{
  "id": 18664,
  "label": "IgG4-related pachymeningitis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0018673",
  "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."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "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"
    },
    {
      "id": 20399,
      "label": "inflammatory disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        29380
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "ICD9:799.89",
          "MEDGEN:452939",
          "NCIT:C93210",
          "SCTID:128139000",
          "UMLS:C1290884"
        ],
        "synonyms": [
          "anatomical structure inflammation",
          "inflammation of anatomical structure",
          "inflammatory disease",
          "inflammatory disorder"
        ],
        "definition": "A disease involving a pathogenic inflammatory response in the anatomical structure."
      },
      "child_count": 94,
      "reference_id": "MONDO:0021166"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 17611,
      "label": "immunoglobulin G4-related sclerosing disease"
    },
    {
      "id": 20399,
      "label": "inflammatory disease"
    }
  ]
}