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- W3217382617 abstract "Interstitial lung disease (ILD) is a common manifestation of the primary Sjögren’s syndrome (SS-ILD). The prevalence of progressive fibrosis in SS-ILD is unknown. The aim of this study was to describe the clinical characteristics of SS-ILD patients. Patients with SS (ACR/EULAR 2016) and ILD (HRCT defined) were retrospectively identified from the electronic files of two ILD reference centers. Patients with another connective tissue disease were excluded. We included 73 patients (58% women), mean age 63 years. At diagnosis, mean FVC was 73±21 % and DLCO 49±18 %. The initial HRCT patterns were: NSIP (n = 23), NSIP with OP (n = 2), OP (n = 4), UIP/probable UIP (n = 12), LIP (n = 5), HP (n = 2), indeterminate (n = 18). Mean follow-up was 5 years. Sixty patients (82%) received corticosteroids (n = 50) and/or immunosuppressants (rituximab, n = 31; cyclophosphamide, n = 25; azathioprine, n = 26; mycophenolate-mofetil, n = 25; hydroxychloroquine, n=11; others, n=7); 8 patients received antifibrotics. Four patients received a lung transplant. Twenty-five patients died. The cause of death was terminal respiratory failure (n=9), lung transplantation (n=2), sepsis (n=4), pulmonary hypertension (n=1), cardiovascular disease (n=3), colon cancer (n=1) and unknown (n=5). Lung function tests were available at follow-up in 69 patients. A relative decline in FVC ≥10% predicted was observed in 30 patients (43%) and a 5-9% decline in 13 patients (19%). Sequential lung-CT were available for review in 66 patients. Fibrosis extent increased in 44 patients (66%). We conclude that a progressive pulmonary fibrosis phenotype is common in patients with SS-ILD with a poor long-term survival." @default.
- W3217382617 created "2021-12-06" @default.
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- W3217382617 date "2021-09-05" @default.
- W3217382617 modified "2023-10-01" @default.
- W3217382617 title "Progressive fibrosis is common in patients with Sjögren’s syndrome and Interstitial lung disease" @default.
- W3217382617 doi "https://doi.org/10.1183/13993003.congress-2021.pa2245" @default.
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