Concurrent Churg-Strauss syndrome and temporal arteritis in a young patient with pulmonary nodules

Abstract

Very few cases of pulmonary vasculitis that cannot be classified into a single category of vasculitis have been described. We report the first case of a vasculitic process in which pulmonary involvement with asthma, eosinophilic interstitial infiltrates, and small nodules were seen in association with jaw claudication and temporal arteritis with giant cells found on biopsy. Other signs of systemic involvement were also present such as peripheral neuropathy, hematuria with erythrocytic casts and proteinuria, pericardial effusion, and a dilated cardiomyopathy. The histopathologic picture was complex and unique. The early age of onset, the multisystemic involvement, and the prompt response to cyclophosphamide pointed to a diagnosis of "polyangiitis overlap syndrome," with some aspects of Churg-Strauss syndrome and also temporal arteritis. Physicians should be aware of these polymorphous and life-threatening pulmonary vasculitic syndromes, which require aggressive immunosuppressor therapy.