Autoimmune Hepatitis

Autoimmune Hepatitis
(Hepatology 2002;36:479-97)
DEFINITION:
  • Autoimmune Hepatitis (AIH) is an unresolving inflammation of liver, unknown cause:
    • Characterized by interface hepatitis (histology), autoantibodies & hypergammaglobulinemia (serum)
  • Types of AIH:

    Features

    Type 1 (Classic)

    Type 2

    Type 3 (Least Established Form)

    Predominant age

    Adult (some children)

    Child (girls > boys)

    All ages ? if really a variant of Type 1 AIH

    Auto Antibodies

    ASMA*

    LKM1ˆ

    anti-SLA

    ANA, p-ANCA

    Liver cytosol 1

    ANA

    Anti-Actin

    Organ-specific Ab

    ± (thyroid)

    ± (thyroid, parietal cells)

    ?

    Autoantigen

    Unknown

    P450 2D6 (CYP2D6)

    tRNP

    HLA phenotype

    B8, DR3, DR4

    DR3, DR7

    DR3

    Fulminant onset

    Possible (but rare)

    Possible

    Unknown

    Concurrent disease

    40% (see below)

    32% (see below)

    60% (see below)

    Gamma globulin ↑

    Yes

    Slight

    Moderate

    Progress Cirrhosis

    35%

    80%

    Probably

    Steroid responsive

    Yes

    Yes

    Yes

    * anti-smooth muscle antibody,ˆ anti-liver/kidney microsome type 1,anti-soluble liver antigen

  • Overlap or Variant Syndrome:
    • Autoimmune Hepatitis & PBC or PSC; Has high titers of AMA and cholestasis; See also Liver- PBC (Chapter 4.23)
EPIDEMIOLOGY:
  • F:M = 5:1, can occur at any age but typically before 4th decade; Represents <10% of all chronic hepatitis in the U.S. but 6% of transplants
  • Concurrent immunologic diseases can be present in patients, most common: autoimmune thyroiditis, ulcerative colitis, Graves, synovitis, RA

Others:

Celiac sprueCoomb’s + hemolytic anemiaCyroglobulinemiaDermatitis herpetiformis

Erythema nodosumFocal myositisGingivitisGlomerulonephritis

ITPInsulin DMIritisLichen planus

Myasthenia gravisNeutropeniaPericarditisPeripheral neuropathy

Pernicious anemiaPleuritisPyoderma gangrenosum

Sjogren’sSLEUrticariaVitiligo

ETIOLOGIES:

▪

Differential diagnosis

Diagnostic tests (See each corresponding diagnosis/chapter for details)

Wilson’s

Copper studies: ↓ ceruloplasmin, ↓ serum copper, ↑ urinary copper

PSC

Cholangiography: Focal biliary strictures; Liver biopsy: fibrous obliterate changes

PBC

Auto Antibodies: AMA >1:40; Liver biopsy: florid duct lesion, ↑ hepatic copper

Autoimmune cholangitis

Liver biopsy: cholangitis, ductopenia

Chronic Hepatitis C

Viral labs: anti-HCV/RIBA positive, HCV RNA present

Drug-induced Hepatitis

Clinical history: exposure to minocycline, nitrofurantoin, propylthiouracil, isoniazide, methyldopa

Hemochromatosis

Genetics: C282Y, H63D mutations; Labs: ↑ Iron/Ferritin; Liver biopsy: iron overload, HII >1.9

α1-AT

Phenotype: ZZ or MZ; Liver biopsy: hepatic inclusions, PAS +

NASH

Clinical: obesity, DM, hyperlipidemia; Liver biopsy: macrosteatosis; U/S: hyperechogenicity

PATHOPHYSIOLOGY:
Aug 24, 2016 | Posted by in GASTROENTEROLOGY | Comments Off on Autoimmune Hepatitis

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