Gastrointestinal system manifestations in juvenile systemic lupus erythematosus

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18 years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68.4%). The GIS manifestations due to SLE were autoimmune hepatitis (AIH) (n = 8) and lupus enteritis (n = 1). Manifestations associated with SLE were hepatomegaly and hypertransaminasemia due to macrophage activation syndrome (MAS) (n = 3) and hepatic steatosis (n = 1). GIS manifestations as a result of the adverse events of drugs were as follows: toxic hepatitis (n = 3; associated with methotrexate and nonsteroidal anti-inflammatory drugs in one, methotrexate in another, and azathioprine in another patient), azathioprine-induced cholestatic hepatitis (n = 1), and gastritis associated with corticosteroid (n = 1). In one patient, acute appendicitis occurred as a coincidence. In this study, one of every five pediatric SLE patients had GIS-related manifestations. GIS involvement may occur as an initial manifestation of the disease.

Autoimmune hepatitis in 847 childhood-onset systemic lupus erythematosus population: a multicentric cohort study

“…Of note, the majority of our patients had a Results are presented in n (%) and median (range), *P -value according to Bonferroni correction for multiple comparisons (p < 0.0033), SLEDAI-2 K -SLE Disease Activity Index 2000 complete response to the classical AIH prednisone and azathioprine combination therapy. Reinforcing this finding none of our patients had hepatic failure, portal hypertension and cirrhosis suggesting that AIH phenotype in lupus was a non-severe pattern [1,3]. Additionally, none of our cSLE patients had anti-LKM1 antibodies.…”

“…Childhood-onset systemic lupus erythematosus (cSLE) is an autoimmune and inflammatory disease that affects multiple organs and systems, including liver [1][2][3].…”

“…Of note, autoimmune hepatitis (AIH) is characterized by elevated hepatic enzymes, hypergammaglobulinemia, presence of autoantibodies and liver histology abnormalities, particularly interface hepatitis and lymphocytic infiltrates [4,5]. To our knowledge the prevalence of overlap AIH and cSLE in a large population was not studied and analysis of this very rare association is restricted to few case reports or case series [1,3,6,7].…”

Overlap Syndrome Involving Systemic Lupus Erythematosus and Autoimmune Hepatitis in Children: A Case Report and Literature Review

“…Overlap syndrome was usually used in the context of overlap of autoimmune hepatitis with primary sclerosing cholangitis (PSC) or primary biliary cholangitis (PBC), also called autoimmune sclerosing cholangitis (ASC). Although the case number is few, overlapping case of SLE and AIH has been occasionally diagnosed and reported (12, 13). Pediatric sclerosing cholangitis also has autoimmune features similar to AIH type 1, and the differential diagnosis depends on cholangiography (8).…”

“…However, there seems to be a non–relapsing association in the case reports of AIH-SLE overlapping in the recent years. Sönmez et al (12) reported 8 pediatric SLE-AIH overlapping patients with good response to immunosuppressive treatment and good prognosis of AIH. Since the case number is still few, further discussion should be done if more cases reported in the future.…”