Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients

Koh, E.; Lee, P.; Gladman, Dafna D.; Abu‐Shakra, Mahmoud

doi:10.1093/rheumatology/35.10.989

Cited by 283 publications

(213 citation statements)

References 27 publications

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“…These differences may reflect varying approaches to screening, referral criteria and diagnostic strategies. Survival in IPAH, PAH-SSc and CTEPH was superior to that described in historical series [42][43][44][45] and age at diagnosis in IPAH has increased from 34 yrs in the National Institutes of Health (Bethesda, MD, USA) registry to 55¡16 yrs in our cohort, in keeping with other observations [12,29,30,42]. Notably, the proportion of patients with PAH associated with HIV was lower than observed elsewhere [29,30], which may be related to a lower prevalence of HIV in the UK [46] and differing epidemiological factors in HIV acquisition.…”

Section: Discussionmentioning

confidence: 81%

ASPIRE registry: Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre

Hurdman¹,

Condliffe²,

Elliot³

et al. 2011

Eur Respir J

374

362

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Pulmonary hypertension (PH) is a heterogeneous condition. To date, no registry data exists reflecting the spectrum of disease across the five diagnostic groups encountered in a specialist referral centre.Data was retrieved for consecutive, treatment-naïve cases diagnosed between 2001 and 2010 using a catheter-based approach. 1,344 patients were enrolled, with a mean follow-up of 2.9 yrs.The 3-yr survival was 68% for pulmonary arterial hypertension (PAH), 73% for PH associated with left heart disease, 44% for PH associated with lung disease (PH-lung), 71% for chronic thromboembolic PH (CTEPH) and 59% for miscellaneous PH. Compared with PAH, survival was inferior in PH-lung and superior in CTEPH (p,0.05). Multivariate analysis demonstrated that diagnostic group independently predicted survival. Within PAH, Eisenmenger's survival was superior to idiopathic PAH, which was superior to PAH associated with systemic sclerosis (p,0.005). Within PH-lung, 3-yr survival in sleep disorders/alveolar hypoventilation (90%) was superior to PH-lung with chronic obstructive pulmonary disease (41%) and interstitial lung disease (16%) (p,0.05). In CTEPH, long-term survival was best in patients with surgically accessible disease undergoing pulmonary endarterectomy.In this large registry of consecutive, treatment-naïve patients identified at a specialist PH centre, outcomes and characteristics differed between and within PH groups. The current system of classification of PH has prognostic value even when adjusted for age and disease severity, emphasising the importance of systematic evaluation and precise classification.

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Section: Discussionmentioning

confidence: 81%

ASPIRE registry: Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre

Hurdman¹,

Condliffe²,

Elliot³

et al. 2011

Eur Respir J

374

362

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“…The prevalence of resting PAH in CTD is ϳ2.3-10 cases per million (5), typically mostly SSc patients (5-50%) (6)(7)(8)(9)(10)(11)(12), patients with mixed CTD (21-29%) (13), and patients with systemic lupus erythematosus (5-43%) (14)(15)(16). Based on right heart catheterization, the prevalence of resting PAH in SSc is likely 7.9-20% (7,17).…”

