Epidemiology and Treatment of Peripheral Neuropathy in Systemic Sclerosis

Abstract

Objective The epidemiology and treatment of peripheral neuropathy in systemic sclerosis (SSc) is poorly understood. The objectives of this study were to evaluate the incidence, prevalence, risk factors, and treatments of peripheral neuropathy in SSc. Methods A systematic review of Medline, Embase and CINAHL databases for literature reporting peripheral neuropathy in SSc was performed. Studies evaluating incidence, prevalence, risk factors, and treatments were synthesized. Meta-analysis using a random effects model was used to evaluate the prevalence of peripheral neuropathy. Results 113 studies reported 949 subjects with at least one type of peripheral neuropathy out of 2143 SSc patients studied. The mean age was 48.5 years. The mean time between SSc onset and detection of peripheral neuropathy was 8.85 years. The pooled prevalence of neuropathy was 27.4% (95%CI 22.4% - 32.7%). Risk factors for peripheral neuropathy in SSc included advanced diffuse disease, anticentromere antibodies, calcinosis cutis, ischemia of the vasa nervosum, iron deficiency anemia, metoclopramide, pembrolizumab, silicosis and uremia. There were 73 subjects with successful treatments (n=36 restoring sensation, n=37 restoring motor or sensorimotor function). Treatments included decompression surgery, prednisone, cyclophosphamide, carbamazepine, transcutaneous electrical nerve stimulation, tricyclic antidepressants and IVIG. Conclusion All-cause peripheral neuropathy is not uncommon in SSc. Compression neuropathies can be treated with decompression surgery. Observational data reporting immunosuppressive and anticonvulsants to treat peripheral neuropathy in SSc is limited and conflicting. This data provides the signal of effect to justify RCT to evaluate the efficacy of these interventions.

Management of Calcinosis Cutis in Rheumatic Diseases

“…(7) Lesions may be asymptomatic, or associated with pain, soft tissue swelling, ulcers with superimposed infections, or even deformities leading to functional disability (5). Calcinotic lesions can lead to compression neuropathies resulting in motor and/or sensory deficits (34). Ulceration of the overlying skin may occur, with a higher tendency in the forearm, elbows, fingers (particularly the volar aspect of fingertips), the metacarpophalangeal or the interphalangeal joints.…”

Prevalence of polyneuropathies among systemic sclerosis patients and impact on health-related quality of life

“…Furthermore, the materials and methods used in those studies provide a large range of results. A recent systematic review of 113 studies [4] showed a pooled prevalence of neuropathy involvement in 27.37% of cases, including 26% (n = 556/2,143) with SFN and 10.8% (n = 231/2,143) with LFN when neuropathies were assessed based on small and large fibres.…”

“…trigeminal neuropathy, carpal tunnel syndrome, ulnar nerve entrapment) and non-compression (e.g. sensorimotor neuropathy, sensory ataxic neuropathy, multiple mononeuropathies) neuropathies [4].…”

“…However, recent studies have shown that neurological involvement is fairly common. The prevalence of peripheral neuropathy in SSc ranges from 17% [6] to 40% [7], with a pooled prevalence close to 30% [3,4]. Probably due to the rarity of the disease, the methods used in these studies and the characteristics of the study groups, the results differ and the extent of peripheral nervous system involvement remains unclear.…”

High-resolution ultrasound of peripheral neuropathies in rheumatological patients: An overview of clinical applications and imaging findings

“…In regard to PNs, in a cross-sectional study by Raja et al ( 73 ) and Paik et al ( 74 ), the prevalence of PN in SSc varied from 28 to 36.6%. In a recent systematic review from AlMehmadi et al ( 75 ), compression neuropathies were reported in 26.5% of the studies, and according to Yagci et al ( 76 ) and Sriwong et al ( 77 ), the prevalence of median neuropathy in SSc is estimated to be 35%. Most CTS in patients with SSc were asymptomatic.…”