{
  "id": 17611,
  "label": "immunoglobulin G4-related sclerosing disease",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0017287",
  "properties": {
    "xrefs": [
      "DOID:0080356",
      "GARD:0012521",
      "MEDGEN:473761",
      "MedDRA:10071569",
      "NANDO:1200923",
      "NANDO:1200924",
      "NCIT:C95992",
      "Orphanet:284264",
      "Orphanet:596448",
      "UMLS:C3203653",
      "icd11.foundation:99883782"
    ],
    "synonyms": [
      "IgG4-RD",
      "IgG4-related disease",
      "IgG4-related sclerosing disease",
      "IgG4-related systemic disease",
      "immunoglobulin G4-related sclerosing disease",
      "IgG4-associated disease",
      "IgG4-positive multiorgan lymphoproliferative syndrome",
      "IgG4-related autoimmune disease",
      "IgG4-related systemic sclerosing disease",
      "IgG4-syndrome",
      "hyper-IgG4 disease",
      "multifocal fibrosclerosis",
      "multifocal idiopathic fibrosclerosis",
      "systemic IgG4-related plasmacytic syndrome",
      "systemic IgG4-related sclerosing syndrome"
    ],
    "categories": [
      {
        "ref": "MONDO:0005046",
        "name": "immune system disorder"
      }
    ],
    "definition": "A recently described mass-forming lesion that occurs in the pancreas, submandibular glands, lacrimal glands, lymph nodes, and hepatobiliary tract. It is characterized by the presence of marked tissue sclerosis and infiltration by numerous plasma cells. The plasma cells show immunohistochemical staining for IgG4 and the serum IgG4 levels are often increased."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 14,
  "parents": [
    {
      "id": 8586,
      "label": "autoimmune disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6778
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:417",
          "EFO:0005809",
          "ICD9:279.4",
          "ICD9:279.49",
          "ICD9:720",
          "MEDGEN:2135",
          "MESH:D001327",
          "NCIT:C2889",
          "OBI:1110054",
          "OMIM:109100",
          "SCTID:85828009",
          "UMLS:C0004364"
        ],
        "synonyms": [
          "autoimmune disease",
          "autoimmune disease or disorder",
          "autoimmune disorder",
          "disease, autoimmune",
          "autoimmune hypersensitivity disease",
          "hypersensitivity reaction type II disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "A disorder resulting from loss of function or tissue destruction of an organ or multiple organs, arising from humoral or cellular immune responses of the individual to their own tissue constituents. It may be systemic (e.g., systemic lupus erythematosus), or organ specific, (e.g., thyroiditis)."
      },
      "child_count": 47,
      "reference_id": "MONDO:0007179"
    }
  ],
  "children": [
    {
      "id": 16099,
      "label": "autoimmune pancreatitis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        2997,
        3004,
        6723,
        17611
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0040091",
          "GARD:0010911",
          "MEDGEN:750633",
          "MESH:D000081012",
          "MedDRA:10069002",
          "NANDO:1200925",
          "NANDO:2200943",
          "Orphanet:103919",
          "SCTID:448542008",
          "UMLS:C2609129",
          "icd11.foundation:2057951941"
        ],
        "synonyms": [
          "AIP",
          "lymphoplasmocytic sclerosing pancreatitis"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "Autoimmune pancreatitis (AIP) is a rare pancreatic disease characterized by chronic non-alcoholic pancreatitis that presents with abdominal pain, steatorrhea, obstructive jaundice and responds well to steroid therapy and is seen in two subforms: type 1 AIP which affects elderly males, involves other organs and has increased immunoglobin G4 (IgG4) levels and type 2 AIP which affects both sexes equally but presents at a younger age and has no other organ involvement or increased IgG4 levels."
      },
      "child_count": 8,
      "reference_id": "MONDO:0015175"
    },
    {
      "id": 17039,
      "label": "IgG4-related mesenteritis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17611
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0008169",
          "MEDGEN:75639",
          "Orphanet:238593",
          "SCTID:1092381000119100",
          "UMLS:C0267770",
          "icd11.foundation:1312565896"
        ],
        "synonyms": [
          "Mesenteric Panniculitis",
          "isolated mesenteric lipodystrophy",
          "lipomatous mesenteritis",
          "liposclerotic mesenteritis",
          "mesenteric lipogranuloma",
          "mesenteric panniculitis",
          "sclerosing mesenteritis"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "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)."
