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Granulomatosis with Polyangiitis - Epidemiology Forecast to 2030

Granulomatosis with Polyangiitis - Epidemiology Forecast to 2030

  • April 2021
  • 60 pages
  • ID: 6064463
  • Format: PDF
  • Delve Insight

Summary

Table of Contents

‘Granulomatosis with polyangiitis (GPA) – Epidemiology Forecast – 2030’ report delivers an in-depth understanding of the historical and forecasted epidemiology of GPA in the United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan.

Granulomatosis with polyangiitis (GPA): Disease Understanding

GPA Overview
Granulomatosis with polyangiitis (GPA, formerly known as Wegener’s granulomatosis) is an autoimmune small-vessel vasculitis that is highly associated with anti-neutrophil cytoplasmic antibodies (ANCA). According to the Chapel Hill criteria (2012), GPA is defined as necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small-to-medium vessels (e.g., capillaries, venules, arterioles, arteries, and veins).
The disease was initially described by Klinger in 1931 as a variant of polyarteritis nodosa and was defined for the first time as a separated syndrome by Friedrich Wegener. Godman and Churg (1954) introduced the term ‘Wegener’s granulomatosis’ and further delineated the clinical and pathological features.
Two phenotypes are distinguished in GPA that are localized and systemic with the possible transition during disease. Typically, patients initially suffer from a limited form that may consist of constitutional symptoms and localized manifestations, such as chronic sinusitis, rhinitis, otitis media, ocular conditions, and/or skin lesions. More serious manifestations may arise in later stages, including pulmonary complications and glomerulonephritis, although the skin, eyes, and heart may also be involved. Classically, the acronym ELK is used to describe the clinical involvement of the ear, nose, and throat (ENT), lungs, and kidneys.
The etiology of GPA is linked to environmental and infectious triggers inciting disease onset in genetically predisposed individuals. Anti-neutrophil cytoplasmic antibodies are pathogenic and play an important role in the pathogenesis of this disease. Pathological processes involved include aberrant epigenetic expression of proteinase-3 on the cell membrane of neutrophils and the formation of antibodies against proteinase-3 (PR3-ANCA). The binding of PR3-ANCA to PR3 activates neutrophils, and there is a release of neutrophilic inflammatory mediators, the formation of neutrophil extracellular traps that lead to complement activation and damage to endothelial cells of small blood vessels.
Diagnosis of GPA is achieved through clinical assessment, serological tests for anti-neutrophil cytoplasmic antibodies (ANCA), and histological analysis. Biopsy, imaging, and laboratory tests are involved. Symptoms of microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), Goodpasture syndrome (GPS), polyarteritis nodosa (PAN), lymphomatous granulomatosis (LyG), and systemic rheumatological disorders can be similar to those of GPA for which differential diagnosis is useful.
The treatment of GPA involves Induction therapy as the first phase, which aims to quickly put the disease into remission and lasts about 3–6 months according to the clinical response, and maintenance therapy as the second phase that lasts 12–24 months to consolidate the remission and limit the risk of relapse. The standard therapy for GPA is a combination of glucocorticoids and cyclophosphamide/ rituximab. Adjuvant therapy and plasma exchange are also involved.
The early diagnosis and specific treatment of GPA are important but difficult to achieve because of the multiplicity of presenting symptoms and unclear histological features. Talking about the pathogenesis of GPA, it has undergone many advances and progressed over the years. Hopefully, this will be translated into the development of new and better therapies for patients.

Granulomatosis with polyangiitis: Epidemiology
The GPA epidemiology division provides insights into the historical and current patient pool, along with the forecasted trend for every seven major countries. It helps recognize the causes of current and forecasted trends by exploring numerous studies and views of key opinion leaders. This part of the report also provides the diagnosed patient pool and their trends, along with assumptions undertaken.

Key Findings
The disease epidemiology covered in the report provides historical and forecasted GPA epidemiology segmented as the Incident cases of granulomatosis with polyangiitis, Gender-specific cases of granulomatosis with polyangiitis and Age-specific cases of granulomatosis with polyangiitis. The report includes the incident scenario of GPA in the 7MM covering the United States, EU5 countries (Germany, France, Italy, Spain, and the United Kingdom), and Japan from 2018 to 2030.

