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Review
. 2019 Nov 5;8(11):e1086.
doi: 10.1002/cti2.1086. eCollection 2019.

Idiopathic and immune-related pulmonary fibrosis: diagnostic and therapeutic challenges

Andrew McLean-Tooke  1   2 , Irene Moore  3 , Fiona Lake  4
Affiliations
Review

Idiopathic and immune-related pulmonary fibrosis: diagnostic and therapeutic challenges

Andrew McLean-Tooke et al. Clin Transl Immunology. .

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.

Keywords: connective tissue disease; diagnosis; interstitial lung diseases (ILDs); pulmonary fibrosis; therapeutics.

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Conflict of interest statement

IM has received funding to attend an educational meeting from Boerigher Ingelheim, the manufacturers of nintenadib. AMT and FL declare no conflicts of interest.

Figures

Figure 1
Figure 1
Disease overlap between idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILD). The shaded conditions represent the focus of this paper. The size of the ovals reflects the approximate prevalence of individual diseases. IPAF: interstitial pneumonitis with autoimmune features; c‐NSIP: cellular fibrotic non‐specific interstitial pneumonia; CTD: connective tissue disease; f‐NSIP: fibrotic non‐specific interstitial pneumonia; HP: hypersensitivity pneumonitis. (adapted from Wells et al.75).
Figure 2
Figure 2
ABCDE of idiopathic pulmonary fibrosis care. GERD, gastro‐oesophageal reflux disease; OSA, obstructive sleep apnoea (reproduced from van Manen et al. 46 with permission).
Figure 3
Figure 3
Schematic diagram of sequence of profibrotic processes implicated in the current understanding of IPF pathogenesis which results in fibrosis rather than normal repair. All of these stages are targets for potential therapeutic intervention.
See this image and copyright information in PMC

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