Efficacy of adalimumab therapy for life-threatening pulmonary vasculitis in Beh‡et's disease

Inflammatory large-vessel vasculitis in Beh double dagger et's disease may cause life-threatening arterial aneurysms that are prone to rupture. We report a patient with Beh double dagger et's disease with right ventricular thrombus and large aneurysms of the pulmonary arteries that led to recurrent episodes of hemoptysis. Following relapses and only partial response to repeated courses of cyclophosphamide and steroids, the patient was treated with adalimumab (Humira) and is now in clinical remission for over 30 months, with regression of her pulmonary lesions. Anti-TNF alpha treatment is a potential therapeutic option in patients with life-threatening complications due to large-vessel vasculitis.