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
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
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‡
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.