Nouran Abaza

Vitamin D Deficiency in Egyptian Systemic Lupus Erythematosus Patients: How Prevalent and Does It Impact Disease Activity?

BACKGROUND The emerging role of vitamin D in immunology and autoimmune disorders has been a worldwide interest in the last decade. Systemic lupus erythematosus (SLE) patients are particularly at a delicate position predisposing them to suffer from vitamin D deficiency due to the multiple risk factors accompanying the disease. Whether vitamin D deficiency is also involved as a risk factor for developing SLE and affecting its course is a considerable concern. OBJECTIVES The objective of this study was to estimate the prevalence of vitamin D deficiency in SLE patients and its relation to disease. MATERIALS AND METHODS In our observational cross-sectional study, serum levels of vitamin D [25(OH)D] in 60 SLE patients and 30 age- and sex-matched healthy controls were assessed and estimated for deficiency and insufficiency at 10 and 30 ng/mL, respectively. Disease activity was evaluated by SLE disease activity index (SLEDAI), irreversible organ damage by Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR DI), and severity by Severity of Disease Index. Fatigue was measured by visual analog scale. RESULTS Significantly lower levels of 25(OH)D were found in SLE patients (17.6 ± 6.9 ng/mL) in comparison to controls (79.0 ± 28.7 ng/mL), with a statistically high significant difference (t = −11.2, P < 0.001). High prevalence of vitamin D insufficiency and deficiency was detected as 73.3% and 23.3%, respectively. Vitamin D had a highly significant negative correlation with SLEDAI (r = −0.495, P < 0.001), SLICC (r = −0.431, P < 0.05), and fatigue (r = −0.436, P < 0.05). CONCLUSION Vitamin D deficiency and insufficiency were found to be prevalent in SLE patients in our study and related to disease activity and fatigue. If needed, routine screening and consequent repletion of vitamin D are recommended in SLE patients. Restoring adequate vitamin D levels in SLE patients should be more explored as a potential yet simple measure to their usual management to improve their condition.

Anti-dsDNA titre in female systemic lupus erythematosus patients: relation to disease manifestations, damage and antiphospholipid antibodies:

Background Attempts are ongoing to unveil unresolved queries about anti-double-stranded deoxyribonucleic acid (anti-dsDNA), their precise pathogenic effects and to what extent blocking them would be a useful therapeutic goal. Objectives The aim of the present study was to determine the anti-dsDNA antibodies titre in systemic lupus erythematosus (SLE) patients and investigate their relation to the disease characteristics, activity, damage and antiphospholipid autoantibodies (aPL). Methods Seventy female SLE patients and 35 age- and sex-matched controls were included. The anti-dsDNA level and aPL were measured. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) were assessed. Results The mean age of the patients was 27.5 ± 5.1 years, disease duration 7.7 ± 5.4 years, and SLEDAI and SLICC/ACR-DI scores were 6.8 ± 8.04 and 1.2 ± 1.3, respectively. Anti-dsDNA was positive in 61.4% of the patients and the titre (133.2 ± 100.5 IU/ml) was significantly higher compared to controls (22.03 ± 17.2 IU/ml) (p < 0.0001). The anti-dsDNA level was significantly increased in those with musculoskeletal manifestations (p = 0.007) and positive anti-β2 glycoprotein (anti-β2GP) (p = 0.037) and decreased in those with neuropsychiatric manifestations (p = 0.004) and those receiving cyclophosphamide (CYC) (p = 0.013). The anti-dsDNA level tended to be higher in active patients. The anti-dsDNA titre significantly correlated with the erythrocyte sedimentation rate (p = 0.001), anticardiolipin IgG and IgA antibodies (p = 0.008) and anti-β2GP IgG (p = 0.03) and IgA (p = 0.002) and inversely with the total leucocytic count (p < 0.0001) and SLICC/ACR-DI (p = 0.001). Conclusion Anti-dsDNA is remarkably increased in SLE patients especially those with musculoskeletal manifestations and aPL. A protective role seems likely in those with neuropsychiatric manifestations and those receiving CYC and may form a shield against disease tissue damage.

