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

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.

Hidden in plain sight: how to look behind the veil of cocaine-induced vasculitis

“…This is perhaps shown indirectly by the observation that the number of included patients in published case series is growing. Granulomatosis with polyangiitis and microscopic polyangiitis affect men and women equally, which was also the case in this study on cocaine-induced vasculitis [ 5 ]. A higher prevalence in men has been reported previously [ 2 ], but it should be noted that men are more likely to use cocaine [ 1 ].…”

“…It is a clinical challenge for rheumatologists, nephrologists, pulmonologists, dermatologists and ENT specialists to differentiate a cocaine-induced vasculitis from AAV. In this issue of Rheumatology Advances in Practice , Gill et al [ 5 ] were able to carry out retrospective analysis of a large number of patients who presented during a 5-year period in tertiary centres in London and Birmingham. In total, 42 patients with severe nasal damage mimicking AAV were included who disclosed cocaine use or had positive urine toxicology.…”

“…A higher prevalence in men has been reported previously [ 2 ], but it should be noted that men are more likely to use cocaine [ 1 ]. The mean age of patients with cocaine-induced vasculitis, however, was ∼20 years younger than that of AAV patients [ 5 ].…”

The role of ANCA in the management of cocaine‐induced midline destructive lesions or ENT pseudo‐granulomatosis with polyangiitis: a London multicentre case series

“…27,28 Nonetheless, the inverse association we observed in our p-ANCA patients (PR3 + MPO-) is not novel and corroborates previous findings showing that p-ANCA pattern is the most common pattern in CIMDL patients 5,29 with up to 57% of them being PR3 + . 20,30 In a recent large case series conducted in the United Kingdom, which included 42 patients with CIMDL and vasculitis compatible with GPA, Gill and colleagues 31 found that only 29% of their patients had a p-ANCA pattern with PR3+, a lower percentage that could have been influenced by the inclusion of patients with GPA (c-ANCA PR3+). In one of the largest case series of LIV, patients showed a clear predominance of p-ANCA pattern (93%) which, however, was directed against both PR3 and MPO (PR3 + MPO+) in 57% of cases or against MPO alone (PR3-MPO+) in the remaining ones, whereas isolated c-ANCA or isolated PR3 positivity was less frequent.…”

A variety of death modes of neutrophils and their role in the etiology of autoimmune diseases

“…105,106 The presence of NETs has been detected in several typical lesion sites, including the kidney, skin, and thrombus of individuals with AAV. 107 Specifically, NETs have been recognized in the neuropathy of individuals with AAV, while they are absent in patients with nonvasculitic demyelinating neuropathy. 108 Some studies demonstrated an overall increase in NETosis in patients with active vasculitis, along with the presence of fragmented NETs in affected tissues and the bloodstream of AAV patients.…”