Pregnancy Complicated by Systemic Lupus Erythematosus, Autoimmune Hepatitis and Portal Hypertension.

Abstract

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.

Learning points: Pregnancy in women with concurrent systemic lupus erythematosus (SLE), autoimmune hepatitis and portal hypertension is rare but can achieve favourable maternal and neonatal outcomes with individualized multidisciplinary care.Thrombocytopenia in this setting is often multifactorial (hypersplenism, immune-mediated, pregnancy-related). A stable platelet count above 50 ×10⁹/l without bleeding may allow expectant management rather than intervention.The bleeding-thrombosis paradox requires balancing prothrombotic tendencies (pregnancy, SLE) against bleeding risks (portal hypertension, varices, and thrombocytopenia). Low-dose aspirin may be used cautiously; low molecular weight heparin is not mandatory in antiphospholipid antibody-negative patients with significant bleeding risk.