Effective therapies and increased awareness mean that general practitioners, doctors in training and nurses, as well as specialists in cardiology, respiratory medicine, rheumatology and HIV and liver medicine, need a working knowledge of the basic principles of screening and management of pulmonary arterial hypertension. Rare diseases are often overlooked or forgotten, but advances in the field of pulmonary arterial hypertension mean that a hitherto rapidly fatal disease is now, for many patients, a controllable condition. The known causes of pulmonary hypertension are many and ever growing and a patient may present to one of many specialities. An enhanced awareness of the condition and willingness to work across specialty boundaries, to investigate early with relevant tests, to share care and refer appropriately for further specialist advice and management will undoubtedly improve the quality and quantity of life for our patients.
Professor Dame Carol Black DBE
PULMONARY HYPERTENSION IN SCLERODERMA (SYSTEMIC SCLEROSIS)
Pulmonary hypertension is recognised as a lethal complication of all forms of systemic sclerosis. Endothelin has been postulated as having a pivotal role in the pathogenesis of the pulmonary vascular disease associated with this condition, which has all the hallmarks pathologically of PAH. There are, however, significant clinical differences compared to other forms of PAH, relating principally to late presentation and/or recognition of the pulmonary vascular abnormality. This occurs in the main because of the significant co-morbidities associated with the underlying condition that often dominate the clinical presentation early in the disease. As a consequence patients with systemic sclerosis often present in advanced stage of right ventricular dysfunction and functional decline and as a result, treatment outcomes are generally less satisfactory when compared to idiopathic PAH for example.
PAH most commonly complicates limited cutaneous systemic sclerosis (also known as CREST syndrome), and is not to be confused with the pulmonary hypertension tension complicating systemic sclerosis-related interstitial lung disease/pulmonary fibrosis.
For patient information on Pulmonary Hypertension please visit the PHA website at http://www.phassociation.uk.com/
Below are the Pulmonary Hypertension Specialist Centres in the UK & Ireland.
Click on the hospital below for further information:
Freeman Hospital - Newcastle Upon Tyne - Tel: 0191 233 6161
Golden Jubilee National Hospital - Tel: 0141 951 5000
Great Ormond Street Hospital - London - Tel: 020 7405 9200 : Ext 1005
Hammersmith Hospital - London - Tel: 020 8383 2330
Mater Misericodiae Hospital - Eire - Tel: 00 3531 8032000
Papworth Hospital NHS Trust - Cambridgeshire - Tel: 01480 830541
Royal Brompton Hospital - London - Tel: 020 7351 8121
Royal Free Hospital - London - Tel: 020 7794 0500
Royal Hallamshire Hospital - Sheffield - Tel: 0114 271 1719