Large fiber peripheral neuropathy in systemic sclerosis: A prospective study using clinical and electrophysiological definition

Abstract

Aim The reported prevalence of peripheral neuropathy in systemic sclerosis (SSc) is variable between 0.01% to 28%, probably due to differences in sample size, study design and population. Our aim is to determine the prevalence of large fiber peripheral neuropathy in SSc and to identify any contributing factors. Method A prospective cross‐sectional study of 60 SSc patients were evaluated for large fiber neuropathy using the modified clinical Total Neuropathy Score (cTNS) and nerve conduction study (NCS) of the upper and lower limbs. A combination of clinical (cTNS score ≥ 2) and NCS criteria (≥2 abnormal nerves including 1 sural [symmetrical polyneuropathy] and NCS abnormalities consistent with individual nerves/nerve roots [focal neuropathy]) was used to diagnose peripheral neuropathy. Results The majority had limited cutaneous subset (75%). Mean age was 55.73 (SD ± 13.04) years and mean disease duration was 8.61 (SD ± 8.09) years. Twenty‐two (36.7%) had combined clinical and NCS criteria for peripheral neuropathy, 14 (23.3%) with symmetrical polyneuropathy and 8 (13.3%) with focal neuropathy. Symmetrical polyneuropathy patients had significantly lower hemoglobin levels (11.2 vs. 12.35 g/L; P = .047). Serum vitamin B12 levels were normal, therefore excluding vitamin B12 deficiency. No other associations were found for both polyneuropathy and focal neuropathy with demography, co‐morbid diseases and SSc disease factors such as Raynaud's phenomenon and modified Rodnan skin score. Conclusion Large fiber neuropathy is common in SSc patients, which could contribute to non‐lethal burden in SSc with sensory loss and muscle weakness. Apart from lower hemoglobin in polyneuropathy, there were no associations with disease‐specific features or co‐morbid diseases.

Prevalence of various forms of peripheral neuropathy in patients with systemic connective tissue diseases: A clinical and electrophysiological study

“…There were 39 patients with a limited disease form in our study, while diffuse disease was found in 17 patients. Raja et al (42) determined the prevalence of large fiber peripheral neuropathy in the group of 60 patients diagnosed with SSc and their results showed that 22 patients (36,7%) had peripheral neuropathy, which is in correlation with the results of our study. Since in the studied population axonal neuropathy was seen at a higher percentage, for which low amplitudes were specific electrophysiologically, our aim was to investigate the measure of the impact of disease activity and disease duration on the reduction of amplitude since it was the most important parameter in the initial neuropathy stages in autoimmune diseases.…”

Peripheral Neuropathy in Diabetes Mellitus: Pathogenetic Mechanisms and Diagnostic Options

“…Nerve conduction studies (NCS) are the gold standard exam for diagnosing DPN [ 119 ]. NCS allows an evaluation of the large fibers (myelinated alpha and beta), while with this tool it is not possible to identify the function of the small fibers (small myelinated Aδ-fibers and unmyelinated C-fibers) [ 120 , 121 ]. The application of skin electrodes permits nerve stimulation in μV and the detection of electrical nerve activity through a series of parameters that are useful for diagnosing nerve and muscle fiber pathologies [ 120 , 122 , 123 , 124 , 125 ].…”

Prevalence and impact of foot peripheral neuropathy in Sytemic Sclerosis (SSc): results from a single centre cross-sectional study

“…In fact, there are studies that identify dcSSc and anti-centromere positivity as major risk factors for peripheral neuropathy [4,30], whereas others found a significant association with lcSSc and positivity for anti-topoisomerase I and anti-U1-RNP [9,31]. Although, there are also studies that have not highlighted a particular influence of cutaneous involvement or autoantibody profile [6,32]. In this regard, larger cohort, multicentre studies are needed to confirm association with specific diseae characteristics.…”