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Systemic Lupus Erythematosus in a Male Patient With Autoimmune Hepatitis

Soumaya Zaouga1, Soumaya Mrabet1, Raida Harbi1, Imen Akkari1, Elhem Ben Jazia1

1 Department of Gastroenterology, Farhat Hached Hospital, Sousse, Tunisia.

Conflict-of-interest statement: The author(s) declare(s) that there is no conflict of interest regarding the publication of this paper.

Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http: //creativecommons.org/licenses/by-nc/4.0/

Correspondence to: Zaouga Soumaya, Department of Gastroenterology, Farhat Hached Hospital, Sousse, Tunisia.
Email: zaouga.Soumaya@gmail.com

Received: May 23, 2021
Revised: June 14, 2021
Accepted: June 18, 2021
Published online: August 21, 2021

ABSTRACT

Overlap syndrome involving autoimmune hepatitis (AIH) and systemic lupus erythematosus (SLE) is a rare condition. The difference between the hepatic involvement of SLE and autoimmune hepatitis has not been clearly defined due to the presence of clinical and laboratory common features. We report a 42-year-old male patient with autoimmune hepatitis who presented with intermittent fever, jaundice, and dyspnea. CT scan showed pleural and pericardial effusion, high titer of antinuclear antibodies and positive anti-DNA antibody were found. Our patient fulfilled both the international criteria of SLE and AIH. Clinical symptoms and laboratory findings improved with treatment by corticosteroids.

Key words: Cirrhosis; Autoimmune hepatitis; Lupus; Overlap

© 2021 The Author(s). Published by ACT Publishing Group Ltd. All rights reserved.

Zaouga S, Mrabet S, Harbi R, Akkari I, Jazia EB. Systemic Lupus Erythematosus in a Male Patient With Autoimmune Hepatitis. Journal of Gastroenterology and Hepatology Research 2021; 10(4): 3565-3567 Available from: URL: http://www.ghrnet.org/index.php/joghr/article/view/3190

INTRODUCTION

Autoimmune hepatitis is a chronic inflammatory liver disease that affects mainly women in early adulthood. The usual presentation includes non-specific symptoms such as fatigue, general ill health, right upper quadrant pain, nausea, pruritus, and fluctuating jaundice[1]. Immunosuppression, generally results in clinical and laboratory improvement[2]. The disease, if untreated, often leads to cirrhosis and liver failure. Other autoimmune diseases may be associated with AIH such as primary biliary cholangitis (PBC), celiac disease, autoimmune thyroiditis and SLE. SLE is a systemic autoimmune disease, with multisystemic involvement and several presentations[3]. The term overlap syndrome is usually used in the context of overlap of AIH with PBC. Overlapping case of SLE and AIH has been occasionally diagnosed and reported. In this report, we present a patient with an overlap syndrome involving autoimmune hepatitis and SLE.

Case report

A 42-year-old male without a notable personal history, and without a family history of autoimmune diseases, presented in November 2019 with liver dysfunction and ascites. Laboratory data showed: Serum alanine aminotransferase (ALT) 230 IU/L (N: 5-40 IU/L), aspartate aminotransferase (AST) 390 IU/L (N: 5-40 IU/L), gamma-glutamyl transpeptidase (GGT ): 50 IU/L (N: 5-40 IU/L), alkaline phosphatase 80 IU/L (N: 35-140 IU/L), total bilirubin 44 µmol/L (N: 3-17 µmol/L), conjugated bilirubin 39mg/L (N:1-3 µmol/L), and a low prothrombin activity at 37%. Hemoglobin rate was 14 g/dL White blood cells: 4030 elements/mm3 Platelets: 140 000 elements/mm3.

Positive results for serum antibodies against nuclear antigen (ANA): 1/800 IU/L and anti-double-stranded DNA. Anti-soluble liver antigens/liver-pancreas (SLA/LP) were also positive. Gamma globulin levels were very high at 45 g/L (N: 8-13.5 g/L). Viral serology was negative for hepatitis B and C virus.

The patient was diagnosed to have probable AIH according to diagnostic criteria for AIH.

