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The Pathogenesis of Pulmonary Sarcoidosis and Implications for Treatment

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

Deadline for manuscript submissions: closed (30 September 2021) | Viewed by 2933

Special Issue Editors


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Guest Editor
INSERM U1272 “Hypoxia and the Lung”, Université Sorbonne Paris Nord, Bobigny, France
Interests: sarcoidosis; granuloma; hypoxia; interstitial lung disease; fibrosis; macrophages

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Co-Guest Editor
INSERM U1135, CIMI (Centre d'immunologie et des maladies infectieuses), Sorbonne Université, APHP - Pitié-Salpètrière Hospital –Immunology Department
Interests: sarcoidosis; granuloma; interstitial lung disease; fibrosis; immunoregulation; T cells

Special Issue Information

Dear Colleagues,

Sarcoidosis is a rare systemic granulomatous disease of unknown cause(s) that initiates in genetically predisposed individuals a disproportionate inflammatory response leading to granuloma formation. For most patients, spontaneous resolution or short courses of glucocorticoids will take place; however, absence of response to existing lines of treatment will lead to a reduced quality of life with dyspnea, fatigue, and severe disabilities associated with pulmonary, cardiac, and nervous system impairment. Both the innate and adaptive immune systems are involved in sarcoidosis pathogenesis. Factors influencing the disease course towards clinical resolution versus chronic disease and fibrosis remain unknown and represent major targets for the development of new treatment strategies to replace/enhance current therapies, including steroids and/or biologic agents. Identification of new mechanisms in the pathogenesis of sarcoidosis should help us to better understand the variability in disease course amongst patients and to develop more effective treatments for sarcoidosis.

Dr. Valérie Besnard
Guest Editor
Dr. Makoto Miyara
Co-Guest Editor

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Keywords

  • sarcoidosis
  • granulomatosis
  • genetics
  • environment
  • immunopathology
  • models
  • corticosteroids
  • biologic treatments

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Published Papers (1 paper)

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Research

12 pages, 2226 KiB  
Communication
Tumor-Associated Granulomas Preceding a Diagnosis of Thoracic Sarcoidosis: A Retrospective, Single-Center Cohort Study
by Patricia Muñoz-Hernández, Mariel Valdivia-Mazeyra, Julio Ancochea, Tamara Alonso Pérez, Olga Rajas, Claudia Valenzuela, Susana Hernández-Muñiz, Amparo Esteban-Peris and José A. Jiménez-Heffernan
J. Clin. Med. 2021, 10(18), 4151; https://doi.org/10.3390/jcm10184151 - 15 Sep 2021
Cited by 2 | Viewed by 2248
Abstract
There is a relationship between systemic sarcoidosis (SS) and malignancy. Sarcoidosis results from an exaggerated immune response in genetically susceptible individuals. In oncologic patients with sarcoidosis, tumoral antigens and antineoplastic treatment are considered potential triggering factors. The observation of a patient with granulomas [...] Read more.
There is a relationship between systemic sarcoidosis (SS) and malignancy. Sarcoidosis results from an exaggerated immune response in genetically susceptible individuals. In oncologic patients with sarcoidosis, tumoral antigens and antineoplastic treatment are considered potential triggering factors. The observation of a patient with granulomas in a parotid carcinoma who later developed SS led us to review the previous tumors of patients with SS. The aim of the study is to see whether granulomas were already present in the tumors that preceded sarcoidosis. We identified 196 sarcoidosis patients, 47 of whom had previously had a tumor. We were able to review 29 cases, 12 of which showed tumor-associated granulomas (TAGs) (41.4%). This ratio is much higher than that of the normal population (4.4–13.8). We analyzed five control patients without sarcoidosis for each tumor. In conclusion, we observed an increased number of TAGs in patients who later developed SS. This finding reinforces a pathogenic relationship between SS and neoplasia. The histology of tumors in patients with SS should be reviewed in an attempt to identify granulomas. Full article
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