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Liver Update: Aggravation of Autoimmune Hepatitis-Primary Biliary Cholangitis Overlap Syndrome by COVID-19

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eMediNexus    22 July 2021

COVID-19 caused  by  severe  acute  respiratory  syndrome  coronavirus  2  infection,  has emerged as a global  pandemic.  Although, initially  considered  a  respiratory  disease,  it  can  causes severe  complications  including gastrointestinal,  neurological,  thromboembolic  and  cardiovascular leading to multiple organ dysfunction and death. The authors of the present study reviewed a case of 57-year-old man with a medical history of hypertension, prediabetes and beta thalassemia minor. The patient developed shortness of breath, and cough and was diagnosed with COVID-19 by RT-PCR. He was quarantined at home for 2 weeks and his respiratory symptoms resolved. However, he further developed fatigue  and  multiple  joint  pain  involving  the  hand,  wrist,  knee  and  shoulder which  progressively  aggravated.  He had no history of nausea, vomiting, abdominal pain, itching, rash, bleeding from the nose or mouth, or blood in stools, and alcohol or drug abuse, blood transfusions or iron supplementation. 

His blood workup showed that hepatitis  A  IgM  antibodies  (HA  Ab-IgM),  hepatitis  B  surface antigen (HBsAg), hepatitis B IgM core antibody (HBcAb-IgM), and hepatitis C antibodies (HC Ab) was negative, HIV was negative. Other laboratory investigations showed  hyperferritinemia,  elevated  liver  enzymes  (AST/ALT/GGT), hypergammaglobulinemia,  anti-smooth  muscle  antibody,  anti-mitochondrial  antibody  and  anti-double-stranded  DNA  antibodies). Ultrasound  of  the  abdomen  revealed  a  normal  sized  liver  with  mild  heterogeneous  parenchyma  and  a slightly  lobulated  contour.  There  was  a  12  mm  cyst  in  the  right  hepatic  lobe  posteriorly.  No evidence of intra-hepatic biliary ductal dilatation was seen and the common bile duct diameter was 6 mm. There was no evidence of gallstones.  Endoscopy showed gastritis and colonoscopy showed internal haemorrhoids. The patient was taking fenofibrate which has been associated with autoimmune hepatitis (AIH).

The patient was diagnosed with autoimmune hepatitis–primary biliary cholangitis (PBC) overlap syndrome stimulated by COVID-19. He was then started on ursodeoxycholic acid and continued to be followed up.

AIH–PBC overlap syndrome is diagnosed when two or three criteria for PBC and AIH are met. AIH is diagnosed when two  out  of  the  following  three  criteria  are  present;  (a)  alanine  aminotransferase  (ALT)  lev ls  >5×ULN,  (b)  serum  immunoglobulin  G  (IgG)  levels  >2×ULN  or  a  positive  test  for  anti-smooth  muscle  antibodies  (ASMA),  and  (c)  liver  biopsy  specimen  showing  moderate  or  severe  periportal  or  periseptal  lymphocytic  piecemeal  necrosis.  PBC  is  diagnosed  when  two  out  of  the  following  three  criteria  are  present:  (a)  alkaline  phosphatase  (AP)  levels  >2×ULN  or  γ-glutamyl  transpeptidase  (GGT)  levels  >5×ULN,  (b)  a  positive  test  for  antimitochondrial  antibodies (AMA), and (c) liver biopsy specimen showing florid bile duct lesions.  This  patient  met  2/3  criteria  for  each  of  AIH  and  PBC, though a liver biopsy was not conducted. Therefore, healthcare professionals should be cautious of uncommon  manifestation  of  COVID-19  so  that  early  diagnostic  and  therapeutic  strategies  can  be  implemented.

Source: Singh B, Kaur P, Maroules M. Autoimmune Hepatitis-Primary Biliary Cholangitis Overlap Syndrome Triggered by COVID-19. Eur J Case Rep Intern Med. 2021 Feb 4;8(3):002264. 

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