IgG4-Related Disease Market Synopsis

IgG4-Related Disease Market Size Was Valued at USD 3.65 Billion in 2023 and is Projected to Reach USD 5.30 Billion by 2032, Growing at a CAGR of 4.22% From 2024-2032.

Immunoglobulin G4-related disease (IgG4-RD) is a chronic immune-mediated disease that may present with tumefactive lesions, fibrosis, and a polyclonal IgG4-positive (IgG4) plasma cell-enriched infiltrate in nearly any anatomic site. IgG4-related disease (IgG4-RD) was described in Japan in the first years of the 21st century, characterized pathologically by the infiltration of IgG4-bearing plasma cells into involved organs.

• IgG4-RD presents with a wide range of symptoms, mimicking other conditions like cancers, infections, and autoimmune diseases. Therefore, accurate diagnosis often requires a combination of clinical evaluation, imaging, histopathological examination, and serological tests. The identification of elevated serum IgG4 levels and specific histopathological features (such as dense lymphoplasmacytic infiltrate and storiform fibrosis) are crucial for confirming the diagnosis​.
• Advances in identifying biomarkers for IgG4-RD have the potential to revolutionize its diagnosis and monitoring. Reliable biomarkers could enable early detection and assess disease activity and response to therapy, facilitating more personalized treatment plans. Ongoing research is focused on discovering and validating these biomarkers.
• Traditional treatments for IgG4-RD, such as corticosteroids and immunosuppressants, often lead to significant side effects and require long-term management. The development of targeted biological therapies, such as monoclonal antibodies (e.g., rituximab and obexelimab), represents a major advancement. These therapies specifically target components of the immune system involved in IgG4-RD, potentially offering more effective and safer treatment options​.

IgG4-Related Disease Market Trend Analysis

Rising prevalence of IgG4-related disease

• IgG4-RD, first recognized as a distinct clinical entity in the early 2000s, has seen a steady increase in reported cases. This condition is now understood to affect multiple organs, making it a key area of focus in autoimmune and inflammatory disease research.
• IgG4-RD represents a very young entity of disease, having emerged within only 1 decade. Although it is generally thought to be a rare condition, it is likely that with growing awareness and recent advances in research, the number of patients diagnosed with IgG4-RD will increase in the future.
• By analysing identified IgG4-RD cases. The mean age was 56.5 years with 57.6% female and 66% White. The incidence of IgG4-RD increased during the study period from 0.78 to 1.39 per 100,000 person-years in 2015 and 2019, respectively. The point prevalence in recent years was 5.3/100 000 persons. During follow-up, there were 39 and 164 deaths among 515 IgG4-RD cases and 5160 comparators, resulting in a mortality rate of 3.42 and 1.46/100 person-years, respectively, and adjusted HR of 2.51 (95% CI 1.76 to 3.56). Thus, raising the disease simultaneously prevalence of IgG4-related disease is increasing. 

Advanced Diagnostic Tools

• IgG4-Related Disease (IgG4-RD) is a complex and rare autoimmune condition that often mimics other diseases, making accurate and timely diagnosis challenging. The development of advanced diagnostic tools presents a significant market opportunity.
• IgG4-RD can affect multiple organs, including the pancreas, bile ducts, salivary glands, and kidneys. Symptoms often overlap with other conditions, leading to misdiagnosis or delayed diagnosis. Current diagnostic methods rely on a combination of clinical observation, imaging, serology, and histopathology, often requiring invasive biopsies. IgG4 serum levels, although commonly measured, are not specific and can be elevated in other conditions, leading to potential false positives.
• The development of non-invasive diagnostic tools, such as advanced imaging techniques and molecular assays, could revolutionize the management of IgG4-RD. Innovations in imaging, like positron emission tomography (PET) scans using novel radiotracers, offer the potential for early and accurate detection of organ involvement in IgG4-RD. Additionally, molecular diagnostics, including next-generation sequencing (NGS) and gene expression profiling, could identify specific biomarkers associated with IgG4-RD. These tools can enhance the accuracy of diagnosis and facilitate differentiation from other similar conditions.

