Sarcoidosis, Vasculitis, and Diffuse Lung Diseases最新文献

The aim of this study was to evaluate if CT findings in patients with pulmonary Post Covid syndrome represent residua after acute pneumonia or if SARS-CoV 2 induces a true ILD. Consecutive patients with status post acute Covid-19 pneumonia and persisting pulmonary symptoms were enrolled. Inclusion criteria were availability of at least one chest CT performed in the acute phase and at least one chest CT performed at least 80 days after symptom onset. In both acute and chronic phase CTs 14 CT features as well as distribution and extent of opacifications were independently determined by two chest radiologists. Evolution of every single CT lesion over time was registered intraindividually for every patient. Moreover, lung abnormalities were automatically segmented using a pre-trained nnU-Net model and volume as well as density of parenchymal lesions were plotted over the entire course of disease including all available CTs. 29 patients (median age 59 years, IQR 8, 22 men) were enrolled. Follow-up period was 80-242 days (mean 134). 152/157 (97 %) lesions in the chronic phase CTs represented residua of lung pathology in the acute phase. Subjective and objective evaluation of serial CTs showed that CT abnormalities were stable in location and continuously decreasing in extent and density. The results of our study support the hypothesis that CT abnormalities in the chronic phase after Covid-19 pneumonia represent residua in terms of prolonged healing of acute infection. We did not find any evidence for a Post Covid ILD.

Background: In Primary Health Care (PHC) many interstitial lung disease (ILD) cases may remain at diagnostic delay, due to their challenging presentation and the limited experience of general practitioners (GPs) in recognizing their early symptoms.

Objective: We have designed a feasibility study to investigate early ILD case-finding competency between PHC and tertiary care.

Methods: A cross-sectional prospective case-finding study was launched at two private health care centers of Heraklion, Crete, Greece, during nine months (2021-2022). After clinical assessment by GP, PHC attenders, who agreed to participate in the study, were referred to the Respiratory Medicine Department, University Hospital of Heraklion, Crete, underwent Lung Ultrasound (LUS) and those with an overall suspicion for ILDs underwent high resolution computed tomography (HRCT) scan. Descriptive statistics and chi-square tests were used. Multiple Poisson regression analysis was performed to explain positive LUS and HRCT decision with selected variables.

Results: One hundred and nine patients out of 183 were finally included (54.1% females; mean age 61, SD: 8.3 years). Thirty-five (32.1%) were current smokers. Overall, two out of ten cases were assessed to need HRCT due to a moderate or high suspicion (19.3%; 95%CI 12.7, 27.4). However, in those who had dyspnea in relation to counterparts, a significantly higher percentage of patients with LUS findings (57.9% vs. 34.0%, p=0.013) was found, as in those who had crackles (100.0% vs. 44.2%, p= 0.005). Detected possible ILD provisional labelling cases were 6, and most importantly, 5 of those cases were considered highly suspicious for further evaluation based on LUS findings.

Conclusions: This is a feasibility study exploring potentials by combining data of medical history, basic auscultation skills, as crackles detection, and inexpensive and radiation-free imaging technique, such as LUS. Cases of ILD labeling may be hidden within PHC, sometimes, much before any clinical manifestation.

Background: Sarcoidosis may seriously hamper patients' quality of life despite fairly good prognosis.

Objectives: To assess the relationship between the Big Five personality traits, chronotype and severity of fatigue symptoms, in the context of selected clinical variables and general mental health among patients with sarcoidosis.

Methods: The study group comprised 60 patients with a confirmed diagnosis of sarcoidosis. They were asked to share relevant clinical data and complete a set of questionnaires: Fatigue Asessment Scale (FAS), General Health Questionnaire (GHQ-28), the NEO Five Factor Inventory and Composite Scale of Morningness.

Results: In linear regression analysis, FAS score was predicted by female sex, active sarcoidosis status, Morning Affect and Conscientiousness. In principal component analysis, FAS score and all GHQ-28 subscale scores (somatic symptoms, anxiety/insomnia, social dysfunction and depressive symptoms) formed a single component explaining 60% of variance. The factor loading for each variable exceeded 0.6.

Conclusions: The psychological burden appeared to rise with the severity of the fatigue, regardless the inactive/active phase of sarcoidosis. The severity of fatigue may be linked to patient's poor morning affect. The profile of psychological burden presented by the patients may be associated with their personality and clinical presentation of sarcoidosis.

Background and aims: Autophagy is a highly regulated, complex intracellular recycling process that is vital to maintaining cellular homeostasis in response to diverse conditions and stressors. Despite the presence of robust regulatory pathways, the intricate and multi-step nature of autophagy creates opportunity for dysregulation. Errors in autophagy have been implicated in the development of a broad range of clinical pathologies including granulomatous disease. Specifically, activation of the mTORC1 pathway has been identified as a key negative regulator of autophagic flux, prompting the study of dysregulated mTORC1 signaling in the pathogenesis of sarcoidosis. Our review: We conducted a thorough search of the extant literature to identify the regulatory pathways of autophagy, and more specifically the implication of upregulated mTORC1 pathways in the pathogenesis of sarcoidosis. We review data showing spontaneous granuloma formation in animal models with upregulate mTORC1 signaling, human genetic studies showing mutation in autophagy genes in sarcoidosis patients, and clinical data showing that targeting autophagy regulatory molecules like mTORC1 may provide new therapeutic approaches for sarcoidosis.

