Pulmonary arterial hypertension screening practices in scleroderma patients among Canadian rheumatologists (original) (raw)
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Screening for Pulmonary Hypertension in Systemic Sclerosis—A Primer for Cardio-Rheumatology Clinics
Diagnostics, 2021
Systemic sclerosis (SSc) is a rare disease, with unfavorable clinical course and prognosis, characterized by progressive multisystemic involvement. SSc associated pulmonary hypertension (SSc-PAH) and interstitial lung disease (ILD) are the most important factors for morbi-mortality in these patients, being responsible for more than 60% of total deaths. Though pulmonary arterial hypertension (PAH) is the dominant subtype seen in SSc, PH secondary to ILD, left-heart pathology, and pulmonary veno-occlusive disease (PVOD) are also possible occurrences. Initial evaluation of a SSc case is complex and should be performed with a multidisciplinary approach. Early detection of SSc-PAH is imperative, given the fact that new and effective medications are available and early treatment was shown to improve outcomes. Therefore, screening algorithms must be used adequately and in a cost-effective manner. Sensitivity and negative predictive value (NPV) are the most important performance measures in...
Clinical and experimental rheumatology
Pulmonary arterial hypertension in patients with systemic sclerosis is a disease involving multiple organ systems. We investigated the differences in perceptions of how to measure PAH-SSc among cardiologists, pulmonologists and rheumatologists. We also examined how a Delphi exercise can improve agreement among these subspecialties. The outcome measures derived from the recent Delphi survey were used for a detailed analysis of the contribution of the various specialties contributing to it. We compared rheumatologists and cardiologist/pulmonologists with regards to preferences and ratings of various endpoints and the actual use of tools to measure these outcomes. We also examined the effects of the Delphi process among these groups. We could show that the different expert groups each tended to contribute differently to the development of the core set of measures and that interactions in the Delphi process resulted in convergence of rankings. Despite agreement on the high importance of...
The Journal of rheumatology, 2005
To estimate the prevalence of elevated pulmonary arterial pressures (PAP) as a correlate for pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc) in rheumatology centers in Canada. During the one-year study period (June 2002-May 2003), charts of patients with SSc were reviewed to determine demographics, SSc characteristics, percentage of patients with a PAH diagnosis, and the site criteria for such diagnosis. Subjects with no PAH that had symptoms of dyspnea and/or fatigue were invited to undergo Doppler echocardiography to estimate their systolic PAP (sPAP). A total of 539 patients with SSc (age 56 yrs +/- 13 SD, 84% female, 41% with diffuse SSc, 58% limited SSc, SSc disease duration 9 yrs +/- 7 SD) at 8 rheumatology centers were reviewed. Twenty-three percent of patients were diagnosed with elevated sPAP based on the site diagnosis criteria (i.e., > 30 mm Hg or > 35 mm Hg). From the non-PAH, not recently screened patients that had symptoms of dyspn...
CHEST Journal, 2012
Pulmonary arterial hypertension (PAH) causes substantial mortality and morbidity in systemic sclerosis (SSc). Current PAH screening recommendations result in a high false positive rate whilst the rate of missed diagnoses is unknown. DETECT aimed to develop an evidence-based screening algorithm for PAH in SSc that would limit the number of missed PAH diagnoses. METHODS: In this prospective, multicenter, cohort study [NCT00706082], adult patients with SSc for >3 years, a diffusing capacity of the lung for carbon monoxide (DLCO) <60% of predicted, and no previous diagnosis of pulmonary hypertension (PH) underwent multiple non-invasive screening tests followed by right heart catheterization (RHC). Univariable and multivariable logistic regression, followed by clinical expert input addressing clinical feasibility was used to select the best discriminatory variables for identifying PAH. These were incorporated into a 2-step screening algorithm. RESULTS: Of 466 SSc patients, 87 had RHC-confirmed PAH, 58 had other forms of PH and 321 had PH excluded. Six non-echocardiographic screening tests were used in step 1 of the algorithm to determine referral to echocardiography with a high sensitivity (97%) in order to minimize missed PAH
Arthritis & Rheumatism, 2011
Methods. Two incident cohorts of patients with SSc-PAH from the same management era (2002/2003) were studied. The first cohort (designated the routine practice cohort) included consecutive adult patients with symptomatic SSc in whom a diagnosis of PAH was made by right-sided heart catheterization (RHC) at the time of recruitment into the French PAH Registry. The second cohort (designated the detection cohort) comprised consecutive patients with SSc who entered a systematic PAH detection program and were subsequently found to have PAH on RHC. Clinical characteristics at diagnosis of PAH and subsequent 8-year mortality were compared between the cohorts.
