Search for Articles:
Title / Keyword
Author / Affiliation / Email
Journal
Article Type
 
 
Advanced Search
 
Section
Special Issue
Volume
Issue
Number
Page
 
5.4
3.9
Journals
JCM
Special Issues
Management of Sarcoidosis: Challenges and Solutions
share announcement

Management of Sarcoidosis: Challenges and Solutions

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Pulmonology".

Deadline for manuscript submissions: closed (31 December 2019) | Viewed by 51689

Special Issue Editors

Prof. Dominique Valeyre

E-Mail Website
Guest Editor
Paris 13 University, INSERM U 1272; Hopital Avicenne, AP HP, Serv Pneumol, Bobigny, France
Interests: sarcoidosis; interstitial lung diseases; idiopathic pulmonary fibrosis
Special Issues, Collections and Topics in MDPI journals
Dr. Pr. Jean-François Bernaudin

E-Mail Website
Guest Editor
Sorbonne University, Paris INSERM UMR 1272, Paris 13 University, 74 Rue Marcel Cachin, F-93017 Bobigny, France
Interests: pulmonary pathology; respiratory histophysiology; sarcoidosis; granuloma; comparative physiology; interstitial lung diseases
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Sarcoidosis is a systemic granulomatous disease whose cause remains unknown and which is still raising important challenges. This Special Issue will be focused on critical challenges for both clinical practice and understanding of the disease’s pathophysiology. Dyspnea and fatigue are frequent, major symptoms with multiple underlying mechanisms, even in a single patient. These mechanisms are essential to decipher in order to choose the most appropriate treatment. Quality of life is considered a priority by patients, and its assessment is crucial for monitoring treatments. While corticosteroid treatment is still the first-line treatment, adverse events are now better taken into account, and corticosteroid-sparing agents are proposed when appropriate. Danger sarcoidosis, associated with a lower survival or significant handicap, relies on specific criteria that are crucial for optimizing the treatment and in order to design trials with new drugs. Finally, recent advances on pathophysiology, including using models of sarcoidosis, should help to better understand such a mysterious disease as sarcoidosis.

Prof. Dominique Valeyre
Dr. Pr. Jean-François Bernaudin
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • sarcoidosis
  • dyspnea
  • fatigue
  • cardiac sarcoidosis
  • quality of life
  • danger sarcoidosis
  • corticosteroids
  • immunology
  • models
  • environment

Published Papers (11 papers)

Download All Papers
Order results
Result details
Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:

Editorial

Jump to: Research, Review

7 pages, 223 KiB  
Editorial
Management of Sarcoidosis, a Selection of Topical Items Updating
by Dominique Valeyre and Jean-François Bernaudin
J. Clin. Med. 2020, 9(10), 3220; https://doi.org/10.3390/jcm9103220 - 7 Oct 2020
Viewed by 1179
Abstract
First of all, we would like to thank all the authors for their contribution and the editorial staff who enabled the achievement of this “Management of Sarcoidosis: Challenges and Solutions” Special Issue [...] Full article
(This article belongs to the Special Issue Management of Sarcoidosis: Challenges and Solutions)

