This site uses cookies to improve your browsing experience. By using this site, you agree to their use. Cookie Information

close

PULMONARY FIBROSIS CAN
LEAD TO EARLY MORTALITY
IN CONNECTIVE TISSUE
DISEASES LIKE SSc1,2

Patient showing a lung scan
  • SSc explanation SSc is a heterogeneous, often devastating CTD that affects the skin and other organs3,4
  • SSc Incidence SSc typically occurs between the ages of 30 to 605
  • SSc in women and men SSc affects 3x more women than men3
  • ILD as the leading cause of death in patients with SSc ILD is the leading cause of mortality in the SSc population6
ILD accounts for almost 35% of SSc-related deaths6

HEIGHTENED VIGILANCE IS A NECESSITY IN MONITORING
CONNECTIVE TISSUE DISEASE FOR DEVELOPMENT OF ILD

Pulmonary involvement appearance in patients with SSc

For most patients with SSc,
pulmonary involvement

APPEARS EARLY

often evident within three years
of diagnosis7

 

Pulmonary fibrosis incidence on patients with diffuse cutaneous SSc

Pulmonary fibrosis
can affect

UP TO 53%

of patients with
diffuse cutaneous SSc8

Pulmonary fibrosis incidence on patients with limited cutaneous SSc

Pulmonary fibrosis
can affect

UP TO 35%

of those with
limited cutaneous SSc8

Baseline high-resolution computed tomography (HRCT) in SSc is critical for ILD detection1

MONITORING PATIENTS WITH SSc-ILD

Anna-Maria Hoffman-Vold, Postdoctoral Researcher at Oslo University Hospital,
talks about how she monitors patients with SSc-ILD

WHAT SHOULD YOU DO FOR ILD PATIENTS?

 

Pulmonary fibrosis is unpredictable—deterioration in pulmonary function tests (PFTs) should trigger urgency for further investigation9,10

  • Prompt consideration of HRCT may be in order for patients with connective tissue diseases susceptible to development of ILD when loss of pulmonary function is observed1,9

Non-CTD ILD patient profiles

 

Download profiles of patients with a range of ILDs, to view their background, diagnostic history and pulmonary function evaluation

WHAT SHOULD YOU DO FOR CTD-ILD PATIENTS?

 

Make regular pulmonary function tests (PFTs) routine in patients susceptible to CTD-ILD2

  • Conduct proactive and regular monitoring for any deterioration in respiratory symptoms such as cough or difficulty breathing11,12
  • For at-risk patients, HRCT should be evaluated at the first suspicion of ILD involvement, if possible at baseline diagnosis, and repeated upon worsening of either PFT or respiratory symptoms9,13

CTD-ILD patient profiles

 

Download profiles of patients with a range of CTD-ILDs, to view their background, diagnostic history and pulmonary function evaluation

Early supportive care can help maintain physical and emotional wellbeing in patients with ILD12

Your vigilance matters when it comes to progressive fibrosing ILD.

References
  1. 1.

    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;27:180076.

  2. 2.

    Koo S-M, Uh S-T. Treatment of connective tissue disease-associated interstitial lung disease: the pulmonologist's point of view. Korean J Intern Med. 2017;32(4):600-610.

  3. 3.

    Muangchan C, Baron M, Pope J; Canadian Scleroderma Research Group. The 15% rule in scleroderma: the frequency of severe organ complications in systemic sclerosis. A systematic review. J Rheumatol. 2013;40(9):1545-1556.

  4. 4.

    Van den Hoogen F, Khanna D, Frasen J, et al. 2013 Classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheum. 2013;65(11):2737-2747.

  5. 5.

    Olsen AL, Gifford AH, Inase N, Fernández Pérez ER, Suda T. The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressivefibrosing phenotype. Eur Respir Rev. 2018;27:180077.

  6. 6.

    Tyndall AJ, Bannert B, Vonk M, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69(10):1809-1815.

  7. 7.

    Solomon J, Olsen AL, Fisch3er A, Bull T, Brown KK, Raghu G. Scleroderma lung disease. Eur Respir Rev. 2013;22(127):6-19.

  8. 8.

    Walker UA, Tyndall A, Czirják L, Brown KK. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis. 2007;66(6):754-763.

  9. 9.

    Raghu G, Collard HR, Egan JJ, et al; on behalf of the ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.

  10. 10.

    Martinez FJ, Flaherty K. Pulmonary function testing in idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006;3(4):315-321.

  11. 11.

    Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of ... in patients with progressive fibrosing interstitial lung disease. BMJ Open Resp Res. 2017;4(1):e000212.

  12. 12.

    Theodore AC, Tseng C-H, Li N, Elashoff RM, Tashkin DP. Correlation of cough with disease activity and treatment with cyclophosphamide in scleroderma interstitial lung disease: findings from the Scleroderma Lung Study. Chest. 2012;142(3):614-621.

  13. 13.

    Raghu G, Nyberg F, Morgan G. The epidemiology of interstitial lung disease and its association with lung cancer. Br J Cancer. 2004;91(suppl 2):S3-S10.

  14. 14.

    Kreuter M, Swigris J, Pittrow D, et al. Health related quality of life in patients with idiopathic pulmonary fibrosis in clinical practice: insights-IPF registry. Respir Res. 2017;18(1):139.