Sarcoidosis, Vasculitis, and Diffuse Lung Diseases最新文献

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Introduction:   activity tracker device usage can help analyze the impact of disease state and therapy on patients in clinical practice.  factors such as age, race, and gender may contribute to difficulties with using such technology.  Objective: we evaluated the effect of age, race, and gender on the usability of the Fitbit OneTM activity tracking device in sarcoidosis patients and the impact of device on sarcoidosis patients' activity.

Method: patients participated in a six-month prospective study where were asked to wear a Fitbit OneTM activity tracker daily. device usage education was provided at study enrollment.  weekly data download and submission reports to participating centers was required. patients were asked to complete a post-study questionnaire reviewing the motivation of the activity tracker on daily activity.

Results: at three centers, 91 patients completed all study visits and the post study questionnaire with a mean age of 55 and 75% were female and 34% african american. accurate downloads occurred >75% of the time, regardless of age, race, or sex. results of the post-study questionnaire did not show a correlation between the likelihood of wearing the device and motivation to increase activity.

Conclusion: using an activity tracking device to evaluate and/or correlated with quality of life (QOL) instruments may prove beneficial for gathering more data on patients.  age, race, and gender did not contribute to differences in usability among sarcoidosis patients.

Sarcoidosis is a multisystem inflammatory disease manifesting as noncaseating epithelioid cell granulomas. 25 to 30% of individuals with systemic sarcoidosis show variable cutaneous manifestations. A 59-year-old female was seen with reticular purplish-red nodules and plaques on the legs for three months. A skin biopsy of the livedo area revealed non-caseating epithelioid cell granulomas surrounding blood vessels in the dermis. She was diagnosed with sarcoidosis livedo, and cutaneous lesions subsided with oral prednisone. Sarcoidosis livedo (SL) assumes a uncommon livedo reticularis-like presentation. This is the first Chinese patient with SL, and more patients are needed to unveil the unique characters of SL.

Objective and aim:  Sarcoidosis, a multisystemic granulomatous disease, generally results in a lower quality of life (QoL) because of its unexpected course and diverse clinical symptoms. The Sarcoidosis Health Questionnaire (SHQ) evaluates the QoL for people with sarcoidosis in terms of their health. This study set out to validate the SHQ in a group of Turkish sarcoidosis patients.

Methods:  The study included a total of 146 adult sarcoidosis patients (63 male and 83 female; mean age, 44±3.6 years; range, 27-63 years) between May 2019 and September 2021. Preparation, forward translation, reconciliation, back translation/back translation review, harmonization, finalization, and proofreading comprised the steps of the testing procedure for translation and cultural adaptation. The participants filled out three questionnaires, including the SHQ, 36-Item Short Form (SF-36) Health Survey, and King's Sarcoidosis Questionnaire (KSQ), and underwent pulmonary function tests (PFTs).

Results:  Of the patients, 95% had lung involvement, with a mean number of 1.3 organs involved. Each SHQ component displayed a moderate to high internal consistency, ranging from 0.806 to 0.844. The whole scale's Cronbach's alpha value was 0.781. The SHQ total score significantly correlated with physical component summary (p< 0.001, r=0.360) and mental component summary (p<0.001, r=0.352) scores of SF-36, and the general health status (p< 0.001, r=0.478), medication component (p<0.001, r=0.456), and eye component scores of KSQ (p<0.001, r=0.545). When patients were divided into groups based on organ involvement (p=0.01), oral steroid medication (p<0.001), and types of symptoms (P=0.021), there were significant differences in the total SHQ scores.

Conclusion:  The Turkish version of SHQ can be a valid and accurate instrument for assessing the health of sarcoidosis patients in Turkey. When combined with normal physiological, radiological, and serological examinations, SHQ can assess the QoL of sarcoidosis patients and give useful new information.

