The patient was a 71‐year‐old white man with systemic lupus erythematosus who presented with confusion, gait impairment, and left‐sided visual neglect. A brain MRI showed multiple areas of ischemic infarct involving bilateral cerebral hemispheres and left cerebellum. Laboratory workup showed elevated ESR and CRP, decreased complement levels, thrombocytopenia, and positive lupus anticoagulant along with beta 2 glycoprotein1 antibodies. Transesophageal echocardiogram ruled out cardiac vegetations. Cerebral angiogram was negative for characteristic CNS vasculitis findings, however, showed thrombotic occlusion of multiple medium‐size vessels in bilateral hemispheres, suggesting presence of antiphospholipid antibody syndrome (APS). His hospital course was complicated by acute kidney injury with active urinary sediments for which renal biopsy was performed and revealed focal proliferative lupus nephritis (WHO class III). Based on these results, our patient was thought to have a combination of APS and active systemic small‐vessel vasculitis. He was started on high‐dose corticosteroids and monthly cyclophosphamide for his renal failure, with gradual clinical improvement. He was started on therapeutic anticoagulation for APS to prevent further vascular complications. He tolerated the treatment well and was discharged on chronic immunosuppressants and Coumadin anticoagulation to prevent recurrent thrombosis.
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder with various clinical presentations, including myriad neurological manifestations ranging from psychosis to frank stroke. It usually presents in young adults, with higher female predilection. The diagnosis is challenging, given the various possible etiologies of such presentations, including vasculitis, embolic disease, and thrombosis from antiphospholipid antibody syndrome. In patients who have a combination of the above etiologies, the clinical dilemma arises regarding the proper therapeutic approach. In addition to initiation of high‐dose immunosuppressive therapy, one of the most important considerations is regarding anticoagulation in patients with high risk of recurrent thrombosis, as well as risk of evolution of acute ischemic infarcts into catastrophic hemorrhagic lesions. We are presenting this rare case of coexistence of 2 autoimmune processes in an elderly male patient to stress the importance of prompt diagnostic evaluation and formulation of a therapeutic approach when facing important and time‐sensitive clinical dilemmas in the hospital setting.
Primary CNS antiphospholipid antibody syndrome is an unusual presentation in elderly patients with SLE. In patients with SLE who present with acute ischemic stroke, prompt diagnostic evaluation is necessary to differentiate between primary CNS vasculitis and APS because their therapeutic implications are largely different.
To cite this abstract:Wiegley N, Kalva N. Simultaneous Cns Antiphospholipid Antibody Syndrome and Lupus Nephritis in Systemic Lupus Erythematosus. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 310. https://www.shmabstracts.com/abstract/simultaneous-cns-antiphospholipid-antibody-syndrome-and-lupus-nephritis-in-systemic-lupus-erythematosus/. Accessed December 10, 2018.