Introduction
Definition :
Fibrosis & nodular regeneration resulting from hepatocellular injury
Epidemiology :
Cirrhosis & CLD accounted for > 25.000 death & 375.000 hospitalization
Etiology
¡ Alcohol
¡ Virus Hepatitis : chronic HBV,HCV, HDV infection
¡ Autoimmune hepatitis (female, ?IgG, +ANA, +ASMA)
¡ Cogenital : hemocromatosis, wilson’s disease, -?1antitrypsin deficiency, cogenital hepatic fibrosis
¡ Metabolic disease : NASH/NAFLD
¡ Biliary tract disease : PBC/PSC, secondary biliary cirrhosis (calculus, neoplasm,post-op stricture, biliary atresia)
¡ Vascular disease : Budd-Chiari syndrome, R-side heart failure, constrictive pericarditis
¡ Cryptogenic : may reflect terminal progression of NAFLD or some may be non/missed diagnosed AIH
Pathopysiology
• A late stage of progressive hepatic fibrosis characterized by distortion of hepatic architecture & formation of regenerative nodules
• Form of chronic hepatitis
• Inflammatory infiltration of hepatic portal
• Necrosis of hepatocytes (parenchyme or portal areas)
Clinical Manifestation
? Subclinical or present at progressive liver dysfunction (jaundice, coagulopathy, encephalopathy) and/or portal hypertension (ascites, varices)
? Liver : enlarged, palpable, firm, nodular >> shrunken & nodular
? Sign of liver failure :
Jaundice, spider angiomata (marker of chronicity), palmar erytema, duputyren’s contracture, white nail lines (muehrcke’s line) & proximal nail beds (terry’s nail), parotid & lacrimal glands, gynecomasti, asterixis, enchephalopathy, fetor hepaticus
? Sign of portal hipertension
Splenomegaly, ascites, dilated superficial abdominal vein (caput medusae), epigastric “cruveilhier-Baumgarten” venous hum
Laboratory Studies
? bilirubin, PT, albumin, ± aminotransferases, alkaline phosphatase
? Na
? Anemia (bone marrowsupression, hypersplenism, iron and/or folate deficiencies), neutropenia (hypersplenism), thrombocytopenia (hypersplenism, thrombopoietin production)
Diagnostic Studies
? Liver biopsy (percutaneus/transjugular)
? Abdominal U/S doppler
? Hepatic serologies (HBsAg, AntiHCV)
? Autoimmune hepatitis study (IgG, ANA, ASMA)
? Fe and Cu studies
? a1-AT phenotype
? AMA (PBC), p-ANCA (PSC)
? Echocardiogram (right sided heart failure)
? AFP
Complication
? Portal hypertension : ascites, varices, hepatohydrotorax
? Coagulopathy
? Hepatic encephalopathy
? Hepatorenal syndrome
? Hepatopulmonary syndrome
? Liver failure
? Infection
? Hepatocellular carcinoma
Prognosis
Correlates with Child-Pugh class
| 1 | 2 | 3 | |
| ascites | none | Easy control | poor |
| encephalopathy | none | Grade I/II | Grade III/IV |
| Bilirubin | < 2 | 2-3 | >3 |
| Albumin | > 3.5 | 2.8-3.5 | <2.8 |
| PT | <4 | 4-6 | >6 |
| A | B | C | |
| Total point | 5-6 | 7-9 | 10-15 |
| 1-y survival | 100% | 81% | 45% |
| 2-y survival | 85% | 57% | 35% |