Abstract

BackgroundSystemic sclerosis (SSc) is one of the most severe autoimmune rheumatic diseases with high morbidity and mortality rates 1. Peripheral neuropathy in SSc is reported with a broad prevalence variable between 0.01% to 28% 2. Developed in 1978, the Health Assessment Questionnaire Disability Index (HAQ-DI) for rheumatoid arthritis, nowadays is widely used to measure functional status in patients with SSc as well, but data are lacking in SSc patients with polyneuropathy (PN) 3.ObjectivesTo determinate the impact of PN on disability in SSc patients.MethodsThis was cross-section study in patients with SSc. Study was conducted in two University hospitals in Latvia for adults. Study included patients diagnosed with SSc that met the ACR / EULAR 2013 accepted classification criteria and have been consulted by rheumatologists between 1st January 2016 and 31st July 2021. Patients who agreed to comply in the study were evaluated by one rheumatologist and were surveyed and clinically evaluated according to The European Scleroderma Trials and Research group accepted domains. Disease duration was defined as time from first non-Raynaud SSc symptom to date of examination. SSc subtype was evaluated in each patient by clinical presentation that included modified Rodnan score (mRSS) as well: limited cutaneous systemic sclerosis (lcSSc) with skin thickening distal to the elbow/knee and diffuse cutaneous systemic sclerosis (dcSSc) with skin thickening proximal to the elbow/knee. For neuropathic pain assessment, the Neuropathic Pain Diagnostic Questionnaire (DN4) was used. Peripheral neuropathy symptoms were assessed using total neuropathy score (TNS). Peripheral nerve conduction study (NCS) and quantitative sensory testing (QST) was used to detect PN.Functioning and disability were assessed using HAQ-DI. All patients completed the HAQ-DI form in the same day as they were evaluated, filling the form in native language.ResultsFrom 109 acknowledged patients, 67 were able to come for a visit, 54 females, 13 males. Mean age for females 62.54±12.02 years, for males 58.69±14.50 years.After assessment by rheumatologist, 50 were classified as lcSSc, 17 were classified as dcSSc. The overall ratio of lcSSC and dcSSc was 50:17, in females 41:13, in males 9:4. Disease duration ranges from 1 to 39 years (mean 15.49±10.18), 16.61±10.47 years for females, 10.85±7.55 years for males. Large fiber PN was diagnosed in 47.8% of subjects by NCS (n=32/67) and small fiber PN in 40.3% of subjects by QST (n=27/67), while 11.9% had neither (n=8/67). Mean HAQ-DI score was 1.22. There was no statistically significant difference in HAQ-DI score between males (1.08±0.89) and females (1.26±1.01), (p=0.13). As well there was no difference between HAQ-DI score in patients with large fiber/small fiber neuropathy and without neuropathy symptoms (p=0.6817). But we found that neuropathy symptoms (assessed by TNS) have moderately strong and statistically significant correlation with HAQ-DI (r=0.39, p=0.0012). As well we found moderately strong correlation between HAQ-DI and neuropathic pain (assessed by DN4) (r=0.45, p=0.00015).ConclusionPN is more common complication in SSc that was previously reported, with significant impact on QoL.

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