IgG4-Related Disease - Epidemiology Forecast - 2032

Key Highlights:

• IgG4-Related Disease is a systemic disease that causes fibrosis, tumor-like nodules, and lymphoid hyperplasia with infiltration of IgG4-positive plasma cells. It is noticeable in many organ systems.
• Many patients with IgG4-Related Disease may have no signs or symptoms for months or even years before the diagnosis, which can cause organ damage even while the patient is feeling well, long before they require medical attention.
• The most frequent localizations include the pancreas and salivary glands. Other common manifestations are tubulointerstitial nephritis, dacryoadenitis, and periaortitis.The IgG4-Related Disease may be diagnosed as an incidental finding when someone undergoes a routine radiological or surgical investigation. The condition often presents as swelling of or within an organ (such as skin, orbit, and lung), known as an inflammatory pseudo tumor.
• Diagnosing IgG4-Related Disease can be difficult, as multiple organs may be involved simultaneously. Diagnostic criteria have not been fully developed. The condition requires a combination of clinical, endoscopic, radiological, and serological tests looking for organ involvement and end-organ damage (e.g., hormonal abnormalities).
• In 2022, the total prevalent cases of IgG4-Related Disease were ~314,200 cases in the 7MM. These cases are expected to increase by 2032.
• The United States, in 2022, accounted for the highest number of prevalent cases of IgG4-Related Disease, which were ~123,300 cases. These cases are expected to increase by the year 2032.
• In 2022, Japan accounted for ~85,150 cases of total prevalent IgG4-Related Disease, approximately 27% of the total prevalent IgG4-Related Disease cases in the 7MM.
• Among the EU4 countries, the highest number of prevalent cases of IgG4-Related Disease were found in Germany, i.e., ~27,300 cases in the year 2022, which are estimated to increase by the year 2032.

DelveInsight's "IgG4-Related Disease - Epidemiology Forecast - 2032" report delivers an in-depth understanding of the IGG4, historical and forecasted epidemiology in the United States, EU4 (Germany, Spain, Italy, and France) and the United Kingdom, and Japan.

Geography Covered:

• The United States
• EU4 (Germany, France, Italy, and Spain) and the United Kingdom
• Japan

Study Period: 2019-2032.

IgG4-Related Disease Understanding and Diagnostic Algorithm:

IgG4-Related Disease Overview

IgG4-Related Disease (IgG4-RD) is a systemic fibroinflammatory disease characterized by dense infiltration of IgG4-positive plasma cells in the affected tissue(s) with or without elevated plasma levels of IgG4. It is a chronic-fibroinflammatory disorder affecting a wide range of organs. Elevation of serum IgG4 concentrations and abundant infiltration of IgG4-expressing plasma cells are key diagnostic features of this autoimmune Disease. Although common organ involvement of IgG4-Related Disease includes the salivary glands, pancreas, and bile duct, hepatic involvement is less well established. In all organs, corticosteroids and drugs that deplete B cells, such as rituximab, can reduce inflammation.

Patients with IgG4-Related Disease have relapses that respond to primary therapy. Intense fibrosis accompanies the inflammatory response, leading to permanent organ damage and insufficiency. Death from IgG4-Related Disease is rear. Typically, lymphoplasma cellular inflammation, storiform fibrosis, and obliterative phlebitis are found in IgG4-Related Disease biopsies, and the tissue invading plasma cells largely produce IgG4. IgG4-related Disease (IgG4-RD) is a fibro-inflammatory, immune-mediated, systemic disease first identified in patients with type 1 autoimmune pancreatitis (AIP1). Increased titers of autoantibodies against a spectrum of self-antigens and response to steroids have characterized it as an autoimmune disease.

IgG4-Related Disease Diagnosis

A definitive diagnosis of IgG4-Related Disease requires both histopathological confirmation and clinicopathological correlation. Serological and radiological features lack adequate sensitivity and specificity for diagnostic purposes. Histological examination is also mandatory to exclude neoplastic or inflammatory conditions that can mimic IgG4-Related Disease.

In the case of tumefactive lesions or infiltrating processes, it is self-evident that malignancy is usually the main differential diagnosis, especially for IgG4-related pancreatitis. Lymphoproliferative disorders, such as multicentric Castleman's Disease or antineutrophil cytoplasmic antibody-associated vasculitides, such as granulomatosis with polyangiitis, may mimic IgG4-Related Disease in certain cases and can also present with an increased number of IgG4+ plasma cells in lesions.

