DOI: 10.12890/2026_007352
Pregnancy complicated by systemic lupus erythematosus, autoimmune hepatitis and portal hypertension
Authors
lusisahakyan@gmail.com
Keywords
Systemic lupus erythematosus, autoimmune hepatitis, portal hypertension, pregnancy, case reportAbstract
Introduction: Pregnancy in women with concurrent systemic lupus erythematosus (SLE), autoimmune hepatitis and portal hypertension is exceptionally rare, with fewer than 10 reported cases of any two conditions together and none describing the full triad with detailed haemostatic assessment.
Case description: A 26-year-old woman at 9–10 weeks of gestation presented with abnormal liver enzymes, thrombocytopenia, and later developed subacute cutaneous SLE. Autoimmune evaluation revealed positive anti-double-stranded deoxyribonucleic acid (45 IU/ml), low complement C3 (0.68 g/l), and elevated serum IgG (18.2 g/l) supporting a diagnosis of SLE and probable autoimmune hepatitis. Doppler ultrasound confirmed compensated portal hypertension with splenomegaly. Multidisciplinary management included methylprednisolone, hydroxychloroquine, and low-dose aspirin. The platelet count remained stable (85–105 × 109/l) throughout pregnancy. Second-trimester endoscopy showed grade I varices without red signs. Planned caesarean section was performed at 34–35 weeks after foetal lung maturation, delivering a female neonate weighing 2,270 g with Apgar scores 8/9. Maternal and neonatal outcomes were favourable.
Conclusion: Favourable outcomes are achievable despite the concurrent burden of SLE, autoimmune hepatitis (AIH) and portal hypertension in pregnancy. Key points include interpreting thrombocytopenia as a multifactorial sign, balancing bleeding and thrombosis risks, and delivering individualised multidisciplinary care.
References
- Andreoli L, Bertsias GK, Agmon-Levin N, Brown S, Cervera R, Costedoat-Chalumeau N, et al. EULAR recommendations for women’s health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome. Ann Rheum Dis. 2017;76(3):476-485. doi: 10.1136/annrheumdis-2016-209770.
- Sammaritano LR, Bermas BL, Chakravarty EE, Chambers C, Clowse MEB, Lockshin MD, et al. 2020 American College of Rheumatology Guideline for the Management of Reproductive Health in Rheumatic and Musculoskeletal Diseases. Arthritis Rheumatol. 2020;72(4):529-556. doi: 10.1002/art.41191.
- European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Autoimmune hepatitis. J Hepatol. 2015;63(4):971-1004. doi: 10.1016/j.jhep.2015.06.030. Epub 2015 Sep 1. Erratum in: J Hepatol. 2015;63(6):1543-4.
- Dalekos GN, Arvaniti P, Gatselis NK, et al. EASL Clinical Practice Guidelines on the management of autoimmune hepatitis. J Hepatol. 2025;82(1):179–210. doi: 10.1016/j.jhep.2024.10.020.
- Kaplan DE, Bosch J, Ripoll C, Thiele M, Fortune BE, Simonetto DA, et al. AASLD practice guidance on risk stratification and management of portal hypertension and varices in cirrhosis. Hepatology. 2024;79(5):1180–1211. doi: 10.1097/HEP.0000000000000647.
- Garcia-Tsao G, Abraldes JG, Berzigotti A, Bosch J. Portal hypertension and variceal bleeding: pathophysiology and management. Gastroenterology. 2017;153(3):656–674. doi: 10.1053/j.gastro.2017.06.051.
- James AH, Pacheco LD, Konkle BA. Management of pregnant women who have bleeding disorders. Hematology Am Soc Hematol Educ Program. 2023;2023(1):229-236. doi: 10.1182/hematology.2023000475.
- Gisbert JP, Chaparro M. Pregnancy in patients with inflammatory bowel disease and autoimmune hepatitis. World J Gastroenterol. 2023;29(15):2250-2265. doi:10.3748/wjg.v29.i15.2250.
