THU0529 CAPILLAROSCOPIC ALTERATIONS IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 504.1-504
Author(s):  
V. Kachkovska ◽  
A. Kovchun ◽  
E. Prystupa ◽  
L. Prystupa

Background:Nailfold capillaroscopy (NFC) is a useful, noninvasive, widely available diagnostic tool in rheumatology practice. We commonly use it to describe patterns of abnormalities in systemic sclerosis however we have enough data which proves NFC usefulness for monitoring idiopathic inflammatory myopathies (IIM) considering it as diagnostic tool, monitoring of disease activity and treatment efficiency. Despite evident clinical relevance of NFC in IIM patients we still do not have clear capillaroscopic images for IIM definition.Objectives:That’s why, the goal of our research was aimed to analyze capillaroscopic peculiarities among IIM patients and find possible associations with clinical and activity data.Methods:69 patients with IIM were examined and 47 IIM patients with capillaroscopic alterations were included in the study, 26 patients with dermatomyositis (DM) and 21 patients with polymyositis (PM) according to the Targoff Criteria (1997). NFC we performed usingDino-Lite CapillaryScope with 200 magnification. We assessed nailfold capillary density (NCD), presence of microhemorrhages, giant, dilated and ramified capillaries, scleroderma patterns (defined as an early, active or late pattern) and neovascular pattern (defined as an active and late scleroderma patterns). To asses disease activity we use Manual Muscle Testing 8 (MMT8), Health Assessment Questionnaire (HAQ), Myositis Disease Activity Assessment Tool (MDAAT), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), physician’s VAS, patient’s VAS, serum muscle enzymes levels. We divided patients into 4 groups: 1stgroup – 17 DM patients with active disease (8 of them with newly onset disease), 2ndgroup included 9 DM patients with inactive disease, 3rdgroup – 11 PM patients with active disease (5 of them with newly onset disease) and 4thgroup included 10 PM patients with inactive disease.Results:The most common finding was low NCD, 70% of all patients had NCD lower than 6 per 1 mm. NCD for the 1stgroup was 4,4±1,12, 2ndgroup – 6,0±1,0, 3rdgroup – 5,8±1,1, 4thgroup – 9,2±2,1 (F=26,27, p<0,001). Hemorrhages were significantly more common among DM patients and active disease and were observed among 47,1% (p=0,036,χ2=8,574) with no significant difference with regard to the disease onset. Analyzing scleroderma patterns, among 1stgroup of patients – 17,6% had early, 47,1% – active, 35,3% – late pattern, in the 2ndgroup – 66,7% had early pattern, in the 3rdgroup – 27,3% patients with early and 18,2% with active pattern and in the 4thgroup – 50% of patients presented with early pattern (p=0,001,χ2=31,87). Neovascular pattern was found significantly more often among patients with active DM (p=0,001) with no regard to the disease onset. No statistically significant difference in giant and ramified capillaries distribution was found.Conclusion:According to our results, we can admit that the most common capillaroscopic finding was decreased NCD, which were significantly lower among patients with active DM, the same as microhemorrhages and neovascular scleroderma pattern. This data suggests that NCD, microhemorrhages and neovascular scleroderma pattern could be consider as biomarkers of DM activity but not PM, therefore more detailed research with larger numbers of patients are required.Disclosure of Interests:None declared

Author(s):  
Lisa G. Rider ◽  
Frederick W. Miller

Due to their rarity, heterogeneity, and multispecialty nature, the myositis syndromes have limited data-driven consensus on appropriate outcome measures. Recently, two international, multispecialty consortia developed new tools and consensus on core set measures of myositis disease activity and damage, as well as response criteria that are now recommended for use as clinical trial endpoints but will also be useful in clinical practice. Magnetic resonance imaging, muscle ultrasound, selected laboratory tests, and immunological biomarkers—including cytokines, chemokines, lymphocyte flow cytometry, and endothelial activation markers—can all be helpful adjuncts to serum muscle enzyme levels in assessing disease activity and damage, but these have not yet been fully validated. Definitions of clinically inactive disease, complete clinical response, and remission have also been proposed but require further validation. These advances should enhance the development of therapies by standardizing our ability to demonstrate their efficacy in treating the idiopathic inflammatory myopathies.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 935.2-936
Author(s):  
S. Lanni ◽  
O. De Lucia ◽  
S. Orsi ◽  
S. Costi ◽  
G. Beretta ◽  
...  

