Intracranial hypertension, nephritis and serositis complicating unexpected pregnancy in a lupus patient

Abstract

Background: Systemic lupus erythematosus (SLE) flare in pregnancy is challenging. The simultaneous occurrence of intracranial hypertension (IH) is unusual and raises the possibility of being a presentation of neuropsychiatric SLE. Aim of the work: We here discuss the presentation and management of a complicated case of lupus associated with IH, lupus nephritis (LN) and serositis in unplanned pregnancy. Case presentation: A 26-year old SLE patient presented 10 weeks pregnant with marked pleural effusion, hypertension, grade V papilledema, impaired renal functions, proteinuria (2.2 g/24 h), high anti-double stranded deoxyribonucleic acid and consumed complement (C3). Renal biopsy revealed LN (class IV and V). MRI brain demonstrated features consistent with IH and perimetry revealed early optic nerve affection. Ultrasound-guided pleurocentesis and relieving lumbar puncture were performed. Pulse methylprednisolone and cyclosporine A (CsA) (2 mg/kg/day) were provided. Acetazolamide was added to manage the IH and meticulous control of BP was achieved. In 2 weeks renal functions, proteinuria and papilloedema improved. The patient gave birth via vaginal delivery at full term to a normal healthy boy. On follow-up after delivery, proteinuria ranged from 100 to 300 mg/24 h with normal renal functions and fundus. The patient was maintained on CsA, hydroxychloroquine and tapering doses of steroids with no relapses. Conclusion: Pregnant SLE cases presenting with IH, LN and serositis are challenging concerning the management of SLE activity. IH may be a sign of lupus activity and its resolution coincides with the control of the flare. Lower dose CsA is potentially effective in treating LN in pregnancy with no reported side effects.