[Lung and systemic sclerosis].

Interstitial lung disease (ILD) is a frequent and potentially life-threatening complication of systemic sclerosis, affecting 40 to 50% of patients. Systematic screening with high-resolution computed tomography and pulmonary function tests (PFTs), including DLCO, is recommended at diagnosis. ILD may be present even in patients with normal PFTs. Prognosis depends on the initial extent of ILD, assessed by imaging and forced vital capacity (FVC). Current guidelines (PNDS, ACR/CHEST, EULAR) recommend a personalized management approach based on progression risk. Mycophenolate mofetil (MMF) is considered the first-line treatment. Rituximab (RTX), evaluated in several trials, has shown a favorable effect on FVC stabilization or improvement. Tocilizumab has also demonstrated a slowing of FVC decline in ILD subgroups in two randomized trials, although the primary endpoint based on the modified Rodnan skin score was negative. Nintedanib demonstrated efficacy in the SENSCIS trial by significantly reducing the annual rate of FVC decline, including in patients receiving MMF. In cases of disease progression despite appropriate initial treatment, hematopoietic stem cell transplantation or a combination of MMF and RTX may be considered. Lung transplantation can be an option for refractory forms. A comprehensive approach including oxygen therapy, pulmonary rehabilitation, and infection prophylaxis is essential.