Background Pulmonary hypertension (PH) complicating systemic sclerosis (SSc)-related interstitial lung disease (ILD) is usually associated with a poor prognosis. However, data are either lacking or ...scarce on prognostic factors in this condition. The objectives of this study were to compare the survival of patients with ILD-associated PH (PH-ILD) or pulmonary arterial hypertension (PAH) and to determine whether the severity of PH has prognostic value in SSc-associated PH-ILD. Methods Consecutive patients with SSc and PH-ILD (n = 47) or PAH (n = 50) confirmed by right-sided heart catheterization were included in a cross-sectional analysis. PH was classified as mild (mean pulmonary arterial pressure mPAP ≤ 35 mm Hg) or moderate to severe (mPAP > 35 mm Hg). Results As compared with patients with PAH, subjects with PH-ILD were younger, were more frequently men with a history of smoking, had more frequently diffuse SSc, less frequently anticentromere antibodies, and a lower FVC/diffusing capacity of lung for carbon monoxide (D lco) ratio. They had a worse prognosis than patients with PAH (3-year survival of 47% vs 71%, respectively; P = .07). Patients with mild PH-ILD had similar poor outcomes when compared with those with moderate to severe PH-ILD. Pericardial effusion (hazard ratio HR, 2.44; P = .04) and lower D lco (HR, 0.96; P = .01) were the only independent factors predictive of a poor survival in the PH-ILD group. Conclusions Patients with SSc with PH-ILD had a different phenotype and a worse prognosis than those with SSc and PAH. Lower D lco and presence of pericardial effusion were predictive of a poor outcome in PH-ILD, whereas mPAP seemed to have no prognostic significance.
Abstract Objectives Ischemic digital ulcers (DU) represent a major complication of systemic sclerosis (SSc). We investigated the impact of controlling the ulcerative disease on disability, pain, and ...quality of life in SSc patients receiving bosentan. Methods ECLIPSE (Study AC-052-517) is a 2-year prospective, multicenter, and observational study. Patients with SSc who experienced at least 1 DU in the previous year and received bosentan were included between October 2009 and March 2011. Disability scores Cochin Hand Function Scale (CHFS) and Health Assessment Questionnaire Disability Index (HAQ-DI), pain scores (visual analog scale), and quality-of-life scores (SF-36) were collected at inclusion and 1 year later (primary endpoint). A controlled ulcerative disease was defined by the absence of ongoing/new DU episode between inclusion and 1-year follow-up. Results Data were available at 1 year for 120 patients out of 190 included. During follow-up, 46 (38.3%) patients experienced a new DU episode. The number of DU per patient decreased from 1.4 ± 1.8 at inclusion to 0.6 ± 1.6 ( p < 0.0001) at 1 year. Disability scores decreased from 1.0 ± 0.7 to 0.9 ± 0.7 ( p = 0.04) for the HAQ-DI and from 29 ± 20 to 25 ± 20 ( p = 0.005) for the CHFS; the pain score decreased from 4.3 ± 3.1 to 2.9 ± 2.8 ( p < 0.0001). This improvement was attributed to patients with a controlled ulcerative disease (48.3%), who significantly improved HAQ-DI ( p = 0.04), CHFS ( p = 0.04), and pain score ( p = 0.046). Conclusions In patients with SSc, control of the ulcerative disease for 1 year was associated with significant attenuation of hand disability.
Abstract Background Objective: The objective of this study was to modify the EVAPIL, a questionnaire designed to assess user-reported tolerability of combined oral contraceptives (COCs) in ...cross-sectional studies, to make it appropriate for assessing the tolerability of intrauterine systems (IUSs), subdermal implants, and COCs in longitudinal studies. Methods Development of the EVAPIL-Revised (EVAPIL-R) was informed by a targeted literature review, qualitative interviews with IUS-prescribing gynecologists (n = 5), and IUS and implant users in the United States, Germany, and France (n = 36). Results Evidence generated supports the content validity of the EVAPIL-R for assessing user-reported tolerability of COCs, IUSs, and implants. Modifications to improve the relevance and usability of the questionnaire in longitudinal studies included specification of a defined recall period, addition of separate assessments of frequency and intensity (where relevant), and inclusion of additional items measuring concepts of importance to IUS and implant users (eg, vaginal discharge). Conclusions The EVAPIL-R is a valuable tool for use in research and clinical practice to identify tolerability concerns in hormonal contraceptive users. Future research will evaluate the psychometric validity and responsiveness of the EVAPIL-R. Understanding of user-reported tolerability of contraceptive methods is critical for facilitating patient adherence and potentially reducing the number of unintended pregnancies. The EVAPIL-R may be used to facilitate “women-centered” research and contraceptive counseling and provision.
