The Diagnosis and Treatment of Pulmonary Fibrosis

The different types of pulmonary fibrosis are a subgroup of the interstitial lung diseases (ILDs). They are associated with a chronic and often progressive course.

This review is based on pertinent publications retrieved by a selective search in the EMBASE and PubMed databases, with an emphasis on articles published from 2000 to 2020.

The most common type of pulmonary fibrosis is idiopathic pulmonary fibrosis (IPF). Among other relevant types, the most important ones are fibrosing hypersensitivity pneumonitis (fHP) and ILDs associated with systemic diseases, all of which are rare and generally carry a poor prognosis. The essential prerequisite to accurate diagnosis is aninterdisciplinary approach, taking account of the clinical, histological, and radiological aspects. The main complications of pulmonary fibrosis are acute exacerbations and pulmonary hypertension; comorbidities are also of prognostic relevance. Treatment of pulmonary fibrosis depends on the subtype and clinical behavior. For IPF, antifibrotic therapy is indicated; fHP, on the other hand, is mainly treated by antigen avoidance and immune modulation. The predominant mode of treatment for systemic disease-associated pulmonary fibrosis is immune suppression. Antifibrotic agents can also be useful in the treatment of other types of progressivepulmonary fibrosis besides IPF.

The differential diagnosis of pulmonary fibrosis, though complex, is clinically essential, as different types of pulmonary fibrosis are treated differently.