Cocaine-induced granulomatosis with polyangiitis—an under-recognized condition. Issue 1 (4th April 2023)

• Record Type: Journal Article
• Authors: Gill, Charn; Sturman, Joseph; Ozbek, Leyla; Henderson, Scott R; Burns, Aine; Hamour, Sally; Pepper, Ruth J; McClelland, Lisha; Chanouzas, Dimitrios; Gane, Simon; Salama, Alan D; Harper, Lorraine
• Is Part Of: Rheumatology advances in practice. Volume 7:Issue 1(2023)
• Publication Date: 2023-04-04
• Subjects: cocaine -- drug-induced vasculitis -- granulomatosis with polyangiitis -- ANCA -- treatment; Rheumatology -- Periodicals; Rheumatology; Rheumatic Diseases; Rheumatology; Periodicals; Electronic journals; Periodical; 616.723005
• DOI: 10.1093/rap/rkad027
• Languages: English
• ISSNs: 2514-1775

Abstract

Abstract: Objectives: Cocaine and cocaine mixed with levamisole are increasingly used in the UK and result in significant direct nasal damage in addition to promoting vasculitis. Our aims were as follows: (1) to identify the main symptoms and presentation of cocaine-induced vasculitis; (2) to provide evidence regarding the best practice for the investigation and diagnosis of cocaine-induced vasculitis; and (3) to analyse the clinical outcomes of patients in order to understand the optimal management for the condition. Methods: We performed a retrospective case series analysis of patients presenting with cocaine-induced midline destructive lesions or vasculitis compatible with granulomatosis with polyangiitis (GPA) from two large tertiary vasculitis clinics between 2016 and 2021. Results: Forty-two patients (29 Birmingham, 13 London) with cocaine-induced midline lesions or systemic disease were identified. The median age was 41 years (range 23–66 years). Current cocaine use was common, and 20 of 23 samples provided were positive when routine urine toxicology was performed; 9 patients who denied ever using cocaine were identified as using cocaine based on urine toxicology analysis, and 11 who stated they were ex-users still tested positive. There was a high incidence of septal perforation (75%) and oronasal fistula (15%). Systemic manifestations were less common (27%), and only one patient had acute kidney injury. Fifty-six per cent of our patients were PR3-ANCA positive, with none testing positive for MPO-ANCA. Symptom remission required cocaine discontinuation even when immunosuppression was administered. Conclusion: Patients with destructive nasal lesions, especially young patients, should have urine toxicology performed for cocaine before diagnosing GPA and considering immunosuppressive therapy. The ANCA pattern is not specific for cocaine-induced midline destructive lesions. Treatment should be focused on cocaine cessation and conservative management in the first instance in the absence of organ-threatening disease. Lay Summary: What does this mean for patients? Cocaine causes a form of inflammation of the blood vessels that primarily leads to a skin rash and destruction of the nasal structures. This can mimic an illness called granulomatosis with polyangiitis (GPA), in which inflammation of the blood vessels occurs for an unknown reason. There is little evidence about the best way to detect and treat cocaine-induced disease and whether it should be treated with strong immunosuppression like GPA. In our study, we reviewed data from 42 patients with cocaine-induced disease over a 5-year period. Our results show that 32% denied cocaine use but were positive on urine testing. The antibody ANCA is commonly found in patients with cocaine-induced disease and GPA; the pattern of ANCA does not differentiate between the two conditions. Patients found resolution of their symptoms only once they stopped using cocaine, and treatment with strong immunosuppression alone did not cause resolution of the symptoms. This study suggests that all patients presenting with skin rashes and nasal symptoms similar to GPA should have urine testing. Furthermore, the most important intervention for a good clinical outcome is cessation of cocaine use. This is more important than the use of immunosuppression, which could potentially harm patients using cocaine.