Real World Profile, Management and Outcomes of Patients Seen at a Joint Interstitial Lung Clinic

I ntroduction: Interstitial lung disease (ILD) is associated with significant morbidity and mortality.Effective therapeutic intervention to halt or reverse its progression is needed.Systemic autoimmune rheumatic diseases (SARDs) are associated with ILD, and it has been shown that therapies developed initially for SARDs may also impact favourably on ILD.This has led to the growth of joint ILD clinics, frequently run by chest physicians in tandem with rheumatologists.However, little data are available on the profile, treatment and outcome of patients treated in these clinics across the UK.Methods: We established a clinic for patients with ILD and a diagnosis of proven or suspected SARDs in 2023.We collected data from each patient comprising age, sex, SARD diagnosis, ILD subtype on high-resolution computed tomography (HRCT), baseline pulmonary function tests (PFTs), autoantibody profile, smoking history, therapeutic intervention, changes in both HRCT and PFTs over time and survival.We analysed these for the first 130 patients attending our clinic to produce information on source and appropriateness of referral, demographics, therapeutic approaches used, outcomes and predictors of death or deterioration across the whole cohort.Results: Of the 130 patients referred to the clinic in the first 18 months, only 110 had definite ILD on HRCT.Their mean (standard deviation [SD]) age was 68.4 (11.7) years at referral, and 56 (50.9%) patients were female.Seventy-one patients met the criteria for a definite SARD, of which 41 were patients with rheumatoid arthritis (RA).Twelve additional patients had biopsy-proven sarcoidosis.Of the remaining 27 patients, 12 had autoantibodies associated with SARDs.In the study, 12 (11%) patients died during a median follow-up period of 12 months, with five of them from respiratory causes.Across the 110 patients with ILD, the most prescribed therapeutic agents included mycophenolate (53%), rituximab (35%) and methotrexate (17%), usually with bridging steroids.Fewer patients were treated with cyclophosphamide (10%), anti-tumour necrosis factor (TNF) therapy (10%) or azathioprine (4%), and these were often later switched to another therapy.Anti-fibrotics were given to just 11%.Death or deterioration in both lung function and HRCT appearances occurred in 21 patients who had a male:female ratio of 2:1 and a mean (SD) age of 71.3 (11.4) years.Predictors for death or deterioration included male sex (relative risk [RR] 1.89), the absence of autoantibodies (1.60), a usual interstitial pneumonia (UIP) pattern on HRCT (1.59), baseline reduction in gas transfer (1.43) and a non-SARD-ILD or sarcoid diagnosis (RR 1.31).Discussion: We describe a real-world experience of a joint ILD clinic.Our results suggest that therapeutic intervention can be effective in patients with SARD-ILD, but that a meaningful response can be achieved in patients with a similar autoantibody profile, even in the absence of clinical evidence of SARD.Patients with sarcoidosis also responded well to intervention.Sex, antibody status, HRCT pattern, baseline gas transfer and diagnosis influenced the outcome of the study.Longer follow-up and a larger database are essential to clarify and confirm these findings.