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Initial evaluation for ILDs

Identifying pulmonary fibrosis in patients as early as possible may help to improve their burden of disease, slow decline in daily functioning and quality of life, and reduce the risk of early mortality1–4

ILD patients with the HRCT scans in their hands.

For at-risk patients, HRCT should be evaluated at the first suspicion of ILD involvement, where appropriate at baseline diagnosis in the case of an underlying connective tissue disease (CTD), and repeated upon worsening of either PFT or respiratory symptoms.5–9

SIGNS AND SYMPTOMS THAT SHOULD RAISE SUSPICION OF IPF

A patient, typically over 60 years old, with dyspnea on exertion, non-productive cough, finger clubbing, or, most tellingly, velcro-like crackles on auscultation, should raise suspicion.10–12

Dyspnea / breathlessness icon.

Dyspnea

Icon of person coughing representing worsening respiratory symptoms.

Cough

Icon of patient with finger clubbing.

Finger clubbing

Icon of stethoscope representing Velcro cracks.

Velcro crackles

SIGNS AND SYMPTOMS OF NON-IPF ILDs

CLINICAL PRESENTATION OF IDIOPATHIC NON-SPECIFIC INTERSTITIAL PNEUMONIA (iNSIP)

Patients with iNSIP typically present with breathlessness and cough of usually 6–7 months’ duration, and inspiratory crackles.13,14

Proportion of comorbid symptoms in patients with iNSIP13,14

Fever, weight loss and arthralgias are the most prevalent comorbid symptoms in patients with iNSIP

CLINICAL PRESENTATION OF HYPERSENSITIVITY PNEUMONITIS (HP) 

Subacute HP may present with fever, and subacute HP or cHP may present with exertional dyspnea, cough, fatigue, malaise, anorexia, and/or weight loss.15

Physical examination typically reveals tachypnea and bibasilar inspiratory crackles.15 Wheezing may occur in some patients

Female patient with fibrotic cHP

CLINICAL PRESENTATION OF SARCOIDOSIS-ASSOCIATED INTERSTITIAL LUNG DISEASE

Sarcoidosis-associated interstitial lung disease may present with dyspnea, cough, chest pain and lung crackles lung disease can be typically found by abnormal chest X-ray:16

  • If asymptomatic, bilateral mediastinal adenopathy is typically found17

  • If symptomatic, dyspnea, cough, nonspecific chest discomfort and crackles are commonly found17

  • Wheezing is often a sign of fibrosis18

Female patient with sarcoidosis-associated interstitial lung disease

SCREENING FOR SYSTEMIC SCLEROSIS-ASSOCIATED INTERSTITIAL LUNG DISEASE (SSc-ILD)

It is recommended that all patients should be screened for ILD at baseline SSc diagnosis.7–9 Screening for ILD in patients with SSc should involve a thorough clinical assessment including chest imaging with HRCT, PFTs, DLCO and auscultation.3,8,19,20

SCREENING FOR RHEUMATOID ARTHRITIS-ASSOCIATED INTERSTITIAL LUNG DISEASE (RA-ILD)

It is recommended that patients with risk factors for ILD or pulmonary symptoms are evaluated using PFTs and HRCT at baseline RA diagnosis.21–24

How can you confirm an ILD diagnosis in your patients following screening?

Footnotes
  • cHP, chronic hypersensitivity pneumonitis; CTD, connective tissue disease; DLCO, diffusing capacity of the lung for carbon; HP, hypersensitivity pneumonitis; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; iNSIP, idiopathic non-specific interstitial pneumonia; IPF, idiopathic pulmonary fibrosis; PFT, pulmonary function test; RA, rheumatoid arthritis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.

  1. Chaudhuri N, Spencer L, Greaves M, et al. A Review of the Multidisciplinary Diagnosis of Interstitial Lung Diseases: A Retrospective Analysis in a Single UK Specialist Centre. J Clin Med. 2016;5(66):1–9. 

  2. Cottin V, Hirani N, Hotchkin D, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076.  

  3. Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc–ILD). Respir Res. 2019a;20(1):13.
  4. Wong AW, Ryerson C, Guler S. Progression of fibrosing interstitial lung disease. Respir Res. 2020:29;21(1):32.
  5. Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: A double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Resp Res. 2017;4(1):e000212. 
  6. Theodore AC, Tseng C-H, Li N, Elashoff RM, Tashkin DP. Correlation of cough with disease activity and treatment with cyclophosphamide in scleroderma interstitial lung disease: findings from the Scleroderma Lung Study. Chest. 2012;142(3):614–621.
  7. 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. 
  8. 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.
  9. 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.
  10. Raghu G, Collard HR, Egan JJ, et al; on behalf of the ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788–824.
  11. Molina-Molina M, Aburto M, Acosta O, et al. Importance of early diagnosis and treatment in idiopathic pulmonary fibrosis. Exp Rev Resp Med. 2018;12(7):537–539.
  12. Oldham JM, Noth I. Idiopathic pulmonary fibrosis: early detection and referral. Respir Med. 2014;108(6):819–829.
  13. Travis WD, Hunninghake G, King Jr TE, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Care Med. 2008;177:1338–1347. 
  14. Tomassetti S, Ryu JH, Piciucchi, et al. Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management? Semin Respir Crit Care Med. 2016;37:378–394. 
  15. Selman M, Buendia-Roldan I. Immunopathology, diagnosis, and management of hypersensitivity pneumonitis. Semin Respir Crit Care Med. 2012;33:543–554. 
  16. American Thoracic Society. Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Med. 1999;160:736–755.
  17. Ianuzzi MC, Sah BP. Sarcoidosis. Merck Manual Professional Version website. Available at: https://www.merckmanuals.com/professional/pulmonary-disorders/sarcoidosis/sarcoidosis. August 2017. Accessed: November 2020. 
  18. Patterson KC, Strek ME. Pulmonary fibrosis in sarcoidosis. Clinical features and outcomes. Ann Am Thorac Soc. 2013;10:362–370. 
  19. Roofeh D, Jaafar S, Vummidi D, Khanna D. Management of systemic sclerosis-associated interstitial lung disease. Curr Opin Rheumatol. 2019;31(3):241–249.
  20. Hoffmann-Vold A, Aaløkken TM, Lund MB, et al. Predictive Value of Serial High-Resolution Computed Tomography Analyses and Concurrent Lung Function Tests in Systemic Sclerosis. Arthritis Rheumatol. 2015;67:2205–2212. 
  21. Esposito AJ, Chu SG, Madan R, et al. Thoracic manifestations of rheumatoid arthritis. Clin Chest Med. 2019;40(3):545–560.
  22. 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.
  23. Cottin V and Cordier JF. Subclinical interstitial lung disease: no place for crackles? Am J Respir Crit Care Med. 2012;186(3):289–290.
  24. Lake F and Proudman S. Rheumatoid arthritis and lung disease: from mechanisms to a practical approach. Semin Respir Crit Care Med. 2014;35(2):222–238.
  25. Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958–968. 

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