Pulmonary Sarcoidosis

2017 ◽  
Vol 38 (04) ◽  
pp. 437-449 ◽  
Author(s):  
Martina Bonifazi ◽  
Stefano Gasparini ◽  
Veronica Alfieri ◽  
Elisabetta Renzoni
Keyword(s):  
Tissue Characterization ◽  
Wide Spectrum ◽  
Diagnostic Techniques ◽  
Pulmonary Sarcoidosis ◽  
Unknown Etiology ◽  
Local Resources ◽  
Clinical Scenarios ◽  
Risk Of Progression ◽  
Prognostic Implications ◽  
Granulomatous Disorder

AbstractSarcoidosis is a systemic granulomatous disorder of unknown etiology, with a predilection for the respiratory system, involved in more than 90% of cases, with considerable variability in terms of patterns and prognostic implications. A protean interplay between genetic susceptibility and antigen exposure in selected environmental contexts is likely to define not only the risk for disease but also the different clinical phenotypes. Advances in imaging and functional tests over the past decades have increasingly broadened the awareness of the wide spectrum of features potentially occurring in pulmonary sarcoidosis, while the evolution of diagnostic techniques in interventional pulmonology has increased the possibility of obtaining tissue characterization more easily, by means of ultrasound-guided tools and cryotechnology. However, histological proof is not always needed, as, in selected scenarios with compatible clinical and imaging picture, a confirmatory biopsy is perceived as redundant. The diagnostic workup should be tailored on a case-by-case basis, according to an integrated assessment of clinical features, local resources, and expertise availability. Owing to the heterogeneity of possible clinical scenarios and the paucity of evidence-based data on treatments, a standardized clinical management has yet to be established, and general approach usually reflects expert opinion from accumulated clinical data. Key elements of decision-making process are to assess the baseline severity of disease, to stratify the risk of progression, and to define the goals of treatment.

scholarly journals T Lymphocyte Maturation Profile in the EBUS-TBNA Lymph Node Depending on the DLCO Parameter in Patients with Pulmonary Sarcoidosis

Cells ◽  
2021 ◽  
Vol 10 (12) ◽  
pp. 3404
Author(s):  
Elżbieta Rutkowska ◽  
Iwona Kwiecień ◽  
Joanna Bednarek ◽  
Rafał Sokołowski ◽  
Agata Raniszewska ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
T Cells ◽  
T Lymphocytes ◽  
Cd8 T Cells ◽  
Pulmonary Sarcoidosis ◽  
Unknown Etiology ◽  
Median Proportion ◽  
Normal Diffusion ◽  
Granulomatous Disorder ◽  
Inflammatory Changes

Sarcoidosis (SA) is a systemic granulomatous disorder of unknown etiology with lung and mediastinal lymph nodes (LNs) as the main location. T lymphocytes play important role in the formation of granulomas in SA, but still little is known about the role of maturation profile in the development of inflammatory changes. The aim of this study was to determine the CD4+ and CD8+ T cells maturation profile in LNs and in peripheral blood (PB) and its relation to disease severity expressed by diffusing capacity of the lung for carbon monoxide (DLCO). 29 patients with newly pulmonary SA were studied. Flow cytometry was used for cells evaluation in EBUS-TBNA samples. We observed lower median proportion of T lymphocytes, CD4+ T and CD8+ T cells in patients with DLCO< 80% than in patients with normal diffusion (DLCO > 80%). Patients with DLCO < 80% had lower median proportion of effector and higher median proportion of central memory CD4+ and CD8+ T cells than patients with DLCO > 80%. We reported for the first time that LNs CD4+ and CD8+ T cells maturation differs depending on the DLCO value in sarcoidosis. Lymphocytes profiles in LNs may reflect the immune status of patients with SA and can be analysed by flow cytometry of EBUS-TBNA samples.

scholarly journals Imaging Diagnosis of Right Ventricle Involvement in Chagas Cardiomyopathy