Section: Methodsmentioning

confidence: 99%

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

Saggar

Khanna

Fürst

et al. 2010

Arthritis & Rheumatism

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Objective. Exercise-induced pulmonary hypertension (PH) may represent an early but clinically relevant phase in the spectrum of pulmonary vascular disease. There are limited data on the prevalence of exerciseinduced PH determined by right heart catheterization in scleroderma spectrum disorders. We undertook this study to describe the hemodynamic response to exercise in a homogeneous population of patients with scleroderma spectrum disorders at risk of developing pulmonary vascular disease. Methods.Patients with normal resting hemodynamics underwent supine lower extremity exercise testing. A classification and regression tree (CART) analysis was used to assess combinations of variables collected during resting right heart catheterization that best predicted abnormal exercise physiology, applicable to each individual subject.Results. Fifty-seven patients who had normal resting hemodynamics underwent subsequent exercise right heart catheterization. Four distinct hemodynamic groups were identified during exercise: a normal group, an exercise-induced pulmonary venous hypertension (ePVH) group, an exercise out of proportion PH (eoPH) group, and an exercise-induced PH (ePH) group. The eoPH and ePVH groups had higher pulmonary capillary wedge pressure (PCWP) than the ePH group (P < 0.05). The normal and ePH groups had exercise PCWP <18 mm Hg, which was lower than that in the ePVH and eoPH groups (P < 0.05). During submaximal exercise, the transpulmonary gradient and pulmonary vascular resistance (PVR) were elevated in the ePH and eoPH groups as compared with the normal and ePVH groups (P < 0.05). CART analysis suggested that resting mean pulmonary artery pressure (mPAP) >14 mm Hg and PVR >160 dynes/seconds/cm ؊5 were associated with eoPH and ePH (positive predictive value 89% for mPAP 14-20 mm Hg and 100% for mPAP >20 mm Hg).Conclusion. We characterized the exercise hemodynamic response in at-risk patients with scleroderma spectrum disorders who did not have resting PH. Four distinct hemodynamic groups were identified during exercise. These groups may have potentially different prognoses and treatment options.

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“…Inability to evaluate PASP due to the lack of tricuspid regurgitation and/or due to insufficient quality of the images obtained was reported in several studies - Table 5 In summary, inability to evaluate PASP by echo-Doppler was shown in several studies including different populations of SSc patients. All these studies evaluated heterogeneous populations of patients with and without PAH/PH or, if including separate analyses of PAH/PH patients, were of quality levels below A1 (highest quality level A2) [34,38]. Thus, until studies on the ability of echo-Doppler to evaluate PASP exclusively in PAH-SSc (level A1) are available, this aspect of feasibility was judged "unclear".…”

Section: Feasibilitymentioning

confidence: 99%

Echocardiography as an Outcome Measure in Scleroderma-related Pulmonary Arterial Hypertension: A Systematic Literature Analysis by the EPOSS Group

Kowal-Bielecka¹,

Avouac²,

Pittrow³

et al. 2009

J Rheumatol

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Lewis J Rubin has received consulting income from Actelion and Encysive; and he has served as an investigator for Actelion and Encysive. James R. Seibold, has consultancy relationships and research funding from Actelion, Pfizer, Encysive, FibroGen, Centocor, Bristol-Myers Squibb, Genzyme, Lilly, Gilead and United Therapeutics in the area of potential treatments of scleroderma and its complications. He has received lecture honoraria from Actelion, Pfizer, Encysive and United Therapeutics. His spouse is a full-time employee of Actelion Vibeke Strand has consultancy relationships with Pfizer, FibroGen, and Bristol-Myers Squibb in the area of potential treatments of scleroderma and its complications Daniel E Furst has consultancy relationship, has been a member of scientific advisory board, and/or has received research funding from Abbott, Actelion, Amgen, BMS, BiogenIdec, Centocor, Genentech, Gilead, GSK, Merck, Nitec, Novartis, Roche, UCB, Wyeth and Xoma. He has received lecture honoraria and/or lectured on behalf of Abbott, Actelion, Amgen, BMS, Biogen, Biogenidec, Centocor, Genentech, Gilead, Merck, Nitec and UCB. Oliver Distler has consultancy relationships and/or has received research funding from Actelion, Pfizer, Encysive, FibroGen, Ergonex, NicOx, and Biovitrum in the area of potential treatments of scleroderma and its complications. He has received lecture honoraria from Actelion, Pfizer, Encysive and Ergonex.This study was partially supported by unrestricted educational grants from Actelion,

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Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients

Cited by 283 publications

References 27 publications

ASPIRE registry: Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre

ASPIRE registry: Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre

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

Echocardiography as an Outcome Measure in Scleroderma-related Pulmonary Arterial Hypertension: A Systematic Literature Analysis by the EPOSS Group

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