Adult-onset Still Disease - Market Insight, Epidemiology, and Market Forecast - 2034

Key Highlights:

• According to DelveInsight's estimates, in 2023, there were approximately 7,464 diagnosed prevalent cases of Adult-Onset Still's Disease (AOSD) in the 7MM. Of these, the US which accounted for approximately 31% of the cases, EU4 and the UK countries accounted for around 37%, followed by Japan which represented nearly 32%.
• The AOSD market is set for steady growth, with a robust compound annual growth rate (CAGR) of 5% anticipated from 2024 to 2034. This expansion in the 7MM is driven by the introduction of innovative therapies such as Tadekinig Alfa, as well as growing awareness and diagnosis, and advancements in treatment options.
• A major unmet need in the treatment of AOSD is the lack of personalized therapeutic strategies, as current treatments often lead to variable efficacy and adverse effects, necessitating more targeted and individualized approaches.
• FDA-approved drugs like Canakinumab (Novartis), Anakinra (Swedish Orphan Biopharma), and Tocilizumab (Roche) for AOSD indicate strong market potential. Their approval underscores the growing recognition of AOSD as a distinct therapeutic target.
• A significant market barrier in AOSD is the rarity and diagnostic complexity of the condition, leading to underdiagnosis and delayed treatment. This limits patient identification, thereby constraining the market growth potential for AOSD therapies.

DelveInsight's "Adult-onset Still's Disease (AOSD) - Market Insights, Epidemiology, and Market Forecast - 2034" report delivers an in-depth understanding of AOSD, historical and forecasted epidemiology, as well as the AOSD market trends in the United States, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan.

The AOSD market report provides current treatment practices, emerging drugs, market share of individual therapies, and current and forecasted 7MM AOSD market size from 2020 to 2034. The report also covers AOSD treatment practices/algorithms and unmet medical needs to curate the best opportunities and assess the market's potential.

AOSD Understanding and Treatment Algorithm

AOSD overview

AOSD is a rare systemic inflammatory disorder characterized by an unknown etiology, leading to significant morbidity. Clinically, it presents with a constellation of symptoms, including high fever, a distinctive rash, prolonged joint pain, and systemic manifestations such as sore throat, splenomegaly, hepatomegaly, and lymphadenopathy. While the precise causes remain elusive, AOSD may be triggered by environmental factors or infections in genetically predisposed individuals. Risk factors include a history of autoimmune diseases and age, as it typically affects adults in their 20s to 40s. The complexity of diagnosis and variability in symptom presentation contribute to challenges in management and treatment, highlighting the need for greater awareness and research into this condition.

AOSD diagnosis

The diagnosis of AOSD necessitates a comprehensive clinical evaluation, including a detailed patient history and the identification of characteristic clinical findings. As AOSD is primarily a diagnosis of exclusion, it requires ruling out other inflammatory and autoimmune disorders. Diagnostic procedures typically include blood tests that reveal specific hematological changes, such as elevated white blood cell counts and anemia. A critical component is the erythrocyte sedimentation rate (ESR) test, which assesses systemic inflammation. Additional tests may encompass C-reactive protein (CRP) levels, liver function tests, and serological evaluations for other autoimmune diseases. Ultimately, a combination of clinical criteria and laboratory findings guides the diagnosis, emphasizing the need for a systematic approach to effectively identify AOSD.

AOSD treatment

The treatment landscape for AOSD is characterized by limited options that primarily include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and antirheumatic agents. Given the disease's highly variable clinical course-ranging from self-limiting forms to potentially life-threatening complications-no single optimal therapy has been universally established; treatment often requires individualization based on patient response. In cases where NSAIDs and corticosteroids are insufficient, the introduction of biologic disease-modifying antirheumatic drugs (bDMARDs) represents a significant advancement. The recent FDA and European approvals of Anakinra, Canakinumab, and Tocilizumab have notably transformed AOSD management, offering targeted therapies that address the underlying inflammatory processes. These biologics provide clinicians with new avenues for effectively managing the disease, thereby improving patient outcomes and overall quality of life.

AOSD Epidemiology

As the market is derived using a patient-based model, the AOSD epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented by total prevalent cases of AOSD, total diagnosed prevalent cases of AOSD, gender-specific diagnosed prevalent cases of AOSD, and complication associated diagnosed prevalent cases of AOSD in the 7MM covering the United States, EU4 countries (Germany, France, Italy, and Spain) and the United Kingdom, and Japan from 2020 to 2034.

