Autoimmune pancreatitis type 1
Synonyms: AIP type 1 · IgG4-related pancreatitis · Lymphoplasmacytic sclerosing pancreatitis
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Type 1 autoimmune pancreatitis is a form of autoimmune pancreatitis seen in elderly males (>60 years) and presenting with abdominal pain, steatorrhea, obstructive jaundice and other organ (bile duct, kidneys and retroperitoneum) involvement. It is thought to be due to an immunoglobulin G4 (IgG4)-associated systemic disease.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Autoimmune pancreatitis type 1 | AIP type 1 · IgG4-related pancreatitis · Lymphoplasmacytic sclerosing pancreatitis |
| fr | Pancréatite auto-immune type 1 | AIP type 1 · Pancréatite associée à IgG4 · Pancréatite sclérosante lymphoplasmocytaire |
| de | Pankreatitis, autoimmune, Typ 1 | AIP Typ 1 · Autoimmun-Pankreatitis Typ 1 · Pankreatitis, IgG4-assoziierte · Pankreatitis, lymphoplasmozytäre sklerosierende |
| es | Pancreatitis autoinmune tipo 1 | AIP tipo 1 · Pancreatitis asociada a IgG4 · Pancreatitis linfoplasmocitaria esclerosante |
| it | Pancreatite autoimmune, tipo 1 | AIP, tipo 1 · Pancreatite IgG4-correlata · Pancreatite sclerosante linfoplasmocitaria |
| nl | Auto-immune pancreatitis type 1 | AIP type 1 · IgG4-gerelateerde pancreatitis · Lymfoplasmacytaire scleroserende pancreatitis |
| pt | Pancreatite autoimune 1 | AIP tipo 1 · Pancreatite esclerosante linfoplasmocítica |
| pl | Autoimmunologiczne zapalenie trzustki typu 1 | AIP typu 1 · Limfoplazmatyczne stwardniające zapalenie trzustki |
| cs | Autoimunitní pankreatitida, typ 1 | AIP typ 1 · IgG4 asociovaná pankreatitida · Lymfoplazmocytární sklerozující pankreatitida |
| uk | Аутоімунний панкреатит, тип 1 | AIP тип 1 · Панкреатит, повязаний з IgG4 · Лімфоплазмоцитарний склерозуючий панкреатит |
| zh | 自身免疫性胰腺炎1型 | 淋巴浆硬化性胰腺炎 |
| tr | Otoimmün pankreatit tip 1 | AIP tip 1 · IgG4-ilişkili pankreatit · Lenfoplazmasitik sklerozan pankreatit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K86.1 | NTBT | attributed | validated |
| ICD-11 | DC33 | NTBT | index | validated |
| MONDO | 0017227 | E | validated | |
| UMLS | C4302243 | 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 (2):
- ORPHA:103919Autoimmune pancreatitis
rare gastroenterological diseases - ORPHA:596448IgG4-related systemic disease
rare systemic and rheumatological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/280302
- This record as JSON: /data/d/280302.json