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Drug-induced vasculitis
A rare secondary vasculitis characterized by inflammation of blood vessels caused by various drugs, including antibiotics, anti-tumor necrosis factor-alpha agents, immunotherapeutic drugs, and psychoactive agents, among others. The skin is most commonly affected, but other tissues and organs, such as the subcutis, kidneys, or lungs, may also be involved. Systemic disease develops only in a minority of patients, typically when treated with the causative drug over a prolonged period of time. Presenting signs and symptoms include skin rash, myalgia, arthralgia, fever, and malaise.
ORPHA:251325
The incidence and prevalence of drug-induced vasculitis remains unknown. No population-based study has been performed.
Drug-induced vasculitis typically presents with a petechial rash, purpura and skin necrosis. Symptoms are usually mild and localized. Rarely, systemic involvement of the lungs, kidneys, central nervous system, and/or liver can occur; this can be severe and potentially life-threating. Clinical features include rash, fever, malaise and myalgia, with a temporal relationship with drug administration.
The etiology of drug-induced vasculitis (DIV) is incompletely understood, however, aberrant NETosis, breakdown in immune tolerance and epitope spreading, implicated in the development of autoimmunity, have been identified as potential etiological mechanisms.
Drug-induced vasculitis is a diagnosis of exclusion. There is no laboratory diagnostic tool for drug-induced vasculitis. Eosinophil count may be raised; this is seen more frequently in the case of systemic involvement. A comprehensive drug history of prescribed and over-the-counter medicines is paramount. Tissue biopsy can be beneficial in confirming the diagnosis.
Differential diagnosis includes systemic lupus erythematosus, other non-drug-induced ANCA-vasculitis, infections, and malignancies.
Treatment is focused on stopping administration of the offending agent, and is enough to induce remission in mild cases. In addition, short-course and low-dose steroids therapy can be beneficial. For severe presentations, higher doses of steroids for longer durations may be required. In severe systemic presentations patients can be treated according to ANCA-vasculitis flare protocols. Plasma exchange can also be considered. Relapse is likely upon reintroduction of the offending drug.
Most cases are mild and resolve with cessation of the offending drug without any long-term sequelae. However, in cases with severe systemic involvement, drug-induced vasculitis can result in critical illness and death.
Last update: March 2023 - Expert reviewer(s): Dr Eithne NIC AN RÍOGH
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