Open AccessSignificance of extractable nuclear antigens in childhood autoimmune liver disease
Author(s) -
GREGORIO G. V.,
DAVIES E. T.,
MIELIVERGANI G.,
VERGANI D.
Publication year - 1995
Publication title -
clinical & experimental immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.329
H-Index - 135
eISSN - 1365-2249
pISSN - 0009-9104
DOI - 10.1111/j.1365-2249.1995.tb03782.x
Subject(s) - autoimmune hepatitis , anti nuclear antibody , extractable nuclear antigens , immunology , medicine , autoimmune disease , liver disease , overlap syndrome , antibody , mixed connective tissue disease , antigen , hepatitis , autoantibody , pathology , disease
SUMMARY Antinuclear antibody (ANA) is found in connective tissue disorders and in autoimmune disease. While ANA‐positive connective tissue disorders are subdivided according to possession of specific antibodies to extractable nuclear antigens (ENA) (anti‐ribonucleoprotein (anti‐RNP), anti‐Smith (anti‐Sm), anti‐Ro, anti‐La), little is known about the presence and significance of ENA in autoimmune liver disease. To investigate this, we have tested 35 children with autoimmune hepatitis (AIH) (19 ANA and/or smooth muscle antibody‐positive (ANA/SMA +ve); 16 liver kidney microsomal 1‐positive (LKM‐1 +ve)) and 14 with ANA/SMA +ve autoimmune sclerosing cholangitis (ASC), using both double dimension immunodiffusion and ELISAs. Eighty children with non‐autoimmune liver disease (20α 1 ‐antitrypsin deficiency, 20 Wilson's disease. 20 Alagille's syndrome and 20 chronic hepatitis B virus infection) and 20 healthy controls were also tested. ENA were detected in seven (20%) patients with AIH: two ANA‐positive, one SMA‐positive and four LKM‐1‐positive. Three were positive for anti‐Sm, two for anti‐La, one for anti‐Sm/anti‐La and one for anti‐Sm/anti‐La/anti‐Ro. ENA‐positive had more severe liver disease than ENA‐negative patients ( P < 0·03). ENA were not detected in ASC, non‐autoimmune liver diseases and controls. Our results indicate that ENA reactivity, including anti‐Sm and anti‐La, characteristic of systemic lupus erythematosus and Sjögren's syndrome, respectively, are present in some patients with AIH even in the absence of ANA, and may characterize a particularly severe form of the disease.