Disease information

Systemic sclerosis (SSc) is a rare and heterogeneous connective tissue disease (CTD) characterized by fibrosis affecting the skin and multiple organs, including the lungs3,4

Interstitial lung disease (ILD) is a common manifestation of SSc3,5,6

ILD can often develop within 3 years of diagnosis in up to:5,7*

Patient Data With SSc

ILD is the leading cause of mortality in SSc, responsible for up to 35% of SSc-related deaths8,9

Causes Of Systemic Sclerosis Related Deaths

ILD can develop early in patients with SSc, so regular screening is critical6,10

CTD, connective tissue disease; ILD, interstitial lung disease; SSc, systemic sclerosis.
*As defined by physiological, radiographic, or bronchoalveolar lavage abnormalities.7


  1. Cyendiv® India pack insert version dated
  2. Distler O, et al; the SENSCIS Trial Investigators. Nintedanib for systemic sclerosis-associated lung disease. N Engl J Med. 2019;380(26):2518-2528. doi:10.1056/NEJMoa1903076.
  3. Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc-ILD). Respir Res. 2019;20(13):1-10. doi:10.1186/s12931-019-0980-7.
  4. Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390(10103):1685-1699. doi: 10.1016/S0140-6736(17)30933-9.
  5. Walker UA, et al. 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. doi:10.1136/ ard.2006.062901.
  6. Jaeger VK, et al. Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: a longitudinal EUSTAR study. PLoS One. 2016;11(10):e0163894. doi:10.1371/journal.pone.0163894.
  7. McNearney TA, et al. Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Rheum. 2007;57(2):318-326. doi:10.1002/ art.22532.
  8. Tyndall AJ, 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. doi:10.1136/ ard.2009.114264.
  9. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007;66(7):940-944. doi:10.1136/ard.2006.066068.
  10. Solomon JJ, et al. Scleroderma lung disease. Eur Respir Rev. 2013;22(127):6-19. doi:10.1183/09059180.00005512.