Interstitial Lung Disease and Pulmonary Damage in Primary Sjögren’s Syndrome: A Systematic Review and Meta-Analysis

Abstract

Background: Pulmonary lung involvement is the most common extra-glandular manifestation in patients with primary Sjögren’s syndrome (pSS), leading to a worsening of the patient’s prognosis. To date, different studies have assessed the prevalence of pulmonary involvement and interstitial lung disease (ILD) in pSS patients with different results. Methods: We performed a systematic literature review and meta-analysis on ILD pooled prevalence in pSS according to the PRISMA and MOOSE guidelines. Furthermore, we explored the pooled prevalence of the two main presentations of pSS-ILD, nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP). Results: We analysed the pSS-ILD prevalence in 30 studies including 8255 pSS patients. The pSS-ILD pooled prevalence was 23% (95% CI: 16–30). For NSIP, we found a pooled prevalence of 52% (CI 41–64), and for UIP we found a pooled prevalence of 44% (CI: 32–55). Regarding the meta-regression analysis, male gender, DLco value, country, and HRCT seem to contribute to the ILD presence. Conclusions: At least 20% of pSS patients have a comorbid ILD, usually NSIP. Male gender and alteration in DLco value may be considered the most important independent factors supporting an active search of lung complications during the clinical history of pSS patients.

Pulmonary Manifestations of Sjögren's Disease

“…Nonspecific interstitial pneumonia (NSIP) is the most commonly identified ILD subtype, present in 39 to 45.5% of patients with SjD-ILD. 12,15 This is followed by usual interstitial pneumonia (UIP) pattern (►Fig. 2) in 10 to 16%, organizing pneumonia (OP) in 4 to 7%, and LIP in 4 to 17%.…”

“…Recent data suggest a pooled prevalence between 13 and 23% for SjD-related ILD. 11,12 Male sex and older age were identified as risk factors for SjD-ILD in large meta-analyses. [11][12][13] Interestingly, male sex has also been reported to predict disease progression in mixed connective tissue disease (CTD)-related ILD.…”

“…11,12 Male sex and older age were identified as risk factors for SjD-ILD in large meta-analyses. [11][12][13] Interestingly, male sex has also been reported to predict disease progression in mixed connective tissue disease (CTD)-related ILD. 14 Cigarette smoking has also been identified as a potential risk factor in the development of SjD-ILD.…”

“…A recent large meta-analysis found a 25% pooled prevalence of SjD-related ILD based on HRCT diagnosis. 12 Common symptoms exhibited in SjD-ILD include cough and dyspnea with severity of symptoms varying from mild to severe. 15 Prevalence of ILD in patients with SjD increases over time, from 10% in their first year, to 20% after 5 years, and 47% after 15 years following the diagnosis of SjD.…”

Long-term functional course of Sjögren's disease-associated interstitial lung disease

“…Pulmonary involvement includes parenchymal disease, airway abnormalities, lymphoproliferative disorders and more rarely, vascular, muscular or pleural damage. Interstitial lung disease associated with primary SS (pSD-ILD), a common manifestation [2], can lead to acute or chronic respiratory failure and is associated with an increased mortality risk [3]. In recent years, a progressive pulmonary fibrosis (PPF) phenotype has been defined, characterized by an increase in respiratory symptoms, a decline in respiratory function and an increase in chest high resolution computed tomography (HRCT) abnormalities, despite appropriate treatment, over a period of 1 to 2 years [4,5].…”