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1.

Background

Systemic sclerosis (SSc) is an autoimmune chronic disease characterized by vascular impairment, immune dysfunction and collagen deposition. Raynaud’s phenomenon (RP) and digital ulcers (DU) are prominent features of SSc. Intravenous (IV) iloprost (ILO), according to the recently updated EULAR recommendations, is indicated for RP after failure of oral therapy. Moreover, IV ILO could be useful in DU healing. IV ILO is currently available mainly on the European market approved for RP secondary to SSc with 3–5 days infusion cycle. Unfortunately, data published varies regarding regimen (dosage, duration and frequency). Up to now, ILO has been studied in small cohorts of patients and in few randomized controlled trials.

Methods

A systematic review of studies on IV ILO in patients with SSc complicated by DU and RP was performed. Insufficient data were available to perform a meta-analysis according to the GRADE system. We performed a three-stage internet-based Delphi consensus exercise.

Results

Three major indications were identified for IV ILO usage in SSc: RP non-responsive to oral therapy, DU healing, and DU prevention. IV ILO should be administered between 0.5 and 2.0 ng/kg/min according to patient tolerability with a frequency depending on the indication.

Conclusions

Although these suggestions are supported by this expert group to be used in clinical setting, it will be necessary to formally validate the present suggestions in future clinical trials.  相似文献   

2.

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   

3.
4.

Background

To estimate patient acceptable symptom state (PASS) and minimal clinically important difference (MCID) for patient-reported outcomes in systemic sclerosis (SSc).

Methods

We conducted a secondary analysis of the SCLEREDUC trial, a 12-month randomized controlled trial comparing the efficacy of physical therapy to usual care in 220 SSc patients followed-up from September 2005 to October 2010. Self-rated state and change in patient health at 12 months were assessed by using 2 external anchors extracted from the Medical Outcomes Study 36-Item Short-Form. Patients who self-rated their health as “excellent”, “very good” or “good” were the PASS group and those who self-rated their health change as “somewhat better” were the MCID group. Main outcomes were the estimates of PASS by using the 75th percentile method and of MCID by using the mean change in scores method for pain and activity limitation.

Results

PASS (95% confidence interval) and mean (SD) MCID estimates at 12 months were 53.75 (34.00 to 68.00) and ?6.74 (32.02) for the joint-pain visual analog scale (range 0–100), 1.41 (1.13 to 1.63) and ?0.21 (0.48) for the Health Assessment Questionnaire (HAQ, range 0–3), 1.27 (1.07 to 1.62) and ?0.13 (0.45) for the scleroderma HAQ (range 0–3), 26.00 (17.00 to 37.00) and -3.38 (9.87) for the Cochin Hand Function Scale (range 0–90), and 19.40 (17.20 to 21.90) and ?5.69 (6.79) for the McMaster-Toronto Arthritis Patient Preference Disability Questionnaire (range 0–30), respectively.

Conclusions

We provide, for the first time, the PASS and MCID estimates for pain and activity limitation in SSc.

Trial registration

ClinicalTrials.gov Identifier: NCT00318188. First Posted: April 26, 2006.  相似文献   

5.

Background

We compared pre-emptive transplant rates between SLE and non-SLE end-stage renal disease (ESRD) from the U.S. Renal Data System (USRDS) and investigated the potential influence of frequency matching and primary ESRD causes in the non-SLE group.

Methods

4830 adult SLE patients with incident ESRD from USRDS 2005–2009 were frequency matched by age, sex and race to 4830 patients with incident non-SLE ESRD. Multivariable logistic regression models were used to estimate the odds of pre-emptive transplantation in SLE and non-SLE, and with the non-SLE subgroups by primary ESRD cause.

Results

The odds ratios (OR) of receiving a pre-emptive transplant were similar among non-SLE and SLE (referent group): OR?=?1.18 (95% CI: 0.92, 1.50; p?=?0.20). However, the ORs for receiving a pre-emptive transplant were 0.19 (95% CI: 0.08, 0.42) in type 2 diabetes ESRD, 0.42 (95% CI: 0.23, 0.75) for hypertension-associated ESRD, 1.67 (95% CI: 1.10, 2.54) in type 1 diabetes ESRD, and 2.06 (95% CI: 1.55, 2.73) for “other” ESRD. In contrast to non-SLE, younger SLE patients were less likely to receive a pre-emptive transplant than older SLE patients.

