Primary biliary cirrhosis: Clinical and laboratory criteria for its diagnosis

Table 1 Classification of the clinical course of primary biliary cirrhosis

Clinical classification
Ref.	Clinical stages
Sasaki et al[44], 1985	Asymptomatic stage	Itching stage	Stage of jaundice	End-stage
Poupon[45], 1991	Preclinical, asymptomatic stage	Clinical stage	End-stage	-

Table 2 The most common extrahepatic diseases associated with primary biliary cirrhosis

Autoimmune hepatitis

Sjogren’s syndrome (Sicca syndrome)[99]

CREST syndrome

Raynaud’s phenomenon

Rheumatoid arthritis

Autoimmune thyroiditis

Scleroderma

Pulmonary fibrosis

Polymyositis

Sarcoidosis[100]

Hemolytic anemia

Celiac disease

Inflammatory bowel disease

Table 3 Levels of bile acids, cholesterol, lecithin, and orthophosphate in the hepatic bile portion and serum of patients with primary biliary cirrhosis

Parameters		Parameter values inthe groups		mean1/mean2
		Control(mean1± SE)	Patients with PBC(mean2± SE)
Bile acids (g/L)	Bile	3.9 ± 0.8	0.65 ± 0.02	6.0
	Blood	0.012 ± 0.008	0.054 ± 0.008	-0.2
Cholesterol (μmol/L)	Bile	0.91 ± 0.06	0.38 ± 0.08	2.4
	Blood	4.2 ± 0.6	11.8 ± 1.6	-0.4
Lecithin1	Bile	2.1 ± 0.3	0.5 ± 0.1	4.6
Orthophosphate1	Bile	1.2 ± 0.3	0.30 ± 0.07	4.0

¹The data given in the table show the average statistical integral intensity of the corresponding ³¹Р-NMR spectral signals in conventional units. Patients with primary biliary cirrhosis (n = 16) and control (n = 14).

Table 4 Lipoproteins of blood in primary biliary cirrhosis patients and control group

Lipoproteins		Parameter values in the groups
		Control (n = 21)	PBC (n = 18)
β-lipoproteins	mean ± SD	50.1 ± 6.0	56.4 ± 16.6
	Range	39.2-59.1	34.8-86.4
α-lipoproteins	mean ± SD	28.4 ± 6.5	23.9 ± 12.2
	Range	15.5-42.7	6.4-46.8
Pre-β-lipoproteins	mean ± SD	20.5 ± 7.5	18.2 ± 8.5
	Range	10.5-34.7	6.3-43.5

Table 5 Classification of morphological stages of primary biliary cirrhosis

Ref.	Morphological stages of PBC
	I	II	III	IV
Popper et al[233], 1970	Cholangitis	Ductular proliferation	Precirrhosis	Cirrhosis
Scheuer[234], 1974	Ductular damage	Ductular proliferation	Scarring	Cirrhosis
Ludwig et al[235], 1978	Portal stage	Periportal stage	Septal stage	Cirrhosis

PBC: Primary biliary cirrhosis.