Pulmonary fibrosis
Pulmonary fibrosis (PF) comprises a group of chronic fibrotic ILDs that carry a substantial risk of progressive respiratory failure and death. As such, PF represents an important indication for LTx. Specific ILD subtypes may be idiopathic, related to rheumatological disease or environmental exposures, or of other rare or unknown causes. Appropriate treatment depends both on ILD classification and disease behaviour, and may involve addressing underlying causes, immunomodulatory or antifibrotic pharmacological treatment, supplemental oxygen, and other nonpharmacological interventions. Despite optimal medical therapy, a substantial proportion of patients develop progressive PF, such that LTx remains an important treatment for appropriate patients. Genetic risk factors, extrapulmonary manifestations of systemic ILD causes, and other factors impact on patient selection and affect the appropriate surgical approach for LTx.
