Case 2
A 39-year-old man of Malagasy origin was seen on an outpatient basis in August 2018 for progressive worsening jaundice with dark urine and discolored stools. The patient had no specific pathological history. He did not report drug addiction, hepatotoxic drugs and viral contagion. The physical examination found frank conjunctival jaundice. The patient is well aware, without hepatomegaly and without ascites. No signs of advanced liver disease and signs of heart failure were detected. Laboratory examinations reported ALT at 1590 (< 45) IU/L, AST at 983 (< 35) IU/L, PAL at 387 (53-128) IU/L, γGT at 960 (< 55) IU/L, total bilirubin at 175 (< 20) µmol/L, conjugated bilirubin at 134 (< 9) µmol/L, albuminemia 33 (40.20-47.60) g/L, serum ferritin at 1700.42 (21.81-274.66) ng/mL, γ-globulin at 26 (8-13.5) g/L and prothrombin level at 60% (70-100) with INR at 1.2 (0.80-1.30). Abdominal ultrasound showed normal liver parenchyma without focal lesions, absence of bile ducts dilation and absence of intrahepatic lithiasis (Image 2a). Hepatitis viral markers A, B, C and E were negative. The anti-tissue antibodies (ANA, anti-SMA, anti-LKM1, anti-SLA, anti-mitochondria type M2, native anti-DNA) were all negative. The serum transferrin saturation, serum ceruloplasmin, serum copper and urinary copper were normal. Liver biopsy was strongly recommended but not available. The aggregate score without treatment calculated from laboratory examinations was 11 points indicating probable AIH (Table 1). Acute autoimmune seronegative hepatitis has been suspected. The chest X-ray was normal (Image 2b). Blood cultures and cytobacteriological examinations of urine were negative. Trial corticosteroid therapy (Prednisone) at an initial dose of 1 mg/kg/day was started, for a period of 8 weeks followed by a reduction. Normalization of transaminases was obtained after 8 weeks of treatment. Corticosteroid therapy was stopped due to side effects. Monthly follow-up has recommended. A progressive increase in transaminases was been demonstrated with respective levels of ALT and AST at 3xULN and 1.7xULN at 2 months then 6.5xULN and 4.2xULN at 3 months, motivating the resumption of corticosteroid therapy. The aggregate score with treatment was 16 points indicating Probable seronegative AIH (Table 1). Azathioprine (Imurel) at a dose of 2 mg/kg/day was introduced one month after resumption of corticosteroid therapy. The combination corticosteroid therapy and azathioprine were well tolerated. A second search for anti-tissue antibodies was carried out in October 2019. The anti SMA, LKM1, anti-mitochondria type M2, native anti-DNA remained negative. ANA and Anti-SLA were positive with a respective rate of 1/160 and 1/80. The final diagnostic score was 21 points indicating a definite type 1 AIH (Table 1). The reassessment in November 2019 and January 2021 showed a good general condition and persistence of the normalization of transaminases, gamma globulins signing a prolonged remission. The combination therapy has been continued and discontinuation of treatment is probably impossible in the absence of a biopsy.