A liver biopsy was not performed due to the presence of ascites and coagulopathy. Prednisolone therapy was not used because the patient had an advanced decompensated cirrhosis at the time of diagnosis.

In July 2020, the patient was admitted for fever with cough and dyspnea. On physical examination he had: Fever at 38.3°C, Pulse: 74 bpm, jaundice and large volume ascites. The laboratory findings showed: White blood cells: 7400 E/mm3 Hemoglobin: 10.7 g/dL, platelets: 134 000 /mm3; CRP: 90 mg/L; AST: 57 IU/L, ALT: 21 IU/L, GGT: GGT: 14 IU/L, ALP: 76 IU/L, Total bilirubin: 73 µmol/L Direct bilirubin: 17 µmol/L; a positive direct coombs test, C3: 0.25 g/L (VN: 0.9-1.8 g/L) C4: 0.05 (VN: 0.1 - 0.4 g/L) positive ANA: 1/1600 UI /L positive native anti DNA bodies.

Chest X-ray (Figure 1) and computed tomography showed pericardial and pleural effusion. These clinic-biological data allow us to make the diagnosis of SLcE acording to SLICC criteria and EULAR/ ACR 2019 criteria.

Figure 1 Chest X-ray showing pleural effusion.

The Patient was treated by corticosteroid: prednisolone 0.5 mg/kg with good outcome: disappearance of fever, regression of biological inflammatory syndrome, marked decrease in bilirubin and decrease in pleural effusion.

DISCUSSION

Systemic lupus erythematous (SLE) is a multisystem autoimmune disorder involving various organs including the liver, skin, kidney, and joints. Increased serum levels of liver enzymes are common in SLE[4]. This can reflect liver disease as a component of SLE or a liver disorder associated with another immune disease such as AIH.

Autoimmune hepatitis is a chronic progressive inflammatory liver disorder of unknown etiology with fluctuating course and potentially progressing to cirrhosis.

HAI and SLE overlap syndrome has previously been reported in some cases but it is considered to be rare however its exact frequency is not clear.

Our patient, was diagnosed with SLE according to the criteria of EULAR/ACR 2019 and to the SLICC criteria[5,6].

The criteria for the diagnosis of AIH in adult patients have been established by the International Autoimmune Hepatitis Group (IAIHG). Diagnostic criteria are based on elevation of Immunglobulin G (IgG), demonstration characteristic autoantibodies, histological features of hepatitis and the absence of viral disease[7] (Table 1). AIH score in our patient was 6 suggesting probable AIH.

Table 1 Simplified criteria for diagnosis of autoimmune hepatiti [7].
Feature /ParameterDiscriminatorScore
ANA ou SMA + ≥ 1 :401
ANA ou SMA + ≥ 1 :802
Or LKM1 + ≥ 1 :402
Or SLA/LP+ 2
IgG ou Gamma globulins levels> Upper limit of normal1
> 1,1 Upper limit of normal2
Liver biopsyTypical of HAI2
Compatible with AIH1
Absence of viral hepatitisYes2
Definite autoimmune hepatitis: ≥ 7; Probable autoimmune hepatitis: ≥ 6.

Liver biopsy plays an important role in the diagnostic regimen. It would have been of major diagnostic and prognostic interest[8].

Histological findings in AIH are periportal piecemeal necrosis and hepatocyte rosette formation abundant plasma cell and lymphocyte infiltrate. Percutaneous Liver Biopsy was contraindicated for our patient due to the presence of ascites and coagulopathy[9].

Transjugular liver Biopsy (TJLB) is performed in patients when liver biopsy is essential for the diagnosis and management but percutaneous biopsy is contraindicated due to deranged bleeding parameters or ascites[10]. Nevertheless, TJLB is not available in our hospital.

The difference between the hepatic involvement in SLE and AIH has not been clearly defined both have features of an autoimmune disorder, such as the presence of hypergammaglobulinemia and positive tests for ANA. It is important to distinguish between these two entities because the prognosis and the treatment are different. Differential diagnosis between SLE hepatitis and AIH must be made in order to avoid potential complications associated with inadequate therapy.

We believe that our patient presents an overlap syndrome (AIH and SLE ) because of the advanced liver disease at diagnosis: decompensated cirrhosis classified Child-Pugh C 10, the negativity of Anti- ribosomal P antibody, which are strongly correlated with SLE associated hepatitis[11], and the presence of specific markers for AIH, which usually do not occur in SLE: soluble liver antigen (SLA)[12].

Overlap syndrome (SLE and HAI) has been reported to respond rapidly to corticosteroid therapy[13], the prognosis is generally good[14].

AIH patients with decompensated cirrhosis constitute a subgroup with a poor prognosis and a high mortality rate[15].

For our patient the response to corticosteroid was good: The patient’s condition improved rapidly with a medium dose of prednisolone. However, the prognosis is considered to be poor since the diagnosis was made at an advanced stage of chronic liver failure[15].

Conclusion

AIH and SLE with hepatic involvement are two autoimmune diseases that share several clinical and biological similarities. The association of these two diseases in one patient is rare. Autoimmune hepatitis is associated with a poor prognosis, higher risk of progression to cirrhosis. It’s needs to be considered in SLE patients with elevated liver enzymes.

Acknowledgement

The authors express deep appreciation to Mr. Akira Mukaitani, Chairperson of Japan Aloe Science Association, supporting the information on the case reports.

REFERENCES

1. Czaja AJ. Diagnosis and Management of Autoimmune Hepatitis: Current Status and Future Directions. Gut Liver. 2016 Mar; 10(2): 177-203 [PMID: 26934884]; [PMCID: PMC4780448]; [DOI: 10.5009/gnl15352]

2. Dhruv Lowe, Savio John. Autoimmune hepatitis: Appraisal of current treatment guidelines. World J Hepatol. 2018 Dec 27; 10(12): 911-923. [PMID: 30631396]; [PMCID: PMC6323516]; [DOI: 10.4254/wjh.v10.i12.911]

3. T. B et O. Nj, Systemic lupus erythematosus diagnosis and management, Rheumatology (Oxford, England), janv. 04, 2017.

4. Runyon BA, LaBrecque DR, Anuras S. The spectrum of liver disease in systemic lupus erythematosus: Report of 33 histologically-proved cases and review of the literature. The American Journal of Medicine. 1980; 69(2): 187-194. [DOI: 10.1016/0002-9343(80)90378-2]

5. Michelle Petri, Ana-Maria Orbai, Graciela S Alarcón, Caroline Gordon, Joan T Merrill, Paul R Fortin, Ian N Bruce, David Isenberg, Daniel J Wallace, Ola Nived, Gunnar Sturfelt, Rosalind Ramsey-Goldman, Sang-Cheol Bae, John G Hanly, Jorge Sánchez-Guerrero, Ann Clarke, Cynthia Aranow, Susan Manzi, Murray Urowitz, Dafna Gladman, Kenneth Kalunian, Melissa Costner, Victoria P Werth, Asad Zoma, Sasha Bernatsky, Guillermo Ruiz-Irastorza, Munther A Khamashta, Soren Jacobsen, Jill P Buyon, Peter Maddison, Mary Anne Dooley, Ronald F van Vollenhoven, Ellen Ginzler, Thomas Stoll, Christine Peschken, Joseph L Jorizzo, Jeffrey P Callen, S Sam Lim, Barri J Fessler, Murat Inanc, Diane L Kamen, Anisur Rahman, Kristjan Steinsson, Andrew G Franks Jr, Lisa Sigler, Suhail Hameed, Hong Fang, Ngoc Pham, Robin Brey, Michael H Weisman, Gerald McGwin Jr, Laurence S Magder. Derivation and Validation of Systemic Lupus International Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheum. 2012 Aug; 64(8): 2677-2686. [PMID: 22553077]; [PMCID: PMC3409311]; [DOI: 10.1002/art.34473]

6. Aringer M. EULAR/ACR classification criteria for SLE, Seminars in Arthritis and Rheumatism. Semin Arthritis Rheum. 2019 Dec; 49(3S): S14-S17. [PMID: 31779843]; [DOI: 10.1016/j.semarthrit.2019.09.009]

7. Elke M Hennes, Mikio Zeniya, Albert J Czaja, Albert Parés, George N Dalekos, Edward L Krawitt, Paulo L Bittencourt, Gilda Porta, Kirsten M Boberg, Harald Hofer, Francesco B Bianchi, Minoru Shibata, Christoph Schramm, Barbara Eisenmann de Torres, Peter R Galle, Ian McFarlane, Hans-Peter Dienes, Ansgar W Lohse, International Autoimmune Hepatitis Group. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology. 2008 Jul; 48(1): 169-76. [PMID: 18537184]; [DOI: 10.1002/hep.22322]

8. Szabó Z. The role of liver biopsy in the diagnosis of autoimmune hepatitis. Orv Hetil. 2006; 147(35): 1697-1702

9. James Neuberger, Jai Patel, Helen Caldwell, Susan Davies, Vanessa Hebditch, Coral Hollywood, Stefan Hubscher, Salil Karkhanis, Will Lester, Nicholas Roslund, Rebecca West, Judith I Wyatt, Mathis Heydtmann. Guidelines on the use of liver biopsy in clinical practice from the British Society of Gastroenterology, the Royal College of Radiologists and the Royal College of Pathology. Gut. 2020 Aug; 69(8): 1382-1403. [PMID: 32467090]; [PMCID: PMC7398479]; [DOI: 10.1136/gutjnl-2020-321299]

10. Shyamkumar N Keshava, Thomas Mammen, Nrs Surendrababu, Vinu Moses. Transjugular liver biopsy: What to do and what not to do. Indian J Radiol Imaging. 2008 Aug; 18(3): 245-8. [PMID: 19774169]; [PMCID: PMC2747432]; [DOI: 10.4103/0971-3026.41839]

11. E Koren, W Schnitz, M Reichlin. Concomitant development of chronic active hepatitis and antibodies to ribosomal p proteins in a patient with systemic lupus erythematosus. Arthritis Rheum. 1993 Sep; 36(9): 1325-8. [PMID: 8216426]; [DOI: 10.1002/art.1780360917]

12. Avinash Adiga, Kenneth Nugent. Lupus Hepatitis and Autoimmune Hepatitis (Lupoid Hepatitis). Am J Med Sci. 2017 Apr; 353(4): 329-335. [PMID: 28317620]; [DOI: 10.1016/j.amjms.2016.10.014]

13. Maame B Amissah-Arthur, Caroline Gordon. Contemporary treatment of systemic lupus erythematosus: an update for clinicians. Ther Adv Chronic Dis. 2010 Jul; 1(4): 163-75. [PMID: 23251736]; [PMCID: PMC3513867]; [DOI: 10.1177/2040622310380100]

14. Jun Tojo, Hiromasa Ohira, Kazumichi Abe, Junko Yokokawa, Junko Takiguchi, Tsuyoshi Rai, Shoichiro Shishido, Yukio Sato, Reiji Kasukawa. Autoimmune Hepatitis Accompanied by Systemic Lupus Erythematosus. Intern Med. 2004 Mar; 43(3): 258-62. [PMID: 15098613]; [DOI: 10.2169/internalmedicine.43.258]

15. Zhaoyue Wang , Li Sheng, Yue Yang, Fan Yang, Xiao Xiao, Jing Hua, Canjie Guo, Yiran Wei, Ruqi Tang, Qi Miao, Jun Zhang, Yanmei Li, Jingyuan Fang, Dekai Qiu, Edward L Krawitt, Christopher L Bowlus, M Eric Gershwin, Qixia Wang, Xiong Ma. The Management of Autoimmune Hepatitis Patients with Decompensated Cirrhosis: Real-World Experience and a Comprehensive Review. Clin Rev Allergy Immunol. 2017 Jun; 52(3): 424-435. [PMID: 27515672]; [DOI: 10.1007/s12016-016-8583-2]

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