Monitoring and management of ILD progression in pSS-ILD

Regular monitoring for progression of primary Sjögren’s syndrome-associated intersitial lung disease (pSS-ILD) can inform appropriate treatment decisions1

Julie

MONITORING FOR PROGRESSION OF PSS-ILD USING PFTS IS RECOMMENDED EVERY 3–6 MONTHS IN THE FIRST 1–2 YEARS1

It is estimated that 24% of patients with pSS-ILD develop a progressive fibrosing phenotype2,3

The progressive fibrosing phenotype is defined by the presence of:4-7

If ILD is progressive

Increase extent of fibrosis on HRCT

If ILD is clinically significant (symptoms, severity)

Worsening respiratory symptoms

Declining lung function

Declining lung function

Risk factors for ILD progression in pSS-ILD

Age

Older8,9

HRCT pattern

UIP8–10

NSIP8–10

Clinical

Elevated ESR8

Other

Digestive involvement9

 

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

Age

Older age at disease onset has been shown to be associated with ILD progression in patients with pSS-ILD (p=0.015)8,9*
 

un

Clinical

Elevated ESR at baseline is significantly associated with ILD progression in patients with pSS-ILD (p=0.030)8*
 

hi

Other

In a retrospective multicenter study, pSS patients with progressive ILD (n=7) more commonly had digestive involvement than patients with stable/improved ILD (n=12) [57.1% vs. 8.3%, p=0.038]9*

The 2020 consensus guideline describes severity of ILD in patients with pSS according to symptoms (defined by the NYHA Functional Classification), imaging, and PFT results:1

y

Moderate

shortness of breath on exercise (NHYA II) or PFTs restricted to FVC between 60–80% predicted or DLCO between 40%–70% predicted

rheum

Severe/High

shortness of breath at rest (NHYA III, IV) or PFTs with FVC <60% predicted or DLCO <40% predicted

PROGRESSION AND SEVERITY OF ILD ARE THE MAIN FACTORS TO CONSIDER WHEN MAKING TREATMENT DECISIONS FOR pSS-ILD1

Critical review of HRCT should be conducted to determine the primary pattern in patients with pSS-ILD who fail to respond to first-line treatment or deteriorates and different treatment options should be considered.1

Treatment for pSS-ILD should be escalated when ILD progresses.1 If patients with pSS-ILD develop a progressive fibrotic ILD phenotype, appropriate treatment should be immediately considered.1

HOW COULD A MULTIDISCIPLINARY TEAM IMPROVE THE MANAGEMENT OF pSS-ILD?

Multidisciplinary management incorporating rheumatologists and respiratory specialists has a vital role in optimizing the care of patients with pSS-ILD1,11

A multidisciplinary team including a range of specialists should be involved in ILD diagnosis
Footnotes
  • * Progression of ILD is defined by decreases of ≥10% in FVC and/or ≥15% in DLCO.
    CTD-ILD, connective tissue disease-associated interstitial lung disease; DLCO, diffusing capacity of the lung for carbon monoxide; ESR, erythrocyte sedimentation rate; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; NSIP, non-specific interstitial pneumonia; NYHA, New York Heart Association; PFT, pulmonary function test; pSS, primary Sjögren’s syndrome; pSS-ILD, primary Sjögren’s syndrome-associated interstitial lung disease; UIP, usual interstitial pneumonia.

  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. Olson AL, Hartmann N, Padmaja P, et al. Estimation of the Prevalence of Progressive Fibrosing Interstitial Lung Diseases: Systematic Literature Review and Data from a Physician Survey. Adv Ther. 2020; https://doi.org/10.1007/s12325-020-01578-6.
  3. Zamora-Legoff JA, Krause ML, Crowson CS, et al. Progressive decline of lung function in rheumatoid arthritis associated interstitial lung disease. Arthritis Rheumatol. 2017;69(3):542–549.
  4. Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: a doubleblind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Respir Res. 2017;4(1):e000212. doi: 10.1136/bmjresp-2017-000212.
  5. Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076. doi: 10.1183/16000617.0076-2018.
  6. Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev. 2019;28(151):180100. doi: 10.1183/16000617.0100-2018.
  7. Kolb M and Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20(1):57. doi: 10.1186/s12931-019-1022-1.
  8. Zhang T, Yuan F, Xu L, Sun W, Liu L, Xue J. 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.
  9. Roca F, Dominique S, Schmidt J, et al. Interstitial lung disease in primary Sjögren’s syndrome. Autoimmun Rev. 2017;16(1):48–54.
  10. 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.
  11. 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.
  12. Chowaniec M, Skoczyńska M, Sokolik R, Wiland P. Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. Reumatologia. 2018;56(4):249–254.
  13. Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc–ILD). Respir Res. 2019a;20(1):13.
  14. 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. 
  15. Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968-980. 
  16. Maher TM, Wuyts W. Management of Fibrosing Interstitial Lung Diseases. Adv Ther. 2019;doi:10.1007/s12325-019-00992-9. [Epub ahead of print]. 
  17. 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.
  18. Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958–968.

Resources for patients