ANCA Associated Vasculitis Epidemiology

Key Highlights

• ANCA Associated Vasculitis is a collection of relatively rare autoimmune diseases of unknown cause, characterized by inflammatory cell infiltration causing necrosis of blood vessels. AAVs include three different conditions - MPA, GPA, and EGPA.
• Because of the rarity of these conditions, very few studies have been done to determine their prevalence to date. Additionally, the prevalence of these conditions is geographically heterogeneous, such as GPA being more prevalent in European countries while in Asian countries (especially Japan), MPA is more prevalent.
• In 2022, the total diagnosed prevalent cases of AAV were ~547,150 in the 7MM and China, which might reach ~719,020 cases by 2034 at a CAGR of 2.8%. Among these, approximately 44% of cases are of GPA, 36% are of MPA, and 20% are of EGPA.
• Although the incidence of MPA is higher compared to GPA, the prevalence is much lower than GPA as patients of MPA present severe manifestations at the time of diagnosis leading to a high mortality rate and lower recorded prevalence.
• AAVs affect multiple organs and are associated with a high disease burden. If left untreated, patients develop multiple organ failures, such as ESRD in the case of MPA and GPA and lung failure in the case of EGPA.
• According to published literature and primary market research findings, the presence of PR3-ANCAs has been reported as approximately 73% in GPA, 17% in EGPA, and 4% in MPA in the 7MM, while in China, it is around 38% in GPA, 1% in EGPA, and 2% in MPA.

DelveInsight’s “ANCA Associated Vasculitis – Epidemiology Forecast – 2034” report delivers an in-depth understanding of the ANCA Associated Vasculitis, historical and forecasted epidemiology in the United States, EU4 (Germany, Spain, Italy, and France) and the United Kingdom, Japan, and China.

Geography Covered

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

Study Period: 2021-2034

ANCA Associated Vasculitis Understanding and Diagnostic Algorithm

ANCA Associated Vasculitis Overview

ANCA Associated Vasculitis is a rare, potentially life-threatening, and heterogeneous group of rare autoimmune conditions that causes an inflammation of blood vessels with various manifestations, which attacks and injures the kidney, lungs, etc. It includes three main diseases, which are granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). ANCA Associated Vasculitis may present with constitutional symptoms and symptoms associated with specific organ involvement. The clinical spectrum of ANCA Associated Vasculitis is broad, and hence the presentation can be quite varied, ranging from a skin rash to fulminant multisystem disease.

The exact cause of ANCA Associated Vasculitis is unknown but is probably multifactorial. Several genetic and environmental risk factors have been identified. Triggers for developing ANCA Associated Vasculitis include microbial infections, reactions to certain medications, genetic variations, or exposure to toxins. ANCA Associated Vasculitis often has the presence of circulating autoantibodies (ANCA) that are usually directed against myeloperoxidase (MPO) or proteinase 3 (PR3) antigens.

ANCA Associated Vasculitis Diagnosis

The diagnosis of ANCA Associated Vasculitis is clinical and supported by serological and histological data. The key to diagnosis is prompt recognition of an inflammatory disease pattern when multiple symptoms emerge, especially if more than one organ system is implicated or combined with chronic systemic symptoms. The diagnosis of ANCA Associated Vasculitis is based on ANCA antibody testing detects and measures the amount of these autoantibodies in the blood. Two of the most common ANCAs are the autoantibodies that target the proteins MPO and PR3, and these are called pANCAs and cANCAs, respectively. A positive C-ANCA immunofluorescence test or a strongly positive PR3-ANCA or MPO-ANCA ELISA result is highly suspicious for diagnosing ANCA-associated vasculitis.

Blood tests are done to look for abnormal blood counts and an increase in eosinophils, and special antibody testing is called ANCA. Blood tests that detect inflammation include the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) tests. In patients with EGPA, the complete blood cell count (CBC) with differential typically demonstrates eosinophilia, usually with at least 10% eosinophils (or 5000–9000 eosinophils/µL), and anemia.

In the case of EGPA, commonly found radiographic patterns are lobar or segmental opacity, diffuse interstitial or miliary patterns, migratory infiltrates of the lower lobe or subpleural, hilar, or mediastinal lymphadenopathy, pleural effusion, pulmonary hemorrhage, ground glass opacity, and hyperinflation. Sinonasal CT Scan is the imaging test of choice due to the optimal viewing and discrimination of pneumatic bone, solid bone, and soft tissue offered.