2017 ◽  
Vol 2017 ◽  
pp. 1-14 ◽  
Author(s):  
Minna M. D. Romano ◽  
Henrique T. Moreira ◽  
André Schmidt ◽  
Benedito Carlos Maciel ◽  
José Antônio Marin-Neto
Keyword(s):  
Right Ventricle ◽  
Tissue Characterization ◽  
Low Cost ◽  
Diagnostic Techniques ◽  
Diagnostic Tools ◽  
Deformation Analysis ◽  
Geometrical Shape ◽  
Gold Standard Method ◽  
Chagas Cardiomyopathy ◽  
Health And Disease

Right ventricle (RV) is considered a neglected chamber in cardiology and knowledge about its role in cardiac function was mostly focused on ventricular interdependence. However, progress on the understanding of myocardium diseases primarily involving the RV led to a better comprehension of its role in health and disease. In Chagas disease (CD), there is direct evidence from both basic and clinical research of profound structural RV abnormalities. However, clinical detection of these abnormalities is hindered by technical limitations of imaging diagnostic tools. Echocardiography has been a widespread and low-cost option for the study of patients with CD but, when applied to the RV assessment, faces difficulties such as the absence of a geometrical shape to represent this cavity. More recently, the technique has evolved to a focused guided RV imaging and myocardial deformation analysis. Also, cardiac magnetic resonance (CMR) has been introduced as a gold standard method to evaluate RV cavity volumes. CMR advantages include precise quantitative analyses of both LV and RV volumes and its ability to perform myocardium tissue characterization to identify areas of scar and edema. Evolution of these cardiac diagnostic techniques opened a new path to explore the pathophysiology of RV dysfunction in CD.

Potential use of biomarkers for the clinical evaluation of sarcoidosis

2021 ◽  
pp. jim-2020-001659
Author(s):  
Amir Mousapasandi ◽  
Cristan Herbert ◽  
Paul Thomas
Keyword(s):  
Clinical Evaluation ◽  
Clinical Presentation ◽  
Current Knowledge ◽  
Epithelioid Cell ◽  
Pulmonary Sarcoidosis ◽  
Biological Molecules ◽  
Unknown Etiology ◽  
Chronic Antigenic Stimulation ◽  
Host Genetic ◽  
Potential Use

Sarcoidosis is a systemic granulomatous disease of unknown etiology and pathogenesis with a heterogeneous clinical presentation. In the appropriate clinical and radiological context and with the exclusion of other diagnoses, the disease is characterized by the pathological presence of non-caseating epithelioid cell granulomas. Sarcoidosis is postulated to be a multifactorial disease caused by chronic antigenic stimulation. The immunopathogenesis of sarcoidosis encompasses a complex interaction between the host, genetic factors and postulated environmental and infectious triggers, which result in granuloma development.The exact pathogenesis of the disease has yet to be elucidated, but some of the inflammatory pathways that play a key role in disease progression and outcomes are becoming apparent, and these may form the logical basis for selecting potential biomarkers.Biomarkers are biological molecules that are altered pathologically. To date, there exists no single reliable biomarker for the evaluation of sarcoidosis, either diagnostically or prognostically but new candidates are emerging. A diagnosis of sarcoidosis ideally requires a biopsy confirming non-caseating granulomas, but the likelihood of progression that requires intervention remains unpredictable. These challenging aspects could be potentially resolved by incorporating biomarkers into clinical practice for both diagnosis and monitoring disease activity.This review outlines the current knowledge on sarcoidosis with an emphasis on pulmonary sarcoidosis, and delineates the understanding surrounding the implication of biomarkers for the clinical evaluation of sarcoidosis.

scholarly journals Pneumatosis Intestinalis: A Case Report and Approach to Management

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Sean Donovan ◽  
Joseph Cernigliaro ◽  
Nancy Dawson
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Bowel Wall ◽  
Surgical Intervention ◽  
Wide Spectrum ◽  
Pneumatosis Intestinalis ◽  
Rapid Evaluation ◽  
Thought Process ◽  
Diagnostic Dilemma ◽  
Clinical Scenarios

