Clinical Presentation of Connective Tissue Disease Patients with and without Interstitial Lung Disease: A Retrospective Study.
Antisynthetase syndrome
Idiopathic inflammatory myopathy
Interstitial lung disease
Rheumatoid arthritis
Sjӧgren’s syndrome
Systemic sclerosis
Journal
Respiration; international review of thoracic diseases
ISSN: 1423-0356
Titre abrégé: Respiration
Pays: Switzerland
ID NLM: 0137356
Informations de publication
Date de publication:
2023
2023
Historique:
received:
09
02
2023
accepted:
11
04
2023
medline:
22
6
2023
pubmed:
26
5
2023
entrez:
26
5
2023
Statut:
ppublish
Résumé
Connective tissue diseases (CTDs) are responsible for about 20% of interstitial lung disease (ILD) cases, but their diagnosis in a pulmonary unit (PU) is not always straightforward due to a heterogeneous clinical picture. The aim of this study was to evaluate the clinical presentation of rheumatoid arthritis (RA) and CTD-ILD cases diagnosed in PU, compared to RA and CTD patients diagnosed in a rheumatologic unit (RU). Patients with RA, systemic sclerosis (SSc), primary Sjӧgren's syndrome (pSS), and idiopathic inflammatory myopathy were retrospectively enrolled from an RU and a PU designated to manage ILD during a period from January 2017 to October 2022. The classification of CTD-PU was carried out in a multidisciplinary setting, including the same rheumatologists that diagnosed CTD in the RU. ILD-CTD-PU patients were prevalently male and older. Progression from undifferentiated CTD to a specific condition was more common in ILD-CTD-PU, and those patients generally obtained a lower score on specific classification criteria. RA-PU patients resembled polymyalgia rheumatica in 47.6% of cases, also showing a greater proportion of typical joint deformities (p = 0.02). SSc-PU patients showed a usual interstitial pneumonia pattern in 76% of cases and, compared with SSc-RU, were more commonly seronegative (p = 0.03) and generally lacked fingertip lesions (p = 0.02). The majority of the diagnoses of pSS-PU were in patients with previously diagnosed ILD, in which seropositivity and sicca syndrome developed during follow-up. CTD-ILD patients diagnosed in the PU show severe lung involvement and a nuanced autoimmune clinical picture.
Sections du résumé
BACKGROUND
Connective tissue diseases (CTDs) are responsible for about 20% of interstitial lung disease (ILD) cases, but their diagnosis in a pulmonary unit (PU) is not always straightforward due to a heterogeneous clinical picture.
OBJECTIVES
The aim of this study was to evaluate the clinical presentation of rheumatoid arthritis (RA) and CTD-ILD cases diagnosed in PU, compared to RA and CTD patients diagnosed in a rheumatologic unit (RU).
METHODS
Patients with RA, systemic sclerosis (SSc), primary Sjӧgren's syndrome (pSS), and idiopathic inflammatory myopathy were retrospectively enrolled from an RU and a PU designated to manage ILD during a period from January 2017 to October 2022. The classification of CTD-PU was carried out in a multidisciplinary setting, including the same rheumatologists that diagnosed CTD in the RU.
RESULTS
ILD-CTD-PU patients were prevalently male and older. Progression from undifferentiated CTD to a specific condition was more common in ILD-CTD-PU, and those patients generally obtained a lower score on specific classification criteria. RA-PU patients resembled polymyalgia rheumatica in 47.6% of cases, also showing a greater proportion of typical joint deformities (p = 0.02). SSc-PU patients showed a usual interstitial pneumonia pattern in 76% of cases and, compared with SSc-RU, were more commonly seronegative (p = 0.03) and generally lacked fingertip lesions (p = 0.02). The majority of the diagnoses of pSS-PU were in patients with previously diagnosed ILD, in which seropositivity and sicca syndrome developed during follow-up.
CONCLUSIONS
CTD-ILD patients diagnosed in the PU show severe lung involvement and a nuanced autoimmune clinical picture.
Identifiants
pubmed: 37231870
pii: 000530785
doi: 10.1159/000530785
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
405-415Informations de copyright
© 2023 S. Karger AG, Basel.