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| Pulmonary Hypertension Diagnosis |
| This page was written by Shelley Ensz, and has not been medically edited. Systemic sclerosis (scleroderma) affects everyone differently. Just because something is listed here does not mean an individual patient will ever experience it. See Disclaimer |
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| Overview of Diagnosis of PH/PAH in Systemic Sclerosis (Scleroderma) |
| All scleroderma patients should be screened for PAH and PH on a regular basis. |
| Early, Simple and Reliable Detection of Pulmonary Arterial Hypertension (PAH) in Systemic Sclerosis (SSc) (DETECT). A two-stage prospective observational cohort study in scleroderma patients to evaluate screening tests and the incidence of pulmonary arterial hypertension and pulmonary hypertension. This study is currently recruiting participants. Verified by Actelion, October 2008. ClinicalTrials.gov. (Also see: Clinical Trials) |
| Assessment of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis: Comparison of Noninvasive Tests with Results of Right-Heart Catheterization (RHC). Echo appeared to be the most useful among the noninvasive tests. However, due to the low sensitivity of noninvasive testing, RHC should remain the gold standard. (PubMed) J Rheumatol. 2008 Jan 15. |
| “PH cannot be diagnosed without right heart catheterization.” |
How is PH/PAH Diagnosed? Pulmonary function tests can be helpful. Blood pressure in the lung can be estimated and the size and function of the right ventricle assessed by Doppler echocardiography. However, Doppler echocardiograms are notoriously inaccurate when the PH is mild or when there is simultaneous presence of lung scarring. PH cannot be diagnosed without right heart catheterization. James R. Seibold, MD, International Scleroderma Network Newsletter. 09-30-07. |
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| How is Pulmonary Arterial Hypertension diagnosed? Diagnosis can be delayed for months or even years while other possible causes are investigated and as a result, Pulmonary Arterial Hypertension (PAH) is frequently only identified when it is already quite advanced. Actelion Pharmaceuticals. |
| Dyspnea Due to Pulmonary Hypertension and Interstitial Lung Disease in Scleroderma: Room for Improvement in Diagnosis and Management. Rigorous assessment for pulmonary disease in patients with scleroderma, will reliably define the cause of dyspnea and exercise intolerance in the majority of patients. J Rheumatol 2006 September;33:1723. Editorial: H. Racz, S. Mehta. (Also see: Pulmonary Fibrosis) |
| Screening for Pulmonary Hypertension (PH) in Scleroderma: How and When to Look? As experience with newer imaging modalities increases and are rigorously assessed in future clinical studies, novel noninvasive technologies for the assessment of PH will help clinicians determine when and how scleroderma patients should be screened and how that information should be used in therapeutic decision-making. J Rheumatol 2006 February;33:204. Editorial. |
| Screening for PAH in Scleroderma: Identifying Hallmarks of the Disease and Optimal Treatment Strategies Pulmonary Hypertension Association. |
| Diagnostic Algorithm for Pulmonary Hypertension with Scleroderma |
| Pulmonary Diagnostic Algorithm for Patients With Scleroderma Pulmonary Hypertension Association. |
| Progressive Diagnostic Techniques and Procedures for the Diagnosis of Pulmonary Arterial Hypertension in Subjects Affected by Systemic Sclerosis. The disease activity criteria do not predict the presence of PAH in SSc patients, while ACA autoantibodies are strictly related with PAH. A good correlation was also found between echocardiography and RHC (right heart catheterization) in the assessment of PAH. S. Tonini. FRI0144 EULAR 2005. |
| DLCO in Pulmonary Hypertension |
| Carbon Monoxide Diffusing Capacity (DLCO) Correlates With Functional Pulmonary Vascular Surface Area In Patients With Pulmonary Arterial Hypertension (PAH) From Connective Tissue Disease (CTD). This is the first demonstration that the reduction in DLCO in PAH-CTD is at least in part related to loss of functional capillary surface area, and is proportional to its severity. D. Langleben THU0285 EULAR 2007. (Also see: Connective Tissue Disease) |
| Echocardiograms for Pulmonary Hypertension |
| Accuracy of Doppler Echocardiography in the Hemodynamic Assessment of Pulmonary Hypertension. Transthoracic Doppler echocardiography is recommended for screening for the presence of pulmonary hypertension. However, some recent studies have suggested that Doppler echocardiographicpulmonary artery pressure estimates may frequently be inaccurate. M. R. Fisher. Am. J. Respir. Crit. Care Med. January 22, 2009. |
