Ischemic stroke as the first manifestation of systemic lupus erythematosus in a 13-year-old patient: a case report

Resumo

INTRODUCTION: Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease of unknown cause that is associated with substantial morbidity and mortality. Its pathophysiology involves interactions among genetic, environmental, and hormonal factors, resulting in heterogeneous clinical manifestations. Patients younger than 18 years account for 10%–20% of all SLE cases; compared with adults affected by the disease, they have a more severe phenotype and a less favorable prognosis. CASE REPORT: A 13-year-old girl presented to an emergency care unit accompanied by her mother with nonspecific symptoms, including odynophagia, productive cough, headache, and a low-grade fever for two days, followed by food refusal, behavioral changes, mental confusion, and difficulty speaking for one day. Physical examination showed mental confusion, right-sided central facial paresis, and monosyllabic speech, for a Glasgow Coma Scale score of 12. She was urgently transferred to the pediatric intensive care unit of a tertiary hospital for investigation of an acute neurologic deficit. Infectious, neoplastic, and ischemic etiologies were considered in particular; magnetic resonance imaging confirmed an ischemic etiology—an acute left-sided ischemic stroke. During the etiologic investigation of the acute ischemic event, the patient met clinical and laboratory EULAR/ACR classification criteria for SLE, confirming the diagnosis. She also developed complete and proportional right hemiparesis, appendicular ataxia, absence seizures, pericardial and pleural effusions, left cephalic vein thrombosis, nonhemolytic anemia, lupus psychosis, and acute kidney injury with nephrotic syndrome. SLE-specific treatment was initiated with methylprednisolone and cyclophosphamide pulse therapy, and prophylactic treatment for severe leukopenia was required. DISCUSSION: Pediatric SLE is rare and has a guarded prognosis, especially when ischemic stroke is the first manifestation. In presentations compatible with this condition, clinical suspicion is essential for early diagnosis and timely disease-specific intervention to minimize associated morbidity and mortality.