EC Paediatrics

Childhood-onset systemic lupus erythematosus (cSLE) is a chronic autoimmune disease with an incompletely understood etiology that commonly presents with fatigue, arthralgia, fever, malar rash, oral ulcers, and alopecia. Although the liver is less frequently involved than other organ systems, hepatic abnormalities are an important and under-recognized manifestation. Hepatic dysfunction in cSLE may result from immune-mediated lupus hepatitis (LH), drug-induced liver injury, infection, metabolic disease, or overlap with other immune-mediated disorders such as autoimmune hepatitis (AIH), making timely and accurate diagnosis essential. This mini-review presents recent evidence on the epidemiology, proposed pathogenic mechanisms, histopathologic features, and differential diagnosis of hepatic involvement in cSLE, and provides a diagnostic approach and management principles.

 Keywords: Childhood-Onset Systemic Lupus Erythematosus (cSLE); Lupus Hepatitis (LH); Autoimmune Hepatitis (AIH)

1. Aggarwal A., et al. “Childhood-onset systemic lupus erythematosus (cSLE): An international perspective”. Current Allergy and Asthma Reports 10 (2024): 559-569.
2. Sura A., et al. “Childhood-onset systemic lupus erythematosus”. Pediatrics in Review 6 (2024): 316-328.
3. Mariniello G., et al. “Liver involvement in juvenile systemic lupus erythematosus (SLE)”. Pediatric Rheumatology 1 (2011): P254.
4. Piga M., et al. “Liver involvement in systemic lupus erythematosus: incidence, clinical course and outcome of lupus hepatitis”. Clinical and Experimental Rheumatology 4 (2010): 504-510.
5. Runyon BA., et al. “The spectrum of liver disease in systemic lupus erythematosus. Report of 33 histologically-proved cases and review of the literature”. American Journal of Medicine 2 (1980): 187-194.
6. Tsokos GC. “Systemic lupus erythematosus”. New England Journal of Medicine 365 (2011): 2110-2121.
7. Takahashi A., et al. “Liver dysfunction in patients with systemic lupus erythematosus”. Internal Medicine 13 (2013): 1461-1465.
8. Zheng RH., et al. “Clinical and immunopathological features of patients with lupus hepatitis”. Chinese Medical Journal (England)2 (2013): 260-266.
9. Fiel MI., et al. “Systemic disease and the liver-part 1: Systemic lupus erythematosus, celiac disease, rheumatoid arthritis, and COVID-19”. Surgical Pathology Clinics 3 (2023): 473-484.
10. Afzal W., et al. “Lupus hepatitis, more than just elevated liver enzymes”. Scandinavian Journal of Rheumatology 6 (2020): 427-433.
11. Vaiphei K., et al. “Liver pathology in collagen vascular disorders highlighting the vascular changes within portal tract”. Indian Journal of Pathology and Microbiology 1 (2011): 25-31.
12. Bessone F., et al. “Challenge of liver disease in systemic lupus erythematosus: clues for diagnosis and hints for pathogenesis”. World Journal of Hepatology 6 (2014): 394-409.
13. Schmeltzer PA., et al. “Liver injury from nonsteroidal anti-inflammatory drugs in the United States”. Liver International 4 (2016): 603-609.
14. Conway R., et al. “Risk of liver injury among methotrexate users: a meta-analysis of randomized controlled trials”. Seminars in Arthritis and Rheumatism 2 (2015): 156-162.
15. Chertoff J., et al. “Azathioprine-induced hepatitis and cholestasis occurring 1 year after treatment”. BMJ Case Reports (2014): bcr2014206859.
16. Abdel Galil SM. “Hydroxychloroquine-induced toxic hepatitis in a patient with systemic lupus erythematosus: a case report”. Lupus6 (2015): 638-640.
17. Mieli-Vergani G., et al. “Diagnosis and management of pediatric autoimmune liver disease: ESPGHAN hepatology committee position statement”. Journal of Pediatric Gastroenterology and Nutrition 2 (2018): 345-360.
18. Gurung A., et al. “Histologic features of autoimmune hepatitis: a critical appraisal”. Human Pathology 82 (2018): 51-60.
19. Mori S., et al. “Hepatitis B virus reactivation associated with antirheumatic therapy: risk and prophylaxis recommendations”. World Journal of Gastroenterology 36 (2015): 10274-10289.
20. Balbi VA., et al. “Autoimmune hepatitis in 847 childhood-onset systemic lupus erythematosus population: a multicentric cohort study”. Advances in Rheumatology 1 (2018): 43.
21. Adiga A., et al. “Lupus hepatitis and autoimmune hepatitis (lupoid hepatitis)”. American Journal of the Medical Sciences 4 (2017): 329-335.
22. Huang D., et al. “Prevalence and risk factors for liver biochemical abnormalities in Canadian patients with systemic lupus erythematosus”. Journal of Rheumatology 2 (2012): 254-261.