• According to DelveInsight's epidemiology model, in the US, the total diagnosed prevalent cases of AOSD were approximately 2,325 in 2023. This number is anticipated to rise during the forecast period (2024-2034), driven by increased awareness and screening, along with advancements in genetic testing.
• Among the EU4 and the UK, Germany accounted for the highest number of diagnosed prevalent cases of AOSD, with approximately 691 cases in 2023, followed by France with approximately 560 cases, and the UK with nearly 550 cases.
• Among the gender-specific diagnosed prevalent cases of AOSD in the US in 2023, there were approximately 1,627 cases for females and around 697 cases for males.
• In 2023, Japan had the highest number of diagnosed prevalent cases of AOSD among the 7MM, totaling approximately 2,362 cases.
• In 2023, complications were categorized into Reactive Hemophagocytic Syndrome (RHS) and Macrophage Activation Syndrome (MAS). Notably, Macrophage Activation Syndrome (MAS) had a higher prevalence, with approximately 373 cases, compared to 307 cases of Reactive Hemophagocytic Syndrome (RHS) reported in Japan.

AOSD Drug Chapters

Marketed Drugs

ILARIS (canakinumab): NOVARTIS

ILARIS (canakinumab), a human monoclonal anti-human IL-1B antibody of the IgG1/? isotype, was approved by the FDA in June 2020, for the treatment of AOSD in patients aged 2 years and older, including both systemic juvenile idiopathic arthritis (SJIA) and AOSD. Canakinumab works by binding to IL-1B and neutralizing its activity, thus blocking its interaction with IL-1 receptors, a crucial mechanism in controlling the inflammatory response in conditions like AOSD. The FDA granted ILARIS Priority Review designation, recognizing its potential to significantly improve the safety or effectiveness of treating a serious condition.

KINERET (anakinra): Swedish Orphan Biovitrum

KINERET (anakinra) is indicated in Europe for the treatment of rheumatoid arthritis (RA) in adults who have had an inadequate response to methotrexate alone, and for treating cryopyrin-associated periodic syndromes (CAPS) in patients aged 8 months and older with a body weight of 10 kg or more. It is also approved for treating Still's disease, including SJIA and AOSD with moderate to high disease activity or continued disease activity after NSAIDs or glucocorticoids. KINERET can be administered as monotherapy or in combination with other anti-inflammatory drugs and DMARDs. Anakinra received US orphan drug status from the FDA in September 2015.

ACTEMRA (tocilizumab): Genentech (ROCHE)

ACTEMRA (tocilizumab), a humanized anti-human IL-6 receptor monoclonal antibody, is approved for the treatment of AOSD in both the United States and Japan. The FDA granted approval in 2017, recognizing ACTEMRA's efficacy in targeting IL-6 receptors to mitigate the inflammatory response associated with AOSD. In May 2019, Chugai Pharmaceutical announced that it had secured regulatory approval from Japan's Ministry of Health, Labour and Welfare (MHLW) for ACTEMRA Intravenous Infusion (80 mg, 200 mg, and 400 mg) for adult Still's disease, particularly in cases unresponsive to existing therapies, along with an additional dosing regimen. ACTEMRA'S approval underscores its therapeutic significance in addressing unmet needs in this challenging condition.

Emerging Drugs

Tadekinig Alfa: AB2 Bio

Tadekinig alfa is a recombinant Interleukin-18 Binding Protein (r-hIL-18BP) designed to target IL-18, a key inflammatory cytokine involved in various inflammatory diseases. In healthy individuals, naturally occurring IL-18BP maintains low levels of free IL-18, but in patients with certain inflammatory conditions, this balance is disrupted, leading to elevated free IL-18 levels and pathological inflammation. By administering AB2 Bio's exogenous recombinant human IL-18BP, the IL-18/IL-18BP balance is restored, effectively removing excess free IL-18 and reducing inflammation.

Drug Class Insights

The treatment of AOSD involves several classes of medications tailored to address varying degrees of inflammation and disease severity. Nonsteroidal anti-inflammatory drugs (NSAIDs) serve as first-line therapies to alleviate pain and reduce inflammation, though their efficacy may be limited in more severe cases. When NSAIDs prove inadequate, corticosteroids are commonly prescribed to provide rapid anti-inflammatory effects and immune suppression. For patients who do not respond to these conventional therapies, biologic disease-modifying antirheumatic drugs (bDMARDs) are increasingly utilized, targeting specific pathways in the inflammatory process to improve clinical outcomes. Additionally, antirheumatic agents may be employed to help modulate the immune response and control disease activity. The strategic use of these drug classes underscores the importance of individualized treatment plans that address the unique clinical presentation and progression of AOSD in each patient.

