IgG4-related mesenteritis
Synonyms: Isolated mesenteric lipodystrophy · Lipomatous mesenteritis · Liposclerotic mesenteritis · Mesenteric lipogranuloma · Mesenteric panniculitis · Sclerosing mesenteritis
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Sclerosing mesenteritis (SM) is a rare pathological disease causing inflammation of the adipose tissue of the small bowel mesentery and is commonly associated with abdominal pain, diarrhea, nausea, weight loss, bloating and loss of appetite. The two subforms include mesenteric panniculitis (where inflammation and fatty necrosis are dominant features) and retractile mesenteritis (where fibrosis and retraction dominate).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | IgG4-related mesenteritis | Isolated mesenteric lipodystrophy · Lipomatous mesenteritis · Liposclerotic mesenteritis · Mesenteric lipogranuloma · Mesenteric panniculitis · Sclerosing mesenteritis |
| fr | Mésentérite associée à IgG4 | Lipodystrophie isolée du mésentère · Lipogranulome mésentérique · Mésentérite lipomateuse · Mésentérite liposclérotique · Mésentérite sclérosante · Panniculite mésentérique |
| de | Mesenteritis, IgG4-assoziierte | Lipodystrophie, isolierte mesenterische · Lipogranulom, mesenterisches · Mesenteritis, lipomatöse · Mesenteritis, liposklerotische · Pannikulitis, mesenterische |
| es | Mesenteritis asociada a IgG4 | Lipodistrofia mesentérica aislada · Lipogranuloma mesentérico · Mesenteritis esclerosante · Mesenteritis lipoesclerótica · Mesenteritis lipomatosa · Paniculitis mesentérica |
| it | Mesenterite IgG4-correlata | Lipodistrofia mesenterica isolata · Lipogranuloma mesenterico · Mesenterite lipomatosa · Mesenterite liposclerotica · Mesenterite sclerosante · Panniculite mesenterica |
| nl | IgG4-gerelateerde mesenteritis | Geïsoleerde mesenterische lipodystrofie · Lipomateuze mesenteritis · Liposclerotische mesenteritis · Mesenterisch lipogranuloom · Mesenterische panniculitis · Scleroserende mesenteritis |
| pt | Mesenterite esclerosante | Mesenterite esclerosante |
| pl | Stwardniające zapalenie krezki jelita cienkiego | Izolowana lipodystrofia krezki jelitowej · Lipomatous mesenteritis · Tłuszczowo-stwardniające zapalenie krezki jelitowej · Zapalenie tkanki tłuszczowej krezki jelitowej · Ziarniniak tłuszczowy krezki jelitowej |
| cs | IgG4-asociovaná mezenteritis | Izolovaná mezenterická lipodystrofie · Lipomatózní mezenteritis · Liposklerotická mezenteritis · Mezenterický lipogranulom · Mezenterická panikulitida · Sklerotizující mezenteritis · Panniculitis peritonealis |
| uk | IgG4-асоційований мезентерит | Ізольована мезентеріальна ліподистрофія · Ліпоматозний мезентерит · Ліпосклеротичний мезентерит · Мезентеріальна ліпогранульома · Мезентеріальний паннікуліт · Склерозуючий мезентерит |
| zh | IgG4相关性肠系膜炎 | 孤立性肠系膜脂肪代谢障碍 |
| tr | IgG4-ilişkili mezenterit | Lipomatöz mezenterit · Mezenterik pannikülit · Sklerozan mezenterit · Mezenterik lipogranülom · Liposklerotik mezenterit · İzole mezenterik lipodistrofi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K65.8 | NTBT | attributed | validated |
| ICD-11 | 4A43.0 | NTBT | index | validated |
| MONDO | 0016544 | E | validated | |
| UMLS | C0267770 | E | validated | |
| GARD | 8169 | E | validated | |
| MedDRA | 10063031 | 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:596448IgG4-related systemic disease
rare systemic and rheumatological diseases - ORPHA:165711Rare abdominal surgical disease
rare abdominal surgical 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/238593
- This record as JSON: /data/d/238593.json