Rheumatic Manifestations of Cocaine Use

Jonathan Graf


Curr Opin Rheumatol. 2013;25(1):50-55. 

In This Article

Abstract and Introduction


Purpose of review: Cocaine use is associated with several rheumatic syndromes. This review summarizes these clinical manifestations and highlights recent developments linked to levamisole-adulterated cocaine.

Recent findings: Cocaine use has been linked to several distinctive syndromes that can be difficult to distinguish from idiopathic rheumatic diseases. These disorders can range in severity from purely cosmetic damage to organ and/or life-threatening disease that includes sinonasal destruction and vasculitis. Many of these illnesses are associated with antineutrophil cytoplasmic antibodies (cytoplasmic, perinuclear and atypical perinuclear patterns). With the recent introduction of levamisole as a cocaine adulterant, a newly reported syndrome has emerged that is associated with neutropenia, retiform purpura with cutaneous necrosis and autoantibodies consisting of high-titre perinuclear antineutrophil cytoplasmic antibodies (p-ANCAs) with specificities for 'atypical' antigens.

Summary : Cocaine use is associated with clinical syndromes that closely mimic other primary rheumatic diseases. Given the high prevalence of cocaine use and its adulteration with levamisole, clinicians should be familiar with these rheumatic manifestations in order to avoid misdiagnosis and unnecessary treatment with potentially toxic therapies.


Illicit drug use is common in many societies across the globe and causes significant morbidity and mortality relating to well known harmful physiologic effects of these substances. Many of these drugs, including but not limited to amphetamines (including prescription and over-the-counter medications containing amphetamine-like substances) and cocaine, have also been linked to unusual rheumatic syndromes and vasculitis. Recently, a novel rheumatic syndrome has been reported in cocaine users, and this review will therefore focus primarily on cocaine and its link to newly recognized as well as previously established rheumatic diseases.

Approximately 1.9 million Americans are estimated to have actively used cocaine in 2008, with as many as 1.5% of young adults between the ages of 18 and 25 years having reported using cocaine in the month prior to being surveyed.[1] Cocaine is a powerful, addictive stimulant with well defined pharmacologic effects on the cardiovascular and cerebrovascular systems.[2] Cocaine use has also been implicated in a variety of unusual autoimmune syndromes that can vary in clinical presentation from frank vasculitis (organ-specific or systemic), to necrotic and destructive sinonasal lesions, to autoantibody production.[3–5] The evaluation of a patient with a suspected cocaine-associated rheumatic syndrome requires a high index of suspicion and can be quite complicated to pursue. First, many patients are unwilling to admit to cocaine use. Second, it can induce a clinical syndrome of inflammation, vasculitis and/or autoantibody production, and can closely resemble a primary, idiopathic vasculitis. Finally, cocaine use can promote atherogenesis, arterial stenoses and aneurisms in the absence of vasculitis or systemic autoimmunity.

Many of these syndromes have previously been well described, including in earlier editions of this journal.[5] However, a novel cocaine-associated syndrome has recently emerged as a public health concern and has become the subject of several medical case series and government-issued public health advisories. This recent flurry of cases has been linked not to cocaine itself but to the increasing use of the antihelminthic drug, levamisole, to process and 'cut' cocaine. Exposure to levamisole can lead to a distinctive syndrome that includes neutropenia, necrotizing skin lesions with retiform purpura and a characteristic autoantibody profile.[6] Given the widespread use of cocaine, clinicians should recognize the classic features of this levamisole-associated syndrome while also being aware that its full extent has yet to be completely characterized clinically or mechanistically.