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Cocaine-induced granulomatosis with polyangiitis-an under-recognized condition

Gill, Charn
Sturman, Joseph
Ozbek,Leyla
Henderson, Scott, R.
Burns, Aine
Hamour, Sally
Pepper, Ruth, J.
McClelland, Lisha
Chanouzas, Dimitrios
Gane, Simon
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04/04/2023
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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.
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Gill C, Sturman J, Ozbek L, Henderson SR, Burns A, Hamour S, Pepper RJ, McClelland L, Chanouzas D, Gane S, Salama AD, Harper L. Cocaine-induced granulomatosis with polyangiitis-an under-recognized condition. Rheumatol Adv Pract. 2023 Apr 4;7(1):rkad027. doi: 10.1093/rap/rkad027.
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