Disease Progression in Systemic Sclerosis Associated Pulmonary Arterial Hypertension

Condliffe, Robin

doi:10.1007/978-1-84882-637-3_17

Cited by 2 publications

(1 citation statement)

References 42 publications

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“…Analysis of the National United Kingdom Registry population showed that the median baseline walking distance was only 332 m and that while those who walked further had a better prognosis, change in 6MWD at 3 months did not predict outcome [62]. As in idiopathic PAH, baseline, but not change in 6MWD, predicts outcome, but 500 m is an unrealistic target for most SSc patients.…”

Section: Goal-directed Therapy In Scleroderma-associated Pulmonary Armentioning

confidence: 82%

An Update on the Evaluation and Management of Pulmonary Hypertension in Scleroderma

Coghlan

Schrieber

2012

Curr Rheumatol Rep

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Pulmonary arterial hypertension associated with scleroderma (SScPAH) is a debilitating, highly lethal condition that responds to an array of therapies. Quality of life and prognosis are substantially improved by treatment, and early diagnosis and treatment are associated with improved outcomes. There are serious limitations to current screening programs. Many more questions need to be addressed. Why is PAH so common in SSc? Why is the tolerance of pulmonary hypertension so poor in scleroderma? What are the best measures of response to therapy in SSc patients with PAH? Should we use different parameters in prognostic scores in SScPAH? Why is postcapillary pulmonary hypertension so common in SSc? How do we reliably differentiate lung disease-associated pulmonary hypertension from PAH? The aim of this review is to summarize the main areas of progress over the past decade and to look to the challenges for the next decade.

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Section: Goal-directed Therapy In Scleroderma-associated Pulmonary Armentioning

confidence: 82%

An Update on the Evaluation and Management of Pulmonary Hypertension in Scleroderma

Coghlan

Schrieber

2012

Curr Rheumatol Rep

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Complex Challenges of Pulmonary Hypertension

Coghlan

2011

Scleroderma

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Disease Progression in Systemic Sclerosis Associated Pulmonary Arterial Hypertension

Cited by 2 publications

References 42 publications

An Update on the Evaluation and Management of Pulmonary Hypertension in Scleroderma

An Update on the Evaluation and Management of Pulmonary Hypertension in Scleroderma

Complex Challenges of Pulmonary Hypertension

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