Background:The ankle is one of the most commonly affected sites in juvenile idiopathic arthritis (JIA). This region has a complex anatomical structure owing to the presence of multiple joint recesses and surrounding tendons. While the prognostic value of ultrasound (US)-detected arthritis has been investigated in recent studies, the role of tenosynovitis in JIA remains still unexplored.Objectives:To investigate: 1) US features of ankle involvement in JIA at disease onset; 2) the predictive value of US-detected tenosynovitis in ankles with clinically active disease of children with new-onset JIA.Methods:The clinical charts of all consecutive patients with new-onset JIA between May 2018 and January 2020 at study centres (Policlinico and G.Pini Hospitals of Milan) and with clinically active ankle disease among the joints affected were reviewed retrospectively. Data on ankle US assessment were retrieved and patients were then stratified as follows: 1) patients with detection on US of isolated arthritis in at least one of the joint recesses of the ankle region; 2) patients with detection on US of tenosynovitis in at least one of the tendon compartments of the ankle irrespective of the presence of concomitant arthritis. For each of these two categories, estimation of patients who were able to achieve clinical disease remission at 12 months since disease onset was evaluated.Results:Twenty-seven new-onset JIA patients were found to have clinical involvement of the ankle among the joints affected. Nine of them (33.3%) showed on US isolated arthritis of the ankle, whereas US-detected tenosynovitis was found in 18 (66.7%) patients. The amount of patients who were able to achieve disease remission at 12-months was the same (66.7%) for both patients with and without US-detected tenosynovitis in the ankle (12/18 and 6/9 patients, respectively). In patients with US-detected tenosynovitis and clinical disease remission at 12 months, the lateral tendon compartment (LTC) was the tendon site more frequently affected by pathology (75.0%). Patients with US-detected tenosynovitis that did not achieve clinical disease remission at follow-up had the highest frequency of tendon pathology on US in the medial tendon compartment (MTC) (83.3%). The anterior tendon compartment was the less frequently affected tendon compartment of the ankle in all patients (33.3% in both patients with and without clinical remission of disease at the 12-months follow-up visit).Conclusion:US-detected tenosynovitis of the ankle is a common finding in patients with new-onset JIA with clinically ankle disease activity and is more frequent than the detection on US of isolated arthritis. The MTC and LTC are the tendon compartments more commonly affected on US. The detection on US of tenosynovitis at disease onset in ankles with clinical disease activity did not seem to affect the change to achieve the overall clinical disease remission compared to patients without tendon pathology but with joint disease in the ankle region.Disclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1582.1-1582
Author(s):  
B. H. Egeli ◽  
S. Ergun ◽  
Y. K. Gursoy ◽  
A. Cetin ◽  
S. Ugurlu

Background:Idiopathic inflammatory myopathies (IIM) are essentially treated aiming improvement of muscle function and extra muscular disease manifestations. The backbone of the treatment is corticosteroids enhancing the survival and patient quality of life. The lack of consensus on target-specific immunosuppressive treatment highlights the need for further studies evaluating alternative treatment methods. Rituximab is potentially a glucocorticoid-sparing agent which was reviewed in multiple studies with small sample sizes due to the rarity of the disease.Objectives:Higher statistical power can enhance the trustworthiness of alternative treatment methods yielding the main objective of this study.Methods:This retrospective study was conducted at a tertiary rheumatology center. Patients were diagnosed with an idiopathic inflammatory myopathy (dermatomyositis [DM], polymyositis [PM]) and were treated with rituximab in order to be included in this study. Clinical signs and symptoms of the presentation were noted during the first patient encounter as well as the follow-up. Parameters of disease activity including acute phase reactants, muscle enzyme levels, and disease-specific autoantibodies were analyzed.Results:The study includes 28 patients (20 DM, 8 PM). The age of diagnosis was 43.44 ± 15.77 years, follow-up duration was 60.7 ± 70.7 months. The presenting signs and symptoms of the patients are shown in Figure 1. The parameters of disease activity before and after treatment are summarized in Table 1. The mean corticosteroid dose decreased from 31.429 ±23.934 mg to 10.278 ±12.001 (p=0.001). Other treatment methods were methotrexate (n=18), Intravenous Immunoglobulin (IVIG) (n=7), and cyclophosphamide (n=2). There were not any deaths during the follow-up. Two patients were lost to follow-up.Table 1.The Parameters of Disease Activity Before and After TreatmentBefore TreatmentAfter TreatmentP ValueCPK, mean ± std (U/L)1426 ± 2049.92263.44 ± 265.630.004LDH, mean ± std (U/L)557.5 ± 365379.78 ± 192.10.03AST, mean ± std (U/L)62.52 ± 5930.16 ± 27.590.01ALT, mean ± std (U/L)56.48 ± 49.2127.64 ± 24.520.008ESR, mean ± std (mm/hour)26.38 ± 28.9820.39 ± 18.760.36CRP, mean ± std (mg/L)19.23 ± 46.1512.53 ± 26.670.5RF, mean ± std (U/mL)0 (0)N/AN/AANA, n (%)3 (10.71)N/AN/AFigure 1.The Presenting Signs and Symptoms of the PatientsConclusion:Rituximab is shown to be effective in treating myositis along with corticosteroids as well as a corticosteroid-sparing agent in retrospective studies and open-label clinical trials; however, lack of statistical power should be underlined. Long term decrease in steroid use and decrease in disease activity markers hints the effective use of rituximab as a glucocorticoid sparing agent as well as its safety with minimal side effects.Disclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 553.1-553
Author(s):  
K. Ulu ◽  
F. Demir ◽  
T. Coşkuner ◽  
Ş. Çağlayan ◽  
B. Sözeri

