Immunosuppressive therapy of systemic lupus erythematosus associated peripheral neuropathy: A systematic review

Abstract

Introduction Peripheral neurologic manifestations may be associated with most of the collagen vascular diseases including systemic lupus erythematosus (SLE), yet most of the times it is not clear what therapy should be prescribed. EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations suggest the use of glucocorticoids and immunosuppressive agents for the treatment of SLE associated peripheral neuropathy (PN) (strength of statement A, category of evidence 1), however these recommendations are based on studies that did not focus specifically on PN but rather on neuropsychiatric manifestations of SLE out of which only one was a randomized controlled clinical trial that included 7 patients with peripheral neuropathy. The objective of this systematic review is to determine whether the pathogenic treatments (corticosteroids, immunosuppressive agents, intravenous immunoglobulins, plasmapheresis) are effective for SLE associated PN. Methods We searched MEDLINE for all the studies that included the pathogenic treatment of SLE associated PN. The purpose was to identify randomized clinical trials, and in the absence of these, we included observational studies and case reports or case series. Results The search returned only retrospective case reports or case series. Only one prospective study, a randomized controlled study, was focused on neuropsychiatric SLE and included few patients with PN (7). Some studies reported cases of PN responsive to glucocorticoids (GC), cyclophosphamide (CYC), rituximab (RTX), azathioprine (AZA), plasmapheresis (PPH), intravenous immunoglobulin (IVIG), mycophenolate mofetil (MMF) or different combinations of these immunosuppressive agents, whereas others noticed effectiveness of sequential treatments (i.e. administration of a therapeutic agent after another single agent or a combination of agents had previously failed). Many studies did not mention how the outcomes were objectively measured. Conclusions There are no interventional studies dedicated to the SLE associated PN, only retrospective case reports or case series which not only did they show contradictory results, but they also represent the lowest level of evidence. There is a strong need for new analytical studies dedicated to SLE associated PN. Protocol registered with PROSPERO (number CRD42019121748).

Three cases of systemic lupus erythematosus presenting with ischemic stroke as the initial symptom: Case reports and literature review

“…For patients with a confirmed diagnosis of NPSLE, current treatment options include glucocorticoids (GCs), immunosuppressants, biologics, intravenous immunoglobulins (IVIg), plasma exchange, and anticoagulant therapy 30–33 . Among the 20 cases reported in our study, only 12 reported treatment regimens.…”

Peripheral Neuropathy in Systemic Autoimmune Rheumatic Diseases—Diagnosis and Treatment

“…As for intravenous immunoglobulin, the previously mentioned systematic review reported good responses for all the five patients analyzed. No conclusions could be drawn from cyclophosphamide, and rituximab was effective in six out of the eight cases reported [43]. Finally, one retrospective study showed good results with azathioprine [34].…”

“…Methylprednisolone or cyclophosphamide are considered as second-line treatments [6]. A systematic review for SLE-associated PN reported similar treatments, including glucocorticoids in different dosages [43]. Because they were used frequently with other immunosuppressive drugs, it is hard to grasp their real efficiency.…”

The Challenge of Neuropsychiatric Systemic Lupus Erythematosus: From Symptoms to Therapeutic Strategies

“…Peripheral neuropathy is quite common and be challenging to attribute to SLE. SLE patients also have high rates of small fiber neuropathies [ 15 ].…”