Pneumatosis intestinalis (PI), defined as gas within the bowel wall, is an uncommon radiographic sign which can represent a wide spectrum of diseases and a variety of underlying diagnoses. Because its etiology can vary greatly, management of PI ranges from surgical intervention to outpatient observation (see, Greenstein et al. (2007), Morris et al. (2008), and Peter et al. (2003)). Since PI is infrequently encountered, clinicians may be unfamiliar with its diagnosis and management; this unfamiliarity, combined with the potential necessity for urgent intervention, may place the clinician confronted with PI in a precarious medical scenario. We present a case of pneumatosis intestinalis in a patient who posed a particularly challenging diagnostic dilemma for the primary team. Furthermore, we explore the differential diagnosis prior to revealing the intervention offered to our patient; our concise yet inclusive differential and thought process for rapid evaluation may be of benefit to clinicians presented with similar clinical scenarios.

Antenatal diagnosis and perinatal care of anterior abdominal wall defects

1995 ◽  
Vol 7 (3) ◽  
pp. 109-127 ◽  
Author(s):  
Hratch L Karamanoukian ◽  
Stuart J O'Toole ◽  
Philip L Glick
Keyword(s):  
Parenteral Nutrition ◽  
Abdominal Wall ◽  
Antenatal Diagnosis ◽  
Anterior Abdominal Wall ◽  
Wide Spectrum ◽  
Perinatal Care ◽  
Postoperative Management ◽  
Abdominal Wall Defects ◽  
Anaesthetic Care ◽  
Prognostic Implications

Improvements in anaesthetic care and postoperative management over the last two decades have significantly improved survival of neonates with ventral abdominal wall defects, from a dismal 47% in 1971, to 96% for both gastroschisis and isolated omphalocele in two recent series. This increased survival has generally been attributed to result from improvements in the pre and postoperative management of these fragile neonates. Specifically, the routine use of total parenteral nutrition, and staged repairs for cases with severe “viscero-abdominal disproportion” have been implicated in a decreased incidence of sepsis, morbidity and mortality. In addition, an appreciation of the wide spectrum of anomalies uniquely associated with gastroschisis and omphalocele have helped improve survival, as each has unique pathophysiologic features that have prognostic implications for the fetus before, during and after delivery.

scholarly journals CD39+ EXPRESSION BY REGULATORY T CELLS IN PULMONARY SARCOIDOSIS AND LOFGREN’S SYNDROME

2019 ◽  
Vol 21 (3) ◽  
pp. 467-478
Author(s):  
I. V. Kudryavtsev ◽  
N. M. Lazareva ◽  
O. P. Baranova ◽  
A. S. Golovkin ◽  
D. V. Isakov ◽  
...  
Keyword(s):  
T Cells ◽  
Regulatory T Cells ◽  
Peripheral Blood ◽  
Relative Number ◽  
Epithelioid Cell ◽  
Pulmonary Sarcoidosis ◽  
Peripheral Blood Cell ◽  
Unknown Etiology ◽  
Löfgren’S Syndrome ◽  
Acute Form

