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Article

A syndromic framework for progressive pulmonary fibrosis.

Respiratory research - 17 Apr 2026

Perrotta Fabio, Luppi Fabrizio, Sebastiani Marco, Bianco Andrea

Abstract excerpt

Pulmonary fibrosis comprises a heterogeneous group of interstitial lung diseases (ILDs) with diverse aetiologies but often convergent clinical behaviour. Idiopathic pulmonary fibrosis (IPF) represents the prototypical fibrotic ILD; however, a substantial proportion of patients with non-IPF fibrotic ILDs develop a progressive pulmonary fibrosis (PPF) phenotype characterised by irreversible functional decline,...

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Identifiers

PMID: 41998741
DOI: 10.1186/s12931-026-03649-8
PMCID: PMC13267752

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 17 Oct 2026.

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