Conclusion

The results of this study provide compelling evidence that major improvements need to be made in optimizing access to pre-emptive transplantation in SLE by addressing sociodemographic disparities and the unique challenges faced by SLE patients. Applying careful matching and selecting appropriate comparison groups in future studies may facilitate the development of effective strategies to address these barriers and to increase the number of pre-emptive renal transplants among SLE patients.  相似文献   

6.

Background

Idiopathic pulmonary fibrosis (IPF) is a chronically progressive lung disease with exceptionally poor prognosis. While lung transplantation (LTx) is considered the last-resort therapeutic option, dismal waitlist mortality still hampers the salvage of patients with IPF. Pirfenidone, originally designed for IPF treatment, has increasingly been utilized. This study aimed to evaluate whether Pirfenidone could influence outcomes of patients with IPF on the Japanese LTx waitlist.

Methods

This retrospective single-center cohort study included 25 consecutive patients with IPF who were registered as LTx candidates at our institution between July 1999 and August 2016. Patients with a history of pretransplant Pirfenidone therapy (Pirfenidone group) were compared with those with no history (non-Pirfenidone group).

Results

In total, 6 (24%) patients received Pirfenidone as pretransplant therapy for 45.2 (range, 18.6–66.8) months. During the treatment period, the Pirfenidone group achieved a significant reduction in the decline rate of the forced vital capacity (-6.2% vs. -0.3%, p = 0.04) and a lower lung allocation score (31 vs. 41, p = 0.013) compared with the non-Pirfenidone group. The Pirfenidone group exhibited 100% waitlist survival three years after registration that was comparable to other indications, and 66% of the patients were still alive at the time of organ availability. No patient in the Pirfenidone group developed Pirfenidone-related surgical complications postoperatively.

Conclusions

Patients with IPF successfully managed with long-term Pirfenidone therapy achieved favorable outcomes after LTx registration, comparable to other patients with LTx indications. The tolerability to antifibrotic therapy can be a predictor of waitlist survival.  相似文献   

7.

Introduction

End-stage renal disease (ESRD) due to lupus nephritis (LN) occurs in 10%-30% of patients. Initially systemic lupus erythematosus (SLE) was a contraindication for kidney transplantation (KT). Today, long-term graft survival remains controversial. Our objective was to compare the survival after KT in patients with SLE or other causes of ESRD.

Methods

All SLE patients who had undergone KT in a retrospective cohort were included. Renal graft survival was compared with that of 50 controls, matched for age, sex, and year of transplantation. Survival was evaluated by the Kaplan-Meier test and the Cox proportional hazards model.

Results

Twenty-five subjects with SLE were included. The estimated 1-year, 2- and 5-year survival rates for patients with SLE were 92%, 66% and 66%. Renal graft survival did not differ between patients with SLE and other causes of ESRD (P = .39). The multivariate analysis showed no significant difference in graft survival between the two groups (hazard ratio, HR = 1.95, 95% confidence interval [CI] 0.57-6.61, P = .28). The recurrence rate of LN was 8% and was not associated with graft loss. Acute rejection was the only variable associated with graft loss in patients with SLE (HR = 16.5, 95% CI 1.94-140.1, P = .01).

Conclusions

Renal graft survival in SLE patients did not differ from that reported for other causes of ESRD.  相似文献   

8.

Objective

To determine characteristics of the foot and ankle in people with systemic lupus erythematosus (SLE).

Methods

Medline, CINAHL, Sports-Discus, Scopus and Cochrane Library databases were searched up to January 2018. Studies reporting foot- and ankle-related outcomes in the following domains were included: vascular, neurological, musculoskeletal, cutaneous (skin and nail) or pain/function. The Quality Index tool was used to assess methodological quality. Where appropriate, odds ratio (OR) and mean difference meta-analyses were conducted for case-control studies; and pooled mean prevalence meta-analyses for studies assessing characteristics in SLE.

Results

Forty-nine studies were included with mean (range) quality scores of 75% (38–100%). Twenty-three studies assessed vascular characteristics, followed by musculoskeletal (n?=?16), neurological (n?=?11), cutaneous (n?=?5) and pain/function (n?=?4). Foot and ankle characteristics in people with SLE included impaired vascular supply, abnormal nerve function, musculoskeletal pathology, skin and nail pathology, and pain and functional disability. Twenty-four studies were included in meta-analyses. Pooled OR for abnormal ankle brachial index was 3.08 for SLE compared with controls. Pooled mean difference in brachial-ankle pulse-wave velocity between SLE and controls was significant (161.39?cm/s, P?=?0.004). Pooled prevalence was 0.54 for intermittent claudication, 0.50 for Raynaud's phenomenon, 0.28 for chilblains, 0.00 for gangrene, 0.30 for hallux valgus, 0.15 for onychomycosis, 0.76 for history of foot pain, and 0.36 for current foot pain.

