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1.

Background

Numerous tools to assess activity of rheumatoid arthritis (RA) are available to use. For any marker to be a more appropriate indicator of disease activity, it should be more authentic to the patho-physiologic basis of the disease.

Aim of the work

To determine the performance of serum adenosine deaminase (sADA) in measuring disease activity in RA.

Patients and Methods

100 RA patients and 100 matched controls were included in the study. The disease activity score (DAS28) with erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were assessed. sADA level was determined by spectrophotometry. The sADA level was integrated in the DAS28 formulae and the corresponding values were determined.

Results

The mean age of the RA patients was 61.8?±?9.7?years, 68% were females and they had a disease duration of 12.5?±?3.7?years. The mean DAS28-ESR was 4.2?±?1.3 and DAS28-CRP 3.5?±?1.1. The mean sADA was significantly higher in the patients (33.6?±?11.6?U/L) compared to the control (25.1?±?9.9?U/L) (p?<?0.001). The sADA level and DAS28-sADA did not differ according to the gender, methotrexate use, rheumatoid factor or anti-citrullinated protein autoantibodies positivity. The mean DAS28-sADA significantly increased in higher activity categories (p?<?0.001). sADA significantly correlated with the disease activity parameters. DAS28-sADA significantly correlated with DAS28-ESR (r?=?0.57, p?<?0.001) and DAS28-CRP (r?=?0.604, p?<?0.001). DAS28-sADA showed a sensitivity of 0.9 and specificity 0.69 for detection of disease activity measured with DAS28-ESR and was 0.88 and 0.65 when measured with DAS28-CRP.

Conclusion

Integration of sADA in the DAS28 index can be a useful marker that reflects RA activity.  相似文献   

2.

Aim of the work

To determine the serum progranulin levels in rheumatoid arthritis (RA) patients and to study its relation with disease activity assessed clinically and by ultrasound (US).

Patients and methods

The study included 52 RA patients and 19 age and sex matched controls. Disease activity score (DAS-28) and modified health assessment questionaire were assessed. Progranulin was measured by ELISA. Ultrasound examination was performed and the German US7 score (USS) recorded.

Results

The patients mean age was 42.8?±?10.5?years; disease duration was 4.9?±?5.02?years; 47 females and 5 males with a mean DAS28 of 4.4?±?0.9 (3 in remission; 5 low activity; 31 moderate and 13 high). The mean serum progranulin level in patients (9.5?±?45.5?ng/ml) was significantly elevated compared to control (32.74?±?9.2?ng/ml) (p?<?0.0001). There was a significant difference in the progranulin levels and USS according to the grades of disease activity (p?<?0.0001 and p?=?0.037 respectively). The progranulin and USS significantly correlated with the DAS28 (r?=?0.64, r?=?0.58; p?<?0.0001 respectively) and erythrocyte sedimentation rate (p?<?0.0001). The progranulin and USS significantly correlated with each other (r?=?0.32, p?=?0.02). At a cut-off value 51.5?ng/ml, progranulin would discriminate between patients and control at sensitivity 96.2%, specificity 100% and accuracy 99%.

Conclusion

Serum progranulin levels were higher in RA patients than age and sex matched controls. It significantly correlated with disease activity measured by DAS28, ESR and ultrasound activity measured by German US7 score. Serum progranulin levels may be a useful biomarker in RA disease. Ultrasound correlated with ESR and DAS28 in RA patients.  相似文献   

3.

Aim of the work

To measure serum peptidyl arginine deiminase (PADI4) enzyme level in rheumatoid arthritis (RA) patients and to assess its role in diagnosis and monitoring patient improvement.

Patients and methods

The study included 31 RA patients and 10 age and sex matched healthy controls. Serum PADI4 and anti-cyclic citrullinated peptide (anti-CCP) were measured. Disease activity score (DAS28) was calculated. Patients were followed-up at 3 months with measurement of serum PADI4, anti-CCP and DAS28.