Conclusions: Given the incomplete understanding of sarcoidosis pathogenesis and the toxicities of current treatments, a more complete understanding of sarcoidosis pathogenesis is crucial for the development of more effective and safer therapies. In this review, we propose a strong molecular pathway driving sarcoidosis pathogenesis at which autophagy is at the center. A more complete understanding of autophagy and its regulatory molecules, like mTORC1, may provide a window into new therapeutic approaches for sarcoidosis.

Background: Individuals with self-declared sarcoidosis are at increased risk of COVID-19 related morbidity and mortality for which vaccination can be lifesaving. Despite this, vaccine hesitancy remains a large barrier to global acceptance of vaccination against COVID-19. We aimed to identify individuals with sarcoidosis who had and had not been vaccinated against COVID-19 vaccine to 1) establish a safety profile of COVID-19 vaccination in those with sarcoidosis and 2) to elucidate factors that contribute to COVID-19 vaccine hesitancy.

Methods: A questionnaire inquiring about COVID-19 vaccination status, vaccination side effects, and willingness for future vaccination was distributed from December 2020 to May 2021 to individuals with sarcoidosis living in the US and European countries. Details regarding sarcoidosis manifestations and treatment were solicited. Vaccine attitudes were classified as pro or anti-COVID-19 vaccination for subgroup analysis.

Results: At the time of questionnaire administration, 42% of respondents had already received a COVID-19 vaccination, most of whom either denied side effects or reported a local reaction only. Those off sarcoidosis therapy were more likely to report systemic side effects. Among subjects who had not yet received a COVID-19 vaccine, 27% of individuals reported they would not receive one once available. Reasons against vaccination were overwhelmingly related to the lack of confidence in vaccine safety and/or efficacy and less related to concerns associated with convenience or complacency. Black individuals, women, and younger adults were more likely to decline vaccination.

Conclusions: Among individuals with sarcoidosis, COVID-19 vaccination is well-accepted and well-tolerated. Subjects on sarcoidosis therapy reported significantly less vaccination side effects, and thus the correlation between side effects, vaccine type, and vaccine efficacy requires further investigation. Strategies to improve vaccination should focus on improving knowledge and education regarding vaccine safety and efficacy, as well as targeting sources of misinformation, particularly in young, black, and female subpopulations.

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Background: The 6-minute walk test (6MWT) is a potential tool for assessing the severity of interstitial lung disease (ILD).

Objectives: To explore the relationship between 6MWT results and traditional measures including pulmonary function and chest computed tomography(CT) and to determine factors that might influence the 6-minute walk distance (6MWD).

Methods: Seventy-three patients with ILD were enrolled at Peking University First Hospital. All patients underwent 6MWT, pulmonary CT, and pulmonary function tests and their correlations were analyzed. Multivariate regression analysis was used to identify factors that might impact 6MWD.  Results: Thirty (41.4%) of the patients were female and the mean age was 66.1 ± 9.6 years. 6MWD was correlated with forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), total lung capacity (TLC), diffusing capacity for carbon monoxide (DLCO) and DLCO%pred. The decrease in oxygen saturation (SpO2) after the test was correlated with FEV1%pred, FVC%pred, TLC, TLC%pred, DLCO, DLCO%pred and the percentage of normal lung calculated by quantitative CT. The increase in Borg dyspnea scale was correlated with FEV1, DLCO and the percentage of normal lung. The backward multivariate model (F = 15.257, P < 0.001, adjusted R2 = 0.498) indicated that 6MWD was predicted by age, height, body weight, increase in heart rate, and DLCO.

Conclusions: The 6MWT results were closely correlated with pulmonary function and quantitative CT in patients with ILD. However, in addition to disease severity, 6MWD was also influenced by individual characteristics and the degree of patient effort, which should thus be considered by clinicians when interpreting 6WMT results.

Background and aim: The prognosis of sarcoidosis is challenging and largely depends on the persistence of disease activity and the degree of organ dysfunction. Various biomarkers have been evaluated for diagnosis, disease activity assessment, and prognosis. This study aimed to determine if the ratios of monocytes to high-density lipoprotein cholesterol (MHR), platelets to lymphocytes (PLR), neutrophils to lymphocytes (NLR), and lymphocytes to monocytes ratio (LMR) could be used as novel sarcoidosis activity markers.

Methods: In a case-control study, 54 patients with biopsy-confirmed sarcoidosis were divided into two groups; group 1: consisted of 27 patients with active sarcoidosis who were newly diagnosed and treatment-naive, and group 2: consisted of 27 patients with inactive sarcoidosis who had been on treatment for at least 6 months. All patients were subjected to a comprehensive history, physical examination, laboratory tests, chest imaging, spirometry, and screening for extrapulmonary organ involvement by means of electrocardiogram and eye examination.

Results: The mean age of the patients was 44 ± 11 years (79.6% were females & 20.4% were males). MHR, NLR, and LMR were significantly higher in patients with active sarcoidosis than in an inactive disease with a cut-off value of 8.6, a sensitivity of 81.5%, and a specificity of 70.4% (P-value < 0.001), a cut-off value of 1.95, sensitivity of 74% and specificity of 66.7% (P-value 0.007) and a cut-off value of < 4, a sensitivity of 81.5%, and a  specificity of 85.2% (P-value < 0.001), respectively. In contrast, PLR was not statistically significant between active and inactive sarcoidosis patients.

Conclusions: The lymphocytes monocytes ratio is a highly sensitive and specific biomarker that could be used to assess the disease activity in sarcoidosis patients.