systemic sclerosis associated interstitial lung disease ssc ild market insight

DelveInsight’s ‘Systemic Sclerosis-associated Interstitial Lung Disease (SSc-ILD)—Market Insights, Epidemiology, and Market Forecast–2030’ report deliver an in-depth understanding of the SSc-ILD, historical and forecasted epidemiology as well as the SSc-ILD market trends in the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom) and Japan.

The SSc-ILD market report provides current treatment practices, emerging drugs, and market share of the individual therapies, current and forecasted 7MM SSc-ILD market size from 2018 to 2030. The Report also covers current SSc-ILD treatment practice, market drivers, market barriers, SWOT analysis, reimbursement, and market access, and unmet medical needs to curate the best of the opportunities and assesses the underlying potential of the market.

Geography Covered

• The United States
• EU5 (Germany, France, Italy, Spain, and the United Kingdom)
• Japan

Study Period: 2018–2030

Systemic Sclerosis-associated Interstitial Lung Disease (SSc-ILD) Disease Understanding and Treatment Algorithm

SSc-ILD Overview

Systemic sclerosis (SSc), also referred to as scleroderma, is a rare chronic, multisystem connective tissue disease characterized by microangiopathy, diffuse fibrosis, and vascular abnormalities in the skin, joints, and internal organs (especially the esophagus, lower gastrointestinal tract, lungs, heart, and kidneys). SSc encompasses a spectrum of related disorders, most of which share a characteristic clinical feature of skin thickening due to an excess of collagen fibers. The classification of SSc can be done based on limited systemic sclerosis, generalized systemic sclerosis, and systemic sclerosis sine scleroderma (Cutolo et al., 2019)

Limited systemic sclerosis is characterized by slow progression. Patients develop skin tightening over the face and distal to the elbows and knees and are often complicated by pulmonary hypertension.

Generalized systemic sclerosis evolves rapidly with diffuse skin involvement. Interstitial lung disease and scleroderma renal crisis are the major complications. In systemic sclerosis sine scleroderma, patients have systemic sclerosis-related antibodies and visceral manifestations of the disease but no skin tightening (Nevares, 2020).

Additionally, systemic sclerosis patients are sorted into two additional subgroups based on the extent of skin involvement. These subgroups are diffuse cutaneous scleroderma and limited cutaneous scleroderma.

Diffuse cutaneous scleroderma is characterized by thick or tight skin on the arms, above and below the elbows, and frequently on the legs, above and below the knees, with or without the involvement of the face. The skin thickening in diffuse cutaneous scleroderma may continue for 1–3 years before this process slows down and levels off. After 1–2 years of stability, the skin thickening usually begins to recede, and the skin begins to thin or soften.

People with Limited cutaneous scleroderma suffer from thick, tight, or hard skin on areas below, but not above, the elbows and knees, with or without the involvement of the face. Thickening of skin frequently develops gradually and is relatively unobtrusive. When measured repeatedly over time, the skin score (modified Rodnan skin score [mRSS]) in patients with limited cutaneous scleroderma is usually small and changes very little, even over many years.

Interstitial lung disease (ILD) is a frequent complication and is the leading cause of death in patients with SSc. ILD is a group of lung diseases characterized by thickening of the interstitium surrounding pulmonary alveolar walls. It is related to specific radiographic features in lung imaging and/or the presence of restrictive disorders in pulmonary function tests (PFTs).

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SSc-ILD Diagnosis

SSc-ILD is challenging to diagnose at an early stage because of the lack of specific symptoms. The disease could be a mild, clinically asymptomatic and stable disease or an aggressive disease that progresses rapidly. Therefore, the initial diagnosis of SSc-ILD is based on clinical symptoms, such as exertional dyspnea and nonproductive cough.

When a high-resolution computed tomography (HRCT) is conducted, majorly two main types of SSc-ILD are detected: nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP). The NSIP pattern is more frequent in systemic sclerosis and concerns about 78% of patients than the UIP pattern, which develops in about 10–15% of patients. In the NSIP type, the dominant changes include basal ground-glass opacities, while in UIP, most commonly reticular changes and honeycombing with mainly peripheral and basilar distribution are observed.

