{
  "$schema": "https://ui.shadcn.com/schema/registry-item.json",
  "name": "autoimmune-hepatitis",
  "title": "Autoimmune hepatitis",
  "description": "Autoimmune hepatitis at two scales: the whole liver, where it ends in cirrhosis and portal hypertension, and a liver lobule, with the portal infiltrate, interface hepatitis and periportal injury. Affected and spared structures each say what the disease does there. Models licensed CC BY 4.0 (liver) and CC0 1.0 (lobule).",
  "registryDependencies": [
    "@organui/disease-viewer",
    "@organui/liver",
    "@organui/liver-lobule"
  ],
  "files": [
    {
      "path": "registry/base-nova/autoimmune-hepatitis/autoimmune-hepatitis.tsx",
      "content": "import { diseaseModel, type Disease } from \"@/components/disease-model\"\nimport {\n  DiseaseViewer,\n  type DiseaseViewerProps,\n} from \"@/components/disease-viewer\"\nimport { liverModel } from \"@/components/liver\"\nimport { liverLobuleModel } from \"@/components/liver-lobule\"\n\n// Autoimmune hepatitis on the whole liver, where its course shows, and on a\n// liver lobule, where the injury happens under the microscope.\nexport const autoimmuneHepatitis: Disease = {\n  name: \"Autoimmune hepatitis\",\n  code: \"K75.4\",\n  summary:\n    \"The immune system attacks hepatocytes. Portal inflammation spills into the lobule as interface hepatitis and, untreated, scars the liver towards cirrhosis.\",\n  scales: [\n    {\n      id: \"liver\",\n      label: \"Liver\",\n      model: liverModel,\n      findings: {\n        liver: {\n          label: \"Hepatic parenchyma\",\n          affected: true,\n          description:\n            \"Inflammation is diffuse, so the whole liver is involved; switch to the lobule to see where it starts. The liver can be tender and enlarged, and a severe flare turns the skin yellow.\",\n        },\n        portal: {\n          affected: false,\n          description:\n            \"Portal pressure stays normal until scarring makes blood flow through the liver harder.\",\n        },\n        biliary: {\n          affected: false,\n          description:\n            \"The bile ducts are usually spared. Strictures on cholangiography suggest an overlap with primary sclerosing cholangitis.\",\n        },\n        \"hepatic-veins\": {\n          affected: false,\n          description:\n            \"Not involved. Hepatic vein obstruction (Budd–Chiari syndrome) can look like acute hepatitis, so imaging excludes it.\",\n        },\n      },\n    },\n    {\n      id: \"liver-lobule\",\n      label: \"Lobule\",\n      model: liverLobuleModel,\n      findings: {\n        \"portal-tract-stroma\": {\n          label: \"Portal tracts\",\n          affected: true,\n          description:\n            \"A dense infiltrate of lymphocytes and plasma cells fills the portal tracts, the histological hallmark with interface hepatitis.\",\n        },\n        \"limiting-plate\": {\n          affected: true,\n          description:\n            \"Interface hepatitis: the infiltrate breaks through the limiting plate and destroys the hepatocytes along it, the injury once called piecemeal necrosis.\",\n        },\n        \"periportal-hepatocytes\": {\n          affected: true,\n          description:\n            \"Injured hepatocytes regenerate as rosettes, and lymphocytes can be seen inside them (emperipolesis).\",\n        },\n        \"midzonal-hepatocytes\": {\n          affected: false,\n          description:\n            \"Spared while inflammation stays at the interface. Severe disease bridges across this zone.\",\n        },\n        \"centrilobular-hepatocytes\": {\n          affected: false,\n          description:\n            \"Spared in typical chronic disease. An acute severe onset can show centrilobular necrosis, sometimes as the only finding.\",\n        },\n        \"central-vein\": {\n          affected: false,\n          description:\n            \"Usually spared. Inflammation around it (central perivenulitis) is seen in some acute presentations.\",\n        },\n        \"bile-ducts\": {\n          affected: false,\n          description:\n            \"Usually spared. Duct injury on biopsy suggests an overlap with primary biliary cholangitis or primary sclerosing cholangitis.