This document describes a case of a 48-year-old woman with autoimmune hepatitis type 1 who was admitted with abdominal pain, vomiting, and loose stools. She had been diagnosed with autoimmune hepatitis in July 2017 and had multiple admissions since then. On physical examination, she had jaundice and hepatosplenomegaly. Her labs showed features of hepatitis and anemia. She was treated with IV fluids, blood transfusions, and continued steroids. Her condition improved and she was discharged on steroid monotherapy.

1. Autoimmune Hepatits
Dr. Tanweer Baksh
Case and Theory

2.  48 year old, Comoron lady, k/c of Autoimmune Hepatitis type 1, diagnosed in July
2017; ( High ALT, Direct Hyperbilirubinemia, high serum globulin and igG, ANA + ,
ASMA +, SA and B +).
 She has multiple admissions due to jaundice and fatigability since last July 2017 >
September 2017 > May 2018.
 She was started on Prednisolone and Azathioprine since she was diagnosed, July
2017.
History

3.  June 29: ER with Abdominal pain, Vomiting and loose stools.
She vomited 3 times since the morning after drinking pineapple juice. And had
crampy abdominal pain, with a h/o passing watery stool twice.
 No h/o hematemesis or Melena.
 Yellowish discoloration of eyes since last 3 days and complains of generalized
weakness.
 No h/o respiratory or urinary symptoms.
 No other systemic complains.
 Azathioprine 40mg, Prednisolone 50mg bid, Esmoprazole 40mg, Cholecalciferol
10,000IU, Calcium Carbonate 600mg bid.
 No other co-morbidities.
 No family hx of Liver Cirrhosis in either Parents or siblings.

4. Physical Examination
 Vitals: RR – 19 BP – 121/75 SpO2 – 99%
 General: Alert & oriented, not in acute distress, No flapping tremors
 Eye: Pallor + & Icterus +
 No pedal edema.
 Skin – some purpuric patches seen.
 Abdomen – Soft, non-tender, not distended, Hepatosplenomegaly +
 Lungs: Bilateral equal air entry, no added sounds
 CVS : s1 s2 +

5. Labs & Investigation
WBC 7.84x10mcL
Neutro 59.90%
Hgb 4.80 gm/dL
Hct 15.60%
MCV 102.60 fL
RDW 18%
Platelets 129x10/mcL
Sodium 136 mmol/L
Potassium 2.7 mmol/L
Creatinine 88 umol/L
Glucose 7.2 mmol/L

6. Total Protien 87
Albumin 14.2 gm/L
Total Bilirubin 312 umol/L
Bilirubin Direct 23.2 umol/L
ALT 53
Alkaline Phosphatase 203 IU/L
Amylase 46 IU/L
Ammonia 74 umol/L
CRP 33.6 mg/L
Labs & Investigation

7. Urine colour Dark Yellow
Urine Bilirubin 2+
pH 6.5
PT 17 secs
PTT 54.9 secs
INR 1.38
LDH 376 IU/L
DAT Positive
Labs & Investigation

8.  CXR: Within Normal Limits.
 US Abdomen (28th June) :
Cirrhotic Liver, Splenomegaly, Minimal Ascites, small Cortical cyst in the
left Kidney, Retroverted Uterus.
Labs & Investigation

9. Management
 IV fluids was started (kcl 60mEq+Nacl 0.9% 1.5L)
 Blood transfusion 4.8gm/dL >> 5.7gm/dL >> 7.10gm/dL
 Azathioprine was stopped (due to leucopenia and decreased Platelets)
 Steroids was continued (Prednisolone 60mg).

10. Clinical Course
 She was improving clinically with no new complains.
 No complains of fever/vomiting/melena.
 Hgb level improved from 4.8gm/dL >> 5.7gm/dL >> 7.10gm/dL >> 7.30g/dL.
 Potassium level improved from 2.70 mmol/L >> 3.50 mmol/L
 Patient was discharged after 5 days of admission on Steroid monotherapy and was
asked to follow up in 2 weeks.
Diagnosis
 Autoimmune Hepatitis with Autoimmune Hemolytic anemia.
(Inderect Hyperbilirubinemia, features of hemolysis on Blood film & Positive Coombs
test.)

11. Autoimmune Hepatitis

12. Autoimmune Hepatitis
 Autoimmune hepatitis is a chronic disorder characterized by continuing
hepatocellular necrosis and inflammation, which can progress to cirrhosis and liver
failure.
 Prevalence : 0.1-1.2 cases per 100,000 individuals in the US.
 Women are affected more often than men (70-80% of patients are women).
 Bimodal age distribution, with a first peak of incidence at age
10-20 years and
45-70 years.

13. Pathophysiology
 Involves the combination of genetic predisposition and environmental triggers.
 Genetic - HLA haplotypes B8, B14, DR3, DR4, and Dw3.
 Environmental triggers – Drugs and Several viruses including Rubella, EBV, and
Hepatitis A, B, and C.
 Display of human leukocyte antigen (HLA) class II on the surface of hepatocytes
facilitates the exposure of normal liver cell membrane constituents to antigen-
presenting cells (APCs).
 APC’s attract the T helper cells which produces macrophages and antibodies.

14.  Type 1 autoimmune hepatitis – Type 1, or classic autoimmune hepatitis, is
associated with marked hyperglobulinemia, lupoid features and has circulating
antibodies to nuclei (ANA) and/or smooth muscle (ASMA);
 Type 2 autoimmune hepatitis – Often seen in children and is defined by the
presence of antibodies to liver/kidneymicrosomes (ALKM-1) and/or to a liver
cytosol antigen (ALC-1).

15.  widely varies. Some may be asymptomatic or have nonspecific symptoms (eg,
fatigue, anorexia, weight loss, behavioral changes, amenorrhea).
 Epistaxis, bleeding gums, and bruises with minimal trauma are frequent complaints.
 Other Common findings include :
- Hepatomegaly
- Jaundice
- Splenomegaly
- Spider angiomata
- Ascites
- Encephalopathy
Clinical Feature

16. Diagnosis
Laboratory findings include:
 Elevated serum aminotransferase levels.
 Elevated IgG’s.
 Mild to moderately elevated serum bilirubin and alkaline phosphatases.
 Positive ANA, SMAs, or liver-kidney microsomal type 1 (LKM-1) or anti–liver cytosol 1
(anti-LC1) antibodies
 Hypoalbuminemia and prolongation of prothrombin time
Other hematologic abnormalities may include the following:
 Mild leukopenia
 Normochromic anemia
 Coombs-positive hemolytic anemia
 Thrombocytopenia
 Elevated ESR
 Eosinophilia (uncommon)

17. Management
 Initial treatment is with glucocorticoid monotherapy rather than combination
therapy with a glucocorticoid and azathioprine.
 Corticosteroid is usually started at a dose of 60 mg, followed by a taper.
 Patients with mild disease (eg, asymptomatic patients with aminotransferase levels
<10 times the upper limit of normal), lower dose prednisone monotherapy (20 mg
per day) is an alternative.
 Patients with an increased risk for side effects from glucocorticoids, using a lower-
dose glucocorticoid (eg, prednisone 30 mg per day) for monotherapy or in
combination with azathioprine (50 mg per day) for initial treatment is an
alternative.

18. Thank You