Abstract
Interstitial lung disease (ILD) represents a frequent extra-glandular manifestation of primary Sjögren's Syndrome (pSS). Limited published data regarding phenotyping and treatment exists. Advances in managing specific ILD phenotypes have not been comprehensively explored in patients with coexisting pSS. This retrospective study aimed to phenotype lung diseases occurring in a well-described pSS-ILD cohort and describe treatment course and outcomes. Between April 2018 and February 2020, all pSS patients attending our Outpatient clinic were screened for possible lung involvement. Clinical, laboratory and high-resolution computed tomography (HRCT) findings were analyzed. Patients were classified according to HRCT findings into five groups: usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), combined pulmonary fibrosis and emphysema (CPFE), and non-specific-ILD. Lung involvement was confirmed in 31/268 pSS patients (13%). One-third (10/31) of pSS-ILD patients were Ro/SSA antibody negative. ILD at pSS diagnosis was present in 19/31 (61%) patients. The commonest phenotype was UIP n = 13 (43%), followed by NSIP n = 9 (29%), DIP n = 2 (6 %), CPFE n = 2 (6 %), and non-specific-ILD n = 5 (16%). Forced vital capacity (FVC) and carbon monoxide diffusion capacity (DLCO) appeared lower in UIP and DIP, without reaching a significant difference. Treatment focused universally on intensified immunosuppression, with 13/31 patients (42%) receiving cyclophosphamide. No anti-fibrotic treatments were used. Median follow-up was 38.2 [12.4–119.6] months. Lung involvement in pSS is heterogeneous. Better phenotyping and tailored treatment may improve outcomes and requires further evaluation in larger prospective studies.
Highlights
Primary Sjögren’s Syndrome is an increasingly recognized autoimmune disease, primarily affecting secretory gland tissue
All symptomatic patients underwent Pulmonary function testing (PFT), 36/51 had pathological findings defined as forced vital capacity (FVC) ≤ 80% predicted and/or DLCO ≤ 70% predicted
Our data raises a number of important aspects regarding interstitial lung disease (ILD) and primary Sjögren’s Syndrome (pSS) despite small cohort size and limited follow-up
Summary
Primary Sjögren’s Syndrome (pSS) is an increasingly recognized autoimmune disease, primarily affecting secretory gland tissue. Lung Disease in Sjögren Syndrome relatively common, affecting 9–22% and confers major adverse effects on both life quality and mortality, resulting in a 4-fold increase in 10 years mortality [3, 4]. Lung involvement typically presents with exertional dyspnea and a persisting dry cough. Pulmonary symptoms can be the first manifestation of pSS. Nannini et al reported on 105 pSS patients, 10% of whom displayed respiratory manifestations at diagnosis or within the 1st year. At 5 years, prevalence had risen to 20% (+/– 4%) [8]. Dry cough was the predominating symptom, affecting 41–61% of patients, with higher than anticipated rates for respiratory infections and pneumonia at 10–35% [3, 9]
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