Research

Jump to: Editorial, Review

16 pages, 2418 KiB  
Article
Child–Adult Transition in Sarcoidosis: A Series of 52 Patients
by Simon Chauveau, Florence Jeny, Marie-Emeline Montagne, Rola Abou Taam, Véronique Houdouin, Ulrich Meinzer, Christophe Delacourt, Ralph Epaud, Fleur Cohen Aubart, Catherine Chapelon-Abric, Dominique Israël-Biet, Karine Juvin, Antoine Dossier, Bahram Bodaghi, Grégoire Prévot, Jean-Marc Naccache, Sarah Mattioni, Antoine Deschildre, Jacques Brouard, Abdellatif Tazi, Roderich Meckenstock, Morgane Didier, Julien Haroche, Annick Clement, Jean-François Bernaudin, Hilario Nunes, Dominique Valeyre, Nadia Nathan and for the French Sarcoidosis Group (GSF)add Show full author list remove Hide full author list
J. Clin. Med. 2020, 9(7), 2097; https://doi.org/10.3390/jcm9072097 - 3 Jul 2020
Cited by 12 | Viewed by 2458
Abstract
(1) Background: Pediatric sarcoidosis is a rare and mostly severe disease. Very few pediatric series with a prolonged follow-up are reported. We aimed to evaluate the evolution of pediatric sarcoidosis in adulthood. (2) Material and methods: Patients over 18-years-old with a pediatric-onset sarcoidosis [...] Read more.
(1) Background: Pediatric sarcoidosis is a rare and mostly severe disease. Very few pediatric series with a prolonged follow-up are reported. We aimed to evaluate the evolution of pediatric sarcoidosis in adulthood. (2) Material and methods: Patients over 18-years-old with a pediatric-onset sarcoidosis (≤15-year-old) who completed at least a three-year follow-up in French expert centers were included. Clinical information at presentation and outcome in adulthood were studied. (3) Results: A total of 52 patients were included (34 prospectively in childhood and 18 retrospectively in adulthood), with a mean age of 12 (±2.7) at diagnosis. The median duration time of follow-up was 11.5 years (range 3–44.5). Relapses mostly occurred during treatment decrease (84.5%), others within the three years after treatment interruption (9.1%), and rarely when the disease was stable for more than three years (6.4%). Sarcoidosis was severe in 11 (21.2%) in adulthood. Patients received a high corticosteroid cumulative dose (median 17,900 mg) for a median duration of five years (range 0–32), resulting in mostly mild (18; 35.3%) and rarely severe (2; 3.8%) adverse events. (4) Conclusions: Pediatric-onset sarcoidosis needed a long-term treatment in almost half of the patients. Around one fifth of pediatric-onset sarcoidosis patients had severe sarcoidosis consequences in adulthood. Full article
(This article belongs to the Special Issue Management of Sarcoidosis: Challenges and Solutions)
►▼ Show Figures

Figure 1

9 pages, 785 KiB  
Article
Presence of Antibodies Binding to Negative Elongation Factor E in Sarcoidosis
by Niklas Baerlecken, Nils Pursche, Torsten Witte, Katja Kniesch, Marius Höpfner, Diana Ernst, Frank Moosig, Benjamin Seeliger and Antje Prasse
J. Clin. Med. 2020, 9(3), 715; https://doi.org/10.3390/jcm9030715 - 6 Mar 2020
Cited by 3 | Viewed by 2412
Abstract
Sarcoidosis is characterized by multiorgan involvement and granulomatous inflammation. Its origin is unknown and the potential role of autoimmunity has not been sufficiently determined. We investigated the presence of autoantibodies in sarcoidosis using protein array technology. The derivation cohort consisted of patients with [...] Read more.
Sarcoidosis is characterized by multiorgan involvement and granulomatous inflammation. Its origin is unknown and the potential role of autoimmunity has not been sufficiently determined. We investigated the presence of autoantibodies in sarcoidosis using protein array technology. The derivation cohort consisted of patients with sarcoidosis (n = 25) and controls including autoimmune disease and blood donors (n = 246). In addition, we tested a validation cohort including pulmonary sarcoidosis patients (n = 58) and healthy controls (n = 13). Initially, sera of three patients with sarcoidosis were screened using a protein array with 28.000 proteins against controls. Thereby we identified the Negative Elongation Factor E (NELF-E) as an autoantigen. With confirmatory Enzyme-linked Immunosorbent Assay (ELISA)testing, 29/82 patients (35%) with sarcoidosis had antibodies against NELF-E of the Immunoglobulin (Ig) G type, whereas 18/253 (7%) sera of the controls were positive for NELF-E. Clinically, there was an association of the frequency of NELF-E antibody detection with lung parenchymal involvement and corresponding x-ray types. NELF-E autoantibodies are associated with sarcoidosis and should be further investigated. Full article
(This article belongs to the Special Issue Management of Sarcoidosis: Challenges and Solutions)
►▼ Show Figures

