Seronegative autoimmune hepatitis in children: a single-center experience

Abstract

Background: Seronegative autoimmune hepatitis (AIH) is a diagnostic challenge with unclear prognosis. This study describes the features and outcomes of seronegative AIH in children. Patients and methods: Patients under 18 years of age, who had been diagnosed with AIH between April 2014 and April 2020, were retrospectively evaluated. Seronegative AIH was identified by the absence of the three conventional non-organ-specific autoantibodies (anti-nuclear antibody [ANA], anti-smooth muscle antibody [ASMA], and anti-liver kidney microsomal [anti-LKM] type 1 antibody), alongside the characteristic AIH liver histopathology and a positive response to immunosuppressive therapy in the absence of other liver diseases. Results: The study included 54 patients with AIH. 15 (27.77%) were seronegative at the time of diagnosis. 13 of the 15 seronegative patients presented with acute hepatitis or acute liver failure (ALF). Mean follow-up duration was 27.48 months in seronegative patients. Two seronegative patients had lymphocytopenia on admission, and, although the liver disease improved after corticosteroid treatment, they developed aplastic anemia (AA). Other seronegative patients responded well to immunosuppressive therapy. Conclusions: Patients with seronegative AIH present frequently with acute hepatitis or ALF. AIH diagnosis can be confirmed by observing the effects of corticosteroid therapy in seronegative patients with characteristic AIH liver histopathological features. However, the presence of lymphocytopenia in seronegative patients is a sign of bone marrow failure.