Double Immunosuppression With or Without Anti-fibrotic in Scleroderma ILD

Systemic sclerosis (scleroderma) is a rare systemic autoimmune disease. It primarily affects the skin, but it can also involve other vital organs and systems, and is characterized by fibrosis, vascular abnormalities, and the production of autoantibodies. Its main pathological feature is the excessive production and deposition of collagen in the skin and other organs.

Mortality in scleroderma varies depending on the type and severity of the disease, particularly on whether vital organs are involved. Patients with limited cutaneous scleroderma generally have a better prognosis and life expectancy, whereas those with diffuse scleroderma face a higher risk of death from disease-related complications, mainly due to lung involvement.

Pulmonary involvement in scleroderma is common, either affecting the pulmonary blood vessels (pulmonary hypertension) or the supporting structure (interstitium) of the lungs, leading to interstitial fibrosis.

Objective:

The aim of the study is to compare patients receiving only dual immunosuppressive therapy (control group) with patients receiving dual immunosuppressive therapy combined with antifibrotic treatment (e.g., nintedanib).