Background/Purpose: Interstitial lung disease is a major cause of morbidity and mortality in patients with connective tissue disease (CTD-ILD). Evaluation of ILD severity and progression relies on symptoms along with pulmonary function tests (PFT) and high-resolution computed tomography (HRCT). It has been previously reported that Ro52 antibodies are associated with more severe ILD phenotype. The goal of this study is to evaluate PFT characteristics across CTD-ILDs and whether anti-Ro52 presence determines more severe ILD in scleroderma, Sjogren’s, idiopathic inflammatory myopathy (IIM), and Mixed Connective Tissue Disease (MCTD) subgroups.

Methods: This is a retrospective analysis of the Northwell CTD-ILD cohort, meeting classification criteria for scleroderma, IIM, Sjogren’s, or MCTD. Cases with rheumatoid arthritis ILD and post-transplant were excluded. We combined 22 previously reported cases of IIM-ILD with 101 CTD-ILD patients followed between 2012 – 2024. Longitudinal analysis of PFTs was performed over a 2-year period. HRCT chest were recorded as improved, stable or worsened by radiologist read. Descriptive statistics, Anova (with Geisser-Greenhouse correction, Welch’s ANOVA and repeated measures ANOVA) and Mann-Whitney (non-parametric) tests were used.

Results: 123 patients were included in the analysis. Our cohort comprised of 45 IIM, 30 scleroderma, 21 Sjogren’s, and 27 MCTD patients. The median age was 58 (22-83) years with a predominantly females (80.5%) with ~5 years follow-up (1-12 years), Table 1. A quarter of patients (26.8%) were Ro-52+ with most distributed between Sjogren’s (47.6%) and IIM (42.2%) subgroups. NSIP was the most common ILD pattern. Most patients in our cohort were on at least 1 immunosuppressive agent, led by mycophenolate mofetil (Cohort: 58.5%; Scleroderma 76.7%, IIM 53.3%, Sjogren’s 38.1%, and MCTD 63%). At baseline, mean FVC % (p=0.67) and DLCO% (p=0.42) were similar across subgroups, Fig1a,c and Ro-52+ patients (all cohort and IIM subgroup) had significantly lower DLCO% compared to Ro52- patients (Cohort: 46.3 vs. 54.8, p=0.02; IIM 43.1 vs. 56.4, p=0.01), while this was not observed for any other subgroups of ILD, Fig 2a,b. When we looked at change in disease over time in individual CTD-ILD subgroups, a trend for improvement in both FVC% and DLCO% was noted only in the IIM subgroup (FVC% p=0.05 with baseline to year 1 p=0.02 and to year 2 p=0.04; DLCO% p=0.14 with baseline to year 1 p=0.09 and to year 2 p=0.4, data not shown). When the ILD subgroups were compared to each other, there was no significant change in FVC% or DLCO% over the 2-year period, Fig1b,d. Similarly, there was no significant change in FVC% or DLCO% between Ro-52+ and negative groups over 2 years (p=0.889). However, Ro52 negative patients consistently maintained higher DLCO% over time (p=0.003) Fig 2c,d. Most patients had stable HRCT over 2-year period in all subgroups.

Conclusion: While all CTD-ILD share similar evolution when compared to each other, myositis-ILD has a higher chance of improvement by PFT parameters. Anti-Ro52 positivity is associated with consistently lower DLCO% over time and may serve as harbinger of more severe ILD across all CTD-ILD subgroups.

Patient Demographics and Characteristics

Baseline and Longitudinal Differences in FVC and DLCO Between CTD-ILD Subgroups

Baseline and Longitudinal Differences in FVC and DLCO Between Ro52+ and Ro52- Subgroups

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/baseline-and-2-year-follow-up-of-pulmonary-function-among-ctd-ild-subtypes/