Idiopathic interstitial lung diseases - treatment options

Idiopathic interstitial pneumonias (IIPs) represent a heterogeneous group of rare lung diseases characterized by distinct radiological and histological phenotypes. The new 2025 classification provides a more detailed definition of individual entities, including the incorporation of idiopathic eosinophilic pneumonias and rare forms, without yet altering current therapeutic approaches. The most common form is idiopathic pulmonary fibrosis (IPF), where antifibrotic therapy with pirfenidone and nintedanib has been shown to improve prognosis. Recent clinical trials (FIBRONEER, TETON) suggest further therapeutic options. The treatment of other IIPs, particularly nonspecific interstitial pneumonia (NSIP), is based on systemic corticosteroids and immunosuppressive therapy, though evidence remains limited. Complications such as pulmonary hypertension and acute exacerbations significantly impact disease course. Non-pharmacological strategies, including oxygen therapy, pulmonary rehabilitation, and, in selected cases, lung transplantation, play an important role.