Idiopathic Intracranial Hypertension as the Initial Manifestation of Systemic Lupus Erythematosus: A Case Report and Literature Review.

BACKGROUND Idiopathic intracranial hypertension (IIH) is an uncommon but serious neurological condition that can precede systemic lupus erythematosus (SLE). Early recognition is essential to prevent complications, such as permanent vision loss. In this report, we describe a case of IIH as the initial presentation of SLE and review the relevant literature. CASE REPORT A 23-year-old previously healthy Saudi woman presented with a 1-week history of persistent headache and blurred vision, along with fatigue and bilateral leg swelling. Neurological examination revealed papilledema and mild left abducens nerve palsy. Brain magnetic resonance imaging and magnetic resonance venography were normal. Laboratory workup showed pancytopenia, positive antinuclear antibody and anti-dsDNA antibodies, low complement levels, and proteinuria. She was given a diagnosis of SLE with lupus nephritis and presumed IIH. Treatment included high-dose corticosteroids, hydroxychloroquine, mycophenolate mofetil, and acetazolamide. While her symptoms initially improved, persistent papilledema and worsening proteinuria were noted during follow-up, indicating ongoing disease activity. CONCLUSIONS IIH can be an atypical and isolated early manifestation of SLE, especially in young women. This case underscores the importance of considering autoimmune etiologies in patients with unexplained intracranial hypertension. Prompt diagnosis and a multidisciplinary approach to treatment are critical to reducing the risk of vision loss and long-term neurological damage. Further research is needed to better understand the pathophysiological connection between SLE and IIH.