Results

Mean age of the patients was 42.4?±?7.8 years, female:male?=?5.2:1 and disease duration 8.1?±?5.4 years. The DAS28 was 5?±?1.2 and modified health assessment questionnaire (MHAQ) was 0.8?±?0.6. Mean anti-CCP (129.3?±?74.8?ng/ml) and PADI-4 levels (532.9?±?240.9?pg/ml) in patients were significantly higher than in controls (8.4?±?4.4?ng/ml and 156?±?31?pg/ml respectively; p?<?0.001 both). At a cut-off value?≥?250?pg/ml, PADI4 was highly diagnostic for RA (sensitivity 90.3%, specificity 100%; area under curve?=?0.97 with no significant difference from anti-CCP at a cut-off of 15 ng/ml (sensitivity 93.5%, specificity 100%; AUC ?=?0.99). Serum PADI4 level showed a significant correlation with DAS28 (r?=?0.49, p?=?0.047). At follow up (n?=?20), no significant difference in PADI4, anti-CCP level or DAS28 scores was found but changes in serum PADI4 level significantly correlated with changes in DAS28 (r?=?0.82, p?<?0.001). Mean serum PADI4 level was significantly lower in improved patients (184.4?±?10.2?pg/ml) compared to unimproved (563.3?±?251.9?pg/ml, p?<?0.001).

Conclusion

Serum PADI4 was diagnostic for RA and comparable with anti-CCP. It correlated with disease activity and could be a promising follow up marker of remission.  相似文献   

4.

Background

The diagnosis of early rheumatoid arthritis (RA) is challenging. B-cell chemokine (CXCL13) plays a critical role in the disease pathogenesis.

Aim of the work

To assess the diagnostic value of serum CXCL13 in early RA and compare it with rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) antibodies.

Patients and methods

The study included 60 RA patients; 30 early, 30 established RA and 30 healthy controls. The modified health assessment questionnaire (MHAQ), modified Sharp-van der Heijde score (MSS) and disease activity score (DAS28) were assessed in RA patients. RF, anti-CCP and serum level of CXCL13 were measured.

Results

Patients had a mean age of 39?±?7.4?years and disease duration of 4.4?±?5.7?years; they were 46 females and 12 males (F:M 3.8:1). Serum CXCL13 was significantly higher in early (191.7?±?74.4?pg/ml) compared to established (136.4?±?79?pg/ml) RA (p?=?0.007) which were not observed with RF and anti-CCP; both were higher than in control (30.4?±?13.5?pg/ml) (p?<?0.001). In early RA, the frequencies of CXCL13, RF and anti-CCP positivity were 90%, 73.3% and 56.7% while in the established cases the frequencies were 36.7%, 66.7% and 63.3% respectively. CXCL13 significantly correlated with DAS28 (early: 0.49, p?=?0.006; established: r?=?0.38, p?=?0.04) but not with MHAQ or MSS. The CXCL13 significantly correlated with both the RF and anti-CCP in both early and established cases (p?<?0.001).

Conclusion

CXCL13 is an important for the diagnosis of early RA with a superior diagnostic performance compared to RF and anti-CCP. It may also be considered a potential biomarker of disease activity.  相似文献   

5.

Background

Evaluation of remission in Rheumatoid Arthritis (RA) largely relies on composite scores based on clinical and laboratory assessments however, patients can fulfill clinical remission criteria as defined by composite scores, yet still have evidence of synovitis detectable on imaging.

Aim of the work

To evaluate hand and wrist joints in patients with RA in clinical remission using power Doppler (PD) ultrasonography and to study the association between ultrasonographic findings and composite index scores.

Patients and methods

This study was conducted on 50 RA patients in clinical remission. Ten matched healthy subjects were included as control. The modified health assessment questionnaire (MHAQ) was assessed in the patients; disease activity was calculated using a composite index score including disease activity score (DAS28) and clinical disease activity index (CDAI). Ultrasonographic assessment of the hand and wrist joints was performed.

Results

The mean age of the patients was 50.9?±?9.2?years, disease duration was 10.6?±?5.5?years and were 38 females and 12 males. The mean DAS28 was 2.3?±?0.3. On ultrasonographic examination, 14 (28%) patients had normal synovium, while 18 (36%) showed synovial hypertrophy without evidence of inflammation and 18 (36%) had PD signals. DAS28 was higher in patients with PD signals (2.36?±?0.3) compared to those without synovitis (2.3?±?0.28). There was a significant correlation between PD activity and CDAI (p?=?0.005), MHAQ (p?=?0.002) and disease duration (p?=?0.023).

Conclusion

Power Doppler ultrasound can detect residual inflammation in RA patients in clinical remission and its scores were signficantly associated with the clinical disease activity index and functional status.  相似文献   

6.

