The IgG4-Related Disease (IgG4-RD) market, valued at around USD 170 million in 2024, is anticipated to expand substantially by 2034. IgG4-RD is a rare, systemic fibroinflammatory disorder that can impact various organs, such as the pancreas, kidneys, lungs, and salivary glands. Across the 7MM, there were approximately 148,970 diagnosed prevalent cases in 2024, with the United States accounting for the largest share and Japan reporting nearly 38,690 cases. Diagnosis is often delayed or missed due to the condition’s recent recognition, its diverse clinical manifestations, and its higher occurrence in males.
Current management primarily revolves around corticosteroids – especially prednisone – with immunosuppressive therapies and Rituximab reserved for refractory or relapsing patients. Despite these options, no curative treatment is currently available. A pivotal advancement came in April 2025, when the FDA approved Amgen’s UPLIZNA as the first approved therapy for IgG4-RD, following its Breakthrough Therapy Designation. Other promising candidates in development include obexelimab (Zenas BioPharma/Bristol Myers Squibb) and rilzabrutinib (Sanofi). UPLIZNA and obexelimab are expected to be strong competitors, while rilzabrutinib – an oral therapy – could shift treatment preferences by offering an alternative to injectable options. Major players driving innovation include Zenas BioPharma, Bristol Myers Squibb, Amgen, Sanofi, and others, setting the stage for significant evolution within this underserved therapeutic area.
DelveInsight’s report, “IgG4-Related Disease Market Insights, Epidemiology, and Market Forecast – 2034,” provides a comprehensive overview of IgG4-RD, including historical and projected epidemiology, as well as detailed market dynamics across the United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan. The analysis covers current treatment approaches, emerging therapies, individual drug market shares, and market size projections from 2020 to 2034 across the seven major markets. In addition, the report reviews treatment algorithms, key market drivers and challenges, unmet needs, and strategic opportunities – offering a complete assessment of the growth potential within the IgG4-RD landscape.
The IgG4-Related Disease market is set for major growth with the approval of UPLIZNA and a robust pipeline including obexelimab and rilzabrutinib @ IgG4-related disease Market Forecast
Some facts of the IgG4-related disease Market Report are:
- In April 2025, Amgen announced that the FDA had approved UPLIZNA as the first and only treatment for adults living with IgG4-RD.
- In March 2025, Zenas BioPharma announced that the company anticipates reporting topline results for the INDIGO trial by the end of 2025.
- In November 2024, Amgen presented new data at the annual American College of Rheumatology (ACR) Convergence 2024, demonstrating a significant reduction in disease activity in patients with IgG4-RD following treatment with UPLIZNA.
- In August 2024, Amgen announced that the US FDA had granted Breakthrough Therapy Designation (BTD) to UPLIZNA for the treatment of IgG4-RD based on data from the MITIGATE study.
- The IgG4-Related Disease market size is anticipated to grow with a significant CAGR during the study period (2020-2034).
- DelveInsight estimates that the market size for IgG4-RD is expected to grow from USD 170 million in 2024, with a significant CAGR by 2034.
- IgG4 related disease is a systemic disease that causes fibrosis, tumor-like nodules, and lymphoid hyperplasia with infiltration of IgG4-positive plasma cells. It can manifest in many organ systems.
- There are no approved therapies for IgG4-related disease (IgG4-RD) specifically and hence, the current treatment landscape is driven by off-label therapies such as glucocorticoids, immunomodulators, and rituximab.
- Glucocorticoids (GCs) have been considered the first-line therapy in IgG4-related disease (IgG4-RD), whereas rituximab accounted for the majority of the market share as annual treatment cost is significantly higher for patients taking rituximab.
- The United States accounted for the largest market size (around USD 115 million) of IgG4-related disease treatment market, in comparison to EU4 (Germany, Spain, Italy, France), the United Kingdom, and Japan.
- Amgen recorded USD 379 million in UPLIZNA sales in 2024 from its original indication alone. However, the recent approval for IgG4-related disease presents a significant growth opportunity, as this new market includes a treatable patient population that is roughly twice as large as that for NMOSD.
