Anti-glomerular basement membrane disease
Synonyms: Anti-GBM syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Elderly
- Status
- active
A rare, fulminant small vessel vasculitis that affects the capillary beds of the kidneys and lungs and characterized by the presence of anti-glomerular basement membrane (GBM) and, in its full-blown form, anti-alveolar basement membrane (ABM) antibodies. Consequently, it may manifest as a rapidly progressive, isolated glomerulonephritis (anti-GBM nephritis) or as a pulmonary-renal syndrome with severe lung hemorrhage.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Anti-glomerular basement membrane disease | Anti-GBM syndrome |
| fr | Maladie des anticorps anti-membrane basale glomérulaire | Glomérulonéphrite médiate anti-GBM · Maladie à anticorps anti-GBM |
| de | Antikörper vermittelte Krankheit der glomerulären Basalmembran | Anti-Basalmembran-Glomerulonephritis · Anti-GBM-Nephritis · Anti-GBM-Syndrom |
| es | Enfermedad por anticuerpos antimembrana basal glomerular | Síndrome anti-MBG |
| it | Malattia da anticorpi anti membrana basale glomerulare | Sindrome da anti-GBM · Sindrome di Goodpasture |
| nl | Antiglomerulaire-basaalmembraanziekte | Anti-GBM-syndroom |
| pt | Doença de anticorpos anti-membrana basal glomerular | Glomerulonefrite por anticorpos anti-membrana basal glomerular · Síndrome pneumo-renal de Goodpasture |
| pl | Zespół Goodpasture'a | Choroba wywołana przeciwciałami przeciwko błonie podstawnej kłębuszków nerkowych |
| cs | Antirenální glomerulonefritida | Anti-GBM syndrom · Goodpastureův syndrom |
| uk | Хвороба антигломерулярної базальної мембрани | Анти-GBM-синдром · Синдром Гудпасчера |
| zh | 抗肾小球基膜疾病 | Goodpasture综合征 |
| tr | Anti-glomerüler bazal membran hastalığı | Anti-GBM sendromu · Goodpasture sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 233450 | E | validated | |
| ICD-10 | M31.0+ | NTBT | inclusion | validated |
| ICD-10 | N08.5* | NTBT | inclusion | validated |
| ICD-11 | MF85 | E | specific | validated |
| MONDO | 0009303 | E | validated | |
| MeSH | D019867 | E | validated | |
| UMLS | C0403529 | E | validated | |
| GARD | 2551 | E | validated | |
| MedDRA | 10081981 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (3):
- ORPHA:156146Predominantly small-vessel vasculitis
rare systemic and rheumatological diseases; rare circulatory system diseases - ORPHA:264949Secondary interstitial lung disease in childhood and adulthood associated with a systemic disease
rare respiratory diseases; rare transplant-related disorders - ORPHA:567560Systemic vasculitis associated with glomerulopathy
rare renal diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
5 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.06 / 100 000 | China | Value and class | Validated | 15056270[PMID] |
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
| Annual incidence | <1 / 1 000 000 | 0.08 / 100 000 | Europe | Value and class | Validated | [EXPERT]_ORPHANET |
| Annual incidence | 1-9 / 1 000 000 | 0.179 / 100 000 | New Zealand | Value and class | Validated | 22032242[PMID] |
| Point prevalence | 1-9 / 1 000 000 | 0.2 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2018 [OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
19 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001903 | Anemia | |
| HP:0002960 | Autoimmunity | |
| HP:0100749 | Chest pain | |
| HP:0012735 | Cough | |
| HP:0100820 | Glomerulopathy | |
| HP:0002105 | Hemoptysis | |
| HP:0006335 | Persistence of primary teeth | |
| HP:0000093 | Proteinuria | |
| HP:0002113 | Pulmonary infiltrates | |
| HP:0002093 | Respiratory insufficiency | |
| HP:0002633 | Vasculitis | |
| Frequent (79-30%) | ||
| HP:0000790 | Hematuria | |
| Occasional (29-5%) | ||
| HP:0002829 | Arthralgia | |
| HP:0001369 | Arthritis | |
| HP:0001945 | Fever | |
| HP:0003326 | Myalgia | |
| HP:0000979 | Purpura | |
| HP:0000083 | Renal insufficiency | |
| HP:0000541 | Retinal detachment | |
Run the differential on the 11 obligate / very frequent terms
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/375
- This record as JSON: /data/d/375.json