Objective

To assess the 2-year effect on disease activity and health-related quality of life (HRQoL) of implementing a clinical practice treat-to-target (T2T) strategy in patients with rheumatoid arthritis (RA).

Methods

Patients in the Norwegian Very Early Arthritis Cohort 2.0 (NOR-VEAC 2.0), included 2010–2015, were treated according to T2T principles with visits at baseline, 3, 6, 9, 12 months, then every 6 months plus monthly visits until DAS28?<2.6. These patients were compared to a pre-T2T cohort of patients included in the Norwegian Disease Modifying Anti-Rheumatic Drug (NOR-DMARD) register 2006–2009. Both groups had a clinical diagnosis of RA (≤1 year) and were DMARD naïve. Disease activity and HRQoL outcomes were analysed, and the primary outcome was SDAI remission (≤3.3) at 2years.

Results

The T2T cohort included 293 patients (mean (SD) age 54 (13) years, 66% females, disease duration median (25,75 perc) 98 (57,164) days) and the routine care cohort 392 patients (age 54 (13) years, 68% females, 4 (0,30) days since diagnosis). At 2years, the proportion of patients achieving SDAI remission was 46% in the T2T cohort compared to 31% in the routine care cohort. EQ-5D was similar at baseline, but differed significantly between groups at 2years (median (25,75 perc) 0.77 (0.69, 0.85) vs 0.73 (0.59, 0.80), p?<?0.001). Methotrexate monotherapy was the dominant DMARD regimen used to achieve SDAI remission in both cohorts.

Conclusion

Higher remission rates and better HRQoL were achieved in patients following a T2T strategy in clinical practice compared to routine care.  相似文献   

7.

Aim of the work

The aim of this study was to evaluate interleukin-23 (IL-23) level in the sera of rheumatoid arthritis (RA) patients and to determine its relation with disease activity and severity.

Patients and methods

This study was carried out on 40 RA patients and 40 healthy control subjects. All patients were subjected to full history taking, thorough clinical examination, radiological and laboratory investigations including C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), rheumatoid factor (RF), anti-cyclic-citrullinated peptide (anti-CCP) antibodies. Serum IL-23 was measured by enzyme-linked immunosorbent assay. Disease activity score (DAS-28) and rheumatoid arthritis severity scale (RASS) were assessed.

Results

Patient's mean age was 43.3?±?10.4?years and they were 37 females and 3 males. The mean disease duration of the patients was 4.98?±?4.1?years (1–15?years) with a mean DAS-28 of 4.8?±?1.2 (2.4–7.6) and RASS of 41.1?±?16.9 (16.7–85). The mean IL-23 serum level was significantly higher in RA patients (67.6?±?39.2?pg/ml) compared to the control (37.7?±?15.6?pg/ml) (p?<?0.001). There were significant correlations between IL-23 levels with the DAS-28 (r?=?0.35,?p?=?0.02), RASS (r?=?0.31, p?=?0.04), CRP (r?=?0.39, p?=?0.02), ESR (r?=?0.45, p?=?0.004), RF (r?=?0.48, p?=?0.002) and anti-CCP antibodies (r?=?0.35, p?=?0.04). At a cut-off value of 45?(pg/ml), IL-23 had a sensitivity of 77.8% and a specificity of 75% for detection of active disease and at 43.5?pg/ml the sensitivity was 88.2% and specificity 83.3% for occurrence of physical damage.

Conclusion

IL-23 could be a useful marker for disease activity in RA. Its correlation with RASS suggested that IL-23 might be a therapeutic target for prevention of disability.  相似文献   

8.

Aim of the work

Cardiovascular diseases represent a major source of morbidity and mortality for patients with rheumatoid arthritis (RA). The increase in aortic stiffness, carotid intima-media thickness (CIMT) and serum osteoprotegerin (OPG) have been shown to be independent risk factors for cardiovascular events. This work aimed to investigate the clinical significance of these parameters in RA patients.

Patients and methods

60 RA patients and 30 control with no primary cardiovascular risk factors were included. Disease activity score (DAS28) was assessed in patients. Aortic stiffness was evaluated by transthoracic echocardiography and CIMT evaluated by Doppler ultrasonography. OPG was determined by ELISA.