| “Echocardiography cannot be relied upon to exclude pulmonary hypertension when pre-test probability is high.” |
Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. The positive predictive accuracy of currently used non-invasive tests are adequate for the diagnosis of advanced PAH provided sufficiently high thresholds (TG 45 mmHg or DLCO < 55% predicted) are used. These tests cannot be relied upon to exclude pulmonary hypertension where pre-test probability is high. PubMed. Rheumatology (Oxford). 2004 Apr;43(4):461-6. |
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| Evaluation of Asymptomatic Changes in Left Ventricular Function in Scleroderma Patients Using Echocardiographic Strain Imaging. Besides being a cause of pulmonary hypertension, severity of pulmonary involvement may be a contributing factor to subtle ventricular abnormalities observed in scleroderma patients as strain values were found to correlate significantly with DLCO (carbon monoxide diffusion capacity). A. Kepez. FRI0364 EULAR 2006. (Also see: Cardiac Involvement) |
| Lung lesion is a most common organ change in systemic scleroderma (SSD) detectable in approximately 70% of the patients at autopsy. This study has provided evidence that echocardiography is of high informative value in detecting pulmonary hypertension (PH) and right cardiac changes in patients with systemic scleroderma, which shows this technique to be a valuable screening in these cases. PubMed. Klin Med (Mosk). 2004;82(5):47-50. (Also see: Cardiac Involvement) |
| Exercise Echocardiography of Pulmonary Hypertension |
| Noninvasive detection of early pulmonary vascular dysfunction in scleroderma. Non-invasive assessment of pulmonary circulation during physical exercise and/or hypoxia enables screening for pulmonary vascular dysfunction in scleroderma. Reichenberger F. (PubMed) Respir Med. 2009 Jun 2. |
Using a bicycle ergometer
after physical activity,
and doing an echocardiogram
may help identify PAH early,
before it becomes irreversible. |
A new method allows for the early diagnosis of pulmonary hypertension. Using the bicycle ergometer to measure pulmonary artery systolic pressure of the patient after physical activity and performing an echocardiogram are very reliable methods, since they may help to identify scleroderma earlier. This confirms the importance of performing an echocardiogram on patients suffering from scleroderma, since this fatal complication may be indentified before it becomes irreversible. EurekAlert! 11/13/07. |
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| Exercise Doppler Echocardiography (Ex-Echo) as a Tool to Detect Pulmonary Arterial Hypertension (PAH) in Systemic Sclerosis (SSc) Patients in a Pre-Clinic Asymptomatic Phase. Ex-Echo is an useful tool for identifying a cluster of lSSc patients with sub-clinical PAH, that, possibly, could be treated early with a therapy able to reverse PAH and to halt the progression of pulmonary vessel involvement. A. Pignone. SAT0225 EULAR 2006. |
| Early Detection of Pulmonary Hypertension in Systemic Sclerosis by Exercise Echocardiography. Exercise echocardiogram detected 68% of abnormal pulmonary responses missed on the resting evaluation. This procedure may become a useful tool in the screening of PAH in SS patients. A. C. Cordeiro. FRI0335 EULAR 2006. |
| Reduced exercise capacity and stress-induced pulmonary hypertension in patients with scleroderma. Stress Doppler echocardiography identifies scleroderma patients with an abnormal rise in PASP (pulmonary artery systolic pressure) during exertion. PubMed. Chest. 2006 Jul;130(1):176-81. |
| The Incidence of Pulmonary Hypertension and Myocardial Ischemia in Systemic Sclerosis by Echocardiography Exercise Testing. Echocardiography exercise test is more sensitive, baseline method as compared to the rest echocardiography. The confirmation of PAH with right heart catheterization (RHC) might be applied to all the patients with systemic sclerosis. B. N. Stamenkovic. FRI0127 EULAR 2005. (Also see: Cardiac Involvement) |
| Assessment of Pulmonary Hypertension in Systemic Sclerosis by Exercise Echocardiography. Exercise echocardiography can identify patients that develop tricuspid gradient during exercise, even with normal echo values at rest. Albeit these small numbers it seems to be a safe and sensitive tool that can be used in the screening of scleroderma patients for the presence of PAH. M. J. Santos. FRI0305 EULAR 2004. |
