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.


  • 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?


  • 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.

Table of contents

  • Disease Landscape & Forecast
    • COVID-19
    • Commercial Outlook
      • Key Findings
      • Drivers and Constraints
        • What Factors Are Driving Sales in Scleroderma (Systemic Sclerosis)?
        • What Factors Are Constraining Sales in Scleroderma (Systemic Sclerosis)?
      • Drug-Class-Specific Trends
        • Tyrosine Kinase Inhibitors
        • Cannabinoid CB2 Receptor Agonists
        • Antioxidant Inflammation Modulators
      • Alternative Market Scenarios
    • Forecast
    • Etiology and Pathophysiology
      • Disease Overview
      • Etiology
      • Pathophysiology
      • Clinical Presentation
      • Expert Insight
      • Key Pathways and Drug Targets
    • Epidemiology
      • Key Findings
      • Epidemiology Populations
        • Diagnosed Prevalent Cases
        • Drug-Treated Prevalent Cases
        • Diagnosed Prevalent Cases by Severity
    • Current Treatment
      • Key Findings
      • Diagnosis
        • Treatment Providers and Referral Patterns
      • Treatment Goals
      • Key Current Therapies
        • Overview
        • Angiotensin-Converting Enzyme Inhibitors
        • Autologous Hematopoietic Stem-Cell Transplantation
        • B-Cell Modulators
        • Calcium-Channel Blockers
        • Endothelin Receptor Antagonists
        • Guanylate Cyclase Stimulants
        • Immunosuppressants
        • Phosphodiesterase-5 Inhibitors
        • Prostacyclin Analogues
        • Tyrosine Kinase Inhibitors
      • Medical Practice
        • Overview
        • Treatment Guidelines
        • Region-Specific Treatment Practices
    • Unmet Need Overview
      • Current and Future Attainment of Unmet Needs in Scleroderma (Systemic Sclerosis)
    • Emerging Therapies
      • Key Findings
      • Key Emerging Therapies
        • Notable Developments Among Key Emerging Therapies for Scleroderma (Systemic Sclerosis)
        • Cannabinoid Receptor Agonists
        • Antioxidant Inflammation Modulators
        • Antifibrotics
        • T-Cell Costimulation Inhibitors
        • Autologous Stem-Cell Therapies
      • Early-Phase Pipeline Analysis
      • Key Discontinuations and Failures in Systemic Sclerosis (Scleroderma)
      • Patient Registries
        • Patient Organizations
      • Orphan Drug Designation
        • Orphan Drug Provisions: United States
        • Orphan Drug Provisions: Europe
    • Access & Reimbursement Overview
      • Region-Specific Reimbursement Practices
        • United States
        • EU5
      • Looking for More?
    • Methodology
      • Bottom-Up Forecasting Overview
        • Patient Populations
        • Drug-Specific Assumptions
      • Bottom-Up Forecast Assumptions
        • General Sources of Data
        • General Statements About Pricing
        • Dosing, Days of Therapy, and Compliance
        • Generic Erosion
        • Out-Year Forecasting
        • Emerging Therapy Prices
      • Primary Market Research
        • Experts Interviewed
    • Appendix
      • Scleroderma (Systemic Sclerosis) Bibliography

Author(s): Raina Priyadarshini, Ph.D; Sunali D. Goonesekera, SM

Raina is a senior analyst on the Infectious, Rare, and Niche Diseases team at Decision Resources Group. Before joining DRG, Raina worked in drug development for novel disease targets. She gained experience assessing market research and competitive intelligence in rare disorders in multiple therapy areas. She holds a in molecular biology from the National Institute of Immunology in India.

Sunali Goonesekera is an Associate Epidemiologist at Decision Resources Group. Sunali holds a Master’s degree in Epidemiology from the Harvard School of Public Health and a in Biology (Honors) from Dartmouth College. Prior to joining Decision Resources Group, Sunali conducted epidemiological research and lead authored two manuscripts on racial/ethnic disparities in metabolic diseases at the New England Research Institutes. She has contributed to multiple publications in peer-reviewed journals in epidemiology and in the biological sciences.

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