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Such uncommon presentations of granulomatosis with polyangiitis (Wegener's granulomatosis), classical polyarteritis nodosa and unclassifiable vasculitides are described here with the relevant review of literature.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>All patients diagnosed as having systemic vasculitides and classified as having granulomatosis with polyangiitis (Wegener's granulomatosis), classic polyarteritis nodosa, microscopic polyangiitis and unclassifiable vasculitis according to <jats:styled-content style=\"fixed-case\">EMEA<\/jats:styled-content> consensus methodology and followed up prospectively from June 2007 to December, 2011 were included. Details of uncommon presentations of these disorders were identified.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Seventy\u2010nine patients with systemic vasculitides were seen under our rheumatology services during this period. These included 45 patients with granulomatosis with polyangiitis (Wegener's granulomatosis), 18 with classic polyarteritis nodosa, five with microscopic polyangiitis, four with Churg\u2013Strauss syndrome and seven with unclassifiable vasculitis. The uncommon presentations of granulomatosis with polyangiitis were a tumefactive subcutaneous mass in the thigh; prostatomegaly with obstructive uropathy and advanced renal failure; and predominant gastrointestinal (<jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content>) vasculitis with thrombocytopenia and coagulopathy at presentation. The uncommon manifestations of classic polyarteritis nodosa were secondary antiphospholipid antibody syndrome and Budd\u2013Chiari syndrome. One patient with massive lower <jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content> bleeding required surgical resection of the large bowel which showed isolated necrotizing granulomatous <jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content> vasculitis. Single organ vasculitis of the <jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content> tract was diagnosed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Systemic necrotizing vasculitides may present with uncommon manifestations and a high index of suspicion is required for early diagnosis and prompt treatment to prevent adverse outcomes.<\/jats:p><\/jats:sec>","DOI":"10.1111\/1756-185x.12223","type":"journal-article","created":{"date-parts":[[2013,11,15]],"date-time":"2013-11-15T05:10:50Z","timestamp":1384492250000},"page":"562-572","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Uncommon presentations of primary systemic necrotizing vasculitides: the Great Masquerades"],"prefix":"10.1111","volume":"17","author":[{"given":"Aman","family":"Sharma","sequence":"first","affiliation":[{"name":"Department of Internal Medicine Post Graduate Institute of Medical Education and 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