Background:The TNF-α inhibitor adalimumab is a biological disease modifying anti-rheumatic drug (bDMARD) that has been used in different rheumatic diseases with a resistant course. ABP-501 is a biosimilar product (BP) of adalimumab, recently approved by the FDA and EMA. To our knowledge, there is no study assess the efficacy and safety of these two molecules on pediatric patients.Objectives:We aimed to compare the efficacy and safety of the original and biosimilar adalimumab (ABP-501) molecules in childhood rheumatic diseases.Methods:This non-interventional, retrospective, single-centre analysis carried out in Umraniye Training and Resrach Hospital, Pediatric Rheumatology Clinic, Istanbul, Turkey. The study group consisted of patients who were followed due to chronic rheumatic disease between January 1, 2016 and June 1, 2020, and received reference or biosimilar adalimumab therapy for at least three months. Demographic and clinical data of patients were collected at baseline, 3rd, 6th, and 12th months of treatment. Disease activity assessment was made with JADAS-27 in JIA patients, with SUN criteria in uveitis patients, and with Behçet’s Disease Activity Index in BD patients. Efficacy and safety of treatments were compared between reference and biosimilar adalimumab groups.Results:A total of 89 patients (65 with original and 24 with biosimilar molecule) treated with adalimumab, were included in the study. There were 45 female and 44 male in the study, and the median age at the initiation of the adalimumab was 166 months (min-max: 36-231). Of the 89 patients evaluated, the primary diagnoses of 62 were juvenile idiopathic arthritis, 13 were idiopathic uveitis, eight were Behçet’s disease, three were Blau syndrome, two were chronic recurrent multifocal osteomyelitis and one was Vogt-Koyanagi-Harada syndrome. 63 of the patients were biologic-naïve, and 13 were switched from etanercept, 11 from infliximab, and two from other bDMARDs. The median exposure time of adalimumab was 16 months (min-max:3-70) in RP and 14.5 months (min-max: 3-23) in BP. All patients had active disease before treatment. In the group treated with RP, inactive disease was achieved in 60%, 76.6% and 87.2% of the patients at the 3rd, 6th and 12th months, respectively. Also, inactive disease was achieved in 62.5%, 78.2% and 78.2% of the patients at the 3rd, 6th and 12th months in the group treated with BP, respectively. There was no statistically significant difference in efficacy between the groups at the 3rd, 6th and 12th months (p=0.83, 0.07 and 0.32). Serious adverse events were seen in one patient in each groups (lymphoma in RP group, tuberculous meningitis in BP group). Non-serious adverse events were observed in eight patients (12.3%) in the RP group and in two patients (8.3%) in the BP group, without statistically significant difference between groups (p=0.86).Conclusion:No significant difference was observed between the biosimilar adalimumab ABP-501 and RP adalimumab in terms of efficacy and safety.References:[1]Renton, William D et al. Pediatr Rheumatol Online J. 2019;17(1):67.[2]Lovell DJ, Ruperto N, Goodman S, et al. N Engl J Med. 2008;359(8):810-820.[3]Kingsbury, Daniel J et al. Clin Rheumatol 2014;33(10):1433-41.Disclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1629.2-1629
Author(s):  
K. Ben Abdelghani ◽  
Y. Gzam ◽  
A. Fazaa ◽  
S. Miladi ◽  
K. Ouenniche ◽  
...  

