Newbie with lung tumor

[newbie]

Hello everyone.

I am in the CNY area and have recently been diagnosed with Wegener's GPA. The dx is not complete because they cannot get to the tumor in my lung to get a biopsy of it. It is in a terrible location according to both the thoracic surgeon and the radiologist who have reviewed the ct and pet scans. Cancer hasn't been ruled out either.
The rheumatologist I am seeing wants to start me on methyltrexate and prednisone anyway as he is convinced the weg dx is correct. I am frightened, to say the least.
My dx story:
June 2nd; my feet swell throughout the day and into the evening...goes away overnight. I was tired and short of breath when hiking with hubby over the weekend but think nothing of it. just getting old and already out of shape.

June 9th, went to urgent care with horrible lower abdominal pain. Abdominal CT and Ultrasound show nothing except an enlarged uterus and an ovarian cyst. I am referred to ob/gyn

July 2nd; finally see ob/gyn. still having abdominal pain and decreased appetite. I can eat only a bite or two at a time. I have now lost 15lbs since June 1st. He takes uterine biopsy and does some blood work for hormones and such. I have fibroids-have had them forever so no surprise-and my uterus is enlarged because of them but he does not think this is the cause of my problem. My primary care doc has been trying to talk me into a hysterectomy for nearly two years due to heavy bleeding and anemia(more severe right now) so I am referred for second opinion. He finds a strange area of skin on my left breast. I am referred for mammogram.

July 11th; mammo and ultrasound of left breast...all clear for cancer. referred to dermatologist.

July 14th; new ob/gyn says of course your pain/weight loss is from uterus. Schedules hysterectomy. I have now lost 20 lbs.

July 30th; dermatologist has no idea. gives me 3 different topical treatments.

Aug 19th: endoscopy for gi issues-shows nothing but inflammation. I have now lost 25 lbs. weight loss is slowing because I have figured out how/what to eat throughout the day. I still have no appetite and I can go 24 hours without eating and my stomach doesn't give a gurgle.

Aug 22nd; no change in skin lesion so dermatologist takes a biopsy.

Aug 29th; Dermatologist informs me the lesion on my breast is Morphea and I am sent for another battery of tests, including chest x-ray all of which I do on Sept 1st. By now I have lost 30 lbs and I have developed a cough to go with my ongoing headache. I have no energy to exercise and get short of breath walking from my desk to the bathroom. I have eaten more ice cream than ever this summer...and still I am down two pant sizes.

Sep 4th; my primary care doc's office calls and tells me to go for a CT scan the following day and schedules fu appointment with pcm for Sept 8.

Sept 8th; pcm tells me I have a 3.9x3.3x3 cm mass in my lung. that plus the scleroderma prompts a referral to rheumatologist. I am also scheduled for a pet scan, cardiology work up and pulmonary function test.

Sept 11th; see rheumy for first time. Determines this is not systemic scleroderma but localized morphea. Asks about my cough and do I have issues with my ears and sinuses-yes, for quite a long time. why? orders 8 more vials worth of blood tests

Sept 12th; heart checks out fine

Sept 15th; pet scan performed

Sept 18th; back to pcm for results of pet. not good. shows activity. I am having all over body aches, swollen and sore glands and sinus headache that won't quit. Still coughing. She gives me something for cough and anxiety. Rheumy calls me at 6:30 pm to say he suspects Wegener's disease based on symptoms and test results. Asks when the biopsy of mass is scheduled and when I tell him it isn't scheduled yet and won't be done until mid-October at the earliest, he throws a fit and sits in it, tells me he is going to call my pcm and see what can do to hurry it up....but don't worry...oh and go to emergency room if you start coughing up blood. Perfect day to start on anti-anxiety meds!

Sept 22nd; pft results ok

Oct 1st; consult with thoracic surgeon-no way to biopsy without taking half your lung. too close to too many blood vessels. repeat scan nov 4th. see me again Nov 13th. I call the rheumy and he gets upset-and reminds me to go to emergency room if I start coughing up blood. (duh!)

So, still no biopsy and he wants to start meds anyway. My appetite is returning, no weight loss or gain but my stomach makes noises again :). All other symptoms persist. I am exhausted. I am confused. I am frightened. I started off the summer with stomach pains, scheduled a hysterectomy which got cancelled because now I have Morphea, a mass in my lung we can't identify and a rare AI disease.

To summarize....wtf.

Thank you for giving me someplace to vent and feel less alone.

[drz]

Welcome. Starting treatment now seems like right call. Hope it helps quickly!

[Rose]

I agree. It could also give you a Wegeners diagnosis as if you improve rapidly and the mass in your lungs shrinks, you will know you are on the right track. They usually wont start treatment (because of the toxic drugs used in the treatment) until a biopsy of the lung is performed but in this case if they cant get to the mass, I think they should treat you for WG without further delay. I did have lung and kidney involvement. Following a positive kidney biopsy for WG treatment was commenced and within days the masses in my lungs started to shrink.

Rose

[mrtmeo]

Rose is right.
If you have any signs of abnormal kidney function, get a biopsy to confirm glomerulonepthritis.
A urinalysis might show red blood cell casts, blood in the urine and/or protein in the urine.

If they haven't already, they should do a C-Reactive Protein, Sediment Rate and Rheumatoid Factor test, ANCA and MPO and PR3 for wegener's or microscopic polyangiitis.

As far as food, I got Bariatric Fusion which gives you a powder you mix with 4 or 6 oz of water or milk that provides a good nutritional profile and 27gms of protein.

Treating this disease early is key and may prevent relapses.
The masses in the lungs are usually blood clots, but they can't tell this with their scans.
Lung biopsies are very dangerous and deadly and they tend to be non conclusive.