Conclusion

People with SLE experience a wide range of foot and ankle manifestations. Published research highlights the impact of peripheral arterial disease, peripheral neuropathy, musculoskeletal deformity, skin and nail pathology and patient-reported foot pain and disability.  相似文献   

9.

Objectives

The study aimed at assessing the epidemiology and clinical features of systemic lupus erythematosus (SLE) in Reunion Island, South West of Indian Ocean.

Patients and methods

A retrospective study was conducted at the University Hospital of La Reunion (Saint-Denis) by charts review from 2004 to 2015. Patients with a SLE diagnosed over 15 years according to SLICC 2012 criteria were included. Incidence and prevalence were inferred from national health insurance database and population census results.

Results

In total, 123 patients met inclusion criteria: 116 were women (94%) and mean age at diagnosis was 34.7 ± 13.4 years. Ten percent of all patients had a least one parent with autoimmune disease, and 4% with lupus. The main manifestations were musculoskeletal (89%) and mucocutaneous with acute or subacute lesions (76%), alopecia (25%), ulcers (15%) and discoid lupus (11%). Lupus nephropathy occurred in 39%, serositis in 31% and neurological features in 15%. Antinuclear antibodies were positive in 99% (threshold >1/80), and associated to anti-DNA (70%), anti-SSA (47%), anti-RNP (42%), and anti-Sm (37%). APL syndrome was diagnosed in 15%. The average annual standardized incidence between 2010 and 2016 was 6.3 cases per 100,000 inhabitants (95% confidence interval [CI]: 5.6–6.9). The prevalence was 76 cases per 100,000 inhabitants in 2016 (95% CI: 70–82).

Conclusion

Lupus in the multi-ethnic population of Reunion Island is characterized by high incidence and high rates of articular and renal manifestations, as well as anti-ENA antibodies.  相似文献   

10.

Objective

Systemic lupus erythematosus (SLE), which is associated with increased stroke risk, is more prevalent and often more severe among Blacks, Asians, and Hispanics than Whites. We examined racial/ethnic variation in stroke rates and risks, overall and by hemorrhagic versus ischemic subtype, among SLE patients.

Methods

Within Medicaid (2000–2010), we identified patients aged 18–65 with SLE (≥ 3 ICD-9 710.0 codes, ≥ 30days apart) and ≥12 months of continuous enrollment. Subjects were followed from index date to first stroke event, death, disenrollment, or end of follow-up. Race/ethnicity-specific annual event rates were calculated for stroke overall and by subtypes (hemorrhagic vs. ischemic). We used Cox proportional hazard models to estimate hazard ratios (HR) of stroke by race/ethnicity, adjusting for comorbidities and the competing risk of death.

Results

Of 65,788 SLE patients, 93.1% were female. Racial/ethnic breakdown was 42% Black, 38% White, 16% Hispanic, 3% Asian, and 1% American Indian/Alaska Natives. Mean follow-up was 3.7 ± 3.0years. After multivariable adjustment, Blacks were at increased risk of overall stroke (HR 1.34 [95%CI 1.18–1.53), hemorrhagic stroke (HR 1.42 [1.00–2.01]), and ischemic stroke (HR 1.33 [1.15–1.52]) compared to Whites. Hispanics were at increased risk of overall stroke (HR 1.25 [1.06–1.47)] and hemorrhagic stroke (HR 1.79 [95% CI 1.22–2.61]), but not ischemic stroke, compared to Whites.

Conclusion

Among SLE patients enrolled in Medicaid, we observed elevated stroke risk (overall and by subtype) among Blacks and Hispanics compared to Whites, suggesting the importance of early recognition and screening for stroke risk factors among Blacks and Hispanics.  相似文献   

11.

Background

Cigarette smoking in patients with asthma leads to poor symptom control. As patients who are current smokers have been excluded from enrollment in many clinical trials on asthma, there are few reports on the treatment in current smokers with asthma. In this study, we aimed to assess how respiratory physicians manage asthma in current smokers in Japan.

