Title:Neuropsychiatric Systemic Lupus Erythematosus: A Remaining Challenge
Volume: 28
Issue: 11
Author(s): Daniel Mrak, Michael Bonelli*Helga Radner
Affiliation:
- Division of Rheumatology, Medical University of Vienna, Spitalgasse 23, 1090 Vienna, Austria
Keywords:
Central nervous system, peripheral nervous system, neuropsychiatric systemic lupus erythematosus, systemic lupus erythematosus, autoimmune disease, constitutional symptoms.
Abstract: Systemic Lupus Erythematosus (SLE) is an autoimmune disease, which affects a wide range of organs
with variable clinical features. Involvement of the nervous system is a challenging and multifaceted manifestation
of the disease, presenting with a broad range of symptoms. Neuropsychiatric lupus (NPSLE) encompasses
seven syndromes of the peripheral and 12 of the central nervous system, associated with a high disease
burden. Despite advances in the management of SLE, NP manifestations still pose a challenge to clinicians.
First, diagnosis and attribution of SLE are difficult due to the lack of specific biomarkers or imaging modalities.
Second, therapeutic options are limited, and evidence is mainly based on case reports and expert consensus, as
clinical trials are sparse. Moreover, no validated outcome measure on disease activity exists. Current recommendations
for treatment include supportive as well as immunosuppressive medication, depending on the type
and severity of manifestations. As NPSLE manifestations are increasingly recognized, a broader spectrum of
therapeutic options can be expected.
