Autoimmune hepatitis type 2 associated with an unexpected and transient presence of primary biliary cirrhosis-specific antimitochondrial antibodies: a case study and review of the literature

Table 2 Characteristics of paediatric patients with antimitochondrial antibody (AMA) positivity in primary biliary cirrhosis (PBC) and other liver or non-liver related diseases

Reference	Age	Sex	Pathology	Antibodies Detected	Histology
Zamfir et al.	6 yrs	M	ITP	AMA, ANA	-
	15 yrs	M	AHA	AMA, ANA	-
Gregorio et al.	12 yrs	F	AIH	AMA	Portoseptal mononuclear cell infiltration and extensive interface hepatitis, with lymphocytic periportal necrosis. Consistent with chronic hepatitis.
Hannam et al.	Birth	F	NNH	AMA
	Birth	M	NNH	AMA	Cholestasis, hepatitis, mild cholangiolitic changes and multi-nucleated giant hepatocytes. Mild portal fibrosis.
Aoki et al.	6 months	M	IL-2Rα deficiency	AMA	Intense mononuclear lymphocyte infiltration of the portal tracts with preservation of the lobular architecture.
Tsuda et al.	11 yrs	M	IPEX	AMA	-
Melegh et al.	6 yrs	F	PBC	AMA	At 6 years: Fibrotic degeneration of the portal tracts, loss of bile ducts, ductal proliferation, periportal hepatocytes separated by mononuclear inflammatory cells.
Dahlan et al.	11 yrs	F	PBC	AMA	At 16 years: Stage II PBC At 21 years: Stage IV PBC (explanted liver)
	15 yrs	F	PBC	AMA, ANA	At 17 years: Stage II PBC

Detailed description of cases is given within the text. ITP: Idiopathic thrombocytopaenic purpura; AHA: Autoimmune haemolytic anaemia; AIH: Autoimmune hepatitis; NNH: Neonatal hepatitis; IPEX: Immunodysregulation polyendocrinopathy enteropathy X-linked syndrome; ANA: Antinuclear antibody.

ISSN: 1471-230X