Efzofitimod is a first-in-class biologic based on a naturally occurring splice variant of histidyl-tRNA synthetase (HARS) that downregulates immune responses via selective modulation of neuropilin-2 (NRP2). Preclinical data found high expression of NRP2 in sarcoidosis granulomas. Treatment with efzofitimod reduced the granulomatous inflammation induced by P. acnes in an animal model of sarcoidosis. A dose escalating trial of efzofitimod in sarcoidosis with chronic symptomatic pulmonary disease found that treatment with efzofitimod was associated with improved quality of life with a trend towards reduced glucocorticoid use and stable to improved pulmonary function. These studies have led to a large Phase 3 trial of efzofitimod in symptomatic pulmonary sarcoidosis.

Background and aim Prednisone is used as first-line therapy for patients with pulmonary sarcoidosis. There is however no clear association between prednisone dose and FVC change in patients with pulmonary sarcoidosis. In order to improve our standard of care we introduced a more conservative prednisone protocol. Methods This study is a single centre observational study, applying value-based healthcare (VBHC) and quality improvement (QI) principles. Prednisone intake was reduced from a starting dose of 40 mg to a starting dose of 20 mg. Primary outcomes evaluated were FVC, FEV1 and DLCO % predicted. The secondary outcome measure was BMI. Results 369 patients were included in the old-cohort and 215 in the new-cohort. In the old-cohort, 182 (49.0%) of the patients were treated with prednisone. In total, 114 patients (62.6%) were treated according to the old protocol with a mean initial prednisone dose of 32.1 ±14.2 mg. In the new-cohort, 93 patients (45.0%) were treated with prednisone of which 53 patients (57.0%) received prednisone according to the new protocol. The mean initial prednisone dose in the new-cohort was 21.4 ±9.8 mg. Changes in FVC and FEV1 % predicted did not vary. Change in % predicted DLCO was 2.4 ±9.3 for the old-cohort and -1.3 ±11.4 for the new-cohort (p = 0.01). No statistically significant changes in BMI were observed. Conclusions Our results indicate that in more than half of the patients the new protocol was followed. Data support the observation that a more conservative prednisone regimen might be equally effective, looking at changes in pulmonary function and BMI.

Background and aim: To outline the observations of studies evaluating the prominence of Neutrophil to Lymphocyte Ratio (NLR) in sarcoidosis.

Methods: The search was performed on PubMed, Scopus, and web of science up until November 21, 2021. Eventually, a number of 17 papers were incorporated into this review.

Results: The results of this analysis showed no significant difference of NLR values between sarcoidosis patients and tuberculosis patients (SMD=-0.36, 95% CI= -0.92-0.21). The results showed high heterogeneity (I2=90.83%, p<0.001). So, we used random-effects model. However, NLR can be utilized to identify the radiological severity and staging of pulmonary sarcoidosis due to statistically significant variations. An elevation in NLR values may assist both sarcoidosis diagnosis and lung parenchyma involvement. Also, extra-pulmonary involvement was just more probable to be found in individuals diagnosed with sarcoidosis inhibiting high rates of NLR. High NLR levels were found to be associated with an accelerated rate of progression, revealing that NLR might be used to detect Pulmonary Hypertension (PH) as a complication of sarcoidosis.

Conclusions: In the visualizations of the disease, NLR was revealed to be a beneficial and straightforward fundamental laboratory biomarker connected to disease severity and requirement for therapy.

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with elevated mortality. Delay in diagnosis lead to worse outcomes. Guidelines developed at academic medical centers are difficult to replicate in the community.

Objectives: Our primary objective was to ascertain consistency with the 2011 IPF guidelines. Our secondary objective was to conduct an interdisciplinary review to ascertain whether the evidence supported the original diagnosis of IPF or not.

Methods: We asked permission from pulmonologists to review records of patients diagnosed with IPF after 2011. We collected physician demographics and training data; patient demographics, clinical and diagnostic/management data. The clinical data and available images were reviewed by the interdisciplinary review panel.

