Still's disease in pregnancy complicated by anakinra-related hepatotoxicity and rescued by intravenous immunoglobulin: a case-based review.

Still's disease (SD) in pregnancy is a rare autoinflammatory disorder presenting considerable diagnostic and therapeutic challenges. Balancing maternal disease control with foetal safety is essential, and evidence supporting biologic therapies - particularly interleukin-1 (IL-1) blockade - during gestation remains scarce. A 34-year-old primigravida developed de novo SD at 27 weeks' gestation with high-grade fever, evanescent maculopapular rash, polyarthralgia, neutrophilic leucocytosis, and hyperferritinaemia (peak 27,967 ng/mL; CRP 342 mg/L; WCC 18.1 × 10⁹/L; ALT 69 IU/L). Intravenous methylprednisolone, oral prednisolone, and subcutaneous anakinra (100 mg daily) achieved disease control, but anakinra was complicated by suspected drug-induced liver injury (ALT 1138 IU/L) with biochemical improvement after withdrawal. Intravenous immunoglobulin (IVIg; 0.4 g/kg/day for 5 days) was administered as rescue therapy. Caesarean delivery at 34 weeks resulted in a healthy neonate. Postpartum control was achieved with tocilizumab, which the patient self-discontinued after two years, remaining in drug-free remission. A CABARET-compliant search of PubMed/MEDLINE, EMBASE and DOAJ (to May 2026) identified 23 publications describing 29 pregnancies; only five referenced anakinra with two cases of new antenatal exposure with no complications, underscoring the paucity of evidence for IL-1 blockade in pregnancy. SD in pregnancy demands prompt multidisciplinary assessment, exclusion of mimics (Haemophagocytic Lymphohistiocytosis (HLH)/Macrophage Activation Syndrome (MAS), Haemolysis, Elevated Liver Enzymes, Low Platelets Syndrome (HELLP), sepsis, intrahepatic cholestasis of pregnancy), and vigilant monitoring for treatment-related toxicity. This case documents suspected anakinra-related hepatotoxicity and supports IVIg as a potential rescue option, with tocilizumab as a viable postpartum strategy.