Other common differential diagnoses depend on the organ systems or regions where IgG4-Related Disease is suspected. Concerning liver lesions, primary sclerosing cholangitis has to be kept in mind, while Sjogren's syndrome or sialolithiasis has to be distinguished from IgG4-related sialadenitis and dacryoadenitis. Diagnosis of pulmonary manifestations of IgG4-Related Disease can be especially challenging as signs may be very unspecific and subtle. Besides malignancy, a broad spectrum of interstitial lung diseases may mimic pulmonary IgG4-Related Disease.

Further details related to diagnosis are provided in the report…

IgG4-Related Disease Epidemiology

The epidemiology forecast model of IgG4-Related Disease for the 7MM is based on the prevalence of IgG4 in the overall population to calculate the prevalent cases of IgG4. From this, the diagnosis rate was applied to calculate the diagnosed cases of IgG4.

As the market is derived using a patient-based model, the IgG4 epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented by total prevalent cases of IGG4, total diagnosed cases of IgG4, gender-specific cases of IgG4 and diagnosed cases of major organ involvement in IgG4-Related Disease in the 7MM covering the United States, EU4 countries (Germany, France, Italy, and Spain), United Kingdom, and Japan from 2019 to 2032. The total prevalent cases of IgG4 in the 7MM comprised approximately 12,082,000 cases in 2022, projected to increase during the forecasted period.

• In the UK, out of the total prevalent cases, ~21,700 cases and ~15,190 and ~6,180 cases accounted for IgG4-Related Disease in males and females, respectively, in 2022. These cases are expected to increase by 2032.
• The UK accounted for ~21,700 cases of IgG4-Related Disease in 2022, which formed approximately 7% of the total number of cases of IgG4-Related Disease in the 7MM.
• The EU4 and the UK accounted for ~91,600 cases of IgG4-Related Disease in 2022, which formed approximately 50% of the total number of IgG4-Related Disease cases in the 7MM.
• Among the EU4 countries, Spain had the least cases of IgG4-Related Disease, which were ~15,100 cases in 2022.
• In 2022, out of the total diagnosed cases, Germany accounted for ~13,700 cases of IgG4-Related Disease.
• Out of the total diagnosed cases, Japan accounted for ~42,600 cases of IgG4-Related Disease in adults in 2022.

Scope of the Report:

• The report covers a segment of key events, an executive summary, and a descriptive overview of IgG4-Related Disease, explaining its causes, signs and symptoms, pathogenesis, and diagnosis.
• Comprehensive insight into the country-wise epidemiology segments and forecasts, the future growth potential of diagnosis rate, and insights on disease progression have been provided.
• Patient stratification based on gender, age, and severity is an inclusion.
• A detailed review of current challenges in establishing the diagnosis.

IgG4-Related Disease Report Insights

• IgG4-Related Disease Patient Population
• Gender-wise, and major organ involvement in IgG4-Related Disease
• Country-wise Epidemiology Distribution

IgG4-Related Disease Report Key Strengths

• Ten years Forecast
• The 7MM Coverage
• IgG4-Related Disease Epidemiology Segmentation

IgG4-Related Disease Report Assessment

• Epidemiology Segmentation
• Current Diagnostic Practices

Key Questions:

Epidemiology Insights

• What are the disease risks, burdens, and unmet needs of IgG4-Related Disease? What will be the growth opportunities across the 7MM with respect to the patient population of IgG4-Related Disease?
• What is the historical and forecasted IgG4-Related Disease patient pool in the United States, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan?
• Which gender is the largest contributor to the IgG4-Related Disease patient pool?
• Which age group contributes more to IgG4-Related Disease in the 7MM?
• Which severity has the most number of cases of IgG4-Related Disease in the 7MM?
• What factors are affecting the increase in the diagnosis rate of IgG4-Related Disease?

Reasons to Buy:

• 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.
• To understand the change in IgG4-Related Disease diagnosed prevalent cases in varying geographies over the coming years.
• A detailed overview of age-specific, severity-specific, and gender-specific cases of IgG4-Related Disease is included.
• To understand the perspective of key opinion leaders around the current challenges with establishing the diagnosis and insights on the treatment-eligible patient pool.
• Detailed insights on various factors hampering disease diagnosis and other existing diagnostic challenges.