tailieunhanh - Báo cáo y học: "Cold hands — strained heart? Advances in the management of Raynaud’s phenomenon and pulmonary hypertension"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học General Psychiatry cung cấp cho các bạn kiến thức về ngành y đề tài: Cold hands — strained heart? Advances in the management of Raynaud’s phenomenon and pulmonary hypertension. | Arthritis Research Therapy June 2005 Vol 7 No 3 Hall Viewpoint Cold hands - strained heart Advances in the management of Raynaud s phenomenon and pulmonary hypertension Frances C Hall University of Cambridge School of Clinical Medicine Addenbrooke s Hospital Cambridge UK Corresponding author Frances C Hall fch22@ Published 18 April 2005 This article is online at http content 7 3 126 2005 BioMed Central Ltd Arthritis Research Therapy 2005 7 126-128 DOI ar1755 Vasculopathy is an increasingly recognised partner to inflammatory rheumatological disease. Raynaud s phenomenon RP is generally considered a benign vascular manifestation. The natural history of systemic sclerosis SScl however suggests that we underestimate the significance of RP in this context. RP occurs in approximately 90 of SScl patients often decades before the diagnosis is recognised and is a harbinger of generalised vasculopathy. Pulmonary arterial hypertension PAH is another manifestation of this process which occurs in approximately 12 of SScl patients 1 . PAH accounts for approximately 50 of mortality in limited SScl and accounts for approximately 7 of mortality in diffuse SScl. It is probable that the vasculopathic processes underlying RP and PAH contribute to the familiar pattern of skin renal and gastrointestinal pathology. The emergence of effective and conveniently administered therapy for PAH increases the importance of diagnosis and monitoring of this complication. Furthermore since many principles of PAH management translate to the management of RP this raises the possibility that the generalised vasculopathy of SScl may also be modifiable. In the characteristic microangiopathy of SScl luminal narrowing results from a combination of intimal proliferation medial hypertrophy and adventitial fibrosis 2 . This leads to a state of progressive chronic organ ischaemia. Dysfunction of cellular components of the arterial wall and dysfunction of .