scholarly journals Characteristics of Joint Involvement and Relationships with Systemic Inflammation in Systemic Sclerosis: Results from the EULAR Scleroderma Trial and Research Group (EUSTAR) Database

2010 ◽  
Vol 37 (7) ◽  
pp. 1488-1501 ◽  
Author(s):  
JEROME AVOUAC ◽  
ULRICH WALKER ◽  
ALAN TYNDALL ◽  
ANDRÉ KAHAN ◽  
MARCO MATUCCI-CERINIC ◽  
...  

Objective.To determine the prevalence of and independent factors associated with joint involvement in a large population of patients with systemic sclerosis (SSc).Methods.This study was cross-sectional, based on data collected on patients included in the European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) registry. We queried this database to extract data regarding global evaluation of patients with SSc and the presence of any clinical articular involvement: synovitis (tender and swollen joints), tendon friction rubs (rubbing sensation detected as the tendon was moved), and joint contracture (stiffness of the joints that decreased their range of motion). Overall joint involvement was defined by the occurrence of synovitis and/or joint contracture and/or tendon friction rubs.Results.We recruited 7286 patients with SSc; their mean age was 56 ± 14 years, disease duration 10 ± 9 years, and 4210 (58%) had a limited cutaneous disease subset. Frequencies of synovitis, tendon friction rubs, and joint contractures were 16%, 11%, and 31%, respectively. Synovitis, tendon friction rubs, and joint contracture were more prevalent in patients with the diffuse cutaneous subset and were associated together and with severe vascular, muscular, renal, and interstitial lung involvement. Moreover, synovitis had the highest strength of association with elevated acute-phase reactants taken as the dependent variable.Conclusion.Our results highlight the striking level of articular involvement in SSc, as evaluated by systematic examination in a large cohort of patients with SSc. Our data also show that synovitis, joint contracture, and tendon friction rubs are associated with a more severe disease and with systemic inflammation.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 407.1-407
Author(s):  
M. G. Lazzaroni ◽  
S. Zingarelli ◽  
P. Airò ◽  
Y. Allanore ◽  
O. Distler

Background:Anti-PM/Scl antibodies positivity has been associated with frequent skeletal muscle involvement in patients with Systemic Sclerosis (SSc) in different studies, including the EUSTAR cohort (1). Moreover, although myositis has been previously associated with heart involvement in SSc patients (2), this issue has never been explored among anti-PM/Scl+ patients.Objectives:To evaluate the cardiac involvement in anti-PM/Scl patients with SSc in the large multicentre EUSTAR database, with focus on the subgroup of patients with muscle involvement.Methods:Patients from the EUSTAR database were included when the item anti-PM/Scl was fulfilled in at least one visit.Results:Anti-PM/Scl status was available in 7,353 SSc patients from EUSTAR database: 295 were anti-PM/Scl+. After exclusion of 151 patients with multiple autoantibody positivity, 144 anti-PM/Scl + patients were compared with 7,058 anti-PM/Scl- patients. Among them, 3,120 (44.2%) were positive for ACA, 2,361 (33.5%) for anti-Topo I and 274 (3.88%) for anti-RNAP3.Regarding the specific cardiac outcomes, in the anti-PM/Scl+ as compared to the anti-PM/Scl- group, a decreased rate of elevated sPAP at ECHO was recorded (12.8% vs 25.0%, p:0.001), while no differences were observed in the frequency of conduction blocks (26.2% vs 23.7%, p:0.526), abnormal diastolic function (33.9% vs 36.4%, p:0.582), pericardial effusion (10.2% vs 10.9%, p:1.000) and LVEF ≤50% (4.76% vs 6.11%, p:0.818). In multivariate analysis, adjusted for age at disease onset, sex, and disease duration, the negative association of anti-PM/Scl with elevated sPAP was not confirmed (p:0.061).When comparing anti-PM/Scl+ patients with (n=47) and without (n=87) CK elevation, the former group had a higher frequency of conduction blocks (43.2% vs 17.5%, p:0.005; OR 95% CI 3.47, 1.51-7.97) and left ventricular dysfunction, both diastolic (45.6% vs 27.2%, p:0.050; OR 95% CI 2.25, 1.05-4.81) and systolic (LVEF ≤50% 13.3% vs 0%, p:0.018; OR 95% CI 16.8, 0.87-324). Moreover, anti-PM/Scl+ patients with CK elevation had significantly increased rate of lung fibrosis on HRCT (p:0.045), intestinal symptoms (p:0.017), joint contractures (p:0.045) and tendon friction rubs (p:0.034).Conclusion:In the largest series of anti-PM/Scl positive SSc patients so far reported, muscle involvement in anti-PM/Scl+ patients (defined as increased serum CK) seems to represent a marker of a more severe disease phenotype, including a higher frequency of cardio-pulmonary involvement.References:[1]Lazzaroni MG, et al. Ann Rheum Dis 2018. 77 (2), 421-2.[2]Follansbee WP, et al. Am Heart J 1993. 125: 194-203.Acknowledgments:Authors would like to thank the patients’ association GILS (Gruppo Italiano Lotta Sclerodermia) for the grant that supported the project.Disclosure of Interests:Maria Grazia Lazzaroni: None declared, Stefania Zingarelli: None declared, Paolo Airò: None declared, Yannick Allanore Grant/research support from: BMS, Inventiva, Roche, Sanofi, Consultant of: Actelion, Bayer AG, BMS, BI, Oliver Distler Grant/research support from: Grants/Research support from Actelion, Bayer, Boehringer Ingelheim, Competitive Drug Development International Ltd. and Mitsubishi Tanabe; he also holds the issued Patent on mir-29 for the treatment of systemic sclerosis (US8247389, EP2331143)., Consultant of: Consultancy fees from Actelion, Acceleron Pharma, AnaMar, Bayer, Baecon Discovery, Blade Therapeutics, Boehringer, CSL Behring, Catenion, ChemomAb, Curzion Pharmaceuticals, Ergonex, Galapagos NV, GSK, Glenmark Pharmaceuticals, Inventiva, Italfarmaco, iQvia, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Roche, Sanofi and UCB, Speakers bureau: Speaker fees from Actelion, Bayer, Boehringer Ingelheim, Medscape, Pfizer and Roche