Sarcoidosis is a disorder of unknown etiology characterized by development of necrosis-free epithelioid cell granulomas in various tissues. There are two main phenotypes of pulmonary sarcoidosis (PS): Lofgren’s syndrome (LS) is an acute form with favorable outcome, while non-Lofgren’s syndrome (nLS) is a chronic type of disease that can lead to pulmonary fibrosis in 20% of cases.Our study was aimed at investigating changes in the main cell-surface differentiation antigens on peripheral blood regulatory T cells (Tregs) from the patients with first diagnosed PS without treatment (LS, n = 11) and nLS (n = 46) compared to healthy volunteers (HC, n = 26).These indexes might be used as immunological markers for predicting severity of this disorder. Flow cytometry analysis of peripheral blood cell samples demonstrated that the nLS patients had decreased relative numbers of CD3+ cells vs healthy controls, as well as diminished CD3+CD4+ cells vs HC and LS patients. Furthermore, the relative and absolute Treg numbers were also decreased in nLS group vs HC (2.83% (2.47; 3.36) vs 3.33% (2.79; 3.84), p = 0.021), and 37 (29; 52) cells vs 50 (42; 65), p = 0.004, respectively) per one microliter of peripheral blood. Relative number of CD39-positive Тregs in chronic vs acute sarcoidosis patients was associated with 51.02% (38.20; 61.62) vs 48.64% (41.46; 63.72) that was significantly (p < 0.001 and p = 0.007, respectively) higher than in HC (39.52% (11.55; 46.34). We have found that “naïve” (CD45R0-CD62L+) Тregs did not significantly differ in percentage of CD39- and CD73-positive cells in all the groups tested. Moreover, CD45R0+CD62L+ Тregs in LS and nLS patients contained significantly more CD39-positive cells (69.66% (61.92; 79.34) and 67.62% (61.92; 79.34), respectively, compared to 47.55% (15.74; 65.32) in HC (p < 0.001 and p = 0.004, respectively). In case of CD45R0 + CD62LTregs able to exit from the circulation and migrate to the site of inflammation, an increased percentage of CD39-positive subset was noted only in patients with chronic sarcoidosis and HC (61.79% (55.12; 73.09) and 57.27% (16.03; 66.98), p = 0.006). Enhanced CD39 expression on Tregs seems to be related to chronic immune response, so that antigen elimination becomes impossible due to Treg overactivation, as shown in patients with sarcoidosis and some other chronic autoimmune and infectious disorders.

scholarly journals Biomarkers in systemic sclerosis-associated interstitial lung disease: review of the literature

Rheumatology ◽  
2019 ◽  
Vol 58 (9) ◽  
pp. 1534-1546 ◽  
Author(s):  
Olivier Bonhomme ◽  
Béatrice André ◽  
Fanny Gester ◽  
Dominique de Seny ◽  
Catherine Moermans ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Wide Spectrum ◽  
Unknown Origin ◽  
Multiple Organ ◽  
Correct Identification ◽  
Review Of The Literature ◽  
Clinical Value ◽  
Diagnosis And Prognosis ◽  
Risk Of Progression

Abstract SSc is a rare disease of unknown origin associated with multiple organ involvement. One of the major complications that drives the mortality of SSc patients is interstitial lung disease. The course of SSc-interstitial lung disease progression has a wide spectrum. Since the treatment is based on aggressive immunosuppression it should not be given to stable or non-progressing disease. The correct identification of disease with high risk of progression remains a challenge for early therapeutic intervention, and biomarkers remain urgently needed. In fact, eight categories of biomarkers have been identified and classified according to the different biological pathways involved. The purpose of this article is to describe the main biomarkers thought to be of interest with clinical value in the diagnosis and prognosis of SSc-interstitial lung disease.

scholarly journals Sacroiliac Pain: A Clinical Approach for the Neurosurgeon

2017 ◽  
Vol 08 (04) ◽  
pp. 622-627 ◽  
Author(s):  
Luis Rafael Moscote-Salazar ◽  
Hernando Raphael Alvis-Miranda ◽  
Andrei Fernandes Joaquim ◽  
Jessica Amaya-Quintero ◽  
Huber S. Padilla-Zambrano ◽  
...  
Keyword(s):  
Public Health Problem ◽  
Diagnostic Techniques ◽  
Unknown Etiology ◽  
Clinical Approach ◽  
Clinical Criteria ◽  
Positive Data ◽  
Health Related ◽  
Important Amount ◽  
Rule Out

ABSTRACTPain originating from sacroiliac joint may also cause pain in the lumbar and gluteal region in 15% of the population. The clinical manifestation represents a public health problem due to the great implications on the quality of life and health-related costs. However, this is a diagnosis that is usually ignored in the general clinical practice; probably because of the unknown etiology, making harder to rule out the potential etiologies of this pathology, or maybe because the clinical criteria that support this pathology are unknown. By describing several diagnostic techniques, many authors have studied the prevalence of this pathology, finding more positive data than expected; coming to the conclusion that even though there is no diagnostic gold standard yet, an important amount of cases might be detected by properly applying several tests at the physical examination. Thus, it is necessary to have knowledge of the physiopathology and clinical presentation so that diagnosis can be made to those patients that manifest this problem. We present a clinical approach for the neurosurgeon.

