![]() ![]() It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. Six patients (43% died during followup to 2008. Seven patients (50% had glomerulonephritis, 11 (79% pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50% had overlap CTD features, and the majority (79% had limited variant of scleroderma. The majority (71% were female, with mean age at vasculitis diagnosis 53 years. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD features, type of scleroderma (limited or diffuse ANCA serology, vasculitic organ system manifestations and death. These diagnoses were confirmed by retrospective review of complete medical records. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. In 8, manual occupation with local muscle strain apparently caused the hyperCKemia despite aĪNCA-associated vasculitis in scleroderma: a case series of fourteen patientsĭirectory of Open Access Journals (Sweden)įull Text Available Antimyeloperoxidase (MPO, perinuclear antineutrophil cytoplasmic antibodies (pANCA, and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. Clinical and laboratory findings did not point to any established neuromuscular disorder. Persistent hyperCKemia: fourteen patients studied in retrospectįourteen patients with persistently raised serum creatine kinase activity (hyperCKemia) were studied in retrospect. ![]()
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