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.