Abstract
Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease with a broad clinical spectrum, with 14-95% of patients presenting with neurological manifestations. At the neurological level, patients may present with intracranial hypertension (ICH), manifested by headache, nausea, vomiting, papilledema, decreased visual acuity, and cranial nerve palsies. Despite its clinical relevance, ICH is not classified as a specific neuropsychiatric manifestation of SLE by the American College of Rheumatology (ACR).
Case presentation: We report the case of a preadolescent girl with systemic signs, cutaneous involvement, and neurologic deterioration at the onset of SLE. She developed papilledema as a manifestation of ICH. Although it has been reported that most patients with ICH and SLE respond favorably to treatment with corticosteroids and acetazolamide as first-line therapy, in this case the response was inadequate. The patient received corticosteroids and immunomodulators, but due to the persistence of neurological symptoms, cyclophosphamide was added, which eventually resulted in a significant improvement in her condition.
Discussion: This case is relevant due to the few pediatric reports of SLE presenting with ICH. It highlights the presentation of ICH syndrome in the debut of autoimmune disease and emphasizes the importance of increased surveillance for this neurological condition in the early diagnosis and management of pediatric SLE.
Conclusion: This report, together with the findings in the literature, leads us to consider ICH as a potential neuropsychiatric manifestation of SLE.
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