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Demographic, Clinical, and Immunologic Correlates among a Cohort of 50 Cocaine Users Demonstrating Antineutrophil Cytoplasmic Antibodies

Objective. Cocaine/levamisole-associated autoimmunity syndrome (CLAAS) is a poorly understood form of drug-induced autoimmunity. Our goals were to better characterize the spectrum of clinical and immunologic features of CLAAS, to identify demographic risk factors, and to generate new hypotheses regarding pathogenesis. Methods. CLAAS subjects were identified between 2001 and 2015 at the University of Washington Medical Center, Harborview Medical Center, and affiliated clinics in Seattle, Washington, USA. Demographic, clinical, and immunologic variables were collected and correlated using contingency and logistic regression analyses. We used similar analyses to compare CLAAS subjects with all individuals exhibiting antineutrophil cytoplasmic antibodies (ANCA+) or cocaine use (Cocaine+) in an associated deidentified clinical data repository. Results. We identified 50 CLAAS subjects. Compared to all Cocaine+ individuals (n = 2740), CLAAS subjects were more likely to be female and less likely to self-identify as black/African American. CLAAS subjects showed several ANCA patterns, including anti-MPO (myeloperoxidase)/anti-PR3 (proteinase 3) dual reactivity, a finding that appears to be specific to CLAAS. Hematologic, renal, and skin abnormalities were most frequently reported, including neutropenia and skin purpura. Finally, we observed strong, independent associations between the cytoplasmic ANCA (C-ANCA) pattern and mortality. Conclusion. We identify sex and race as important risk modifiers in the developing CLAAS among cocaine users. The development of C-ANCA was associated with increased mortality. Moreover, we confirm the enriched presence of anti-MPO/anti-PR3 dual reactivity in CLAAS, further supporting the diagnostic utility of this feature.