{"orpha":238593,"name":"IgG4-related mesenteritis","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Isolated mesenteric lipodystrophy","Lipomatous mesenteritis","Liposclerotic mesenteritis","Mesenteric lipogranuloma","Mesenteric panniculitis","Sclerosing mesenteritis"],"definition":"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).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-238593/","orphanet_url":"https://www.orpha.net/en/disease/detail/238593","names":{"fr":{"name":"Mésentérite associée à IgG4","synonyms":["Lipodystrophie isolée du mésentère","Lipogranulome mésentérique","Mésentérite lipomateuse","Mésentérite liposclérotique","Mésentérite sclérosante","Panniculite mésentérique"],"definition":"La mésentérite sclérosante est une maladie rare causant l'inflammation de tissus adipeux du mésentère. Douleur abdominale, diarrhée, nausée, perte de poids, ballonnement et perte d'appétit sont couramment associés à cette maladie. Les 2 sous-formes de la mésentérite sclérosante incluent la panniculite mésentérique (lorsque l'inflammation et la nécrose graisseuse sont les caractéristiques dominantes) et la mésentérite rétractile (lorsque la fibrose et la rétraction dominent)."},"de":{"name":"Mesenteritis, IgG4-assoziierte","synonyms":["Lipodystrophie, isolierte mesenterische","Lipogranulom, mesenterisches","Mesenteritis, lipomatöse","Mesenteritis, liposklerotische","Pannikulitis, mesenterische"],"definition":"Die Sklerosierende Mesenteritis ist eine entzündliche Erkrankung des Fettgewebes im Dünndarm-Mesenterium. Symptome sind Leibschmerzen, Diarrhoe, Nausea, Gewichtsverlust, Auftreibung des Abdomens und Appetitlosigkeit. Zwei Formen werden unterschieden: Mesenterische Pannikulitis (es dominieren Entzündung und Fettgewebsnekrose) und Retraktile Mesenteritis (es dominieren Fibrose und Retraktion)."},"es":{"name":"Mesenteritis asociada a IgG4","synonyms":["Lipodistrofia mesentérica aislada","Lipogranuloma mesentérico","Mesenteritis esclerosante","Mesenteritis lipoesclerótica","Mesenteritis lipomatosa","Paniculitis mesentérica"],"definition":"La mesenteritis esclerosante (SM) es una enfermedad patológica poco frecuente que causa inflamación del tejido adiposo del mesenterio del intestino delgado y está asociada normalmente con dolor abdominal, diarrea, náuseas, pérdida de peso, hinchazón y pérdida de apetito. Las dos subformas son la paniculitis mesentérica (en la que la inflamación y la necrosis grasa son las características dominantes) y la mesenteritis retráctil (en la que dominan la fibrosis y la retracción)."},"it":{"name":"Mesenterite IgG4-correlata","synonyms":["Lipodistrofia mesenterica isolata","Lipogranuloma mesenterico","Mesenterite lipomatosa","Mesenterite liposclerotica","Mesenterite sclerosante","Panniculite mesenterica"],"definition":"La mesenterite sclerosante (SM) è una malattia rara dovuta all'infiammazione del tessuto adiposo del mesentere dell'intestino tenue, che si associa di solito a dolore addominale, diarrea, nausea, perdita di peso, distensione dell'addome e perdita dell'appetito. I due quadri clinici con cui si estrinseca la malattia sono la panniculite mesenterica (nella quale dominano l'infiammazione e la necrosi grassa) e la mesenterite retrattile (nella quale prevalgono la fibrosi e la retrazione)."},"nl":{"name":"IgG4-gerelateerde mesenteritis","synonyms":["Geïsoleerde mesenterische lipodystrofie","Lipomateuze mesenteritis","Liposclerotische mesenteritis","Mesenterisch lipogranuloom","Mesenterische panniculitis","Scleroserende mesenteritis"],"definition":"Een zeldzame pathologische aandoening die inflammatie van vetweefsel van mesenterium van dunne darm veroorzaakt, en doorgaans geassocieerd is met abdominale pijn, diarree, misselijkheid, gewichtsverlies, opgeblazen gevoel, en verlies van eetlust. De twee subtypes van scleroserende mesenteritis zijn mesenterische panniculitis (waarbij inflammatie en necrose van vetweefsel dominante kenmerken zijn) en retractiele mesenteritis (waarbij fibrose en retractie domineren)."},"pt":{"name":"Mesenterite esclerosante","synonyms":["Mesenterite esclerosante"]},"pl":{"name":"Stwardniające zapalenie krezki jelita cienkiego","synonyms":["Izolowana lipodystrofia krezki jelitowej","Lipomatous mesenteritis","Tłuszczowo-stwardniające zapalenie krezki jelitowej","Zapalenie tkanki tłuszczowej krezki jelitowej","Ziarniniak tłuszczowy krezki jelitowej"]},"cs":{"name":"IgG4-asociovaná mezenteritis","synonyms":["Izolovaná mezenterická lipodystrofie","Lipomatózní mezenteritis","Liposklerotická mezenteritis","Mezenterický lipogranulom","Mezenterická panikulitida","Sklerotizující mezenteritis","Panniculitis peritonealis"]},"uk":{"name":"IgG4-асоційований мезентерит","synonyms":["Ізольована мезентеріальна ліподистрофія","Ліпоматозний мезентерит","Ліпосклеротичний мезентерит","Мезентеріальна ліпогранульома","Мезентеріальний паннікуліт","Склерозуючий мезентерит"],"definition":"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)."},"zh":{"name":"IgG4相关性肠系膜炎","synonyms":["孤立性肠系膜脂肪代谢障碍"],"definition":"硬化性肠系膜炎（SM）是一种罕见的引起小肠系膜脂肪组织炎症的病理性疾病，通常伴有腹痛、腹泻、恶心、体重减轻、腹胀和食欲减退。包括两种亚型：肠系膜脂膜炎（主要特征为炎症和脂肪坏死）和退缩性肠系膜炎（主要特征为纤维化和退缩）。"},"tr":{"name":"IgG4-ilişkili mezenterit","synonyms":["Lipomatöz mezenterit","Mezenterik pannikülit","Sklerozan mezenterit","Mezenterik lipogranülom","Liposklerotik mezenterit","İzole mezenterik lipodistrofi"]}},"xrefs":[{"source":"ICD-10","code":"K65.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"4A43.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1312565896"},{"source":"MONDO","code":"0016544","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0267770","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"8169","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10063031","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":165711,"name":"Rare abdominal surgical disease"},"parents":[{"orpha":596448,"name":"IgG4-related systemic disease","classifications":[196]},{"orpha":165711,"name":"Rare abdominal surgical disease","classifications":[182]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}