ORPHA:449395
IgG4-related kidney disease
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare renal disease occurring in the setting of a systemic IgG4 related disease (IgG4-RD). The disorder is characterized by a fibrosing tubulointerstitial nephritis consisting of predominantly IgG4+ plasma cells with/without glomerulonephritis, retroperitoneal fibrosis and hydronephrosis.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | IgG4-related kidney disease | |
| fr | Maladie rénale associée à IgG4 | |
| de | IgG4-assoziierte Nierenkrankheit | Nephropathie, IgG4-assoziierte |
| es | Enfermedad renal relacionada con IgG4 | |
| it | Malattia renale correlata a IgG4 | |
| nl | IgG4-gerelateerde nierziekte | |
| pt | Doença renal IgG4-relacionada | |
| pl | Choroba nerek związana z IgG4 | |
| cs | IgG4 asociované postižení ledvin | |
| uk | Захворювання нирок, повязані з IgG4 | |
| zh | IgG4相关肾病 | |
| tr | IgG4-ilişkili böbrek hastalığı |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | N11.8 | NTBT | attributed | validated |
| ICD-11 | 4A43.0 | NTBT | index | validated |
| MONDO | 0018671 | E | validated | |
| UMLS | C5392056 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:596448IgG4-related systemic disease
rare systemic and rheumatological diseases - ORPHA:567558Non-genetic systemic disease with glomerulopathy as a major feature
rare renal diseases; rare transplant-related disorders
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET_26666884[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
59 annotations · validated 2021-05-19| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0003237 | Increased circulating IgG level | |
| HP:0032300 | Increased circulating IgG4 level | |
| HP:0001970 | Tubulointerstitial nephritis | |
| Frequent (79-30%) | ||
| HP:0001919 | Acute kidney injury | |
| HP:0003493 | Antinuclear antibody positivity | |
| HP:0012622 | Chronic kidney disease | |
| HP:0004431 | Complement deficiency | |
| HP:0005421 | Decreased circulating complement C3 concentration | |
| HP:0045042 | Decreased circulating complement C4 concentration | |
| HP:0003259 | Elevated circulating creatinine concentration | |
| HP:0000790 | Hematuria | |
| HP:0032298 | Increased circulating IgG1 level | |
| HP:0000093 | Proteinuria | |
| HP:0000083 | Renal insufficiency | |
| HP:0032616 | Renal interstitial immunoglobulin deposits | |
| HP:0002923 | Rheumatoid factor positive | |
| Occasional (29-5%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0001679 | Abnormal aortic morphology | |
| HP:0031093 | Abnormal breast morphology | |
| HP:0002088 | Abnormal lung morphology | |
| HP:0045026 | Abnormal mediastinum morphology | |
| HP:0025633 | Abnormal ureter morphology | |
| HP:0100016 | Abnormality of mesentery morphology | |
| HP:0011747 | Abnormality of the anterior pituitary | |
| HP:0012592 | Albuminuria | |
| HP:0012089 | Arteritis | |
| HP:0001082 | Cholecystitis | |
| HP:0011109 | Chronic sinusitis | |
| HP:0000620 | Dacryocystitis | |
| HP:0001410 | Decreased liver function | |
| HP:0031032 | Decreased retinol-binding protein level | |
| HP:0000105 | Enlarged kidney | |
| HP:0012378 | Fatigue | |
| HP:0003212 | Increased circulating IgE concentration | |
| HP:0001880 | Increased total eosinophil count | |
| HP:0011123 | Inflammatory abnormality of the skin | |
| HP:0006515 | Interstitial pneumonitis | |
| HP:0002840 | Lymphadenitis | |
| HP:0002716 | Lymphadenopathy | |
| HP:0031549 | Lymphocytoma cutis | |
| HP:0012578 | Membranous nephropathy | |
| HP:0001287 | Meningitis | |
| HP:0012593 | Nephrotic range proteinuria | |
| HP:0001733 | Pancreatitis | |
| HP:0001701 | Pericarditis | |
| HP:0002102 | Pleuritis | |
| HP:0000024 | Prostatitis | |
| HP:0005200 | Retroperitoneal fibrosis | |
| HP:0030991 | Sclerosing cholangitis | |
| HP:0031281 | Sialadenitis | |
| HP:0100646 | Thyroiditis | |
| HP:0006000 | Ureteral obstruction | |
| HP:0500006 | Urethritis | |
| HP:0100577 | Urinary bladder inflammation | |
| HP:0001824 | Weight loss | |
| Very rare (<4-1%) | ||
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0000126 | Hydronephrosis | |
| HP:0010741 | Pedal edema | |
| Excluded (0%) | ||
| HP:0100586 | Sterile pyuria | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 32488771[PMID]_32234380[PMID]_25594543[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/449395
- This record as JSON: /data/d/449395.json