Significance of Pulmonary Arterial Pressure and Diffusion Capacity of the Lung as Prognosticator in Patients With Idiopathic Pulmonary Fibrosis

Incidence of Pulmonary Hypertension and Its Clinical Relevance in Patients with Interstitial Pneumonias: Comparison between Idiopathic and Collagen Vascular Disease Associated Interstitial Pneumonias

“…The finding of significant association of PH with DLCO in IIPs was consistent with the report of Nadrous et al (2), who showed that sPAP at rest was inversely correlated with DLCO and PaO2 in patients with IPF/UIP. In a previous study in our institution, it was also shown that the mean pulmonary artery pressure measured by right heart catheterization negatively correlated with %DLCO in patients with IPF/UIP (25). On the other hand, there was no significant correlation between %DLCO and sPAP in CVD-IPs, and some patients had higher levels of sPAP in CVD-IPs compared with IIPs (Fig.…”

A Novel Genomic Signature with Translational Significance for Human Idiopathic Pulmonary Fibrosis

“…In addition, the translational gene set was also able to ORIGINAL RESEARCH segregate control subjects from patients with IPF in two publicly available, independent clinical IPF/control datasets (GSE10667 and GSE24206), thereby supporting the findings ( Figure E17). Furthermore, in patients with IPF, the expression levels of the translational gene markers, GREM1, MMP7, CTHRC1, and FHL2, showed a significant negative correlation with percent diffusing capacity of carbon monoxide (%DL CO ) (GREM1, r = 20.68, adjusted P = 4.2e-25; MMP7, r = 20.64, adjusted P = 3.4e-22; CTHRC1, r = 20.64, adjusted P = 3.4e-22; and FHL2, r = 20.61, adjusted P = 1.7e-19), a parameter associated with disease severity (34,35). IPF samples obtained from lung transplantation (category 1, lung explant single; and category 2, lung explant bilateral) showed higher MMP7 expression and a lower %DL CO compared with IPF samples obtained from lung biopsies (category 3, lung lobectomy; or category 4, lung biopsy) ( Figure 5).…”

Exercise‐induced pulmonary hypertension associated with systemic sclerosis: Four distinct entities

“…Importantly, reanalysis after exclusion of these ILD patients (n ϭ 9) did not change the hemodynamic response to exercise in each of the 4 categories. Interestingly, IPF is a particularly well studied ILD, with a spectrum of pulmonary complications similar to those of SSc, and in fact, PAH complicates IPF in ϳ40% of patients during rest (40,46,47) and in 80% during exercise (40). We hypothesize that a pathophysiology similar to IPF exists within the scleroderma spectrum disorders, especially given the high prevalence of ILD in the latter; as such, earlier diagnosis and potential intervention may modify the natural history of this disease.…”