2017
DOI: 10.7861/clinmedicine.17-1-60
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ANCA-associated vasculitis

Abstract: The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity. The ANCA-associated vasculitides (AAV) are three separate conditions - granulomatosis with polyangiitis (GPA - formerly known as Wegener’s granulomatosis); microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA … Show more

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Cited by 169 publications
(170 citation statements)
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“…The pathogenesis of AAV involves the interplay of many cell types of the immune system, including neutrophils, monocytes and B cells, all of which contribute to T cell differentiation and activation and controlled by cytokines released by T cells [2][3][4][21][22][23]. CD4 1 T cells produce Th1 and Th17 cytokines, such as interferon (IFN)-g and interleukin (IL)-17, respectively, are expanded in the circulation of patients with GPA and MPA [24,25], whereas Th2 cells, producing IL-4, IL-5 and IL-13, are more frequent in patients with EGPA [24,[26][27][28][29].…”
Section: Discussionmentioning
confidence: 99%
“…The pathogenesis of AAV involves the interplay of many cell types of the immune system, including neutrophils, monocytes and B cells, all of which contribute to T cell differentiation and activation and controlled by cytokines released by T cells [2][3][4][21][22][23]. CD4 1 T cells produce Th1 and Th17 cytokines, such as interferon (IFN)-g and interleukin (IL)-17, respectively, are expanded in the circulation of patients with GPA and MPA [24,25], whereas Th2 cells, producing IL-4, IL-5 and IL-13, are more frequent in patients with EGPA [24,[26][27][28][29].…”
Section: Discussionmentioning
confidence: 99%
“…GPA has a wide spectrum of clinical manifestations, including symptoms from the upper and lower respiratory system (hearing loss, epistaxis, inflammation, etc), cardiovascular symptoms (vasculitis, occlusive vascular disease, pericarditis, cardiomyopathy, ischemic heart disease, etc), gastrointestinal symptoms (mesenteric vasculitis, peritonitis), cutaneous and mucocutaneous symptoms. Renal participation may even lead to end‐stage renal failure …”
Section: Introductionmentioning
confidence: 99%
“…Renal participation may even lead to end-stage renal failure. 6,7 Involvement of the nervous system, even as the initial manifestation, is not uncommon, with reported frequencies ranging from 22 to 54% of patients. In older studies, peripheral nervous system (PNS) involvement seemed to dominate, predominantly as polyneuropathy or mononeuritis multiplex due to vasculitis of the vasa nervorum, whereas lesions of the central nervous system (CNS) were considered rather rare, with reported frequencies ranging from 7 to 11% (modern authors exclude cranial nerve palsies from CNS manifestations).…”
Section: Introductionmentioning
confidence: 99%
“…AAV is common in older adults, and slightly more common in men. 2 The ANCA-positive rate is much higher in patients with T1DM than in healthy individuals. 3 The database searches (PubMed, J-STAGE, and Ichushi) using the key words "type 1 diabetes mellitus" and "ANCA-associated vasculitis" identified just 10 T1DM cases of ANCA-associated vasculitis ( Table 1; File S1).…”
mentioning
confidence: 98%