|Year : 2013 | Volume
| Issue : 2 | Page : 138-139
A case of coexistent primary biliary cirrhosis and celiac disease
Moushumi Lodh1, Ashif Ali Ahmed2, Debakanta Pradhan3
1 Department of Biochemistry, The Mission Hospital, Durgapur, West Bengal, India
2 Department of Gastroenterology, The Mission Hospital, Durgapur, West Bengal, India
3 Department of Microbiology, The Mission Hospital, Durgapur, West Bengal, India
|Date of Web Publication||4-Jan-2014|
Department of Biochemistry, The Mission Hospital, Immon Kalyan Sarani, Sector 2C, Bidhannagar, Durgapur - 713 212, West Bengal
Source of Support: None, Conflict of Interest: None
Both celiac disease and primary biliary cirrhosis share several features, including a higher prevalence in females, autoimmune comorbidities and specific autoantibodies. We report such a case in a male, with symptoms of chronic diarrhea and marked weight loss. A high level of suspicion of autoimmune disease, autoantibody assays and biopsies helped in a quick diagnosis and management. At 3-month follow-up, the patient had gained 3 kg. Reciprocal screening for both diseases is recommended, as an early diagnosis with the appropriate treatment can improve the outcome of these patients.
Keywords: Antimitochondrial antibody, celiac disease, primary biliary cirrhosis, tissue transglutaminase
|How to cite this article:|
Lodh M, Ahmed AA, Pradhan D. A case of coexistent primary biliary cirrhosis and celiac disease. Indian J Allergy Asthma Immunol 2013;27:138-9
|How to cite this URL:|
Lodh M, Ahmed AA, Pradhan D. A case of coexistent primary biliary cirrhosis and celiac disease. Indian J Allergy Asthma Immunol [serial online] 2013 [cited 2020 Jan 29];27:138-9. Available from: http://www.ijaai.in/text.asp?2013/27/2/138/124397
| Case Report|| |
Autoimmune disorders are a matter of great concern to the medical fraternity. We recently diagnosed a case of primary biliary cirrhosis (PBC) and celiac disease (CD), in a 51-year-old male, admitted to the hospital with the complaints of chronic diarrhea, bloating and borborygmi. He complained of marked weight loss since 2 years. His medical history was unremarkable other than persistently abnormal liver function since the last one year. His stool was negative for occult blood, ova parasites or culture.
Other laboratory investigations were as under: Hemoglobin was 10.4 g/dl (13-17), iron, Total iron-binding capacity, ferritin, cerruloplasmin, transferrin, antifreeze peptides, Immunoglobulins (Ig) A, G and M were within reference range and blood picture was that of pancytopenia; red blood cell 3.7 million/cumm (4.5-5.5), white blood cell (WBC) 2000/cumm (4000-10,000) and platelet, 1.26 lakhs/cumm (1.5-4).
All liver enzymes were elevated with alkaline phosphatase 489 U/L (30-279), gamma glutamyltransferase 343 U/L (1-94). Serology for hepatitis B and C were negative. Anti-nuclear antibody was positive at 1:160 titer (normal <1:40), with granular filamentous pattern. Anti-mitochondrial antibody (AMA) was positive at 1:160 titer (normal <1:40) [Figure 1] confirmed by immunochromatography [Figure 2]. Anti-smooth muscle antibody was negative and. Tissue transglutaminase (tTg) was high at 57.09 U/ml (0.50-20 U/ml). Ig A endomysial antibody was positive. Ultrasonography of abdomen revealed dilated portal vein, enlarged spleen and ascites. Gastroduodenoscopy revealed esophageal varices with duodenal ulcer. Liver biopsy revealed ill-defined granulomas centered on bile ducts. Lymphocytes, histiocytes and plasma cells were present in the portal tracts. Duodenal endoscopic biopsy revealed mild villous blunting, intraepithelial lymphocytes to be increased >40/100 expected count, lamina propria having chronic inflammatory cell infiltrate. All these features were suggestive of CD modified Marsh Grade 3a. 