IgG4-Related Disease Market Segment Analysis:

IgG4-Related Disease Market Segmented based on Disease Type, Type, Drug Type, Route of Administration, and end-users.

By Disease Type, Type 1 (IgG4-Related) Autoimmune Pancreatitis (AIP) segment is expected to dominate the market during the forecast period

• Type 1 AIP is the most common subtype of autoimmune pancreatitis. Its prevalence is higher compared to other types, contributing to a larger patient pool and market demand. Improvements in diagnostic techniques have led to better identification and understanding of Type 1 AIP. This increased detection contributes to its dominant position in the market. Therapeutic developments tailored specifically for Type 1 AIP have enhanced patient outcomes. As treatment options become more effective and tailored to this subtype, it further solidifies its dominance in the market.
• Although commonly observed, approximately 32% (7–40%) of the patients with type 1 AIP may develop pancreatic calcification or chronic pancreatitis. The association of malignant tumors with type 1 AIP or IgG4-RD is controversial, several cases of pancreatic cancer or other malignancies have been described in patients with AIP or IgG4-RD.22,40–44) In recent reports,22,40–44) in about 1,000 patients, 8.5% (10.1–13.9%) of IgG4-RD cases and 1% of AIP cases (2.1–13.9%) exhibited malignant tumors. Therefore, it is noted that pancreatic cancer or other malignant tumors may be complicated with AIP or IgG4-RD.
• A Japanese multicenter study of 108 cases of AIP reported that the highest risk for cancer is in the first year after AIP diagnosis. The absence of a relapse after successful treatment of the coexisting cancers suggests that AIP may develop as a paraneoplastic syndrome in some patients.

By Drug Type, the Prednisone segment held the largest share in 2023

• Treatment for IgG4-RSD traditionally has involved prednisone, a steroid medication. Prednisone has proven very effective in treating this condition.  The initial recommended dose of oral prednisolone for induction of remission is 0.6 mg/kg/day, administered for 2-4 weeks. This dose is gradually tapered to a maintenance dose of 2.5-5 mg/day over a period of 2-3 months.
• Prednisone is often the first-line treatment for IgG4-related disease (IgG4-RD) due to its potent anti-inflammatory properties. IgG4-RD is characterized by tissue inflammation and fibrosis, and prednisone helps reduce inflammation and suppress the immune response, which can alleviate symptoms and prevent disease progression.
• Other drugs used to treat IgG4-RD include immunosuppressants like azathioprine and methotrexate, as well as rituximab, which targets B-cells involved in the immune response. However, prednisone is frequently chosen as the initial therapy because of its effectiveness and relatively low cost compared to some of these alternatives.
• Additionally, prednisone is widely available and has a long history of use in treating various inflammatory conditions, making it a familiar option for many healthcare providers. Its dominance in the IgG4-RD market is thus a combination of its efficacy, accessibility, and familiarity among clinicians.

IgG4-Related Disease Market Regional Insights:

Asia Pacific is Expected to Dominate the Market Over the Forecast Period

• Igg4-related disease is more prevalent in certain Asian populations compared to others. Japan, for example, has a relatively high prevalence of the disease. There may be higher awareness and better diagnostic capabilities for IgG4-Related Diseases in the Asia Pacific region, leading to more accurate diagnoses and higher reported cases. The Asia Pacific region is home to many pharmaceutical and biotech companies conducting research and development in the field of rare diseases, including IGg4-related diseases. These companies may be more focused on addressing the needs of their local populations, driving innovation and investment in this area.
• For Instance, In a Japanese cohort, reportedly 70% of patients later diagnosed with IgG4-RD presented with symptoms, while 30% were found incidentally. Most frequently, symptoms were related to tumefaction, such as obstructive jaundice and exophthalmos (41%), followed by general abdominal symptoms (18%) and malaise (4%). Renal (4%) and pulmonary symptoms (3%) were only present in a minority of patients. Another frequently reported symptom preceding the diagnosis of IgG4-RD is weight loss.
• With a large and diverse population, there may be a significant number of patients with IGg4-related disease in the Asia Pacific region, creating a sizable market for related treatments and therapies. Improvements in healthcare infrastructure and access to medical care in countries across the Asia Pacific region may also contribute to better detection, diagnosis, and management of IgG4-Related Disease, further driving market growth.