Market Outlook

AOSD is an autoinflammatory syndrome driven by the innate immune system, characterized by symptoms such as fever, rash, arthritis, pharyngitis, and hepatic and splenic involvement. Due to the rarity of AOSD and a lack of large-scale studies, diagnosis is often challenging, leading to an empirical treatment approach based on small retrospective case series.

The current treatment options include NSAIDs and corticosteroids, which serve as first-line therapies. However, many patients do not achieve remission with NSAIDs, prompting the use of DMARDs and biologics when corticosteroids fail. Biologic agents targeting the inflammatory pathways, such as interleukin (IL) receptor antagonists and TNF inhibitors, have emerged as promising treatments. Notably, KINERET (anakinra), an IL-1 receptor antagonist, is commonly prescribed for refractory AOSD patients, while ILARIS (canakinumab) has also been approved for treating AOSD, establishing a significant therapeutic advance. In Japan, ACTEMRA (tocilizumab), an anti-IL-6 receptor monoclonal antibody, has been approved for AOSD patients who do not respond sufficiently to existing therapies.

Emerging therapies include Tadekinig alfa, a recombinant IL-18 Binding Protein under development by AB2 Bio, which has shown promise in earlier Phase II trials. However, a lack of recent data regarding its efficacy in AOSD limits its inclusion in future forecasts. Overall, the treatment landscape for AOSD continues to evolve, with ongoing research aimed at enhancing therapeutic options for affected individuals.

AOSD Drugs Uptake

This section focuses on the uptake rate of potential drugs expected to be launched in the market during 2020-2034. For example, Tadekinig alfa is expected to enter the US market in 20XX and is projected to have a XX uptake during the forecast period.

AOSD Pipeline Development Activities

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

Pipeline development activities

The report covers information on collaborations, acquisitions and mergers, licensing, and patent details for emerging therapies for AOSD.

KOL Views

To keep up with current market trends, we take KOLs and SMEs' opinions working in the domain through primary research to fill the data gaps and validate our secondary research. Industry Experts contacted for insights on AOSD evolving treatment landscape, patient reliance on conventional therapies, patient therapy switching acceptability, and drug uptake, along with challenges related to accessibility, including Medical/scientific writers, Medical Professionals, Professors, Directors, and Others.

DelveInsight's analysts connected with 50+ KOLs to gather insights; however, interviews were conducted with 15+ KOLs in the 7MM. Centers like the Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy; San Joaquin General Hospital * French Camp, USA; Department of Rheumatology, The University of Chicago Medical Center, Chicago, IL, USA, among others, were contacted. Their opinion helps understand and validate current and emerging therapy treatment patterns or AOSD market trends. This will support the clients in potential upcoming novel treatments by identifying the overall scenario of the market and the unmet needs.

Physician's View

As per the KOLs from the US, There are often delays in diagnosis due to the infrequency of the condition and the nonspecific clinical presentation. A definitive diagnostic test has yet to be discovered. Measures of ferritin may only be useful when they are extremely elevated, and clinical suspicion is already high, or as a part of fulfilling diagnostic criteria.

Qualitative Analysis

We perform Qualitative and market Intelligence analysis using various approaches, such as SWOT and Conjoint Analysis. In the SWOT analysis, strengths, weaknesses, opportunities, and threats in terms of disease diagnosis, patient awareness, patient burden, competitive landscape, cost-effectiveness, and geographical accessibility of therapies are provided. These pointers are based on the Analyst's discretion and assessment of the patient burden, cost analysis, and existing and evolving treatment landscape.

Conjoint Analysis analyzes multiple emerging therapies based on relevant attributes such as safety, efficacy, frequency of administration, route of administration, and order of entry. Scoring is given based on these parameters to analyze the effectiveness of therapy.

To analyze the effectiveness of these therapies, have calculated their attributed analysis by giving them scores based on their ability to improve atrial and ventricular dimension/function and ability to regulate heart rate.

Further, the therapies' safety is evaluated wherein the adverse events are majorly observed, and it sets a clear understanding of the side effects posed by the drug in the trials, which directly affects the safety of the molecule in the upcoming trials. It sets a clear understanding of the side effects posed by the drug in the trials. In addition, the scoring is also based on the route of administration, order of entry and designation, probability of success, and the addressable patient pool for each therapy. According to these parameters, the final weightage score and the ranking of the emerging therapies are decided.