- Limited number of therapies are being investigated for the treatment of IgG4 related disease. Some of the key players involved in the development are Zenas Biopharma (obexelimab), Amgen/Horizon Therapeutics (inebilizumab), and Sanofi (rilzabrutinib).
- Approval of ZB012 (obexelimab) and UPLIZNA (inebilizumab) will change the treatment pattern drastically over the forecast period as these will be the first few drugs to be launched for the treatment of IgG4-related disease.
- IgG4-related disease is a rare disease, with an estimated prevalence of 6 cases per 100,000 individuals. This condition primarily affects middle-aged to elderly men.
- The 7MM accounted for approximately 300,000 prevalent cases of IgG4-related disease in 2023. These cases are expected to increase due to increased awareness and improved diagnostic methods that contribute to the rise in identified cases of IgG4-related disease, especially among aging populations with higher incidences of autoimmune disorders.
- Key IgG4-Related Disease Companies: Zenas BioPharma, Horizon Therapeutics, Sanofi, Roche, AbbVie, Gilead Sciences, Takeda Pharmaceutical Company, and others
- Key IgG4-Related Disease Therapies: ZB012 (obexelimab), UPLIZNA (inebilizumab), Rilzabrutinib, and others
- The IgG4-Related Disease market is expected to surge due to the disease’s increasing prevalence and awareness during the forecast period. Furthermore, launching various multiple-stage IgG4-Related Disease pipeline products will significantly revolutionize the IgG4-Related Disease market dynamics.
IgG4-related disease Overview
IgG4-related disease (IgG4-RD) is a rare, chronic fibroinflammatory condition characterized by elevated IgG4-positive plasma cells and tissue fibrosis. It can affect multiple organs, including the pancreas, salivary glands, kidneys, lungs, and lymph nodes, often presenting with organ enlargement or mass-like lesions. The disease is frequently underdiagnosed due to its diverse symptoms and relatively recent recognition. Although corticosteroids remain the standard first-line therapy, many patients experience relapses, requiring immunosuppressants or biologics like Rituximab. With no curative treatment currently available, ongoing research and emerging therapies aim to improve long-term disease control and address significant unmet medical needs.
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IgG4-Related Disease (IgG4-RD) Market Outlook
In 2024, the United States dominated the IgG4-RD market with revenues close to USD 90 million, followed by the EU4 and the UK, which collectively generated around USD 80 million, led primarily by Germany. Japan accounted for approximately USD 6 million, though its market is expected to expand swiftly through 2034.
Key innovators – including Zenas BioPharma, Amgen, and Sanofi – are advancing late-stage therapies aimed at addressing critical unmet needs. Successful approvals could significantly reshape the treatment landscape, enhance patient outcomes, and unlock substantial growth across the 7MM.
Glucocorticoids remain the standard first-line treatment, with prednisone (30–40 mg/day or 0.6 mg/kg/day) widely used to induce remission. In select cases, DMARDs are added, while plasmablast levels help guide B-cell–targeted therapeutic decisions. Although several promising pipeline therapies are in development, pricing pressures, reimbursement hurdles, limited regulatory precedent, and the need for consistent clinical endpoints may influence uptake. Rituximab – effective and relatively affordable – continues to face insurance-related restrictions in Japan, further shaping treatment dynamics.
IgG4-related disease Epidemiology
In 2024, the United States recorded an estimated 56,800 diagnosed cases of IgG4-related disease (IgG4-RD), with a clear male predominance – about 39,100 males compared to 17,700 females. In the EU4 and the UK, the diagnosed prevalence reached approximately 53,550 cases, including 38,850 males and 14,750 females. Cases were distributed across six age groups: 0–17, 18–29, 30–44, 45–64, 65–74, and ≥75 years. In Japan, individuals aged 75 years and older represented the highest burden, totaling around 12,800 cases in 2024. Overall, the diagnosed prevalence of IgG4-RD is expected to rise consistently from 2025 to 2034, supported by growing clinical awareness, enhanced diagnostic capabilities, and improved disease reporting.