Figure 1

15 pages, 2131 KiB  
Article
Bone Marrow-Derived Mesenchymal Stromal Cells (MSCs) Modulate the Inflammatory Character of Alveolar Macrophages from Sarcoidosis Patients
by Ian McClain Caldwell, Christopher Hogden, Krisztian Nemeth, Michael Boyajian, Miklos Krepuska, Gergely Szombath, Sandra MacDonald, Mehrnoosh Abshari, Joel Moss, Lynn Vitale-Cross, Joseph R Fontana and Eva Mezey
J. Clin. Med. 2020, 9(1), 278; https://doi.org/10.3390/jcm9010278 - 19 Jan 2020
Cited by 13 | Viewed by 3596
Abstract
Sarcoidosis is a devastating inflammatory disease affecting many organs, especially the lungs and lymph nodes. Bone marrow-derived mesenchymal stromal cells (MSCs) can “reprogram” various types of macrophages towards an anti-inflammatory phenotype. We wanted to determine whether alveolar macrophages from sarcoidosis subjects behave similarly [...] Read more.
Sarcoidosis is a devastating inflammatory disease affecting many organs, especially the lungs and lymph nodes. Bone marrow-derived mesenchymal stromal cells (MSCs) can “reprogram” various types of macrophages towards an anti-inflammatory phenotype. We wanted to determine whether alveolar macrophages from sarcoidosis subjects behave similarly by mounting an anti-inflammatory response when co-cultured with MSCs. Fifteen sarcoidosis and eight control subjects underwent bronchoscopy and bronchoalveolar lavage (BAL). Unselected BAL cells (70–94% macrophages) were isolated and cultured with and without MSCs from healthy adults. Following stimulation of the cultured cells with lipopolysaccharide, the medium was removed to measure interleukin 10 and tumor necrosis factor alpha (IL-10 and TNF-α). In two additional sarcoidosis subjects, flow cytometry was used to study intracellular cytokines and surface markers associated with alveolar macrophages to confirm the results. Unselected BAL cells from sarcoidosis subjects co-cultured with MSCs showed a reduction in TNF-α (pro-inflammatory M1) and an increase in IL-10 (anti-inflammatory M2) in 9 of 11 samples studied. Control subject samples showed few, if any, differences in cytokine production. Unselected BAL cells from two additional patients analyzed by flow cytometry confirmed a switch towards an anti-inflammatory state (i.e., M1 to M2) after co-culture with MSCs. These results suggest that, similarly to other macrophages, alveolar macrophages also respond to MSC contacts by changing towards an anti-inflammatory phenotype. Based on our results, we hypothesize that mesenchymal stromal cells applied to the airways might alleviate lung inflammation and decrease steroid need in patients with sarcoidosis. Full article
(This article belongs to the Special Issue Management of Sarcoidosis: Challenges and Solutions)
►▼ Show Figures

