Pathophysiology of eosinophilic granulomatosis with polyangitis (Churg-Strauss).

[Pathophysiology of eosinophilic granulomatosis with polyangitis (Churg-Strauss)]. Rev Med Interne. 2016 Mar 9; Authors: Chaigne B, Dion J, Guillevin L, Mouthon L, Terrier B Abstract Eosinophilic granulomatosis with polyangitis (EGPA) (formerly Churg-Strauss syndrome) is a rare small-sized vessel vasculitis belonging to the group of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides. MPO-ANCA is present in only 31 to 38% of patients. In this review, we describe the pathophysiology of EGPA, which is characterized by a genetic predisposition, an environmental association, and a cellular dysfunction of eosinophils, neutrophils, and T and B cells. PMID: 26971201 [PubMed - as supplied by publisher]
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research

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Conclusion: Neurological morbidity commonly accompanies systemic vasculitis. Nervous system disease does not affect the survival significantly in these patients.
Source: Neurology India - Category: Neurology Authors: Source Type: research
We report a case of a 15-year-old male who presented with indurated and pruritic papules on both legs and peripheral eosinophilia without other system involvement. Histopathological findings from a skin biopsy were suggestive of eosinophilic granulomatosis with polyangiitis. Anti-neutrophil cytoplasmic antibodies were negative. PMID: 31360325 [PubMed]
Source: Oman Medical Journal - Category: Middle East Health Tags: Oman Med J Source Type: research
Abstract Eosinophilic granulomatosis with polyangiitis (EGPA) is one form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Identical to what has been called Churg-Strauss syndrome, EGPA exhibits both allergic and vasculitis features. EGPA was first described as a syndrome consisting of asthma, fever, eosinophilia, and organ involvement including heart failure, neuropathy, and kidney damage, by Churg and Strauss in 1951. On the basis of the 2012 Chapel Hill Consensus Conferences Nomenclature of Vasculitis, EGPA comprises three typical allergic components, including asthma, peripheral eosinophili...
Source: Yonsei Medical Journal - Category: Universities & Medical Training Authors: Tags: Yonsei Med J Source Type: research
Co-occurrence of PLA2R-positive membranous nephropathy without crescents, and PR3-positive eosinophilic granulomatosis with polyangiitis
. Clin Nephrol. 2019 Jul 26;: Authors: Zhu Y, Chang Q, Cao X, Zheng S, Li P, Luan J, Zhou H Abstract Primary membranous nephropathy (PMN) is a common cause of adult nephrotic syndrome, most commonly associated with autoantibodies against M-type phospholipase A2 receptor (PLA2R). Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a rare disorder characterized by asthma, eosinophilia, and multiorgan vasculitis. Here, ...
Source: Clinical Nephrology - Category: Urology & Nephrology Authors: Tags: Clin Nephrol Source Type: research
Conclusion: Most common etiology of PRS is ANCA positive vasculitis in India. With high degree of suspicion for DAH in patients presenting with haemoptysis, breathlessness and alveolar opacities in chest x-ray and carefully investigating by simple urine examination for evidence of GN, timely diagnosis of PRS can be made. With timely appropriate treatment survival is 68%. Patients with PRS due to leptospirosis or dengue have features suggestive of underlying disease (like icterus with raised bilirubin but
Source: Journal of the Association of Physicians of India - Category: General Medicine Tags: J Assoc Physicians India Source Type: research
Shanshan Zhang1, Dongli Yuan2 and Ge Tan1* 1Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China 2The Institute of Medical Information, Chongqing Medical University, Chongqing, China Primary systemic vasculitis can affect every structure in both the central and peripheral nervous system, causing varied neurological manifestations of neurological dysfunction. Early recognition of the underlying causes of the neurological symptoms can facilitate timely treatment and improve the prognosis. This review highlights the clinical manifestations of primary systemic vasc...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
This report represents one of the possible presentations of EGPA according to the antineutrophil cytoplasmic antibody status – which in our case was negative, with a low prognostic Five-Factor Score – that was successfully treated with oral steroids and azathioprine as a steroid-sparing agent. Our objective was to add a case report to the scarce existing literature in order to learn more about therapeutic options for EGPA. This case report demonstrates that oral steroids, as induction treatment, and azathioprine, as maintenance treatment, are effective in elderly patients with EGPA without involvement of any ot...
Source: Case Reports in Dermatology - Category: Dermatology Source Type: research
We present the case report of a 38-year-old male with a history of asthma. After a month-and-a-half of progressive weakness, no fever, diarrhea, vomiting and abdominal pain associated with weight loss, he was diagnosed of intestinal parasitosis. He later died of a massive myocardial necrosis due to EGPA with multiple organs affected. PMID: 30720008 [PubMed - as supplied by publisher]
Source: Anales del Sistema Sanitario de Navarra - Category: General Medicine Authors: Tags: An Sist Sanit Navar Source Type: research
Systemic vasculitis, which can involve the brain, may be one of the causes of stroke in young adults; therefore, a test panel for systemic vasculitis is considered for some young stroke patients. However, little is known about this test ’s yield as a screening test in young adults with ischemic stroke. We evaluated the yield of a panel for systemic vasculitis as a screening test in young patients with ischemic stroke. Consecutive patients aged 18–45 years with ischemic stroke between January 2010 and December 2017 were included . They all underwent screening tests for systemic vasculitis including rheumatoid fa...
Source: European Neurology - Category: Neurology Source Type: research
Semin Respir Crit Care Med 2018; 39: 471-481 DOI: 10.1055/s-0038-1669454Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg–Strauss syndrome, is a systemic necrotizing vasculitis of small- and medium-size vessels, characterized by asthma and blood eosinophilia. EGPA typically occurs in patients with preexisting asthma, and involves the skin, lungs, and peripheral nerves. Poor-prognosis factors (i.e., involvement(s) of the gastrointestinal tract, heart, and/or kidney) have been assessed with the Five-Factor Score (FFS). One-third of the patients have anti-myeloperoxidase antineutrophil cytoplas...
Source: Seminars in Respiratory and Critical Care Medicine - Category: Respiratory Medicine Authors: Tags: Review Article Source Type: research
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