scholarly journals Biatrial Cardiac Metastases in a Patient with Uterine Cervix Malignant Melanoma

2015 ◽  
Vol 2015 ◽  
pp. 1-5
Author(s):  
Caglayan Geredeli ◽  
Melih Cem Boruban ◽  
Necdet Poyraz ◽  
Mehmet Artac ◽  
Alpay Aribas ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Uterine Cervix ◽  
Tissue Characterization ◽  
Primary Melanoma ◽  
Diagnostic Techniques ◽  
Cardiac Metastasis ◽  
Cardiac Tumors ◽  
Malignant Melanomas ◽  
Cardiac Metastases

Primary malignant melanomas of uterine cervix are quite rarely seen neoplasms, and long-life prognosis of patients with this disease is poor. Immunohistochemical methods and exclusion of other primary melanoma sites are used to confirm the diagnosis. As with other melanomas, cervix malignant melanomas may also cause cardiac metastases. Cardiac metastases are among rarely seen but more commonly encountered cases, compared to primary cardiac tumors. Here, we present a case of biatrial cardiac metastases in a 73-year-old patient with uterine cervix malignant melanomas. The patient underwent echocardiography, cardiac magnetic resonance imaging, and computed tomography. Our report shows the importance of advanced diagnostic techniques, such as cardiac magnetic resonance, not only for the detection of cardiac masses, but for a better anatomic definition and tissue characterization. Although the cases of malignant melanomas leading to multiple cardiac metastasis were reported in literature, the metastatic concurrence of malignant melanomas in both right and left atriums is quite rarely encountered as metastatic malignant melanomas. Also, another intriguing point in our case is that the primary lesion of our case was stemmed from uterine cervix, but not skin.

scholarly journals Sarcoidosis exosomes stimulate monocytes to produce pro-inflammatory cytokines and CCL2

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Casper J. E. Wahlund ◽  
Gozde Gucluler Akpinar ◽  
Loïc Steiner ◽  
Ahmed Ibrahim ◽  
Elga Bandeira ◽  
...  
Keyword(s):  
Reactive Oxygen Species Generation ◽  
Cell Types ◽  
Lavage Fluid ◽  
Pulmonary Sarcoidosis ◽  
Unknown Etiology ◽  
Cysteinyl Leukotriene ◽  
Asthma Drug ◽  
Cell Sources ◽  
Cysteinyl Leukotriene Receptor ◽  
Dose Dependent

Abstract Pulmonary sarcoidosis has unknown etiology, a difficult diagnostic procedure and no curative treatment. Extracellular vesicles including exosomes are nano-sized entities released from all cell types. Previous studies of exosomes from bronchoalveolar lavage fluid (BALF) of sarcoidosis patients have revealed pro-inflammatory components and abilities, but cell sources and mechanisms have not been identified. In the current study, we found that BALF exosomes from sarcoidosis patients, but not from healthy individuals, induced a dose-dependent elevation of intracellular IL-1β in monocytes. Analyses of supernatants showed that patient exosomes also induced release of IL-1β, IL-6 and TNF from both PBMCs and enriched monocytes, suggesting that the observed effect is direct on monocytes. The potently chemotactic chemokine CCL2 was induced by exosomes from a subgroup of patients, and in a blocking assay the exosome-induced CCL2 was reduced for 13 out of 19 patients by the asthma drug Montelukast, a cysteinyl leukotriene receptor antagonist. Further, reactive oxygen species generation by PBMCs was induced to a higher degree by patient exosomes compared to healthy exosomes. These findings add to an emerging picture of exosomes as mediators and disseminators of inflammation, and open for further investigations of the link between CCL2 and exosomal leukotrienes in sarcoidosis.

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