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The impact of
fibrotic ILD

Progressive pulmonary fibrosis (PPF) in interstitial lung disease (ILD) can be deadly – your early recognition of fibrotic ILD on HRCT is critical1–4

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A COMMON THREAT ACROSS A WIDE RANGE OF FIBROTIC ILDs1–4

Progressive pulmonary fibrosis (PPF) in ILDs is a key driver of irreversible harm and early mortality1–4

Idiopathic pulmonary fibrosis (IPF), by definition, is a progressive fibrosing ILD.5,6 In addition to IPF, disease progression occurs in a range of other ILDs.5,7–11

Patients with a range of ILDs may develop PPF, including connective tissue disease-associated ILDs (CTD-ILDs)11

Patients with a range of ILDs may develop PPF, including connective tissue disease-associated ILDs (CTD-ILDs)

The coral shaded area represents the estimated proportion of patients with various types of ILD who manifest PPF.

Antifibrotic treatment is indicated for patients diagnosed with IPF.12 Antifibrotic treatment of the other types of ILD upon manifesting PPF is as suggested/recommended in the referenced guideline.

*The guideline committee acknowledges that eosinophilic pneumonia of unknown cause was not included in the IIP classification. †Myositis includes PM/DM/antisynthetase syndrome, which may be amyopathic. ‡Although respiratory bronchiolitis interstitial lung disease (RBILD) is acknowledged to be a consequence of exposure to cigarette smoke in virtually all patients with RBILD, RBILD and desquamative interstitial pneumonia (DIP) often coexist. Although DIP is also related to exposure with cigarette smoke in a majority of patients, DIP is also seen in some patients with connective tissue disease, without exposure to cigarette smoke, and without a known cause.

AFOP, acute fibrinous and organizing pneumonia; AIP, acute interstitial pneumonia; COP, cryptogenic organizing pneumonia; HP, hypersensitivity pneumonitis; iDIP, idiopathic DIP; IIP, idiopathic interstitial pneumonia; iLIP, idiopathic lymphoid interstitial pneumonia; iNSIP, idiopathic non-specific interstitial pneumonia; iPPFE, idiopathic pleuroparenchymal fibroelastosis; LAM, lymphangioleiomyomatosis; LCH, Langerhans cell histiocytosis; MCTD, mixed connective tissue disease; PAP, pulmonary alveolar proteinosis; PM, polymyositis; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus; SSc, systemic sclerosis.

Adapted from: Raghu G, et al. Am J Respir Crit Care Med. 2022;205(9):e18–e47.

ILD often develops early in the course of a CTD13–16

ILD may even be the first manifestation of a previously undiagnosed or unrecognized CTD.13–16

PPF in ILDs can be deadly4,17–27

PPF in ILDs can be deadly

cHP, chronic hypersensitivity pneumonitis; IPF, idiopathic pulmonary fibrosis; RA-ILD, rheumatoid arthritis-associated ILD; SSc-ILD, systemic sclerosis-associated ILD; uILD, unclassifiable interstitial pneumonitis.

Your early recognition of fibrotic ILD on HRCT can lead to treatment that can slow disease progression and reduce the risk of early mortality5,7,28-30

Footnotes

AFOP, acute fibrinous and organizing pneumonia; AIP, acute interstitial pneumonia; cHP, chronic hypersensitivity pneumonitis; COP, cryptogenic organizing pneumonia; CTD, Connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; HP, hypersensitivity pneumonitis; HRCT, high-resolution computed tomography; iDIP, idiopathic desquamative interstitial pneumonia; IIP, idiopathic interstitial pneumonia; ILD, interstitial lung disease; iLIP, idiopathic lymphoid interstitial pneumonia; iNSIP, idiopathic non-specific interstitial pneumonia; IPF, idiopathic pulmonary fibrosis; iPPFE, idiopathic pleuroparenchymal fibroelastosis; LAM, lymphangioleiomyomatosis; LCH, Langerhans cell histiocytosis; MCTD, mixed connective tissue disease; PAP, pulmonary alveolar proteinosis; PPF, progressive pulmonary fibrosis; RA, rheumatoid arthritis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; RBILD, respiratory bronchiolitis interstitial lung disease; SLE, systemic lupus erythematosus; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease; uILD, unclassifiable interstitial pneumonitis.

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