Morning Report

September 9, 2011

Case
HPI: 17Y previously healthy girl with two bouts of debilitating fatigue over the past year ROS: + for low-grade fevers, arthralgias, fatigue PMHx/PSHx: syncopal episode several months ago

Allergies: NKMA
Meds: prednisone 10mg QD FamHx: brother with T1DM, multiple family members with rheumatoid arthritis, grandmother with celiac and thyroid disease, GGM with liver disease and unspecified liver cancer

Details
PE: Normal exam for age
Mild hyperpigmentation in creases of fingers

Labs:
ALT 521, AST 359, alk phos 192, albumin 3.4, Na 133, bili 1.8 EBV, CMV,and hepB negative, normal CBC, CRP 0.9

Imaging:
Abd US showed common bile duct in upper limits of normal, no other notable findings MRCP revealed mild changes that may be consistent with sclerosing cholangitis

Thoughts
GI: fatty liver, hepatic steatosis, non-alcoholic steatohepatitis (NASH), celiac, autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) Genetic: hemochromatosis, Wilsons disease

Rheum: rheumatoid arthritis, Sjogrens syndrome,

Endocrine: thyroid disease, adrenal insufficiency MSK: inborn errors of muscle metabolism, acquired muscle disorders (polymyositis), seizures or heavy exercise Infectious: viral hepatitis B or C, EBV, CMV Other: medications, supplements, alcohol, anorexia

Workup
Further labs:
ANA 1:160, pANCA 1:640, F-actin 72 Elevated anti-thyroid Ab, TTG and gliadin Ab Iron studies normal, ceruloplasmin 30 Saccharmocyces IgG/IgA and mitochondrial Ab negative

Liver biopsy/EGD:
Esophagitis, gastritis, duodenitis, and villous atrophy Broad-based antral mass

Abd CT:
Antral mass (GIST?) Lymphadenopathy of gastrohepatic ligament and small bowel mesentary with ascites Multiple kidney lesions

Autoimmune Hepatitis
3 types:
Type I classic
Affects females > males between ages 10-20 years and 45-70 years Associated with smooth muscle antibodies (f-actin) and/or antinuclear antibodies (ANA)

Type II occurs in younger children

Males = females Liver-kidney microsomal-1 antibody (anti-LKM-1)

Type III seen in adults

Anti-soluble liver antigen (anti-SLA)

Autoimmune Hepatitis
Presentation:
Extremely variable, but most commonly patients present with malaise, weight loss, or anorexia Jaundice +/ Serious symptoms (variceal bleeds) may not present until cirrhosis and portal HTN have occurred Look for family history of other autoimmune diseases

Autoimmune Hepatitis
Diagnosis:
Elevated aminotransferases
Elevated total protein due to hypergammaglobulinemia Positive serum antibody markers (ANA, f-actin)

Usually need liver biopsy

Portal lymphoplasmacytic infiltrates extending into surrounding hepatic lobule Exclude other causes, particularly viral hepatitis E.g. hepatitis C can have elevated ANA or LKM antibodies

Autoimmune Hepatitis
Associations:
PSC (positive pANCA) -> IBD Other autoimmune conditions such as adrenal or thyroid disease, celiac sprue, T1DM, ulcerative colitis or rheumatoid arthritis Hemolytic anemia, idiopathic thrombocytopenic purpura

Autoimmune Hepatitis
Treatment
Immunosuppression with corticosteroids Others include:
Azathioprine Cyclosporine Tacrolimus

Liver transplant for refractory cases

AIH can recur

Complications
Similar to those seen in any progressive liver disease (primary hepatocellular carcinoma)