Long-term clinical course and outcome in patients with primary Sj\xf6gren syndrome-associated interstitial lung disease

Yun Jae Kim,Ho Jeong Kim,Jooae Choe,Jin Woo Song

doi:10.1038/s41598-021-92024-2

Yun Jae Kim, Ho Jeong Kim + Show 2 more

Open Access

https://doi.org/10.1038/s41598-021-92024-2

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Abstract

Interstitial lung disease (ILD) is the most common lung manifestation in patients with Sjögren syndrome (SJS) and is associated with poor outcomes. This study aimed to investigate the long-term clinical course and prognostic factors in patients with SJS-ILD. Clinical data and high-resolution computed tomography (HRCT) images of 62 patients with primary SJS-ILD were retrospectively analyzed (biopsy-proven cases, n = 16). The mean patient age was 59.8 years; 83.9% of the patients were females, and 38.7% showed a usual interstitial pneumonia (UIP) pattern on HRCT. The median follow-up period was 61.5 months. During follow-up, 15 patients (24.2%) died, 7 (11.3%) experienced acute exacerbation (AE), and 27 (43.5%) progressed. The 1-, 3- and 5-year survival rates were 93.5%, 85.8%, and 81.1%, respectively. Age (hazard ratio [HR]: 1.158, P = 0.003), C-reactive protein (CRP) level (HR: 1.212, P = 0.045), FVC (HR: 0.902, P = 0.005), and a UIP pattern on HRCT (HR: 4.580, P = 0.029) were significant prognostic factors in multivariable Cox analysis. In conclusion, death, AE, and ILD progression occurred in 25%, 10%, and 50% of the patients with SJS-ILD, respectively. Older age, higher CRP level, lower FVC, and a UIP pattern on HRCT indicated poor prognosis.

Highlights

Primary Sjögren syndrome (SJS) is a chronic systemic inflammatory disorder characterized by impaired functioning of the lacrimal and salivary glands[1,2,3]
Previous studies were conducted on a small number of patients, and the long-term clinical course and prognostic factors are still not well-defined in patients with SJS-Interstitial lung disease (ILD)
(HR 0.902; 95% confidence interval (CI) 0.839–0.969; P = 0.005), and a UIP pattern on high-resolution computed tomography (HRCT) (UIP to non-UIP, hazard ratio (HR) 4.580; 95% CI 1.167–17.975; P = 0.029) were independently associated with survival

Summary

Introduction

Primary Sjögren syndrome (SJS) is a chronic systemic inflammatory disorder characterized by impaired functioning of the lacrimal and salivary glands[1,2,3]. The long-term clinical course and prognostic factors in patients with SJS-ILD are not well-defined. Ito et al assessed 33 patients with primary SJS showing lung involvement and reported that a lower baseline arterial oxygen pressure ( PaO2) and presence of microscopic honeycombing on surgical lung biopsy were significant prognostic factors for m ortality[15]. Enomoto et al assessed 33 patients with SJS-ILD (all biopsy-proven cases) and reported that a higher arterial dioxide pressure (PaCO2), the extent of reticular abnormality on high-resolution computed tomography (HRCT), and the severity of fibroblastic foci on surgical lung biopsy were risk factors for m ortality[14]. Our study aimed to evaluate the long-term clinical course and prognostic factors in a large number of patients with SJS-ILD

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