Cutaneous vasculitis in systemic lupus erythematosus patients: potential key players and implications:

Objectives The aim of the present work was to study the clinical characteristics of cutaneous vasculitis (CV) in systemic lupus erythematosus (SLE) patients and find possible potential key players in its development and implicated associations with the disease manifestations. Patients and methods Fifty adult female SLE patients underwent full history taking, thorough clinical examination and laboratory investigations. The SLE Disease Activity Index (SLEDAI) and accumulated damage using the Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SLICC/ACR DI) were assessed. Results The mean age of the patients was 29.1 ± 6.1 years and was significantly lower in those with CV (p = 0.018). The disease duration was 4.9 ± 3.7 years. CV was present in 30% of the patients. Musculoskeletal manifestations and hypocomplementemia were present in all patients with CV. The SLEDAI and SLICC/ACR DI tended to be higher in those with CV. Complement (C3 and C4) was significantly consumed in CV patients (p < 0.0001). Antiphospholipids were comparable between those with and without CV. Lupus nephritis, cardiovascular manifestations and Sjögren syndrome were significantly linked to the development of CV (p = 0.025, p = 0.023 and p < 0.0001, respectively). Both C3 and C4 showed a high sensitivity (93.3% and 86.7%) to detect CV in SLE at cut-off values below 81.4 mg/dl and 16.8 mg/dl, respectively. Conclusion CV is closely related to hypocomplementemia but not to antiphospholipids and is associated with lupus nephritis, musculoskeletal manifestations and Sjögren syndrome.

The second lumbrical-interossei latency difference in carpal tunnel syndrome: Is it a mandatory or a dispensable test?

Abstract Objective To assess the value of the 2L-INT latency difference in the electrodiagnosis of the carpal tunnel syndrome (CTS) and evaluate its sensitivity in comparison to other routine median motor and sensory studies. Methods The study was conducted on 100 hands with symptoms and signs suggestive of CTS and 100 non-CTS hands as the control group. All were subjected to routine median motor nerve conduction study with stimulation at midpalm, wrist and elbow, median-versus-radial sensory comparison study and Second lumbrical-versus-interosseus (2L-INT) motor comparison study. Results The results showed that the most sensitive tests were the median-radial sensory test and the 2LINT test and that both were correlated suggesting that the motor fibers of the median nerve can be compressed as early as sensory fibers. Conclusion The 2L-INT test is as sensitive and important as the median-radial sensory test. Significance We recommend the routine use of the 2L-INT test in clinically suspected cases of CTS especially in cases where routine median motor studies are normal together with the median-radial sensory test even if the sensory studies are normal.

Serum Calprotectin in Rheumatoid Arthritis: A Promising Diagnostic Marker, How Far Is It Related to Activity and Sonographic Findings?

Background: In the past 2 decades, there has been increasing interest in calprotectin. It is released and detected in serum and body fluids as a potentially useful clinical inflammatory marker. The protein has been described in synovial tissue in rheumatoid arthritis (RA) patients, specifically in the lining layer adjacent to the cartilage–pannus junction, which is the primary site of cartilage destruction and bone erosion. Assessment of inflammatory activity in RA is of pivotal importance for the optimal treatment. Our aim in this study is to measure the serum calprotectin levels in RA patients and to assess its association—if there is any—with disease activity score and radiological findings using the musculoskeletal ultrasound. Patients and methods: In our case control study, we included 44 RA patients (Group I) and 20 age- and sex-matched healthy volunteers who served as the control group (Group II). Both groups were subjected to full history taking and thorough clinical examination. Assessment of RA disease activity state was done for all RA patients using the Disease Activity Score 28. Laboratory investigations included the measurement of complete blood cell count, erythrocyte sedimentation rate, C-reactive protein, rheumatoid factor, anticitrullinated peptide antibodies, kidney, liver functions; serum calprotectin levels were determined using enzyme-linked immunosorbent assay and radiological joint assessment was done using musculoskeletal ultrasound score. Results: There was a statistically significant elevation of serum calprotectin levels among RA patients when compared with healthy controls. Statistically significant correlations were also found between serum calprotectin and the ultrasound grading score, Disease Activity Score 28, and erythrocyte sedimentation rate, which reflect the degree of inflammatory activity in the affected joints in RA patients. Moreover, the study yielded a significant correlation between serum calprotectin levels and rheumatoid autoantibodies (rheumatoid factor and anticitrulli-nated peptide antibodies), which are strong predictors of the aggressiveness of the disease. Serum calprotectin at a cutoff level of 93.9 μg/dL had 88.6% sensitivity and 100% specificity for diagnosis of RA. Conclusion: Calprotectin was found to have high association with laboratory and ultrasonography markers of inflammation in RA patients, so it is recommended for use as a marker of inflammatory activity in RA patients especially for the follow-up of patients on biological therapy to assess its efficacy.

