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Keywords = fibrotic sarcoid

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20 pages, 1772 KB  
Review
Sarcoidosis-Associated Pulmonary Hypertension
by Dominique Israël-Biet, Jean Pastré and Hilario Nunes
J. Clin. Med. 2024, 13(7), 2054; https://doi.org/10.3390/jcm13072054 - 2 Apr 2024
Cited by 8 | Viewed by 5020
Abstract
Sarcoidosis-associated pulmonary hypertension (SAPH) is a very severe complication of the disease, largely impacting its morbidity and being one of its strongest predictors of mortality. With the recent modifications of the hemodynamic definition of pulmonary hypertension (mean arterial pulmonary pressure >20 instead of [...] Read more.
Sarcoidosis-associated pulmonary hypertension (SAPH) is a very severe complication of the disease, largely impacting its morbidity and being one of its strongest predictors of mortality. With the recent modifications of the hemodynamic definition of pulmonary hypertension (mean arterial pulmonary pressure >20 instead of <25 mmHg,) its prevalence is presently not precisely known, but it affects from 3 to 20% of sarcoid patients; mostly, although not exclusively, those with an advanced, fibrotic pulmonary disease. Its gold-standard diagnostic tool remains right heart catheterization (RHC). The decision to perform it relies on an expert decision after a non-invasive work-up, in which echocardiography remains the screening tool of choice. The mechanisms underlying SAPH, very often entangled, are crucial to define, as appropriate and personalized therapeutic strategies will aim at targeting the most significant ones. There are no recommendations so far as to the indications and modalities of the medical treatment of SAPH, which is based upon the opinion of a multidisciplinary team of sarcoidosis, pulmonary hypertension and sometimes lung transplant experts. Full article
(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Sarcoidosis)
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18 pages, 372 KB  
Review
High-Risk Sarcoidosis: A Focus on Pulmonary, Cardiac, Hepatic and Renal Advanced Diseases, as Well as on Calcium Metabolism Abnormalities
by Dominique Israël-Biet, Nicol Bernardinello, Jean Pastré, Claudio Tana and Paolo Spagnolo
Diagnostics 2024, 14(4), 395; https://doi.org/10.3390/diagnostics14040395 - 11 Feb 2024
Cited by 15 | Viewed by 4993
Abstract
Although sarcoidosis is generally regarded as a benign condition, approximately 20–30% of patients will develop a chronic and progressive disease. Advanced pulmonary fibrotic sarcoidosis and cardiac involvement are the main contributors to sarcoidosis morbidity and mortality, with failure of the liver and/or kidneys [...] Read more.
Although sarcoidosis is generally regarded as a benign condition, approximately 20–30% of patients will develop a chronic and progressive disease. Advanced pulmonary fibrotic sarcoidosis and cardiac involvement are the main contributors to sarcoidosis morbidity and mortality, with failure of the liver and/or kidneys representing additional life-threatening situations. In this review, we discuss diagnosis and treatment of each of these complications and highlight how the integration of clinical, pathological and radiological features may help predict the development of such high-risk situations in sarcoid patients. Full article
(This article belongs to the Special Issue Advances in Diagnostics and Management of Respiratory System)
18 pages, 18762 KB  
Article
Sarcoid-Like Granulomatous Disease: Pathologic Case Series in World Trade Center Dust Exposed Rescue and Recovery Workers
by Vasanthi R. Sunil, Jared Radbel, Sabiha Hussain, Kinal N. Vayas, Jessica Cervelli, Malik Deen, Howard Kipen, Iris Udasin, Robert Laumbach, Jag Sunderram, Jeffrey D. Laskin and Debra L. Laskin
Int. J. Environ. Res. Public Health 2019, 16(5), 815; https://doi.org/10.3390/ijerph16050815 - 6 Mar 2019
Cited by 8 | Viewed by 5178
Abstract
Sarcoid-like granulomatous diseases (SGD) have been previously identified in cohorts of World Trade Center (WTC) dust-exposed individuals. In the present studies, we analyzed lung and/or lymph node biopsies from patients referred to our clinic with suspected WTC dust-induced lung disease to evaluate potential [...] Read more.
Sarcoid-like granulomatous diseases (SGD) have been previously identified in cohorts of World Trade Center (WTC) dust-exposed individuals. In the present studies, we analyzed lung and/or lymph node biopsies from patients referred to our clinic with suspected WTC dust-induced lung disease to evaluate potential pathophysiologic mechanisms. Histologic sections of lung and/or lymph node samples were analyzed for markers of injury, oxidative stress, inflammation, fibrosis, and epigenetic modifications. Out of seven patients examined, we diagnosed four with SGD and two with pulmonary fibrosis; one was diagnosed later with SGD at another medical facility. Patients with SGD were predominantly white, obese men, who were less than 50 years old and never smoked. Cytochrome b5, cytokeratin 17, heme oxygenase-1, lipocalin-2, inducible nitric oxide synthase, cyclooxygenase 2, tumor necrosis factor α, ADP-ribosylation factor-like GTPase 11, mannose receptor-1, galectin-3, transforming growth factor β, histone-3 and methylated histone-3 were identified in lung and lymph nodes at varying levels in all samples examined. Three of the biopsy samples with granulomas displayed peri-granulomatous fibrosis. These findings are important and suggest the potential of WTC dust-induced fibrotic sarcoid. It is likely that patient demographics and/or genetic factors influence the response to WTC dust injury and that these contribute to different pathological outcomes. Full article
(This article belongs to the Special Issue Long-Term Health Effects of the 9/11 Disaster)
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