IgG4-related disease Epidemiology Segmented by:
- Total diagnosed prevalent cases of IgG4-RD
- Gender-specific diagnosed prevalent cases of IgG4-RD
- Age-specific diagnosed prevalent cases of IgG4-RD
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IgG4-related disease Drugs Uptake
Obexelimab (XmAb5871) – Developed by Zenas BioPharma and Bristol Myers Squibb, this bifunctional monoclonal antibody targets CD19 and FcγRIIb to inhibit B-cell activity. It is administered subcutaneously (SC) and is currently in Phase III development.
Rilzabrutinib (PRN1008/SAR444671) – A small molecule oral Bruton’s Tyrosine Kinase (BTK) inhibitor from Sanofi, positioned in Phase II clinical development for IgG4-RD.
IgG4-related disease Market Drivers and Barriers
IgG4-related disease Market Drivers
- Rising Diagnosis and Awareness – Improved recognition of IgG4-RD by clinicians, supported by growing research and clinical guidelines.
- Unmet Medical Need – Lack of approved therapies until recently creates strong demand for effective, targeted treatments.
- Novel Therapeutics Pipeline – Approval of UPLIZNA (Amgen, 2025) and advancement of obexelimab (Zenas/BMS) and rilzabrutinib (Sanofi) drive innovation.
- Increasing Prevalence – Rising diagnosed patient pool in the US, EU, and Japan supports market expansion.
- Supportive Regulatory Designations – Breakthrough and orphan drug designations accelerate drug development.
- Shift Toward Targeted Therapies – Growing interest in B-cell modulation and BTK inhibitors to replace long-term steroid dependence.
IgG4-related disease Market Barriers
- Underdiagnosis & Misdiagnosis – Due to nonspecific symptoms and overlap with malignancies/autoimmune diseases.
- Limited Epidemiological Data – True prevalence remains unclear, impacting investment and healthcare planning.
- Pricing & Reimbursement Challenges – High-cost biologics face payer resistance, especially where off-label rituximab is used as a cheaper alternative.
- Clinical Development Risks – Lack of established regulatory precedent and standardized endpoints for IgG4-RD trials.
- Steroid Dependence – Corticosteroids remain the standard first-line therapy, delaying adoption of novel agents.
- Regional Variations – Limited drug access in markets like Japan, where rituximab use is restricted.
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IgG4-related disease Therapeutics Assessment
Major key companies are working proactively in the IgG4-related disease Therapeutics market to develop novel therapies which will drive the IgG4-related disease treatment markets in the upcoming years are Zenas BioPharma, Amgen Inc (NASDAQ: AMGN), Horizon Therapeutics (NASDAQ: HZNP), Sanofi (EPA: SAN), Roche (SWX: ROG), AbbVie (NYSE: ABBV), Gilead Sciences (NASDAQ: GILD), Takeda Pharmaceutical Company (TYO: 4502), and others.
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IgG4-related disease Report Key Insights
1. IgG4-related disease Patient Population
2. IgG4-related disease Market Size and Trends
3. Key Cross Competition in the IgG4-related disease Market
4. IgG4-related disease Market Dynamics (Key Drivers and Barriers)
5. IgG4-related disease Market Opportunities
6. IgG4-related disease Therapeutic Approaches
7. IgG4-related disease Pipeline Analysis
8. IgG4-related disease Current Treatment Practices/Algorithm
9. Impact of Emerging Therapies on the IgG4-related disease Market
Table of Contents
1. Key Insights
2. Executive Summary
3. IgG4-related disease Competitive Intelligence Analysis
4. IgG4-related disease Market Overview at a Glance
5. IgG4-related disease Disease Background and Overview
6. IgG4-related disease Patient Journey
7. IgG4-related disease Epidemiology and Patient Population
8. IgG4-related disease Treatment Algorithm, Current Treatment, and Medical Practices
9. IgG4-related disease Unmet Needs
10. Key Endpoints of IgG4-related disease Treatment
11. IgG4-related disease Marketed Products
12. IgG4-related disease Emerging Therapies
13. IgG4-related disease Seven Major Market Analysis
14. Attribute Analysis
15. IgG4-related disease Market Outlook (7 major markets)
16. IgG4-related disease Access and Reimbursement Overview
17. KOL Views on the IgG4-related disease Market
18. IgG4-related disease Market Drivers
19. IgG4-related disease Market Barriers
20. Appendix
21. DelveInsight Capabilities
22. Disclaimer
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