Diagnosis of pSS-ILD

High-resolution computed tomography (HRCT) is considered the gold standard for diagnosis of interstitial lung disease (ILD) in primary Sjögren’s syndrome (pSS)1,2




HRCT should be used to confirm or exclude ILD in patients with pSS when there is reason to suspect ILD, according to symptoms, clinical examination, chest radiography or pulmonary function abnormalities.4,7

A multidisciplinary approach, including a rheumatologist, pulmonologist, radiologist and pathologist represents the best strategy to identify signs and symptoms of 

Find out more about how to diagnose pSS-ILD using HRCT


Suggested algorithm from consensus guidelines to screen for and identify ILD in patients with pSS7

Algorithm guidelines to screen and identify ILD in patients with pSS through CXR / clinical assessment / PFTs / HRCT /DLco

* The benefit of obtaining baseline PFTs in asymptomatic pSS patients with regards to long-term outcomes is not clear. This paucity of evidence and the potential costs of the test should be taken into account and discussed with individual patients prior to proceeding with screening PFTs.

Complete PFTs includes spirometry, DLCO, lung volumes, ideally measured by body plethysmography. 

Adapted from: Lee AS, et al. Chest. 2020 Oct 20. doi: 10.1016/j.chest.2020.10.011.

How can you break the news of an ILD diagnosis to your patients with pSS-ILD?

Breaking bad news is a complex communication task. The SPIKES model involves delivering bad news in clinical practice using six elements

Learn more about the SPIKES model that can help you break the bad news of an ILD diagnosis to your patients

Once you have reached a diagnosis, how should you manage your patients with pSS-ILD?

  • CXR, chest X-ray; DLCO, diffusing capacity of the lung for carbon monoxide; HRCT, high-resolution computed tomography; interstitial lung disease; PFT, pulmonary function test; pSS, primary Sjögren’s syndrome; pSS-ILD, primary Sjögren’s syndrome-associated interstitial lung disease. 

  1. Guisado-Vasco P, Silva M, Duarte-Millán M A, et al. Quantitative assessment of interstitial lung disease in Sjögren’s syndrome. PLoS One. 2019;14(11):e0224772.

  2. Li X, Xu B, Ma Y, et al. Clinical and laboratory profiles of primary Sjögren’s syndrome in a Chinese population: a retrospective analysis of 315 patients. Int J Rheum Dis. 2015;18(4):439–446.

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

  4. Luppi F, Sebastiani M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A. Lung complications of Sjogren syndrome. Eur Respir Rev. 2020;29(157):200021.

  5. 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.

  6. Manfredi A, Sebastiani M, Cerri S, et al. Prevalence and characterization of non-sicca onset primary Sjögren syndrome with interstitial lung involvement. Clin Rheumatol. 2017;36(6):1261–1268.

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

  9. Ryerson CJ, Cayou C, Topp F, et al. Pulmonary rehabilitation improves long-term outcomes in interstitial lung disease: a prospective cohort study. Respir Med. 2014;108(1):203-210. 

  10. Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968-980. 

  11. Maher TM, Wuyts W. Management of Fibrosing Interstitial Lung Diseases. Adv Ther. 2019;doi:10.1007/s12325-019-00992-9. [Epub ahead of print]. 

  12. Sgalla G, Cerri S, Ferrari R, et al. Mindfulness-based stress reduction in patients with interstitial lung diseases: a pilot, single-centre observational study on safety and efficacy. BMJ Open Respir Res. 2015;2(1):e000065. 

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