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Introduction Systemic sclerosis (SSc) is a rare disease characterized by widespread collagen deposition resulting in fibrosis. Although skin involvement is the most common manifestation and also the one that determines the classification of disease, mortality in SSc is usually a result of respiratory compromise in the form of interstitial lung disease (ILD) or pulmonary hypertension.Material and Methods To assess the efficacy of rituximab (RTX) treatment in 2 female pts (44 and 39 years old) with the diffuse type of SSc-associated ILD and polymyositis (Pm), hospital records were reviewed between 2015 and 2025 yy.Results The disease duration was of 16 and 11 years. The ILD was joined in the fifth and second year after the onset of the disease, Pm - in the ten and second year after the onset of the disease, respectively. Both pts were treated low-dose prednisone until 2015. The first patient received at least 10 cycles of RTX (cumulative dose of 10 g), the second patient - 7 cycles of RTX (cumulative dose of 8 g). The pts were also treated with mycophenolate mofetil 2000 mg/day continuously for the last 8 and 7 years, respectively. Both pts received low-dose prednisone. Pulmonary function tests (PFTs) and chest high-resolution computed tomography (HRCT) were performed before and after treatment RTX to assess the response. RTX therapy prevented the further decline of FVC and DLCO in PFT (FVC 69.4% / DLCO 42,1% and FVC 110.2%/ DLCO 55%, respectively (before RTX) and FVC 84,2%/ DLCO 41,6% and FVC 88%/ DLCO 55%, respectively (after RTX)). There was a stabilization of the condition against the background of RTX therapy, but according to HRCT data, there was a decrease in the volume of the lower lobes of the lungs. These results provide further evidence that RTX is an effective treatment for SSc-ILD. Our clinical observation suggests that therapy with RTX and MMF is well-tolerated, safe, and potentially effective for pulmonary involvement in SSc-Pm overlap syndrom.Conclusions The stabilization of pulmonary disease was observed in SSc-Pm pts with longer disease duration and worse pulmonary function. Rituximab might be useful in this patient group.