LESSON OF THE MONTH Concurrence of sarcoidosis and aortitis: case report and review of the literature

Takayasu arteritis (TA) is a rare manifestation of systemic large vessel vasculitis which aVects predominantly the aorta and its main branches, but often remains unrecognised owing to delayed diagnosis and non-characteristic clinical features. Sarcoidosis, too, is a systemic inflammatory disease which can aVect 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 recurrent uveitis, which dated back to 1980 and was attributed to sarcoidosis. At least 12 years later aortic valve insuYciency with progressive cardiac failure 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 thorough check up failed to detect further manifestations of TA; thus, possibly, the patients had aortitis similar to, but not identical with, TA. Several related cases previously reported are discussed, suggesting that both diseases may be inherently related as they are characterised by certain non-specific, immunoinflammatory abnormalities. This case report suggests that the prevalence of TA, or related forms of arteritis, may be higher than expected and should be considered, especially in younger patients with non-characteristic cardiovascular symptoms and suspected systemic inflammatory disease. Moreover, the association with sarcoidosis in this and other previously described cases suggests that the two diseases may be related and that TA or TA-like vasculitis may even be a complication of sarcoidosis. (Ann Rheum Dis 2000;59:850–853) Takayasu’s arteritis (TA) is a chronic inflammatory disease of the large vessels which aVects mainly the thoracic aorta and its branches but can also occur elsewhere in the vascular system—for example, in the pulmonary, the coronary, mesenterial, and renal arteries. Prevalence is highest in younger women and the incidence in the Western hemisphere is around two to three cases per million personyears. Clinically, the disease is characterised by claudication, pulselessness, and asymmetric blood pressure. Hypertension is seen in about half of the patients, especially if the renovascular system is aVected. In the early stage non-specific symptoms, such as fatigue, low grade fever, or weight loss, prevail; arthralgia is also a common complaint. Some patients may be asymptomatic, depending on the amount of collateral perfusion. Laboratory findings are non-specific and are usually confined to raised erythrocyte sedimentation rate and mild leucocytosis or normochromic anaemia. There are no serological markers specific for TA. Diagnosis can be made by angiography, showing changes of the large arteries with stenosis and post-stenotic dilatation. Histologically, focal pan-arteritis is found, including granulomatous inflammation with infiltration of the media and adventitia by mononuclear cells, histiocytes, and multinucleated giant cells; endothelial cell proliferation and fibrosis of the media lead to progressive narrowing of the lumen. 2 Sarcoidosis is a systemic granulomatous disease with an incidence of 10 to 40 per 100 000 people a year. The lungs are most commonly aVected, but often there is also aVection of the musculoskeletal system, the cutaneous and central nervous systems, and the heart. Histology shows granulomas consisting of a central zone with macrophages, epithelioid cells, and multinucleated giant cells in addition to activated CD4 lymphocytes, and a peripheral zone with macrophages, fibroblasts, and CD4 and CD8 lymphocytes. Inclusion bodies are commonly found. Vasculitis may be found occasionally in patients with sarcoidosis. 7 It has not been confirmed, however, whether vasculitis is a true manifestation of the disease or an occasional, incidentally concurring abnormality. There are a few reports of the concurrence of sarcoidosis and various immunological disorders, including diVerent forms of vasculitis. Until now it has remained uncertain whether this coincidence implies any immunological relation. Here we present a rare case of a patient who had aortitis with Ann Rheum Dis 2000;59:850–853 850 Second Department of Internal Medicine, Centre for the Rheumatic Diseases, Lainz Hospital, Vienna, Austria