CTD-ILD patient cases

Patients like Anne and John have connective tissue disease-associated interstitial lung diseases (CTD-ILDs) that demand vigilance Explore these patient cases for fibrotic CTD-ILDs and their impact on patients

Steph hero image
Picture of a female patient with RA-ILD


Anne was diagnosed with rheumatoid arthritis-associated ILD one year ago

Age: 55

Underlying diagnosis: rheumatoid arthritis

Treatment history: DMARD

Pulmonary complications: RA-ILD with UIP pattern on HRCT 1 year ago

Over the past 6 months, Anne’s respiratory symptoms have worsened with coughing that has started to keep her awake most nights1

This recent evidence suggests that Anne has developed a progressive phenotype
“It was a real shock to be diagnosed with RA-ILD. I really feel, ‘Why me?’ I’ve always been so active and now I’ll have to rethink life”

Patients with RA-ILD like Anne are at risk of early death2,3


Patients with RA-ILD who experience a 10% decline in FVC % predicted at any point during follow-up are at increased risk of mortality2

A progressive fibrosing ILD phenotype develops in 40% of patients with RA-ILD4

Delays in the diagnosis of underlying ILD in patients with RA can significantly increase patients’ risk of death – ILD in patients with RA can significantly reduce survival by 7.3 years (p<0.001)3


Patients with RA-ILD have significantly worse survival than those without ILD3†
Curve showing survival rate from ILD diagnosis in RA patients with ILD vs. without ILD


Kaplan–Meier curve showing survival rate from ILD diagnosis in RA patients with ILD vs. without ILD3
Adapted from: Bongartz T, et al. Arthritis Rheum. 2010;62:1583–1591.

RA-ILD patients with UIP on HRCT have a shorter survival time than those with NSIP on HRCT2

Additionally, acute exacerbation of ILD is a serious event for all patients with RA-ILD5,6

17% of patients with RA-ILD experienced acute exacerbation of ILD over 33 months in a retrospective review of 84 patients with RA-ILD5,6

Patients with RA-ILD who experience acute exacerbation of ILD have a high risk of death in the 1.5 months immediately after the event5,6
Picture of a male patient with fibrotic iNSIP


John was diagnosed with mixed connective tissue
disease-associated ILD three years ago

Age: 44

Underlying diagnosis: mixed connective tissue disease

Pulmonary complications: MCTD-ILD 3 years ago

John has been experiencing increasing levels of dyspnea that have begun to affect his everyday activities7

This recent evidence suggests that John has developed a progressive phenotype
“Having two school-age children means I should be running them round to clubs and activities, but my CTD-ILD means I barely have the energy to play ball games in the park with my children without getting short of breath”



Patients with MCTD-ILD involving ≥5% total lung volume have significantly worse survival than those involving <5% total lung volume7‡

10-year cumulative survival rate for MCTD patients with ILD of ≥10% TLV was 60% (95% CI=29%, 81%)7‡

Significantly worse survival for patients with MCTD-ILD involving 5% or greater total lung volume compared to lower

What could you do to monitor fibrotic CTD-ILD progression in patients like John?

What can you do to help your patients like Anne and John?

How can you identify fibrotic ILD early in your patients with CTDs?

CI, confidence interval; CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; DMARD, disease-modifying antirheumatic drug; FVC, forced vital capacity; HR, hazard ratio; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; MCTD, mixed connective tissue disease; MCTD-ILD, mixed connective tissue disease-associated interstitial lung disease; RA, rheumatoid arthritis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; TLV, total lung volume; UIP, usual interstitial pneumonia.

* Hypothetical patient. Patient quotes for illustrative purposes only.

† After adjusting for age, sex and smoking (HR=2.86; 95% CI=1.98, 4.12; p<0.001).3

‡ From a long-term observational study of patients with mixed CTD (N=135) who had HRCT scans at baseline. The study examined the prevalence, extent, progression and functional effect of ILD. 10-year cumulative survival rates were 87% (95% CI=79%, 92%) and 70% (95% CI=49%, 84%) for MCTD patients with ILD of <5% TLV and ≥5% TLV, respectively (p=0.015).

  1. Iqbal K, Kelly C. Treatment of rheumatoid arthritis associated interstitial lung disease: a perspective review. Ther Adv Musculoskel Dis. 2015;7(6):247–267.

  2. Solomon JJ, Chung JH, Cosgrove GP, et al. Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J. 2016;47:588–596.

  3. Bongartz T, Nannini C, Medina-Velasquez YF, et al. Incidence and mortality of interstitial lung disease in rheumatoid arthritis: A population based study. Arthritis Rheum. 2010;62(6):1583–1591.

  4. 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. 

  5. Song JW, Lee H, Lee C, et al. Clinical Course and outcome of rheumatoid arthritis-related usual interstitial pneumonia. Sarcoidosis Vasc Dif. 2013;30:103–112.

  6. Kolb M and Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20:57.

  7. Reiseter S, Gunnarrson R, Aalokken TM, et al. Progression and mortality of interstitial lung disease in mixed connective tissue disease: a long-term observational nationwide cohort study. Rheumatology. 2018;57:255–262.

  8. 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.

  9. Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc–ILD). Respir Res. 2019a;20(1):13.

  10. Geerts S, Wuyts W, de Langhe E, et al. Connective tissue disease associated interstitial pneumonia: a challenge for both rheumatologists and pulmonologists. Sarcoidosis Vasc Dif. 2017;34:326–335.

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

  12. 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.

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

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

  15. 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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