Misdiagnosis of ANCA-Associated Vasculitis in Patients With Cocaine/Levamisole–Associated Autoimmune Syndrome and Cocaine-Induced Midline Destructive Lesions: A Case Series

Abstract

Background

Cocaine/Levamisole-Associated Autoimmune Syndrome (CLAAS) encompasses a spectrum of autoimmune and vasculitic phenomena, which includes Cocaine-Induced Midline Destructive Lesions (CIMDL), which can mimic ANCA-associated vasculitis (AAV) due to overlapping clinical features and the potential for ANCA positivity. These similarities can lead to misdiagnosis and inappropriate immunosuppressive therapy.

Methods

This study highlights a case series of seven patients (from 2015 to 2024) with CLAAS with its subset of CIMDL, initially misdiagnosed as active AAV, in patients who were referred to various clinicians in the Rheumatology unit of a Tertiary Hospital in the United Kingdom.

Results

All patients presented with nasal symptoms, and they all exhibited additional systemic manifestations consistent with CLAAS. Five were ANCA-positive at initial evaluation, leading to the initiation of immunosuppressive therapy; however, symptoms persisted. The diagnoses were then revised to CIMDL in all cases within the broader context of CLAAS following the identification of cocaine use after further patient inquiry and urine toxicology for drug of abuse (DOA) screening found cocaine metabolites.

Conclusion

A comprehensive drug history and urine toxicology screening are crucial in patients with suspected AAV, as ANCA positivity can occur in CLAAS as well as its subset of CIMDL, complicating the diagnosis. Differentiating between AAV and CIMDL related to CLAAS is essential to avoid unnecessary immunosuppression.