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Scleroderma & Raynaud's UK (SRUK)
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Hi is there anyone out there whose Scleroderma started in their lungs, with nowhere else affected and no other asdociated illnesses. I know in one way I'm lucky, when i read about the problems other people have, but i am on oxygen nearly all the time and even small tasks take a lot of effort. I am wondering if anyone else has the same experiences and have they seen any improvement with treatment?

6 Replies

Hi Mandy, yes I am in the same position as you, although because of the lack of any other symptoms, it has not been confirmed as Scleroderma, only as an unclassified ILD most likely related to Scleroderma. Like you I am grateful for the 'current' lack of other symptoms but who knows what is ahead. I have had a 6 month course of Cyclophosphamide followed by 2 years on mycophenolate . It's difficult to say whether any of it has worked as the disease is still progressing, but maybe the drugs have slowed it down.


Hi Kirsty, thanks for your reply. I've had 4 infusions of cyclophosphamide, 2 more to go. There is a slight improvement, I've been able to reduce the oxygen level a bit. I hope for more, but doctors just seem pleased to have stopped the progression. Yours is the only reply to my post, so there don't seem to be any others in the same position. I'm finding this hard, i've applied for ill health retirement but as no one can say how the disease wii progress, or how treatment will work over the next few years i may not get it. Are you able to work?

I hope you are keeing well. Mandy


Hi Mandy, yes I am still working full time and to be honest I have not told anyone other than my husband about the condition, I have been able to carry on but for how long? I was diagnosed 3 years ago and my gas transfer level was 50% of a woman my age. I then had 6 rounds of cyclophosphamide , followed by 2 years of mycophenolate . The gas transfer %age has gone up and down a bit but the last one has dropped to 39% which is a bit of concern. I know exactly how you feel, the doctors don't know what it is, how it will progress, what to treat it with, not that there are lots of options. I

have been offered the chance to go on a drugs trial for pirfenidone and have been told to get my head around the possibility of a lung transplant.

How long did it take you to be diagnosed, what was it that prompted you to go to the doctor in the first place. Did you exacerbated ray aids bfoehand.


This all started for me with Flu in Feb 16 i went to doctors with persistant cough and occasional breathlessness. They did lots of tests and diahnosed asthma! Luckily i was doing pulmonary rehab as they had no other suggestions for treatment and the clinical lead is a wonderful man who didn't accept it was asthma and pushed for other tests and arranged for quick appointments. They found i had a hole in my heart, but this Doesn't seem to be related. Eventually my gp sent me to hospital as breathing getting worse and heart doctors said if i stayed in they could do all the planned tests and get to the bottom of it. 3 weeks later CT scan showed inflammation in my lungs, not visible 12 months ago and blood tests showed ILD, further tests indicate Scleroderma sine scleroderma, but not 100% definate diagnosis.

So it took 18 months for this diagnosis. What are exacerbated ray aids? I've not heard of them. I have pulmonary hypertension, but no damage to the right side of my heart, which is unusual, but good of course!

Lung transplant was mentioned, but only in the context of available treatment if nothing else worked. I think my gas transfer is improving, but it was about 25%, hence the oxygen. I am under Hammersmith hospital as well as Bournemouth, although some talk of the Royal free as well.

How did you hear about the drugs trial?

What sort of benefits do they think it could bring?


Sorry Mandy that it's taken me so long to reply, but I have been away with work. I meant to ask if you had previously had raynauds but predictive texts mixed it up. I am amazed that you have gone from being 'alright' albeit a bit breathless to needing oxygen within the space of a year. I thought this was a slowish moving disease, it took them 7 months to start my treatment after it had been diagnosed.I heard about the trial from my consultant but I may end up with the placebo and even if I get the drugs there is no way of knowing if they will help. But it may help people in the future. What have you had to try and stop the progression of the disease?


Don't worry, it's nice to be able to talk to someone with similar version of this disease. I don't have Raynauds or any other symptoms, except the pulmonary hypertension. They diagnosed it in July and started me on cyclophosphamide straight away, within 2 weeks of diagnosis, but it had taken 18 months to get diagnosis with no treatment in that time. I'm just having the 4th infusion now. I've been told that i have a rare form of this, part of that i suppose is how fast it has developed, on the other hand they hope that the treatment will be effective because it hasn't been too long.

I had pneumonia in February and now i think this made things worse and progressed the disease faster.

I've been able to reduce the oxygen level over night in the last few days so hopefully this means it's beginning to work. It's difficult to tell because the chemo makes me very tired.

How are you?


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