Country-wise GPA Epidemiology
The epidemiology segment also provides the GPA epidemiology data and findings across the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan.
The total incident population of GPA in the 7MM countries was estimated to be 7,618 cases in 2020.
As per the estimates, the United States had the largest incidence of granulomatosis with polyangiitis in 2020. Among the EU5 countries, Germany had the largest incident population of GPA with 2,722 cases, followed by the UK in 2020. On the other hand, Italy had the lowest incident population of 150 cases in 2020.

Scope of the Report
• GPA report covers a detailed overview explaining its causes, symptoms, classification, pathophysiology, diagnosis, and treatment patterns.
• GPA Epidemiology Report and Model provide an overview of the risk factors and global GPA trends in the seven major markets (7MM: US, France, Germany, Italy, Spain, UK, and Japan).
• The report provides insight into the historical and forecasted patient pool of GPA in seven major markets covering the United States, EU5 (Germany, Spain, France, Italy, UK), and Japan
• The report helps recognize the growth opportunities in the 7MM concerning the patient population.
• The report assesses the disease risk and burden and highlights the unmet needs of granulomatosis with polyangiitis.
• The report provides the segmentation of the GPA epidemiology by incident cases of GPA in the 7MM
• The report provides the segmentation of the GPA epidemiology by gender-specific cases of GPA in the 7MM.
• The report provides the segmentation of the GPA epidemiology by age-specific cases of GPA in the 7MM.

Report Highlights

• 10-year Forecast of GPA epidemiology
• 7MM Coverage
• Incident Cases of Granulomatosis with polyangiitis
• Gender-specific Cases of Granulomatosis with polyangiitis
• Age-specific Cases of Granulomatosis with polyangiitis

KOL Views
We interview KOLs and obtain SME’s opinion through primary research to fill the data gaps and validate our secondary research. The opinion helps understand the total patient population and current treatment pattern. This will support the clients in potential upcoming novel treatment by identifying the overall scenario of the indications.

Key Questions Answered
• What will be the growth opportunities in the 7MM for the patient population pertaining to GPA?
• What are the key findings pertaining to the granulomatosis with polyangiitis epidemiology across 7MM, and which country will have the highest number of patients during the forecast period (2018–2030)?
• What would be the total number of patients with granulomatosis with polyangiitis across the 7MM during the forecast period (2018–2030)?
• Among the EU5 countries, which country will have the highest number of patients during the forecast period (2018–2030)?
• At what CAGR the patient population is expected to grow by in the 7MM during the forecast period (2018–2030)?
• What are the disease risk, burdens, and unmet needs of granulomatosis with polyangiitis?
• What are the currently available treatments for granulomatosis with polyangiitis?

Reasons to buy
GPA Epidemiology report will allow the user to:
• Develop business strategies by understanding the trends shaping and driving the global GPA market
• Quantify patient populations in the global granulomatosis with polyangiitis market to improve product design, pricing, and launch plans
• Organize sales and marketing efforts by identifying the age groups and sex that present the best opportunities for granulomatosis with polyangiitis therapeutics in each of the markets covered
• Understand the magnitude of the granulomatosis with polyangiitis population by its incident cases.
• Understand the magnitude of the granulomatosis with polyangiitis population by its gender-specific cases.
• Understand the magnitude of the granulomatosis with polyangiitis population by its age-specific cases.
• The GPA epidemiology report and model were written and developed by Masters and PhD level epidemiologists
• The GPA Epidemiology Model developed is easy to navigate, interactive with dashboards, and epidemiology based on transparent and consistent methodologies. Moreover, the model supports data presented in the report and showcases disease trends over a 10-year forecast period using reputable sources

Key Assessments
• Patient Segmentation
• Disease Risk and Burden
• Risk of disease by the segmentation
• Factors driving growth in a specific patient population

Geographies Covered
• The United States
• EU5 (Germany, France, Italy, Spain, and the United Kingdom)
• Japan

Study Period: 2018–2030
Granulomatosis with Polyangiitis (GPA), formerly known as Wegener’s Granulomatosis is a rare systemic necrotizing vasculitis that affects small and medium-sized blood vessels. As per the classification on the Modern Nomenclature of Systemic Vasculitides laid down at the Chapel Hill Consensus Conference in 2012, GPA is a component of a vast spectrum of disorders entitled the anti-neutrophil-cytoplasmic-antibody (ANCA) associated vasculitides (AAV). AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA or Churg–Strauss Syndrome). Among these three ANCA-associated vasculitides, GPA is the most common. The annual worldwide incidence of GPA is estimated to be 10-20 cases per one million based upon the geographical location. A higher incidence is noted in the colder regions.

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