Age and Risk of Pulmonary Arterial Hypertension in Scleroderma*

Schachna, Lionel; Wigley, Fredrick M.; Chang, Betty; White, Barbara; Wise, Robert A.; Gelber, Allan C.

doi:10.1378/chest.124.6.2098

Cited by 106 publications

(75 citation statements)

References 39 publications

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“…In a Canadian prospective study, only 4.9% of SSc patients were diagnosed as having PAH using Doppler echocardiography (2). Similar surveys in the UK found prevalences of 13% and 12% (3,9), and surveys in the US found prevalences of 35% and 38.6% (19,20). This variation is probably due to differences in the definition of PAH and in the populations of patients studied.…”

Section: Prevalence Of Undiagnosed Pah In Community Practicesmentioning

confidence: 96%

“…Physical findings may be absent in early disease, and chest radiographs are usually nondiagnostic. There are risk factors that should make the physician more suspicious of the presence of PAH, including a diagnosis of connective tissue disease (especially limited SSc), a late age at onset of SSc (20), progressive decline in the DLCO in SSc (21), and the presence of certain autoantibodies, including ACAs, anti-B23, anti-U3 RNP, anti-U1 RNP, or anti-Th/To antibodies (4). Rapidly progressive PAH presents more often as isolated PAH in patients with limited SSc (9).…”

Section: Prevalence Of Undiagnosed Pah In Community Practicesmentioning

confidence: 99%

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The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of community‐based rheumatologists (the UNCOVER study)

Wigley

Lima

Mayes

et al. 2005

Arthritis & Rheumatism

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170

103

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Objective. Most of the data about the prevalence of pulmonary arterial hypertension (PAH) are from tertiary centers that are biased toward seeing more severe cases; therefore, the true prevalence of PAH among patients with connective tissue disease is unknown. We sought to determine the point prevalence of undiagnosed PAH in community-based rheumatology practices.Methods. The study design was a multicenter, prospective and retrospective survey and analysis of clinical cases in 50 community rheumatology practices. We evaluated a total of 909 patients with either scleroderma ( Conclusion. A significant number of patients with SSc or MCTD (13.3%) followed up in a community rheumatology practice setting have undiagnosed elevated ERVSP consistent with PAH.

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Section: Prevalence Of Undiagnosed Pah In Community Practicesmentioning

confidence: 96%

Section: Prevalence Of Undiagnosed Pah In Community Practicesmentioning

confidence: 99%

The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of community‐based rheumatologists (the UNCOVER study)

Wigley

Lima

Mayes

et al. 2005

Arthritis & Rheumatism

Self Cite

170

103

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“…56 This makes it often uncertain if the pulmonary hypertension is due to the lung disease, pulmonary vascular disease, or both. 57 Most series show that a marked reduction in diffusing capacity for carbon monoxide is a consistent feature of the patients with connective tissue diseases who have pulmonary hypertension, 58,59 but it does not correlate with the degree of pulmonary fibrosis.…”