Clinical characteristics of patients from two large published cohorts

IgG4-Related Disease Market Top Active Key Players:

• Zydus Group (India)
• Hikma Pharmaceuticals PLC (U.K.)
• Lannett (U.S.)
• Sun Pharmaceutical Industries Ltd. (India)
• Baxter (U.S.)
• Viatris Inc. (U.S.)
• Amgen Inc. (U.S.)
• Sandoz Group AG (Switzerland)
• Fresenius Kabi AG (Germany)
• Pfizer Inc. (U.S.)
• Cipla Inc. (India)
• Celltrion Healthcare Co., Ltd. (South Korea)
• Antares Pharma (U.S.)
• Amneal Pharmaceuticals LLC (U.S.)
• Accord-UK Ltd. (U.K.)
• Genentech, Inc. (U.S.)
• Ingenus Pharmaceuticals (U.S.)
• STI PHARMA (Canada)
• Sebela Pharmaceuticals (France)
• ANI Pharmaceuticals, Inc. (U.S.), and Other Active Player.  

Key Industry Developments in the IgG4-Related Disease Market:

• In June 2024, Amgen announced positive topline results from its randomized, double-blind, multicenter, placebo-controlled Phase 3 clinical trial (NCT04540497) evaluating the efficacy and safety of UPLIZNA® (ibalizumab-con) for the treatment of Immunoglobulin G4-related disease (IgG4-RD). The trial met its primary endpoint, showing a statistically significant 87% reduction in the risk of IgG4-RD flare compared to placebo (Hazard Ratio 0.13, p<0.0001) during the 52-week placebo-controlled period.
• In August 2023, Zenas BioPharma released Phase 2 trial results evaluating obexelimab for treating IgG4-related disease (IgG4-RD). Based on positive outcomes, a Phase 3 trial is underway, aiming to further assess the efficacy and safety of obexelimab via subcutaneous injection, showcasing promising advancements in treating IgG4-related disease within the market.

