Case 1
A 39-year-old woman of Malagasy origin was seen in an outpatient clinic
on May 24, 2018 for physical asthenia that has been evolving for 4
months. She is neither alcoholic nor smoking. The patient is allergic to
peanuts. She did not report any notion of drug addiction, viral
contagion, or risky sexual behavior. The patient was not taking any
hepatotoxic drug. On clinical examination, she was well conscious,
without asterixis. She had no signs of advanced liver disease or signs
of heart failure. She did not have palpable lymphadenopathy. Laboratory
studies showed hemoglobin level at 14.10 g/dL (normal range, 12-18
g/dL), leukocytes at 4.9 G/L (normal range, 4-10 G/L), platelets at 270
G/L (normal range, 150-400 G/L), alanine aminotransferase (ALT) at 661
(< 45) IU/L, aspartate aminotransferase (AST) at 597
(< 35) IU/L, γ-glutamyl-transpeptidase (γGT) at 120 (7-32)
IU/L, alkaline phosphatases (ALP) at 178 (53-128) IU/L, total bilirubin
at 16.9 (< 18.8) µmol/L, albuminemia at 46.83 (40.20-47.60)
g/l, prothrombin level at 100% (70-100%), International Normalized
Ratio (INR) at 0.949 (0.80-1.30), serum creatinine at 66 (44-97) µmol/L,
C-reactive Protein (CRP) at 14.9 (0-5) mg/L, serum ferritin at 576.70
(4.63-204) ng/mL and γ-globulin at 24.84 (8-13.5) g/L. Abdominal
ultrasound showed normal liver parenchyma with no focal lesions, absence
of bile ducts dilation and absence of intrahepatic stones (Image 1a).
Hepatitis viral markers A, B, C and E were negative. The polymerase
chain reaction (PCR) were negative for cytomegalovirus (CMV), herpes
simplex virus (HSV), Epstein bar virus (EBV). The serum transferrin
saturation, serum ceruloplasmin, serum copper and urinary copper were
normal. Antinuclear antibodies (ANA) were positive at 1/160 speckled in
appearance. Anti-smooth muscle antibodies (anti-SMA) was positive at
1/120. Anti-SLA (Soluble Liver Antigen), anti-LKM1
(Liver-Kidney-Microsome type 1) and native anti-DNA antibodies were
negative. A liver biopsy was recommended but not available. The
aggregate score without treatment calculated from laboratory studies was
16 points indicating definite AIH (Table 1). Type I AIH was retained on
clinical-biological, radiological and immunological criteria. The chest
X-ray was normal (Image 1b). Blood cultures and cyto-bacteriological
examinations of urine were negative. Trial corticosteroid therapy
(Prednisone) at an initial dose of 1 mg/kg/day was started on
06/15/2018, for a period of 8 weeks followed by a reduction.
Azathioprine (Imurel) at a dose of 2 mg/kg/day was introduced 2 months
after the start of corticosteroid therapy (08/19/2018). The combination
corticosteroid therapy and azathioprine were well tolerated. Laboratory
assessment after 8 weeks of treatment showed marked improvement in
cytolysis with normalization of transaminases. The aggregate score with
treatment was 18 points (Table 1). The diagnosis of type I AIH was
maintained. In addition, a second search for anti-tissue antibodies was
carried out on 12/23/2018 reporting positive anti-SMA, confirmed our
diagnosis of type I AIH. At 18 months of treatment (12/12/2019), the
liver function tests remained normal. The combination therapy was
continued.