Lung Involvement in Primary Sjögren's Syndrome-An Under-Diagnosed Entity.
ESSDAI—EULAR Sjögren's Syndrome Disease Activity Index
Sjögren's Syndrome (SS)
interstitial lung disease (ILD)
lung fibrosis
sicca syndrome
Journal
Frontiers in medicine
ISSN: 2296-858X
Titre abrégé: Front Med (Lausanne)
Pays: Switzerland
ID NLM: 101648047
Informations de publication
Date de publication:
2020
2020
Historique:
received:
15
12
2019
accepted:
04
06
2020
entrez:
9
8
2020
pubmed:
9
8
2020
medline:
9
8
2020
Statut:
epublish
Résumé
Interstitial lung disease (ILD) represents a frequent extra-glandular manifestation of primary Sjögren's Syndrome (pSS). Limited published data regarding phenotyping and treatment exists. Advances in managing specific ILD phenotypes have not been comprehensively explored in patients with coexisting pSS. This retrospective study aimed to phenotype lung diseases occurring in a well-described pSS-ILD cohort and describe treatment course and outcomes. Between April 2018 and February 2020, all pSS patients attending our Outpatient clinic were screened for possible lung involvement. Clinical, laboratory and high-resolution computed tomography (HRCT) findings were analyzed. Patients were classified according to HRCT findings into five groups: usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), combined pulmonary fibrosis and emphysema (CPFE), and non-specific-ILD. Lung involvement was confirmed in 31/268 pSS patients (13%). One-third (10/31) of pSS-ILD patients were Ro/SSA antibody negative. ILD at pSS diagnosis was present in 19/31 (61%) patients. The commonest phenotype was UIP
Identifiants
pubmed: 32766261
doi: 10.3389/fmed.2020.00332
pmc: PMC7378373
doi:
Types de publication
Journal Article
Langues
eng
Pagination
332Informations de copyright
Copyright © 2020 Sogkas, Hirsch, Olsson, Hinrichs, Thiele, Seeliger, Skripuletz, Schmidt, Witte, Jablonka and Ernst.
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