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granulomatosis with polyangiitis - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

granulomatosis with polyangiitis

MONDO:0012105

Also known as:ANCA-associated vasculitisWgGPA

Find Clinical TrialsTrials

Overview

A small-vessel necrotizing vasculitis characterized by the association of inflammation of the vessel wall and peri- and extravascular granulomatosis.

GeneticsPolygenic (multifactorial)

TreatmentsNo approved treatments · 2 in pipeline

Clinical Trials27 registered · 17 recruiting

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include very common findings: Cytoplasmic antineutrophil antibody positivity; and common findings: Nasal mucosa vasculitis. 32 total HPO annotations.

Organ System	Phenotype Count	Example Features
Lungs and breathing	5	Hemosiderin-laden macrophages in bronchoalveolar fluid, Elevated bronchoalveolar lavage fluid neutrophil proportion, Pulmonary infiltrates

Diagnosis

Biomarker and diagnostic research for granulomatosis with polyangiitis has been reported in the published literature.

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

No approved treatments are currently available for granulomatosis with polyangiitis. An additional 2 compounds hold orphan drug designation.

Orphan Drug Designations

While no drugs are FDA-approved specifically for granulomatosis with polyangiitis, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.

The following drugs have received orphan drug designation from the FDA for granulomatosis with polyangiitis. Orphan designation reflects regulatory interest and does not indicate approval for treatment.

Brand Name	Generic Name	Sponsor

Prognosis

Phenotype severity distribution: 1 very common feature, 1 common feature.

Estimated prevalence: 1-9 in 100,000 (Uncommon).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

27 clinical trials registered, 17 recruiting. Interventions under study include drug therapy, other interventions, biologic therapy, and procedural interventions. Pipeline includes 2 PHASE4, 7 PHASE3, 5 PHASE2. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT07087912](https://clinicaltrials.gov/study/NCT07087912)

Data assembled from 7 of 12 sources · Last updated Sep 19, 2026, 1:04 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:608710

Orphanet

European rare disease database

Orphanet:900

GARD

Genetic and Rare Diseases Info Center

GARD:7880

Questions for Your Care Team

→Are any of the 27 active clinical trials a good fit for my situation?
→What is the current development status of gusperimus trihydrochloride, and when might it become available?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Research Type	Count	% of Total
Research summaries	34	27%
Patient case studies	33	26%
Clinical study results	22	18%
Disease patterns and progression	15	12%
Laboratory research	11	9%
Testing and diagnosis research	6	5%
Other research	2	2%
New treatment approaches	2	2%

Kisho

granulomatosis with polyangiitis

Overview

Clinical Presentation

Diagnosis

Treatment

Orphan Drug Designations

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Clinical Trials

Recent Publications

Latest News & Developments

Transethmoidal encephalocele associated with granulomatosis with polyangiitis: A case report.

A Case Report of Granulomatosis with Polyangiitis Presenting with Chronic Conjunctivitis.

Association of rhinocerebral mucormycosis and granulomatosis with polyangiitis (Wegener's granulomatosis).

granulomatosis with polyangiitis

Overview

Clinical Presentation

Diagnosis

Treatment

Orphan Drug Designations

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Clinical Trials

Recent Publications

Latest News & Developments

Transethmoidal encephalocele associated with granulomatosis with polyangiitis: A case report.

A Case Report of Granulomatosis with Polyangiitis Presenting with Chronic Conjunctivitis.

Association of rhinocerebral mucormycosis and granulomatosis with polyangiitis (Wegener's granulomatosis).