| Functional Classification of Pulmonary Hypertension |
| A New Classification of Pulmonary Hypertension. Includes World Health Organization (WHO) Functional Classification of Pulmonary Hypertension. Pulmonary Hypertension Association. |
| High Resolution Chest CT for Pulmonary Hypertension is Not Helpful for Diagnosing PH in Patients with Advanced Pulmonary Fibrosis |
| High-Resolution Chest CT Findings Do Not Predict the Presence of Pulmonary Hypertension in Advanced Idiopathic Pulmonary Fibrosis. Chest CT-determined fibrosis score, ground-glass opacity score, honeycombing score, total profusion score, diameter of the main pulmonary artery, and the ratio of the pulmonary artery to aorta diameter did not differ between those with and without PH . There was no significant correlation between mean pulmonary artery pressure and any of the chest CT-determined measures. Chest. 2007; 132:773-779. (Also see: Pulmonary Fibrosis) |
| CT assessment of main pulmonary artery diameter. Right cardiac catheterization is considered to be a gold standard for measuring pulmonary artery pressure (PAP). However, this is an invasive procedure and carries a risk of mortality and morbidity (1, 2). Therefore, researchers have carried out several studies seeking a reliable and reproducible diagnostic imaging method for the assessment of the pulmonary artery diameter in order to predict the PAP. Diagn Interv Radiol 2008; 14:72-74. |
| Markers for Pulmonary Hypertension in Systemic Sclerosis (Scleroderma) |
| Ventricular mass index (VMI) correlates with pulmonary artery pressure and predicts survival in suspected systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH). The VMI, as demonstrated by magnetic resonant imaging (MRI), correlates well with mean pulmonary artery pressure in patients with SSc and may have a role in non-invasively excluding clinically significant PAH in breathless SSc patients in whom echocardiographic screening has failed. Dan Hagger. Rheumatology. July 14, 2009. (Also see: Cardiac MRI) |
| Serum Vascular Endothelial Growth Factor (VEGF) levels are related to the presence of pulmonary arterial hypertension (PAH) in systemic sclerosis. Serum VEGF levels are increased in systemic sclerosis patients with systolic pulmonary artery pressure(sPAP) ≥ 35 mmHg. The correlation between VEGF levels and sPAP may suggest a possible role of VEGF in the pathogenesis of PAH in systemic sclerosis. Andriana I Papaioannou. BMC Pulmonary Medicine 2009, 9:18. (Also see: VEGF) |
| Potential biomarkers for detecting pulmonary arterial hypertension (PAH) in patients with systemic sclerosis. The elevation of the serum levels of endoglin and endothelin-1 could represent a useful tool as PAH biomarkers. Nevertheless, the diagnostic value of these markers needs to be determined by prospective studies. P. Coral-Alvarado. (SpringerLink) Rheumatology International 01/07/2009. |
| Identification of Target Antigens of Antifibroblast Antibodies(AFAs) in Pulmonary Arterial Hypertension (PAH). AFAs detected in patients with PAH recognize cellular targets playing key roles in cell biology and maintenance of homeostasis. B. Terrier. American Journal of Respiratory and Critical Care Medicine Vol 177. pp. 1128-1134, (2008). (Also see: Antibodies) |
| Antiphospholipid antibody syndrome and autoimmune diseases. Evidence is growing that antiphospholipid antibodies may have a pathogenic role in pulmonary hypertension and accelerated atherosclerosis of autoimmune diseases. (PubMed) Hematol Oncol Clin North Am. 2008 Feb; 22(1):53-65. (Also see: Antibodies, Lupus, and Cardiac) |
| Anticardiolipin antibodies (aCL) are significantly associated with pulmonary arterial hypertension in systemic sclerosis (scleroderma) patients. |
Anticardiolipin antibodies (aCL) are significantly associated with pulmonary arterial hypertension in systemic sclerosis (scleroderma) patients. (Also see: What is Scleroderma?, Types of Scleroderma, and Antiphospholipid Syndrome) |
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| Plasma Brain Natriuretic Peptide as a Marker of Right Ventricular Overload in Systemic Sclerosis. NT-proBNP is a good marker of diastolic dysfunction frequently found in SSc, determined by various degrees of pulmonary hypertension, well correlated with inflammatory markers. A. E. Musetescu. SAT0217 EULAR 2006. (Also see: Cardiac Involvement) |
| Anti-endothelial cell antibodies in idiopathic and systemic sclerosis associated pulmonary arterial hypertension. IgG antibodies from patients with idiopathic or SSc associated PAH express distinct reactivity profiles with macrovascular and microvascular endothelial cell antigens. PubMed. Thorax. 2005 Sep;60(9):765-772. (Also see: Antibodies) |
