Thymic Stromal Lymphopoietin Is Associated With Disease Activity in Systemic Lupus Erythematosus

Objective To investigate the association between Thymic stromal lymphopoietin (TSLP) and disease activity in patients with Systemic Lupus Erythematosus (SLE).Methods In this study, concentrations of serum TSLP in 65 SLE patients, 50 sex and age-matched control subjects were determined by enzyme-linked immunosorbent assay (ELISA).Results Serum TSLP concentrations in SLE patients were dramatically higher than healthy controls. The levels of serum TSLP displayed a significant increase as compared with healthy controls. More importantly, TSLP levels were significantly correlated with SLE disease activity features such as ESR, CRP, Anti-dsDNA Ab, and SLEDAI-2K,. The predictive value of TSLP on high disease activity was superior to those of CRP, ESR, and Anti-dsDNA Ab. A note worthy correlation in our study was observed between the serum TSLP levels and laboratory parameters, particularly serum lipids. Furthermore, serum TSLP levels could be significantly down-regulated after effective integrative treatment.Conclusion TSLP may serve as a novel sensitive biomarker to assist disease activity assessment and monitor therapeutic effects in active SLE patients.

Nail Fold Capillaroscopy in a Cohort of Egyptian Dermatomyositis/Polymyositis Patients

ABASTRACT Background Nailfold capillaroscopy (NC) has been demonstrated to be an important tool in the diagnosis and treatment of patients with juvenile dermatomyositis (JDM) and systemic sclerosis. However, NC has been scarcely studied in adult patients with DM. Objectives Is to visualize capillary changes in DM/PM patients, trying to find a specific capillary pattern and its correlation if any- with disease activity in those patients. Methodology This is a cross sectional observational study included 20 patients (DM.Diagnosed on base of clinical, laboratory and histopathological findings. We excluded patients with hypertension or diabetes mellitus. The NFC performed using videocapillaroscope. A semiquantitative rating scale was used to score capillaroscopy changes. Myositis disease activity assessment tools were used to assess disease activity [Manual Muscle Testing (MMT), Myositis Disease Activity Assessment Visual Analogue Scales (MYOACT), Health Assessment Questionnaire (HAQ), physician’s VAS, patient’s VAS, skin scoring, serum muscle enzymes levels] Result The median age of our patients was 36 (S.D.12) Mean disease duration was 24months (S.D.19).The results presented mean value 3,8 (S.D.3,3) for muscle disease activity score,2(S.D.3) for skin score and 3,92 (S.D.3,09) for global disease activity .Enlarged capillaries was found in12 patients (100%), avascular areas in 10 patients (83.3%), capillaries hemorrhage in 8 patients (66,7%), and branched capillaries in 6 patients (50%).Sixteen patient (80%) had scleroderma like pattern. We found that disease activity positively correlate with branched capillaries(p = 0,04), muscle disease activity correlate positively with capillaries density score(p = 0,002) while hemorrhage correlate positively with skin score(p = 0,024). In our study major capillaroscopic abnormalities appeared to be virtually confined to patients with DM while patients with PM had only minor changes. No signifcant correlations between NFC and other demographic, clinical and laboratory parameters were observed. Conclusion NFC may provide helpful information about patients with PM/DM by: (a) contributing to the diagnosis and (b) correlating with disease activity parameters. Additional investigations with larger series of patients and prospective studies may be useful to reinforce our data.

