No abstract

Background:Systemic sclerosis (SSc) is a diffuse connective tissue disease characterized by varying degrees of cutaneous and visceral fibrosis, presence of autoantibodies and vasculopathy. In addition, central nervous system (CNS) involvements are often observed [1].Objectives:The aim of the present study was to investigate the presence of axonal dysfunction in systemic sclerosis (SSc) and to determine if clinical, laboratory and treatment features are associated with its occurrence.Methods:In this longitudinal study we included 38 SSc patients (32 woman, mean age of 50.86, SD±11.66 years; range 31- 74) and 38 healthy volunteers (32 woman, mean age of 49.23, SD±12.03 years; range 26 - 77). All individuals were evaluated (neuropsychiatric evaluation and MRI) at study entry and after 12 months. Cognitive evaluation was performed using the Montreal Cognitive Assessment (MoCA), mood disorders were determined through Beck’s Depression (BDI) and Beck’s Anxiety Inventories (BAI). Individual with scores: MoCA ≤26, BDI ≥11 and BAI ≥7 were considered impaired. SSc patients were further assessed for clinical and laboratory SSc manifestations, disease activity (Valentini Activity Index), severity activity (Medsger Severity Index). We performed multi-voxel ¹H-MRSI over the superior-posterior region of the corpus callosum. Our MRI/MRSI protocol consisted of: T1-weighted images; 2D pulse sequence (PRESS); excitation angle of 90°; Long TE: 144ms and TR: 2000ms; VOI (MRSI) size (mm) ≈ (116 x 79 x 16) grid with 208 spectra. Scans were performed with a Philips 3.0T MRI scanner. We measured signals from N-acetyl-compounds (NAA), creatine (Cr), choline (Cho), glutamate (Glu), glutamine (Gln) and Glx (the sum of Glu and Gln) using TARQUIN software.Statistics was performed according nature of the variable.Results:We observed a significant reduction in NAA/Cr (mean value=1.72; SD=2.4) and Cho/Cr (mean value=2.4; SD=2.04) ratio in SSc when compared to controls (NAA/Cr mean value=1.85; SD=2.7; p = 0.36; Cho/Cr mean value=0.377; SD=0.169; p<0.001). Reduction in NAA/Cr ratio was associated with cognitive impairment (p = 0.017), presence of migraine (p = 0.001), current use of prednisone (p=0.010) and current use of methotrexate (p < 0.001). NAA/Cr ratio correlated with MOCA scores (r=0.4; p=0,015).Follow up study showed a reduction in NAA/Cr values when compared to patients’ baseline values (p = 0.0341).Conclusion:Our results showed a significant reduction in NAA/Cr ratio in SSc patients associated with cognitive impairment and the use of some drugs (MTX and prednisone) during the treatment. Therefore, NAA/Cr ratios may be a useful biomarker in follow-up studies of SSc.References:[1] Hietaharju A, Jaaskelainen S, Hietarinta M, Frey H. Central nervous system involvement and psychiatric manifestations in systemic sclerosis (scleroderma): clinical and neurophysiological evaluation. Acta neurologica Scandinavica. 1993[2] Mountford, C. E., Stanwell, P., Lin, A., Ramadan, S., and Ross, B., Neurospectroscopy: the past, presente and future,” Chemica...

No abstract

Background/Purpose Proton magnetic resonance spectroscopy (1H-MRS) has been shown to be an important non-invasive tool to quantify neuronal loss or damage in the investigation of central nervous system (CNS) disorders. The purpose of this article is to discuss the clinical utility of 1H-MRS in determining CNS involvement in individuals with rheumatic autoimmune diseases. Methods This study is a systematic review of the literature, conducted during the month of November and December of 2019 of articles published in the last 16 years (2003-2019). The search for relevant references was done through the exploration of electronic databases (PubMed/Medline and Embase). We searched for studied including systemic lupus erythematosus (SLE), systemic sclerosis (SSc), juvenile idiopathic arthritis, rheumatoid arthritis (RA), psoriasis, Sjögren's syndrome (pSS), vasculitis and Behçet. Only studies published after 2003 and with more than 20 patients were included. Results We included 26 articles. NAA/Cr ratios were significant lower and Cho/Cr ratios increased in several brain regions in SLE, SS, RA, SSc. Associations with disease activity, inflammatory markers, CNS manifestations and comorbidities was variable across studies and diseases. Conclusion The presence of neurometabolite abnormalities in patients without ouvert CNS manifestations, suggests that systemic inflammation, atherosclerosis or abnormal vascular reactivity may be associated with subclinical CNS manifestations. MRS may be a usefull non-invasive method for screening patients with risk for CNS manifestations.

No abstract