1. INTRODUCTION
   1. RESEARCH OBJECTIVES
   2. RESEARCH METHODOLOGY
   3. RESEARCH PROCESS
   4. SCOPE AND COVERAGE
      1. Market Definition
      2. Key Questions Answered
   5. MARKET SEGMENTATION
2. EXECUTIVE SUMMARY
3. MARKET OVERVIEW
4. GROWTH OPPORTUNITIES BY SEGMENT
5. MARKET LANDSCAPE
   1. PORTER’S FIVE FORCES ANALYSIS
      1. Bargaining Power Of Supplier
      2. Threat Of New Entrants
      3. Threat Of Substitutes
      4. Competitive Rivalry
      5. Bargaining Power Among Buyers
   2. INDUSTRY VALUE CHAIN ANALYSIS
   3. MARKET DYNAMICS
      1. Drivers
      2. Restraints
      3. Opportunities
      4. Challenges
   4. MARKET TREND ANALYSIS
   5. REGULATORY LANDSCAPE
   6. PESTLE ANALYSIS
   7. PRICE TREND ANALYSIS
   8. PATENT ANALYSIS
   9. TECHNOLOGY EVALUATION
   10. MARKET IMPACT OF THE RUSSIA-UKRAINE WAR
       1. Geopolitical Market Disruptions
       2. Supply Chain Disruptions
       3. Instability in Emerging Markets
   11. ECOSYSTEM
6. IGG4-RELATED DISEASE MARKET BY LUXURY GOODS (2017-2032)
   1. IGG4-RELATED DISEASE MARKET SNAPSHOT AND GROWTH ENGINE
   2. MARKET OVERVIEW
   3. TYPE 1 (IGG4-RELATED) AUTOIMMUNE PANCREATITIS (AIP)
      1. Introduction And Market Overview
      2. Historic And Forecasted Market Size in Value (2017 – 2032F)
      3. Historic And Forecasted Market Size in Volume (2017 – 2032F)
      4. Key Market Trends, Growth Factors And Opportunities
      5. Geographic Segmentation Analysis
   4. RETROPERITONEAL FIBROSIS
   5. IGG4-RELATED TUBULOINTERSTITIAL NEPHRITIS (TIN)
   6.  SERUM IGG4 CONCENTRATION
   7. IGG4-RELATED THYROID DISEASE
   8. OTHERS
7. IGG4-RELATED DISEASE MARKET BY TYPE (2017-2032)
   1. IGG4-RELATED DISEASE MARKET SNAPSHOT AND GROWTH ENGINE
   2. MARKET OVERVIEW
   3. DIAGNOSTIC
      1. Introduction And Market Overview
      2. Historic And Forecasted Market Size in Value (2017 – 2032F)
      3. Historic And Forecasted Market Size in Volume (2017 – 2032F)
      4. Key Market Trends, Growth Factors And Opportunities
      5. Geographic Segmentation Analysis
   4. TREATMENT
8. IGG4-RELATED DISEASE MARKET BY DRUG TYPE (2017-2032)
   1. IGG4-RELATED DISEASE MARKET SNAPSHOT AND GROWTH ENGINE
   2. MARKET OVERVIEW
   3. CORTICOSTEROIDS
      1. Introduction And Market Overview
      2. Historic And Forecasted Market Size in Value (2017 – 2032F)
      3. Historic And Forecasted Market Size in Volume (2017 – 2032F)
      4. Key Market Trends, Growth Factors And Opportunities
      5. Geographic Segmentation Analysis
   4. BIOLOGICS
   5. IMMUNOSUPPRESSANTS
   6. ANTIMETABOLITES
   7. PREDNISONE
9. IGG4-RELATED DISEASE MARKET BY ROUTE OF ADMINISTRATION (2017-2032)
   1. IGG4-RELATED DISEASE MARKET SNAPSHOT AND GROWTH ENGINE
   2. MARKET OVERVIEW
   3. PARENTERAL
      1. Introduction And Market Overview
      2. Historic And Forecasted Market Size in Value (2017 – 2032F)
      3. Historic And Forecasted Market Size in Volume (2017 – 2032F)
      4. Key Market Trends, Growth Factors And Opportunities
      5. Geographic Segmentation Analysis
   4. ORAL
   5. OTHERS
10. IGG4-RELATED DISEASE MARKET BY END-USER (2017-2032)
    1. IGG4-RELATED DISEASE MARKET SNAPSHOT AND GROWTH ENGINE
    2. MARKET OVERVIEW
    3. HOSPITALS
       1. Introduction And Market Overview
       2. Historic And Forecasted Market Size in Value (2017 – 2032F)
       3. Historic And Forecasted Market Size in Volume (2017 – 2032F)
       4. Key Market Trends, Growth Factors And Opportunities
       5. Geographic Segmentation Analysis
    4. SPECIALTY CLINICS
    5. OTHERS
11. IGG4-RELATED DISEASE MARKET BY DISTRIBUTION CHANNEL (2017-2032)
    1. IGG4-RELATED DISEASE MARKET SNAPSHOT AND GROWTH ENGINE
    2. MARKET OVERVIEW
    3. HOSPITAL PHARMACY
       1. Introduction And Market Overview
       2. Historic And Forecasted Market Size in Value (2017 – 2032F)
       3. Historic And Forecasted Market Size in Volume (2017 – 2032F)
       4. Key Market Trends, Growth Factors And Opportunities
       5. Geographic Segmentation Analysis
    4. RETAIL PHARMACY
    5. ONLINE PHARMACY
    6. OTHERS
12. COMPANY PROFILES AND COMPETITIVE ANALYSIS
