{
  "id": 19698,
  "label": "pauci-immune glomerulonephritis with ANCA",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0019988",
  "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."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 19431,
      "label": "Pauci-immune glomerulonephritis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4542
      ],
      "type_id": 0,
      "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."
      },
      "child_count": 2,
      "reference_id": "MONDO:0019641"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 19431,
      "label": "Pauci-immune glomerulonephritis"
    }
  ]
}