2018 ◽  
Vol 2018 ◽  
pp. 1-6 ◽  
Author(s):  
O. Zargari ◽  
E. Kazemnezhad Leyli ◽  
S. Z. Azimi

Background. Psoriatic arthritis (PsA) results in an increased burden of psoriasis and impairs both quality of life and an individual’s functional capacity. The relationship between nail involvement and PsA in psoriasis is not fully characterized. Aim. To evaluate the frequency and characteristics of nail involvement in psoriatic patients and to assess the relationship with joint involvement. Methods. A total of 197 patients with moderate-to-severe psoriasis were consecutively invited to participate in this cross-sectional study. The patients are divided into two groups: those with and those without psoriatic arthritis. Results. 69.5% of psoriatic (137 out of 197) patients had nail involvement. The most common nail abnormality was onycholysis, followed by pitting and oil droplet changes. Nail involvement was more common in patients with psoriatic arthritis (82.1% versus 57.8%, p=0.001). Conclusion. Nail involvement is commonly associated with PsA. Onycholysis, splinter hemorrhage, and oil drop were significantly more common in the PsA group as opposed to patients with just skin findings. In general, psoriatic patients with arthritis had more severe disease.


2019 ◽  
Vol 47 (8) ◽  
pp. 1198-1203 ◽  
Author(s):  
Marco Di Carlo ◽  
Pietro Muto ◽  
Devis Benfaremo ◽  
Michele Maria Luchetti ◽  
Fabiola Atzeni ◽  
...  

Objective.To evaluate the prevalence and factors associated with the neuropathic pain features in a cohort of patients with psoriatic arthritis (PsA).Methods.A cross-sectional evaluation was conducted in consecutive patients having PsA with prevalent peripheral joint involvement, referred to 3 rheumatological centers. For each patient, a comprehensive assessment of disease activity, physical function, and disease effect was carried out. The presence of comorbid fibromyalgia syndrome (FMS) was evaluated. Acute-phase reactants were also recorded. The neuropathic pain features were investigated through the PainDETECT Questionnaire (PDQ). A logistic regression analysis was therefore conducted using the PDQ as the dependent variable.Results.The final evaluation included 118 patients. A comorbid FMS was detectable in 30 of the 118 patients with PsA (25.4%). Probable characteristics of neuropathic pain (PDQ ≥ 19) were found in 30 (25.4%) patients overall, ambiguous (PDQ > 12 and < 19) in 21 (17.8%) patients, and unlikely (PDQ ≤ 12) in 67 (56.8%) patients. Using logistic regression analysis, the only independent variable among those investigated that could explain the neuropathic pain features was the presence of a comorbid FMS (p = 0.0127). Excluding patients with comorbid FMS, an association with disability (measured by Health Assessment Questionnaire–Disability Index) emerges (p = 0.0489). In patients with PsA and comorbid FMS, PDQ scores were significantly higher than in patients without comorbid FMS.Conclusion.Neuropathic pain features are common in patients with PsA, and the presence of pain sensitization (comorbid FMS) seems to be its main predictor.