| Raised levels of circulating endothelial cells in systemic sclerosis (SSc). Raised counts of CECs may represent direct evidence of active vascular disease in SSc as regard as visceral involvement, the association between CECs and pulmonary hypertension suggest a relevant role for CECs as a marker of prominent endothelial involvement. PubMed. Reumatismo. 2005 Jan-Mar;57(1):29-35. |
| Low Prevalence of Antiphospholipid Antibodies in Systemic Sclerosis (SSc) and Association with Pulmonary Arterial Hypertension. APL antibodies have a low prevalence in SSc with few thromboembolic events but are significantly associated with pulmonary arterial hypertension. N. Assous. FRI0298 EULAR 2004. (Also see: Antiphospholipid Syndrome) |
| P-Wave Amplitude |
| P-wave amplitude and pulmonary artery pressure in scleroderma. P-wave amplitude analysis on the ECG may be helpful in the assessment of pulmonary hypertension in patients with scleroderma. PubMed. J Electrocardiol. 2006 Aug 17. |
| Right Heart Catheterization (RHC) for Pulmonary Hypertension |
| “PH cannot be diagnosed without right heart catheterization.” |
How is PH/PAH Diagnosed? Pulmonary function tests can be helpful. Blood pressure in the lung can be estimated and the size and function of the right ventricle assessed by Doppler echocardiography. However, Doppler echocardiograms are notoriously inaccurate when the PH is mild or when there is simultaneous presence of lung scarring. PH cannot be diagnosed without right heart catheterization. James R. Seibold, MD, International Scleroderma Network Newsletter. 09-30-07. |
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| Risk Factors for Pulmonary Hypertension |
| Determinants of Pulmonary Arterial Hypertension (PAH) in Scleroderma. The present analysis identified pulmonary fibrosis and Raynaud's phenomenon preceding SSc skin manifestations by at least 3 years as risk factors for PAH in our scleroderma cohort. PubMed. Semin Arthritis Rheum. 2007 Jan 2. |
| Natural History of Mild–Moderate Pulmonary Hypertension (PH) and the Risk Factors for Severe Pulmonary Hypertension in Scleroderma. Development of severe PH is uncommon in certain subgroups of SSc patients. Risk factors for progression of PH include older age, limited skin disease, and elevated pulmonary artery pressures at the time of initial evaluation. J Rheumatol 2006 February;33:269-74. |
| Six-Minute Walk for Pulmonary Hypertension in Scleroderma Patients |
| Lack of Specificity of the 6-Minute Walk Test as an Outcome Measure for Patients with Systemic Sclerosis. The 6MWD relates to broad factors in SSc and these results raise doubts about the specificity of the 6MWD in this systemic disease, and its relevance to monitoring therapy. Yoland Schoindre. The Journal of Rheumatology. vol. 36 no. 7 1481-1485. July 2009. (Also see: Pulmonary Fibrosis) |
| Limitations to the 6-Minute Walk Test in Interstitial Lung Disease and Pulmonary Hypertension in Scleroderma. Pain may cause failure to reach a dyspnea limitation during 6MWT, especially in SSc patients without both ILD and PH. 6 MWT interpretation should include consideration of vascular, pulmonary, and musculoskeletal exercise limitations. M.C. Garin. Journal of Rheumatology February 1, 2009 vol. 36 no. 2 330-336 . (Also see: Pulmonary Fibrosis) |
| Six-minute walk test for the evaluation of pulmonary disease severity in scleroderma patients. Hemoglobin desaturation during a 6 minute walk test (MWT) provides additional information regarding severity of disease in scleroderma patients with pulmonary manifestations. PubMed. Chest. 2007 Jan;131(1):217-22. (Also see: Pulmonary Fibrosis) |
| Mediators and modulators of pulmonary arterial hypertension. Pulmonary hypertension, defined as a mean pulmonary arterial pressure of >25 mmHg at rest or >30 mmHg during exercise, is characterized by a progressive and sustained increase in pulmonary vascular resistance that eventually leads to right ventricular failure. PubMed. Am J Physiol Lung Cell Mol Physiol. 2006 Oct;291(4):L547-58. |
| TAPSE as Predictor of Patient Survival |
| A simple, noninvasive test measures survival time in adult pulmonary hypertension. A test called tricuspid annular plane systolic excursion (TAPSE) measures echocardiographic abnormalities in the tricuspid valve. "Our results suggest that TAPSE is a robust measure of right ventricular function and a powerful predictor of patient survival in pulmonary hypertension." EurekAlert! 11/01/06. |
