Lung fibrosis progression

[alive and kicking]

I am after some advice, i am getting conflicting stories, so thought i would come straight to the ones in the know with experience.

I have WG and had a double diffuse alveolar hemorrhage 2 years ago, i now have lung fibrosis, i have a good capacity of 72% but my transfer values are 57%

My original Resp Consultant said that in time my fibrosis will just increase, but they want to make it as slow as possible

Another,said that it will stay as is,no progressive deterioration.

I have come down on pred,as low as 4mg now,and i am coughing again and a bit more breathless.

Anyone with experience of this be able to shed some light for me??? when you look at google for lung fibrosis it doesnt look good but thats idiopathic,not WG related

Many thanks for reading

[renidrag]

I must admit no knowledge other than what I just read at WebMD but I would see a rheumatologist who is learned in vasculitis and WG. From the article, WG can be a cause of the fibrosis and seems to be able to be controlled with our best friend prednisone and AI meds such as Methotrexate. Definitely would see the proper Rheumy though. Do you already have one? By the way I also have Wegs but am in remission at this time.
Dale

[Dirty Don]

I didn't have a 'progression' as such, but my lungs were quite the mess upon the first and worst flare. With large doses of pred my lungs cleared up pretty well. With the exception of a new found susceptibility to upper bronchial and pneumonia problems, my lungs are maintaining well enough. I mean, we're talking 'fractured glass' here in the Xrays! As I've tapered on pred, it seems I have become more susceptible to the above stuff. Best of luck...and I 'ditto' Dale's advice...get to a WG experienced rheumy if at all possible.

[pberggren1]

It will most likely just get worse over time. I also have scar tissue in my bronchials so my breathing is not that great either. I have to go for dilations every 6 months or so now.

[Sangye]

I agree with Dale that it's best to see a pulmonologist with a lot of Wegs experience. Lung fibrosis does not always get worse with time. I don't know how they determine when it might or might not, which is why you need an expert. :wink1:

It's worth traveling to a major center for a consultation if you need to.

[marylz]

The doctors also told me that my lungs looked like ground glass, for what it's worth. I can't give advice, as I am newly diagnosed and not nearly as knowledgeable as others.:wink1:

[mishb]

Hi Marylz and welcome to the group. Glad you have found us but sorry that you had too :sad:

Don't worry about not knowing much about this disease, we all learn different things about it on a quite regular basis and you will learn very fast from some very knowledgeable people on here and you will find that you will also be able to help with answers to things that we may not know about.

Do you have a wegs specialist and what have they done so far with the lungs and other symptoms (if any)?

[Sangye]

The doctors also told me that my lungs looked like ground glass, for what it's worth. I can't give advice, as I am newly diagnosed and not nearly as knowledgeable as others.:wink1:
The ground glass appearance can be caused by a number of conditions, including certain types of pneumonia, lung hemorrhage, some types of lung cancer, lymphoma, sarcoidosis and interstitial lung disease. Did you have lung hemorrhage? If so, your lungs will clear up just fine once the treatment starts working.

[alive and kicking]

Do you have a wegs specialist and what have they done so far with the lungs and other symptoms (if any)?

Hi I am being seen at Addenbrooks by Dr Jaynes team,but havent seen a Respiratory consultant for a year, my last lung function test was requested by my rheumatologist,who i now realise does not know as much about WG as I was led to believe, but i cant change as there is no one else at our hospital.
Those test results showed deterioration and that my muscles arent working as should be. My first resp doc said that with this type of fibrosis it would just get gradually worse, if this is true,i would rather know,and live life now as best i can.
This has already taken the use of my legs,without all the rest of the rubbish it brings.
I had a diffuse alveolar hemmorrhage,both lungs. They would fill up, i'd cough, hemmorrhage then fill up again,and so on,they did this for 2 days before they put me on a ventilator and gave me chemo. The alveoli are scarred now.

Thanks all for your answers :)