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Antimitochondrial Antibody-Negative Primary Biliary Cholangitis: A Retrospective Diagnosis

Primary biliary cholangitis (PBC) is an inflammatory cholestatic disease that tends to worsen, leading to hepatic cirrhosis and portal hypertension. We present a case of a middle-aged female who presented with progressively worsening generalized itch; the examination was significant only for urticar...

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Autores principales: Al-Handola, Rami, Chinnappan, Justine, Hussain, Murtaza, Mahgoub, Abdullahi, Bachuwa, Ghassan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10106268/
https://www.ncbi.nlm.nih.gov/pubmed/37073193
http://dx.doi.org/10.7759/cureus.36309
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author Al-Handola, Rami
Chinnappan, Justine
Hussain, Murtaza
Mahgoub, Abdullahi
Bachuwa, Ghassan
author_facet Al-Handola, Rami
Chinnappan, Justine
Hussain, Murtaza
Mahgoub, Abdullahi
Bachuwa, Ghassan
author_sort Al-Handola, Rami
collection PubMed
description Primary biliary cholangitis (PBC) is an inflammatory cholestatic disease that tends to worsen, leading to hepatic cirrhosis and portal hypertension. We present a case of a middle-aged female who presented with progressively worsening generalized itch; the examination was significant only for urticarial rash and facial swelling. Investigation revealed direct hyperbilirubinemia, mildly elevated transaminase, and significant elevation of alkaline phosphatase. A differential was performed with labs including antimitochondrial antibodies (AMA) for PBC, hepatitis panel, anti-smooth muscle antibodies for autoimmune hepatitis, and tissue transglutaminase IgA for celiac disease, all of which were unremarkable. The patient was empirically treated with ursodeoxycholic acid (UDCA). Given the excellent clinical response at the three-week follow-up to treatment despite negative AMA, further testing with anti-sp100 and anti-gp210 was pursued, which returned positive for anti-sp100, confirming the diagnosis of PBC.
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spelling pubmed-101062682023-04-17 Antimitochondrial Antibody-Negative Primary Biliary Cholangitis: A Retrospective Diagnosis Al-Handola, Rami Chinnappan, Justine Hussain, Murtaza Mahgoub, Abdullahi Bachuwa, Ghassan Cureus Internal Medicine Primary biliary cholangitis (PBC) is an inflammatory cholestatic disease that tends to worsen, leading to hepatic cirrhosis and portal hypertension. We present a case of a middle-aged female who presented with progressively worsening generalized itch; the examination was significant only for urticarial rash and facial swelling. Investigation revealed direct hyperbilirubinemia, mildly elevated transaminase, and significant elevation of alkaline phosphatase. A differential was performed with labs including antimitochondrial antibodies (AMA) for PBC, hepatitis panel, anti-smooth muscle antibodies for autoimmune hepatitis, and tissue transglutaminase IgA for celiac disease, all of which were unremarkable. The patient was empirically treated with ursodeoxycholic acid (UDCA). Given the excellent clinical response at the three-week follow-up to treatment despite negative AMA, further testing with anti-sp100 and anti-gp210 was pursued, which returned positive for anti-sp100, confirming the diagnosis of PBC. Cureus 2023-03-17 /pmc/articles/PMC10106268/ /pubmed/37073193 http://dx.doi.org/10.7759/cureus.36309 Text en Copyright © 2023, Al-Handola et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Internal Medicine
Al-Handola, Rami
Chinnappan, Justine
Hussain, Murtaza
Mahgoub, Abdullahi
Bachuwa, Ghassan
Antimitochondrial Antibody-Negative Primary Biliary Cholangitis: A Retrospective Diagnosis
title Antimitochondrial Antibody-Negative Primary Biliary Cholangitis: A Retrospective Diagnosis
title_full Antimitochondrial Antibody-Negative Primary Biliary Cholangitis: A Retrospective Diagnosis
title_fullStr Antimitochondrial Antibody-Negative Primary Biliary Cholangitis: A Retrospective Diagnosis
title_full_unstemmed Antimitochondrial Antibody-Negative Primary Biliary Cholangitis: A Retrospective Diagnosis
title_short Antimitochondrial Antibody-Negative Primary Biliary Cholangitis: A Retrospective Diagnosis
title_sort antimitochondrial antibody-negative primary biliary cholangitis: a retrospective diagnosis
topic Internal Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10106268/
https://www.ncbi.nlm.nih.gov/pubmed/37073193
http://dx.doi.org/10.7759/cureus.36309
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