|Figure 1: Immunofluorescence study showing HEp2 cells with granular cytoplasmic filamentous staining around the nucleus, suggestive of anti-mitochondrial antibody pattern|
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|Figure 2: Liver profile line immunoassay showing presence of immunoglobulin G autoantibodies to M2 (pyruvate dehydrogenase complex) and borderline presence of cytosolic liver antigen type 1 (LC-1)|
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Patient was managed symptomatically, endoscopic variceal ligation and patient at discharge put on gluten free diet and ursodeoxycholic acid (UDCA). At a 3-month follow-up visit, the patient had gained 3 kg; and his hemoglobin, platelet count and WBC count were increased. Stool frequency and consistency became normal.
| Discussion|| |
CD has been reported in up to 6% of patients of PBC  and in 4.37% patients of chronic liver disease undergoing endotherapy.  PBC has a female predominance with an 8:1 female-to-male ratio.  The association of CD and PBC presumably represents a shared susceptibility of biliary and small bowel epithelium to attack by autoimmune mechanisms. The role of infection in the course of CD and PBC, have been discussed by Abenavoli et al. 
In CD, immunologically active molecules generated from the cross-linking between tTg and food/bacterial antigens reach the liver through the portal circulation owing to the increased intestinal permeability. A molecular mimicry between bacterial antigens and the pyruvate dehydrogenase E2 component, recognized by AMA, may have a role in PBC pathogenesis. An aberrant intestinal T lymphocyte homing to the liver may contribute to trigger immune hepatic damage. 
Villous atrophy and malabsorption that responded to gluten withdrawal, a positive AMA, cholestatic biochemistry and liver biopsy consistent with PBC were the diagnostic criteria here. The IgG deamidated gliadin peptide and IgA tTg tests used in combination is an excellent screening test for suspected cases of CD. Our reporting this case, in a male and at the age of 51 years, assumes greater importance due to the fact that a coexistent untreated CD (present in 7% of patients with PBC, particularly in Caucasians) may cause a failure to normalize with UDCA. 
There is no causative association between the two diseases nor that the activity of one influences the course of the other,  but presumably a shared susceptibility of biliary and small bowel epithelium to attack by autoimmune mechanisms. Given Logan et al. proposal, is now considered established, screening for possible PBC in patients with CD using AMA tests is recommended; similarly, screening for CD in patients with PBC could be readily performed with antigliadin antibody testing or duodenal biopsy when the presence of varices is being assessed. Furthermore since malabsorption, weight loss and osteoporosis  are clinical manifestations common to both entities, screening for CD in PBC and vice-versa, for AMA are advisable in adult CD patients for early diagnosis and institution of specific therapy.
| References|| |
|1.||Ghadir M, Iranikhah A, Jandaghi M, Joukar F, Rahimabadi MS, Ghanaei FM. Unexplained infertility as primary presentation of celiac disease, a case report and literature review. Iran J Reprod Med 2011;9:135-40. |
|2.||Hohenester S, Oude-Elferink RP, Beuers U. Primary biliary cirrhosis. Semin Immunopathol 2009;31:283-307. |
|3.||Kochhar R, Dutta U, Miglani A, Bhagat S, Poornachandra KS, Vaiphei K, et al. Celiac disease suspected at endoscopy in patients with chronic liver disease. Indian J Gastroenterol 2011;30:166-9. |
|4.||Kumagi T, Heathcote EJ. Primary biliary cirrhosis. Orphanet J Rare Dis 2008;3:1. |
|5.||Abenavoli L, Arena V, Giancotti F, Vecchio FM, Abenavoli S. Celiac disease, primary biliary cirrhosis and Helicobacter pylori infection: One link for three diseases. Int J Immunopathol Pharmacol 2010;23:1261-5. |
|6.||Volta U, Caio G, Tovoli F, De Giorgio R. Gut-liver axis: An immune link between celiac disease and primary biliary cirrhosis. Expert Rev Gastroenterol Hepatol 2013;7:253-61. |
|7.||Kingham JG, Parker DR. The association between primary biliary cirrhosis and coeliac disease: A study of relative prevalences. Gut 1998;42:120-2. |
|8.||Logan RF, Ferguson A, Finlayson ND, Weir DG. Primary biliary cirrhosis and coeliac disease: An association? Lancet 1978;1:230-3. |
[Figure 1], [Figure 2]