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1.
IgG4-related disease is a fibrous-inflammatory process related to immunomodulation. The most commonly affected organs are: the pancreas, bile duct, major salivary glands, lacrimal glands, retroperitoneum and lymphatic ducts.In recent decades, this disease has been recognised as a systemic disorder that includes many single organ disorders, previously unrelated and known as independent entities.The common characteristics shared by the different entities that make up the IgG4-related disease are: raised serum IgG4 levels, alterations in the imaging tests with neoplastic-like swelling of the affected organs, specific histopathological characteristics and in immunostaining, as well as good response to treatment with glucocorticoids.In this work, we will review this pathology with a special emphasis on the characteristics of autoimmune pancreatitis, sclerosing cholangitis related to IgG4 and the involvement of the retroperitoneum, mesenterium and the digestive tract.  相似文献   

2.

Objectives

The aim of the present study was to describe the ultrasound (US) findings in patients with rheumatoid arthritis (RA) in clinical remission, and to evaluate the ability of power Doppler (PD) US to reclassify disease activity in these patients.

Material and methods

We included consecutive patients with RA according to 2010 American College of Rheumatology/European League Against Rheumatism criteria, who were in clinical remission by the Disease Activity Score (DAS28<2.6). Twenty joints of both hands were assessed by US. PD signal was evaluated on a semi-quantitative scale from 0 to 3. Three different US-modified DAS28 (US-DAS28) were constructed, replacing the clinical swollen joint count by the PD US joint count using PD score ≥1, ≥2 or ≥3, respectively.

Results

Eighty-six patients were included. Twenty-three (26.7%) patients had at least one joint with abnormal US-positive PD signal. Thirteen percent of patients were reclassified to low disease activity by applying the US-DAS28 when joints were considered active with a PD signal ≥1; 12%, when a PD signal ≥2 was considered, and 2% of the patients were reclassified when a PD score of 3 was considered. No patients were reclassified to a level of moderate or high activity applying US-DAS28.

Conclusions

Although around a quarter of patients with RA in clinical remission showed PD US features indicating residual activity, only a small percentage were reclassified to a state of low activity and none to a level of moderate or high activity, applying the proposed US-DAS28.  相似文献   

3.

Introduction

There are few published studies on predictors of response to treatment with sofosbuvir and simeprevir in HCV patients.

Objective

The objective of the study was to analyse possible predictors of response to simeprevir (SMV) and sofosbuvir (SOF) in patients infected with hepatitis C genotypes 1 or 4.

Patients and methods

Prospective observational cohort study in 12 hospitals. The primary efficacy endpoint was SVR rate 12 weeks after end of treatment (SVR12).

Results

204 patients (62.3% male, mean age 55 years) were included: 186 (91.2%) genotype 1 (60.3% 1 b 25% 1 a) and 18 (8.8%) genotype 4. 132 (64.7%) cirrhotic (87.9% Child A), 33 (16.2%) F3, 31 (15.2%) F2, 8 (3.9%) F0-1. 80.8% MELD < 10. 93 (45.6%) naive. Ribavirin was added in 68 (33.3%). Mean baseline viral load 2,151,549 IU/ml (SD: 2,391,840). Treatment duration 12 weeks in 93.1%. 4 discontinued therapy: suicide, psychotic attack, hyperbilirubinaemia and liver cancer recurrence. 190 (93.1%) achieved SVR12. There were no differences in SVR12 depending on the genotype, treatment duration, ribavirin use, prior therapy, viral load (VL) or baseline platelets. In univariate analysis, undetectable VL at 4 weeks (p = 0.042), absence of cirrhosis (p = 0.021), baseline albumin ≥ 4 g/dl (p = 0.001) and MELD < 10 (p < 0.0001) were associated with higher SVR12. In multivariate analysis, only baseline MELD score <10 patients had higher SVR12 (p < 0.001).

Conclusions

The combination of simeprevir and sofosbuvir in patients infected with genotype 1 and 4 hepatitis C is highly effective. It is a safe therapy, especially in patients without ribavirin. This combination was more effective in patients with a MELD score below 10.  相似文献   

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Background

Measures of health related quality of life (HRQoL) in patients with α1-antitrypsin deficiency (AATD) can help to determine the impact of the disease and provide an important insight into the intervention outcomes. There is few data regarding this issue in the literature. The aim of this study is to assess the relationship between HRQoL and gender, functional parameters and history of hospitalizations in patients with AATD.

Methods

This is a cross-sectional study of 26 patients with severe AATD recruited in the pulmonology outpatient clinic at a tertiary care medical center. Social-demographic, clinical and functional parameters were recorded and HRQoL was assessed with the Portuguese version of the medical outcome study short form-36 (SF-36) self-administered questionnaire.

