Postinfantile Giant Cell Hepatitis Secondary to Rheumatoid Arthritis

Abstract

Postinfantile giant cell hepatitis (PIGCH), also known as syncytial giant cell hepatitis, continues to be a poorly defined and rare disease presentation in the adult population. Although a common finding in neonates, there is limited literature on the disease process, causes, and treatment success of PIGCH in adults. A strong association between autoimmune disorders and PIGCH, considerably so in the case of autoimmune hepatitis, has been established. However, there have been limited to no reports of PIGCH secondary to rheumatoid arthritis. Our clinical case aims to bring forth a vignette of PIGCH to spotlight this ill-defined disease in the adult population and highlight some of the proposed causes, treatments, and laboratory markers.

Keywords: PIGCH; adult giant cell hepatitis; giant cell hepatitis; post-infantile giant cell hepatitis; syncytial giant cell hepatitis.

Figure 1.

Right liver lobe needle core biopsy with chronic hepatitis, giant cell change, and marked fibrosis: (A) Marked portal inflammation with interface activity (black arrows) and rare plasma cells (inset on the lower left) (H&E, ×11). The inset photograph is of high magnification. (B) Multiple foci inflammation (black arrows) in lobules consistent with lobular hepatitis (H&E, ×7). (C) Numerous markedly enlarged periportal hepatocytes (black arrows) with abundant pink and pale pink cytoplasms with numerous nuclei (ranging up to approximately 25 nuclei) consistent with giant cell change (H&E, ×9). (D) Portal tract shows marked fibrosis with periportal fibrosis (black arrows) and bridging fibrosis (white arrow) (Masson trichrome, ×4). H&E, hematoxylin and eosin.