Background
Interstitial lung disease (ILD) is a severe pulmonary complication of Sjögren disease (SjD), but its prevalence, natural history and survival are not completely understood. Our study aimed to investigate prevalence, incidence, and mortality of SjD-ILD in a cohort of unselected consecutive SjD patients.
Methods
all consecutive SjD patients referred to our centre were enrolled in the study. A careful assessment for respiratory symptoms was periodically performed for each patient, and high-resolution computed tomography (HRCT) was requested in case of new-onset dyspnoea, persistent dry cough, or detection of velcro crackles by mean of electronic auscultation (VECTOR).
Findings
At enrolment, ILD was detected in 61/257 patients with a prevalence of 23.7 %. During a mean follow-up of 42.6 ± 14.6 months, 3 new cases of ILD were recorded, with an incidence of 0.41 new cases per 100 patients/year. Multivariate analysis showed a direct association between ILD and male sex, age at SjD diagnosis, and erythro‑sedimentation rate >40 mm, and an inverse correlation with sicca syndrome. Nonspecific interstitial pneumonia was the most observed HRCT pattern, followed by usual interstitial pneumonia. During the follow-up, 21 patients (8.2 %) died, with a statistically significant difference between the overall survival of patients with (66.5 %±11.7) and without ILD (88.4 %±5.5) (p<0.001). A fibrotic pattern was associated to a worse survival rate, while no difference was observed according to the radiologic pattern. Anti-SSA antibody was a protective factor for death, while the age at diagnosis of SjD, and the extent of ILD at HRCT were directly associated to an increased mortality.
Interpretation
ILD can be identified in a high number of SjD patients, inducing a significant impairment in survival. The ILD extent, but not HRCT pattern of ILD, represents the main predictor of mortality. Therefore, careful monitoring, by a multidisciplinary team, should be ensured to all SjD-ILD patients.
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