Delphi-Based Consensus on Interstitial Lung Disease Screening in Patients with Connective Tissue Diseases (Croatian National-Based Study)

Abstract

The aim of this study was to develop a Croatian Delphi-based expert consensus for screening interstitial lung disease (ILD) associated with connective tissue disease (CTD). A systematic literature review was conducted on risk factors for the development of ILD, prevalence and incidence of ILD, diagnostic and screening methods for ILD, and prognosis of ILD in idiopathic inflammatory myopathy (IIM), mixed connective tissue disease (MCTD), primary Sjögren's syndrome (pSS), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and systemic sclerosis (SSc) were performed. Based on the evidence found, experts developed questionnaires for screening and monitoring ILD in each CTD, which were provided via an online survey. Following the electronic survey, two screening algorithms were developed based on the consensus opinions. The detection strategy for ILD included high-resolution computed tomography (HRCT) in addition to pulmonary function testing for IIM, MCTD, and SSc. and pulmonary function testing for newly diagnosed pSS, RA and SLE. However, in patients with identified risk factors for ILD HRCT, these tests should also be performed. A screening strategy for early identification of patients with various CTD-ILD was first developed by a multidisciplinary team of rheumatologists, pulmonologists, and radiologists to identify early CTD patients at risk of ILD, a severe extra-articular manifestation of CTD.

Keywords: Sjögren’s syndrome; idiopathic inflammatory myopathy; interstitial lung disease; mixed connective tissue disease; national delphi consensus; rheumatoid arthritis; systemic lupus erythematosus; systemic sclerosis.

Figure 1

Screening for ILD in newly discovered MCTD, IIM, or SSc patients. Abbreviations: DLco, diffusion capacity for carbon monoxide; FVC, forced vital capacity; HRCT, high-resolution computed tomography; IIM, idiopathic inflammatory myopathy; ILD, interstitial lung disease; MCTD, mixed connective tissue disease; MDT; NSIP; SSc, systemic sclerosis. * Especially in patients with antisynthetase positive antibodies and anti-MDA5 in idiopathic inflammatory myopathy and anti-Scl-70/anti-topoisomerase I antibody in SSc. ** Clinical deterioration and/or decrease in FVC or DLco below 80% or by more than 10% compared to previous findings. ^† Most common symptoms include shortness of breath, exertional dyspnea, dry cough without phlegm, and weakness/extreme fatigue. Other symptoms include loss of appetite, unexplained weight loss, chest discomfort, dyspnea, tachypnea, and pulmonary hemorrhage.

Figure 2

Screening for ILD in newly discovered RA, pSS, or SLE patients. Abbreviations: DLco, diffusion capacity for carbon monoxide; FVC, forced vital capacity; ILD, interstitial lung disease; HRCT, high-resolution computed tomography; MDT; multidisciplinary team; pSS, primary Sjögren’s syndrome; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus. * In patients with identified risk factors include HRCT as screening methods. ** Clinical deterioration and/or decrease in FVC or DLco below 80% or by more than chest x compared to previous findings. ^† Most common symptoms include shortness of breath, exertional dyspnea, dry cough without phlegm, and weakness/extreme fatigue. Other symptoms include loss of appetite, unexplained weight loss, chest discomfort, dyspnea, tachypnea, and pulmonary hemorrhage.