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.

Imaging Innovations in the Screening, Diagnosis, and Monitoring of Systemic Autoimmune Disease-Related Interstitial Lung Disease

“…antibodies is considered high risk, and should necessitate screening irrespective of pulmonary symptoms. 21,22 In other SARDs, namely pSS, anti-neutrophil cytoplasmic antibody-associated vasculitis, and SLE, screening for ILD is based upon patient symptomatology.…”

Screening for rheumatoid arthritis-associated interstitial lung disease—a Delphi-based consensus statement

“…While there are recognized risk factors for the development of ILD among RA patients, an international recommendation for screening is still lacking. Some national attempts on this topic have been published [ 13 , 14 ]. To date, however, there is no European Alliance of Associations for Rheumatology (EULAR) recommendation concerning screening for ILD in RA patients.…”