Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives

Abstract

Neuropsychiatric systemic lupus erythematosus (NPSLE) is a generic definition referring to a series of neurological and psychiatric symptoms directly related to systemic lupus erythematosus (SLE). NPSLE includes heterogeneous and rare neuropsychiatric (NP) manifestations involving both the central and peripheral nervous system. Due to the lack of a gold standard, the attribution of NP symptoms to SLE represents a clinical challenge that obligates the strict exclusion of any other potential cause. In the acute setting, management of these patients does not differ from other non-SLE subjects presenting with the same NP manifestation. Afterwards, an individualized therapeutic strategy, depending on the presenting manifestation and severity of symptoms, must be started. Clinical trials in NPSLE are scarce and most of the data are extracted from case series and case reports. High-dose glucocorticoids and intravenous cyclophosphamide remain the cornerstone for patients with severe symptoms that are thought to reflect inflammation or an underlying autoimmune process. Rituximab, intravenous immunoglobulins, or plasmapheresis may be used if response is not achieved. When patients present with mild to moderate NP manifestations, or when maintenance therapy is warranted, azathioprine and mycophenolate may be considered. When symptoms are thought to reflect a thrombotic underlying process, anticoagulation and antiplatelet agents are the mainstay of therapy, especially if antiphospholipid antibodies or antiphospholipid syndrome are present. Recent trials on SLE using new biologicals, based on newly understood SLE mechanisms, have shown promising results. Based on what we currently know about its pathogenesis, it is tempting to speculate how these new therapies may affect the management of NPSLE patients. This article provides a comprehensive and critical review of the literature on the epidemiology, pathophysiology, diagnosis, and management of NPSLE. We describe the most common pharmacological treatments used in NPSLE, based on both a literature search and our expert opinion. The extent to which new drugs in the advanced development of SLE, or the blockade of new targets, may impact future treatment of NPSLE will also be discussed.

Monitoring disease activity and severity in lupus

“…It has an annual incidence of 5 per 100,000 of the general population 1 . There are racial and ethnic variations, with higher rates reported in Black and Hispanic peoples 2 .…”

“…There are racial and ethnic variations, with higher rates reported in Black and Hispanic peoples 2 . Usually, this disease with protean manifestations has a remitting relapsing course; however, it has a tendency to vary from acutely progressive to chronic indolent forms 1,2 .…”

Anti-B cell therapy in patients with systemic lupus erythematosus

“…Использованные схемы лечения не имели различий по таким параметрам, как ответ на терапию, переносимость и нежелательные явления. Дли-тельные наблюдения больных с нейропсихическими прояв-лениями СКВ на фоне терапии РТМ отсутствуют, в настоя-щее время РТМ используется в качестве терапии второй ли-нии у пациентов с острой, рефрактерной к стандартной те-рапии СКВ с нейропсихическими проявлениями [44]. Е.М.…”

Feasibility of hydroxychloroquine adjuvant therapy in pregnant women with systemic lupus erythematosus

“…Systemic Lupus Erythematosus (SLE) is a common autoimmune disease with poorly understood pathogenesis [1]. SLE has diversified clinical manifestations and often implicates organ systems, such as skin, kidney, joints, and nervous centralis [2].…”