A young lady with fits and bleeding; a case of acquired hypodysfibrogenemia and cerebral lupus in a patient with systemic lupus erythematosis

Introduction: Systemic lupus erythematosis (SLE) is a multisystem disorder. Although Anti-Nuclear Antibody (ANA) positivity is described as an essential criterion of diagnosis in the 2019 EULAR guideline, ANA negative lupus is recognized up to 2% of all SLE patients who otherwise fulfil clinical and immunological criteria.

 

Case Presentation: We describe a 20-year-old female diagnosed with ANA negative SLE who presented with renal SLE relapse and adult-onset seizures with high blood pressures; MRI was more in favour of cerebral lupus than posterior reversible encephalopathy syndrome, facilities to detect CSF anti ribosomal P antibodies were not available. During hospital stay, she developed severe bleeding manifestations with normal prothrombin and activated partial thromboplastin time, prolonged thrombin time and fibrinogen clauss with low normal fibrinogen levels which partially corrected with cryoprecipitate. Clot solubility was not performed. A diagnosis of acquired hypodysfibrinogenemia with possible factor X111 deficiency was made.

 

Discussion: ANA-negative SLE is a clinical rarity. Differentiation between cerebral lupus and pure hypertensive encephalopathy may be challenging. Acquired coagulation disorders, which may exhibit diverse clinical and biochemical presentations, are well recognized in SLE.