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Screening for ILD in CTDs

Because prevalence and prognosis of interstitial lung disease (ILD) vary among connective tissue diseases (CTDs), different screening approaches are recommended1–7

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Screening for CTD-ILDs can enable early-stage disease identification8 

DIFFERENT ILD SCREENING STRATEGIES ARE GIVEN FOR SPECIFIC CTDs1–7

sscild screening

Patients with SSc should undergo screening for ILD with chest HRCT and PFTs at diagnosis of SSc1–3   

Find out how to investigate ILD in your patients with SSc
ra

Patients with RA with risk factors for ILD or pulmonary symptoms should be evaluated for ILD at baseline RA diagnosis4,5  

Find out how to investigate ILD in your patients with RA
pssild

Patients with primary Sjögren's syndrome should be systematically screened at baseline for pulmonary involvement, especially if they have pre-existing pulmonary symptoms6,7

Find out how to investigate ILD in your patients with primary Sjögren’s syndrome

GENERAL PRINCIPLES FOR ILD SCREENING IN CTDs.

There are no established guidelines for screening patients with CTD for ILD9

However, screening for CTD-ILD can have the dual benefits of identifying early-stage disease and more specifically identifying those at greatest risk for progression and functional decline8

In patients with CTD, screening for ILD through PFTs, and a thorough physical examination (symptom assessment and lung auscultation), enables early ILD diagnosis by HRCT10

Find out how to screen for ILD using HRCT

How can you obtain an accurate ILD diagnosis in your CTD patients with suspected ILD?
Footnotes
  • 6MWD, six-minute walking distance; BAL, bronchoalveolar lavage; CCP, cyclic citrullinated peptide; CEPT, cardiopulmonary exercise testing; CT, computed tomography; CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; CXR, chest X-ray; DLCO, diffusing capacity of the lung for carbon monoxide; FVC, forced vital capacity; HRCT, high-resolution computed tomography; IIM-ILD, idiopathic inflammatory myopathy; ILD, interstitial lung disease; PFT, pulmonary function test; RA, rheumatoid arthritis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; RF, rheumatoid factor; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease; TLC, total lung capacity. 

  1. Hoffmann-Vold AM, Fretheim H, Halse AK, et al. Tracking impact of interstitial lung disease in systemic sclerosis in a complete nationwide cohort. Am J Respir Crit Care Med. 2019;200:1258–1266.

  2. Hoffmann-Vold AM, Maher TM, Philpot EE, et al. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. The Lancet Rheumatology. 2020b;2: e71–e83.

  3. Asano Y, Jinnin M, Kawaguchi Y, et al. Diagnostic criteria, severity classification and guidelines of systemic sclerosis: Guideline of SSc. J Dermatol. 2018;45:633–691.

  4. Esposito AJ, Chu SG, Madan R, et al. Thoracic manifestations of rheumatoid arthritis. Clin Chest Med. 2019;40(3):545–560.

  5. 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.

  6. Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S. Pulmonary manifestations of Sjögren’s syndrome. Eur Respir Rev. 2016;25(140):110–123.

  7. Lee AS, Scofield RH, Hammitt KM, et al. Consensus guidelines for evaluation and management of pulmonary disease in Sjögren’s. Chest. 2020 Oct 20. doi: 10.1016/j. chest.2020.10.011.

  8. Fischer A, Strek ME, Cotton V, et al. Proceedings of the American College of Rheumatology/Association of Physicians of Great Britain and Ireland Connective Tissue Disease–Associated Interstitial Lung Disease Summit: a multidisciplinary approach to address challenges and opportunities. Arthritis Rheumatol. 2019;71(2):182–195.

  9. Castelino FV, Moua T. Detection and Management of Interstitial Lung Diseases Associated With Connective Tissue Diseases. ACROR. 2021;3:295–304.8

  10. Geerts S, Wuyts W, Langhe ED, et al. Connective tissue disease associated interstitial pneumonia: a challenge for both rheumatologists and pulmonologists. Sarcoidosis Vasc Dif. 2017;34:326–335.

  11. Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958–968.