Practical approach to screening for scleroderma‐associated pulmonary arterial hypertension

Fischer, Aryeh; Bull, Todd; Steen, Virginia D.

doi:10.1002/acr.20693

Cited by 33 publications

(32 citation statements)

References 60 publications

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“…Treatment options may also vary according to the different types of rheumatic diseases. For instance, immunosuppressive treatment has been shown to be effective in SLE-associated PAH and the response to CCB is poor in rheumatic disease-associated with PAH particularly in SSc (19,27). The response to CCB is poorly defined in other rheumatic diseases.…”

Section: Discussionmentioning

confidence: 99%

“…Evaluation of the patients using additional parameters that had been shown to be useful in the diagnosis, prognosis, and/or follow-up of PAH such as serum brain natriuretic peptide levels (BNP), diffusing capacity of carbon monoxide (DLCO), and six minute walk distance before RHC may improve the diagnostic approach (16)(17)(18). Currently, new algorithms for the diagnosis of PH that include components such as patient's clinical features, BNP levels, and DLCO have been suggested (19)(20)(21)(22). DETECT (DETECTion of PAH in SSc) algorithm in SSc patients with DLCO<60% and disease duration>3 years may be useful for early diagnosis (22).…”

Section: Discussionmentioning

confidence: 99%

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Is early diagnosis of pulmonary arterial hypertension possible in inflammatory rheumatic diseases? Experience from a single center in Turkey

et al. 2015

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Objective: Pulmonary arterial hypertension (PAH) is a devastating complication of inflammatory rheumatic diseases. The aim of this study was to determine the role of screening for the early diagnosis of pulmonary hypertension (PH) in inflammatory rheumatic diseases.Material and Methods: Data of patients with inflammatory rheumatic diseases and PH who had no obvious cause of PH and who were evaluated by Working Group for Pulmonary Hypertension in Hacettepe University were investigated retrospectively. All patients with inflammatory disease were evaluated by right heart catheterization (RHC) to check if they had systolic pulmonary arterial pressure (sPAP) ≥40 mmHg and/or symptoms related to PH unless explained by other causes.Results: RHC was performed in 47 patients with inflammatory rheumatic diseases and PH out of 50 patients who were to be evaluated by RHC based on clinical and Doppler echocardiographic findings. There was a positive correlation between sPAP estimated by Doppler echocardiography and sPAP determined by RHC in patients with inflammatory rheumatic diseases (r=0.66; p<0.001). The mean pulmonary arterial pressure (mPAP) was found to be <25 mmHg in 27.7% of the patients. New York Heart Association functional capacity (NYHA FC) was class III or IV in 79.0% of the patients with PAH. PAH was more frequent in patients with NYHA FC III-IV compared with patients with NYHA FC I-II [58.7% (15) patients vs. 19.0% (4) patients; p=0.009]. Conclusion:In this study, approximately 80% of the patients with inflammatory disease-associated PAH were diagnosed late in NYHA FC III or IV. There are still unresolved issues in the diagnosis and treatment of PH in inflammatory diseases. Collaboration and multidisciplinary approach are the key points to overcome the challenges in this field.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Is early diagnosis of pulmonary arterial hypertension possible in inflammatory rheumatic diseases? Experience from a single center in Turkey

et al. 2015

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“…53 In patients with advanced PAH, a typical electrocardiographic finding is right axis deviation of the QRS complex, which reflects right atrial dilatation and right ventricular hypertrophy. 50 One observational study of 36 patients with SSc found an abnormal 24-hour Holter monitoring study in more than half of the patients (predominantly ventricular ectopy and monomorphic tachycardias), and these patients were more likely to have PH by echocardiogram. 54,55 This finding has not been confirmed in larger studies.…”

Section: Valenzuela Et Almentioning

confidence: 98%

“…52 The 6-minute walk distance (6MWD) is not specific or sensitive for PAH in patients with SSc because it can also be abnormal because of ILD, anemia, arthritis, or muscle disease. 50 However, the 6MWD correlates with hemodynamics and is useful for monitoring progression of disease, with a minimal clinically important difference, defined as a change of 33 m in a cohort of patients with PAH (24% CTD-PAH). 53 In patients with advanced PAH, a typical electrocardiographic finding is right axis deviation of the QRS complex, which reflects right atrial dilatation and right ventricular hypertrophy.…”

Section: Valenzuela Et Almentioning

confidence: 99%

“…A pulmonary artery diameter greater than 30 mm, right heart enlargement, or pericardial effusion/thickening are HRCT features that suggest PAH and should prompt more precise PAH assessments. 50 In addition, HRCT is the gold standard to diagnose and determine the extent of ILD or pulmonary fibrosis because many patients with PAH also have some underlying ILD. The HRCT can be helpful in differentiating World Health Organization (WHO) group 1 from group 3 PH in that the latter group by definition has severe pulmonary fibrosis.…”

Section: Valenzuela Et Almentioning

confidence: 99%

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Monitoring and Diagnostic Approaches for Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis

Valenzuela

Nandagopal

Steen

et al. 2015

Rheumatic Disease Clinics of North America

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Survival and Predictors of Mortality in Systemic Sclerosis‐Associated Pulmonary Arterial Hypertension: Outcomes From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry

Chung

Domsic

Lingala

et al. 2014

Arthritis Care & Research

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Objective. To assess cumulative survival rates and identify independent predictors of mortality in patients with incident systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) who had undergone routine screening for PAH at SSc centers in the US. 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.

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Practical approach to screening for scleroderma‐associated pulmonary arterial hypertension

Cited by 33 publications

References 60 publications

Is early diagnosis of pulmonary arterial hypertension possible in inflammatory rheumatic diseases? Experience from a single center in Turkey

Is early diagnosis of pulmonary arterial hypertension possible in inflammatory rheumatic diseases? Experience from a single center in Turkey

Monitoring and Diagnostic Approaches for Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis

Survival and Predictors of Mortality in Systemic Sclerosis‐Associated Pulmonary Arterial Hypertension: Outcomes From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry

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