MIAMI — Patients diagnosed with antimitochondrial antibody (AMA)-negative primary biliary cholangitis (PBC) have clinical and histopathological findings consistent with PBC but without positive AMA.

A new study in Liver International pointed out, “The natural history of patients with anti-mitochondrial antibody (AMA)-negative Primary Biliary Cholangitis (PBC) cirrhosis has not been well defined, with prior studies showing discordant results. Furthermore, most studies of AMA-negative PBC have limited numbers of patients with cirrhosis and liver-related outcomes.”1

Researchers from the Bruce W Carter VAMC and the University of Miami Miller School of Medicine, both in Miami, sought to determine the association of AMA-negative PBC and liver-related death or the development of decompensation and hepatocellular carcinoma (HCC), in a large cohort of predominantly male VHA patients with PBC cirrhosis.

The cohort of 521 patients with PBC cirrhosis included 65 with the AMA-negative variety and involved total follow-up of 2504.3 person-years (PY) from cirrhosis diagnosis. The researchers pointed out that AMA-negative PBC patients were younger and more likely to be Black but had similar rates of ursodeoxycholic acid (UDCA) response.

Results indicated that AMA-negative PBC cirrhosis was associated with similar unadjusted rates of liver-related death (4.6 vs. 5.9 per 100 PY, P = 0.44), overall death (7.7 vs. 9.6 per 100 PY, P = 0.31), decompensation (7.3 vs. 5.1 per 100 PY, P = 0.12) and HCC (0.6 vs. 1.0 per 100 PY, P = 0.63) to AMA-positive PBC. The researchers noted that, after adjusting for confounders, AMA-negative PBC cirrhosis was associated with similar rates of liver-related death (sub-Hazard Ratio [sHR] 1.27, 95% CI 0.71-2.28, P = 0.42, death [sHR] 1.24, 95% CI 0.81-1.90, P = 0.32), decompensation (sHR 1.05, 95% CI 0.56-1.98, P = 0.87) and HCC (sHR 0.48, 95% CI 0.11-2.10, P = 0.33) to AMA-positive patients.

“In a cohort of predominantly male patients, AMA-negative PBC cirrhosis was associated with similar rates of overall or liver-related death, HCC or decompensation compared with AMA-positive disease,” the authors concluded.

 

  1. John BV, Dahman B, Deng Y, Khakoo NS, Taddei TH, Kaplan DE, Levy C. Rates of decompensation, hepatocellular carcinoma and mortality in AMA-negative primary biliary cholangitis cirrhosis. Liver Int. 2022 Feb;42(2):384-393. doi: 10.1111/liv.15079. Epub 2021 Oct 12. PMID: 34614294; PMCID: PMC8810619.