Results

Older patients, females and patients with at least one hospitalization in the previous year due to respiratory disease had statistical lower scores in some dimensions of the SF-36 questionnaire. Superior FEV1 and higher distance mark in the 6-min walking test distance influenced positively several dimensions of the questionnaire. Higher scores in the mMRC scale influenced negatively the HRQoL.

Conclusions

These data suggests that older and female patients with AATD have worse HRQoL. Hospitalizations and functional markers of respiratory disease progression influenced negatively the HRQoL, suggesting that the SF-36 questionnaire could be useful as an outcome for AATD patients with lung involvement.  相似文献   

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This positioning document, sponsored by the Asociación Española de Gastroenterología, the Sociedad Española de Endoscopia Digestiva and the Sociedad Española de Anatomía Patológica, aims to establish recommendations for the screening of gastric cancer (GC) in low incidence populations, such as the Spanish. To establish the quality of the evidence and the levels of recommendation, we used the methodology based on the GRADE system (Grading of Recommendations Assessment, Development and Evaluation). We obtained a consensus among experts using a Delphi method. The document evaluates screening in the general population, individuals with relatives with GC and subjects with GC precursor lesions (GCPL). The goal of the interventions should be to reduce GC related mortality. We recommend the use of the OLGIM classification and determine the intestinal metaplasia (IM) subtype in the evaluation of GCPL. We do not recommend to establish endoscopic mass screening for GC or Helicobacter pylori. However, the document strongly recommends to treat H. pylori if the infection is detected, and the investigation and treatment in individuals with a family history of GC or with GCPL. Instead, we recommend against the use of serological tests to detect GCPL. Endoscopic screening is suggested only in individuals that meet familial GC criteria. As for individuals with GCPL, endoscopic surveillance is only suggested in extensive IM associated with additional risk factors (incomplete IM and/or a family history of GC), after resection of dysplastic lesions or in patients with dysplasia without visible lesion after a high quality gastroscopy with chromoendoscopy.  相似文献   

8.

Introduction and objectives

Multidetector computed tomography (MDCT) has been demonstrated as a feasible alternative to invasive coronary angiography (ICA). However, contradictory results have been reported regarding the effect of coronary artery calcium score (CS) on the diagnostic accuracy of MDCT. Our aim was to assess the agreement of MDCT and ICA and to evaluate the influence of CS on this agreement.

Methods

We enrolled 266 consecutive patients who underwent evaluation with 64-slice MDCT and ICA. Standard CS software tools were used to calculate the Agatston score. Stenosis was qualitatively classified as mild, moderate, or severe by 1 blinded observer and the results were compared with those of ICA, which was used as the gold standard.

Results

The mean age of the patients was 65.4 ± 11.2 years, and 188 patients (70.3%) were men. A total of 484 segments with coronary stenosis ≥ mild were qualitatively evaluated and quantified with MDCT. Noninvasive measurements were concordant with ICA in 402 stenoses (83.05%; Kappa, 0.684), with no significant differences between vessels and with no statistically significant influence of CS on this agreement (OR, 0.93; 95%CI, 0.76-1.09; P = .21). Multidetector computed tomography had high sensitivity, specificity, positive predictive value, and negative predictive value on a per-segment, per-vessel, and per-patient basis.

Conclusions

Non-ICA using MDCT showed good agreement with ICA in the qualitative quantification coronary stenosis and CS had no significant impact on this agreement.Full English text available from: www.revespcardiol.org/en  相似文献   

9.

Introduction and objective

Atrophy and intestinal metaplasia are early phenotypic markers in gastric carcinogenesis. White light endoscopy does not allow direct biopsy of intestinal metaplasia due to a lack of contrast of the mucosa. Narrow-band imaging is known to enhance the visibility of intestinal metaplasia, to reduce sampling error, and to increase the diagnostic yield of endoscopy for intestinal metaplasia in Asian patients. The aim of our study was to validate the diagnostic performance of narrow-band imaging using 1.5 × electronic zoom endoscopy (with no high magnification) to diagnose intestinal metaplasia in Mexican patients.

Materials and methods

A retrospective cohort study was conducted on consecutive patients with dyspeptic symptoms at a private endoscopy center within the time frame of January 2015 to December 2016.

Results

A total of 338 patients (63 ± 8.4 years of age, 40% women) were enrolled. The prevalence of H. pylori infection was 10.9% and the incidence of intestinal metaplasia in the gastric antrum and corpus was 23.9 and 5.9%, respectively. Among the patients with intestinal metaplasia, 65.3% had the incomplete type, 42.7% had multifocal disease, and one third had extension to the gastric corpus. Two patients had low-grade dysplasia. The sensitivity of white light endoscopy was 71.2%, with a false negative rate of 9.9%. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of narrow-band imaging (with a positive light blue crest) were 85, 98, 86.8, 97.7, and 87.2%, respectively.

