Immunoglobulin A nephropathy
Synonyms: Berger disease · IgA nephropathy
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare glomerular disease, histologically characterized by glomerular mesangial deposits of IgA, often accompanied by IgG and complement C3 as well as mesangioproliferative changes, clinically mostly manifesting as oligosymptomatic glomerulonephritis, possibly infection-triggered macrohematuria and a variable course ranging from spontaneous remission to slow or rarely rapid progression to kidney failure.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Immunoglobulin A nephropathy | Berger disease · IgA nephropathy |
| fr | Néphropathie à immunoglobuline A | Maladie de Berger · Néphropathie à IgA |
| de | Immunglobulin A-Nephropathie | Berger-Krankheit · IgA-Nephropathie |
| es | Nefropatía por inmunoglobulina A | Enfermedad de Berger · Nefropatía por IgA |
| it | Nefropatia da immunoglobulina A | Malattia di Berger · Nefropatia da IgA |
| nl | Immunoglobuline A-nefropathie | IgA-nefropathie · Ziekte van Berger |
| pt | Nefropatia por imunoglobulina A | Doença Berger · Nefropatia de IgA |
| pl | Choroba Bergera | Nefropatia IgA |
| cs | Imunoglobulin A nefropatie | = en |
| uk | Імуноглобулінова нефропатія А | Хвороба Бергера · Нефропатія IgA |
| zh | 欧洲非罕见病：贝格尔疾病 | IgA肾病 |
| tr | AVRUPA'DA NADİR OLMAYAN: Berger hastalığı | IgA nefropati |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | N02 | NTBT | index | validated |
| ICD-11 | MF8Y | NTBT | index | validated |
| MONDO | 0005342 | E | validated | |
| MONDO | 5342 | E | validated | |
| UMLS | C0017661 | E | validated | |
| MedDRA | 10021263 | 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 (1):
- ORPHA:93548Glomerular disease
rare renal diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
17 annotations · validated 2024-01-19| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0001919 | Acute kidney injury | |
| HP:0000794 | IgA deposition in the glomerulus | |
| HP:0012587 | Macroscopic hematuria | |
| HP:0002907 | Microscopic hematuria | |
| HP:0012595 | Mild proteinuria | |
| Occasional (29-5%) | ||
| HP:0001541 | Ascites | |
| HP:0002608 | Celiac disease | |
| HP:0001394 | Cirrhosis | |
| HP:0030830 | Crackles | |
| HP:0000282 | Facial edema | |
| HP:0031504 | Foamy urine | |
| HP:0033316 | Glomerular crescent formation | |
| HP:0000099 | Glomerulonephritis | |
| HP:0000822 | Hypertension | |
| HP:0003261 | Increased circulating IgA level | |
| HP:0000083 | Renal insufficiency | |
| Very rare (<4-1%) | ||
| HP:0012593 | Nephrotic range proteinuria | |
Annotation source: 34676611[PMID]_31371641[PMID]_30855802[PMID]_32400945[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/34145
- This record as JSON: /data/d/34145.json