Results

The 60 RA patients had a mean age of 40.8?±?8.3?years, disease duration of 6.9?±?4.9?years and were 46 females and 14 males. In RA patients, serum OPG and CIMT (thickest and mean) were significantly higher than the control (60.5?±?32.4?pg/ml vs 29.4?±?16.7?pg/ml, p?<?0.001; 0.73?±?0.18?mm vs 0.63?±?0.13?mm, p?<?0.001; 0.61?±?0.1?mm vs 0, 56?±?0.1?mm, p?=?0.007, respectively). The aortic stiffness tended to be higher in patients (6.9?±?4.8 vs 5.2?±?2.5, p?=?0.114) and in males (9.7?±?7.4) vs females (5.7?±?3.4, p?=?0.013). OPG levels were significantly higher in those with erosions (n?=?41) (68.6?±?34.5?pg/ml vs 49.1?±?22?pg/ml p?=?0.038) and in those seropositive (n?=?54) (65.4?±?32.2?pg/ml vs 36?±?18.3?pg/ml p?=?0.012). In patients, CIMT (thickest and mean) correlated significantly with the aortic stiffness (p?=?0.02 and p?=?0.04 respectively).

Conclusion

RA is an independent risk factor associated with cardiovascular events. For determining this risk, measuring the serum OPG, CIMT and aortic stiffness may be a useful guide.  相似文献   

9.

Aim of the work

This work aimed to measure serum ferritin and transferrin levels and to study the presence of metabolic syndrome (MetS) in Egyptian systemic lupus erythematosus (SLE) females and to correlate them with disease activity, damage, clinical status and subclinical atherosclerosis.

Patients and methods

The study included 50 SLE female patients and 25 matched control. SLE disease activity index (SLEDAI) and damage index (DI) were assessed and the presence of MetS determined. Serum ferritin was measured by enhanced chemi-luminescence and the carotid intima-media thickness (cIMT) was assessed by B-mode ultrasound.

Results

The mean cIMT (0.71?±?0.14?mm) and ferritin (2098?±?132.99?ng/ml) were significantly higher in patients compared to controls (0.62?±?0.05?mm and 71.7?±?18.7?ng/ml; p?=?0.003 and p?<?0.001, respectively). 28% of patients and 12% controls had MetS. 6(12%) had a thickened cIMT (≥0.9?mm), 3 of them had atherosclerotic plaques (≥1.3?mm). The cIMT significantly correlated (p?<?0.05) with age (r?=?0.54), disease duration (r?=?0.55), SLEDAI (r?=?0.37), DI (r?=?0.52), ferritin (r?=?0.31), cholesterol (r?=?0.32), triglycerides (r?=?0.7), fasting blood sugar (r?=?0.72), systolic (r?=?0.68) and diastolic (r?=?0.7) blood pressure and negatively with transferrin (r?=??0.31), low (r?=??0.32) and high-density lipoprotein (r?=??0.53) and C3 (r?=??0.66). Patients with MetS had significantly higher cIMT (0.9?±?0.3?mm) versus those without (0.64?±?0.1?mm)(p?<?0.0001).

Conclusion

MetS in SLE is a associated with accelerated atherosclerosis while serum ferritin and transferrin are strong indicators of SLE activity and damage. Considering the association with MetS and measuring the cIMT in SLE patients is recommended and provides a useful marker for detecting subclinical cases and predicting future cardiovascular events.  相似文献   

10.

Aim of the work

This study aimed to assess long-term articular damage and function in rheumatoid arthritis (RA) patients in relation to the type of treatment. Early disease modifying anti-rheumatic drug (DMARD) therapy has not been evaluated in this study.

Patients and methods

One hundred and fifty RA patients (141 females and 9 males) with disease duration more than five years and disease activity score-28 (DAS-28) <3.2 were included in this study. Sixty patients received disease modifying anti-rheumatic drugs (DMARDs) (group A), 60 received DMARDs and corticosteroids (CS) (group B), and 30 patients received CS only (group C). The functional outcome was assessed using the Modified Health Assessment Questionnaire (MHAQ). The articular damage was measured by using Rheumatoid Arthritis Articular Damage (RAAD) Score. The van der Heijde modification of the Sharp erosion score was used to define the plain X-ray of both hands and feet.

