Features and outcome of autoimmune hepatitis type 2 presenting with isolated positivity for anti-liver cytosol antibody.
OBJECTIVE
Autoimmune hepatitis (AIH) type 2 is identified by the presence in the serum of anti-liver/kidney microsome type 1 autoantibody. Anti-liver cytosol autoantibody has been reported in children with autoimmune liver disorders mostly in association with anti-liver/kidney microsome reactivity. However, its role as a sole marker of AIH type 2 is debated. We describe here a series of 18 children and adolescents (15 girls, 3 boys) with AIH with serum anti-liver cytosol type 1 (aLC1) as the only autoimmune marker.


METHODS
A retrospective review was conducted from 3 pediatric hepatology units of all children with an autoimmune liver disease associated with aLC1 as found by immunofluorescence and/or immunodiffusion or immunoblotting.


RESULTS
Age at first symptoms ranged from 11 months to 14 years; 12 children presented with acute hepatitis, 1 with progressive jaundice, and 5 were asymptomatic. Anti-liver/kidney microsome, antimitochondria, and anti-actin autoantibodies were not detected. Signs of cirrhosis were present in 11 children. Immunosuppressive treatment was effective in all except 2 children who had subfulminant hepatic failure and who required liver transplantation. Sixteen patients (14 with their native liver) currently are alive; 14 patients still are on immunosuppressive therapy after 1 to 22 years. According to the international scoring system for the diagnosis of AIH, 16 patients corresponded to a definite diagnosis and 2 corresponded to a probable diagnosis.


CONCLUSIONS
The presence of aLC1 in children with acute or chronic liver disease of unknown origin strongly supports a diagnosis of AIH and is an indication for early immunosuppressive therapy.
Bridoux-Henno
Laure
L
Service d'Hépatologie Pédiatrique, Hôpital de Bicêtre, Le Kremlin Bicêtre, France.
Maggiore
Giuseppe
G
Johanet
Catherine
C
Fabre
Monique
M
Vajro
Pietro
P
Dommergues
Jean-Paul
JP
Reinert
Philippe
P
Bernard
Olivier
O
D000293:Adolescent; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D005455:Fluorescent Antibody Technique; D005938:Glucocorticoids / Q000627:therapeutic use; D019693:Hepatitis, Autoimmune / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007223:Infant; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D011516:Prothrombin; D012189:Retrospective Studies
D001323:Autoantibodies; D015415:Biomarkers; D005938:Glucocorticoids; C094307:anti-liver cytosolic protein 1; D011516:Prothrombin; D011241:Prednisone