A small-vessel necrotizing vasculitis characterized by the association of inflammation of the vessel wall and peri- and extravascular granulomatosis.
Comprehensive, easy-to-understand information about this condition
How we create this content →Documentation on granulomatosis with polyangiitis is limited due to its relatively rare occurrence, affecting 1-9 individuals per 100,000. This rarity restricts the scope of systematic clinical studies and comprehensive data collection. Additionally, the complex and variable nature of the disease, including overlapping symptoms with other conditions, complicates clinical characterization and understanding.
Key clinical features of granulomatosis with polyangiitis include cytoplasmic antineutrophil antibody positivity (80-99%), which is a significant marker for diagnosis. Patients may experience nasal mucosa vasculitis (30-79%), leading to symptoms such as nasal obstruction or bleeding. Additionally, subglottic stenosis and tracheal stenosis are observed in 5-29% of cases, potentially resulting in airway complications. These manifestations highlight the importance of early recognition and management.
To navigate your care effectively, seek a rheumatologist or an immunologist with expertise in vasculitis. These specialists can provide targeted management for granulomatosis with polyangiitis. Additionally, consider participating in clinical trials, which may offer access to new therapies. For more resources, visit the Genetic and Rare Diseases Information Center at https://rarediseases.info.nih.gov. Although there are no specific patient organizations identified, connecting with broader vasculitis support groups may provide community and resources.
Currently, there are 36 active clinical trials focused on granulomatosis with polyangiitis, reflecting ongoing research efforts to better understand and treat this condition. While there are no orphan drugs designated specifically for GPA, these trials may explore new therapeutic options and improve patient outcomes. For more information on active trials, you can visit ClinicalTrials.gov at https://clinicaltrials.gov/search?cond=granulomatosis%20with%20polyangiitis.
Actionable guidance for navigating care for granulomatosis with polyangiitis
To navigate your care effectively, seek a rheumatologist or an immunologist with expertise in vasculitis. These specialists can provide targeted management for granulomatosis with polyangiitis. Additionally, consider participating in clinical trials, which may offer access to new therapies. For more resources, visit the Genetic and Rare Diseases Information Center at https://rarediseases.info.nih.gov. Although there are no specific patient organizations identified, connecting with broader vasculitis support groups may provide community and resources.
Consider asking your healthcare providers these condition-specific questions
Helpful links for rare disease information and support
Documentation on granulomatosis with polyangiitis is limited due to its relatively rare occurrence, affecting 1-9 individuals per 100,000. This rarity restricts the scope of systematic clinical studies and comprehensive data collection. Additionally, the complex and variable nature of the disease, including overlapping symptoms with other conditions, complicates clinical characterization and understanding.
Key clinical features of granulomatosis with polyangiitis include cytoplasmic antineutrophil antibody positivity (80-99%), which is a significant marker for diagnosis. Patients may experience nasal mucosa vasculitis (30-79%), leading to symptoms such as nasal obstruction or bleeding. Additionally, subglottic stenosis and tracheal stenosis are observed in 5-29% of cases, potentially resulting in airway complications. These manifestations highlight the importance of early recognition and management.
To navigate your care effectively, seek a rheumatologist or an immunologist with expertise in vasculitis. These specialists can provide targeted management for granulomatosis with polyangiitis. Additionally, consider participating in clinical trials, which may offer access to new therapies. For more resources, visit the Genetic and Rare Diseases Information Center at https://rarediseases.info.nih.gov. Although there are no specific patient organizations identified, connecting with broader vasculitis support groups may provide community and resources.
Currently, there are 36 active clinical trials focused on granulomatosis with polyangiitis, reflecting ongoing research efforts to better understand and treat this condition. While there are no orphan drugs designated specifically for GPA, these trials may explore new therapeutic options and improve patient outcomes. For more information on active trials, you can visit ClinicalTrials.gov at https://clinicaltrials.gov/search?cond=granulomatosis%20with%20polyangiitis.
Actionable guidance for navigating care for granulomatosis with polyangiitis
To navigate your care effectively, seek a rheumatologist or an immunologist with expertise in vasculitis. These specialists can provide targeted management for granulomatosis with polyangiitis. Additionally, consider participating in clinical trials, which may offer access to new therapies. For more resources, visit the Genetic and Rare Diseases Information Center at https://rarediseases.info.nih.gov. Although there are no specific patient organizations identified, connecting with broader vasculitis support groups may provide community and resources.
Consider asking your healthcare providers these condition-specific questions
Helpful links for rare disease information and support
Documentation on granulomatosis with polyangiitis is limited due to its relatively rare occurrence, affecting 1-9 individuals per 100,000. This rarity restricts the scope of systematic clinical studies and comprehensive data collection. Additionally, the complex and variable nature of the disease, including overlapping symptoms with other conditions, complicates clinical characterization and understanding.
Key clinical features of granulomatosis with polyangiitis include cytoplasmic antineutrophil antibody positivity (80-99%), which is a significant marker for diagnosis. Patients may experience nasal mucosa vasculitis (30-79%), leading to symptoms such as nasal obstruction or bleeding. Additionally, subglottic stenosis and tracheal stenosis are observed in 5-29% of cases, potentially resulting in airway complications. These manifestations highlight the importance of early recognition and management.
To navigate your care effectively, seek a rheumatologist or an immunologist with expertise in vasculitis. These specialists can provide targeted management for granulomatosis with polyangiitis. Additionally, consider participating in clinical trials, which may offer access to new therapies. For more resources, visit the Genetic and Rare Diseases Information Center at https://rarediseases.info.nih.gov. Although there are no specific patient organizations identified, connecting with broader vasculitis support groups may provide community and resources.
Currently, there are 36 active clinical trials focused on granulomatosis with polyangiitis, reflecting ongoing research efforts to better understand and treat this condition. While there are no orphan drugs designated specifically for GPA, these trials may explore new therapeutic options and improve patient outcomes. For more information on active trials, you can visit ClinicalTrials.gov at https://clinicaltrials.gov/search?cond=granulomatosis%20with%20polyangiitis.
Actionable guidance for navigating care for granulomatosis with polyangiitis
To navigate your care effectively, seek a rheumatologist or an immunologist with expertise in vasculitis. These specialists can provide targeted management for granulomatosis with polyangiitis. Additionally, consider participating in clinical trials, which may offer access to new therapies. For more resources, visit the Genetic and Rare Diseases Information Center at https://rarediseases.info.nih.gov. Although there are no specific patient organizations identified, connecting with broader vasculitis support groups may provide community and resources.
Consider asking your healthcare providers these condition-specific questions
Helpful links for rare disease information and support
Inheritance patterns describe how genetic conditions are passed from parents to children.
Research studies investigating treatments and therapies for this condition.
Active Trials
Total Trials
Data from ClinicalTrials.gov Feb 8, 2026
Consider asking your healthcare providers these condition-specific questions
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
1 peer-reviewed source from PubMed
AI-Generated Content: This summary was generated using AI. Content has been fact-checked. Always consult with qualified healthcare providers for medical guidance.
Organizations with approved therapies for this disease
No approved therapies yet
Research is ongoing — 4 companies have orphan drug designations for this disease
Kisho delivers this disease record via API, including phenotypes (HPO), genes, orphan drug designations, screening status, and PAG mapping, with version history and governance.