Anti-neutrophilic cytoplasmic antibody (ANCA) associated Vasculitis - Epidemiology Forecast - 2032

Key Highlights

• Anti-neutrophilic cytoplasmic antibody (ANCA) associated vasculitis is a heterogeneous group of rare autoimmune conditions.
• ANCA Associated Vasculitis includes three main diseases, which are granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA).
• ANCA Associated Vasculitis triggers inflammation of blood vessels with various manifestations.
• Patients suffering from ANCA Associated Vasculitis often have general symptoms initially. As the disease progresses requires multiple specialties like a rheumatologist, pulmonologist, nephrologist, etc., to diagnose the disease, this could be the primary cause of the low diagnosis rate across the 7MM.
• 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.
• Due to advancements in health care infrastructure and new diagnostic techniques, the tendency toward diagnosis is rising in the US.
• In 2022, the total diagnosed prevalent cases of AAV were ~207,920 in the 7MM, which might rise by 2032 at a significant CAGR. Among these, approximately 52% of cases are of GPA, 32% are of MPA, and 16% 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.

This “ANCA Associated Vasculitis - Epidemiology Forecast - 2032 report delivers an in-depth understanding of the ANCA Associated Vasculitis, historical and forecasted epidemiology in the United States, EU4 (Germany, France, Italy, and Spain), and the United Kingdom, Japan.

Geography Covered

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

Study Period: 2019-2032
ANCA Associated Vasculitis Disease 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 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. 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.

Other tests include EGPA, which can be done to check for specific organ-system involvement: Electrocardiogram (ECG) for cardiac manifestations, gastrointestinal endoscopy for GI bleeding, EMG, and nerve conduction for peripheral neuropathies. If local organ involvement exists, obtaining a biopsy of that organ is most helpful in confirming the diagnosis. Biopsies of the following may be considered: Skin, Lung - Open or video-assisted thoracoscopic biopsy is preferred over transbronchial, renal, etc.

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 country-based variations are provided in the report.

ANCA Associated Vasculitis Epidemiology

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 covering the United States, EU4 countries (Germany, France, Italy, and Spain), United Kingdom, Japan from 2019 to 2032. The total diagnosed prevalent cases of ANCA-associated vasculitis in the 7MM comprised approximately 207,900 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, the UK, and Japan accounted for around 34%, 42%, and 24% 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 the publisher's estimates, Japan had around 23,000, 17,700, and 9,420 diagnosed prevalent cases of MPA, GPA, and EGPA, respectively, in 2022. These cases are projected to increase during the forecasted period.

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.

This analysts connected with 50+ KOLs to gather insights; however, interviews were conducted with 15+ KOLs in the 7MM.

Scope of the Report

• The report covers a segment of key events, an executive summary, descriptive overview of ANCA Associated Vasculitis, 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, and disease progression has been provided.
• A detailed review of current challenges in establishing diagnosis and diagnosis rate is provided.

ANCA Associated Vasculitis Report Insights

• Patient population
• Prevalence pattern
• Diagnosis rate
• Countrywise epidemiology distribution

ANCA Associated Vasculitis Report Key Strengths

• 10 years patient based forecast
• The 7MM coverage
• ANCA Associated Vasculitis epidemiology segmentation

ANCA Associated Vasculitis Report Assessment

• Epidemiology segmentation
• Current diagnostic practices
• Unmet needs

Key Questions Answered

Epidemiology insights

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

Reasons to Buy

• The report will help develop business strategies by understanding the latest trends and changing treatment dynamics driving the ANCA Associated Vasculitis market.
• Insights on patient burden/disease diagnosis prevalence, evolution in diagnosis, and factors contributing to the change in the epidemiology of the disease during the forecast years.
• To understand the existing market opportunity in varying geographies and the growth potential over the coming years.
• To understand the perspective of key opinion leaders around the accessibility, acceptability, and compliance-related challenges of existing treatment to overcome barriers in the future.
• Detailed insights on the unmet need of the existing market so that the upcoming players can strengthen their development and launch strategy.