idiopathic pulmonary fibrosis

MeSH: D054990ORPHA: 2032

Overview

chronic, irreversible and ultimately fatal disease characterized by a progressive decline in lung function

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with idiopathic pulmonary fibrosis, sourced from HPO and Orphanet clinical annotations.

Abnormal pulmonary interstitial morphologyAcrocyanosisGastroesophageal refluxBronchiectasisPulmonary fibrosisExertional dyspneaFatigueCoughHoneycomb lungGround-glass opacificationReticular pattern on pulmonary HRCTCracklesSubpleural honeycombingUsual interstitial pneumoniaReduced forced vital capacityDecreased DLCOClubbing of fingersExercise intolerancePulmonic regurgitationOrthodeoxia

Classification & Codes

MeSH Code

D054990

Orphanet Code

ORPHA:2032
idiopathic pulmonary fibrosis
MeSHD054990
OrphanetORPHA:2032
Treatments0 drug(s)
Symptoms on record20 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO