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AbstractAutoimmune hepatitis also known as Lupoid hepatitis is an autoimmune liver disease characterized by the

presence of autoantibodies including antinuclear antibodies (ANA) and hyper gammaglobulinemia. SLE can beassociated with hepatitis, which is referred to as lupus hepatitis. It is important to distinguish these two entities,as the course of the disease is different in both. It has also been noted that these two entities can co-exist when itis referred to as Autoimmune hepatitis-SLE overlap syndrome. We are reporting a case of Autoimmune hepatitis-SLE overlap syndrome in a 30 years old lady.

Autoimmune Hepatitis SLE Overlap Syndrome

Jency Maria Koshy*, Mary John**

*Assistant Professor, **Professor and Head , Department of Medicine,Christian Medical College and Hospital, Ludhiana 141008, PunjabReceived: 13.10.2011; Accepted: 29.07.2011

Introduction

Dierentiating the hepatic involvement in Systemic lupusErythematosis (SLE) and Autoimmune Hepatitis (AIH) hasbeen challenging, as there are similarities in the clinical featuresand the biochemical parameters in both these entities. AIH isalso known as Lupoid hepatitis, which is an autoimmune liverdisease, caused by the presence of autoantibodies, includingantinuclear antibodies (ANA) and hypergammaglobulinemia.

SLE can also have accompanying liver disease referred toas lupus hepatitis though it is rare. We are reporting a case ofAutoimmune hepatitis- SLE overlap syndrome. This patientfullled the American College of Rheumatology (ACR) criteriafor SLE and was proved to have AIH on liver biopsy.

Case Report30 years old lady presented to our hospital with yellowish

discoloration of sclera and abdominal distention for 1month.She was initiated on anti tubercular treatment for tuberculouslymphadenitis, 10 months prior to admission. She discontinuedthe medicines after 4 months as she developed jaundice followingwhich her jaundice subsided. She also gave history of multiple

blood transfusions in the past for anaemia. There was no historyof obvious blood loss.

Clinical examination revealed icterus, pallor, clubbing,cervical lymphadenopathy and ascitis. There were no peripheralsigns of liver cell failure. Probable diagnoses considered on herwere Disseminated tuberculosis/ autoimmune hepatitis.

Investigations revealed anaemia (Hb 7g%), leucopoenia withlymphopenia (TLC 3700/mm3, ALC 980/mm3), deranged PTand PTTK, direct hyperbilirubinemia and raised transaminases.Ascitic uid examination was transudative. USG Abdomenshowed liver cirrhosis with portal hypertension and ascitis.We further evaluated the patient for the various causes of livercirrhosis.HbsAg and HCV Ag were not detected in serum.

HEVAg was positive. KF rings were absent on slit lampexaminaton.

Prominent esophageal vein was noted on endoscopy. CTabdomen revealed nodular liver, dilated portal vein, multiplelymph nodes measuring 21x 12 mm in the mesentry, portal andaortocaval areas, ascitis and B/L pleural eusion.

Cervical lymph node biopsy and bone marrow aspirationwere done considering a possibility of disseminated tuberculosisor lymphoma. Lymph node biopsy was reported as necrotizinglymphadenitis. Bone Marrow examination revealed plasmacytosisof 10-20 % with normal cell lines.

Serum protein electrophoresis revealed M band in gregion.IgG was1100 U/ml (650-1500), IgA was 488U/ml (50-450) andIgM was 260U/ml(24-332). ANA and AntiDsDNA were stronglypositive [ANA 42.6 U/ml(0-1.4 ), AntiDsDNA 213 U/ml (0-40)]with decreased C3 and C4.

A diagnosis of SLE was made as she fullled ACR criteria.(Presence of ANA, AntiDsDNA, lymphopenia and serositis).Necrotizing lymphadenitis was aributed to SLE.However her

advanced liver cell failure could not be explained by SLE alone.Hence we considered a possibility of Autoimmune Hepatitis SLE overlap syndrome.

Meanwhile patients general condition worsened and sheprogressed to grade 4 encephalopathy. She was pulsed withmethylprednisolone followed by oral prednisolone. Howeverher general condition kept worsening and she succumbed toher illness eventually.

Postmortem liver biopsy was done which revealed large areasof necrosis and brosis replacing the normal lobular architecture.Liver cells were cord like and regenerative rosees were presentat other places.Macrovesicular and micro vesicular steatosiswith intense lymphoplasmacytic inltrates was present in the

brotic area . These ndings were consistent with autoimmunehepatitis with submassive necrosis. So the nal diagnosis wasconrmed as Autoimmune Hepatitis- SLE overlap syndrome.

