X CHIA1, D CORDATO1, A VENKAT1, S SPICER1
1Liverpool Hospital, Sydney, Australia
Background: Idiopathic intracranial hypertension (IIH) is a condition of unknown aetiology characterized by papilloedema due to increased intracranial pressure. Obese women of child-bearing age are at higher risk. IIH has also been well described in patients with systemic lupus erythematosus (SLE), and in particular, those with lupus nephritis (LN), heavy proteinuria, and haematological complications of SLE. The exact relationship of IIH to SLE/LN remains unknown.
Case Report: An obese 23-year-old south-Asian woman (BMI 32) developed severe generalised headache on a background of a 12-month history of Type V (membranous) LN. Treatment of LN with high-dose oral prednisolone and mycophenolate mofetil had been ineffective. Cyclosporine was added, achieving partial remission, with serum albumin reaching 25g/L. Despite this, she acutely developed Evan’s syndrome (thrombocytopaenia and haemolytic anaemia) in conjunction with headache, photophobia and mild visual blurring. She was normotensive, and thrombotic microangiopathy was excluded. She was treated with pulse intravenous methylprednisolone, intravenous immunoglobulin, and rituximab with favourable responses in platelet count and haemoglobin levels, but her headache and visual symptoms persisted. Retinal examination revealed bilateral papilloedema. Cerebral MRI including venography and angiography was normal. Lumbar puncture (LP) revealed an elevated opening pressure of >34 cmH2O and cerebrospinal fluid analysis was unremarkable. She was treated with acetazolamide and therapeutic LPs, leading to headache resolution. She remains on azetazolamide with resolution of headaches and visual symptoms.
Conclusion: Patients with SLE/LN may develop IIH, particularly during periods of active disease. The presence of headache and visual symptoms in these patients should prompt retinal examination, imaging and LP to exclude intracranial hypertension. Treatment is crucial to prevent the potential long-term sequelae of permanent vision loss
Graduated from University of Melbourne in 2011, then worked in Melbourne and Canberra before going to Liverpool Health in Sydney for Renal Advanced Training. Currently completing final year of advanced training at St George Hospital and Prince of Wales Hospital.