Concurrence of sarcoidosis and aortitis: case report and review of the literature

Takayasu arteritis (TA) is a rare manifestation of systemic large vessel va sculitis which affects predominantly the aorta and its main branches, but o ften remains unrecognised owing to delayed diagnosis and non-characteristic clinical features. Sarcoidosis, too, is a systemic inflammatory disease wh ich can affect virtually any organ system. Reports about the coincidence of both diseases have appeared. The case presented here is characterised by a significant time lag between detection of TA and appearance of clinical signs of sarcoidosis. The woman, now 39 years old, had erythema nodosum, circumscript alopecia, and recurre nt uveitis, which dated back to 1980 and was attributed to sarcoidosis. At least 12 years later aortic valve insufficiency with progressive cardiac fa ilure developed. Histology performed at the time of aortic valve prosthesis in 1997 disclosed a diagnosis of TA, which was confined to the aortic root . Incidentally, sarcoidosis was diagnosed in adjacent lymph nodes. A thorou gh check up failed to detect further manifestations of TA; thus, possibly, the patients had aortitis similar to, but not identical with, TA. Several r elated cases previously reported are discussed, suggesting that both diseas es may be inherently related as they are characterised by certain non-speci fic, immunoinflammatory abnormalities. This case report suggests that the p revalence of TA, or related forms of arteritis, may be higher than expected and should be considered, especially in younger patients with non-characte ristic cardiovascular symptoms and suspected systemic inflammatory disease. Moreover, the association with sarcoidosis in this and other previously de scribed cases suggests that the two diseases may be related and that TA or TA-like vasculitis may even be a complication of sarcoidosis.