Although there is a scarcity of guidelines for early detection of ILD in SSc patients, some screening algorithms have been proposed. Following diagnostic approaches are used for SSc-ILD:

High-resolution computed tomography (HRCT): High-resolution computed tomography (HRCT) has become the gold standard for the diagnosis of SSc-related interstitial lung disease (SSc-ILD), especially for early-stage disease. It is a much more sensitive diagnostic test than a traditional chest radiograph, especially for detecting early or mild disease. HRCT of the chest is a noninvasive diagnostic method of SSc-ILD. The most common abnormal findings are ground-glass opacities (GGOs). Differential diagnoses should include pulmonary edema, pneumonia, chronic pulmonary thromboembolism, and alveolar hemorrhage. Other ILD patterns in HRCT images include reticular changes, honeycombing patterns, septal and nonseptal marks, and micronodules. Mediastinal lymphadenopathy is also seen in SSc-ILD patients (41–74%).

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SSc-ILD Treatment

SSc-ILD is a very heterogeneous disease, and therefore, management tends to differ according to the patient’s profile. Treatment of SSc-ILD has historically been with immunosuppressive agents. In patients with severe and/or rapidly progressive disease, both hematopoietic stem cell transplantation (HSCT) and lung transplantation have been successfully used.

• Immunosuppressants: Cyclophosphamide (CYC) and Mycophenolate Mofetil (MMF) are the most broadly prescribed immunosuppressants for SSc-ILD. A study from the European Scleroderma Trials and Research group involving over 3000 patients with SSc-ILD reported that non-selective immunosuppressive therapy was prescribed to 71% (2,681/3,778) of patients (Fischer et al., 2019)
• Tyrosine Kinase Inhibitors: There is some evidence that tyrosine kinase activity may be involved in the pathogenesis of SSc. First, abnormal signaling in the transforming growth factor β (TGF-β) pathway has been implicated, particularly for downstream effects on the intracellular tyrosine kinase c-Abl. Second, abnormalities in the platelet-derived growth factor (PDGF) axis via the PDGF receptor appears to be important. Thus, interest in evaluating tyrosine kinase inhibition as a therapeutic strategy in patients with SSc has been high (Fischer et al., 2019).
• Inhibition of IL-6: Elevated serum IL-6 has been observed in patients with SSc, with positive correlations noted between IL-6 levels, the extent of skin involvement, and SSc-ILD progression. Following the same mechanism, in March 2021, the FDA approved tocilizumab subcutaneous injection for slowing the rate of decline in pulmonary function in adult patients with SSc-ILD (Bernstein et al., 2021)
• Corticosteroids are often used in SSc patients, predominantly in combination with other immunosuppressive treatments since monotherapy with corticosteroids is generally not effective. Corticosteroid pulses have been used in association with CYC in the treatment of SSc-ILD.
• Biological immunotherapies: In terms of biological treatment, Rituximab (RTX) could be used. RTX is considered a valid treatment option in patients who do not tolerate CYC or who have a contraindication for it. RTX may also be used after CYC instead of starting maintenance with MMF or AZA, particularly if the patient fails to respond to CYC..

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SSc-ILD Epidemiology  

The disease epidemiology covered in the report provides historical as well as forecasted epidemiology segmented by Total Prevalence of Systemic Sclerosis (SSc), Total Diagnosed Prevalence of SSc, Total Prevalence of SSc-ILD, Total Diagnosed Prevalence of SSc-ILD, Gender-specific Diagnosed Prevalence of SSc-ILD, Age-specific Diagnosed Prevalence of SSc-ILD, and Severity-specific Prevalence of SSc-ILD scenario in the 7MM covering the United States, EU5 countries (Germany, France, Italy, Spain, and the United Kingdom) and Japan from 2018 to 2030.

