Systemic sclerosis (SSc) is an autoimmune disease mainly affecting skin, lung, and joints. Immunosuppressants (IS) may halt disease progression. No data are available concerning the risk of disease progression after IS withdrawal.
Among the 650 SSc patients enrolled in Padova cohort (2001-2020), 24 withdrew IS due to disease stabilization (according to expert clinician) or intolerance/poor adherence. 58% patients have diffused (dcSSc) and 42% limited SSc. We recorded IS therapies and categorized disease progression as cutaneous (mRSS increase of >=5 points or >=25%), respiratory (decline in FVC>=10%, DLCO>=15%, or extension of radiographic ILD), articular (DAS28 increase >=0.6), or renal (scleroderma renal crisis). We analyzed FVC, DLCO, mRSS and radiographic ILD extent (>= or < 20%) at IS withdrawal, at disease progression, and last follow-up.
The median duration of IS therapy was 42.3 (SD±31) months. After IS withdrawal ten patients (42%) experienced disease progression: 4 respiratory, 2 cutaneous, 3 articular and 1 renal. The average survival time without progression was 5 years. The risk for disease progression for any causes tends to be greater in patients with dcSSc (3.388 CI0.972-11.81, p=0.0554) and lower in patients who withdrew IS for disease stabilization (0.18 CI 0.04-1.2, p=0.0543). No associations were observed between the risk of progression and disease or IS duration, mRSS, and ILD extent.
The IS withdrawal leads to a huge risk of disease progression which tends to be higher in dcSSc patients and lower in those who withdrew IS for disease stabilization (according to expert clinician).