Results

The mean age of the patients was 49.3?±?11.5?years, and disease duration was 12?±?7.9?years. There was a significant difference between the RAAD, visual analogue scale and MHAQ scores among the three groups (p?=?0.001), with higher score in group C followed by B. By using Multiple regression analysis to examine the relationship between RAAD score and other independent variables there was a significant association of the RAAD score with higher X-ray score (p?<?0.001) and with patients taking steroids only (p?=?0.001).

Conclusion

Although, use of corticosteroids may help to control the disease activity, but it increases the risk of overall joint damage.  相似文献   

11.

Background

There have been no reports on the relationship between lung radiological patterns and rheumatoid arthritis (RA) disease activity or RA treatment response in patients with RA-associated lung disease (RA-LD).

Methods

Patients with RA-LD who underwent treatment for RA from April 2005 to March 2015 were retrospectively evaluated. RA-LD patients were divided into three groups based on high-resolution computed tomography (HRCT) patterns [usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), and bronchiolitis]. The disease activity score of 28 joints-erythrocyte sedimentation rate (DAS28-ESR) and the response of RA to treatment, as measured by the European League Against Rheumatism (EULAR) response criteria, were investigated.

Results

A total of 77 patients (21 with UIP, 23 with NSIP, and 33 with bronchiolitis) were enrolled. Median scores (interquartile range) on the DAS28-ESR at baseline were 5.27 (4.76–5.74), 5.48 (4.24–6.34), and 5.04 (3.90–5.66) for UIP, NSIP, and bronchiolitis, respectively; there were no statistical differences between the three groups (p = 0.412). One year after baseline, 19 (90%), 14 (61%), and 19 (58%) of patients in the UIP, NSIP, and bronchiolitis groups, respectively, were considered good or moderate responders, as evaluated using the EULAR response criteria; there was a significant difference between these three groups (p = 0.014). Multiple logistic regression analysis revealed that the UIP pattern was significantly associated with good or moderate response to RA treatment 1 year after baseline (p = 0.012).

Conclusions

These results suggest that NSIP and bronchiolitis HRCT patterns may be risk factors for resistance to RA therapy.  相似文献   

12.

Objectives

To describe the prevalence of comorbidities in patients with RA in Spain and discuss their management and implications using data from the Spanish cohort of the multinational study on COMOrbidities in Rheumatoid Arthritis (COMORA).

Methods

This is a national sub-analysis of the COMORA study. We studied the demographics and disease characteristics of 200 adults patients diagnosed with RA (1987 ACR), and routine practices for screening and preventing the following selected comorbidities: cardiovascular, infections, cancer, gastrointestinal, pulmonary, osteoporosis and depression.

Results

Patients had a mean age of 58 years and a mean RA duration of 10 years. Mean DAS28 score was 3.3 and approximately 25% of patients were in remission (DAS28 <2.6). Forty-four (22%) patients had ≥1 comorbidity, the most frequent being depression (27%) and obesity (26%). A history of myocardial infarction or stroke was observed in 5% and 1% of patients, respectively, and any solid tumor in 6%. Having a Framingham Risk Score >20% (51%), hypercholesterolemia (46%) or hypertension (41%) and smoking (25%) were the most common CV risk factors. For prostate, colon and skin cancers, only 9%, 10% and 18% of patients, respectively, were optimally monitored. Infections were also inadequately managed, with 7% and 17% of patients vaccinated against influenza and pneumococcal, respectively, as was osteoporosis, with 47% of patients supplemented with vitamin D and 56% with a bone densitometry performed.

Conclusions

In Spain, the prevalence of comorbidities and CV risk factors in RA patients with established and advanced disease is relatively high, and their management in clinical daily practice remains suboptimal.  相似文献   

13.

Background

The tolerability and utility of combination doxycycline and ursodeoxycholic acid (ursodiol) amyloid fibril disruption therapy for transthyretin cardiac amyloidosis (ATTR CA) in clinical practice is poorly described.