Verification of an ultrasonographic scoring system in discriminating rheumatoid arthritis from osteoarthritic and normal joints in an Egyptian cohorts

Background The use of musculoskeletal ultrasound in rheumatoid arthritis (RA) has been growing over the last decades mainly to monitor response to treatment and for early detection of erosions. Suggestions to include this technique in the diagnosis of RA have been made, but not yet been implemented (because of the lack of specific sonographic criteria for RA). Objectives To verify the performance of a proposed combined structural and synovial scoring system in differentiating RA from osteoarthritis (OA) and healthy sonographic findings in the small joints of the hand. Patients and methods Twenty RA patients, 20 patients with hand OA, and 10 healthy controls were subjected to musculoskeletal ultrasound of the metacarpophalyngeal and proximal interphalyngeal joints. The novel proposed scoring system was applied characterizing each joint as either RA supported or RA unsupported. Grading of synovitis as mild, moderate, or severe was also performed. In the RA group, disease activity was assessed by Disease Activity Score 28 (DAS28) and anticyclic citrullinated peptide serum levels were measured. Results When one or more RA-supported joints were detected using this scoring system, it had a sensitivity of 100.0% and a specificity of 83.0%, with a diagnostic accuracy of 90.0%, for the diagnosis of RA. If two or more joints were detected, it had a sensitivity of 95.0% and a specificity of 96.7%, with a diagnostic accuracy of 96.0% for the diagnosis of RA. Conclusion The novel suggested combined structural and synovial scoring system showed high performance in differentiating RA from OA and controls.

AB1079 Verification of an Ultrasonographic Scoring System in Discriminating Rheumatoid from Osteoarthritic and Normal Joints in an Egyptian Cohort

Background Musculoskeletal ultrasound (MSUS) use in Rheumatoid arthritis (RA) has been growing over the last decades mainly to monitor response to treatment and for early detection of erosions. Suggestions to involve this technique in RA diagnosis were taking place but not yet implicated (due to absence of specific sonographic criteria confined to RA). Objectives To verify a proposed combined structural and synovial scoring systems (designed by Kunkel et al., 2012) performance in discriminating RA from osteoarthritis and healthy sonographic findings in small joints of the hand. Methods Twenty RA patients, 20 hand OA patients and 10 healthy controls were subjected to MSUS to metacarpophalyngeal (MCP) and proximal interphalyngeal (PIP) joints. The novel proposed scoring system was applied characterizing each joint as either RA-supported (according to presence of synovial thickening>2mm, doppler signal and/or erosion) or RA-unsupported. Grading of synovitis as mild, moderate or severe was also applied. In RA group, disease activity was assessed by DAS28 and Anti-CCP serum levels were measured. Results Upon applying this scoring system, high statistically significant difference was found between study groups (Table 1) as regards presence of synovium>2mm, doppler signal (Figure 1) and erosions. When one or more RA-supported joints were detected, this scoring system had a sensitivity of 100.0% and specificity of 83.0% with diagnostic accuracy of 90.0% for diagnosis of RA. If two or more joints were detected, it had a sensitivity of 95.0% and specificity of 96.7.0% with diagnostic accuracy of 96.0% for diagnosis of RA. Table 1. Comparison between study groups as regards to number of RA-supported joints by US RA OA Control P^ P^ P^ RA/OA RA/Control OA/Control Erosion Median (IQR) 0.0 0.0 0.0 0.014* 0.014* 0.309 (0.0–1.8) (0.0–0.0) (0.0–0.0) Range 0.0–5.0 0.0–2.0 0.0–0.0 Doppler Median (IQR) 1.0 0.0 0.0 <0.001** 0.005* 1.000 (0.0–2.0) (0.0–0.0) (0.0–0.0) Range 0.0–3.0 0.0–0.0 0.0–0.0 Synovim >2mm Median (IQR) 3.0 0.0 0.0 <0.001** <0.001** 1.000 (2.3–4.0) (0.0–0.0) (0.0–0.0) Range 1.0–8.0 0.0–2.0 0.0–1.0 Total Median (IQR) 3.0 0.0 0.0 <0.001** <0.001** 0.476 (2.3–4.0) (0.0–0.0) (0.0–0.0) Range 1.0–8.0 0.0–2.0 0.0–1.0 IQR: Interquartile range. ^Mann Whitney test,* Significant,** Highly significant. Figure 1. Positive Doppler signal in right third MCP joint in an RA patient. Conclusions The novel suggested combined structural and synovial scoring system showed high performance in differentiating RA from OA and controls. References Kunkel GA, Canon GW and Clegg DO: Combined Structural and Synovial Assessment for Improved Ultrasound Discrimination of Rheumatoid, Osteoarthritic, and Normal Joints: A Pilot Study. The Open Rheumatology Journal 2012;6: 199-206. Scheel AK, Hermann KG, Kahler E, Pasewaldt D, Fritz J, Hamm B, Brunner E, Müller GA, Burmester GR, Backhaus M: A novel ultrasonographic synovitis scoring system suitable for analyzing finger joint inflammation in rheumatoid arthritis. Arthritis Rheum 2005; 52: 733-43.12. Disclosure of Interest None declared

