Abstract
Interstitial lung disease often accompanies systemic scleroderma and manifests itself in up to half of patients. It is also the most common cause of death in patients with systemic scleroderma.
According to the literature, 8 % of patients with interstitial lung involvement due to systemic scleroderma should suffer from a progressive phenotype. Recent years have yielded the results of SENSCIS and INBUILD clinical trials, which demonstrated the effect of nintedanib in slowing the decline in forced vital capacity after one year of treatment in patients with interstitial lung disease due to systemic sclerosis and in patients with a progressive phenotype of fibrotic interstitial lung diseases including those based on systemic sclerosis.
Although the side effects of nintedanib treatment (diarrhea) are usually manageable with regimens and loperamide, they are uncomfortable for patients and are more common in patients with systemic sclerosis than in patients with idiopathic pulmonary fibrosis. Therefore, it is appropriate to individually assess the potential benefits and risks of treatment for each patient and to choose the appropriate therapeutic approach accordingly.