ESPN 50th Annual Meeting

ESPN 2017


 
HEPATIC MANIFESTATIONS AT THE ONSET OF SYSTEMIC LUPUS ERYTHEMATOSUS: CASE REPORT
ADRIAN LUNGU 1 ANCA-ELENA MARIN 1 DIANA BADUCU 1 IULIA CONSTANTINESCU 1 ILEANA NEGRU 1 CAMELIA PANTOC 2 ANDREI CAPITANESCU 2 CRISTINA STOICA 1

1- FUNDENI CLINICAL INSTITUTE
2- CAROL DAVILA NEPHROLOGY HOSPITAL
 
Introduction:

Multi-organ involvement is the hallmark of systemic lupus erythematosus (SLE). Clinically significant hepatic disease is generally regarded as unusual in SLE, but studies showed that hepatic disease may be more common in SLE that was initially thought.  Hepatic disease is not a significant cause of morbidity and mortality, but subclinical liver involvement is common. We aimed to determine if whether to classify our patient as having a primary liver disease with associated autoimmune features or having liver disease as a manifestation of SLE.

Material and methods:

We investigated a 14 years old boy who presented with one-year history of elevated serum aminotransferase and complaints of daily fever in the last month, polyarthralgia, swelling of the right knee, hepatosplenomegaly. Family history was negative for autoimmune or inherited liver disease, including Autoimmune hepatitis (AIH) and SLE.

Results:

SLE was confirmed by 4 clinical and 3 immunological criteria (according to SLICC Classification Criteria). Liver biopsy showed chronic hepatitis with discreet inflammatory activity, no fibrosis, no interface hepatitis, no fatty infiltration, so no specific elements were identified. Renal biopsy revealed class IIIC kidney disease.

Conclusions:

The difference between the hepatic involvement in SLE and AIH has not been clearly defined due to similarities in the clinical and biochemical features. Lupus hepatitis is a SLE-related liver dysfunction and it has been described as hypertransaminasemia owing to the fluctuations in the levels of alanine transaminase that are consistent with the activity of SLE. We consider that our patient has SLE and lupus hepatitis. Children with liver dysfunction and SLE should be investigated for Autoimmune Hepatitis (AIH) as these two entities can occur together and their complications are different. AIH may lead to end-stage liver disease, while SLE may result in end-stage renal disease. Liver biopsy might be necessary to establish the diagnostic in SLE patients with persistent increase of liver enzymes.