Granulomatosis With Polyangiitis Epidemiology Forecast Insight

DelveInsight’s ‘Granulomatosis With Polyangiitis  – 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.

Geographies Covered

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

Study Period: 2018–2030

Granulomatosis With Polyangiitis : 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 by DelveInsight 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

1. Key Insights

2. Report Introduction

3. Executive Summary of Granulomatosis with Polyangiitis

4. Disease Background and Overview

4.1. Introduction

4.2. Clinical Manifestation

4.3. Different Phenotypes of GPA

4.4. Etiology

4.5. Pathophysiology

4.6. Diagnosis

4.6.1. Differential Diagnosis

4.7. Treatment

4.8. Treatment Guidelines

5. Epidemiology and Population Patient

5.1. Key Findings

5.2. 7MM Total Incident cases of GPA

5.3. Epidemiology of Granulomatosis with Polyangiitis

5.4. The United States

5.4.1. Incident cases of GPA in the United States

5.4.2. Gender-specific cases of GPA in the United States

5.4.3. Age-specific cases of GPA in the United States

5.5. EU5

5.5.1. Germany

5.5.1.1. Incident cases of GPA in Germany

5.5.1.2. Gender-specific cases of GPA in Germany

5.5.1.3. Age-specific cases of GPA in Germany

5.5.2. France

5.5.2.1. Incident cases of GPA in France

5.5.2.2. Gender-specific cases of GPA in France

5.5.2.3. Age-specific cases of GPA in France

5.5.3. Italy

5.5.3.1. Incident cases of GPA in Italy

5.5.3.2. Gender-specific cases of GPA in Italy

5.5.3.3. Age-specific cases of GPA in Italy

5.5.4. Spain

5.5.4.1. Incident cases of GPA in Spain

5.5.4.2. Gender-specific cases of GPA in Spain

5.5.4.3. Age-specific cases of GPA in Spain

5.5.5. The United Kingdom

5.5.5.1. Incident cases of GPA in the United Kingdom

5.5.5.2. Gender-specific cases of GPA in the United Kingdom

5.5.5.3. Age-specific cases of GPA in the United Kingdom

5.5.6. Japan

5.5.6.1. Incident cases of GPA in Japan

5.5.6.2. Gender-specific cases of GPA in Japan

5.5.6.3. Age-specific cases of GPA in Japan

6. Organizations contributing toward the fight against GPA

7. Patient Journey

8. Case Report

9. Appendix

9.1. Bibliography

9.2. Report Methodology

10. DelveInsight Capabilities

12. Disclaimer

13. About DelveInsight

List of Table

Table 1: Summary of GPA, Market, Epidemiology, and Key Events (2018–2030)

Table 2:  Clinical manifestations of GPA

Table 3: The American College of Rheumatology criteria for GPA diagnosis.

Table 4:  Clinical-imaging involvement and diagnostic technique of choice.

Table 5: Differential diagnosis of GPA

Table 6: Immunosuppression in GPA, based on disease severity

Table7: Short-term side effects and long-term damage associated with treatment in GPA

Table 8: Recommendation statements by EULAR/ERA-EDTA recommendations for managing AAV

Table 9: Recommendation statements by Japan Research Committee of the Ministry of Health, Labor, and Welfare for Intractable Vasculitis for the management of ANCA-associated vasculitis

Table 10: Evidence-based clinical practice guideline related to the treatment and management of systemic vasculitis by  American College of Rheumatology (ACR)

Table 11: Total Incident cases of GPA in 7MM (2018–2030)

Table 12: Incident cases of GPA in the United States (2018–2030)

Table 13: Gender-specific cases of GPA in the United States (2018–2030)

Table 14: Age-specific cases of GPA in the United States (2018–2030)

Table 15: Incident cases of GPA in Germany (2018–2030)

Table 16: Gender-specific cases of GPA in Germany (2018–2030)

Table 17: Age-specific cases of GPA in Germany (2018–2030)

Table 18: Incident cases of GPA in France (2018–2030)

Table 19: Gender-specific cases of GPA in France (2018–2030)

Table 20: Age-specific cases of GPA in France (2018–2030)

Table 21: Incident cases of GPA in Italy (2018–2030)

Table 22: Gender-specific cases of GPA in Italy (2018–2030)

Table 23: Age-specific cases of GPA in Italy (2018–2030)

Table 24: Incident cases of GPA in Spain (2018–2030)

Table 25: Gender-specific cases of GPA in Spain (2018–2030)

Table 26: Age-specific cases of GPA in Spain (2018–2030)

Table 27: Incident cases of GPA in the United Kingdom (2018–2030)

Table 28: Gender-specific cases of GPA in the United Kingdom (2018–2030)

Table 29: Age-specific cases of GPA in the United Kingdom (2018–2030)

Table 30: Incident cases of GPA in Japan (2018–2030)

Table 31: Gender-specific cases of GPA in Japan (2018–2030)

Table 32: Age-specific cases of GPA in Japan (2018–2030)

Table 33: Organizations contributing toward the fight against GPA

List of Figures

Figure 1: Clinical Signs and Symptoms of GPA

Figure 2: Different phenotypes of GPA

Figure 3: Etiology of GPA

Figure 4: Genetic predisposition for GPA (protein whose related genes are thought to be associated with GPA)

Figure 5: Immune response subversion induced by PR3

Figure 6: Treatment Therapies in GPA

Figure 7: Management of GPA cases that present with organ or life-threatening manifestations

Figure 8: Algorithm for management of AAV by Japan Research Committee of the Ministry of Health, Labour, and Welfare for Intractable Vasculitis

Figure 9: Total Incident cases of GPA in 7MM (2018–2030)

Figure 10: Incident cases of GPA in the United States (2018–2030)

Figure 11: Gender-specific cases of GPA in the United States (2018–2030)

Figure 12: Age-specific cases of GPA in the United States (2018–2030)

Figure 13: Incident cases of GPA in Germany (2018–2030)

Figure 14: Gender-specific cases of GPA in Germany (2018–2030)

Figure 15: Age-specific cases of GPA in Germany (2018–2030)

Figure 16: Incident cases of GPA in France (2018–2030)

Figure 17: Gender-specific cases of GPA in France (2018–2030)

Figure 18: Age-specific cases of GPA in France (2018–2030)

Figure 19: Incident cases of GPA in Italy (2018–2030)

Figure 20: Gender-specific cases of GPA in Italy (2018–2030)

Figure 21: Age-specific cases of GPA in Italy (2018–2030)

Figure 22: Incident cases of GPA in Spain (2018–2030)

Figure 23: Gender-specific cases of GPA in Spain (2018–2030)

Figure 24: Age-specific cases of GPA in Spain (2018–2030)

Figure 25: Incident cases of GPA in the United Kingdom (2018–2030)

Figure 26: Gender-specific cases of GPA in the United Kingdom (2018–2030)

Figure 27: Age-specific cases of GPA in the United Kingdom (2018–2030)

Figure 28: Incident cases of GPA in Japan (2018–2030)

Figure 29: Gender-specific cases of GPA in Japan (2018–2030)

Figure 30: Age-specific cases of GPA in Japan (2018–2030)

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