Rheumatic Manifestations of Cocaine Use

Jonathan Graf


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

In This Article

Cocaine-induced Midline Destructive Lesions

Chronic use of cocaine can induce sinusitis and necrosis of the sinus mucosa, nasal and palatal perforation, and midline sinonasal destruction that can mimic a vascultitis such as GPA.[18–20] Although many of the lesions demonstrate mixed inflammatory cellular infiltrates, necrotizing inflammation and occasional leukocytoclastic vasculitis and fibrinoid necrosis, other classic features of GPA are usually absent on histopathology.[5,18–20]

Serologic markers of inflammation, including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), may be elevated in some patients with cocaine-induced midline destructive lesion (CIMDL), making these tests unreliable in distinguishing CIMDL from a systemic vasculitis.[18–20] Unfortunately, ANCA serologies are generally unhelpful as well in distinguishing between these two syndromes, as patients with CIMDL may be either ANCA positive or negative.[20] When positive, ANCA patterns can vary. Some patients test positive for c-ANCA and PR-3.[9,20] More often, CIMDL patients test positive for p-ANCA with specificity for atypical p-ANCA-associated antigens such as HNE.[20,21] Although some CIMDL patients may also test positive for PR-3 antibodies, the presence of antibodies to HNE provides some diagnostic specificity for CIMDL compared to GPA (which is almost universally anti-HNE negative).[21] It is unclear whether patients with CIMDL who test dual-positive for antibodies to HNE and PR-3 have one antibody recognizing cross-reacting epitopes on the structurally similar molecules, or as some evidence suggests, two unique antibodies that recognize separate epitopes on the two molecules.[22] In the latter case, data suggest that antibodies directed against PR-3 in CIMDL patients might target different epitopes than do those from patients with GPA.[22]

Treatment of CIMDL is centred upon cessation of cocaine use, and there are limited data regarding use of adjunctive immunotherapy. One report describes some benefit from high-dose prednisilone in the setting of ongoing cocaine use but little benefit from methotrexate or cyclophosphomide.[19] Therefore, distinguishing CIMDL from a primary vasculitis, although difficult, could spare CIMDL patients from potentially unnecessary immunosuppressive therapy.