Distinction between Mitochondrial Antibody-Positive and -Negative Primary Biliary Cholangitis

Autor: Venkata Vinod Kumar Matli, David F Dies, Sudha Pandit, Gregory Wellman, James D. Morris
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: Case Reports in Gastroenterology, Vol 17, Iss 1, Pp 14-20 (2023)
Druh dokumentu: article
ISSN: 1662-0631
DOI: 10.1159/000528437
Popis: Antimitochondrial antibody-positive primary biliary cholangitis (AMA-pos PBC) is an autoimmune disorder in which monoclonal antibodies are produced against epitopes in the mitochondrial membranes of biliary epithelial cells, resulting in progressive nonsuppurative biliary cholangitis. Up to 5% of patients lack these autoantibodies, termed antimitochondrial antibody-negative (AMA-neg) PBC. Although a somewhat new variant of AMA-pos PBC, it is not an overlapping syndrome. Few studies to date have described this phenomenon. An 87-year-old woman was referred to our clinic with elevated serum alkaline phosphatase (714 U/L). She reported fatigue but no other symptoms. A physical examination revealed a benign lesion and bilateral lower extremity swelling secondary to lymphedema. The serological profile was significant for a high antinuclear antibody titer (>1:2,560) with a centromere pattern and negative for antimitochondrial antibody (AMA). The hepatitis panel was negative for viruses A, B, and C. Her serum immunoglobulin G level was 871 mg/dL (normal,
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