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
Anne was diagnosed with rheumatoid arthritis-associated ILD one year ago
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
HOW LONG COULD PATIENTS LIKE ANNE SURVIVE?
Patients with RA-ILD have significantly worse survival than those without ILD3†
Kaplan–Meier curve showing survival rate from ILD diagnosis in RA patients with ILD vs. without ILD3
Incidence and mortality of interstitial lung disease in rheumatoid arthritis: A population based study. Bongartz T, Nannini C, Medina-Velasquez YF, et al. Copyright © 2022, Arthritis Rheum. Reproduced with permission of John Wiley & Sons Inc.
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
John was diagnosed with mixed connective tissue
disease-associated ILD three years ago
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”
HOW LONG COULD PATIENTS LIKE JOHN SURVIVE?
PATIENTS WITH MCTD-ILD LIKE JOHN ARE AT RISK OF EARLY MORTALITY7
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‡
Silje Reiseter, Ragnar Gunnarsson, Trond Mogens Aaløkken, May Brit Lund, Georg Mynarek, Jukka Corander, Joanna Haydon, Øyvind Molberg, Progression and mortality of interstitial lung disease in mixed connective tissue disease: a long-term observational nationwide cohort study, Rheumatology, Volume 57, Issue 2, February 2018, Pages 255–262, by permission of Oxford University Press.
How can you identify fibrotic ILD early in your patients with CTDs?
Providing palliative/supportive care
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).
Iqbal K, Kelly C. Treatment of rheumatoid arthritis associated interstitial lung disease: a perspective review. Ther Adv Musculoskel Dis. 2015;7(6):247–267.
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.
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.
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.
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.
Kolb M and Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20:57.
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.
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.
Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc–ILD). Respir Res. 2019a;20(1):13.
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.
Wells AU, Denton CP. Interstitial lung disease in connective tissue disease— mechanisms and management. Nat Rev Rheumatol. 2014;10:728–739.
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.
Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968-980.
Maher TM, Wuyts W. Management of Fibrosing Interstitial Lung Diseases. Adv Ther. 2019;doi:10.1007/s12325-019-00992-9. [Epub ahead of print].
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.