Prevalence of

Interstitial lung disease (ILD) develops in up to 20% of primary Sjögren’s syndrome (pSS) patients1–4



ILD can be found in 8%–20% of patients with pSS1–4

Chart showing the prevalence of pSS and pSS-ILD per 100,000

Prevalence of pSS and pSS-ILD per 100,000. Prevalence figures sourced as midpoint values from ranges for pSS and pSS-ILD prevalence as follows: pSS prevalence 90–2700 per 100,000;6,7 pSS-ILD prevalence 8%–20%.2–4


ILD has been found to occur in 8–20% of patients,1–4but according to two recent studies interstitial lung abnormalities (ILAs) were prevalent in 39% and 79% of pSS patients8,9


Among patients with pSS without prior ILD, the cumulative incidence of ILD increases with the duration of pSS: 10% (±3%) ILD at 1 year, 20% (±4%) by 5 years, and 43% (±7%) by 15 years10

Typical patient characteristics in pSS-ILD 3,8,11,12


Female sex*

Older age at diagnosis has been shown to negatively affect survival
(HR 1.04, 95% CI 1.03–1.05)17

Older age




Serology: ANA+‡

             pSS patients with ILD are characteristically female and smoke:3

  • Even though male patients with pSS have a relatively greater risk of developing ILD vs. female patients, the typical pSS-ILD patient tends to be female since pSS in general is 13 times more prevalent in women vs. men3,8
  • pSS with ILD is 8 times more likely with smoking and is 3 times more likely with ANA positivity3

What impact could ILD have on your patients with RA and how can you identify this threat?

  • ANA, antinuclear antibody; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; OR, odds ratio; pSS, primary Sjögren's syndrome; pSS-ILD, primary Sjögren's syndrome-associated interstitial lung disease.

  1. 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.

  2. 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.

  3. 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.

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

  5. 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.

  6. Alamanos Y, Tsifetaki N, Voulgari PV, Venetsanopoulou AI, Siozos C, Drosos AA. Epidemiology of primary Sjögren’s syndrome in north-west Greece, 1982–2003. Rheumatology (Oxford). 2006;45(2):187–191.

  7. Patel R, Shahane A. The epidemiology of Sjögren’s syndrome. Clin Epidemiol. 2014;6:247–255.

  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. Dong X, Zhou J, Guo X, et al. A retrospective analysis of distinguishing features of chest HRCT and clinical manifestation in primary Sjögren’s syndrome-related interstitial lung disease in a Chinese population. Clin Rheumatol. 2018;37(11):2981–2988.

  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. Zhang T, Yuan F, Xu L, Sun W, et al. Characteristics of patients with primary Sjögren’s syndrome associated interstitial lung disease and relevant features of disease progression. Clin Rheumatol. 2020;39(5):1561–1568.

  12. 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.

  13. Yazisiz V, Göçer M, Erbasan F, et al. Survival analysis of patients with Sjögren’s syndrome in Turkey: a tertiary hospital-based study. Clin Rheumatol. 2020;39(1):233–241.

  14. 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.

  15. 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.

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