Search results for "Scleroderma"
showing 10 items of 70 documents
Serum surfactant protein D and exhaled nitric oxide as biomarkers of early lung damage in systemic sclerosis
2017
Background Interstitial lung disease (ILD) complicates the course of systemic sclerosis (SSc), representing the main cause of death in these patients. The identification of parameters that can predict the early onset and progression of ILD in SSc represents an unmet need in clinical practice. The study was designed to explore whether the surfactant proteins (SP) A and D may be used as noninvasive tools for the early identification of ILD in SSc. Alveolar exhaled nitric oxide (NO) was investigated as a surrogate marker of distal inflammation. Methods Unselected consecutive subjects newly diagnosed with scleroderma and subjects free of respiratory and systemic diseases were recruited. All pat…
Esophageal dysfunction and its pathogenesis in progressive systemic sclerosis
1978
In 25 patients with progressive systemic sclerosis esophageal involvement was studied prospectively by analysing subjective symptoms and radiological and manometric criteria. In all patients abnormal motility could be demonstrated by X-ray and/or manometry: radiologically in 18 (72%), manometrically in 22 (88%) cases. Dysphagia was present only in 11 patients (44%). Six patients (24%) had no subjective symptoms in spite of severe objective esophageal abnormalities. Therefore, the absence of esophageal symptoms does not exclude advanced affliction of the esophagus. To determine the pathogenesis of esophageal dysfunction the effects of 3.5 microgram/kg carbachol and of 0.6 microgram/kg pentag…
Evaluation of esophagogastric junction relaxation by 4-second Integrated Relaxation Pressure in achalasia using High Resolution Manometry with water-…
2014
Background Relaxation of the esophagogastric junction (EGJ) is now evaluated calculating 4-second integrated relaxation pressure (4-s IRP) by high resolution manometry (HREPT). Solid-state catheters have been used to define abnormal values. Our aim was to evaluate 4-s IRP in esophageal achalasia using HREPT with perfused catheters. Methods From June 2009 to June 2013, 936 HREPT studies have been performed in our unit. Of these, 194 patients having treated achalasia were excluded. Control group was constituted by 695 patients without achalasia, and 47 patients with untreated achalasia constituted the study group. HREPT was performed with water-perfused catheters. To establish the cut-off val…
Efficacy and Safety of Selexipag in Adults With Raynaud's Phenomenon Secondary to Systemic Sclerosis:A Randomized, Placebo-Controlled, Phase II Study
2017
Objective To determine the effect of selexipag, an oral, selective IP prostacyclin receptor agonist, on the frequency of attacks of Raynaud's phenomenon (RP) in patients with systemic sclerosis (SSc). Methods Patients with SSc-related RP were randomized 1:1 to placebo (n = 38) or selexipag (n = 36) in individualized doses (maximum of 1,600 μg twice daily) during a 3-week titration period. The primary end point was the weekly average number of RP attacks during the study maintenance period, analyzed using a Bayesian approach with a negative binomial model adjusted for baseline number of RP attacks. Other outcome measures included Raynaud's Condition Score (RCS), RP attack duration, and treat…
A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: A EUSTAR exploratory study.
2015
Objective: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. Methods: Data collected between June 2004 and April 2013 were examined with focus on capillaroscopy. In this retrospective exploratory study, series of patients with juvenile-onset SSc were matched with series of adult-onset SSc having the same gender and autoantibody profile. Results: 30 of 123 patients with juvenile-onset and 2108 of 7133 with adult-onset SSc had data on capillaroscopy. Juvenile-onset SSc showed scleroderma pattern more frequently than adult-onset SSc (93.3% and 88%). The…
Iloprost treatment in patients with Raynaud's phenomenon secondary to systemic sclerosis and the quality of life: a new therapeutic protocol
2006
Objectives: to evaluate the clinical efficacy and the effects on the quality of life of Iloprost, a prostacyclin analogue, used, according to a new protocol, in patients with Raynaud’s phenomenon secondary to Systemic Sclerosis. Methods: in this randomized study we treated 30 patients with Iloprost given by intravenous infusion, at progressively increasing doses (starting from 0.5 ng/Kg/min up 2 ng/Kg/min) over a period of 6 hours a day for ten days in two consecutive weeks, with repeated cycles at regular intervals of three months for 18 months. The results were compared with those obtained in 30 other patients, who had received the same drug but with different posologic schemes. Results: …
Predictive role of capillaroscopic skin ulcer risk index in systemic sclerosis: A multicentre validation study
2012
IntroductionThe early detection of systemic sclerosis (SSc) patients at high risk of developing digital ulcers could allow preventive treatment, with a reduction of morbidity and social costs. In 2009, a quantitative score, the capillaroscopic skin ulcer risk index (CSURI), calculated according to the formula ‘D×M/N2’, was proposed, which was highly predictive of the appearance of scleroderma digital ulcers within 3 months of capillaroscopic evaluation.ObjectivesThis multicentre study aims to validate the predictive value and reproducibility of CSURI in a large population of SSc patients.MethodsCSURI was analysed in 229 unselected SSc patients by nailfold videocapillaroscopy (NVC). All pati…
Effects of a nonsurgical exercise program on the decreased mouth opening in patients with systemic scleroderma.
2004
The decreased mouth opening (microstomia) represents a frequent finding in patients with systemic scleroderma (SSD), but little information is available about the efficacy of nonsurgical management of this condition. The aim of this study is to assess the effects of a nonsurgical exercise program on the decreased mouth opening in a group of 10 SSD patients with severe microstomia (maximal mouth opening 0.1). At the end of the 18-week period, all patients commented that eating, speaking and oral hygiene measures were easier. The edentulous subjects also experienced less difficulty inserting their own dentures. These findings suggest that regular application of the proposed exercise program m…
Is abdominal compression a useful stimulation test for analysis of lower esophageal sphincter function?
1984
The change in pressure of competent and incompetent lower esophageal sphincter (LES) due to abdominal compression is still a controversial subject. Therefore, we studied the effect of sustained (SAC) and intermittent (IAC) abdominal compression on lower esophageal sphincter pressure (LESP) in normals (N), patients with hiatus hernia (HH), and patients with scleroderma (S). When resting lower esophageal sphincter pressure exceeded 15 mm Hg, response to SAC and IAC was similar in patients with HH and in normals. On the other hand when basal LESP was below 15 mm Hg, stimulated sphincter pressure during IAC was significantly lower than during SAC. Values recorded during SAC were also falsely hi…
Aortic root dilation in associated with the reduction in capillary density observed at nailfold capillaroscopy in SSc patients
2020
Systemic sclerosis (SSc) is an autoimmune disease characterized by endothelial dysfunction and fibroblasts activation. Microvascular disease may be easily observed by means of nailfold capillaroscopy. Recent evidences emphasized also the involvement of large-medium arteries in SSc, mainly in terms of increased stiffness of the vessel wall. The study aims to measure aortic root diameter in a cohort of SSc patients and to correlate echocardiographic findings with the capillaroscopic pictures. We analyzed the clinical records of 125 consecutive SSc patients (M/F 14/111, mean age 55 ± 12.7 years, median disease duration 11 years) referring in 3 second-level rheumatology centers. All subjects un…