Elsevier

Autoimmunity Reviews

Volume 17, Issue 1, January 2018, Pages 73-77
Autoimmunity Reviews

Review
Cocaine and ANCA associated vasculitis-like syndromes – A case series

https://doi.org/10.1016/j.autrev.2017.11.011Get rights and content

Abstract

Objectives

We analysed the spectrum of clinical manifestations of cocaine associated pseudovasculitis.

Methods

Clinical, serological, radiological and histological features of 14 patients with cocaine pseudovasculitis syndromes were included.

Results

Twelve patients had significant sinus thickening or erosive disease. Other multi-system manifestations included vasculitic rashes, pulmonary lesions and peripheral neuropathy. All patients had positive ANCA titres at presentation. All patients were managed with corticosteroids +/− methotrexate and co-trimoxazole, 2 patients received cyclophosphamide.

Conclusions

Advanced erosive nasal septal defects and atypical ANCA patterns are suggestive of cocaine induced pseudovasculitis. Complete drug cessation may negate the need for exposure to potent immunosuppressive agents.

Introduction

Cocaine is a potent illicit stimulant and ‘class A’ drug. It is the second most commonly used illicit drug amongst 16–59 year olds in England and Wales [1]. Acute intoxication with cocaine can cause euphoria and increased sociability. Cocaine is associated with multiple deleterious effects including acute psychosis, seizures, cerebrovascular accidents, cardiac arrhythmias, myocardial infarction and cardiac arrest. Cocaine has been implicated as a trigger for the development of multi-organ anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis [2], [3], [4], [5], [6]. It is increasingly recognised that cocaine may trigger a ‘pseudovasculitis’ presentation mimicking true idiopathic ANCA associated vasculitis (AAV) [6].

We describe the clinical and serological manifestations of 14 patients from a single centre presenting with syndromes closely mimicking AAV in subjects with a history of chronic cocaine use.

Section snippets

Materials and methods

We retrospectively analysed 14 patients presenting consecutively between 2000 and 2017 to the Rheumatology department at Guy's and St. Thomas' Hospitals, London. All patients disclosed chronic habitual cocaine use, although not necessarily at initial presentation. All patients had blood samples analysed for ANCA antibodies, inflammatory markers, full blood count, liver and renal profiles. Eleven patients had computerised tomography (CT) imaging of the sinuses, 8 had chest CT scans. Ten patients

Results

There were 10 male and 4 female patients, median age 39 years (range 25–52 years). All patients disclosed cocaine exposure, albeit not necessarily at initial presentation. Nine patients admitted regular cocaine use for up to 12 years prior to their first presentation to our service, 4 patients were actively abusing cocaine at first presentation confirmed by positive urine toxicology tests (patients 10, 11, 13 and 14). The mean duration of cocaine use was 9.6 years (range 6–15 years).

Twelve patients

Discussion

Cocaine induced vasculitis may be difficult to distinguish from idiopathic AAV [7], [8]. Careful assessment of clinical, histological and serological patterns is required to make an accurate diagnosis particularly when considering treatment regimens [8].

Cocaine induced disease is usually associated with localized rather than multi-system involvement and systemic upset [9], [10]. Necrosis and inflammation leading to midline sino-nasal destruction is associated with chronic cocaine use. This may

Key messages

  • Differentiating cocaine induced pseudovasculitis from idiopathic AAV is clinically challenging; a thorough drug history and high index of suspicion are key

  • Advanced erosive nasal septal defects and atypical ANCA patterns are suggestive of cocaine induced pseudovasculitis

  • Complete drug cessation may help negate the need for potent immunosuppressive therapy

Ethics and consent

Analysis of the patient's electronic medical records and clinic correspondence was undertaken as part of a service evaluation of routine clinic practice using anonymised data. Application of the NHS Health Regulatory Agency research decision aid toolkit confirmed that formal Research Ethics Committee approval was not required.

Conflicts of interest

The authors have no conflicts of interest to declare.

Funding

No funding was received to support this manuscript.

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