Atypical Presentations of Systemic Lupus Erythematosus by Cerebral Venous Thrombosis and Generalized Lymphadenopathy without Antiphospholipid Antibodies: Case Report

Abstract

Introduction: Atypical presentations of systemic lupus erythematosus SLE) with features outside of the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria can make the diagnosis of SLE elusive. Case Presentation: We describe a case of a healthy 31-year old female who presented with syncope preceded by progressive headache, serositis, and generalized lymphadenopathy. CT-head and MR-venogram confirmed a cerebral venous thrombosis (CVT). Subsequent workup revealed high titers of antinuclear (ANA), anti-double stranded DNA (anti-dsDNA), anti-Smith (anti-Sm) antibodies, as well as low complement levels, lymphopenia and neutropenia. Diagnosis of SLE was confirmed by SLICC classification criteria. Antiphospholipid antibodies (APLA) were negative. While in hospital, she sustained a seizure secondary to the CVT. Conclusion: We discuss considerations for atypical SLE presentations by CVT (without APLA syndrome) and generalized lymphadenopathy, and the nuances of SLE diagnosis using existing classification criteria.