Non-giant cell arteritis of the temporal artery presenting as the polymyalgia rheumatica-temporal arteritis syndrome

We describe a heterogeneous group of 4 elderly patients with atypical vasculitis of the temporal artery who presented clinically with the polymyalgia rheumatica-temporal arteritis syndrome. The first had apparently isolated eosinophilic necrotizing vasculitis without peripheral blood eosinophilia, history of asthma, or pulmonary vasculitis (so called limited form of Churg-Strauss syndrome). Temporal artery biopsy in the 2nd patient revealed small vessel necrotizing vasculitis in the fragment of skeletal muscle surrounding a normal temporal artery (i.e., negative for giant cell arteritis, GCA). This patient subsequently developed rheumatoid polysynovitis. The temporal artery biopsies in the remaining 2 patients showed vasa vasorum vasculitis, without GCA, associated with malignancy in Patient 3 and as an unexpected and apparently isolated finding in Patient 4. All 4 patients responded to the standard corticosteroid therapy for giant cell temporal arteritis. Unusual temporal artery biopsies such as these should be interpreted in relation to clinical findings.