Methods and Results

We report the clinical experience of 53 ATTR CA patients treated with doxycycline and ursodiol. Six patients (11%) did not tolerate the therapy owing to dermatologic and gastrointestinal effects. Of those remaining, the median follow-up was 22 months (range 8–30), mean age was 71 ± 11years, 41 (87%) were male, and 42 (89%) had wild-type and 5 (11%) mutant ATTR. Five patients (11%) died during follow-up. There was no significant change in New York Heart Association (NYHA) functional class, cardiac biomarkers, or echocardiographic parameters during follow-up. Left ventricular (LV) global longitudinal systolic strain (GLS) improved in 16 patients (38%) (?12 ± 4% to ?17 ± 4%; P < .01). Patients whose LV GLS improved were significantly younger and had lower NYHA functional class, troponin-T, N-terminal pro–B-type natriuretic peptide (BNP), and baseline LV GLS levels compared with those whose LV GLS did not improve. Troponin-T improved in follow-up for patients whose LV GLS improved (35 ± 21 to 20 ± 14 ng/L; P?=?.06).

Conclusions

Doxycycline and ursodiol therapy for treatment of ATTR CA was tolerable and was associated with stabilized markers of disease progression. LV GLS improved in patients with less advanced disease.  相似文献   

14.

Aims

To analyze the role of serum miR-125b-5p in reflecting liver damage and predicting outcomes in chronic hepatitis B (CHB) patients with acute-on-chronic liver failure (ACLF).

Methods

CHB patients with normal hepatic function (n?=?100), moderate-to-severe liver damage (n?=?90), and ACLF (n?=?136) were included. Among hepatitis B virus (HBV)-ACLF patients, 86 and 50 were in the training and validation cohorts, respectively. Serum miR-125b-5p level was measured by quantitative real-time PCR.

Results

Serum miR-125b-5p level increased with disease progression, and serum miR-125b-5p level was lower in surviving than in dead HBV-ACLF patients. Among HBV-ACLF patients, miR-125b-5p positively correlated with total bilirubin (TBil; r?=?0.214, p?<?0.05) and model for end-stage liver disease (MELD) score (r?=?0.382, p?<?0.001) and negatively correlated with prothrombin activity(PTA; r?=??0.215, p?<?0.05). MiR-122 showed a contrasting performance compared with miR-125b-5p. Cox regression analysis showed that miR-125b-5p, miR-122, and PTA were independent survival predictors for HBV-ACLF, and low miR-125b-5p and high miR-122 levels may predict a longer survival in HBV-ACLF. MiR-125b-5p (AUC?=?0.814) had a higher performance for survival prediction in HBV-ACLF compared with miR-122 (AUC?=?0.804), PTA (AUC?=?0.762), MELD score (AUC?=?0.799), and TBil (AUC?=?0.670) alone; predictive effectiveness of miR-125b-5p was increased by combination with miR-122 (AUC?=?0.898). MiR-125b-5p was an effective predictor of HBV-ACLF outcomes in the validation cohort.

Conclusions

MiR-125b-5p increase is associated with severity of liver damage; high serum miR-125b-5p may serve as a predictor for poor outcomes in HBV-ACLF cases.  相似文献   

15.

Objectives

The aim of this study was to investigate the prognostic value of echocardiographic deformation imaging in arrhythmogenic right ventricular cardiomyopathy (ARVC) to optimize family screening protocols.

Background

ARVC is characterized by variable disease expressivity among family members, which complicates family screening protocols. Previous reports have shown that echocardiographic deformation imaging detects abnormal right ventricular (RV) deformation in the absence of established disease expression in ARVC.

Methods

First-degree relatives of patients with ARVC were evaluated according to 2010 task force criteria, including RV deformation imaging (n = 128). Relatives fulfilling structural task force criteria were excluded for further analysis. At baseline, deformation patterns of the subtricuspid region were scored as type I (normal deformation), type II (delayed onset, decreased systolic peak, and post-systolic shortening), or type III (systolic stretching and large post-systolic shortening). The final study population comprised relatives who underwent a second evaluation during follow-up. Disease progression was defined as the development of a new 2010 task force criterion during follow-up that was absent at baseline.

Results

Sixty-five relatives underwent a second evaluation after a mean follow-up period of 3.7 ± 2.1 years. At baseline, 28 relatives (43%) had normal deformation (type I), and 37 relatives (57%) had abnormal deformation (type II or III) in the subtricuspid region. Disease progression occurred in 4% of the relatives with normal deformation at baseline and in 43% of the relatives with abnormal deformation at baseline (p < 0.001). Positive and negative predictive values of abnormal deformation were, respectively, 43% (95% confidence interval: 27% to 61%) and 96% (95% confidence interval: 82% to 100%).

