27 Nov 2020

11/18/20: Autoimmune Hepatitis

PGY2 Selena Zhou presented a case of a 73 year old female with history of HTN, hyperlipidemia, amiodarone-induced hyperthyroidism and paroxysmal Afib who presents with 9 days of abdominal pain along with worsening yellowing of the skin.  The abdominal pain is primarily RUQ with occasional radiation to the LUQ, associated with abdominal distention, fatigue and leg swelling.  The pain is not associated with food, nausea or vomiting.  Pain is improved with defecation/flatulence.  Of note, the patient was recently diagnosed with amiodarone induced hyperthyroidism 2 months prior (had taken amiodarone 200mg BID x1 month but stopped for the last 2 months since diagnosis).  The patient was born in El Salvador, immigrated to US in 1986.  She used to work as a babysitter, housekeeper and in the US government in the office that takes care of old people.  Denies any history of alcohol, tobacco or drug use.

Vitals: T 36.3, HR 69, RR 16 BP 101/50, O2 sat 100% on RA

Physical Exam significant for well developed, Spanish speaking female, sclera icteric bilaterally, normal cardiac and pulmonary exam, +ascites, 1+ edema to bilateral legs and +jaundiced skin.  No asterixis appreciated.

CBC: WBC 7.5> Hb 14.6/Hct 44.3 <Plt 213 (MCV 101.8, RDW 16.7%)

CMP:

Na 136/K 3.4/Cl 102/HCO3 20/BUN 21/Cr 1.46 <Glucose 144  (Baseline Cr: 0.9)

ALP 171>T.protein 6.2/Albumin 2.8/AST 595/ALT 295/Tbili 13.1/Dbili 9.3

Ca 7.8, Mg 1.7, Phos 3.7

Coags: PT 21, INR 1.82

Liver workup started with the following significant results:

  • BCx, UCx, UA, COVID-19 negative
  • APAP level normal
  • Tumor markers (AFP, CEA, CA 19-9): wnl
  • Hepatitis panel negative
  • Copper level and ceruloplasmin wnl
  • ANA positive, 1:1280, nuclear, centromere
  • Anti-Actin, anti-LKM, anti-mitochondrial, ANCA all negative
  • IgA: 881 mg/dL (normal: 70-400), IgG: 1495 mg/dL (normal: 700-1600), IgM: 178 mg/dL (normal: 40 – 230)
  • Abdominal US with mildly heterogenous liver, moderate volume ascites

Trans-Jugular liver biopsy done showed features consistent with autoimmune hepatitis.

Treatment: Patient was started on prednisone 60mg qday, fluconazole 200mg qday, Bactrim DS qday and pantoprazole 40mg qday.  Upon follow up in liver clinic 1 month later, his liver enzymes have downtrended and prednisone was decreased to 30mg qday and started on azathioprine 30mg qday.

Teaching points:

  1. Diagnostic criteria for AIH (point system): AutoAb (ANA or SMA), IgG level, liver histology and absence of viral hepatitis. Points >/= 6 indicates autoimmune hepatitis and >/= 7 is definite AIH.
  2. Treatment: first line is prednisone to get to remission with maintenance of prednisone and azathioprine. Second line is MMF.
  3. In patients who receive liver transplant, there is a risk of recurrent: 7-42%

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