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Combined Pulmonary Fibrosis and Emphysema Among Patients With Scleroderma-Associated Pulmonary Hypertension FREE TO VIEW

Majid Shafiq, MD; Paul Hassoun, MD; Stephen Mathai, MD
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Johns Hopkins Hospital, Baltimore, MD

Chest. 2013;144(4_MeetingAbstracts):866A. doi:10.1378/chest.1703893
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SESSION TITLE: DVT/PE/Pulmonary Hypertension Posters

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 30, 2013 at 01:30 PM - 02:30 PM

PURPOSE: Scleroderma (SSc) is often associated with pulmonary hypertension (PH), with or without interstitial lung disease (ILD). A distinct and under-recognized syndrome of combined pulmonary fibrosis and emphysema (CPFE) has been described among patients with connective tissue diseases. However, little is known about CPFE in SSc patients with PH. We sought to assess the prevalence and clinical characteristics of CPFE in our population of SSc-PH patients.

METHODS: Consecutive patients with SSc and PH documented by right heart catheterization (RHC) identified from our clinical database were retrospectively reviewed, excluding patients with PH related to left heart disease, chronic thrombo-emboli or renal disease. Demographic, hemodynamic, computed tomographic (CT) imaging and pulmonary function tests (PFTs) of those with CPFE were recorded and compared to those without CPFE.

RESULTS: We reviewed 114 patients with SSc-associated PH, out of which 81% were female and 95% were White. Mean age at the time of PH diagnosis was 59±13 years. Based upon CT imaging, 27% of the cohort had CPFE. These patients were likely to be older (65±11 years vs. 57±11 years, p<0.01) and more likely to be female. Hemodynamics revealed moderate-to-severe pulmonary hypertension (mean PAP 40±11 mmHg, CO 4.5±1.5 L/min, PVR 7.1±3.9 Wood units); there were no differences between groups except for PA saturation (68±7% vs. 61±11%, p<0.01, non-CPFE vs. CPFE). Interestingly, PFTs demonstrated no significant differences in spirometry, lung volumes or diffusing capacity between groups.

CONCLUSIONS: A considerable proportion of patients with scleroderma-associated pulmonary hypertension have CPFE. Patients with CPFE are likely to be older and more likely to be female. At presentation, there appears to be no difference in the severity of PH or in pulmonary function between SSc-PH patients with and without CPFE.

CLINICAL IMPLICATIONS: A diagnosis of CPFE should be actively sought among SSc-PH patients, especially among certain demographic groups. The impact of CPFE on outcomes in SSc-PH remains to be determined and would be the focus of future research.

DISCLOSURE: The following authors have nothing to disclose: Majid Shafiq, Paul Hassoun, Stephen Mathai

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