{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T03:08:52Z","timestamp":1774408132206,"version":"3.50.1"},"reference-count":16,"publisher":"S. Karger AG","issue":"1","license":[{"start":{"date-parts":[[2023,1,6]],"date-time":"2023-01-06T00:00:00Z","timestamp":1672963200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2023,1,6]],"date-time":"2023-01-06T00:00:00Z","timestamp":1672963200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Case Rep Gastroenterol"],"abstract":"<jats:p>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 (&amp;gt;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, &amp;lt;1,600 mg\/dL). The rest of the serological tests, including anti-smooth muscle antibodies (ASMA) and anti-liver\/kidney microsomal antibodies, were negative. Computed tomography of the abdomen and pelvis without contrast showed normal liver parenchyma and no acute intra-abdominal pathology. Histopathology indicated florid duct lesions. The background parenchyma showed no significant steatosis, and inflammatory changes were limited to the portal areas. Periodic acid-Schiff staining revealed intact hepatic parenchyma and architecture. The patient was diagnosed with AMA-neg PBC and responded well to ursodeoxycholic acid therapy. This case highlights the importance of recognizing AMA-neg PBC as a variant of AMA-pos PBC and differentiating between them. Autoimmune cholangitis is a vague and imprecise condition. All patients with AMA-negative PBC should be tested for other PBC-specific autoantibodies. Although the prognosis and bile duct damage and loss are worse in AMA-neg PBC for unknown reasons, treatment remains the same for both. <\/jats:p>","DOI":"10.1159\/000528437","type":"journal-article","created":{"date-parts":[[2023,1,6]],"date-time":"2023-01-06T22:00:38Z","timestamp":1673042438000},"page":"14-20","source":"Crossref","is-referenced-by-count":2,"title":["Distinction between Mitochondrial Antibody-Positive and -Negative Primary Biliary Cholangitis"],"prefix":"10.1159","volume":"17","author":[{"given":"Venkata Vinod Kumar","family":"Matli","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9230-070X","authenticated-orcid":false,"given":"David F","family":"Dies","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sudha","family":"Pandit","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gregory","family":"Wellman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James D.","family":"Morris","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"127","published-online":{"date-parts":[[2023,1,6]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2008-1068269"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/198070"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.14.3368"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1023\/a:1010620122567"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra043898"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1002\/hep.21472"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1111\/liv.12690"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1002\/hep.30145"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1002\/hep.25511"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.2188\/jea.16.30"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1016\/j.gtc.2008.02.006"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1002\/hep.510250507"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.14309\/ajg.0000000000000690"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.3109\/00365521.2015.1132337"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinre.2015.10.006"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1136\/gut.40.4.440"}],"container-title":["Case Reports in Gastroenterology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/karger.com\/crg\/article-pdf\/17\/1\/14\/4124762\/000528437.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/karger.com\/crg\/article-pdf\/17\/1\/14\/4124762\/000528437.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T15:02:28Z","timestamp":1745420548000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000528437"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,1,6]]},"references-count":16,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2023,12,22]]}},"URL":"https:\/\/doi.org\/10.1159\/000528437","archive":["Portico"],"relation":{"is-supplemented-by":[{"id-type":"doi","id":"10.6084\/m9.figshare.21821508","asserted-by":"subject"}]},"ISSN":["1662-0631"],"issn-type":[{"value":"1662-0631","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,1,6]]}}}