Sarcoidosis Vasc Dif
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Sarcoidosis Vasc Dif · Mar 2016
Comparative StudyNuclear magnetic resonance spectroscopic analysis of salivary metabolome in sarcoidosis.
Sarcoidosis is a systemic granulomatous disease of unknown cause which has diverse clinical impacts, ranging from benign to very severe, which may therefore require systemic treatment. Only a few tools are currently available to monitor management in these patients. ⋯ This study showed that NMR metabolomics can discriminate saliva samples from sarcoidosis patients and controls. According to these preliminary results, saliva studies in sarcoidosis patients would be particularly useful to identify potentially relevant biomarkers. A study based on a larger number of patients at different stages of the disease or with treated patients is needed to assess the clinical relevance of NMR metabolomics in sarcoidosis.
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Sarcoidosis Vasc Dif · Oct 2014
Multicenter Study Clinical TrialAnalysis of lung function and survival in RECAP: An open-label extension study of pirfenidone in patients with idiopathic pulmonary fibrosis.
RECAP is an open-label extension study evaluating pirfenidone in patients with idiopathic pulmonary fibrosis (IPF) who completed the Phase 3 CAPACITY program. ⋯ FVC and survival outcomes in IPF patients newly treated with pirfenidone in RECAP were similar to those in the CAPACITY pirfenidone group. These data provide further evidence to support the use of pirfenidone in patients with IPF.
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Sarcoidosis Vasc Dif · Sep 2013
Long-term management of IPF with pirfenidone - a clinical case study with 5 years follow-up.
Idiopathic pulmonary fibrosis (IPF) is a progressively fibrotic interstitial lung disease that is associated with a median survival of 2-5 years from initial diagnosis. To date, the search for an effective treatment has involved numerous clinical trials of investigational agents but without significant success. Nevertheless, research over the past 10 years has provided us with a wealth of information on its histopathology, diagnostic work-up, and a greater understanding of its pathophysiology. ⋯ Emerging clinical data from recently published and ongoing trials investigating new potential pharmacological agents should be considered in the routine clinical management of these patients. Based upon encouraging results from randomised-controlled trials showing a positive effect in slowing decline in pulmonary function and reducing disease progression, pirfenidone was approved in 2011 as the first treatment in patients with IPF. This case study describes the clinical course of a patient enrolled into the Phase III and open-label extension studies of pirfenidone.
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Sarcoidosis Vasc Dif · Mar 2013
ReviewSurgical lung biopsy for the diagnosis of interstitial lung disease: a review of the literature and recommendations for optimizing safety and efficacy.
Making an accurate diagnosis of a specific type of interstitial lung disease (ILD) requires a structured and comprehensive approach that includes a complete patient history, careful physical examination, appropriate laboratory testing, and thoracic imaging. If invasive procedures are required, bronchoscopy with bronchoalveolar lavage (BAL) and/or endoscopic lung biopsy (ELB) can frequently establish a confident diagnosis. However, surgical lung biopsy (SLB) may be required to make a confident diagnosis. ⋯ The overall 30-day mortality for open lung biopsy (OLB) was 4.3% versus 2.1% for video-assisted thorascopic surgery (VATS) biopsy, and non-lethal complications appeared to occur more frequently with OLB (18.1%) vs. VATS (9.6%) procedures. In addition to presenting the results of our comprehensive literature review on SLB for the diagnosis of ILD, we suggest an approach that minimizes risks to patients and optimizes the diagnostic utility of SLB when SLB must be performed to obtain a confident ILD diagnosis.
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Sarcoidosis Vasc Dif · Mar 2013
Comparative StudyThe internal consistency of PRO fatigue instruments in sarcoidosis: superiority of the PFI over the FAS.
The Fatigue Assessment Scale (FAS) is a 10-item patient reported outcome (PRO) questionnaire that is used to measure fatigue in sarcoidosis. After several months of use, we began to question the reliability of the FAS in our clinic population. Therefore, we administered an additional fatigue PRO, the Patient Reported Outcomes Measurement Information Systems (PROMIS) Fatigue Instrument (PFI). Our hypothesis was that the internal consistency/reliability (Cronbach's alpha) of the PFI would be superior to the FAS in sarcoidosis patients because two of the ten FAS items (items #4 and #10) required reverse scoring (these items were scaled in the opposite direction to the other 8 items). ⋯ We found that the PFI had "excellent" consistency in our sarcoidosis clinic. The FAS did not demonstrate the same degree of internal consistency. The Cronbach's estimate of the FAS with items #4 and #10 removed was vastly superior to the FAS. These data support our contention that FAS items #4 and #10 detract from the internal consistency of this PRO. They also suggest that the PFI is superior to the FAS in terms of reliability.