FRI0417 Vitamin D Deficiency Prevalence in Systemic Lupus Erythematous; Regardless A Cause or a Consequence does it Impact Disease Course, Activity or Severity?

Background Emerging role of vitamin D in immunology and autoimmune disorders has been a worldwide interest in the last decade. Systemic lupus erythematosus (SLE) patients are particularly at a delicate position predisposing them to suffer from vitamin D deficiency due to the multiple risk factors accompanying the disease. Whether vitamin D deficiency is also involved as a risk factor for developing SLE and affecting its course is a considerable concern. Objectives To estimate prevalence of vitamin D deficiency in SLE and its relation to disease activity and severity. Methods In our observational cross-sectional study, serum levels of vitamin D from 60 SLE patients and 30 age & sex matched healthy controls (HC) were assessed and estimated for deficiency or insufficiency at 10 and 30ng/ml respectively. Disease activity was evaluated by SLEDAI, irreversible organ damage by SLICC/ACR index and severity by Lupus SDI. Fatigue was measured by visual analogue scale (VAS). Results Significantly lower levels of 25(OH)D were found in SLE patients (17.6±6.9 ng/ml) in comparison to controls (79.0±28.7 ng/ml)with statistically high significant difference (t=-11.2, p<0.001).High prevalence of vitamin D insufficiency and deficiency was detected 73.3% & 23.3% respectively. Vitamin D had a highly significant negative correlation with SLEDAI (r =-0.495, p<0.001), see figure 1a, SLICC (r =- 0.431, p<0.05) as well as fatigue (r =- 0.436, p<0.05), see figure 1b. After standardization of all clinical variants, regression analysis study showed that there is significantly inverse correlated between vit D and VAS (standardized regression coefficient β=-0.443, p=0.024) and a highly significant correlation between vit D and SLEDAI score (β=-0.940, p=0.012). Figure 1. a. Correlation between serum vitamin D and SLEDAI. b. Correlation between serum vitamin D and VAS of fatigue. Conclusions Vitamin D deficiency & insufficiency were found to be prevalent in SLE patients in our study and related to disease activity & fatigue. Routine screening and consequent repletion of vitamin D if needed is recommended in SLE. Restoring adequate vitamin D level in SLE should be more explored as a potential and simple yet valuable measure to be added to their usual management to alleviate their condition. References Kamen DL, Cooper GS, Bouali H, Shaftman SR, Hollis BW, Gilkeson GS. Vitamin D deficiency in systemic lupus erythematosus. Autoimmun Rev 2006; 5: 114–117. Ruiz-Irastorza G, Egurbide MV, Olivares N, Martinez-Berriotxoa A, Aguirre C. Vitamin D deficiency in systemic lupus erythematosus: prevalence, predictors and clinical consequences. Rheumatology (Oxford) 2008;47:920–3. Fragoso TS, Dantas AT, Marques CD, Rocha Junior LF, Melo JH, Costa AJ, Duarte AL. 25-Hydroxyivitamin D3 levels in patients with systemic lupus erythematosus and its association with clinical parameters and laboratory tests. Rev Bras Reumatol. 2012 Jan-Feb;52(1):60-5 Disclosure of Interest None declared

Effect of endorectal pullthrough on external anal sphincter integrity (in cases of Hirchsprung’s disease) using EMG

