Idiopathic and Immune-related Pulmonary Fibrosis: Diagnostic and Therapeutic Challenges

Overview

Journal Clin Transl Immunology

Specialty Allergy & Immunology

Date 2019 Nov 12

PMID 31709050

Citations 16

Authors

Andrew McLean-Tooke

Irene Moore

Fiona Lake

Affiliations

Soon will be listed here.

Abstract

Interstitial lung disease (ILD) encompasses a large group of pulmonary conditions sharing common clinical, radiological and histopathological features as a consequence of fibrosis of the lung interstitium. The majority of ILDs are idiopathic in nature with possible genetic predisposition, but is also well recognised as a complication of connective tissue disease or with certain environmental, occupational or drug exposures. In recent years, a concerted international effort has been made to standardise the diagnostic criteria in ILD subtypes, formalise multidisciplinary pathways and standardise treatment recommendations. In this review, we discuss some of the current challenges around ILD diagnostics, the role of serological testing, especially, in light of the new classification of Interstitial Pneumonia with Autoimmune Features (IPAF) and discuss the evidence for therapies targeted at idiopathic and immune-related pulmonary fibrosis.

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