Key Findings

• Total prevalent population of Systemic Sclerosis (SSc) in the 7MM was 191,701 in 2020 which is anticipated to increase by 2030.
• The total diagnosed prevalent cases of Systemic Sclerosis-associated Interstitial Lung Disease (SSc-ILD) in 7MM are anticipated increase in 2030 from 52,655 in 2020 during the study period (2018-2030).
• Among the EU5 countries, the United Kingdom had the highest diagnosed prevalent population of SSc-ILD with 4,232 cases, followed by Italy and France. On the other hand, Germany had the lowest diagnosed prevalent population of 2,067 in 2020. Japan had 5,689 diagnosed prevalent cases for SSc-ILD in 2020.
• A higher percentage of diagnosed prevalence was observed for females, in comparison to males, in all the 7MM countries.
• It has been observed that SSc-ILD is mostly diagnosed in the age group of 65 and above in all the 7MM countries. Majority of cases are found to be of moderate severity.

Country-Wise SSc-ILD Epidemiology

The epidemiology segment also provides the SSc-ILD epidemiology data and findings across the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan.

SSc-ILD Drug Chapters

SSc-ILD Emerging Drugs

ACE-1334: Acceleron Pharma

ACE-1334 is an Acceleron-discovered, TGF-beta superfamily-based ligand trap designed to bind and inhibit TGF-beta 1 and 3 ligands but not TGF-beta 2. The drug is administered subcutaneously. TGF-beta 1 and 3 are believed to be key signaling factors in the pathogenesis of fibrotic disease. ACE-1334 has shown robust anti-fibrotic activity in multiple preclinical models of fibrosis. ACE-1334 recently completed an ascending-dose Phase I clinical trial in healthy volunteers. The FDA has granted Fast Track designation to ACE-1334 in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) as well as Orphan Drug designation for the treatment of systemic sclerosis.

The TGF-beta superfamily consists of secreted proteins involved in the development and a range of vital cellular processes, including signaling, differentiation, metabolism, protein synthesis, motility, and invasion in a microenvironment-dependent manner. Ideally, the body can maintain homeostasis, where these proteins achieve an equilibrium supportive of these essential processes. However, imbalances leading to increases or reductions in protein levels can contribute to a host of disease states across multiple therapeutic areas.

FCR001: Talaris Therapeutics

FCR001 is an investigational, allogeneic stem cell therapy (Allo-HSCT) developed by Talaris Therapeutics to induce or restore patients’ immune tolerance (Talaris Therapeutics, 2020). Facilitated Allo-HSCT therapy, which combines administration of FCR001 with a nonmyeloablative conditioning regimen, could additionally offer a less toxic alternative to autologous HSCT (which generally requires a fully myeloablative conditioning regimen) and has the potential to enable broader use of allogeneic HSCT with a lower risk of GvHD than current approaches.

Recently, autologous HSCT has emerged as a promising and potentially disease-modifying treatment for patients with dcSSc at risk for organ failure. Data to date indicate that autologous HSCT for this indication may require a myeloablative regimen to be most effective; higher rates of relapse have been observed when less intensive conditioning regimens have been used. Nevertheless, disease recurrence still was observed in autologous HSCT patients, presumably in part because the patient’s diseased stem cells are being reinfused in the patient.

Products detail in the report…

List to be continued in the report…

SSc-ILD Market Outlook

Systemic Sclerosis (SSc) is a disease characterized by fibrosis, vasculopathy, and inflammation that may affect different organs and systems, with severe prognostic implications. When SSc pathogenetic processes manifest at the lung level, the pulmonary disease may manifest both as interstitial lung disease (ILD) and/or pulmonary arterial hypertension (PAH).

As SSc-ILD is a very heterogeneous disease, management tends to differ according to the patient’s profile. Furthermore, with the advent of new treatment options, it is essential to detect ILD as early as possible and assign the right treatment as early as possible. The treatment of SSc-ILD has evolved, reflecting the understanding of the biological processes contributing to lung injury and progress in developing targeted therapies. Although traditional immunosuppressants remain the mainstay of treatment, they are being supplemented by targeted biological and antifibrotic therapies.