Conclusions

Normal RV deformation in the subtricuspid region is associated with absence of disease progression during nearly 4-year follow-up in relatives of patients with ARVC. Abnormal RV deformation seems to precede the established signs of ARVC. RV deformation imaging may potentially play an important role in ARVC family screening protocols.  相似文献   

16.

Aim of the work

To determine the frequency of critical complications of systemic lupus erythematosus (SLE) admitted to the intensive care unit (ICU), study the risk factors and outcome.

Patients and methods

Fifty SLE patients consequently admitted to the ICU were prospectively studied. The SLE Disease Activity Index (SLEDAI) was assessed.

Results

The mean age of the patients was 29.3?±?8.7?years; they were 42 females (84%) and disease duration of 4.9?±?3.4?years. The overall mortality was 24% (12 patients) and tended to be higher in males (37.5% vs 21.5%). The commonest causes of death were infection (p?<?0.001) and pulmonary complications (p?=?0.04) in all non-survivors. Metabolic acidosis was significantly increased in deceased patients (75%) compared to survivors (23.7%) (p?=?0.003). Cardiac and CNS complications were significantly increased in non-survivors (p?=?0.04 and p?=?0.03 respectively). Acute renal failure was significantly more frequent in mortality case 9/12 compared to survivors (28.9%) (p?=?0.007) as well as abnormal arterial blood gases (100% vs 57.9%; p?=?0.005). The SLEDAI was significantly increased in non-survivors (41.8?±?8.2) compared to survivors (21.4?±?5.1) (p?=?0.001). There was a significant correlation between mortality and SLEDAI (r?=?0.58, p?=?0.001) and inversely with the pH (r?=??0.38, p?=?0.01). On multiple regression, only increasing SLEDAI was a significant predictor of mortality (β0.26, OR 1.29, 95%CI 1.12–1.49; p?<?0.0001). Mortality prediction by SLEDAI showed at a cut-off of 28.5; sensitivity 84% and specificity 90% (p?=?0.001).

Conclusion

SLE patients admitted to the ICU are at an increased risk of mortality especially those with high disease activity. The main causes of mortality were infection, respiratory, cardiac and neurological complications.  相似文献   

17.

Objectives

To detect systolic dysfunction in heart failure with preserved ejection fraction (HFpEF) patients by using global longitudinal strain (GLS).

Methods

This study included 46 heart failure patients: 24 with heart failure with reduced ejection fraction (HFrEF) and 22 with heart failure with preserved ejection fraction (HFpEF), and 20 patients with similar risk factor but no symptoms or signs of heart failure, matched for age and sex, as controls. All patients were screened by echocardiography. The ejection fraction of left ventricle was measured using Simpson’s method and the GLS of the left ventricle was measured by using two-dimensional speckle tracking.

Results

Left ventricular ejection fraction (LVEF) was 61.90?±?2.94% in the controls, 60.45?±?7.4% in the HFpEF group (p?=?0.421), and 32.75?±?8.45% in the HFrEF group (p?=?0.001). The value of left ventricle (LV) GLS (controls?=???19.74?±?1.12%, HFpEF?=???15.03?±?2.03%, HFrEF?=???10.72?±?1.99%, p?=?0.0001) was significantly impaired in the HFpEF group despite normal LVEF.

Conclusion

There is significant left ventricular systolic impairment detected by GLS despite preserved LVEF.  相似文献   

18.

Background

Endothelial cell dysfunction has been described in Behçet disease (BD) not only as a cause of major vascular events but also related to chronic inflammation in different organ systems.

Aim of the work

To study the relation of serum endocan, a marker of endothelial dysfunction, with clinical manifestations and disease activity in BD patients.

Patients and methods

This study included 23 BD patients and 23 matched controls. Disease activity was assessed by the Behcet Disease Current Activity Form (BDCAF). Serum endocan was measured in all subjects.

