Anti-neutrophilic cytoplasmic antibody (ANCA) associated Vasculitis Epidemiology

Key Highlights

• Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of uncommon autoimmune disorders of unknown origin, marked by inflammation and infiltration of immune cells leading to blood vessel necrosis. AAV encompasses three distinct diseases: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).
• AAV presenting symptoms include sinus pain, nasal discharge, or crusting, ear pain, or deafness (from upper airways involvement), cough, shortness of breath, wheeze or haemoptysis (from lung involvement), and painful, red eyes (from scleritis).
• The exact biological mechanisms behind this autoimmune disorder remain unclear; however, studies suggest it arises from a combination of genetic predisposition and environmental influences, including exposure to pollutants, certain medications, and microbial infections.
• The diagnosis of AVV relies on clinical evaluation, laboratory tests (including ANCA testing), imaging studies, and biopsy of affected tissues. Treatment for AVV involves a combination of immunosuppressive therapies aimed at decreasing inflammation and preventing further organ damage.
• In 2024, AAV affected approximately 220,000 individuals across the 7MM, and it is expected to grow with a CAGR of 3% during the forecast period (2025-2034).
• The United States accounted for around 34% of the total diagnosed prevalent cases, with approximately 75,500 cases, the highest among all 7MM countries. This dominance likely reflects a large patient population, higher diagnostic rates, and broader disease awareness.
• Japan contributed nearly 24% of the total AAV burden, reporting around 52,000 cases, indicating significant disease recognition possibly influenced by an aging population and established diagnostic pathways.
• Germany represented about 11% of the total diagnosed prevalence, with approximately 24,000 cases, making it the highest among the EU4 and UK countries. This may be due to consistent clinical surveillance and access to specialized care.
• The growing prevalence of AAV, particularly in high-burden countries like the US and Japan, highlights a clear need for improved diagnostic precision, earlier intervention, and more durable, targeted therapies—offering strong market potential amid rising awareness and evolving clinical practices across the 7MM.

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

The table given below further depicts the key segments provided in the report:

ANCA-associated Vasculitis (AAV): Disease Understanding

ANCA-associated Vasculitis (AAV) Overview, and Diagnosis

ANCA-associated vasculitides (AAV) are a diverse group of rare autoimmune disorders characterized by inflammation of blood vessels and a wide range of clinical features. The group primarily includes three key conditions: granulomatosis with polyangiitis (GPA), formerly called Wegener’s granulomatosis; eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome; and microscopic polyangiitis (MPA). Additional ANCA-related conditions include drug-induced vasculitis and vasculitis limited to the kidneys.

These diseases often have the presence of circulating autoantibodies (ANCA) that are usually directed against myeloperoxidase (MPO) or proteinase 3 (PR3) antigens.

The exact cause of AAV is unknown but is probably multifactorial. Several genetic and environmental risk factors have been identified. Triggers for developing AAV include microbial infections, a reaction to certain medications, certain genetic variations, or exposure to toxins.

The pathogenetic steps involved in AAV include regulation of cytokines (T cells and B cells), priming of neutrophils and monocytes as a key step in the pathogenesis of PR3-and MPO-ANCA vasculitis, activation of neutrophils and monocytes, T-cell and B-cell activation in PR3 and MPO-ANCA, tissue damage and repair followed by endothelial injury and repair.

The diagnosis of AAV 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 in combination with chronic systemic symptoms. Therefore, when two or more of these symptoms develop concurrently, the index of suspicion should be high.

Physicians use a series of tests to identify vasculitis and classify a patient’s type of ANCA vasculitis. Blood and urine are collected to test for autoantibodies, inflammatory markers, and kidney disease. Tissue samples, or biopsies, also are needed to confirm the diagnosis.

ANCA-associated Vasculitis (AAV) Epidemiology

The AVV epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented by the Total Diagnosed Prevalent Cases of AAV, Diagnosed Prevalent Cases of AAV by Type , Diagnosed Prevalent Cases of AAV by Organ Involvement, Diagnosed Prevalent Cases of AAV by Antibody Type,  Diagnosed Prevalent Cases of AAV by Severity and Total Treated Cases of AAV by Type in the United States, EU4  (Germany, France, Italy, Spain) and the United Kingdom, and Japan from 2020 to 2034.