POS0769 VALIDATION OF THE SIMPLE INDEX FOR DISEASE ACTIVITY OF SYSTEMIC LUPUS ERYTHEMATOSUS IN CHINESE PATIENTS

Objectives:To validate the SIMPLE index for systemic lupus erythematosus (SLE) disease activity assessment in Chinese patients.Methods:Adult patients (age ≥18 years) who fulfilled the 2013 SLICC criteria for SLE and were followed in the Rheumatology clinics of Tuen Mun Hospital, Hong Kong were recruited in a cross-sectional study. Participants were invited to complete the SIMPLE questionnaire before seeing doctors on the same day. The two laboratory results were supplemented by research nurses. Physicians, who were blinded to the SIMPLE results, were asked to complete disease activity assessment by the SELENA-SLEDAI and physicians’ global assessment (PGA) after consultation. Correlation was made between the SIMPLE score and the SLEDAI/PGA scores by Spearman’s rank correlation test. Receiver operating characteristic (ROC) analysis was performed to find the best cut-off SIMPLE score that predicted a clinical SLEDAI score of 1-6 (mild SLE activity) and ≥7 (moderate/severe activity).Results:364 SLE patients were studied (94% women; age 45.4±13.4 years; disease duration 13.2±8.0 years). The proportion of patients having a history of neuropsychiatric and renal disease that required immunosuppressive therapies was 9.3% and 56%, respectively. At the time of questionnaire completion, 69 (19%) patients had SLEDAI ≥6 and 192 (53%) had SLEDAI 1-5. The mean SLEDAI was 3.04±2.85 and PGA score was 0.62±0.55. A total of 161 (44%) had SDI score ≥1. The mean SIMPLE index was 26.0±12.9. SIMPLE index correlated significantly with SLEDAI (ρ=0.76; p<0.001) and PGA score (ρ=0.48; p<0.001). ROC analysis showed that a SIMPLE index of >27 points best predicted a clinical SLEDAI score of 1-6 (area under the curve [AUC] 0.78[0.73-0.84]; sensitivity 0.75; specificity 0.71), and >36.8 points best predicted a clinical SLEDAI score of ≥7 (AUC 0.87[0.69-1.00]; sensitivity 0.88, specificity 0.85).Conclusion:SIMPLE shows a good correlation with SELENA-SLEDAI and PGA. It is a simple tool that enables patients to self-report disease activity and communicate with the health care team more efficiently.Disclosure of Interests:None declared

NP-59 Adrenal Scintigraphy as an Imaging Biomarker to Predict KCNJ5 Mutation in Primary Aldosteronism Patients

PurposeSomatic KCNJ5 mutation occurs in half of unilateral primary aldosteronism (PA) and is associated with more severe phenotype. Mutation status can only be identified by tissue sample from adrenalectomy. NP-59 adrenal scintigraphy is a noninvasive functional study for disease activity assessment. This study aimed to evaluate the predictive value of NP-59 adrenal scintigraphy in somatic KCNJ5 mutation among PA patients who received adrenalectomy.MethodsSixty-two PA patients who had NP-59 adrenal scintigraphy before adrenalectomy with available KCNJ5 mutation status were included. Two semiquantitative parameters, adrenal to liver ratio (ALR) and lesion to contralateral ratio of bilateral adrenal glands (CON) derived from NP-59 adrenal scintigraphy, of mutated and wild-type patients were compared. Cutoff values calculated by receiver-operating characteristic (ROC) analysis were used as a predictor of KCNJ5 mutation.ResultsTwenty patients had KCNJ5 mutation and 42 patients were wild type. Patients harboring KCNJ5 mutation had both higher ALR and CON (p = 0.0031 and 0.0833, respectively) than wild-type patients. With ALR and CON cutoff of 2.10 and 1.95, the sensitivity and specificity to predict KCNJ5 mutation were 85%, 57% and 45%, 93%, respectively. Among 20 patients with KCNJ5 mutation, 16 showed G151R point mutation (KCNJ5- G151R) and 4 showed L168R point mutation (KCNJ5-L168R), which former one had significantly lower ALR (p=0.0471).ConclusionPA patients harboring somatic KCNJ5 mutation had significantly higher NP-59 uptake regarding to ALR and CON than those without mutation. APAs with KCNJ5-L168R point mutation showed significantly higher ALR than those with KCNJ5-G151R point mutation.