[Takayasu's arteritis--course, diagnosis and long term results of treatment].

UNLABELLED: Nonspecific aortoarteritis (Takayasu's arteritis) is a systemic disease of unknown cause. No characteristic symptoms may delay diagnosis and treatment as well as deteriorate prognosis. The aim of the study was analysis of prevalence, course, diagnosis and treatment of Takayasu's arteritis. 10 patients (9 female, 1 male) with inflammatory phase of Takayasu's arteritis were seen at the Department of Hypertension of Institute of Cardiology between 1981 and 1992 (0.1% of all patients). The diagnosis was performed on the basis of typical arteriographic picture and laboratory investigations such as elevated of erythrocyte sedimentation rate and increase of immunoglobins, especially G fraction levels. Three patients were in poor general condition (fever, tachypnea, progressive weakness and severe arthralgia); in the remaining the symptoms were less severe, consisting mainly of weakness and arthralgia. All patients had multiple sites of arterial involvement (clinically and angiographically). Hypertension occurred in 9, aortic valve disease in 4, mitral valve disease in 2 and angina pectoris in 3 persons. All patients were treated with prednisone in initial dose of 1-1.5 mg/kg daily. After normalization of inflammatory indices (in average after 3 weeks therapy) this dose was gradually diminished to maintenance dose 5-15 mg daily. 4 patients were treated with prednisone in monotherapy, 6 received combined therapy--prednisone and cyclophosphamide or prednisone and azathioprine. Responses to immunosuppressive treatment were usually very good. In follow-up period (43.4 +/- 30.7 months) in 9 patients the regression of symptoms of inflammatory phase was observed (all patients were treated with maintenance dose of prednisone). Immunosuppressive therapy was ineffective in one woman, despite long term treatment with prednisone combined alternately with cyclophosphamide, azathioprine or methotrexate. She died of progressive heart and renal failure.

CONCLUSIONS: 1. Takayasu's arteritis is serious systemic disease with considerable risk of death. 2. Early and proper management of Takayasu's arteritis can improve prognosis of this disease. 3. In every case with multiple sites of arterial involvement, especially with associated symptoms of unidentified inflammatory disease it is necessary to consider diagnosis of Takayasu's arteritis, which prevalence seems to be underestimated.