2019 ◽  
Vol 1 ◽  
pp. 77-83
Author(s):  
Deepthi Sureshan ◽  
Najeeba Riyaz ◽  
Lekha Thumbayil

Objectives: To study the cutaneous and systemic manifestations of systemic sclerosis (SSc) and correlate the severity of cutaneous disease with extent of fibrosis on histology. Methods and Materials: Patients were evaluated for cutaneous and systemic manifestations of SSc. Cutaneous disease was assessed using modified Rodnan Skin Score (mRSS) with mRSS ≥14 indicating severe disease. Masson’s trichrome stained skin biopsy specimens were graded semi-quantitatively according to severity and extent of dermal fibrosis. Subsequently, the clinicohistological correlation was assessed. Results: Thirty-two patients were studied. Eighteen patients had diffuse cutaneous SSc while 14 had limited cutaneous SSc. Ten patients had mRSS <14 while 22 had mRSS ≥14 with a mean mRSS of 15.8. Gastrointestinal system was involved in 56.3%, respiratory system in 53.1%, musculoskeletal system in 31.3%, renal in 6.3%, and cardiovascular in 3.1%. Anti-centromere antibodies were positive in six patients and Anti-Scl-70 in 12. In the histopathological analysis of fibrosis, 40.6% of patients had moderate fibrosis while 59.4% had severe fibrosis. While patients with higher mRSS also had a higher grade of fibrosis histologically, the clinicohistological correlation was not found to be statistically significant. Limitations: The prognostic significance of mRSS could not be assessed as this was a cross-sectional study. Conclusion: The cutaneous and systemic involvement observed in this study was comparable to the findings in other studies. The changes observed in frequency of specific manifestations in different population groups point to the role of genetic and environmental factors in the disease process.


2010 ◽  
Vol 69 (11) ◽  
pp. 1951-1957 ◽  
Author(s):  
Lijana Krause ◽  
Mike O Becker ◽  
Claudia S Brueckner ◽  
Christina-Julia Bellinghausen ◽  
Corinna Becker ◽  
...  

ObjectiveTo assess and analyse nutritional status in patients with systemic sclerosis (SSc) and identify possible associations with clinical symptoms and its prognostic value.MethodsBody mass index (BMI) and parameters of bioelectrical impedance analysis (BIA) were assessed in 124 patients with SSc and 295 healthy donors and matched for sex, age and BMI for comparisons. In patients with SSc, BMI and BIA values were compared with clinical symptoms in a cross-sectional study. In a prospective open analysis, survival and changes in the nutritional status and energy uptake induced by nutritional treatment were evaluated.ResultsPatients with SSc had reduced phase angle (PhA) values, body cell mass (BCM), percentages of cells, increased extracellular mass (ECM) and ECM/BCM values compared with healthy donors. Malnutrition was best reflected by the PhA values. Of the patients with SSc, 69 (55.7%) had malnutrition that was associated with severe disease and activity. As assessed by multivariate analysis, low predicted forced vital capacity and high N-terminal(NT)-proBNP values discriminated best between good and bad nutritional status. Among different clinical parameters, low PhA values were the best predictors for SSc-related mortality. BMI values were not related to disease symptoms or mortality. Fifty per cent of patients with SSc had a lower energy uptake related to their energy requirement, 19.8% related to their basal metabolism. Nutritional treatment improved the patients' nutritional status.ConclusionsIn patients with SSc, malnutrition is common and not identified by BMI. BIA parameters reflect disease severity and provide best predictors for patient survival. Therefore, an assessment of nutritional status should be performed in patients with SSc.


2009 ◽  
Vol 36 (9) ◽  
pp. 1924-1929 ◽  
Author(s):  
ALESSANDRA VACCA ◽  
CATHERINE CORMIER ◽  
MARTINA PIRAS ◽  
ALESSANDRO MATHIEU ◽  
ANDRE KAHAN ◽  
...  