ObjectiveThe transanal mucosectomy of the aganglionic segment is a critical step in the transanal endorectal pullthrough procedure for the treatment of Hirchsprung’s disease. It exerts considerable traction on the anorectal tissue during dissection. Anal sphincter electromyography (EMG) is an indispensable parameter for the diagnosis of patients with any anorectal dysfunction.The aim of our study was to assess the integrity of the anorectal sphincter after transanal endorectal pullthrough using anal EMG. MethodsThis prospective study was carried out on 25 infants and children with Hirchsprung’s disease who underwent the endorectal pullthrough (soave) procedure. Needle EMG was used to assess the sphincter preoperatively and postoperatively. ResultsPreoperative EMG showed positive neuropathic changes in 28% of the patients. Postoperative EMG showed neuropathic changes in 60% of the patients, of whom 28% showed preoperative changes and 32% showed absolute postoperative findings, mostly related to difficult operative dissection. ConclusionThe functional results of the endorectal pullthrough procedure were acceptable overall. SignificanceThe reduced sphincter function encountered postoperatively was because of a combination of preoperative and intraoperative influences.

THU0191 Visfatin in patients with lupus nephritis. Correlation with disease activity

Background Renal involvement affects about 50% of SLE patients accounting for significant morbidity and mortality in these patients[1]. The adipokine “Visfatin” acting as a growth factor for B-lymphocyte-precursors, exerts several proinflammatory functions[2]. It was demonstrated as a marker of endothelial dysfunction (ED) in chronic kidney disease (CKD) thus could be a factor linking inflammation in SLE and kidney disease[3]. Objectives To assess serum visfatin level in patients with lupus nephritis (LN)and its correlation to disease activity in these patients. Methods The present study included 40 patients with SLE and forty age and sex matched healthy subjects served as control group.Oral consent was obtained from all patients and controls after a full explanation of the study.Clinical assessment od disease activity by SLE Disease activity index (SLEDAI)[4].Lupus nephritis was assessed clinically with the renal SLE disease activity index (renal SLEDAI).Renal biopsies were taken from the patients with LN and were classified according to the modified WHO classification [5].Serum level of visfatin were measured for the patients and controls using enzyme-linked immunosorbent assay (ELISA). Results A significantly higher serum visfatin level was found on comparing SLE patients (mean 109±180 ng/ml, median18) with controls (mean 9.4±11 ng/ml, median2.5) with statistically highly significant difference (z 5.2, P<0.001).Also there was a statistically significant difference as regards serum visfatin level between active SLE patients (mean 173±111 ng/ml, median 14) and inactive patients (mean 139±88 ng/ml, median 5) (z 2.1, p<0.05) as well as between patients with LN (mean 226±180 ng/ml, median18) and patients with no LN (mean 101±140 ng/ml, median 8 (2-229)) (z=2.1, p<0.05). Visfatin had a highly significant positive correlation with disease duration (r=0.48, p<0.001), SLEDAI (r=0.62, p<0.001) as well as ESR, CRP and, renal score (r=0.45, 0.35, and 0.65 respectively) while inverse correlation with estimated GFR (r=-0.614) and C3 & C4 titre (r=-0.26, r=-0.35 respectively) was recorded. Visfatin showed high sensitivity in detecting active SLE & LN 83% and 85% respectively. Conclusions Serum visfatin is strongly associated with LN in SLE patients and is a promising biomarker for prediction of renal involvement in these patients. It reflects SLE activity specially LN activity namely renal score & GFR decline. References Molino C, Fabbian F, Longhini C. Clinical approach to lupus nephritis: recent advances.European Journal of Internal Medicine2009; 20(5):447-53. Fukuhara A, Matsuda M, Nishizawa M, Segawa K, Tanaka M, Kishimoto K et al. Visfatin: a protein secreted by visceral fat that mimics the effects of insulin. Science 2005;307: 426–30. Yilmaz MI, Saglam M, Carrero JJ, Qureshi AR, Caglar K, Eyileten T et al. Serum visfatin concentration and endothelial dysfunction in chronic kidney disease. Nephrol Dial Transplant 2008;23(3): 959-65. Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH, Derivation of the SLEDAI. A disease activity index of lupus patients. Arthritis Rheum 1992; 35: 630-40. Weening JJ, D’Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB et al. The Classification of Glomerulonephritis in Systemic Lupus Erythematosus Revisited. J Am Soc Nephrol 2004; 15:.241-50. Disclosure of Interest None Declared