Figure 1

12 pages, 624 KiB  
Article
Computed Tomography Images of Fibrotic Pulmonary Sarcoidosis Leading to Chronic Respiratory Failure
by Michiru Sawahata, Takeshi Johkoh, Takeshi Kawanobe, Chiyoko Kono, Yosikazu Nakamura, Masashi Bando, Koichi Hagiwara, Tamiko Takemura, Fumikazu Sakai, Noriharu Shijubo, Satoshi Konno and Tetsuo Yamaguchi
J. Clin. Med. 2020, 9(1), 142; https://doi.org/10.3390/jcm9010142 - 5 Jan 2020
Cited by 18 | Viewed by 2726
Abstract
Background: There is currently no consensus on the morphology of severe fibrotic pulmonary sarcoidosis, and we examined computed tomography (CT) findings and progression. Methods: We analyzed findings in 10 consecutive patients (three men, seven women) with pulmonary sarcoidosis requiring oxygen therapy for chronic [...] Read more.
Background: There is currently no consensus on the morphology of severe fibrotic pulmonary sarcoidosis, and we examined computed tomography (CT) findings and progression. Methods: We analyzed findings in 10 consecutive patients (three men, seven women) with pulmonary sarcoidosis requiring oxygen therapy for chronic respiratory failure, who were extracted from >2500 sarcoidosis patients (three hospitals, 2000–2018). Patients with comorbidities causing chronic respiratory failure were excluded. Results: Predominant findings were consolidations along the bronchovascular bundles comprising ‘central-peripheral band’, traction bronchiectasis, peripheral cysts/bullae, and upper lobe shrinkage. Traction bronchiectasis arose from opacities comprising ‘central-peripheral band’. Clustering of traction bronchiectasis at the distal side formed honeycomb lung-like structures in three patients. Upper lobe shrinkage progressed in seven patients together with progression of consolidations, ‘central-peripheral band’, traction bronchiectasis clusters, and cysts, while patients without shrinkage included two patients with severe multiple cysts without traction bronchiectasis. Restrictive ventilatory impairment developed in most patients. Pulmonary hypertension (PH) was detected radiologically in five patients, and chronic progressive pulmonary aspergillosis (CPPA) in four patients. Conclusions: During progression, consolidations comprising ‘central-peripheral band’ progressed together with traction bronchiectasis clusters and peripheral cysts, resulting in upper lobe shrinkage. This may lead to respiratory failure with possible complications such as PH and CPPA. Full article
(This article belongs to the Special Issue Management of Sarcoidosis: Challenges and Solutions)
►▼ Show Figures

Figure 1

Review

Jump to: Editorial, Research

21 pages, 881 KiB  
Review
Current Insights in Genetics of Sarcoidosis: Functional and Clinical Impacts
by Alain Calender, Thomas Weichhart, Dominique Valeyre and Yves Pacheco
J. Clin. Med. 2020, 9(8), 2633; https://doi.org/10.3390/jcm9082633 - 13 Aug 2020
Cited by 43 | Viewed by 4779
Abstract
Sarcoidosis is a complex disease that belongs to the vast group of autoinflammatory disorders, but the etiological mechanisms of which are not known. At the crosstalk of environmental, infectious, and genetic factors, sarcoidosis is a multifactorial disease that requires a multidisciplinary approach for [...] Read more.
Sarcoidosis is a complex disease that belongs to the vast group of autoinflammatory disorders, but the etiological mechanisms of which are not known. At the crosstalk of environmental, infectious, and genetic factors, sarcoidosis is a multifactorial disease that requires a multidisciplinary approach for which genetic research, in particular, next generation sequencing (NGS) tools, has made it possible to identify new pathways and propose mechanistic hypotheses. Codified treatments for the disease cannot always respond to the most progressive forms and the identification of new genetic and metabolic tracks is a challenge for the future management of the most severe patients. Here, we review the current knowledge regarding the genes identified by both genome wide association studies (GWAS) and whole exome sequencing (WES), as well the connection of these pathways with the current research on sarcoidosis immune-related disorders. Full article
(This article belongs to the Special Issue Management of Sarcoidosis: Challenges and Solutions)
►▼ Show Figures

