Skip to main content
For Healthcare Professionals
Banner-Background

AUTOIMMUNE ILDs AND PPF

Patients with autoimmune diseases are at risk of developing interstitial lung disease (ILD)1,2

ILDs are common manifestations that can often present early in the course of disease and can progress into progressive pulmonary fibrosis (PPF)1,2

autoimmune
Quicklinks

RISK OF MORTALITY

Once progressive, patients with autoimmune ILDs face a high risk of mortality5

5-year-mortality

MCTD, mixed connective tissue disease; RA, rheumatoid arthritis; SSc, systemic sclerosis.

DETECTING PROGRESSION

PPF is defined as the occurrence of at least 2 of the 3 criteria within the past year with no alternative explanation11-14

worsening
Worsening of symptoms such as dyspnea, cough, and fatigue
radiological
Radiological evidence of disease progression (eg, increased extent of fibrosis)
physyological
Physiological evidence of disease progression (either ≥5% absolute FVC decline or ≥10% absolute DLco decline within 1 year)

DLco, diffusing capacity for carbon monoxide; FVC, forced vital capacity.

Screening all at-risk individuals at baseline is key for early detection and treatment15,16

idintify
Identify risk factors15,16

Identify high-risk patients by screening for ILD risk factors

screen
Screen at baseline17

Screen high-risk ILD patients with PFTs and HRCTs to establish a baseline

monitor
Monitor for progression16,17

Monitor for ILD progression with HRCTs, PFTs, and ambulatory desaturation testing

HRCT, high-resolution computed tomography; PFT, pulmonary function test.

Help your autoimmune ILD patients with PPF manage their disease with a proven treatment option 

Explore the data
Quicklinks
References

  1. Wallace B, Vummidi D, Khanna D. Management of connective tissue diseases associated interstitial lung disease: a review of the published literature. Curr Opin Rheumatol. 2016;28(3):236-245.

  2. Wells AU, Denton CP. Interstitial lung disease in connective tissue disease―mechanisms and management. Nat Rev Rheumatol. 2014;10(12):728-739.

  3. Podolanczuk AJ, Hunninghake GM, Wilson KC, et al. Approach to the evaluation and management of interstitial lung abnormalities: an official American Thoracic Society clinical statement. Am J Respir Crit Care Med. 2025;211(7):1132-1155.

  4. Wijsenbeek M, Kreuter M, Olson A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin. 2019;35(11):2015-2024.

  5. Kolb M, Bondue B, Pesci A, et al. Acute exacerbations of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27:180071.

  6. Raimundo K, Solomon JJ, Olson AL, et al. Rheumatoid arthritis-interstitial lung disease in the United States: prevalence, incidence, and healthcare costs and mortality. J Rheumatol. 2019;46(4):360-369.

  7. Su R, Bennett M, Jacobs S, et al. An analysis of connective tissue disease-associated interstitial lung disease at a US tertiary care center: better survival in patients with systemic sclerosis. J Rheumatol. 2011;38(4):693-701.

  8. Pang R, Ma X, Guo H, Qi X. Meta-analysis of mortality-associated factors in primary Sjögren's syndrome patients with interstitial lung disease. Clin Rheumatol. 2025;44(1):23-31.

  9. Li Y, Gao X, Li Y, et al. Predictors and mortality of rapidly progressive interstitial lung disease in patients with idiopathic inflammatory myopathy: a series of 474 patients. Front Med (Lausanne). 2020;7:363.

  10. Nasser M, Larrieu S, Boussel L, et al. Estimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study). Respir Res. 2021;22(162):162.

  11. Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2022;205(9):e18-e47.

  12. Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20(1):57.

  13. Swigris JJ, Brown KK, Abdulgawi R, et al. Patients’ perceptions and patient-reported outcomes in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180075.

  14. Reichmann WM, Yu YF, Macaulay D, Wu EQ, Nathan SD. Change in forced vital capacity and associated subsequent outcomes in patients with newly diagnosed idiopathic pulmonary fibrosis. BMC Pulm Med. 2015;15:167.

  15. Castelino FV, Moua T. Detection and management of interstitial lung diseases associated with connective tissue diseases. ACR Open Rheum. 2021;3(5):295-304.

  16. Johnson SR, Bernstein EJ, Bolster MB, et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Screening and Monitoring of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases. Arthritis Rheumatol. 2024;76(8):1201-1213.

  17. Antoniou KM, Distler O, Gheorghiu A-M, et al. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease. Eur Respir J. 2026;67(1):2402533.

MLR ID: PC-AE-102895
Expiry Date: 10/05/2028