Table 1

Major forms of hepatic abnormalities recorded in patients with autoimmune rheumatic diseases

Abnormality	Definition/comment
Chronic active hepatitis	The morphological hallmark of CAH is piecemeal necrosis. This is defined as the destruction of liver cells at the interface between parenchyma and connective tissue together with a predominantly mononuclear inflammatory infiltrate. Clusters of lymphocytes and macrophages encircle or invaginate hepatocytes with a spreading wave of necrosis. As the liver parenchyma is destroyed sheets of connective tissue are laid down, which initially also contain an inflammatory infiltrate resulting in a “maple leaf” configuration to the portal tract.
Chronic persistent hepatitis	CPH is characterised by chronic inflammatory infiltration of portal tracts with preserved lobular architecture and little or no portal fibrosis, although the tracts are expanded. There is no significant piecemeal necrosis. CPH disease is mostly stationary and in many instances resolves spontaneously, far more frequently than CAH.
Hepatomegaly	Defined clinically as a palpable liver and usually but not always confirmed by ultrasound or CT scanning or at post mortem.
Lupoid hepatitis	A combination of CAH with LE cell phenomena. May be distinguished from SLE by the absence of antibodies to double stranded DNA.
Nodular regenerative hyperplasia of the liver	Characterised by diffuse nodularity of the liver with little or no fibrosis, and has been found in association with autoimmune disease, after drug treatment and a variety of haematological disorders.
Primary biliary cirrhosis	PBC is an autoimmune inflammatory disorder associated with a high serum titre of antimitchondrial antibodies. Histological appearances divided into four stages:
	(I) florid bile-duct lesions with lymphoid aggregates
	(II) ductular proliferation
	(III) scarring (septal fibrosis and bridging)
	(IV) cirrhosis.
Primary sclerosing cholangitis	A chronic inflammatory disorder that ultimately results in fibrosis and obliteration of the bile ducts. It is strongly associated with inflammatory bowel disease and runs an unpredictable course.