Background Therapeutic decisions in NPSLE are made on a patient-by-patient basis guided by the severity of symptoms and the suspected underlying pathogenetic mechanism. Evidence for therapeutic decisions is scanty as is data on clinical phenotypes in NPSLE.
Objectives To describe clinical phenotypes per suspected pathogenetic mechanism in NPSLE.
Methods The Leiden NPSLE clinic is a tertiary referral centre that evaluates patients suspected of NPSLE and leads to a prospectively collected database. Standardized evaluation including serological, imaging, clinical and neuropsychological testing is followed by a multidisciplinary consensus meeting. Diagnosis and therapeutic decisions based on the suspected pathogenetic mechanism are made by consensus of all participating medical specialists. We describe clinical phenotypes with sociodemographic and clinical characteristics per pathogenetic mechanism.
Results One hundred consecutive patients were evaluated in the Leiden NPSLE clinic. Three clinical phenotypes could be differentiated in primary NPSLE; 55% inflammatory NPSLE in patients who were advised immunosuppressive therapy, 32% ischemic NPSLE in patients who were advised anticoagulant therapy and 13% undefined NPSLE in patients who were advised symptomatic treatment. Age and disease duration in each group were 42 and 8, 47 and 9, 45 and 2 years, respectively. MRI abnormalities were present in 72%, 100% and 80% of respective phenotypes and SLEDAI scores were 10, 7 and 6. IgG anticardiolipin antibodies and lupus anticoagulans were present in respectively 33% and 48% of inflammatory and in 58% and 67% of ischemic NPSLE and were absent in undefined NPSLE patients. Twenty-five percent of ischemic NPSLE-patients reported transient ischemic attacks oppose to none in other groups. Cognitive dysfunction was present in 62% of inflammatory, 50% of ischemic and 20% of undefined NPSLE patients. As a fourth group secondary NPSLE was recognized in 11% of patients, in this group 75% of patients had a renal disorder and 88% of patients used steroids.
Conclusions To the best of our kowledge this is the first prospective evaluation of a standardized multidisciplinary assessment of neuropsychiatric symptoms in SLE-patients. A remarkable feature of inflammatory NPSLE is a high SLEDAI and high prevalence of cognitive dysfunction. Whereas in ischemic NPSLE IgG anticardiolipin antibodies and lupus anticoagulans are highly prevalent and patients are distinguished by the report of TIA’s, also in this group all patients had abnormalities on MRI of the brain.
Disclosure of Interest None Declared