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Intern Med. 2013;52(10):1095-9. Epub 2013 May 15.

Systemic vasculitis associated with anti-neutrophil cytoplasmic antibodies against bactericidal/permeability increasing protein.

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Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Japan.


Myeloperoxidase- and proteinase 3-anti-neutrophil cytoplasmic antibodies (ANCAs) are often negative in cases in which systemic vasculitis is highly suspected. We herein present a case of bactericidal/permeability increasing protein (BPI)-ANCA-positive systemic vasculitis. This case highlights the possible role of BPI-ANCA in the pathogenesis of systemic vasculitis as well as the possible use of BPI as a diagnostic tool. The accumulation of further case-based reports is expected to shed some light on the pathogesis of systemic vasculitis.

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