Results: 26 practicing pulmonologists located in the Southeast of the United States consented to participate. Mean age was 48, 70% were male and all had current certification. We reviewed data from 96 patients. The mean age was 71.4 and most were male. Only 23% had the recommended screening for a connective tissue disease and 42.6% were screened for exercise-induced hypoxemia. Among patients with available images for review (n=66), only 50% had a high-resolution CT scan. 22% of patients underwent a surgical biopsy and in only 33% of the cases three lobes were sampled. No patient had documentation that a multidisciplinary discussion occurred. In 20% of the cases with available images, the evidence supported an alternative diagnosis. 56% of eligible candidates were ever started on anti-fibrotics.

Conclusions: Our findings suggest that consistency with the IPF guidelines is low in non-academic settings.

Background and aim: Serum Soluble Interleukin-2 Receptor (sIL-2R) levels are used clinically as a disease activity marker for systemic sarcoidosis. Studies have investigated the diagnostic role of serum soluble interleukin-2 receptor (sIL-2R) level for sarcoidosis relative to biopsy. We performed a systematic review and meta-analysis of studies evaluating the diagnostic utility of sIL-2R.

Methods: We carried out an electronic search in Medline, Embase, Google Scholar, and Cochrane databases using keyword and Medical Subject Heading (MeSH) terms: sarcoidosis and sIL-2R. Studies evaluating the sIL-2R levels as a diagnostic tool in clinically diagnosed or biopsy-proven sarcoidosis patients compared to control groups with non-sarcoidosis patients were included. Forest plots were constructed using a random effect model depicting pooled sensitivity, specificity, positive and negative predictive values, and diagnostic accuracy.

Results: We selected ten studies comprising 1477 patients, with 592 in the sarcoidosis group and 885 in the non- sarcoidosis group. Pooled sensitivity and specificity of sIL-2R levels were 0.88 (95% CI: 0.75-0.95) and 0.87 (95% CI 0.73-0.94) respectively. Pooled negative predictive value and positive predictive value were 0.91 (95% CI 0.77-0.97) and 0.85 (95% CI 0.59-0.96) respectively with diagnostic accuracy of 0.86 (95% CI 0.71- 0.93).

Conclusion: In addition to its utility as a marker of sarcoidosis disease activity, sIL-2R has high diagnostic accuracy. Despite the limitations of the heterogenous sarcoidosis population and different sIL-2R cutoffs, our results suggest that sIL-2R is an important biomarker that can be used to confirm sarcoidosis diagnosis in unconfirmed or unclear cases.

Background   Combined pulmonary fibrosis and emphysema (CPFE) has been recognised as a phenotype of pulmonary fibrosis. We aimed to compare serum surfactant protein-A (SP-A), surfactant protein-D (SP-D) and Krebs von den Lungen-6 (KL-6) levels, functional parameters, in CPFE and  IPF (idiopathic pulmonary fibrosis) patients. Methods Patients diagnosed with 'CPFE' and 'IPF' were consecutively included in 6 months as two groups. The patients with connective tissue diseases are excluded. Results           In this study, 47 patients (41 males, 6 females) with CPFE (n = 21) and IPF (n = 26) with a mean age of 70.12 ± 8.75 were evaluated. CPFE patients were older, had more intense smoking history, had lower DLCO/VA, lower FVC, and worse six-minute walking distance than the IPF group (p=0.005, p=0.027, p=0.02, p<0.001, p=0.001, respectively). Serum KL-6 levels were higher in CPFE group compared to IPF group [264.70 U/ml (228.90-786) vs 233.60 (101.8-425.4), p<0.001]. Serum KL-6 levels of 245.4 U/ml and higher have 81% sensitivity and 73% specificity for the discrimination of CPFE from IPF. Conclusions   Our study has shown that serum KL-6 level is a promising biomarker to differentiate CPFE from IPF. In CPFE cases respiratory and functional parameters are worse than those of pure fibrosis cases.