The hidden pathology: An autopsy diagnosis of unsuspected juvenile systemic lupus erythematosus.

Abstract

Juvenile-onset systemic lupus erythematosus (JSLE) is a severe autoimmune disorder that is rare but frequently manifests with multisystem involvement and can lead to substantial morbidity and mortality. We present the case of an 18-year-old female who was diagnosed with JSLE postmortem following sudden collapse and death. The patient was previously prescribed warfarin for benign intracranial hypertension and cerebral venous thrombosis (CVT). However, she had discontinued the medication months before her demise. She was never diagnosed with systemic lupus erythematosus (SLE) despite her medical history. Postmortem examination revealed several findings characteristic of SLE, including Libman-Sacks endocarditis, immune complex-mediated proliferative glomerulonephritis, pericarditis, and pleuritis. The diagnosis was further substantiated by positive anti-nuclear antibody (ANA) and anti-dsDNA antibodies satisfying the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria. This case emphasizes the diagnostic challenge of JSLE, particularly when it manifests with atypical symptoms, which may impede or obscure the diagnosis. The diagnosis was only determined postmortem following a comprehensive examination of the patient's organs and additional immunological tests. This report features the gross and microscopic features of JSLE with its typical postmortem findings. The case emphasizes the need for heightened clinical suspicion and early intervention in suspected cases of juvenile lupus to prevent fatal outcomes.