An Unusual Case of Idiopathic Intracranial Hypertension (IIH) Headache in 2022 That Later Led to the Diagnosis of Systemic Lupus Erythematosus (SLE) with Lupus Nephritis in 2025

Introduction: IIH typically affects young women and is characterized by elevated cerebrospinal fluid (CSF) pressure without an identifiable cause. However, secondary causes must be considered in atypical progression or recurrence cases. I present an interesting case where recurrent IIH-like symptoms led to the diagnosis of SLE with renal and central nervous system (CNS) involvement. Case Description: A young woman of African descent with sickle cell trait and IIH who had three lumbar punctures and a lumbar drain in the past presented to the ED with headache and transient visual obscuration. She was found to have spontaneous retinal venous pulsation. Extensive infectious, malignant, and thrombophilia screens were negative. She underwent a lumbar puncture, with high opening pressure, CSF analysis revealed markedly elevated protein with mild pleocytosis. Before this admission, her serological studies revealed high-titre ANA, positive Ro/La antibodies, and evidence of immune complex-mediated glomerulonephritis on renal biopsy (Class V lupus nephritis). A renal biopsy was done in the context of renal-rheumatology clinic outcome. Given the neurological symptoms, positive autoantibodies and renal findings, a diagnosis of SLE with CNS involvement was confirmed with an MRI of the head and spine showing meningoradiculitis. She received IV methylprednisolone followed by high-dose oral steroids, immunosuppression with mycophenolate mofetil and hydroxychloroquine, and escalation to intravenous cyclophosphamide was considered once neurology and rheumatology in agreement. Serial ophthalmological monitoring continues alongside neurological, rheumatological, and nephrology multidisciplinary team management. Discussion: This case highlights the diagnostic challenge in SLE when the neurological and renal manifestations develop sequentially. SLE in African and Caribbean descendants is likely overlooked due to co-existing other comorbidities such as hypertension, sickle cell anaemia pain, and social reasons such as being a foster child. A case-specific high index of suspicion for autoimmune disorders and early consideration of renal biopsies are important. In addition, I propose the widespread use of collaborative multi-speciality management including multi-consultant clinics in managing complex systemic autoimmune diseases.