European respiratory review : an official journal of the European Respiratory Society, 2011
Pulmonary arterial hypertension (PAH) is a relatively common complication of systemic sclerosis (SSc) affecting 5-12% of patients, and its development is associated with significant morbidity and a particularly poor prognosis. Deaths associated with other complications of SSc, such as renal crisis, have fallen significantly in recent years in line with improvements in their treatment and management. However, mortality due to PAH in this population, although improved, has shown a less dramatic decline. The early diagnosis of PAH in SSc would allow for earlier treatment, before functional and haemodynamic impairment becomes severe; this may further improve outcome, and evidence suggests that screening of SSc patients for PAH is associated with improved survival. In addition, patients with PAH associated with SSc are not a homogeneous population and they differ in terms of disease haemodynamic severity, functional capacity and rate of disease progression. Likewise, management strategie...
Clin Exp Rheumatol, 2014
The aim of this study was to utilise the Quality Enhancement Research Initiative in Systemic Sclerosis (QuERI-SSc) to measure and reduce a perceived gap in the diagnosis of pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc). Methods. Rheumatologists enrolled patients with SSc (aged ≥18 years) and provided data on a panel of diagnostic tests over 3 years. Pulmonary function testing, echocardiography, 6-minute walk distance, N-terminal pro-brain natriuretic peptide assays, high-resolution computed tomography of the lungs, and ventilation/perfusion scan plus right heart catheterisation (RHC; when appropriate) were emphasised. Exclusion criteria included previously documented PAH, interstitial lung disease, and SSc overlapping with other connective tissue disease. Results. Participating rheumatologists enrolled 207 patients with SSc (90% female; 80% white), with a median age of 57 years and median disease duration of 5 years. A total of 82% of patients were classified as New York Heart Association functional class I and II; of these patients, 177 had an echocardiogram at enrolment and 191 at any time during the study. Of those who met study-specified criteria for RHC at enrolment, only 3 of 7 patients underwent RHC. Conclusion. The screening algorithm was successful in identifying patients with mild impairment. Although specific tools were recommended for screening PAH in patients with SSc, results indicate that significant diagnostic care gaps still exist in the general rheumatology community. Better understanding and adherence to guidelines could improve the care and, ideally, outcomes of these high-risk patients.
Rheumatology, 2004
Objective. A prospective study to evaluate echocardiography and gas transfer (DLCO) by comparison with cardiac catheterization in discriminating between patients with and without systemic sclerosis-associated pulmonary arterial hypertension (SScPAH). Method. A total of 137 (52 with and 85 without pulmonary fibrosis) had echocardiography and lung function tests within 3 months of their definitive invasive study. Results. At cardiac catheterization 99 of these patients were found to have PAH, while PAH was excluded in 38. Echocardiographically estimated tricuspid gradient (TG) showed a moderate positive correlation (r 2 ¼ 0.44, P<0.005) with both mean pulmonary pressure and invasively determined tricuspid gradient. DLCO showed a weak correlation (r 2 ¼ 0.09, P ¼ 0.006), when compared with mean pulmonary arterial pressure. In total, 97% of patients with an echocardiographically determined TG of >45 mmHg were found to have pulmonary hypertension at catheterization. However, no threshold could be defined with either screening test that safely excluded PAH.
Arthritis Care & Research, 2014
Methods. The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma registry is a prospective registry of SSc patients at high risk for PAH or with definite pulmonary hypertension diagnosed by right-sided heart catheterization within 6 months of enrollment. Only patients with World Health Organization group I PAH (mean pulmonary artery pressure >25 mm Hg and pulmonary capillary wedge pressure <15 mm Hg without significant interstitial lung disease) were included in these analyses. Results. In total, 131 SSc patients with incident PAH were followed for a mean ؎ SD of 2.0 ؎ 1.4 years. The 1-, 2-, and 3-year cumulative survival rates were 93%, 88%, and 75%, respectively. On multivariate analysis, age >60 years (hazard ratio [HR] 3.0, 95% confidence interval [95% CI] 1.1-8.4), male sex (HR 3.9, 95% CI 1.1-13.9), functional class (FC) IV status (HR 6.5, 95% CI 1.8 -22.8), and diffusing capacity for carbon monoxide (DLCO) <39% predicted (HR 4.2, 95% CI 1.3-13.8) were significant predictors of mortality. Conclusion. This is the largest study describing survival in patients with incident SSc-associated PAH followed up at multiple SSc centers in the US who had undergone routine screening for PAH. The survival rates were better than those reported in other recently described SSc-associated PAH cohorts. Severely reduced DLCO and FC IV status at the time of PAH diagnosis portended a poor prognosis in these patients. Dr. Chung has received consulting fees, speaking fees, and/or honoraria (less than 10,000each)fromGileadandActelionandresearchsupportfromGilead,UnitedTherapeutics,andPfizer.Dr.Bolsterhasservedasanexpertwitnessontherelationshipbetweenpulmonaryarterialhypertensionandunderlyingautoimmunediseases.Dr.Fischerhasreceivedconsultingfees,speakingfees,and/orhonoraria(morethan10,000 each) from Gilead and Actelion and research support from Gilead, United Therapeutics, and Pfizer. Dr. Bolster has served as an expert witness on the relationship between pulmonary arterial hypertension and underlying autoimmune diseases. Dr. Fischer has received consulting fees, speaking fees, and/or honoraria (more than 10,000each)fromGileadandActelionandresearchsupportfromGilead,UnitedTherapeutics,andPfizer.Dr.Bolsterhasservedasanexpertwitnessontherelationshipbetweenpulmonaryarterialhypertensionandunderlyingautoimmunediseases.Dr.Fischerhasreceivedconsultingfees,speakingfees,and/orhonoraria(morethan10,000 each) from Gilead and Actelion. Dr. Furst has received consulting fees, speaking fees, and/or honoraria (less than 10,000each)fromGileadandActelion.Dr.Gomberg−Maitlandhasreceivedconsultingfees,speakingfees,and/orhonoraria(lessthan10,000 each) from Gilead and Actelion. Dr. Gomberg-Maitland has received consulting fees, speaking fees, and/or honoraria (less than 10,000each)fromGileadandActelion.Dr.Gomberg−Maitlandhasreceivedconsultingfees,speakingfees,and/orhonoraria(lessthan10,000 each) and research grants from Actelion, Gilead, Glaxo-SmithKline, Medtronic, Novartis, and United Therapeutics. Dr. Khanna has received consulting fees, speaking fees, and/or honoraria (less than 10,000each)fromActelion,Gilead,Genentech,Bristol−MyersSquibb,Bayer,Roche,Digna,andUnitedTherapeuticsandhasreceivedresearchfundingfromActelion,thePulmonaryHypertensionAssociation,theSclerodermaFoundation,andUnitedTherapeutics.Dr.Molitorhasreceivedconsultingfees,speakingfees,and/orhonoraria(lessthan10,000 each) from Actelion, Gilead, Genentech, Bristol-Myers Squibb, Bayer, Roche, Digna, and United Therapeutics and has received research funding from Actelion, the Pulmonary Hypertension Association, the Scleroderma Foundation, and United Therapeutics. Dr. Molitor has received consulting fees, speaking fees, and/or honoraria (less than 10,000each)fromActelion,Gilead,Genentech,Bristol−MyersSquibb,Bayer,Roche,Digna,andUnitedTherapeuticsandhasreceivedresearchfundingfromActelion,thePulmonaryHypertensionAssociation,theSclerodermaFoundation,andUnitedTherapeutics.Dr.Molitorhasreceivedconsultingfees,speakingfees,and/orhonoraria(lessthan10,000) from Actelion. Dr. Preston has received consulting fees, speaking fees, and/or honoraria (less than 10,000each)fromActelion,Bayer,Gilead,Novartis,andUnitedTherapeuticsandresearchgrantsfromActelion,Aires,Gilead,Novartis,andUnitedTherapeutics.Dr.Schiopuhasreceivedconsultingfees,speakingfees,and/orhonoraria(lessthan10,000 each) from Actelion, Bayer, Gilead, Novartis, and United Therapeutics and research grants from Actelion, Aires, Gilead, Novartis, and United Therapeutics. Dr. Schiopu has received consulting fees, speaking fees, and/or honoraria (less than 10,000each)fromActelion,Bayer,Gilead,Novartis,andUnitedTherapeuticsandresearchgrantsfromActelion,Aires,Gilead,Novartis,andUnitedTherapeutics.Dr.Schiopuhasreceivedconsultingfees,speakingfees,and/orhonoraria(lessthan10,000) from United Therapeutics. Dr. Simms has received consulting fees, speaking fees, and/or honoraria (less than 10,000each)fromActelionandGileadandhasreceivedresearchsupportfromGilead,Actelion,andUnitedTherapeutics.Dr.Steenhasreceivedconsultingfees,speakingfees,and/orhonoraria(lessthan10,000 each) from Actelion and Gilead and has received research support from Gilead, Actelion, and United Therapeutics. Dr. Steen has received consulting fees, speaking fees, and/or honoraria (less than 10,000each)fromActelionandGileadandhasreceivedresearchsupportfromGilead,Actelion,andUnitedTherapeutics.Dr.Steenhasreceivedconsultingfees,speakingfees,and/orhonoraria(lessthan10,000 each) from Actelion, Gilead, and United Therapeutics and has received research support from Actelion, Gilead, Pfizer, and United Therapeutics.