Rheumatic Manifestations of Cocaine Use

Jonathan Graf


Curr Opin Rheumatol. 2013;25(1):50-55. 

In This Article

Cocaine-associated Vasculitis

Vascultitis is a rare but well described complication of cocaine use. This vasculitis can vary in the number and type of organs involved and in the overall severity of disease. Several case reports have described what would otherwise be considered a classic picture of primary, idiopathic granulomatosis with polyangiitis (GPA), with the exception that it occurs in the setting of active cocaine use.[7,8] Although the development of GPA in the setting of cocaine use may be coincidental, the authors of these series strongly suggest a causal relationship between the two.[7,8] These patients can present with cutaneous vasculitis, nasal septal destruction, pauci-immune crescentic glomerulonephritis and a characteristic autoantibody profile that includes detection of cytoplasmic-staining antineutrophil cytoplasmic antibodies (c-ANCAs) by immunofluorescence with specificity for the target antigen proteinase-3 (PR-3) confirmed by immunoassay.[7–9] Cerebral angiitis has also been reported in the setting of cocaine use,[10] as have cases of immunoglobulin A (IgA) nephropathy,[11] scrotal vascultitis,[12] urticarial vasculitis[13] and Churg—Struass vasculitis.[14] Many of these cases have been ANCA negative.[10,12,14] Other patients with cocaine-associated vasculitic syndromes have tested positive for perinuclear ANCA (p-ANCA) by immunofluorescence, with a paradoxically positive subserology against PR-3, a target of c-ANCA.[15] In these cases, the target of p-ANCA may actually be atypical p-ANCA-associated antigens, consistent with what is seen in other forms of drug-induced vascultitis.[16] One of these atypical p-ANCA is human neutrophil elastase (HNE), which shares sequence and structural homology with the classic c-ANCA-associated antigen PR-3.[17] A more in-depth discussion of ANCA testing will be discussed later in this article. Treatment of cocaine-induced vascultitis usually consists of abstinence from the inciting agent with or without the addition of immunosuppressive therapy with corticosteroids alone or including cytotoxic therapy depending upon the severity of disease.[8,12,14]