Methods

Respiratory physicians in 16 Japanese hospitals answered a questionnaire on treatment for patients with asthma between December 2014 and February 2015. Medical records were reviewed for 1756 patients with asthma.

Results

The mean patient age was 61.1 years, and 62.9% of the patients were female. A total of 102 patients (5.8%) were current smokers, and 546 patients (31.1%) were former smokers. Long-acting muscarinic antagonists (LAMA) were prescribed more frequently for current smokers with asthma than for former smokers and never smokers with asthma (10.8% vs 4.6%, p = 0.01, 10.8% vs 3.8%, p < 0.01). In contrast, macrolides were prescribed more frequently for former smokers and never smokers with asthma than for current smokers with asthma (7.7% vs 1.0%, p = 0.01, 6.4% vs 1.0%, p = 0.03). Triple therapy, i.e., inhaled corticosteroids, long-acting beta agonists, and LAMA concomitantly, was prescribed for current smokers with asthma more frequently than for former smokers and never smokers with asthma (9.8% vs 4.0%, p = 0.01, 9.8% vs 3.3%, p < 0.01).

Conclusions

According to this survey, current smokers with asthma received more intensive therapy, including LAMA, than did former smokers with asthma.  相似文献   

12.

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   

13.

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   

14.

Objective

MRI-detected inflammation is considered of diagnostic value for rheumatoid arthritis (RA), but its evaluation involves a time-consuming scoring of 61 joint-level features. It is not clear, however, which of these features are specific for RA and whether evaluating a subset of specific features is sufficient to differentiate RA patients. This study aimed to identify a subset of RA-specific features in a case–control setting and validate them in a longitudinal cohort of arthralgia patients.

Methods

The difference in frequency of MRI-detected inflammation (bone marrow edema, synovitis, and tenosynovitis) between 199 RA patients and 193 controls was studied in 61 features across the wrist, metacarpophalangeal, and metatarsophalangeal joints. A subset of RA-specific features was obtained by applying a cutoff on the frequency difference while maximizing discriminative performance. For validation, this subset was used to predict arthritis development in 225 clinically suspect arthralgia (CSA) patients. Diagnostic performance was compared to a reference method that uses the complete set of 61 features normalized for inflammation levels in age-matched controls.

Results

Subset of 30 features, mainly (teno)synovitis, was obtained from the case–control setting. Validation in CSA patients yielded an area of 0.69 (95% CI: 0.59–0.78) under the ROC curve and a positive predictive value (PPV) of 31%, compared to 0.68 (95% CI: 0.60–0.77) and 29% PPV of the reference method with 61 features.

Conclusion

Subset of 30 MRI-detected inflammatory features, dominated by (teno)synovitis, offers a considerable reduction of scoring efforts without compromising accuracy for prediction of arthritis development in CSA patients.  相似文献   

15.

Aim of the work

To investigate the role of endothelial protein C receptor (EPCR) (membrane and soluble forms) as a biomarker of lupus nephritis (LN) in systemic lupus erythematosus (SLE) patients and to study its relation to the prognosis and response to treatment.

Patients and methods

The study included 30 SLE patients and 30 matched healthy volunteers as well as 10 renal biopsies from surgical nephrectomy as a control for membranous (mEPCR) examination. SLE disease activity index-2000 and damage index were assessed. Serum sEPCR was measured. Renal expression of mEPCR was analyzed. All patients were reassessed after 3?months.

Results

Patients were 26 females and 4 males with a mean age of 29.6?±?10.04?years and disease duration of 4.4?±?3.5?years. Their mean SLEDAI was 13.9?±?9.9 and damage index 1?±?1.5. Serum levels of sEPCR were significantly higher in patients with LN (19.9?±?5.7?ng/ml) than those without (8.95?±?4.2?ng/ml) and controls (5.3?±?2.6?ng/ml)(p?<?0.001). SLE patients with cutaneous vasculitis (n?=?9) had significantly higher sEPCR levels than those without (18.1?±?7.8 vs 10.2?±?5.2?ng/ml)(p?=?0.02). There was a significant correlation between sEPCR percentage of change and of SLEDAI-2k with and without LN (p?<?0.01 and p?<?0.05). A significant difference was observed in sEPCR according to the prognosis and treatment response after 3?months. mEPCR stained positively in glomeruli and tubules of LN patients with no relation to histopathological grading.

Conclusion

sEPCR plays a role in the pathogenesis, is related to a bad prognosis and poor response to treatment in LN. mEPCR was not related to LN grading.  相似文献   

16.

