Abstract
BACKGROUND: Patients with systemic sclerosis (SSc) have poor prognosis without cure methods. We began, 10 years ago, to relieve active SSc using short-term intravenous high-dose methylprednisolone pulse (MP-Pulse) and then maintain remission using long-term and low-dose oral glucocorticoids (LTLD-GC). METHODS: Total 46 of SSc patients with interstitial lung disease (ILD) and induration of skin during January 2006 to December 2019 were analyzed retrospectively, who were followed up for 10 years or more. The patients were treated with MP-Pulse (15 mg/kg/day, 4 days/week, for 2 weeks) with (n=21) or without (n=25) LTLD-GC (prednisone 5-10 mg/day or methylprednisolone 4-8 mg/day). The biographic and clinical data, including occurrence of infection or any adverse reactions, were collected at baseline, 6 months, 1 year, and annually through 10 years after treatment. RESULTS: From baseline to 10 years, compared with MP-Pulse alone, MP-Pulse/LTLD-GC significantly reduced skin and lung fibrosis and improved lung function: Rodnan skin score (mRSS: 22.1±12.4 to 8.16±2.5, P<0.001), forced vital capacity (FVC: 71.7% to 89.83%, P<0.001), forced expiratory volume in the first second (FEV1: 75.7% to 87.88%, P<0.001), diffusing capacity of the lung for carbon monoxide (DLCO: 63.4% to 87.73%, P<0.001), and high-resolution chest computerized tomography scan (HRCT score: 3.96±2.81 to 1.42±0.83, P<0.001). None of the 46 patients had femoral head necrosis, compression fracture, death, or life-threatening adverse events. CONCLUSION: These outcomes indicate that intravenous MP-Pulse combined with oral LTLD-GC could achieve significant remission and better long-term (10 years) efficacy without severe adverse effects in SSc patients with ILD and induration of skin.