{
  "id": 19431,
  "label": "Pauci-immune glomerulonephritis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0019641",
  "properties": {
    "xrefs": [
      "GARD:0019175",
      "MEDGEN:575439",
      "Orphanet:93126",
      "UMLS:C0343196",
      "icd11.foundation:1359757645"
    ],
    "categories": [
      {
        "ref": "MONDO:0002118",
        "name": "urinary system disorder"
      }
    ],
    "definition": "Pauci-immune glomerulonephritis (GN) is one of the most frequent causes of rapidly progressive GN (RPGN). It is characterized clinically by renal manifestations of RPGN (hematuria, hypertension) leading to renal failure within days or weeks, and may be associated with manifestations of systemic vasculitis (arthralgia, fever, seizures, mono neuritis and lung involvement). Pauci-immune GN is histologically characterized by focal necrotizing and crescentic GN, with mild or absent glomerular staining for immunoglobulin and complement by fluorescence microscopy, which may manifest either as part of a systemic small vessel vasculitis (including microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis), or rarely as part of renal-limited vasculitis (RLV, idiopathic crescentic GN). Immunologic classification is based on the presence or absence of circulating anti-neutrophil cytoplasmic antibodies (ANCAs), namely pauci-immune-GN with ANCA and pauci-immune GN without ANCA."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 2,
  "parents": [
    {
      "id": 4542,
      "label": "glomerulonephritis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        3410,
        19482
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:2921",
          "GARD:0006516",
          "HP:0000099",
          "ICD9:583.9",
          "MEDGEN:6616",
          "MESH:D005921",
          "NCIT:C26784",
          "SCTID:36171008",
          "UMLS:C0017658"
        ],
        "synonyms": [
          "glomerular nephritis",
          "glomerulonephritis",
          "glomerulonephritis (disease)",
          "nephritis of renal glomerulus",
          "renal glomerulus nephritis",
          "bright's disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          }
        ],
        "definition": "A renal disorder characterized by damage in the glomeruli. It may be acute or chronic, focal or diffuse, and it may lead to renal failure. Causes include autoimmune disorders, infections, diabetes, and malignancies."
      },
      "child_count": 40,
      "reference_id": "MONDO:0002462"
    }
  ],
  "children": [
    {
      "id": 19698,
      "label": "pauci-immune glomerulonephritis with ANCA",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        19431
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0019379",
          "MEDGEN:1843351",
          "Orphanet:97563",
          "UMLS:C5680254"
        ],
        "synonyms": [
          "pauci-immune glomerulonephritis with antineutrophil cytoplasmic antibody"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          }
        ],
        "definition": "Pauci-immune glomerulonephritis (GN) with antineutrophil cytoplasmic antibodies (ANCA) is a form of rapidly progressive GN comprising about 90% of pauci-immune glomerulonephritis, and associated with the presence of circulating ANCA (mostly directed against proteinase-3 (PR3) and myeloperoxidase (MPO)). Patients usually present with hematuria and rapidly declining renal function, often leading to dialysis within weeks without treatment. Cutaneous, pulmonary, musculoskeletal and nervous involvement may be observed in case of systemic disease, and the correlation between ANCA titer and disease activity has been demonstrated."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019988"
    },
    {
      "id": 19699,
      "label": "pauci-immune glomerulonephritis without ANCA",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        19431
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0019380",
          "MEDGEN:1842476",
          "Orphanet:97564",
          "UMLS:C5680253"
        ],
        "synonyms": [
          "antineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritis",
          "pauci-immune glomerulonephritis without antineutrophil cytoplasmic antibody"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          }
        ],
        "definition": "Pauci-immune glomerulonephritis (GN) without antineutrophilic cytoplasmic antibodies (ANCA) is a form of rapidly progressive glomerulonephritis comprising 10-43% of pauci-immune glomerulonephritis and characterized by the absence of ANCA. In comparison with pauci-immune GN with ANCA, patients lacking ANCA may be younger at onset of the disease and have a shorter interval from onset of the disease to diagnosis. They have fewer extra renal manifestations (e.g. involvement of lung, eye, ear, nose and throat), fewer constitutional symptoms (e.g. fever, weight loss, muscle pain and arthralgia) and a high prevalence of nephrotic syndrome and chronic renal lesions. Their prognosis is generally poorer."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019989"
    }
  ],
  "roots": [
    {
      "id": 4542,
      "label": "glomerulonephritis"
    }
  ]
}