Scleroderma & Raynaud's UK (SRUK)
6,790 members3,213 posts

Pulmonary pressure no symptons excellent exercise capacity?

I was diagnosed with scleroderma about 6 years ago and apart from one eposide of systemic inflamation approx 5 years ago I have been well. I have the limited form CREST wihich only seems to cause raynauds and some finger thickeneing. I have had annual lung function screening and Echocardiograms and thus far all has been normal. My last lung function test was fine but the Echo showed an estimated pulmonary pressure of 40 - 45 which I understand is quite high but normal bi ventricular function. From what I have read this level of pulmonary pressure would cause sypmtoms although the "interesting" thing is my exercise capacity is fine for example I can easily complete over 700 metres in the 6 minute walk test and I have no breathlessness or other symptons that I know of. I do have white coat hypertension syndrome and my GP now accepts that it is much better for me to monitor my BP at home when it is normal at 125/80. I find the echocardiogram ( or indeed most medical tests) stressful and I wonder if that can impact the estimated pulmonary pressure . My consultant is on the case and will be seeking additional opinions but at the moment no change in my meds or any further tests. Any opinions

6 Replies

There could be developments in other organs but not necessarily. You canot separate Raynaud's and scleroderma. Mine affect my digestive system and my circulation. I have regular echocardiograms and breathing tests simply to test if my lungs or my heart are affected. It is quite important to have these tests even or (hopefully) they are all right. I am quite old (83) and have had it for quite and long time much of which was undiagnosed, untested and wrongly treated. I used to be able to do quite a lot and I have worked until I was 72. You are obviously young, enjoy it, do everything you want, but be tested, just in case, and if you have any developments they can be treated nowadays to a large extent. Have a full life.


Hi there, I have CREST limited Scleroderma with raynauds and slight lung fibrosis. I am checked with lung function tests every 6 months. I had a right heart catheter done 2 years ago and all was fine, but my cardiologist wants to repeat this just to be on the safe side, he said it's the only way to see what is happening with the transfer, all other tests are not thorough enough so on 25th July I am off for another, day surgery, it's not too bad but have to get through it for peace of mind. I go to The Royal Free Hospital in North London where they study Scleroderma under Prof Denton's team. I hope this is useful information. I have had Sclero for 5 yrs. If you have any other questions please come back to me. All the best.


I have had Raynauds for about 45 years which developed into CREST about 10 years ago. I have been having regular lung function tests & Echocardiograms since then (every year). About 3 months ago I began to feel breathless and I was sent to the Royal Hallamshire Hospital, Sheffield for assessment and was diagnosed with Pulmonary Arterial Hypertension (PAH). PAH is very rare, but one in ten CREST sufferers go on to develop PAH. It is important that PAH is diagnosed quickly - delay in making the diagnosis has the same consequences as delay in those with cancer, according to Professor Dame Carol Black DBE, MD, FRCP,MACP, FMEDSCT.

There are several designated PAH Centres in the UK:

Glasgow, Scotland Scottish Pulmonary Vascular Unit, Western Infirmary,

Glasgow, G11 6NT

Tel: 0141 211 6327 Fax: 0141 211 6334




Northern Pulmonary Vascular Unit, Regional Cardiothoracic

Centre, Freeman Hospital, Newcastle upon Tyne, NE7 7DN

Tel: 0191 223 1084 or 0191 244 8608

Fax: 0191 223 1691

Sheffield, England Pulmonary Vascular Unit, Royal Hallamshire Hospital,

Glossop Road, Sheffield, S10 2JF

Tel: 0114 271 2590 Fax: 0114 271 1718

Cambridge, England Pulmonary Vascular Diseases Unit, Papworth Hospital NHS

Trust, Papworth Everard, Cambridge CB3 8RE

Tel: 01480 830 541 Fax: 01480 831 315


London, England Pulmonary Hypertension Service, Hammersmith Hospital,

Du Cane Road, London, W12 0HS

Tel: 0208 383 2330 Fax: 0208 383 2331


London, England Royal Brompton Pulmonary Hypertension and Adult

Congenital Heart Centre,

Sydney Street, London SW3 6NP

Tel: 0207 351 8362 Fax: 0207 351 8629


London, England Royal Free Hospital, Pond Street, London, NW3 2QG

Tel: 0207 794 0500 ext 8648.


London, England UK Pulmonary Hypertension Service for Children, Great

Ormond Street Hospital,

London, WC1N 1EH

Tel 0207 405 9200 Ext.1005, 1007, 8495

Dublin, Ireland Mater Misericordiae University Hospital

Eccles Street, Dublin 7

Tel 00 353 1803 44 20


Pulmonary hypertension

Thorax 2008;63(Suppl II):ii1–ii41. doi:10.1136/thx.2007.09048.



Sounds like you are tetering on the fence between a diagnosis and not getting a diagnosis. Me too. I do about 650 meters in the 6 minute walk test (female shorter steps). I have raynauds for 45 years and diagnosed with CREST for about 10 years ago after severe GERD episodes. My ecos are borderline and show some slight right side involvement at this stage. My O2 goes down when I exercise, but I am not sure the measuring device (finger meter) is the best thing to use since the raynauds restricts blood flow to the fingers. So far, nothing has been recommended for the possible PAH.

I am wondering what the progression of lung involvement is so I can treat it before it gets to far. About the only thing I have noticed is after I walk up 3 flights of stairs, I get a little winded. Flat surfaces, no issues. But I dont have the expectation that if I push it, I can improve, which is frustrating to me. I am also 62 and have arthritis in my knees, so dont know if age plays a factor here also.

I talked with my pulmonary guy and he indicates that there are now several medicines that can be used to treat the suspected PAH orally. One of them is a type of Viagra for the lungs.

So keep me informed about your progress. I go for another cardiogram and a 6 minute walk test in 2 months to see where I now stand.


Thanks for the answer- I would have thought 650 metres plus is excellent especially for a female . My consultant referred my echo results to the Sheffield Centre in the UK and they said they were not too concerned and simply repeat the echo in. A year or so. My consultant at the QMC in Nottingham has said that for pulmonary hypertension a combination of high lateral pressure and significant function reduction in DLCO on the Lung Function Test to 70% is needed mine is 90% and has actually improved. He also has said that a number of new drugs are available and it can be treated.

I think the best approach is not too worry and only get concerned if you get breathless - remembering of course that half the population in their 60's would be a bit breathless after climbing 3 flights of stairs.


My DLCO is 54%. Not exactly sure what it means. I see the pulmonary doctor in 2 months after another echo. Rhum says probable PAH. Things just seem to move so slow with these doctors!~

Thanks for your response.


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