Wegener's granulomatosis in a patient with severe PiZZ α1-antitrypsin deficiency

Sir, We recently described the clinical presentation and outcome in eight Swedish patients with systemic necrotizing vasculitis and severe arantitrypsin deficiency followed up at three Swedish hospitals during 1968-92, and also reviewed six cases reported in the literature during the same period. 1 We here report another patient with this peculiar association, not included in the article submitted for publication. This patient is a 67-year old female, who has never smoked. She presented with haemoptysis, exertional dyspnoea, fever, and arthritis. A few days after admission, she developed erythematous papules and nodules on her lower extremities. Her blood