Scleroderma (systemic sclerosis [SSc]) is a rare progressive autoimmune disorder characterized by skin fibrosis, systemic inflammation, and varying degrees of vasculopathy that manifest as Raynaud’s disease and, often, painful digital ulcers. Risks of pulmonary arterial hypertension (PAH), interstitial lung disease (ILD), and renal crisis are higher in SSc patients than in the general population and underlie a high mortality rate. No disease-modifying therapy (DMT) has been approved to treat SSc. Systemic immunosuppressants are prescribed to treat ILD and skin fibrosis, while symptoms of PAH and vasculopathy are treated with varying combinations of calcium-channel blockers, endothelin receptor antagonists, PDE-5 inhibitors, and prostacyclin analogues. Renal crisis is treated acutely with ACE inhibitors, which interviewed experts credit with having reduced mortality from this complication. Still, current SSc treatments are only marginally effective, leaving tremendous need for effective DMTs and antifibrotics to treat this debilitating condition. Several marketed drugs (e.g., Boehringer Ingelheim’s Ofev, Roche’s Actemra/RoActemra) are in Phase III studies in SSc.


  • How many people are currentlydiagnosed with SScin the United States and EU5; how many will be diagnosed over time?
  • What is the commercial potential of key emerging therapies? How will emerging therapies fit into the SSc treatment landscape?
  • What is the current treatment landscape in SSc, and how will it change in the next ten years?
  • What do key opinion leaders consider the greatest unmet medical needs in the management of SSc?


Niche & Rare Disease Landscape & Forecast: Comprehensive market intelligence providing world-class epidemiology, keen insight into current treatment paradigms, in-depth pipeline assessments, and drug forecasts supported by detailed primary and secondary research.

Table of contents

  • Disease Landscape & Forecast
    • 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
        • Antifibrotics
        • Cannabinoid CB2 Receptor Agonists
        • IL-6 Inhibitors
        • Tyrosine Kinase Inhibitors
    • 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
        • Severity Status
    • 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
      • 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)
        • Antifibrotics
        • Autologous Stem-Cell Therapies
        • Cannabinoid Receptor Agonists
        • IL-6 Inhibitors
        • Nrf2 Activators
        • Kinase Inhibitors
        • T-Cell Costimulation Inhibitors
      • Early-Phase Pipeline Analysis
        • Notable Developments in the Early-Phase Pipeline for Scleroderma (Systemic Sclerosis)
      • 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
        • Generic Erosion
        • Biosimilar Erosion
        • Out-Year Forecasting
        • Emerging Therapy Prices
      • Primary Market Research
        • Experts Interviewed
    • Appendix
      • Scleroderma (Systemic Sclerosis) Bibliography

Author(s): Ian Love, PhD

Ian is an analyst on the Infectious, Niche, and Rare Diseases team at Decision Resources Group, specializing in niche and rare diseases. Ian conducts primary and secondary research to gauge the market landscapes of these diseases, thoroughly analyzing disease etiology, current treatments, unmet need, and emerging therapies expected to impact their respective markets. He received his doctorate in biomedical sciences from the University of Massachusetts Medical School and his in cell & molecular biology from the Worcester Polytechnic Institute. Prior to joining Decision Resources Group, Dr. Love was an Instructor in the Department of Internal Medicine at Virginia Commonwealth University.

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