Fibrosing interstitial lung disease in primary Sjogren syndrome

• Published in: Joint, bone, spine : revue du rhumatisme bone, spine : revue du rhumatisme, 2021-12, Vol.88 (6), p.105237-105237, Article 105237
• Main Authors: Manfredi, Andreina, Vacchi, Caterina, DellaCasa, Giovanni, Cerri, Stefania, Cassone, Giulia, Di Cecco, Giovanna, Luppi, Fabrizio, Salvarani, Carlo, Sebastiani, Marco
• Format: Article
• Language: English
• Subjects: Fibrosing interstitial lung disease; Radiologic pattern; Sjogren's syndrome
• ISSN: 1297-319X; 1778-7254
• DOI: 10.1016/j.jbspin.2021.105237

•Interstitial lung disease impacts on quality of life and prognosis of patients with primary Sjogren's syndrome.•Our data suggest a high prevalence of fibrosing phenotype in interstitial lung disease related to primary Sjogren's syndrome.•Fibrosing phenotype is frequently diagnosed early in patients with primary Sjogren's syndrome. Interstitial lung disease (ILD) represents the main pulmonary involvement in primary Sjogren syndrome (pSS). A proportion of patients with pSS develop a progressive fibrosing form of ILD, but no data are available about the prevalence of these patterns in pSS patients. Aim of this monocentric, cross-sectional study was to investigate the prevalence of fibrosing patterns in pSS patients with ILD. All consecutive patients fulfilling classification criteria for pSS with a new or previous diagnosis of ILD were enrolled in the study. Diagnosis of ILD was always performed by mean of HRCT and specific patterns were identified according to current classification criteria and divided in two groups according to the detection of a fibrotic pattern. Thirty-four pSS-ILD patients were enrolled in the study (males/females 3/31, median age 69.5 years, median pSS duration 47.5 months). Fibrotic pattern was detected in 52.9% of patients, namely: UIP (13 patients, 38.2%), fibrotic NSIP (4, 11.8%), fibrotic OP (1 2.9%) and group 2 (16 pts, 47.1%) including NSIP (6, 17.6%), OP (4, 11.8%), LIP (2, 5.9%) and unclassifiable (4, 11.8%). These patients were younger and with a shorter pSS duration at ILD diagnosis, in particular ILD diagnosis was prior or concurrent to pSS in 83.3% of cases compared to 62.5% in the group of nonfibrotic pattern (P