American College of Rheumatology/Vasculitis Foundation Guideline for managing ANCA Associated Vasculitis 2021 guidelines and guidance focus on screening, counseling, prevention, specialized serological tests, and monitoring of untreated patients.

Further details related to diagnosis are provided in the report…

ANCA Associated Vasculitis Epidemiology

The epidemiology forecast model of ANCA Associated Vasculitis for the 7MM and China is based on the analysis of the diagnosed prevalent cases of ANCA Associated Vasculitis. The ANCA Associated Vasculitis pool is further segmented by organ involvement, antibody type, and disease severity.

As the market is derived using the patient-based model, the ANCA Associated Vasculitis epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented by, Total Diagnosed Prevalent cases of ANCA Associated Vasculitis, Diagnosed Prevalent cases by Type of ANCA Associated Vasculitis, Diagnosed Prevalent Cases by Organ Involvement of ANCA Associated Vasculitis, Diagnosed Prevalent Cases by Antibody Type of ANCA Associated Vasculitis, Diagnosed Prevalent Cases by Severity of ANCA Associated Vasculitis, and Total Treated Cases by Type of ANCA Associated Vasculitis in the 7MM and China covering the United States, EU4 countries (Germany, France, Italy, and Spain), United Kingdom, Japan, and China from 2021 to 2034. The total diagnosed prevalent cases of ANCA Associated Vasculitis in the 7MM and China comprised approximately 547,150 cases in 2022 and are projected to increase during the forecasted period.

• The total diagnosed prevalent cases of ANCA-associated vasculitis in the United States were around 70,700 cases in 2022.
• In the 7MM, the United States, EU4 and the UK, Japan, and China accounted for around 13%, 16%, 9%, and 62% of the total diagnosed prevalent population share in 2022.
• Among the EU4 countries, Germany accounted for the largest number of ANCA-associated vasculitis cases, followed by the UK, whereas Spain accounted for the lowest number of cases in 2022.
• According to DelveInsight estimates, Japan had around 23,000, 17,170, and 9,420 diagnosed prevalent cases of MPA, GPA, and EGPA, respectively, in 2022. These cases are projected to increase during the forecasted period.
• The total diagnosed prevalent cases of ANCA-associated vasculitis in China were around 339,220 cases in 2022.

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 to understand and validate the patient pool, and forecasted trends included Medical/scientific writers, Medical Oncologists and Professors, Pediatric rheumatologists, ANCA Associated Vasculitis Foundation, and Others.

Delveinsight’s analysts connected with 50+ KOLs to gather insights; however, interviews were conducted with 15+ KOLs in the 7MM and China.

Scope of the Report

• The report covers a segment of key events, an executive summary, and a descriptive overview of ANCA Associated Vasculitis, explaining its causes, signs and symptoms, pathogenesis, and currently available therapies.
• 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.
• A detailed review of the ANCA Associated Vasculitis epidemiology, detailed assumptions, and the rationale behind our approach is included in the report.
• A detailed review of current challenges in establishing the diagnosis.

ANCA Associated Vasculitis Report Insights

• ANCA Associated Vasculitis Patient Population
• Patient Population by Organ Involvement, Antibody Type, and Disease Severity
• Country-wise Epidemiology Distribution

ANCA Associated Vasculitis Report Key Strengths

• Ten years Forecast
• The 7MM and China Coverage
• ANCA Associated Vasculitis Epidemiology Segmentation
• ANCA Associated Vasculitis Report Assessment
• Epidemiology Segmentation
• Current Diagnostic Practices

Key Questions

Epidemiology Insights

• What are the disease risks, burdens, and unmet needs of ANCA Associated Vasculitis? What will be the growth opportunities across the 7MM and China with respect to the patient population of ANCA Associated Vasculitis?
• What is the historical and forecasted ANCA Associated Vasculitis patient pool in the United States, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, Japan, and China?
• Why do only limited patients appear with symptoms? Why is the current year diagnosis rate not high?

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.
• The report will help develop business strategies by understanding the latest trends and changing treatment dynamics driving the ANCA Associated Vasculitis Market.
• 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 various factors hampering disease diagnosis and other existing diagnostic challenges.