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Special Issue Information

Dear Colleagues,

Rare lung diseases are associated with high mortality and morbidity and a mean survival of about 5–6 years from the onset of symptoms. They are chronic, irreversible, progressive group of disorders associated with poor prognosis. It is difficult to diagnose due to similar clinical and radiological signs. Very few treatments are now available and lung transplantation is considered the best therapeutic option in end-stage and progressive form of disease. Risk factors can include smoking, genetic predisposition and environmental variables linked to pathological alteration of lung epithelium. The exact pathogenetic mechanisms leading to the development of these disorders are not fully understood. Molecular and cellular research on this field is useful for the study of these diseases since these aspects are fundamental in the pathogenesis and progression of rare lung disease.

Dr. Laura Bergantini
Dr. Miriana d'Alessandro
Guest Editors

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Keywords

pulmonary fibrosis
rare lung diseases
lung transplant
biomarkers
bronchoalveolar lavage

Published Papers (2 papers)

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Research

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11 pages, 2668 KiB

Open AccessArticle

Immunological Pathways in Sarcoidosis and Autoimmune Rheumatic Disorders—Similarities and Differences in an Italian Prospective Real-Life Preliminary Study

by Miriana d’Alessandro, Laura Bergantini, Sara Gangi, Edoardo Conticini, Dalila Cavallaro, Paolo Cameli, Fabrizio Mezzasalma, Luca Cantarini, Bruno Frediani and Elena Bargagli

Biomedicines 2023, 11(6), 1532; https://doi.org/10.3390/biomedicines11061532 - 25 May 2023

Cited by 5 | Viewed by 940

Abstract

Background: The pathogenesis of sarcoidosis involves T cells and B lymphocytes that produce autoantibodies. We compared the expression of different T and B cell subsets in sarcoidosis and three B-mediated rheumatic diseases that can affect the lungs in an attempt to identify similarities and differences that distinguish these diseases. Methods: The study included patients referred to Siena University Hospital’s respiratory disease and rheumatology units. Patients were enrolled prospectively and consecutively. Healthy volunteers were also included. Multicolor flow cytometry was performed on phenotype T and B cell subsets. Multivariate analysis was carried out to reduce the dimensionality of the data. Results: Fifteen patients had a diagnosis of sarcoidosis, fourteen idiopathic inflammatory myopathies (IIM), five granulomatosis with polyangiitis (GPA), ten microscopic polyangiitis (MPA), and seven were controls. Thirty-five T and B cell subsets were phenotyped, 15 of which were significantly different in sarcoidosis, B-mediated rheumatic disorders, and controls. Principal components analysis distinguished the four groups of patients with a total explained variance of 54.7%. A decision tree was constructed to determine which clustering variables would be most useful for distinguishing sarcoidosis, IIM, MPA, and GPA. The model showed regulatory T helper cells (Th-reg) > 5.70% in 91% of sarcoidosis patients as well as Th-reg ≤ 5.70 and Th17 > 43.27 in 100% of MPA. It also showed Th-reg ≤ 5.70, Th17 ≤ 43.27 and Tfh-reg ≥ 7.81 in 100% of GPA patients, and Th-reg ≤ 5.70, Th17 ≤ 43.27 and Tfh-reg ≤ 7.81 in 100% of IIM patients. Conclusion: The immune cell profile sheds light on similarities and differences between sarcoidosis and B-mediated rheumatic diseases. Sarcoidosis and autoimmune diseases show similar patterns of cellular immune dysregulation, suggesting a common pathogenic pathway that may provide an opportunity for further understanding autoimmunity and exploring biological therapies to treat sarcoidosis. Full article

(This article belongs to the Special Issue Molecular and Cellular Research on Rare Lung Diseases)

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Review

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26 pages, 1417 KiB

Open AccessReview

Markers of Bronchiolitis Obliterans Syndrome after Lung Transplant: Between Old Knowledge and Future Perspective

by Dalila Cavallaro, Marco Guerrieri, Stefano Cattelan, Gaia Fabbri, Sara Croce, Martina Armati, David Bennett, Antonella Fossi, Luca Voltolini, Luca Luzzi, Alberto Salvicchi, Piero Paladini, Adriano Peris, Miriana d’Alessandro, Paolo Cameli, Elena Bargagli, Tuscany Transplant Group and Laura Bergantini

Biomedicines 2022, 10(12), 3277; https://doi.org/10.3390/biomedicines10123277 - 17 Dec 2022

Cited by 4 | Viewed by 2389

Abstract

Bronchiolitis obliterans syndrome (BOS) is the most common form of CLAD and is characterized by airflow limitation and an obstructive spirometric pattern without high-resolution computed tomography (HRCT) evidence of parenchymal opacities. Computed tomography and microCT analysis show abundant small airway obstruction, starting from the fifth generation of airway branching and affecting up to 40–70% of airways. The pathogenesis of BOS remains unclear. It is a multifactorial syndrome that leads to pathological tissue changes and clinical manifestations. Because BOS is associated with the worst long-term survival in LTx patients, many studies are focused on the early identification of BOS. Markers may be useful for diagnosis and for understanding the molecular and immunological mechanisms involved in the onset of BOS. Diagnostic and predictive markers of BOS have also been investigated in various biological materials, such as blood, BAL, lung tissue and extracellular vesicles. The aim of this review was to evaluate the scientific literature on markers of BOS after lung transplant. We performed a systematic review to find all available data on potential prognostic and diagnostic markers of BOS. Full article

(This article belongs to the Special Issue Molecular and Cellular Research on Rare Lung Diseases)

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