Overlap syndrome of autoimmune hepatitis , Primary biliary cholangitis leading to decompensated cirrhosis in a patient of systemic lupus erythematosus: A case report

Dr. Dipankar Das

1 day ago

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Speaker Name: Dr. Dipankar Das
Category: (Poster Presentation)
Authors: Dr Dipankar das ^1, Dr Anurag Tiwari ²

^{DM resident , Department of Gastroenterology, Institute of medical sciences, BHU,}

^{Assistant professor, Department of Gastroenterology, Institute of medical sciences, BHU,}

Global Journal of Gastroenterology & Hepatology Research [GJGHR]

Background:

Systemic lupus erythematosus (SLE) is a multi system autoimmune disorder involving various organs such as kidneys, skin and the central nervous system. Liver involvement is normally not part of the spectrum of SLE, but is seen in up to 60% of SLE patient. ¹ Hepatotoxic drugs, coincident viral hepatitis and non-alcoholic fatty liver disease (often induced by steroids) are the most commonly described causes of elevated liver enzymes in SLE . ² The co-occurrence of autoimmune hepatitis (AIH) and SLE is considered to be rare, Autoimmune hepatitis with primary biliary cholangitis overlap with SLE is even more rarer.

Case report: The patient is a 30yr old female, resident of Uttar Pradesh presented to our hospital, IMS ,BHU with history of hematemesis 1 episode 8 months back , during 3 rd month of her gestation, complaints of multiple joints

Pain, alopecia, abdominal distension , intermittent fever since last one and half months. She was evaluated, found to have cirrhosis (decompensated with ascites, past UGI bleed), pancytopenia, coagulopathy, oesophageal varices, hypoalbuminemia, pleural effusion. Biochemical profile revealed elevated ALT,AST (72, 94 IU/ml), elevated ALP ( 638IU/ml), elevated Bilirubin ( 3 x ULN). Viral hepatitis serology, Iron studies, celiac panel, Wilson disease work up, were insignificant. During etiological work up, antinuclear antibody (ANA by anti Hep2 cells antibodies) titre increased 6.90, anti smooth muscle antibody ( ASMA) negative ,titre 1:20, Anti LKM 1 negative ,serum IgG level increased (2104 mg/dl ), Anti mitochondrial antibody (AMA -M2) was found positive ,124 IU/ml . she also had positive DsDNA level > 150 IU/ml. she had urinary 24 hr protein level > 560 mg/dl. Renal biopsy was avoided in view of severe thrombocytopenia. TransJuglar liver biopsy could not be done due to non-consent. The patient met criteria for AIH -PBC overlap syndrome based on Paris criteria.( ³)The patient was given treatment with oral prednisolone(40 mg to start with), Oral mycophenolate mofetil ( 500 mg BD), oral ursodeoxycholic acid ( 900mg/day). The patient responded well both clinically and symptomatically.

Discussion: The diagnosis of probable AIH was made based on revised original scoring system of the IAIHG. Elevated IgG and ANA positivity are characteristic for both AIH and SLE. Anti Ds DNA is very specific for SLE, ( specificity 95.9%), but also common in patients with type 1 AIH (⁴ )AIH-PBC overlaps are found approximately in 7%-13% AIH patients.^{( 5)} Although autoimmune diseases Currently it is uncertain whether con concomitant SLE, AIH -PBC occurs by chance or they share some common immunological and/or genetic basis.

Conclusions: It is advisable to work up for overlap syndrome in lupus hepatitis patients for co existing AIH / PBC

Reference:

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. Am J Med 1980; 69: 187-194 [PMID: 7405944]
Matsumoto T, Yoshimine T, Shimouchi K, Shiotu H, Kuwabara N, Fukuda Y, Hoshi T. The liver in systemic lupus erythematosus: pathologic analysis of 52 cases and review of Japanese Autopsy Registry Data. Hum Pathol 1992; 23: 1151-1158 [PMID: 1398643
Manns ML (2010) AASLD Practice Guidelines: Diagnosis and Management of Autoimmune Hepatitis. Hepatology 51: 1-31.
Muratori P, Granito A, Pappas G, Pendino GM, Quarneti C, et al. (2009) The serological profile of the autoimmune hepatitis/primary biliary cirrhosis overlap syndrome. Am J Gastroenterol104: 1420-1425.
Czaja AJ (2013) The overlap syndromes of autoimmune hepatitis. Dig Dis Sci 58: 326-343.

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