Objective.To investigate 25-OH vitamin D concentrations in 2 independent systemic sclerosis (SSc) populations from France and Italy.Methods.We studied 156 consecutive SSc patients comparable for demographic characteristics: 90 from Northern France and 66 from Southern Italy. 25-OH vitamin D, intact parathyroid hormone, and serum total calcium and phosphorus were measured in all patients. Vitamin D concentrations < 30 ng/ml were considered insufficiency, while values < 10 ng/ml were classified as deficiency.Results.Vitamin D insufficiency and deficiency rates were very high and comparable between the 2 populations: 74/90 (82%) versus 57/66 (86%) for insufficiency and 29/90 (32%) versus 15/66 (23%) for deficiency, respectively, in the French and Italian patients. They were not influenced by vitamin D supplementation, which was not statistically different in the 2 groups. In the combined populations, a significant negative correlation was found between low vitamin D levels and European Disease Activity Score (p = 0.04, r = −0.17) and an even more significant correlation was found with acute-phase reactants (p = 0.004, r = −0.23 for erythrocyte sedimentation rate), and low levels of vitamin D were associated with the systolic pulmonary artery pressure (sPAP) estimated by echocardiography (p = 0.004). In multivariate analysis, vitamin D deficiency was associated with sPAP (p = 0.02).Conclusion.Vitamin D deficiency was very common in the 2 SSc populations, independent of geographic origin and vitamin D supplementation. This suggests that common vitamin D supplementation does not correct the deficiency in SSc patients, and that a higher dose is probably needed, especially in those with high inflammatory activity or severe disease.


2014 ◽  
pp. 48-56
Author(s):  
Van Thi Tran ◽  
Van Bang Le ◽  
Thị Thu Huong Hoang

Aim: Some studies have linked the present of chronic obstructive oulmonary disease (COPD) to coronary artery disease (CAD). Low grade systemic inflammation occurs in patients with COPD as well as patients with CAD. This study was designed to find out the concentration differences of hs-CRP and TNF-a in patients having both chronic obstructive pulmonary and coronary artery diseases with those having either. Methods: A cross - sectional descriptive study was conducted in 200 patients undergoing a coronary artery angiography in the Heart Institute, Thong Nhat Hospital and 115 People Hospital. COPD was diagnosed using GOLD classification. Result: Our study had shown that the levels of hs-CRP and TNF-a were statistically increased in patients with COPD, CAD as well as in patients who had COPD with CAD (p<0,05). The levels of hs-CRP were higher in CAD than in COPD nad the levels of TNF-a were higher in COPD than in CAD. In patients with COPD and CAD, there were increased the levels of both hs-CRP and TNF-a in serum. Conclusion: Systemic inflammation presents in both COPD and CAD. Key words: hs-CRP, TNF-a, coronary artery disease (CAD).


2018 ◽  
Vol 69 (2) ◽  
pp. 403-406
Author(s):  
Codrina Ancuta ◽  
Cristina Pomirleanu ◽  
Cristina Iordache ◽  
Magda Ecaterina Antohe ◽  
Rodica Chirieac ◽  
...  

Data about lipoprotein changes and their link with cardiovascular disease and atherosclerosis in systemic sclerosis (SSc) are still challenging. We aimed to evaluate serum lipid profile of patients with SSc and to identify potential relation with different disease specific characteristics (clinical, serological, inflammatory tests) in a cross-sectional study. Standard assessments comprised SSc-related parameters (disease subtype, clinical spectrum, immunological tests) and lipid metabolism (total cholesterol and fractions, triglycerides). Impaired lipid profile (low serum HDL- and high LDL-cholesterol, increased serum triglycerides, slightly modification in total cholesterol level) significantly correlated with diffuse SSc, activity (EUSTAR) and severity (MEDSGER), as well as seropositivity for specific antibodies (anti-centromere and anti-topoisomerase 1). The dyslipidemic profile might represent a pathobiological pathway for atherosclerosis in SSc.


2020 ◽  
Vol 16 (4) ◽  
pp. 285-292 ◽  
Author(s):  
Mohsen Elshahaly ◽  
Iman Abd El Latif ◽  
Hassan Bassiouni

Objective: To describe the clinical features in patients with Behcet’s disease suffering from uveitis in Egypt and to compare the findings between both genders. Methods: 453 subjects fulfilling the 1990 Classification criteria for Behcet’s disease and with uveitis in at least one eye. Follow-up was done for two years. Results: Oral ulcers and recurrent iridocyclitis occurred in all patients during their disease course. The prevalence of genital ulcers, erythema nodosum, and joint involvement were similar between both sexes. The prevalence of all ocular findings was higher in females except for retinal hemorrhages. The incidence of secondary cataract and glaucoma in addition to vitreous opacities was similar between both sexes. 13.7% of patients had GIT manifestations. Vascular complications were present in 128 (28.2%) patients. : Aseptic meningitis and hemiplegia were only found in males. 1.7% of males and 3.2% of females suffered from brainstem involvement. Superior sagittal sinus thrombosis was present in females only. Acute phase reactants (ESR & CRP) were significantly higher in females (p<0.0001). Conclusion: The disease characteristics of BD patients with uveitis in Egypt might be different from the BD patients of other countries. The markers of inflammation were higher in females compared to males. The ocular manifestations were more common in females.


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