The systemic sclerosis (SSc) market is expected to grow from $498 million in 2020 to $1.8 billion in 2030 across the seven major markets (7MM*) at a strong compound annual growth rate (CAGR) of 14 per cent, according to GlobalData.

The company’s latest report, ‘Systemic Sclerosis: Global Drug Forecast and Market Analysis to 2030’, reveals that recent and anticipated product launches will create new treatment options for SSc patients, leading to unprecedented market growth. GlobalData’s report focuses on the treatment landscape for SSc-associated skin fibrosis, lung fibrosis, and digital vasculopathy.

Rose Joachim, Managing Pharma Analyst at GlobalData, explains, “For decades, the treatment paradigm for SSc has mainly been limited to the off-label use of generic agents repurposed from other indications. However, there has recently been a major shift in the market landscape for the disease. First, the global launch of Boehringer Ingelheim’s Ofev (nintedanib) in 2019, in the US and Japan, and in 2020, in Europe, made it the first approved therapy for the treatment of progressive SSc-associated interstitial lung disease (SSc-ILD). In 2021, treatment options for SSc-ILD patients expanded further in the US with the approval of Roche/Genentech’s Actemra/RoActemra (tocilizumab). Key opinion leaders (KOLs) interviewed by GlobalData noted that Actemra/RoActemra had previously been used off-label for the treatment of lung and skin fibrosis throughout the 7MM, and that both on- and off-label use of the therapy would likely increase.”

GlobalData expects Ofev and Actemra/RoActemra to reach peak global sales of $508.8 million in 2025 and $163.2 million in 2024, respectively. However, the growth trajectories for the two agents will be limited by the market entry of generic nintedanib in 2026 and biosimilar tocilizumab in 2022.

Joachim continues, “Beyond the recent approvals of Ofev and Actemra/RoActemra, KOLs believe the current late-stage SSc pipeline to be one of the most promising they have ever seen, with new agents targeting skin fibrosis, lung fibrosis, and digital vasculopathy.”

GlobalData’s report highlights six late-stage pipeline therapies for SSc. These include four small molecule drugs—Civi BioPharma/Eicos Sciences’ IV iloprost (Civi-030), Horizon Therapeutics’ HZN-825 (SAR-100842), Kadmon Corporation’s belumosudil mesylate (KD-025), and Mitsubishi Tanabe Pharma’s dersimelagon (MT-7117)—and two monoclonal antibodies currently marketed for the treatment of PSO and PsA—Kyowa Kirin’s Lumicef (brodalumab) and J&J’s Tremfya (guselkumab). In regard to geographical coverage, HZN-825 and dersimelagon are being developed in both the US and 5EU**, Civi-030 and belumosudil mesylate are only in development in the US, and Lumicef and Tremfya are only being developed in Japan.

Joachim adds, “Together, these six pipeline agents are expected to represent more than 60 per cent of the global SSc market in 2030, equivalent to around $1.2 billion. HZN-825 and Civi-030 are expected to be the two highest performers during the forecast period. HZN-825, a LPAR1*** antagonist in development for the treatment of skin and lung fibrosis, is expected to reach global sales of $531.1 million by 2030. As one of the first agents likely to be approved for the treatment of digital vasculopathy in the US, Civi-030, a prostacyclin analog, is expected to see strong uptake, with peak sales reaching $536.7 million in 2028. However, nearly half of these sales will disappear by the end of the forecast period due to the anticipated market entry of generic IV iloprost in 2029.”

Although KOLs were pleased to see such an innovative late-stage pipeline for SSc, they did highlight major remaining unmet needs in the field. These include the need for disease-modifying and anti-fibrotic therapies, earlier diagnosis and treatment, and improved prognostic biomarkers.

Joachim concludes, “None of the currently marketed drugs or pipeline agents in development appear to target the underlying disease process of SSc. Likewise, new anti-fibrotic therapies, such as Ofev and Actemra/RoActemra, have only shown efficacy in halting progressive lung fibrosis, not reversing it. In the absence of drugs that target the root cause of the disease or erase past fibrotic damage, it is crucial for physicians to identify and monitor at-risk patients as quickly as possible. As such, it is necessary to develop strategies promoting earlier rheumatologist referral and the identification of prognostic biomarkers. Ultimately, KOLs were optimistic about the future SSc landscape but believe there remains significant work to be done.”

*7MM = US, France, Germany, Italy, Spain, the UK, and Japan

**5EU = France, Germany, Italy, Spain, and the UK

***LPAR1 = lysophosphatidic acid receptor 1