Anti-glomerular basement membrane disease

Summary

Anti-glomerular basement membrane disease (MONDO:0009303) is a disease and 4 clinical trials. Top therapeutic interventions include cyclophosphamide anhydrous and imlifidase. A subtype of autoimmune disease — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: 1-9 / 1 000 000 (Europe) [Orphanet-validated]
• Phenotypes (HPO): 19
• Clinical trials: 4

Clinical features

Epidemiology

Prevalence records

4 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.08	Europe	Validated
Point prevalence	1-9 / 1 000 000	0.2	Europe	Validated
Annual incidence	1-9 / 1 000 000	0.179	New Zealand	Validated
Annual incidence	<1 / 1 000 000	0.06	China	Validated

Signs & symptoms

Clinical features (HPO)

19 HPO clinical features (Orphanet curated; top 19 by frequency):

HPO ID	Term	Frequency
HP:0000093	Proteinuria	Very frequent (80-99%)
HP:0001903	Anemia	Very frequent (80-99%)
HP:0002093	Respiratory insufficiency	Very frequent (80-99%)
HP:0002105	Hemoptysis	Very frequent (80-99%)
HP:0002113	Pulmonary infiltrates	Very frequent (80-99%)
HP:0002633	Vasculitis	Very frequent (80-99%)
HP:0002960	Autoimmunity	Very frequent (80-99%)
HP:0006335	Persistence of primary teeth	Very frequent (80-99%)
HP:0012735	Cough	Very frequent (80-99%)
HP:0100749	Chest pain	Very frequent (80-99%)
HP:0100820	Glomerulopathy	Very frequent (80-99%)
HP:0000790	Hematuria	Frequent (30-79%)
HP:0000083	Renal insufficiency	Occasional (5-29%)
HP:0000541	Retinal detachment	Occasional (5-29%)
HP:0000979	Purpura	Occasional (5-29%)
HP:0001369	Arthritis	Occasional (5-29%)
HP:0001945	Fever	Occasional (5-29%)
HP:0002829	Arthralgia	Occasional (5-29%)
HP:0003326	Myalgia	Occasional (5-29%)

Identifiers

Disease identifiers

Field	Value
Canonical name	anti-glomerular basement membrane disease
Mondo ID	MONDO:0009303
EFO	EFO:0007290
MeSH	D019867
OMIM	233450
Orphanet	375
DOID	DOID:9808
ICD-11	591736785
NCIT	C84566
SNOMED CT	236432001
UMLS	C0403529
MedGen	140788
GARD	0002551
MedDRA	10018620
NORD	1198
Is cancer (heuristic)	no

Also known as: anti-GBM syndrome · anti-glomerular basement membrane antibody disease · anti-glomerular basement membrane disease · glomerulonephritis - pulmonary haemorrhage · glomerulonephritis - pulmonary hemorrhage · Goodpasture Syndrome · Goodpasture syndrome · pulmonary renal syndrome · rapidly progressive glomerulonephritis with pulmonary haemorrhage · rapidly progressive glomerulonephritis with pulmonary hemorrhage

Disease family

This is a subtype of autoimmune disease. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › immune system disorder › autoimmune disease › anti-glomerular basement membrane disease

Related subtypes (46): autoimmune disease, multisystem, infantile-onset, autoimmune disorder of endocrine system, autoimmune disorder of exocrine system, autoimmune disease of ear, nose and throat, autoimmune disorder of gastrointestinal tract, autoimmune disorder of musculoskeletal system, autoimmune disorder of blood, autoimmune disorder of cardiovascular system, phacolytic glaucoma, Jaccoud syndrome, autoimmune disorder of the nervous system, lupus erythematosus, anti-neutrophil antibody associated vasculitis, cryoglobulinemia, CNS demyelinating autoimmune disease, type III hypersensitivity disease, vitiligo, autoimmune pulmonary alveolar proteinosis, Reynolds syndrome, overlapping connective tissue disease, tempi syndrome, immunoglobulin G4-related sclerosing disease, rheumatic fever, autoerythrocyte sensitization syndrome, autoimmune lymphoproliferative syndrome, secondary neonatal autoimmune disease, euthyroid Graves orbitopathy, Kimura disease, autoimmune thrombocytopenia, autoimmune bullous skin disease, scleroderma, Susac syndrome, undifferentiated connective tissue syndrome, type II hypersensitivity reaction disease, autoimmune urticaria, autoimmune glomerulonephritis, multisystem autoimmune disease due to IKAROS gain of function, autoimmune pulmonary disease due to PD-1 deficiency, non-specific autoimmune supratentorial encephalitis with characteristic antibodies, non-specific autoimmune supratentorial encephalitis without characteristic antibodies, non-specific autoimmune brainstem encephalitis with characteristic antibodies, non-specific autoimmune brainstem encephalitis without characteristic antibodies, non-specific autoimmune cerebellar ataxia with characteristic antibodies, non-specific autoimmune cerebellar ataxia without characteristic antibodies, autoimmune disease with susceptibility to mycobacterium tuberculosis, antiphospholipid syndrome

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

Drugs indicated or in trials for this disease

No drug has an approved disease-direct ChEMBL indication for this disease.

1 drug in clinical trials for this disease (phase 2–3, investigational): efficacy not established — a trial record, not an indication.

Drug	Highest phase
Imlifidase	Phase 2

Clinical trials & evidence

Clinical trials

Clinical trials: 4.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	2
PHASE3	1
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT05679401	PHASE3	TERMINATED	A Study With Imlifidase in Anti-GBM Disease
NCT06607016	PHASE2	ACTIVE_NOT_RECRUITING	A Clinical Trial With KJ103 in Anti-GBM Disease
NCT03157037	PHASE2	COMPLETED	Open-Label Phase II Study to Evaluate the Efficacy and Safety of IdeS in Anti-GBM Disease
NCT07018024	Not specified	RECRUITING	Anti-Glomerular Basement Membrane Disease

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
CYCLOPHOSPHAMIDE ANHYDROUS	4	2
IMLIFIDASE	4	2