{
  "id": 16099,
  "label": "autoimmune pancreatitis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0015175",
  "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."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 2,
  "parents": [
    {
      "id": 2997,
      "label": "autoimmune disorder of endocrine system",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6875,
        8586
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0060005",
          "ICD9:279.49",
          "MEDGEN:575099",
          "SCTID:237822008",
          "UMLS:C0342552"
        ],
        "synonyms": [
          "endocrine system autoimmune disease",
          "endocrine system hypersensitivity reaction type II disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "A hypersensitivity reaction type II disease that involves the endocrine system."
      },
      "child_count": 26,
      "reference_id": "MONDO:0000569"
    },
    {
      "id": 3004,
      "label": "autoimmune disorder of gastrointestinal tract",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6151,
        8586
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0060031"
        ],
        "synonyms": [
          "alimentary part of gastrointestinal system autoimmune disease",
          "alimentary part of gastrointestinal system hypersensitivity reaction type II disease",
          "autoimmune disease of alimentary part of gastrointestinal system"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "A hypersensitivity reaction type II disease that involves the alimentary part of gastrointestinal system."
      },
      "child_count": 14,
      "reference_id": "MONDO:0000588"
    },
    {
      "id": 6723,
      "label": "pancreatitis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4455,
        20399
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:4989",
          "EFO:0000278",
          "MEDGEN:14586",
          "MESH:D010195",
          "NCIT:C3306",
          "SCTID:75694006",
          "UMLS:C0030305"
        ],
        "synonyms": [
          "inflammation of pancreas",
          "pancreas inflammation"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "Inflammation of the pancreas."
      },
      "child_count": 10,
      "reference_id": "MONDO:0004982"
    },
    {
      "id": 17611,
      "label": "immunoglobulin G4-related sclerosing disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        8586
      ],
      "type_id": 0,
      "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."
      },
      "child_count": 14,
      "reference_id": "MONDO:0017287"
    }
  ],
  "children": [
    {
      "id": 17566,
      "label": "autoimmune pancreatitis type 1",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        6741,
        16099
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1000780",
          "GARD:0021076",
          "MEDGEN:927912",
          "Orphanet:280302",
          "PMID:25985088",
          "SCTID:722872000",
          "UMLS:C4302243"
        ],
        "synonyms": [
          "AIP type 1",
          "IgG4-related pancreatitis",
          "autoimmune pancreatitis type 1",
          "lymphoplasmacytic 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": "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."
      },
      "child_count": 0,
      "reference_id": "MONDO:0017227"
    },
    {
      "id": 17567,
      "label": "autoimmune pancreatitis type 2",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16099
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021077",
          "MEDGEN:1679984",
          "Orphanet:280315",
          "UMLS:C5197785"
        ],
        "synonyms": [
          "AIP type 2",
          "duct-centric pancreatitis"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "Type 2 autoimmune pancreatitis is a form of autoimmune pancreatitis (see this term) affecting both sexes and having a younger age of onset (<60 years) and presenting with abdominal pain, steatorrhea and obstructive jaundice."
      },
      "child_count": 0,
      "reference_id": "MONDO:0017228"
    }
  ],
  "roots": [
    {
      "id": 2997,
      "label": "autoimmune disorder of endocrine system"
    },
    {
      "id": 3004,
      "label": "autoimmune disorder of gastrointestinal tract"
    },
    {
      "id": 6723,
      "label": "pancreatitis"
    },
    {
      "id": 17611,
      "label": "immunoglobulin G4-related sclerosing disease"
    }
  ]
}