Anticentromere antibody in primary biliary cirrhosis.

Abstract

Of recent interest has been the description of a serum antibody reacting with the centromere portion of chromosomes in tissue culture cells and found in patients with scleroderma, especially of the CREST type.7'-0 We have looked for anticentromere antibody in serum from 1 10 consecutive patients presenting with PBC, 20 of whom had scleroderma, and from 80 patients with other forms of liver disease, including the autoimmune variety of chronic active hepatitis (CAH). Materials and methodsThe series comprised 110 consecutive patients with biopsy-proved PBC, of whom 97 (88.2%) were women and 13 (11.8%) were men. Antimitochondrial antibody was present in 94 (85 5%) patients, antinuclear antibody in 11 (10%), and anti-

Anticentromere antibodies in primary biliary cirrhosis

“…Bernstein et a1 (15) have recently reported the detection of anticentromere antibody in the serum of 10 of 110 (9.1%) pqtients with primary biliary cirrhosis. All 10 patients had one or more features of the CREST syndrome.…”

CRST Syndrome (Calcinosis Cutis, Raynaud's Phenomenon, Sclerodactyly, and Telangiectasia) Associated with Autoimmune Hepatitis.

“…In approximately 20% of the cases, CRSTsyndrome is accompanied by Sjogren's syndrome, and association with PBC is often seen as well (4,13). Observed at a high frequency, but yet fairly specific to CRST syndrome, anticentromere antibodies have been reported in 10%to 29%of the PBCcases (4,(13)(14)(15)(16).…”

Liver Cirrhosis Due to Autoimmune Hepatitis Combined with Systemic Sclerosis

“…However, AIH may be due, in part, to dysfunction of both cellular and humoral immunity related to SSc, as anti-centromere antibody has been detected in up to 13% of patients with AIH. [11][12][13] There have been only two reports in the literature regarding investigations related to anti-centromere antibodies in patients with AIH, which indicated the presence of these antibodies at a low frequency of 3% in cases of AIH. 14,15 In the Japanese population, the frequency of the discrete speckled pattern of FANA (closely associated with anti-centromere antibody) is increased in AIH patients with anti-centromere antibody, supporting the close association between the AIH and SSc.…”