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Key Findings

The market size of SSc-ILD in the seven major markets is expected to increase by 2026 and is expected to plummet down later in 2027 till 2030 from USD 580 million in 2020 for the study period (2018–2030).

• The United States accounts for the highest market size of SSc-ILD, compared to the other major markets, i.e., EU5 countries (the United Kingdom, Germany, Italy, France, and Spain), and Japan.
• Among the EU5 countries, the UK had the highest market size with USD 26 million in 2020, while Germany had the lowest market size of SSc-ILD with USD 13 million in 2020.
• Of the emerging therapies ACE-1334, and Tocilizumab are expected to enter the treatment market, with their respective products, during the forecast period.
• The expected launch of potential therapies shall increase the market size in the coming years, assisted by an increase in the prevalent population of SSc-ILD during the forecast period (2020–2030).

The United States Market Outlook

This section provides the total SSc-ILD market size and; market size by therapies in the United States.

EU-5 Market Outlook

The total SSc-ILD market size and market size by therapies in Germany, France, Italy, Spain, and the United Kingdom are provided in this section.

Japan Market Outlook

The total SSc-ILD market size and market size by therapies in Japan are provided.

SSc-ILD Drugs Uptake

This section focuses on the rate of uptake of the potential drugs recently launched in the SSc-ILD market or expected to get launched in the market during the study period 2018–2030. The analysis covers the SSc-ILD market uptake by drugs; patient uptake by therapies; and sales of each drug.  

This helps in understanding the drugs with the most rapid uptake, reasons behind the maximal use of new drugs, and allows the comparison of the drugs based on market share and size which again will be useful in investigating factors important in market uptake and in making financial and regulatory decisions.

SSc-ILD Development Activities

The report provides insights into different therapeutic candidates in Phase II, and Phase III stage. It also analyzes key players involved in developing targeted therapeutics.

Pipeline Development Activities

The report covers the detailed information of collaborations, acquisition, and merger, licensing, and patent details for SSc-ILD emerging therapies.

Reimbursement Scenario in Systemic Sclerosis-associated Interstitial Lung Disease (SSc-ILD)

Approaching reimbursement proactively can have a positive impact both during the late stages of product development and well after product launch. In the report, we consider reimbursement to identify economically attractive indications and market opportunities. When working with finite resources, the ability to select the markets with the fewest reimbursement barriers can be a critical business and price strategy.

Competitive Intelligence Analysis

We perform competitive and market Intelligence analysis of the SSc-ILD market by using various competitive intelligence tools that include–SWOT analysis, PESTLE analysis, Porter’s five forces, BCG Matrix, Market entry strategies, etc. The inclusion of the analysis entirely depends upon the data availability. 

Scope of the Report

• The report covers the descriptive overview of SSc-ILD, explaining its causes, symptoms, pathophysiology, genetic basis, and currently available therapies.
• Comprehensive insight has been provided into the SSc-ILD epidemiology and treatment.
• Additionally, an all-inclusive account of both the current and emerging therapies for SSc-ILD is provided, along with the assessment of new therapies, which will have an impact on the current treatment landscape.
• A detailed review of the SSc-ILD market; historical and forecasted is included in the report, covering the 7MM drug outreach.
• The report provides an edge while developing business strategies, by understanding trends shaping and driving the 7MM SSc-ILD market.

Report Highlights

• The robust pipeline with novel MOA and oral ROA, increasing incidence, effectiveness of drugs as both mono and combination therapy will positively drive the SSc-ILD market.
• The companies and academics are working to assess challenges and seek opportunities that could influence SSc-ILD R&D. The therapies under development are focused on novel approaches to treat/improve the disease condition.
• Major players are involved in developing therapies for SSc-ILD. The launch of emerging therapies will significantly impact the SSc-ILD market.
• Our in-depth analysis of the pipeline assets across different stages of development (phase III and phase II), different emerging trends and comparative analysis of pipeline products with detailed clinical profiles, key cross-competition, launch date along with product development activities will support the clients in the decision-making process regarding their therapeutic portfolio by identifying the overall scenario of the research and development activities.

