Abstract
BackgroundEarly diffuse cutaneous systemic sclerosis (dcSSc) has the highest case fatality among rheumatic diseases. We report baseline characteristics, current immunosuppressive therapies, progression of skin and internal organ involvement, and mortality in a multicenter prospective cohort from the United States (US) of America.MethodsWe performed a longitudinal analysis of participants from 12 US centers, from April 2012 to July 2020. All participants had early dcSSc or were at-risk for dcSSc, with ≤2 years since the first non-Raynaud’s phenomenon (RP) symptom.ResultsThree hundred one patients were included with a baseline median disease duration of 1.2 years since RP and a mean modified skin score of 21.1 units. At baseline, 263 (87.3%) had definite dcSSc and 38 (12.7%) were classified as at-risk; 112 (49.6%) patients were positive for anti-RNA polymerase III antibodies. The median follow-up duration was 24.5 months (IQR = 10.3–40.7 months). One hundred ninety (63.1%) participants were treated with an immunosuppressive therapy, of which mycophenolate mofetil was most used at baseline and follow-up. Of 38 who were classified as at-risk at baseline, 27 (71%) went on to develop dcSSc; these patients were characterized by higher baseline mean HAQ-DI (0.8 versus 0.4, p = 0.05) and higher baseline mRSS (8.8 versus 4.4, p < 0.01) in comparison with those who remained as limited cutaneous SSc. In the overall cohort, 48 participants (21.1%) had clinically significant worsening of skin fibrosis, mainly occurring in the first year of follow-up; 41 (23.3%) had an absolute forced vital capacity decline of ≥10%. Twenty participants (6.6%) died, of which 18 died in the first 3 years of follow-up. Cardiac involvement (33.3%), gastrointestinal dysmotility (22.2%), and progressive interstitial lung disease (ILD) (16.7%) were the main causes of death.ConclusionThis US cohort highlights the management of early SSc in the current era, demonstrating progression of skin and lung involvement despite immunosuppressive therapy and high mortality due to cardiac involvement.
Highlights
Diffuse cutaneous systemic sclerosis has the highest case fatality among rheumatic diseases
Jaafar et al Arthritis Research & Therapy (2021) 23:170. This United States (US) cohort highlights the management of early Systemic sclerosis (SSc) in the current era, demonstrating progression of skin and lung involvement despite immunosuppressive therapy and high mortality due to cardiac involvement
Seventy-two participants (28.9%) were positive for the anti-topoisomerase I antibody, 49.6% were positive for anti-RNA polymerase III antibody, and 89% were ANA positive. 53.6% of the subjects had evidence of Interstitial lung disease (ILD) on their baseline HRCT and the pulmonary function test (PFT) revealed a mean Forced vital capacity (FVC) (% predicted) of 81.0 (18.6; n = 256) and DLCO (% predicted) of 70.6 (24.6, n = 243)
Summary
Diffuse cutaneous systemic sclerosis (dcSSc) has the highest case fatality among rheumatic diseases. Systemic sclerosis (SSc or scleroderma) is a rheumatological disorder characterized by occlusive microangiopathy associated with fibrotic features, such as skin or lung fibrosis, and the presence of autoimmune markers including specific antibodies [1]. SSc has the highest case-specific mortality with a detrimental impact on quality of life. Major changes have been made in the management of SSc in recent years, based on the results of randomized control trials (RCTs) demonstrating the positive impact of immunosuppressive drugs and autologous hematopoietic stem cell transplant on visceral involvement [3,4,5]. Management and the use of immunosuppressive therapies have become the cornerstones for the evidence-based management of patients with dcSSc [6]. The clinical impact of these modifications in the last decade and their effective implementation in routine medical care is still to be precisely determined, especially in patients with very early dcSSc, defined by less than 2 years since the onset of the first non-Raynaud’s phenomenon (RP) symptom
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