Aim of the work

To evaluate the level of anti-nucleosome (anti-NCS) antibodies in systemic lupus erythematosus (SLE) patients and study their association with disease activity and lupus nephritis.

Patients and methods

The study was carried out on 50 SLE patients; 47 females and 3 males and 30 matched controls. The SLE disease activity index (SLEDAI) was assessed. Plasma levels of anti-NCS antibodies were measured. Renal biopsy was done in those with lupus nephritis.

Results

The mean age of patients was 28.3?±?12.9?years; disease duration was 5.5?±?6.02?years and the SLEDAI.5?±?7.8. LN was present in 60% of the patients. Anti-nuclear antibodies (ANA) were positive in 48 (96%) and the anti-double stranded deoxyribonucleic acid positive in 38 (76% of the patients. The anti-NCS antibodies level was significantly increased in the patients (470.1?±?369.4?U/ml) compared to the control (36.43?±?14.58?U/ml) (p?=?0.0001). The anti-NCS was detected in 48/50(96%) patients at a cut-off of 65?U/ml. There was no significant difference in the anti-NCS antibodies levels between those with nephritis (456.53?±?362.59?U/ml) and those without (490.4?±?387.96?U/ml) (p?=?0.84). A significant correlation was present between the anti-NCS antibodies and photosensitivity (p?=?0.032), ESR (p?=?0.03), complement (C3) consumption (p?=?0.01) and urinary casts (p?=?0.04). A non-significant correlation was detected between level of anti-NCS and grades of nephritis (p?=?0.49) or SLEDAI (p?=?0.09).

Conclusion

Anti-NSC antibodies could be a useful addition to the laboratory tests that can help in the diagnosis of SLE. Also, it has a significant association to photosensitivity but no relation to with disease activity or renal involvement.  相似文献   

17.

Aims

To describe the methodology of the EPISER 2016 (study of the prevalence of rheumatic diseases in adult population in Spain), as well its strengths and limitations. The aim of this study is to estimate the prevalence of rheumatoid arthritis (RA), psoriatic arthritis (PsA), ankylosing spondylitis (AS), systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), osteoarthritis (knee, hip, hands, and cervical and lumbar spine), fibromyalgia, gout and clinical osteoporotic fracture.

Material and method

Population-based, multicenter, cross-sectional study, with the participation of 45 municipalities in the 17 Spanish autonomous communities. The reference population will consist of adults aged 20 years and over residing in Spain. A computer-assisted telephone interview (CATI) system will be used for data collection. Diagnostic suspicions and diagnoses received by the participants will be studied by rheumatologists in the referral hospitals in the selected municipalities. Statistical analysis: the prevalence of the rheumatic diseases will be calculated using estimators and their 95% confidence intervals. Weights will be calculated in each of the sampling stages in accordance with the probability of selection. The distribution of the population in Spain will be obtained from the Spanish Statistics Institute.

Conclusions

Sociodemographic and lifestyle changes over the last 16 years justify EPISER 2016. This study will provide current data about the prevalences of RA, AS, PsA, SLE, SS, osteoarthritis, fibromyalgia, gout and clinical osteoporotic fracture. The results will allow comparisons with studies from other countries and EPISER 2000.  相似文献   

18.

Objectives

To evaluate large-vessel (LV) abnormalities on serial imaging in patients with giant cell arteritis (GCA) and discern predictors of new lesions.

Methods

Clinical and imaging data from patients with GCA (including subjects diagnosed by LV imaging) enrolled in a prospective, multicenter, longitudinal study and/or a randomized clinical trial were included. New arterial lesions were defined as a lesion in a previously unaffected artery.

Results

The study included 187 patients with GCA, 146 (78%) female, mean (±SD) age at diagnosis 68.5 ± 8.5 years; 39% diagnosed by LV imaging. At least one arterial lesion was present in 123 (66%) on the first study. The most frequently affected arteries were subclavian (42%), axillary (32%), and thoracic aorta (20%). In 106 patients (57%) with serial imaging, new arterial lesions were noted in 41 patients (39%), all of whom had a baseline abnormality, over a mean (±SD) follow-up of 4.39 (2.22) years. New abnormalities were observed in 33% patients by year 2; clinical features of active disease were present at only 50% of these cases. There were no differences in age, sex, temporal artery biopsy positivity, or disease activity in patients with or without new lesions.

