DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20214540

A rare presentation of systemic lupus erythematosus with lupus hepatitis

Sushrutha K. P., Thanuja Basavanagowda, Savitha M. R., Prashanth S., A. Ramu

Abstract


Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammation and the presence of circulating autoantibodies directed against self-antigens. Prevalence of SLE in children and adolescents is 1-6 per 100,000 population. Liver dysfunction occurs in approximately in 50-60% of the patients with SLE.And the incidence of lupus hepatitis in diagnosed SLE patients is 9.3%. We are reporting a case of 11-year-old female child who presented with fever, skin rashes, oral ulcers, irritability and positive antinuclear antibody (ANA) with deranged liver function test (LFT) in the form of elevated transaminases, bilirubin level and altered coagulation profile. Hepatitis serology was negative, with low C3 levels, diagnosed as SLE with lupus hepatitis with lupus nephritis stage IV with psychosis. Clinical improvement along with improvement in terms of laboratory findings was seen on corticosteroids therapy. It is important to differentiate lupus hepatitis from autoimmune hepatitis (AIH) as AIH presents similar to lupus hepatitis, has poor prognosis. While lupus hepatitis being rare cause for mortality in patients with SLE has good response with early intervention with corticosteroids therapy.


Keywords


SLE, Lupus hepatitis, AIH

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References


Sperling MA. Nelson textbook of Pediatrics In: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, et al, eds 21st ed. Philadelphia: Elsevier; 2020;1274-9.

Beisel C, Weiler-Normann C, Teufel A, Lohse AW. Association of autoimmune hepatitis and systemic lupus erythematosis: A case series and review of the literature. World J gastroenterol. 2014;20(35):12662-7.

Avinash Adiga, Kenneth Nugent. Lupus Hepatitis and Autoimmune Hepatitis (Lupoid Hepatitis): Am J med Sci. 2017;353(4):329-35.

Ru-hua Z, Jin-hui W. Clinical and immunopathological features of patients with lupus hepatitis. Chinese med j. 2013;126(2):260-66.

Lai W-T, Cho W-H, Eng H-L, Kuo M-H, Huang F-C. Overlap Syndrome involving Systemic Lupus Erythematosisand Autoimmune Hepatitis in Children: A Case Report and Literature Review. Front. Pediatr. 2019;7:310.

Bessone F, Poles N, Roma MG. challenge of liver disease in systemic lupus erythematosus: clues for diagnosis and hints for pathogenesis. World J Hepatol. 2014;6(6):394-409.

Yeganeh MH, Zafari N, Sardarinia MA. Autoimmune hepatitis as an initial presentation of SLE: Arch Pediatric infect Dis. 2016;4(3):e34653.

Balbi VA, Montenegro B. Autoimmune hepatitis in 847 childhood- onset systemic lupus erythematosus population: a multicentric cohort study. Adv rheumatol. 2018;58:43.

Riyaz A. Paediatric gastroenterology and hepatology: autoimmune hepatitis 3rd edition: PARAS. 2008;496-502.

Beisel C, Weiler- Normann C, Teufel A, Lohse AW. Association of autoimmune hepatitis and systemic lupus erythmatosus; A case series and review of the literature. World J Gastroenterol. 2014;20(35):12662-7.

Mahler M, Kessenbrock K, Raats J, Fritzler MJ. Technical and clinical evaluation of anti-ribosomal P protein immunoassays. J clin Lab Anal. 2004;18:215.

Mahler M, Agmon-Levin N, Van Liempt M. multi-center evaluation of autoantibodies to the major ribosomal P C22 epitope. Rheumatol Int. 2012;32:691.