Screening for ILD in pSS

While all primary Sjögren’s syndrome (pSS) patients are at risk of developing interstitial lung disease (ILD) and should be screened at baseline pSS diagnosis, the screening method depends on respiratory symptoms

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pSS PATIENTS AT RISK OF ILD SHOULD BE SYSTEMATICALLY SCREENED1,2

Up to 20% of pSS patients are affected by ILD3–6

Risk factors for development of ILD in patients with pSS

Age

  • Older age7

Sex

  • Male8

Lifestyle

  • Smoking8

Serological

  • ANA positivity8

  • Elevated CA15-3, NSE, CEA and CA125 (tumor markers, in the absence of tumors)9

  • Clinical

Longer disease duration8,10

  • Higher disease activity9

  • Lower %FVC at baseline11

  • Lower %DLCO at baseline11

  • Elevated ESR8,9

 

Icon of the person with magnifying glass, representing screening of the ILD symptoms.

pSS-ILD is more common in men than in women,8  although pSS is 13 times more common in women

Smoking

Smoking increases the risk of ILD by more than 8-fold (OR 8.55, 95% CI 1.09–67.19, p=0.040) in patients with pSS but it is important to note that pSS-ILD can occur in non-smokers8

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pSS patients with ILD have higher levels of disease activity indicators, such as EULAR Sjögren’s syndrome disease activity index, ESR, and CRP, compared to those without ILD8,9

Learn how to identify the signs and symptoms of pSS in your patients

 

pSS PATIENTS SHOULD BE SYSTEMATICALLY SCREENED AT BASELINE FOR PULMONARY INVOLVEMENT, ESPECIALLY IF THEY HAVE PRE-EXISTING PULMONARY SYMPTOMS1,2

PFTs and HRCT scans should be conducted in newly diagnosed patients with pSS experiencing chronic cough and/or dyspnea.1 Patients with pSS are at risk of ILD and should be systematically screened2,6

A 2020 expert consensus strongly recommends that clinicians must obtain a detailed medical history inquiring about respiratory symptoms in all patients with pSS at the initial and every subsequent visit1

Listening for fine bibasilar, end-inspiratory, ‘Velcro-like’ crackles on lung auscultation may be a useful screen for subclinical ILD in patients with pSS and their presence should invite comprehensive lung function assessment12

Suggested algorithm from consensus guidelines to screen for and identify ILD in patients with pSS1

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.

 

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

Footnotes
  • ANA, antinuclear antibody; CA125; cancer antigen 125; CA15-3, cancer antigen 15-3; CEA, carcinoembryonic antigen; CI, confidence interval; CRP, C-reactive protein; CTD-ILD, connective tissue disease-associated interstitial lung disease; CXR, chest X-ray; DLCO, diffusing capacity of the lung for carbon monoxide; ESR, erythrocyte sedimentation rate; EULAR, European League Against Rheumatism; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; NSE, neuron-specific enolase; OR, odds ratio; PFT, pulmonary function test; pSS, primary Sjögren's syndrome; pSS-ILD, primary Sjögren's syndrome-associated interstitial lung disease.

  1. 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.

  2. 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.

  3. Watanabe M, Naniwa T, Hara M, et al. Pulmonary manifestations in Sjögren’s syndrome: correlation analysis between chest computed tomographic findings and clinical subsets with poor prognosis in 80 patients. J Rheumatol. 2010;37(2):365–373.

  4. Sambataro G, Ferro F, Orlandi M, et al. Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sjögren’s syndrome: A systematic review from the Italian Society of Rheumatology. Autoimmun Rev. 2020;19(2):102447.

  5. Ramos-Casals M, Brito-Zerón P, Seror R, et al. Characterization of systemic disease in primary Sjögren’s syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements. Rheumatology (Oxford). 2015;54(12):2230–2238.

  6. Roca F, Dominique S, Schmidt J, et al. Interstitial lung disease in primary Sjögren’s syndrome. Autoimmun Rev. 2017;16(1):48–54.

  7. Sogkas G, Hirsch S, Olsson KM, et al. Lung involvement in primary Sjögren’s syndrome—an under-diagnosed entity. Front Med (Lausanne). 2020;7:332.

  8. Wang Y, Hou Z, Qiu M, Ye Q. Risk factors for primary Sjögren syndrome-associated interstitial lung disease. J Thorac Dis. 2018;10(4):2108–2117. 

  9. Shi L, Han X-L, Guo H-X, et al. Increased in tumor markers are associated with primary Sjögren’s syndrome-associated interstitial lung disease. Ther Adv Chronic Dis. 2020 Jul 30;11:2040622320944802. doi: 10.1177/2040622320944802.

  10. Nannini C, Jebakumar AJ, Crowson CS, et al. Primary Sjögren’s syndrome 1976–2005 and associated interstitial lung disease: a population-based study of incidence and mortality. BMJ Open. 2013;3(11):e003569.

  11. 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.

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

  13. 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. 

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

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

  16. 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. 

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

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