Objective : To estimate the suppressive effects of initial steroid therapy on subsequent disease flares and disease duration requiring maintenance steroid therapy in patients with mixed connective tissue disease (MCTD).
Methods : Clinical records from the past 15 years at Juntendo Medical School Hospital were reviewed, and 87 MCTD patients were identified (observation; 1148 person-years). The patients were classified into one of three groups based on the initial daily doses of prednisolone (PSL) : [0] 0 mg, n = 16; [L] < 20 mg (median 10 mg/d), n = 32; and [H] ? 20 mg/d (median 36 mg/d), n = 39. The entire disease course of each patient was analyzed for disease flares, which were denoted as MCTD events. Systemic lupus erythematosus disease activity index (SLEDAI) was used for estimating the initial disease activities in the patients.
Results : Means of SLEDI at presentation of MCTD were [0] 3.2 ± 2.1 (n = 16), [L] 4.8 ± 2.3 (n = 32), and [H] 4.3 ± 3.1 (n = 39), and there were no significant differences in these parameters among the three groups based on t-test. Frequencies of major organ involvement in MCTD, i.e., progressive interstitial pneumonia or myositis (IP/myositis), aseptic meningitis, and pulmonary hypertension were [0] 0/16, 0/16, 1/16; [L] 4/32, 3/32, 1/32; and [H] 9/39, 3/39, 2/39, respectively. Kaplan-Meier MCTD eventfree analysis showed that patients in the no-therapy group [0] showed frequent events (p = 0.018, log-rank test), and most patients in the treated groups were IP/myositis event free throughout the observation period (p = 0.033). Patients in group [0] required higher doses of steroid therapy when suffering the subsequent MCTD events compared with group [L] patients (PSL : 49.0 ± 12.4 mg, n = 10 vs 31.6 ± 2.9 mg, n = 5, p = 0.016). Frequency of MCTD events during the initial 3 disease years was not associated with the following relapse rate, and MCTD events were infrequent after the 3 disease years.
Conclusions : The initial steroid therapy for MCTD may suppress the following MCTD events even at a low dose. Initial steroid therapy may be beneficial even for patients with mild MCTD.
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