Graphical abstract

20 pages, 744 KiB  
Review
Models Contribution to the Understanding of Sarcoidosis Pathogenesis: “Are There Good Models of Sarcoidosis?”
by Valérie Besnard and Florence Jeny
J. Clin. Med. 2020, 9(8), 2445; https://doi.org/10.3390/jcm9082445 - 31 Jul 2020
Cited by 11 | Viewed by 5509
Abstract
Sarcoidosis is a systemic, granulomatous, and noninfectious disease of unknown etiology. The clinical heterogeneity of the disease (targeted tissue(s), course of the disease, and therapy response) supports the idea that a multiplicity of trigger antigens may be involved. The pathogenesis of sarcoidosis is [...] Read more.
Sarcoidosis is a systemic, granulomatous, and noninfectious disease of unknown etiology. The clinical heterogeneity of the disease (targeted tissue(s), course of the disease, and therapy response) supports the idea that a multiplicity of trigger antigens may be involved. The pathogenesis of sarcoidosis is not yet completely understood, although in recent years, considerable efforts were put to develop novel experimental research models of sarcoidosis. In particular, sarcoidosis patient cells were used within in vitro 3D models to study their characteristics compared to control patients. Likewise, a series of transgenic mouse models were developed to highlight the role of particular signaling pathways in granuloma formation and persistence. The purpose of this review is to put in perspective the contributions of the most recent models in the understanding of sarcoidosis. Full article
(This article belongs to the Special Issue Management of Sarcoidosis: Challenges and Solutions)
►▼ Show Figures

Figure 1

21 pages, 811 KiB  
Review
Sarcoidosis: Causes, Diagnosis, Clinical Features, and Treatments
by Rashi Jain, Dhananjay Yadav, Nidhi Puranik, Randeep Guleria and Jun-O Jin
J. Clin. Med. 2020, 9(4), 1081; https://doi.org/10.3390/jcm9041081 - 10 Apr 2020
Cited by 76 | Viewed by 10335
Abstract
Sarcoidosis is a multisystem granulomatous disease with nonspecific clinical manifestations that commonly affects the pulmonary system and other organs including the eyes, skin, liver, spleen, and lymph nodes. Sarcoidosis usually presents with persistent dry cough, eye and skin manifestations, weight loss, fatigue, night [...] Read more.
Sarcoidosis is a multisystem granulomatous disease with nonspecific clinical manifestations that commonly affects the pulmonary system and other organs including the eyes, skin, liver, spleen, and lymph nodes. Sarcoidosis usually presents with persistent dry cough, eye and skin manifestations, weight loss, fatigue, night sweats, and erythema nodosum. Sarcoidosis is not influenced by sex or age, although it is more common in adults (< 50 years) of African-American or Scandinavians decent. Diagnosis can be difficult because of nonspecific symptoms and can only be verified following histopathological examination. Various factors, including infection, genetic predisposition, and environmental factors, are involved in the pathology of sarcoidosis. Exposures to insecticides, herbicides, bioaerosols, and agricultural employment are also associated with an increased risk for sarcoidosis. Due to its unknown etiology, early diagnosis and detection are difficult; however, the advent of advanced technologies, such as endobronchial ultrasound-guided biopsy, high-resolution computed tomography, magnetic resonance imaging, and 18F-fluorodeoxyglucose positron emission tomography has improved our ability to reliably diagnose this condition and accurately forecast its prognosis. This review discusses the causes and clinical features of sarcoidosis, and the improvements made in its prognosis, therapeutic management, and the recent discovery of potential biomarkers associated with the diagnostic assay used for sarcoidosis confirmation. Full article
(This article belongs to the Special Issue Management of Sarcoidosis: Challenges and Solutions)
►▼ Show Figures

