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Primary-Sjögren’s-Syndrome-Related Interstitial Lung Disease: A Clinical Review Discussing Current Controversies

Lung involvement, especially interstitial lung disease, is a potentially severe extra-glandular manifestation of Primary Sjogren’s Syndrome (pSS-ILD). ILD can manifest either as a late complication of pSS or anticipate sicca symptoms, likely reflecting two different patho-physiological entities. Pre...

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Autores principales: La Rocca, Gaetano, Ferro, Francesco, Sambataro, Gianluca, Elefante, Elena, Fonzetti, Silvia, Fulvio, Giovanni, Navarro, Inmaculada C., Mosca, Marta, Baldini, Chiara
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10218845/
https://www.ncbi.nlm.nih.gov/pubmed/37240535
http://dx.doi.org/10.3390/jcm12103428
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author La Rocca, Gaetano
Ferro, Francesco
Sambataro, Gianluca
Elefante, Elena
Fonzetti, Silvia
Fulvio, Giovanni
Navarro, Inmaculada C.
Mosca, Marta
Baldini, Chiara
author_facet La Rocca, Gaetano
Ferro, Francesco
Sambataro, Gianluca
Elefante, Elena
Fonzetti, Silvia
Fulvio, Giovanni
Navarro, Inmaculada C.
Mosca, Marta
Baldini, Chiara
author_sort La Rocca, Gaetano
collection PubMed
description Lung involvement, especially interstitial lung disease, is a potentially severe extra-glandular manifestation of Primary Sjogren’s Syndrome (pSS-ILD). ILD can manifest either as a late complication of pSS or anticipate sicca symptoms, likely reflecting two different patho-physiological entities. Presence of lung involvement in pSS subjects can remain subclinical for a long time; therefore, patients should be actively screened, and lung ultrasound is currently being investigated as a potential low cost, radiation-free, easily repeatable screening tool for detection of ILD. In contrast, rheumatologic evaluation, serology testing, and minor salivary gland biopsy are crucial for the recognition of pSS in apparently idiopathic ILD patients. Whether the HRCT pattern influences prognosis and treatment response in pSS-ILD is not clear; a UIP pattern associated with a worse prognosis in some studies, but not in others. Many aspects of pSS-ILD, including its actual prevalence, association with specific clinical–serological characteristics, and prognosis, are still debated by the current literature, likely due to poor phenotypic stratification of patients in clinical studies. In the present review, we critically discuss these and other clinically relevant “hot topics” in pSS-ILD. More specifically, after a focused discussion, we compiled a list of questions regarding pSS-ILD that, in our opinion, are not easily answered by the available literature. We subsequently tried to formulate adequate answers on the basis of an extensive literature search and our clinical experience. At the same, we highlighted different issues that require further investigation.
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spelling pubmed-102188452023-05-27 Primary-Sjögren’s-Syndrome-Related Interstitial Lung Disease: A Clinical Review Discussing Current Controversies La Rocca, Gaetano Ferro, Francesco Sambataro, Gianluca Elefante, Elena Fonzetti, Silvia Fulvio, Giovanni Navarro, Inmaculada C. Mosca, Marta Baldini, Chiara J Clin Med Review Lung involvement, especially interstitial lung disease, is a potentially severe extra-glandular manifestation of Primary Sjogren’s Syndrome (pSS-ILD). ILD can manifest either as a late complication of pSS or anticipate sicca symptoms, likely reflecting two different patho-physiological entities. Presence of lung involvement in pSS subjects can remain subclinical for a long time; therefore, patients should be actively screened, and lung ultrasound is currently being investigated as a potential low cost, radiation-free, easily repeatable screening tool for detection of ILD. In contrast, rheumatologic evaluation, serology testing, and minor salivary gland biopsy are crucial for the recognition of pSS in apparently idiopathic ILD patients. Whether the HRCT pattern influences prognosis and treatment response in pSS-ILD is not clear; a UIP pattern associated with a worse prognosis in some studies, but not in others. Many aspects of pSS-ILD, including its actual prevalence, association with specific clinical–serological characteristics, and prognosis, are still debated by the current literature, likely due to poor phenotypic stratification of patients in clinical studies. In the present review, we critically discuss these and other clinically relevant “hot topics” in pSS-ILD. More specifically, after a focused discussion, we compiled a list of questions regarding pSS-ILD that, in our opinion, are not easily answered by the available literature. We subsequently tried to formulate adequate answers on the basis of an extensive literature search and our clinical experience. At the same, we highlighted different issues that require further investigation. MDPI 2023-05-12 /pmc/articles/PMC10218845/ /pubmed/37240535 http://dx.doi.org/10.3390/jcm12103428 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
La Rocca, Gaetano
Ferro, Francesco
Sambataro, Gianluca
Elefante, Elena
Fonzetti, Silvia
Fulvio, Giovanni
Navarro, Inmaculada C.
Mosca, Marta
Baldini, Chiara
Primary-Sjögren’s-Syndrome-Related Interstitial Lung Disease: A Clinical Review Discussing Current Controversies
title Primary-Sjögren’s-Syndrome-Related Interstitial Lung Disease: A Clinical Review Discussing Current Controversies
title_full Primary-Sjögren’s-Syndrome-Related Interstitial Lung Disease: A Clinical Review Discussing Current Controversies
title_fullStr Primary-Sjögren’s-Syndrome-Related Interstitial Lung Disease: A Clinical Review Discussing Current Controversies
title_full_unstemmed Primary-Sjögren’s-Syndrome-Related Interstitial Lung Disease: A Clinical Review Discussing Current Controversies
title_short Primary-Sjögren’s-Syndrome-Related Interstitial Lung Disease: A Clinical Review Discussing Current Controversies
title_sort primary-sjögren’s-syndrome-related interstitial lung disease: a clinical review discussing current controversies
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10218845/
https://www.ncbi.nlm.nih.gov/pubmed/37240535
http://dx.doi.org/10.3390/jcm12103428
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