Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2025 Jun;13(6):e70215.
doi: 10.1002/iid3.70215.

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

Kehinde Sunmboye  1   2 , Ameen Jubber  2 , Maumer Durrani  2 , Jeremy Royle  2 , Shireen Shaffu  2
Affiliations
Case Reports

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

Kehinde Sunmboye et al. Immun Inflamm Dis. 2025 Jun.

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.

Keywords: ANCA‐associated vasculitis; Cocaine; Cocaine/Levamisole‐associated autoimmune Syndrome; granulomatosis with polyangiitis; misdiagnosis; nasal septum perforation.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Chest x‐ray for patient A, showing Bilateral lung field haziness.
Figure 2
Figure 2
Chest CT for patient A showing ground glass shadowing at the level of the tracheal bifurcation.
Figure 3
Figure 3
CT sinus (axial view), with the red arrow showing nasal perforation.
See this image and copyright information in PMC

References

    1. Shoji M. K., Staropoli P., Tran A. Q., et al., “Periorbital Pyoderma Gangrenosum Associated With a Cocaine‐Induced Midline Destructive Lesion: Case Report and Review of the Literature,” Ophthalmic Plastic and Reconstructive Surgery 39, no. 3 (2023): e91–e96, 10.1097/iop.0000000000002347. - DOI - PMC - PubMed
    1. Gill C., Sturman J., Ozbek L., et al., “Cocaine‐Induced Granulomatosis With Polyangiitis—An Under‐Recognized Condition,” Rheumatology Advances in Practice 7, no. 1 (2023): rkad027, 10.1093/rap/rkad027. - DOI - PMC - PubMed
    1. McGrath M. M., Isakova T., and Rennke H. G., “Levamisole‐Adulterated Cocaine Causing ANCA‐Associated Vasculitis and Pauci‐Immune Glomerulonephritis: A Case Report and Literature Review,” American Journal of Kidney Diseases 58, no. 4 (2011): 568–572.
    1. Trimarchi M., Bussi M., and Sinico R. A., “Cocaine‐Induced Vasculitis and Destructive Sinonasal Disease: An Update,” Journal of Otolaryngology 56, no. 1 (2021): 154–160.
    1. Veronese F. M., Di Capua A., and Abrignani S., “Levamisole‐Induced Autoimmune Syndromes: Diagnostic Dilemmas in Clinical Practice,” Journal of Autoimmunity 71 (2016): 90–98.

Publication types

MeSH terms