Saudi Journal of Gastroenterology
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CASE REPORT Table of Contents   
Year : 2002  |  Volume : 8  |  Issue : 3  |  Page : 99-101
Cryptogenic or autoimmune hepatitis


Department of Medicine, Consultant Gastroenterologist, King Abdulaziz University Hospital, Jeddah, Saudi Arabia

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Date of Submission19-Nov-2001
Date of Acceptance29-Sep-2002
 

How to cite this article:
Akbar HO. Cryptogenic or autoimmune hepatitis. Saudi J Gastroenterol 2002;8:99-101

How to cite this URL:
Akbar HO. Cryptogenic or autoimmune hepatitis. Saudi J Gastroenterol [serial online] 2002 [cited 2019 Nov 14];8:99-101. Available from: http://www.saudijgastro.com/text.asp?2002/8/3/99/33400


Autoimmune hepatitis (AIH) is uncommon disease affecting usually young females and characterized by the presence of circulating autoantibodies with elevated gammaglobulin [1],[2] .Corticosteroids which is the corner stone for treatment of most autoimmune disease, is the treatment of choice of AIH [3] . Since corticosteroids is not effective in other liver diseases, including primary sclerosing cholangitis and primary biliary cirrhosis with underlying immune pathogenesis, two cases of liver disease with unknown cause are reported, with probable AIH and good response to corticosteroids.


   Case report Top


Two patients presented with jaundice and fatigue of 4-6 weeks duration. There were no risk factors or significant drug history. Examination was unremarkable except for the presence of jaundice with no evidence of chronic liver stigmata.

The first patient is a 47-year old male who presented in 1995. Results of his investigation included WBC (2.4xl0 9 /L); Hb (9.lg/dl); Platelets (125x10 9 /L); PT/INR (1.3); SGOT (1248 IU/L); SGPT (1118 IU/L); Alp (869 IU/L); Albumin (29g11); T. bilirubin (143 umol/1); IgG (22 g/1), [Table - 1]. He had ERCP which was normal and subsequently a liver biopsy was done that showed interface hepatitis with infiltration of portal tracts and hepatic lobules with lymphocytes and plasma cells together with piece meal necrosis [Figure - 1],[Figure - 2].

The second patient is a 52-year old female, presented in 2001. Her investigation result included WBC (7x10 9 /L); Hb (11.3g/dl); Platelets (96x10 9 /L); PT/INR (1.8); SGOT (607 IU/L); SGPT (760 IU/L); Alp (220 IU/L); Albumin (27g/1); T. bilirubin (623 umol/1); IgG (28 g/1), [Table - 1]. No liver biopsy was done because of increased bleeding tendency In addition, both had negative tests for viral hepatitis serology (HBsAg, HCV-Ab and HAV-Ab (IgM), negative results for autoantibodies, that was double checked (anti-nuclear antibody (ANA), smooth muscle antibody (SMA), anti-mitochondrial antibody (AMA), liver-kidney microsomal antibody (ALKM-1), normal ceruloplasmin, transferrin saturation and CT-abdomen. Both patients received prednisone (40mg daily) and had a complete clinical response and normalization of liver function test within 4-9 months.

First patient normalized his liver function after nine months, however he relapsed after reduction of prednisone to 5m- and improved after increasing Prednisone dose and adding azathioprine (50 mg daily). Currently he is on 5m prednisone and 50 mg azathioprine daily for the last four years with persistently normal liver function.

The second patient had normal liver enzymes four months after starting prednisone and currently is on 7.5 mg prednisone daily for the last three months with normal liver function test, platelets and prothrombin time for more than seven months. She was advised to have a liver biopsy at this stage but she refused.


   Discussion Top


Autoimmune hepatitis diagnosis depends on the presence of specific autoantibodies. Accordingly, there are two different sub-types of AIH. Type I is characterized by the presence of ANA and/or SMA and typell is characterized by the presence of ALKM-1 [4] . In addition, a scoring system for diagnosis of AIH was established based on different clinical, serologic and immunologic variables (Johnson and McFarlane) [5],[6] . The above two patients reported, were tested negative for autoantibodies (ANA, SMA, ALKM-1), and hence cannot be classified as having neither type I nor type 2 AIH. The only indication of underlying immunological pathogenesis is their response to corticosteroids as well as the relapse in the first case when steroids dose was decreased requiring dose increase and addition of azathioprine which in turn resulted in normalization of liver enzymes again.

After applying the scoring system; they scored 10-12 points before treatment and 12-14 points respectively, after treatment which means they belong to the probable AIH group [5],[6] . Other autoantibodies has been reported in AIH and includes anti-neutrophil cytoplasmic antibodies (ANCA), anti soluble liver antigen (anti-SLA), liver microsomal antibodies (LM), anti-liver cytosolic protein type I (ALC 1), anti-asialoglycoprotein receptor (ASGP-R), which are not frequently tested and some are not available for commercial use [7] . Most of these autoantibodies are markers of AIH, they are not involved in the pathogenesis and do not predict response to treatment. Other autoantibodies possibly exist, that are not discovered yet, which may play a role in the pathogenesis and helps in the diagnosis of more cases of AIH that are initially diagnosed as cryptogenic liver disease.

The above two cases are an example of probable AIH with the absence of currently tested autoantibodies, and if were diagnosed as cryptogenic liver disease, they will miss the response achieved using corticosteroids.


   Conclusion Top


Diagnosis of AIH based on the presence of currently tested autoantibodies may miss a group of patients with AIH and negative autoantibodies and hence will be misdiagnosed as cryptogenic liver disease. Few patients with cryptogenic liver disease, based on negative tests for known underlying liver disease, may have AIH and a trial of corticosteroid treatment for possibility of AIH may be considered in some patients after weighing the benefits and risks of corticosteroids with close patient follow up.

 
   References Top

1.Czaja A. autoimmune hepatitis, evolving concepts and treatment strategies. Dig Dis Sci 1995: 40: 435-56.  Back to cited text no. 1    
2.Johnson PJ, McFarlane IG, Eddleston ALWF. The natural course and heterogeneity of autoimmune type chronic active hepatitis. Semin Liver Dis 1991: 11 :187-96.  Back to cited text no. 2    
3.Summerskill WHJ, Korman MG, Ammon HV, Baggenstoss AH. Prednisone for chronic active liver disease: dose titration, standard dose and combination with azathioprine compound. Gut 1975: 16: 876-83.  Back to cited text no. 3    
4.Desmet VJ. Gerber M, Hoofnaagle JH. Manns M. Scheuer PJ. Classification of chronic hepatitis: diagnosis, grading and staging. Hepatology 1994: 19: 1513-20.  Back to cited text no. 4    
5.Johnson PJ. McFarlane IG. Meeting report: International Autoimmune Hepatitis Group. Hepatology 1993: 18: 998-1005.  Back to cited text no. 5    
6.Alvarez F. Berg PA, Bianchi FB, et al. International Autoimmune Hepatitis Group report: Review of criteria for diagnosis of autoimmune hepatitis. JHepatol 1999: 31: 929.  Back to cited text no. 6    
7.Obermaver-Straub P, Strassburg CP, Manns MP. Autoimmune Hepatitis. Journal of Hepatol 2000: 32:181-197.  Back to cited text no. 7    

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Correspondence Address:
Hisham Osman Akbar
Consultant Gastroenterologist, King Abdulaziz University Hospital, P. O. Box 80215, Jeddah 21589
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


PMID: 19861801

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