DiscussionAutoimmune Hepatitis (AIH) also known as Lupoid hepatitis

is an autoimmune liver disease caused by the presence ofauto antibodies, including antinuclear antibodies (ANA) andhypergammaglobulinemia. Systemic Lupus Erythematosis(SLE), which is an autoimmune disorder aecting multipleorgans can have accompanying liver disease, which is alsoreferred to as lupus hepatitis.

The dierence between the hepatic involvement in SLE andAIH has not been clearly dened due to similarities in the clinicaland biochemical features. Liver involvement in patients with SLEis well documented but is considered rare. It has been suggestedthat patients with AIH may be at an increased risk of developingsystemic connective tissue diseases. Conversely, patients withsystemic connective tissue disease may be at an increased riskof AIH. Therefore it is important to distinguish AIH from SLEsince complications are dierent in the two conditions.1 SLEmay result in end stage renal disease while AIH may lead toend stage liver disease.

Even though hepatic lesions due to pathogenic process of SLEhave been thought to be rare; recent studies have indicated thatliver involvement in patients with SLE is of a more signicantimportance than had been thought.2 The dierence between AIH

and hepatic lesions in SLE has long been an indistinct issue. Oka

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reported that 3% of patients with AIH satised the ACR criteriafor SLE.4 Matsumoto etal, who surveyed 1,468 Japanese patientswith SLE reported that only 1.1 % of them had liver cirrhosis.

AIH-SLE overlap syndrome is considered when patientsfulll the ACR criteria for SLE and International AutoimmuneHepatitis group scoring for AIH. Although AIH and SLEare considered two dierent entity both have features of anautoimmune disease with presence of various antibodies.

However histopathologically they are quite distinct. Periportalpiecemeal necrosis associated with lobular activity, roseingof liver cells or dense lymphoid inltrates is prominent in AIH,while in SLE, inammation is usually lobular and occasionallyperiportal with paucity of lymphoid inltrates.

Our patient fullled the ACR criteria for SLE .Her scoring asper international Autoimmune Hepatitis group was 10.Her lowerscore was aributed to unavailability of other auto antibodiesand HLA typing. She also had Hepatitis E co infection; whichalso aributed to negative scoring. However the hepatotrophicviruses are known triggers for AIH. In our patient Hepatitis Evirus infection would have been the trigger. However roseing ofliver cells and dense lymphoplasmacytic inltrate in our patientwas consistent with AIH. The generalized lymphadenopathy inour patient involving cervical, mesenteric, portal and aortocavallymph nodes were aributed to SLE, as the biopsy was consistentwith necrotizing lymphadenitis.The stormy course with liver cellfailure was quite characteristic of AIH.

Antiribosomal P antibody is a useful marker to dierentiateSLE associated hepatitis from AIH. Anti Sm antibodies is highlyspecic though relatively insensitive to SLE

Corticosteroid therapy is the mainstay of therapy.Immunosuppresants like Azathioprine mycophenolate mofetil,cyclosporine and tacrolimus also have shown promising results.6

ConclusionPatients presenting with AIH-SLE overlap impose diagnostic

and therapeutic dilemma on the physician. It is important todistinguish SLE associated hepatitis (lupus hepatitis) from AIH

associated with SLE since the course of the disease is dierentin both the entities.

Most of the biochemical parameters are inconclusive indifferentiating the two entities. However Liver biopsy isdiagnostic.

References1. Usta Y, Gurakan F, Akcoren Z, etal. An overlap syndrome involving

autoimmune hepatitis and systemic lupus Erythematosis inchildhood. World J Gastroenterol2007;13: 2764-7.

2. Runyon BA, LaBrecque DR, Anuras S.The spectrum of liver diseasein systemic lupus Erythematosis. Report of 33 histologically- provedcases and review of the literature.Am J Med1980;69:187-94.

3. Tojo J, Ohira H, Abe K, etal. Autoimmune hepatitis accompaniedby systemic lupus Erythematosis. Intern Med2004;43:258-62.

4. Oka H.The survey of autoimmune hepatitis in Japan. Annual Reportof the Study Group on Severe Hepatitis. Japanese Ministry of Healthand Welfare 1988;235-41.

5. Matsumoto T, YoshimineT, Shimouchi K, etal The liver in systemiclupus Erythematosis: pathologic analysis of 52 cases and review ofJapanese Autopsy Registry data. Hum Pathol1992;23:1151-1158.

6. Czaja AJ. Treatment of autoimmune hepatitis. Semin Liver Dis2002;22:365-378.