\",\n        },\n      },\n    },\n  ],\n  stages: [\n    {\n      id: \"active\",\n      label: \"Active\",\n      summary:\n        \"Interface hepatitis with high transaminases and IgG. Most people respond to steroids and azathioprine.\",\n    },\n    {\n      id: \"fibrosis\",\n      label: \"Fibrosis\",\n      summary:\n        \"Repeated injury lays down collagen that bridges portal tracts. Treatment can still reverse it.\",\n      findings: {\n        liver: {\n          liver: {\n            label: \"Hepatic parenchyma\",\n            affected: true,\n            description:\n              \"Fibrous bands bridge the portal tracts. The liver often still looks normal on ultrasound; elastography shows it is stiffer.\",\n          },\n        },\n        \"liver-lobule\": {\n          \"midzonal-hepatocytes\": {\n            affected: true,\n            description:\n              \"Bridging fibrosis and necrosis cross this zone, linking portal tracts to each other and to central veins.\",\n          },\n        },\n      },\n      investigations: [\n        { name: \"ALT\", result: \"Raised\", flag: \"high\" },\n        { name: \"IgG\", result: \"Raised\", flag: \"high\" },\n        { name: \"Liver stiffness\", result: \"Increased\", flag: \"high\" },\n        { name: \"Biopsy\", result: \"Bridging fibrosis\", flag: \"abnormal\" },\n      ],\n    },\n    {\n      id: \"cirrhosis\",\n      label: \"Cirrhosis\",\n      summary:\n        \"Regenerative nodules replace the lobules and portal pressure rises. About a third of adults already have cirrhosis at diagnosis.\",\n      findings: {\n        liver: {\n          liver: {\n            label: \"Hepatic parenchyma\",\n            affected: true,\n            description:\n              \"Shrunken and nodular, with an enlarged caudate lobe. Synthetic function falls: albumin drops and clotting slows.\",\n          },\n          portal: {\n            affected: true,\n            description:\n              \"Blood meets resistance through the scarred liver and portal pressure rises, causing varices, ascites and an enlarged spleen.\",\n          },\n        },\n        \"liver-lobule\": {\n          \"midzonal-hepatocytes\": {\n            affected: true,\n            description:\n              \"Fibrous septa run from portal tracts to central veins, cutting the lobule into regenerative nodules.\",\n          },\n          \"central-vein\": {\n            affected: true,\n            description:\n              \"Septa reach the central vein, and the normal portal-to-central flow is lost.\",\n          },\n          sinusoids: {\n            affected: true,\n            description:\n              \"The sinusoids lose their fenestrae and a basement membrane forms (capillarisation), raising resistance to flow.\",\n          },\n        },\n      },\n      investigations: [\n        { name: \"Platelets\", result: \"Low\", flag: \"low\" },\n        { name: \"Albumin\", result: \"Low\", flag: \"low\" },\n        { name: \"INR\", result: \"Prolonged\", flag: \"high\" },\n        { name: \"Ultrasound\", result: \"Nodular liver\", flag: \"abnormal\" },\n      ],\n    },\n  ],\n  investigations: [\n    { name: \"ALT\", result: \"5–10× upper limit\", flag: \"high\" },\n    { name: \"IgG\", result: \"Raised\", flag: \"high\" },\n    { name: \"ANA, anti-SMA\", result: \"Positive\", flag: \"abnormal\" },\n    { name: \"Biopsy\", result: \"Interface hepatitis\", flag: \"abnormal\" },\n  ],\n}\n\n/** The liver in active disease, the scale and stage the viewer opens on. */\nexport const autoimmuneHepatitisModel = diseaseModel(autoimmuneHepatitis)\n\nexport type AutoimmuneHepatitisProps = Omit<DiseaseViewerProps, \"disease\">\n\nexport function AutoimmuneHepatitis(props: AutoimmuneHepatitisProps) {\n  return <DiseaseViewer disease={autoimmuneHepatitis} {...props} />\n}\n",
      "type": "registry:component"
    }
  ],
  "docs": "Autoimmune hepatitis docs: https://organui.com/docs/components/autoimmune-hepatitis. It reuses the liver model, licensed CC BY 4.0 (https://dbarchive.biosciencedbc.jp/en/bodyparts3d/lic.html), and the liver lobule model, licensed CC0 1.0 (https://creativecommons.org/publicdomain/zero/1.0/); keep the viewer's credit visible. An educational model, not for diagnosis.",
  "categories": [
    "disease",
    "digestive"
  ],
  "type": "registry:component"
}