      },
      "child_count": 0,
      "reference_id": "MONDO:0016544"
    },
    {
      "id": 18641,
      "label": "IgG4-related sclerosing cholangitis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        2997,
        3004,
        17611,
        18642
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021867",
          "MEDGEN:927778",
          "NANDO:1200928",
          "Orphanet:447764",
          "SCTID:722870008",
          "UMLS:C4302109",
          "icd11.foundation:1676971795"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0018645"
    },
    {
      "id": 18662,
      "label": "IgG4-related kidney disease",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        6948,
        17611
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021882",
          "MEDGEN:1708840",
          "NANDO:1200930",
          "Orphanet:449395",
          "UMLS:C5392056",
          "icd11.foundation:1877692678"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0018671"
    },
    {
      "id": 18663,
      "label": "IgG4-related aortitis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17611
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021883",
          "MEDGEN:1800431",
          "Orphanet:449400",
          "UMLS:C5569008",
          "icd11.foundation:593151236"
        ],
        "synonyms": [
          "IgG4-related periaortitis"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0018672"
    },
    {
      "id": 18664,
      "label": "IgG4-related pachymeningitis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17611,
        20399
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0013256",
          "MEDGEN:1627405",
          "Orphanet:449427",
          "SCTID:762282007",
          "UMLS:C4545992",
          "icd11.foundation:1140264879"
        ],
        "synonyms": [
          "idiopathic hypertrophic pachymeningitis",
          "idiopathic hypertrophic cranial pachymeningitis",
          "idiopathic hypertrophic craniospinal pachymeningitis",
          "idiopathic hypertrophic spinal pachymeningitis"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "Idiopathic hypertrophic pachymeningitis (IHP) is a rare disorder causing inflammation and thickening of the outer layer (dura) of the brain and/or spinal cord. IHP can be widespread or cause tumor-like lesions. Before IHP can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections (syphilis, tuberculosis, and Lyme disease), and autoimmune diseases (rheumatoid arthritis, Sjogrenbs syndrome, Wegenerbs granulomatosis, and IgG4-related disease) must be ruled out. IHP often presents with headache and cranial nerve impairment. Treatment may involve prednisone and/or an immune suppressing drug. This treatment often improves symptoms, however complete recovery is rare. Surgery may be recommended for people with advanced or severe IHP. Some people with IHP have no symptoms and may not need treatment."
      },
      "child_count": 0,
      "reference_id": "MONDO:0018673"
    },
    {
      "id": 18665,
      "label": "IgG4-related submandibular gland disease",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3003,
        17611
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021884",
          "MEDGEN:1830092",
          "NCIT:C82887",
          "Orphanet:449432",
          "SCTID:448131008",
          "UMLS:C5679995",
          "icd11.foundation:588811750"
        ],
        "synonyms": [
          "IgG4-related sialadenitis",
          "Kuttner tumor",
          "Kuttner tumour",
          "Kuttner's tumor",
          "Kuttner's tumour",
          "Küttner tumor",
          "Küttner tumour",
          "chronic sclerosing sialadenitis"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0024623",
            "name": "otorhinolaryngologic disease"
          }
        ],
        "definition": "A chronic fibrotic inflammatory process affecting the salivary gland. Signs and symptoms include firm and painful swelling of the salivary gland, often associated with the presence of salivary gland stones."
      },
      "child_count": 0,
      "reference_id": "MONDO:0018674"
    },
    {
      "id": 18666,
      "label": "IgG4-related ophthalmic disorder",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        7019,
        17611
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021885",
          "MEDGEN:1800432",
          "Orphanet:449563",
          "UMLS:C5569009"
        ],
        "synonyms": [
          "IgG4-related disease of eye",
          "eye IgG4-related disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0002022",
            "name": "disorder of orbital region"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0024458",
            "name": "disorder of visual system"
          }
        ],
        "definition": "A IgG4-related disease that involves the eye."
