Takayasu arteritis: Follow-up studies for 20 years

Summary

We reviewed retrospectively 126 (5 male, 121 female) patients suffering from Takayasu arteritis who had been treated in our clinics from 1971 to 1990. The patients' ages ranged from 19 to 80yrs old (1990) with a mean age of 48.7 ± 11.8 years. HLA typing analysis in 98 patients revealed that 45 patients (47%) were confirmed as carrying the Bw52 antigen, a high result that is statistically significant as compared with that in healthy Japanese. Arteriograms (performed in 75 patients) revealed that 28 patients (37%) were affected in the aorta and its main branches by this disease (type IV by Nasu's classification) and 23 patients (31%) were affected only in the main branches (type I). The C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) improved significantly from 2.55 ± 0.28(+) and 57.0 ± 5.69 mm/hr to 0.53 ± 0.12(+) and 31.2 ± 3.45 mm/hr, respectively after treatment including steroid and antiplatelet therapy (P < 0.01). Patients with Bw52 exhibited more severe inflammatory conditions than those without Bw52. Lung scintillations performed in 81 patients showed pulmonary arterial lesions in 50 patients (62%). Echocardiograms revealed aortic regurgitation (AR) in 44 patients (35%), with a significant difference noted between the Bw52 positive group and the Bw52 negative group [29/40 (73%) versus 11/47 (23%), respectively,P < 0.001]. Patients with Bw52 were prescribed higher doses of steroids (P < 0.05) for longer periods (P < 0.01) than those without Bw52. Of 11 patients who died during our study period, 7 died of cardiac complications, all of whom were suffering from AR. HLA analysis performed in 6 of these 7 patients revealed that all carried the Bw52 antigen. In conclusion, the retrospective survey revealed that patients carrying the Bw52 antigen showed more severe inflammatory conditions and progressed more rapidly to complications and the fatal morbid condition, as compared with those without Bw52. This suggests the important role of gene disequilibrium with this HLA antigen.