Systemic sclerosis (SSc) is a rare progressive autoimmune disorder characterized by skin fibrosis, systemic inflammation, and vasculopathy that manifests as Raynaud’s disease and, often, painful digital ulcers. Risks of pulmonary arterial hypertension(PAH), interstitial lung disease (ILD), and renal crisis are high in SSc patients. The only disease-modifying therapy (DMT) approved to treat SSc is Ofev, targeted for a subpopulation of SSc patients suffering from ILD.  Boehringer Ingelheim’s Ofev awaits approval in the European Union (EU) for SSc-ILD. Ofev has the potential to advance the treatment of SSc-ILD, which partially underlies the high mortality rate in SSc.ILD and skin fibrosis are treated with systemic immunosuppressants; symptoms of PAH and vasculopathy are treated with varying combinations of vasodilators; and renal crisis is treated acutely with angiotensin-converting exzyme (ACE) inhibitors. SSc is a debilitating condition, and treatments are only marginally effective, leaving tremendous need for effective DMTs and antifibrotics.

QUESTIONS ANSWERED

  • How large is the diagnosed SSc population in the United States and EU5, and how will it change over time?
  • What is the current treatment landscape in SSc, and how will it change in the next ten years?
  • What do key opinion leaders view as the greatest unmet medical needs in the management of SSc?
  • What is the commercial potential of key emerging therapies? How will they fit into the SSc treatment landscape?

CONTENT HIGHLIGHTS

  • Geographies: United States and EU5.
  • Primary research: Six country-specific interviews with thought-leading rheumatologists supported by survey data collected for this study.
  • Epidemiology: Diagnosed and drug-treated prevalent cases of SSc by country, diagnosed prevalent cases segmented by degree of cutaneous involvement.
  • Forecast: Drug-level sales and patient share of key SSc therapies in 2018 and 2028.
  • Emerging therapies: Phase III: 2 drugs; Phase II: 9 drugs; coverage of select Phase I products.

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