• In 2024, in the US, Granulomatosis with polyangiitis (GPA) accounted for ~49%, Microscopic polyangiitis (MPA) ~40%, and Eosinophilic granulomatosis with polyangiitis (EGPA) ~17% of the total diagnosed AAV cases.
• In 2024, renal impairment was the most common organ involvement in MPA cases in the US, affecting approximately 25,700 individuals, followed by lower respiratory tract involvement with around 13,500 cases. Eye involvement was the least common, seen in about 1,500 cases, underscoring MPA’s predominant impact on critical organs like the kidneys and lungs.
• In 2024, in the US, approximately 18,000 cases of Microscopic Polyangiitis (MPA) were classified as limited, accounting for about 60% of the total diagnosed MPA cases, which are approximately 30,000.
• In 2024, the total initial treated cases and relapsed and refractory cases of MPA in the US were 28,000 and 8,500, respectively.
• In 2024, the diagnosed prevalent cases of MPA by antibody type in the US were ~1,200, ~28,000, and ~300 for cANCAs/PR3-ANCAs, pANCAs/MPO-ANCAs, and ANCA-negative, respectively.

KOL Views

To gaze into the epidemiology insights of the real world, we take KOLs and SMEs’ opinions working in the domain through primary research to fill the data gaps and validate our secondary research on disease prevalence.

DelveInsight’s analysts connected with 10+ KOLs to gather insights; however, interviews were conducted with 5+ KOLs in the 7MM. Centers such as the Johns Hopkins Vasculitis Center, US; European Vasculitis Society (EUVAS), National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), US, University of Cambridge, UK – Vasculitis Research Group, Kyushu University, Japan – Rheumatology and Vasculitis research; and others were contacted. Their opinion helps understand and validate current disease prevalence, gender involved with the disease, diagnosis rate, and diagnostic criteria.

Scope of the Report

• The report covers a segment of an executive summary, and a descriptive overview of ANCA-associated Vasculitis (AAV), explaining its causes, signs and symptoms, pathogenesis.
• Comprehensive insight into the epidemiology segments and forecasts, the future growth potential of diagnosis rate, and disease progression have been provided.
• A detailed review of current challenges in establishing diagnosis and diagnosis rate is provided.

ANCA-associated Vasculitis (AAV) Report Insights

• Patient Population
• Country-wise Epidemiology Distribution

ANCA-associated Vasculitis (AAV) Report Key Strengths

• Ten-year Forecast
• The 7MM Coverage
• ANCA-associated Vasculitis (AAV) Epidemiology Segmentation

ANCA-associated Vasculitis (AAV) Report Assessment

• Epidemiology Segmentation
• Current Diagnostic Practices

FAQs

Epidemiology Insights

• What are the disease risks, burdens, and unmet needs of ANCA-associated Vasculitis (AAV)? What will be the growth opportunities across the 7MM with respect to the patient population pertaining to ANCA-associated Vasculitis (AAV)?
• What is the historical and forecasted ANCA-associated Vasculitis (AAV) patient pool in the United States, EU4 (Germany, France, Italy, Spain) and the United Kingdom, and Japan?
• What is the diagnostic pattern of ANCA-associated Vasculitis (AAV)?
• Which clinical factors will affect ANCA-associated Vasculitis (AAV)?
• Which factors will affect the increase in the diagnosis of ANCA-associated Vasculitis (AAV)?

Reasons to buy

• Insights on disease burden, details regarding diagnosis, and factors contributing to the change in the epidemiology of the disease during the forecast years.
• To understand the change in ANCA-associated Vasculitis (AAV) cases in varying geographies over the coming years.
• A detailed overview of total prevalent cases of ANCA-associated Vasculitis (AAV), total diagnosed prevalent cases of ANCA-associated Vasculitis (AAV), type-specific cases of ANCA-associated Vasculitis (AAV), gender-specific cases of ANCA-associated Vasculitis (AAV), age-specific cases of ANCA-associated Vasculitis (AAV) and total treated cases is included.
• Detailed insights on various factors hampering disease diagnosis and other existing diagnostic challenges.