Strek, Mary E.

Interstitial lung disease (ILD) is a frequent manifestation of rheumatoid arthritis (RA) that is associated with high mortality. RA‐ILD may initially be asymptomatic, and lung function may be markedly impaired by the time it is diagnosed. The course of RA‐ILD is highly variable, with some patients experiencing no discernable progression or a slow decline, whereas others experience more rapid deterioration. Some patients develop progressive pulmonary fibrosis, which is associated with high mortality. Although risk factors for the progression of RA‐ILD have been identified, including older age, worse lung function, and a usual interstitial pneumonia pattern on high‐resolution computed tomography, it is not possible to predict the course of RA‐ILD in an individual patient. The association between RA disease activity and progression of RA‐ILD remains unclear. Regular monitoring is important to enable the prompt identification of progression and early intervention to preserve lung function. The management of RA‐ILD requires a multidisciplinary and individualized approach, taking account of the severity and progression of articular and lung disease, risk factors for the progression of RA‐ILD, and the patient's preferences, and may include immunosuppression, antifibrotic therapy, and supportive care.

A usual interstitial pneumonia (UIP) imaging pattern can be seen in both idiopathic pulmonary fibrosis (IPF) and connective tissue disease-related interstitial lung disease (CTD-ILD). The purpose of this multicenter study was to assess whether quantitative imaging data differ between IPF and CTD-ILD in the setting of UIP. Patients evaluated at two medical centers with CTD-ILD or IPF and a UIP pattern on CT or pathology served as derivation and validation cohorts. Chest CT data were quantitatively analyzed including total volumes of honeycombing, reticulation, ground-glass opacity, normal lung, and vessel related structures (VRS). VRS was compared with forced vital capacity percent predicted (FVC%) and percent predicted diffusing capacity of the lungs for carbon monoxide (DLCO%). There were 296 subjects in total, with 40 CTD-ILD and 85 IPF subjects in the derivation cohort, and 62 CTD-ILD and 109 IPF subjects in the validation cohort. VRS was greater in IPF across the cohorts on univariate (p < 0.001) and multivariable (p < 0.001–0.047) analyses. VRS was inversely correlated with DLCO% in both cohorts on univariate (p < 0.001) and in the derivation cohort on multivariable analysis (p = 0.003) but not FVC%. Total volume of normal lung was associated with DLCO% (p < 0.001) and FVC% (p < 0.001–0.009) on multivariable analysis in both cohorts. VRS appears to have promise in differentiating CTD-ILD from IPF. The underlying pathophysiological relationship between VRS and ILD is complex and is likely not explained solely by lung fibrosis.

This study aimed to determine diagnostic and prognostic differences in major forms of interstitial lung disease using quantitative CT imaging. A retrospective study of 225 subjects with a multidisciplinary diagnosis of idiopathic pulmonary fibrosis (IPF), interstitial pneumonia with autoimmune features (IPAF), connective tissue disease (CTD), or chronic hypersensitivity pneumonitis (cHP) was conducted. Non-contrast CT scans were analyzed using the Computer Aided Lung Informatics for Pathology Evaluation and Rating (CALIPER) program. Resulting data were analyzed statistically using ANOVA and Student’s t-test. Univariate, multivariable, and receiver operating characteristic analyses were conducted on patient mortality data. CALIPER analysis of axial distribution on CT scans in those with IPF demonstrated greater peripheral volumes of reticulation than either CTD (p = 0.033) or cHP (p = 0.007). CTD showed lower peripheral ground-glass opacity than IPF (p = 0.005) and IPAF (p = 0.004). Statistical analysis of zonal distributions revealed reduced lower zone ground-glass opacity in cHP than IPF (p = 0.044) or IPAF (p = 0.018). Analysis of pulmonary vascular-related structure (VRS) volume by diagnosis indicated greater VRS volume in IPF compared to CTD (p = 0.003) and cHP (p = 0.003) as well as in IPAF compared to CTD (p = 0.007) and cHP (p = 0.007). Increased reticulation (p = 0.043) and ground glass opacity (p = 0.032) were predictive of mortality on univariate analysis. Increased pulmonary VRS volume was predictive of mortality (p < 0.001) even after multivariate analysis (p = 0.041). Quantitative CT imaging revealed significant differences between ILD diagnoses in specific CT findings in axial and, to a lesser degree, zonal distributions. Increased pulmonary VRS volume seems to be associated with both diagnosis and survival.