Section: Category 3: Patients With Pulmonary Hypertension Associated mentioning

confidence: 99%

Diagnosis and Treatment of Secondary (Non–Category 1) Pulmonary Hypertension

Rich

Rabinovitch

2008

Circulation

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P ulmonary hypertension occurs commonly in cardiopulmonary disease. In 1998, a new clinical classification was proposed that divided pulmonary hypertension into 5 categories on the basis of the presumed underlying etiology of the pulmonary vascular disease 1 (Table 1). Although it was never intended that this classification be used as a guideline to determine appropriate therapy, somewhat surprisingly, the regulatory authorities decided that all medications that were clinically tested and approved for patients with idiopathic pulmonary arterial hypertension (PAH) and patients with pulmonary hypertension associated with connective tissue diseases be applied to all category 1 patients. 2 The wisdom of this can be debated at a later date. However, it has left clinicians somewhat confused about the utilization of therapies to treat patients who fall outside of category 1 pulmonary hypertension, often referred to as secondary pulmonary hypertension.There are 3 classes of approved therapies for PAH, all of which are considered to be pulmonary vasodilators: endothelin receptor blockers, phosphodiesterase-5 inhibitors, and prostacyclins. 3 Their clinical efficacy has been based on a short-term improvement in exercise tolerance, as measured by a 6-minute walk test. In all of the clinical trials, an improvement in walk was apparent within the first 4 weeks of use, allowing a judgment about efficacy to be made quickly. 4 Hemodynamically, their effects are modest, but they tend to raise cardiac output with little effect on pulmonary artery pressure. This has important implications when they are considered for unapproved use. We review the distinctive features of these causes of pulmonary hypertension and the data on the evidence that supports or refutes the use of these therapies in noncategory 1 pulmonary hypertension. Category 2: Pulmonary Venous HypertensionPatients with pulmonary venous hypertension have elevated pulmonary venous pressure (as reflected in the pulmonary capillary wedge pressure), most frequently as a consequence of either mitral valve disease 5 or left ventricular (LV) diastolic dysfunction. 6 Although mitral stenosis was the most common cause of this entity decades ago, LV diastolic dysfunction is the most common cause of pulmonary venous hypertension seen in our referral practice. 7 It is presumed that the mechanism of both is similar. Specifically, a chronic elevation in the diastolic filling pressure of the left heart causes a backward transmission of the pressure to the pulmonary venous system, which triggers vasoconstriction in the pulmonary arterial bed. 8 Histologically, abnormal thickening of the veins and formation of a neointima are seen. 9 The latter can be quite extensive ( Figure 1). As secondary features, medial hypertrophy and, with time, thickening of the neointima on the arterial side of the pulmonary circulation occur as well. There is great potential for reversibility of these changes with improvement in the cause of the venous hypertension (see below). The variability in the response...

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“…Right-ventricular systolic pressure (RVSP), reflecting systolic pulmonary artery pressure, was estimated from the tricuspid regurgitating velocity, following the Bernoulli principle, via tissue Doppler echocardiography. 11,12 Statistics Subjects whose S pO 2 decreased Ն 4% during 6MWT were assigned to the desaturation group, because exercise desaturation of Ն 4% predicts mortality 1 and is an adverse prognostic sign 13 in patients with idiopathic pulmonary fibrosis. The remaining subjects constituted the normoxic group.…”

Section: Methodsmentioning

confidence: 99%

Predictors of Exercise-Induced Oxygen Desaturation in Systemic Sclerosis Patients With Interstitial Lung Disease

Someya

Mugii²,

Hasegawa

et al. 2013

Respir Care

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BACKGROUND:The diffusion capacity of the lung for carbon monoxide (D LCO ) is a good marker of disease severity in patients with idiopathic interstitial pneumonia, and is associated with oxygen saturation; however, little is known about D LCO in systemic sclerosis patients with interstitial lung disease. We studied potential predictors of exercise-induced oxygen desaturation in patients with systemic sclerosis. METHODS: Data were collected prospectively from 80 of 110 consecutive systemic sclerosis patients with normal oxygen saturation (> 95%) at rest, who could perform the 6-min walk test without physical discomfort, including leg pain. Pulmonary function tests and echocardiography were collected from all subjects. RESULTS: Thirty subjects showed a > 4% decline in oxygen saturation during the 6-min walk test (desaturation group). The other subjects were assigned to the normoxic group. The percent-of-predicted values for FVC, FEV 1 , total lung capacity, D LCO , and D LCO /alveolar volume were lower, and FEV 1 /FVC was higher, in the desaturation group. Logistic regression analysis showed the percent-of-predicted D LCO as a highly accurate predictor of exercise-induced oxygen desaturation: the area under the receiver operating characteristic curve was 0.92 (cutoff point 56.3%, sensitivity 0.83, specificity 0.86). Five subjects over the cutoff point of the percent-of-predicted D LCO in the desaturation group could not be distinguished from the normoxic subjects with the lung-volume measurements or right-ventricular systolic pressure. CONCLUSIONS: The factor underlying exercise-induced oxygen desaturation appeared to be reduced percent-of-predicted D LCO , which was useful as a predictor in over 80% of the subjects.

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Age and Risk of Pulmonary Arterial Hypertension in Scleroderma*

Cited by 106 publications

References 39 publications

The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of community‐based rheumatologists (the UNCOVER study)

The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of community‐based rheumatologists (the UNCOVER study)

Diagnosis and Treatment of Secondary (Non–Category 1) Pulmonary Hypertension

Predictors of Exercise-Induced Oxygen Desaturation in Systemic Sclerosis Patients With Interstitial Lung Disease

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