Background:Axial spondyloarthritis (ax-SpA) is a chronic rheumatic disease that mainly affects men. However, the female form of ax-SpA remains insufficiently studied.Objectives:The aim of this study was to determine the clinical characteristics, the disease activity and the functional impact of female ax-SpA in comparison with male ax-SpA.Methods:This is a retrospective study including patients diagnosed with ax-SpA fulfilling the criteria of the Assessment of SpondyloArthritis international Society (ASAS) 2009.Clinical parameters, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), Bath ankylosing spondylitis disease activity index (BASDAI) and Bath ankylosing spondylitis functional index (BASFI) were compared between groups of female and male ax-SpA.Results:Two hundred ax-SpA patients were included with 31% of female (n=62) and a mean age of 43,3 ± 11,2 years.The mean age at onset of symptoms was 31,8 ± 8,9 years for women and 25,3 ± 9,1 years for men (p <0,0001). The mean age at diagnosis was 36,4 ± 9,6 years for women and 31,7 ± 10,4 years for men (p = 0,003). Ax-SpA with juvenile onset was noted in 1,7% of women and 12,1% of men (p = 0,02). Male ax-SpA were significantly more smokers (46.8% vs 5.4%; p <0.001). The mean duration of morning stiffness was 11,3 ± 9,2 minutes for women versus 21,6 ± 19,3 minutes for men (p = 0,005).The mean ESR was 42,4 ± 29,8 mm for women and 28,3 ± 23,4 mm for men (p = 0,001). Radiographic sacroiliitis was present in 69,3% of women versus 84,7% of men (p = 0,01). The use of anti-TNF alpha was less frequent in women (29% vs 48,5%; p = 0,01).Our study didn’t found a statistically significant difference in peripheral manifestations, extraarticular manifestations, CRP, BASDAI and BASFI between the two groups.Conclusion:Female ax-SpA seems to have a better prognosis than male with older age in disease onset, less inflammation, less radiographic sacroiliitis and less use of biological treatments.References:[1]Rusman T, et al. Curr Rheumatol Rep. 2018; 20(6).[2]Siar N, et al. Curr Rheumatol Rev. 2019;Disclosure of Interests:None declared


2010 ◽  
Vol 37 (9) ◽  
pp. 1932-1934 ◽  
Author(s):  
KETNA PAREKH ◽  
WILLIAM J. TAYLOR

Objective.To determine whether the Patient Activity Scale-II (PAS-II) is a generic measure of disease activity by assessing whether the relationship of PAS-II with treatment decision (indicating disease activity) is invariant across disease.Methods.The Health Assessment Questionnaire-II (HAQ-II), a 10 cm visual analog scale for “pain,” and another for “patient global assessment” were recorded from 1000 consecutive patients attending rheumatology outpatient clinics. Active disease was defined as treatment intensity increased and inactive disease was defined as treatment intensity unchanged or decreased. A logistic regression analysis was conducted with active disease as the dependent variable and the predictor variables were PAS-II, diagnostic category, and the interaction between diagnostic category and PAS-II.Results.PAS-II had a weak but statistically significant association with active disease that was independent of diagnosis. An increase of 1 point in PAS-II increased the odds of being in the active disease state by 1.19 (95% CI 1.10 to 1.37). The relationship between active disease state and PAS was not affected by diagnostic category.Conclusion.PAS-II can be used as a generic self-report indicator of active disease across different rheumatic disorders, and not just in rheumatoid arthritis. The strength of the relationship with disease activity is weak and physician-derived indicators remain very important.


Rheumatology ◽  
2020 ◽  
Vol 60 (1) ◽  
pp. 322-332
Author(s):  
Eri Watanabe ◽  
Kazunori Kato ◽  
Takahisa Gono ◽  
Emiko Chiba ◽  
Chihiro Terai ◽  
...  