    1. COMPETITIVE LANDSCAPE
       1. Competitive Positioning
       2. IGG4-RELATED DISEASE Market Share By Manufacturer (2023)
       3. Industry BCG Matrix
       4. Heat Map Analysis
       5. Mergers & Acquisitions
    2. ZYDUS GROUP (INDIA)
       1. Company Overview
       2. Key Executives
       3. Company Snapshot
       4. Role of the Company in the Market
       5. Sustainability and Social Responsibility
       6. Operating Business Segments
       7. Product Portfolio
       8. Business Performance (Production Volume, Sales Volume, Sales Margin, Production Capacity, Capacity Utilization Rate)
       9. Key Strategic Moves And Recent Developments
       10. SWOT Analysis
    3. HIKMA PHARMACEUTICALS PLC (U.K.)
    4. LANNETT (U.S.)
    5. SUN PHARMACEUTICAL INDUSTRIES LTD. (INDIA)
    6. BAXTER (U.S.)
    7. VIATRIS INC. (U.S.)
    8. AMGEN INC. (U.S.)
    9. SANDOZ GROUP AG (SWITZERLAND)
    10. FRESENIUS KABI AG (GERMANY)
    11. PFIZER INC. (U.S.)
    12. CIPLA INC. (INDIA)
    13. CELLTRION HEALTHCARE CO., LTD. (SOUTH KOREA)
    14. ANTARES PHARMA (U.S.)
    15. AMNEAL PHARMACEUTICALS LLC (U.S.)
    16. ACCORD-UK LTD. (U.K.)
    17. GENENTECH, INC. (U.S.)
    18. INGENUS PHARMACEUTICALS (U.S.)
    19. STI PHARMA (CANADA)
    20. SEBELA PHARMACEUTICALS (FRANCE)
    21. ANI PHARMACEUTICALS, INC. (U.S.)
13. GLOBAL IGG4-RELATED DISEASE MARKET BY REGION
    1. OVERVIEW
    2. NORTH AMERICA
       1. Key Market Trends, Growth Factors And Opportunities
       2. Key Manufacturers
       3. Historic And Forecasted Market Size By Disease Type
       4. Historic And Forecasted Market Size By Type
       5. Historic And Forecasted Market Size By Drug Type
       6. Historic And Forecasted Market Size By Route Of Administration
       7. Historic And Forecasted Market Size By End-User
       8. Historic And Forecasted Market Size By Distribution Channel
       9. Historic And Forecasted Market Size By Country
          1. USA
          2. Canada
          3. Mexico
    3. EASTERN EUROPE
       1. Key Market Trends, Growth Factors And Opportunities
       2. Key Manufacturers
       3. Historic And Forecasted Market Size By Segments
       4. Historic And Forecasted Market Size By Country
          1. Russia
          2. Bulgaria
          3. The Czech Republic
          4. Hungary
          5. Poland
          6. Romania
          7. Rest Of Eastern Europe
    4. WESTERN EUROPE
       1. Key Market Trends, Growth Factors And Opportunities
       2. Key Manufacturers
       3. Historic And Forecasted Market Size By Segments
       4. Historic And Forecasted Market Size By Country
          1. Germany
          2. United Kingdom
          3. France
          4. The Netherlands
          5. Italy
          6. Spain
          7. Rest Of Western Europe
    5. ASIA PACIFIC
       1. Key Market Trends, Growth Factors And Opportunities
       2. Key Manufacturers
       3. Historic And Forecasted Market Size By Segments
       4. Historic And Forecasted Market Size By Country
          1. China
          2. India
          3. Japan
          4. South Korea
          5. Malaysia
          6. Thailand
          7. Vietnam
          8. The Philippines
          9. Australia
          10. New-Zealand
          11. Rest Of APAC
    6. MIDDLE EAST & AFRICA
       1. Key Market Trends, Growth Factors And Opportunities
       2. Key Manufacturers
       3. Historic And Forecasted Market Size By Segments
       4. Historic And Forecasted Market Size By Country
          1. Turkey
          2. Bahrain
          3. Kuwait
          4. Saudi Arabia
          5. Qatar
          6. UAE
          7. Israel
          8. South Africa
    7. SOUTH AMERICA
       1. Key Market Trends, Growth Factors And Opportunities
       2. Key Manufacturers
       3. Historic And Forecasted Market Size By Segments
       4. Historic And Forecasted Market Size By Country
          1. Brazil
          2. Argentina
          3. Rest of South America
14. INVESTMENT ANALYSIS
15. ANALYST VIEWPOINT AND CONCLUSION
    1. Recommendations and Concluding Analysis
    2. Potential Market Strategies