Results

The mean age of the patients was 32.5?±?6.8?years and they were 16 males and 7 females (M:F 2.3:1) with mean disease duration of 7?±?5.2?years. Their mean BDCAF was 2.26?±?1.32. A significant difference was found between serum endocan level among active patients 328.24?±?195.3?ng/L, inactive patients (169.8?±?35.7?ng/L) and controls (160.6?±?39.7?ng/L)(p?=?0.001). Patients with genital ulcers, papulopastular lesions and arthritis at the time of the study had higher serum endocan level than those without (p?=?0.002, p?=?0.006 and p?=?0.0001 respectively). Serum endocan levels correlated significantly with the BDCAF, neutrophil/lymphocyte ratio, platelet lymphocyte ratio and C-reactive protein (r?=?0.94, p?=?0.0001; r?=?0.82, p?=?0.0001, r?=?0.44, p?=?0.04 and r?=?0.48, p?=?0.02 respectively). The optimum serum endocan cut-off point for active BD was 191.5?ng/L with a sensitivity and specificity of 100% and 86% respectively (area under curve 0.99, 95% confidence interval 0.96-1).

Conclusion

Serum endocan may serve as a potential marker of disease activity in BD. Patients with genital ulcers, papulopastular lesions and arthritis showed higher serum endocan levels.  相似文献   

19.

Aim of the work

To present the clinical and laboratory disease characteristics in Egyptian patients with progressive systemic sclerosis (SSc) and their nail fold capillaroscopy findings.

Patients and methods

50 SSc patients were included. For all patients detailed history taking and clinical and rheumatological examination were performed. The modified Rodnan skin score (mRSS) and nail fold capillaroscopy were assessed.

Results

The mean age was 41.4?±?12.7?years (range 16–67?years) and median disease duration was 5?years (range 1–40?years). Eight patients (16%) were males and 42 (84%) females (M:F 1:5.25). Skin tightness was present in all patients; mean mRSS was 17.5?±?10.4 (ranges 4–45). 44 (88%) patients had abnormal pulmonary function tests; 42 (84%) restrictive and 2 (4%) obstructive; 25 (50%) presented with interstitial lung disease. Pulmonary artery hypertension was recorded in 9 (18%) patients. Renal manifestations were the least common recorded system involvement (20%). Antinuclear antibodies were positive in 98%, rheumatoid factor in 4%, antitopoisomerase I in 36% and anticentromere in 8% of patients. 96% of patient had abnormal nail fold capillaroscopy. There was a significant negative correlation between mRSS with forced expiratory volume (r?=??0.46, p?=?0.001), forced volume capacity (r?=??0.47, p?=?0.001) and positively with the FEV1/FVC (r?=?0.3, p?=?0.035).

Conclusion

Almost all patients have ANA seropositivity, abnormal PFTs and abnormal nailfold capillaroscopy. Antitopoisomerase I antibody seropositivity, ILD, abnormal PFT, worsening skin score, late pattern of nailfold capillaroscopy are more common in dcSSc. Extensive clinical examination and investigations should be considered for early diagnosis and follow up of Egyptian SSc patients.  相似文献   

20.

Background and aims

Metabolic syndrome (MetS) is a cluster of multiple risk factors including central obesity that may lead to cardiac damage and cardiovascular events. We investigated whether visceral obesity induces cardiac structural and functional remodeling independently from central obesity and other risk factors in subjects with suspected MetS.

Methods and results

We studied 229 participants with suspected MetS. Visceral fat area (VFA) was measured by bioelectrical impedance analysis. Left ventricular (LV) mass index, early diastolic velocity of mitral annulus (e′), and LV global longitudinal strain (GLS) were measured by echocardiography. Subjects were categorized into high and low VFA group (VFAh and VFAl). MetS was more prevalent in the VFAh than in the VFAl (p = 0.004). The VFAh had a higher waist circumference (WC) than the VFAl (p < 0.001). LV mass index was higher, but e' and GLS were lower in the VFAh than in VFAl (all p < 0.05). VFA was well correlated with blood pressure, fasting blood glucose, triglyceride, high-sensitivity C-reactive protein and adiponectin (all p < 0.05). VFA was correlated to LV mass index, e’, and GLS (all p < 0.05) and was independently associated with GLS after adjustment for other risk factors, including WC (p = 0.005).

Conclusions

Visceral obesity assessed by VFA was well correlated with parameters of MetS. Visceral obesity, but not central obesity measured by WC, was independently associated with structural and functional cardiac remodeling in subjects with suspected MetS. It suggests that visceral obesity should be considered as an important risk factor for cardiac damage in dysmetabolic subjects.

Trial registration

NCT02077530 (date of registration: November 1, 2013).  相似文献   

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