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.