Conclusion

The prevalence of H. pylori infection and intestinal metaplasia in dyspeptic Mexican patients was not high. Through the assessment of the microsurface structure and light blue crest sign, non-optical zoom narrow-band imaging had high predictive values for detecting intestinal metaplasia in patients from a general Western setting.  相似文献   

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《Reumatología clinica》2022,18(1):49-55
ObjectiveTo analyse the efficacy and safety of intra-articular injection of expanded Mesenchymal Stromal Cells (MSCs) in knee osteoarthritis.MethodsSystematic Literature Review. A pre-defined search strategy was run in Medline, Embase and Cochrane Library until February 2018. Inclusion criteria: knee osteoarthritis (grades II-IV Kellgren-Lawrence); intra-articular injection of MSCs (without surgical co-treatments); Randomized Controlled Trials (RCTs) or Quasi-experimental Clinical Trials (QCTs) N≥10 and ≥6 months of follow-up were included. Evidence was assigned according to the Scottish Intercollegiate Guidelines Network (SIGN).ResultsThe search identified 252 articles. Nine proof-of-concept trials (3 RCTs, 6 QCTs) were included (N=169). Evidence showed clinical improvement in 60% of patients. Structural benefit was reported in half of patients. Clinical benefit was observed from the 3rd month and structural improvement from the 6th. All studies reported maximum clinical and structural benefit a year following the implant. This benefit was sustained for up to 24 months. Studies with doses ≥40×106 showed more consistent clinical and structural benefits than those with lower doses. No systemic adverse reactions were reported. The most common adverse effect was pain and/or inflammation in the puncture area (13-53%). The use of donor cells was as safe as autologous implants.ConclusionsIntra-articular implants of MSCs seem to be safe with no serious adverse effects. Low-quality evidence precludes conclusions regarding efficacy in this review. However, the clinical and structural benefits observed provide a rationale for using expanded MSCs implants in osteoarthritis patients. High-quality evidence trials are needed to further determine best protocols to maximize clinical and structural improvement.  相似文献   

12.

Objective

To analyze the changes in health-related quality of life (HRQoL) of patients with rheumatoid arthritis (RA) treated with biological therapies.

Method

Observational prospective study performed from October 2006 to May 2011. The inclusion criteria were adult patients, diagnosed with RA, treated for at least one year with anti-tumor necrosis factor therapy (infliximab or etanercept), who had not received other biological treatments previously. A total of 41 patients who completed the study undertook the specific and validated questionnaire QoL-RA Scale 3 times: E1 (September 2006-February 2007), E2 (April 2008-January 2009) and E3 (July 2010- May 2011). Data analysis was conducted using Epi-Info version 3.3 2004 for Windows® and Excel 2007; mean comparisons were evaluated by Student's t-test and the relationship between the 3 outcomes for each patient by lineal regression.

Results

Overall results show a downward trend which was not statistically significant: 7.09 (standard deviation [SD] = 1.15) in E1; 6.90 (SD = 1.60) in E2; and 6.52 (SD = 1.59) in E3. Items with higher scores were those related to psychosocial aspects (help from family, interaction with family and friends), whereas the physical dimension was valued more poorly (physical ability, arthritis pain, arthritis). Between E2 and E3 there was a significant increase in help from family (P=.0008), whereas level of tension (P=.0119) and mood (P=.0451) decreased significantly.

Conclusions

In all, HRQoL reported by patients is good and has remained unchanged after approximately 6 years of study. The stability of HRQoL is probably partly attributable to treatment.  相似文献   

13.

Objective

All commercial assays used to measure the presence of Hepatitis C virus (HCV) antibodies set cut-off points to categorise the results, but the problem of false positive results in screening hepatitis C sera is well known.The aim of this study was to evaluate the results obtained by two chemiluminescent assays in selected sera, and compare these results with the detection of viral RNA in the specimens studied.

Material and methods

Two hundred reactive sera (positive) were selected, although with a low signal to cut-off ratio (S/CO), were selected, using two chemiluminescent assays and were then subjected to genome amplification.

