate and sustain granuloma formation and autoimmunity. [source] Sarcoidosis with cutaneous granulomatous vasculitisAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 3 2010Chia-Hung Wei ABSTRACT Ulceration of non-caseating granulomas is a rare cutaneous presentation of sarcoidosis. Granulomatous vasculitis is classically associated with Wegener's granulomatosis, lymphomatoid granulomatosis or Churg,Strauss syndrome. It is also commonly noted in pulmonary sarcoidosis, but has seldom been reported in cutaneous sarcoidosis, particularly the ulcerative variant. We present a rare case of sarcoidosis with multiple purpuric leg ulcers showing a granulomatous vasculitis histologically. [source] Solar urticaria as a manifestation of Churg,Strauss syndromeCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 2 2007T. Shimauchi No abstract is available for this article. [source] | ||||||||||||||||||||||