Abstract Objectives Galectin-3 is involved in various biological activities, including immune activations and fibrosis. Idiopathic inflammatory myopathies (IIMs) are autoimmune diseases of unknown aetiology, often complicated by interstitial lung disease (ILD). The aim of this study was to evaluate the expression of galectin-3 in sera and tissues of patients with IIM and assess the associations of galectin-3 with patient characteristics and disease activity. Results Serum galectin-3 levels were significantly higher in IIM patients than in healthy controls. The serum galectin-3 levels positively correlated with serum levels of inflammatory markers and proinflammatory cytokines/chemokines and the Myositis Intention-to-Treat Activity Index. Stratification analysis revealed that patients with IIM-associated ILD (IIM-ILD) had significantly higher levels of serum galectin-3 than those without IIM-ILD. In addition, patients with acute/subacute interstitial pneumonia had significantly higher levels of serum galectin-3 than those with chronic interstitial pneumonia. Furthermore, serum galectin-3 levels in IIM-ILD patients correlated with the radiological assessments of parenchymal lung involvement and treatment response. Immunohistochemical analysis revealed that galectin-3 was expressed in inflammatory cells of myositis and dermatitis sections, whereas in ILD sections, galectin-3 was expressed in interstitial fibrosis and inflammatory cells. Conclusion Galectin-3 may be involved in the pathogenesis of inflammatory and fibrotic conditions in IIM and can serve as a potential biomarker of disease activity, especially in patients with IIM-ILD.


1991 ◽  
Vol 37 (1) ◽  
pp. 47-50 ◽  
Author(s):  
Lakana Leohirun ◽  
Phlchal Thuvasethakul ◽  
Vasant Sumethkul ◽  
Trithar Pholcharoen ◽  
VlJitr Boonpucknavig

Abstract Concentrations of neopterin were measured in urine specimens from 35 patients with active and eight with inactive systemic lupus erythematosus (SLE). Compared with those of apparently healthy controls, neopterin concentrations were higher in patients with active disease (P less than 0.001) and with inactive disease (P less than 0.01), those in patients with active disease being significantly higher than those in patients with inactive disease (P less than 0.001). The correlation between the neopterin concentration and evidence of disease activity was good. All of the patients with clinically active SLE had increased neopterin, but for only 37.5% (three of eight) did the neopterin concentration exceed the upper normal limit during clinical remission. The increase in neopterin concentration did not correlate with clinical courses or severity of renal function. Moreover, serial determinations of neopterin in active SLE patients showed a rapid decrease of initially high concentration, paralleling a decline of clinical activity after initiation of medical therapy. Thus, urinary neopterin may be a useful marker for monitoring disease activity in SLE patients.


2017 ◽  
Vol 21 (1) ◽  
pp. 233-239 ◽  
Author(s):  
Joana Sousa Neves ◽  
Daniela Santos Faria ◽  
Marcos Cerqueira ◽  
Maria Carmo Afonso ◽  
Filipa Teixeira

2019 ◽  
Vol 40 (05) ◽  
pp. 638-645 ◽  
Author(s):  
Christian Lottspeich ◽  
Claudia Dechant ◽  
Anton Köhler ◽  
Maximilian Tischler ◽  
Karla Maria Treitl ◽  
...  

Abstract Purpose To assess the diagnostic value of intima media thickness measurements and contrast-enhanced ultrasound (CEUS) of the supraaortic arteries in the assessment of disease activity in Takayasu arteritis (TA). Materials and Methods Patients with TA and involvement of the carotid and/or subclavian/axillary arteries underwent CEUS imaging in addition to B-mode and color duplex ultrasound. The maximum IMT (mIMT) was measured and high-resolution CEUS of the most prominently thickened vessel segment was performed. Two blinded readers reviewed the CEUS movies, with semiquantitative assessment of microbubble enhancement of the arterial wall (grade 0: no or minimal; grade 1: moderate; grade 2: pronounced). Clinical symptoms, acute phase reactants, and established indices of clinical disease activity (NIH criteria, ITAS score) were recorded. Results 40 examinations in 17 patients were analyzed. According to clinical judgement, 27 and 13 cases were classified as clinically inactive and active, respectively. An mIMT-cutoff of > 2.7 mm identified active disease with a sensitivity and specificity of 69.2 % and 88.9 %, respectively (area under the curve 0.83). The interobserver agreement of CEUS analysis was substantial (Cohen’s kappa 0.76). By consensus reading, 17, 15, and 8 cases were classified as uptake grade 0, grade 1 and grade 2, respectively. Grade 0 uptake was exclusively present in 17 clinically inactive patients. Grade 1 uptake was seen in 10 patients with clinically inactive disease and 5 patients with clinically active disease. Grade 2 uptake was exclusively present in 8 patients with active disease. Conclusion Both sonographic IMT measurements and high-resolution CEUS are promising in the ad-hoc assessment of disease activity in patients with TA.


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