Results and discussion

Viral RNA could be only be detected in 8 (4%) of the selected specimens. Taking these results into account, we believe that the design of the current chemiluminescent assays do not provide sufficient specificity when they are used as the only tests for the diagnosis of hepatitis C.  相似文献   

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ObjectivesTo describe the clinical-biological characteristics of patients with scleroderma (SSc) and pulmonary artery hypertension (PAH).To establish the relationship between pulmonary functional tests (PFT), Doppler echocardiography (ECHO) and the severity of the PAH.Material and methodsRetrospective study of patients with scleroderma treated at a tertiary center. All participants received a protocol study, which included a complete analysis and additional tests: Doppler Echocardiography and pulmonary functional tests (PFT) with carbon monoxide diffusing capacity (DLCO).ResultsOverall, 331 patients were treated, including 68 (20.5%) with PAH. The limited subtype of Scleroderma was the most prevalent. The Person‘s correlation coefficient was used for the following variables: FVC-sPAP, FVC/DLCO-sPAP, DLCO-sPAP and TRV-sPAP, showed a significant moderate linear association in the relationship DLCO-sPAP and TRV-sPAP.29 deaths occurred, with 12 of them related to PAH. The median time between the PAH diagnosis and death was 1.8 years.ConclusionsThe decrease in DLCO and the increase in TRV are negative predictor factor of PAH which, at the same time, means a worsening prognosis for patients with Scleroderma.  相似文献   

16.
ObjectivesTo determine whether primary drug prevention of osteoporotic hip fracture is a useful measure in Catalonia and what would be their budgetary impact.Material and methodsWe performed a cost-utility social perspective study with a time horizon of 10 to 20 years. Univariate sensitivity analysis was performed. Doing nothing is compared with an intervention that includes screening for osteoporosis in women> 64 years and in those diagnosed and who received treatment with generic alendronate for 10 years. Decision trees are developed for groups of 65-69, 70-74 and 75-79. HF data is from 2009 hospital discharges .Costs are derived from fees paid by public insurance.ResultsIn 2009 there were 9262 HF. The direct cost was € 55 million (€ 5,943.4/patient). The total cost was € 227 million for 10 years. The intervention dominates in all age groups in a 20 year perspective. In any horizon and age group, the different scenario puts the value per QALY below or within the proposed values for Spain. The budgetary impact is estimated at € 8.9 million which increased by 31% the actual direct cost, and 0.5% of the public pharmacy budget. Considering the total costs and the prospect of 20 years, annual savings of 7.4 million € were seen.ConclusionsThe prevention of HF with alendronate in osteoporotic women > 64 years is cost-useful in the long term (20 years) with a low budgetary impact in the 75-79 year group.  相似文献   

17.
IntroductionInstrumental Activities of Daily Life (IADL) requires a higher level of personal autonomy and interaction with the environment than the basic activities of daily life (BADL). Few studies analyze the deficit on IADL. Early detection is important because an early action can delay the evolution of the dependency process.Aimto know the prevalence of functional deficit in IADL of hemodialysis patients, its relationship with the functional deficit in the BADL and its influence on the health related quality of life (HRQoL).MethodsCross-sectional study in 66 patients on HD. Different scales were used: Lawton and Brody (AIVD dependency), Delta (BADL dependency), and COOP-WONCA (HRQOL). Clinical data were collected (age, sex, CKD etiology, months in HD, marital status, employment situation).Results80.3% of the patients had some degree of dependence for the IADL (57.5% in moderate/severe degree) and 40.8% for the BADL (9% in moderate/severe degree). There was a correlation between dependence to the BADL and AIVD. Age and employment status influence the dependence on IADL and BADL. Patients with greater dependence on IADL had worse HRQoL (P < .000). The dimensions in which they showed greater differences were “Daily activities”, “Social activities” and “Social support”.ConclusionsHD patients are more dependent on IADL than on ADL and with a higher degree of severity and the level of dependence in both types of activities has important influence on HRQoL. Early detection of the occurrence of disability in the realization of IADL can be used to initiate intervention programs that help to prevent, reverse or delay the progression.  相似文献   

18.
《Reumatología clinica》2019,15(6):e111-e113
Idiopathic inflammatory myopathies are a heterogeneous group of potentially treatable myopathies. They are classified, on the basis of clinical and histopathological features, into four subtypes: dermatomyositis, polymyositis, necrotizing autoimmune myositis and inclusion-body myositis. Myositis-associated antibodies and myositis-specific autoantibodies are frequently found in patients with idiopathic inflammatory myopathies, and are useful in the diagnosis and classification. Anti-histidyl transfer RNA synthetase antibody is the most widely prevalent and is highly specific for polymyositis. Signal recognition particle antibody is also a specific autoantibody for polymyositis, but it is infrequent and rarely found in patients having other myositis-specific autoantibodies.We present a man with polymyositis who had both antibodies in serum, which is considered an extremely rare clinical situation. Here we analyze the clinical course and findings, and examine the effect of the coexistence and possible interaction on prognosis.  相似文献   

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