Market Access and Reimbursement

The high cost of therapies for the treatment is a major factor restraining the growth of the global drug market. Because of the high cost, the economic burden is increasing, leading the patient to escape from proper treatment.

The report provides detailed insights on the country-wise accessibility and reimbursement scenarios, cost-effectiveness scenarios, programs making accessibility easier and out-of-pocket costs more affordable, insights on patients insured under federal or state government prescription drug programs, etc.

Scope of the Report:

• The report covers a segment of key events, an executive summary, and a descriptive overview of AOSD explaining its causes, signs and symptoms, pathogenesis, and currently available therapies.
• Comprehensive insight into the epidemiology segments and forecasts, the future growth potential of diagnosis rate, disease progression, and treatment guidelines have been provided.
• Additionally, an all-inclusive account of the current and emerging therapies and the elaborative profiles of late-stage and prominent therapies will impact the current treatment landscape.
• A detailed review of the AOSD market, historical and forecasted market size, market share by therapies, detailed assumptions, and rationale behind our approach is included in the report, covering the 7MM drug outreach.
• The report provides an edge while developing business strategies by understanding trends through SWOT analysis and expert insights/KOL views, patient journey, and treatment preferences that help shape and drive the 7MM AOSD market.

AOSD report insights

• Patient Population
• Therapeutic Approaches
• AOSD Pipeline Analysis
• AOSD Market Size and Trends
• Existing and Future Market Opportunity

AOSD report key strengths

• 11 years Forecast
• The 7MM Coverage
• AOSD Epidemiology Segmentation
• Key Cross Competition
• Conjoint analysis
• Drugs Uptake and Key Market Forecast Assumptions

AOSD report assessment

• Current Treatment Practices
• Unmet Needs
• Pipeline Product Profiles
• Market Attractiveness
• Qualitative Analysis (SWOT and Conjoint Analysis)

Key Questions:

Market Insights

• What was the total market size of AOSD, the market size of AOSD by therapies, and market share (%) distribution in 2020, and what would it look like by 2034? What are the contributing factors for this growth?
• How will Tadekinig alfa affect the treatment paradigm of AOSD?
• How will KINERET compete with the upcoming therapies?
• Which drug is going to be the largest contributor by 2034?
• What are the pricing variations among different geographies for approved and marketed therapies?
• How would future opportunities affect the market dynamics and subsequent analysis of the associated trends?

Epidemiology Insights

• What are the disease risks, burdens, and unmet needs of AOSD? What will be the growth opportunities across the 7MM with respect to the patient population pertaining to AOSD?
• What is the historical and forecasted AOSD patient pool in the United States, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan?
• Out of the countries mentioned above, which country would have the highest diagnosed prevalent AOSD population during the forecast period (2024-2034)?
• What factors are contributing to the growth of AOSD cases?

Current Treatment Scenario, Marketed Drugs, and Emerging Therapies

• What are the current options for the treatment of AOSD? What are the current clinical and treatment guidelines for treating AOSD?
• How many companies are developing therapies for the treatment of AOSD?
• How many emerging therapies are in the mid-stage and late stage of development for treating AOSD?
• What are the recent novel therapies, targets, mechanisms of action, and technologies developed to overcome the limitations of existing therapies?
• What is the cost burden of current treatment on the patient?
• Patient acceptability in terms of preferred treatment options as per real-world scenarios?
• What are the accessibility issues of approved therapy in the US?
• What is the 7MM historical and forecasted market of AOSD?

Reasons to Buy:

• The report will help develop business strategies by understanding the latest trends and changing treatment dynamics driving the AOSD market.
• Insights on patient burden/disease prevalence, evolution in diagnosis, and factors contributing to the change in the epidemiology of the disease during the forecast years.
• Understand the existing market opportunities in varying geographies and the growth potential over the coming years.
• The distribution of historical and current patient share is based on real-world prescription data in the US, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan.
• Identifying upcoming solid players in the market will help devise strategies to help get ahead of competitors.
• Detailed analysis and ranking of class-wise potential current and emerging therapies under the conjoint analysis section to provide visibility around leading classes.
• Highlights of Access and Reimbursement policies for AOSD, barriers to accessibility of approved therapy, and patient assistance programs.
• To understand Key Opinion Leaders' perspectives around the accessibility, acceptability, and compliance-related challenges of existing treatment to overcome barriers in the future.
• Detailed insights on the unmet needs of the existing market so that the upcoming players can strengthen their development and launch strategy.