Figure 1

10 pages, 253 KiB  
Review
In Which Patients with Sarcoidosis Is FDG PET/CT Indicated?
by Ruth G.M. Keijsers and Jan C. Grutters
J. Clin. Med. 2020, 9(3), 890; https://doi.org/10.3390/jcm9030890 - 24 Mar 2020
Cited by 42 | Viewed by 4818
Abstract
Sarcoidosis is a granulomatous disease of which the etiology remains unknown. The diverse clinical manifestations may challenge clinicians, particularly when conventional markers are inconclusive. From various studies, it has become clear that fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT aids in sarcoidosis care. In [...] Read more.
Sarcoidosis is a granulomatous disease of which the etiology remains unknown. The diverse clinical manifestations may challenge clinicians, particularly when conventional markers are inconclusive. From various studies, it has become clear that fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT aids in sarcoidosis care. In this article, an update on FDG PET/CT in sarcoidosis is provided. The use of FDG PET/CT in the diagnostic process of sarcoidosis is explained, especially in determining treatable inflammatory lesions in symptomatic patients with indecisive conventional tests. Furthermore, FDG PET/CT for evaluating the potential benefit of additional inflammatory treatment is described and its use in cardiac sarcoidosis is highlighted. Full article
(This article belongs to the Special Issue Management of Sarcoidosis: Challenges and Solutions)
14 pages, 932 KiB  
Review
Comprehensive Care for Patients with Sarcoidosis
by Catharina C. Moor, Vivienne Kahlmann, Daniel A. Culver and Marlies S. Wijsenbeek
J. Clin. Med. 2020, 9(2), 390; https://doi.org/10.3390/jcm9020390 - 1 Feb 2020
Cited by 26 | Viewed by 9273
Abstract
Sarcoidosis is a multisystem granulomatous disease, associated with significant morbidity and impaired quality of life. Treatment is aimed at recovering organ function, reducing symptom burden and improving quality of life. Because of the heterogeneity and variable disease course, a comprehensive, multidisciplinary approach to [...] Read more.
Sarcoidosis is a multisystem granulomatous disease, associated with significant morbidity and impaired quality of life. Treatment is aimed at recovering organ function, reducing symptom burden and improving quality of life. Because of the heterogeneity and variable disease course, a comprehensive, multidisciplinary approach to care is needed. Comprehensive care includes not only pharmacological interventions, but also supportive measures aimed at relieving symptoms and improving quality of life. The purpose of this review is to summarize the most recent knowledge regarding different aspects of care and propose a structured approach to sarcoidosis management. Full article
(This article belongs to the Special Issue Management of Sarcoidosis: Challenges and Solutions)
►▼ Show Figures

Figure 1

17 pages, 738 KiB  
Review
Role of Bronchoscopic Techniques in the Diagnosis of Thoracic Sarcoidosis
by Cecília Pedro, Natália Melo, Hélder Novais e Bastos, Adriana Magalhães, Gabriela Fernandes, Natália Martins, António Morais and Patrícia Caetano Mota
J. Clin. Med. 2019, 8(9), 1327; https://doi.org/10.3390/jcm8091327 - 28 Aug 2019
Cited by 20 | Viewed by 3562
Abstract
The diagnosis of sarcoidosis relies on clinical and radiological presentation, evidence of non-caseating granulomas in histopathology and exclusion of alternative causes of granulomatous inflammation. Currently, a proper diagnosis, with a high level of confidence, is considered as key to the appropriate diagnosis and [...] Read more.
The diagnosis of sarcoidosis relies on clinical and radiological presentation, evidence of non-caseating granulomas in histopathology and exclusion of alternative causes of granulomatous inflammation. Currently, a proper diagnosis, with a high level of confidence, is considered as key to the appropriate diagnosis and management of the disease. In this sense, this review aims to provide a brief overview on the role of bronchoscopy in the diagnosis of thoracic sarcoidosis, incorporating newer techniques to establish, including endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA), endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), transesophageal ultrasound-guided needle aspiration with the use of an echo bronchoscope (EUS-B-FNA) and transbronchial lung cryobiopsy (TBLC). Most of the literature reports the diagnostic superiority of endosonographic techniques, such as EBUS-TBNA alone or in combination with EUS-FNA, over conventional bronchoscopic modalities in diagnosing Scadding stages I and II of the disease. Moreover, TBLC may be considered a useful and safe diagnostic tool for thoracic sarcoidosis, overcoming some limitations of transbronchial lung biopsy (TBLB), avoiding more invasive modalities and being complementary to endosonographic procedures such as EBUS-TBNA. Full article
(This article belongs to the Special Issue Management of Sarcoidosis: Challenges and Solutions)
►▼ Show Figures

Figure 1

Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:
 
Displaying articles 1-11
Back to TopTop