Conclusions

In this cohort of patients with GCA, LV abnormalities on first imaging were common. Development of new arterial lesions occurred in patients with arterial abnormalities at first imaging, often in the absence of symptoms of active disease. Arterial imaging should be considered in all patients with GCA at diagnosis and serial imaging at least in patients with baseline abnormalities.  相似文献   

19.

Objectives

Systemic sclerosis (SSc) causes functional and structural microcirculatory dysfunction, affecting also distal extremities. Optical Near-InfraRed Spectroscopy (NIRS) of blood HbO2 saturation (stO2) is able to evaluate O2 delivery/consumption balance in the explored tissue. The NIRS-sensitive camera non-invasively detects stO2 values in superficial tissues, automatically generating 2D-imaging maps in real time. We aimed at testing whether NIRS hand imaging may evaluate peripheral microcirculatory dysfunction and its spatial heterogeneity in SSc patients compared to controls.

Methods

Forty SSc patients (aged 55.1?±?15.6 years) and twenty-one healthy controls (aged 54.3?±?14.5years, p?=?0.89) were studied by palmar hand NIRS-2D imaging. A blood pressure cuff was applied to the forearm and 3 min ischemia was induced. Images were acquired at basal conditions and every 10 seconds during 3 minutes of ischemia and 5 minutes of reperfusion. Five regions of interest were positioned on each fingertip, from the second to the fifth finger and one on the thenar eminence.

Results

A significant difference was found between controls and SSc patients in basal stO2 (84.3?±?7.5?vs. 75.4?±?10.9%, p?<?0.001), minimum stO2 (65.2?±?8.0?vs. 53.4?±?10.1%, p?<?0.001) and time to maximum stO2 during hyperemia (63?±?38?vs. 85?±?49?s, p?<?0.05). Among clinical characteristics, anti-Scl70 antibody positivity, digital ulcers history and smoke exposure affected NIRS parameters, as well as sildenafil and statins therapy. Conversely, no significant differences were found in NIRS-2D values between different nailfold-videocapillaroscopy patterns.

Conclusion

NIRS-2D imaging is a simple, automated tool to non-invasively detect regional microcirculatory impairment in SSc, which seems to add significant functional information to the morphological picture of nailfold-videocapillaroscopy.  相似文献   

20.

Background

Spondyloarthritis (SpA), a chronic inflammatory, rheumatic disease, and hidradenitis suppurativa (HS), a chronic, debilitating, inflammatory skin disease, share several clinical and pathophysiological features, such as the association with inflammatory bowel disease and elevated cytokine levels IL-17 and TNF-α. Recently, SpA was reported to be more prevalent (2.3–28.2%) in patients with HS than in the general population. Conversely, the prevalence of HS in SpA is not exactly known.

Objective

To determine the prevalence of HS in patients with axial SpA, a subtype of SpA primarily of the axial skeleton. Secondly, to identify patient characteristics associated with the presence of HS in axial SpA.

Methods

In this cross-sectional study, a self-screening questionnaire based on validated diagnostic HS questions was sent to all participating axial SpA patients from the Groningen Leeuwarden Axial Spondyloarthritis (GLAS) cohort fulfilling the ASAS axial SpA criteria. Self-reported HS symptoms were confirmed by previous medical diagnosis or verification by phone using highly specific validated questions.

Results

In total, 75.6% (449/592) questionnaires were eligible for analyses. Included patients had a mean age of 50 ± 13 years, 64% was male, mean symptom duration was 23 ± 13 years, and 79% was HLA-B27 positive. HS diagnosis could be confirmed in 41 patients, resulting in an estimated prevalence of 9.1%. In comparison to patients without a positive history of HS, these patients were more often female (54% vs. 35%, P = 0.02), showed higher axial SpA disease activity (mean BASDAI 4.5 vs. 3.6, p = 0.01 and ASDASCRP 2.6 vs. 2.2, P = 0.003) and worse quality of life (QoL) (median ASQoL 9.0 vs. 4.0, P < 0.001). Also, a history of heel enthesitis and dactylitis was more prevalent (34% vs. 19%, P = 0.03 and 15% vs. 6%, P = 0.05, respectively). Multivariable analysis showed that a higher score on ASDAS was independently associated with HS (OR: 1.639, 95% CI: 1.176–2.284).

Conclusion

In our cohort of axial SpA patients, HS is more prevalent than in the general population (9.1% versus 0.053–4.1%). HS is associated with female gender, lower QoL, and especially higher axial SpA disease activity.  相似文献   

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