      },
      "child_count": 2,
      "reference_id": "MONDO:0018675"
    },
    {
      "id": 18667,
      "label": "eosinophilic angiocentric fibrosis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3003,
        17611
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021886",
          "MEDGEN:1814179",
          "Orphanet:449566",
          "UMLS:C5578050",
          "icd11.foundation:57609544"
        ],
        "synonyms": [
          "IgG4-related eosinophilic angiocentric fibrosis",
          "eosinophilic angiocentric fibrosis",
          "EAF",
          "Sinonasal eosinophilic angiocentric fibrosis"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0024623",
            "name": "otorhinolaryngologic disease"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0018676"
    },
    {
      "id": 18670,
      "label": "primary cutaneous plasmacytosis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        6820,
        17611
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021888",
          "MEDGEN:1672491",
          "Orphanet:451602",
          "UMLS:C4736227",
          "icd11.foundation:1669369613"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0018679"
    },
    {
      "id": 18671,
      "label": "cutaneous pseudolymphoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        6820,
        17611
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021889",
          "MEDGEN:81385",
          "NCIT:C62776",
          "Orphanet:451607",
          "SCTID:128862000",
          "UMLS:C0311220",
          "icd11.foundation:1620802923"
        ],
        "synonyms": [
          "lymphadenosis Benigna cutis",
          "lymphocytoma cutis",
          "pseudolymphoma of Spiegler"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "A pseudolymphoma of the skin."
      },
      "child_count": 0,
      "reference_id": "MONDO:0018680"
    },
    {
      "id": 18784,
      "label": "IgG4-related retroperitoneal fibrosis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        17611
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0009568",
          "ICD9:593.4",
          "MEDGEN:20554",
          "MESH:D012185",
          "MedDRA:10038979",
          "NCIT:C26876",
          "NORD:1665",
          "Orphanet:49041",
          "SCTID:197808006",
          "UMLS:C0035357",
          "icd11.foundation:900354709"
        ],
        "synonyms": [
          "Ormond disease",
          "Retroperitoneal Fibrosis",
          "idiopathic retroperitoneal fibrosis",
          "retroperitoneal fibrosis",
          "Ormond's disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "Retroperitoneal fibrosis (RPF) is characterized by the development of a fibrotic mass surrounding retroperitoneal structures, such as aorta, vena cava, ureters and psoas muscle."
      },
      "child_count": 1,
      "reference_id": "MONDO:0018848"
    },
    {
      "id": 18897,
      "label": "IgG4-related mediastinitis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        6815,
        17611
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0008337",
          "MEDGEN:82683",
          "MESH:C536136",
          "MedDRA:10027074",
          "NORD:1146",
          "Orphanet:63999",
          "UMLS:C0264573",
          "icd11.foundation:123840075",
          "icd11.foundation:791747341"
        ],
        "synonyms": [
          "Fibrosing Mediastinitis",
          "fibrosing mediastinitis",
          "mediastinal fibrosis",
          "sclerosing mediastinitis",
          "idiopathic mediastinal fibrosis"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005087",
            "name": "respiratory system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0018978"
    },
    {
      "id": 18908,
      "label": "IgG4-related thyroid disease",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        7262,
        17611
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:14351",
          "GARD:0018866",
          "MEDGEN:509536",
          "MedDRA:10039142",
          "NCIT:C35827",
          "Orphanet:64744",
          "SCTID:89024000",
          "UMLS:C0154162",
          "icd11.foundation:1357889668"
        ],
        "synonyms": [
          "Riedel disease",
          "Riedel fibrosing thyroiditis",
          "Riedel thyroiditis",
          "Riedel's fibrosing thyroiditis"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "Riedel thyroiditis is a fibroinflammatory disorder of the thyroid gland, occurring more frequently in females, characterized a large, hard thyroid mass, and presenting with pressure symptoms (breathing difficulB,ties and dysphagia) or voice hoarseness and aphonia (impingement of recurrent laryngeal nerve). It can often be associated with extracervical fibroinflammatory disorders such as retroperitoneal fibrosis, primary scleroisng cholangitis and autoimmune diseases such as Hashimoto struma, Addison disease, and Biermer disease."
      },
      "child_count": 0,
      "reference_id": "MONDO:0018992"
    }
  ],
  "roots": [
    {
      "id": 8586,
      "label": "autoimmune disease"
    }
  ]
}