SSc-ILD Report Insights

• Patient Population
• Therapeutic Approaches
• SSc-ILD Pipeline Analysis
• SSc-ILD Market Size and Trends
• Market Opportunities
• Impact of upcoming Therapies

SSc-ILD Report Key Strengths

• 10 Years Forecast
• 7MM Coverage
• SSc-ILD Epidemiology Segmentation
• Key Cross Competition
• Highly Analyzed Market
• Drugs Uptake

SSc-ILD Report Assessment

• Current Treatment Practices
• Unmet Needs
• Pipeline Product Profiles
• Market Attractiveness
• Market Drivers and Barriers
• SWOT analysis

Key Questions

Market Insights:

• What was the SSc-ILD market share (%) distribution in 2018 and how it would look like in 2030?
• What would be the SSc-ILD total market size as well as market size by therapies across the 7MM during the forecast period (2021–2030)?
• What are the key findings pertaining to the market across the 7MM and which country will have the largest SSc-ILD market size during the forecast period (2021–2030)?
• At what CAGR, the SSc-ILD market is expected to grow at the 7MM level during the forecast period (2021–2030)?
• What would be the SSc-ILD market outlook across the 7MM during the forecast period (2021–2030)?
• What would be the SSc-ILD market growth till 2030 and what will be the resultant market size in the year 2030?
• How would the market drivers, barriers, and future opportunities affect the market dynamics and subsequent analysis of the associated trends?

Epidemiology Insights:

• What are the disease risk, burdens, and unmet needs of SSc-ILD?
• What is the historical SSc-ILD patient pool in the United States, EU5 (Germany, France, Italy, Spain, and the UK), and Japan?
• What would be the forecasted patient pool of SSc-ILD at the 7MM level?
• What will be the growth opportunities across the 7MM with respect to the patient population pertaining to SSc-ILD?
• Out of the above-mentioned countries, which country would have the highest incident population of SSc-ILD during the forecast period (2021–2030)?
• At what CAGR the population is expected to grow across the 7MM during the forecast period (2021–2030)?

Current Treatment Scenario, Marketed Drugs, and Emerging Therapies:

• What are the current options for the treatment of SSc-ILD along with the approved therapy?
• What are the current treatment guidelines for the treatment of SSc-ILD in the US and Europe?
• What are the SSc-ILD marketed drugs and their MOA, regulatory milestones, product development activities, advantages, disadvantages, safety, and efficacy, etc.?
• How many companies are developing therapies for the treatment of SSc-ILD?
• How many emerging therapies are in the mid-stage and late stages of development for the treatment of SSc-ILD?
• What are the key collaborations (Industry–Industry, Industry-Academia), Mergers and acquisitions, licensing activities related to the SSc-ILD therapies?
• What are the recent novel therapies, targets, mechanisms of action, and technologies developed to overcome the limitation of existing therapies?
• What are the clinical studies going on for SSc-ILD and their status?
• What are the key designations that have been granted for the emerging therapies for SSc-ILD?
• What are the 7MM historical and forecasted market of SSc-ILD?

Reasons to buy

• The report will help in developing business strategies by understanding trends shaping and driving SSc-ILD.
• To understand the future market competition in the SSc-ILD market and Insightful review of the key market drivers and barriers.
• Organize sales and marketing efforts by identifying the best opportunities for SSc-ILD in the US, Europe (Germany, Spain, Italy, France, and the United Kingdom), and Japan.
• Identification of strong upcoming players in the market will help in devising strategies that will help in getting ahead of competitors.
• Organize sales and marketing efforts by identifying the best opportunities for the SSc-ILD market.
• To understand the future market competition in the SSc-ILD market.