Review article: Drug‐induced anti‐neutrophil cytoplasmic antibody‐associated vasculitis

Abstract

SUMMARY:A recent development in the field of vasculitis is the increasing recognition that certain medications such as propylthiouracil can induce anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). This review focuses on the data on causal drugs, possible pathogenesis, clinical description, diagnosis, treatment and prognosis of patients with drug-induced AAV. The pathogenesis of drug-induced AAV might be multifactorial. The clinical manifestations are similar to those of primary AAV, but ANCA with multi-antigenicity may help to differentiate it from primary AAV. The diagnosis of drug-induced AAV is based on the temporal relationship between clinically evident vasculitis and administration of the offending drugs, and excluding medical conditions that mimic vasculitis and other definable types of vasculitis. After the diagnosis of drug-induced AAV was made, the offending drugs should be withdrawn immediately, and appropriate immunosuppressive therapy should be administered only for patients with vital organ involvement. The duration of immunosuppressive therapy should be much shorter than that in primary AAV and long-term maintenance therapy might not be necessary. The prognosis of patients with drug-induced AAV is good as long as the offending drug is discontinued in time.

Does this patient have vasculitis?

“…4 Another well recognised cause is hydralazine. Vasculitis has also been documented with biologic therapies (eg tumour necrosis factor-targeted therapies) following their increasing use.…”

“…The prognosis is good provided that the offending drug is withdrawn in time. 4 When faced with an acutely sick patient with signs consistent with either systemic infection or vasculitis, it can be difficult in the early stages to narrow the differential diagnosis. In some cases therefore, the combination of empirical immunosuppression and antibiotics can be justified pending further laboratory, imaging and histological information.…”

The clinical presentation and therapy of diseases related to anti-neutrophil cytoplasmic antibodies (ANCA)

“…Studies have shown that MPO-ANCA can be found in around 20 % of patients taking the drug and among these around 20 % develop vasculitic symptoms [46]. In most cases there are only mild skin lesions, but severe multi-organ failure may occur.…”

Cutaneous Vasculopathy Associated with Levamisole-Adulterated Cocaine

“…18 ANCA-associated vasculitis has been linked to various drugs, such as hydralazine, propylthiouracil, and minocycline. 19 Immunologic abnormalities found in drug-induced ANCAassociated vasculitis include high titer anti-MPO antibodies or combined positivity of both anti-MPO and anti-PR3, ANA, and anti-cardiolipin antibodies, similar to that observed in cocaine-levamisole cutaneous vasculopathy. 19 Typically, a prolonged exposure to a causative drug is required before the development of drug-induced autoantibody production.…”