PR3-ANCA-positive Necrotizing Multi-organ Vasculitis Following Cocaine Abuse

Abstract

A 22-year-old man with a history of cocaine abuse from 2003 to 2005 developed recurrent bleeding of the nasal septum and a progressive cough and dyspnoea. He was admitted to the intensive care unit because of fulminant pneumonia, impaired renal function and progressive general deterioration. While hospitalized, he developed cutaneous vasculitis, thrombosis of the right subclavian and right jugular veins, testicular pain and, eventually, expanding red papules and plaques on the limbs. The symptoms were a diagnostic challenge, until skin biopsy showed immunoglobulin deposits in small vessels and kidney biopsy focal and segmental pauci-immune, crescentic glomerulonephritis. This led, together with anti-neutrophil cytoplasmic antibodies (cANCA and PR3-ANCA), to the diagnosis of Wegener granulomatosis. The number of affected organ systems in our patient exceeds that commonly found in the literature. Several clinical observations of cocaine abuse followed by Wegener granulomatosis suggest an active induction of a PR3-ANCA-positive vasculitis by cocaine.