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Annals of the Rheumatic Diseases logoLink to Annals of the Rheumatic Diseases
. 2001 Mar;60(3):293–295. doi: 10.1136/ard.60.3.293

Systemic antineutrophil cytoplasmic antibody vasculitis associated with lymphoid neoplasia

M Hamidou 1, D El Kouri 1, M Audrain 1, J Grolleau 1
PMCID: PMC1753562  PMID: 11171697

Abstract

Two cases of systemic antineutrophil cytoplasmic antibody (ANCA) vasculitis in the setting of chronic lymphocytic leukaemia and angioimmunoblastic lymphadenopathy type T cell lymphoma are reported. The two patients had fever of unknown origin associated with cutaneous vasculitis and "pulmonary-renal syndrome" with alveolar haemorrhage. Despite anti-infectious treatments, steroids, and chemotherapy, the vasculitis had a fatal paraneoplastic course in several weeks. When infection is excluded in patients with malignancy, atypical features should be promptly investigated for systemic vasculitis, and an ANCA test performed.



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