Check out some of the stories at the Vasculitis foundation website
Vasculitis Stories (http://www.vasculitisfoundation.org/support/vasculitis-stories/)

You can also, contact them for help.

[Debbie C]

Hi and welcome to the "family" We are a very close knit group here and since it is worldwide there is usually someone here day or night if you have guestions,need to vent or just talk.
I also have involvement in my lungs although they say I have been in remission for about 3 yrs now. I never knew I was sick until I got rear ended in my car and went to the er to get checked out.They did a ct scan of my lungs and showed several nodules in both lungs and they were afraid I had lung cancer. My pulmy did 2 needle biopsys but could not get enough to dxed me so I had to have a partial right middle lobectomy to prove it was wg.I was then put on cytoxin (ctx ), prednisone and Bactrim. The nodules started going away.You need to make sure all your drs. are familiar with wg but it sounds like yr rhuemy is on top of things. But diffently get bloodwork and uinylisis done..this will show a lot.The anca test isn't much prove since mine kept coming up neg. Good luck and keep us posted

[Dirty Don]

Hi wtf...I think that is a common reaction to a dx of WG...LOL. OK, not funny, but sounds like your docs have a grip on it. Thus, your job now is to get a grip on yourself as I'm sure you have or will. Patience, fortitude, and drug protocols are what one needs in this situation. It will take time for your drugs to get going, so keep at it. Best to you.

[jlove]

Welcome to the gang! "Wtf" pretty much sums up this disease. It manifests in different ways in everyone, so no two Wegener's stories are the same. One of the most important things for me is having a rheumatologist I trust. It sounds like you're in good hands with your rheum based on what you said. When I told my previous rheumatologist that I was having trouble breathing, he brought me into his office and explained basic physics to me. If a pipe has a small diameter, less liquid can flow through it. Duh. So basically he was saying "tough luck, your throat is narrow and life is just going to be hard for you." I was also 21 at the time and had just taken college level physics. I still get angry thinking about it. As soon as I left his office, I cancelled my follow up with him and never saw him again. My current doctor, on the other hand, came with me to get a CT scan because he felt it was urgent. The staff at radiology asked me if he was my dad.

Going through a flare is not fun but there are times when I feel good. The lows can be very low but they will really make you appreciate the periods when you feel good. Like others have said, blood work and urinalysis are important. The best thing you can do is learn as much as you can and ask any questions you have here. This site has been so helpful for me. Our doctors know a lot but there is a real wealth of knowledge here in all of the people who have gone through similar experiences and know what has worked for them. Best of luck to you and, again, welcome! :smile:

[Psyborg]

Welcome to the forum. Feel free to vent...we've all been in the same place to varying extents.

[Allisonquast]

I like wtf too! Sums up it up nicely! I would keep fighting to get started on meds as soon as the rule out infection. And as Rose says get them to keep an eye on your kidneys. I hope you get relief soon.

[newbie]

Thank you, everyone!
They've done urine and blood several times. I have tried making sense of it, but it is difficult. My sed rate was only 17 and my most recent urine seemed better than the previous two. the docs said no sign of kidney involvement. The whole thing is overwhelming and confusing. Especially the solitary mass....I can only find anecdotal evidence of that with Wegener's. It seems lung involvement presents as multiple nodules not solitary, large masses.
I am seeing another rheumy tomorrow morning for second opinion and I am still waiting to see a pulmonologist.
I am not in complete denial, I know something is wrong. Some days I can't even get out of bed. Two days ago, out of the blue, my right shoulder was in such pain, I couldn't lift my arm. Today, it's my knee. And, my coworker asked me what was wrong with my hand today, I looked down and my left hand was white and bluish(it was cold in the office)...again...wtf??? I feel like a stranger in my own body.
I have these:
Antimyeloperoxidase (MPO) Abs
<9.0 U/mL 0.0-9.0 N
Antiproteinase 3
(PR-3) Abs
94.4 H U/mL 0.0-3.5 N
Cytoplasmic (C-ANCA)
1:80 H titer Neg:<1:20 N
Perinuclear (P-ANCA)
<1:20 titer Neg:<1:20 N

[mrtmeo]

Thank you, everyone!
They've done urine and blood several times. I have tried making sense of it, but it is difficult. My sed rate was only 17 and my most recent urine seemed better than the previous two. the docs said no sign of kidney involvement. The whole thing is overwhelming and confusing. Especially the solitary mass....I can only find anecdotal evidence of that with Wegener's. It seems lung involvement presents as multiple nodules not solitary, large masses.
I am seeing another rheumy tomorrow morning for second opinion and I am still waiting to see a pulmonologist.
I am not in complete denial, I know something is wrong. Some days I can't even get out of bed. Two days ago, out of the blue, my right shoulder was in such pain, I couldn't lift my arm. Today, it's my knee. And, my coworker asked me what was wrong with my hand today, I looked down and my left hand was white and bluish(it was cold in the office)...again...wtf??? I feel like a stranger in my own body.
I have these:
Antimyeloperoxidase (MPO) Abs
<9.0 U/mL 0.0-9.0 N
Antiproteinase 3
(PR-3) Abs
94.4 H U/mL 0.0-3.5 N
Cytoplasmic (C-ANCA)
1:80 H titer Neg:<1:20 N
Perinuclear (P-ANCA)
<1:20 titer Neg:<1:20 N

With the high C-ANCA and PR3, it sure looks like weg's.
Vasculitis lung nodules can shrink and enlarge back n forth like waxing and waning of the disease activity, so don't wait on it.
The quicker you can get into remission, the better.
I don't know if a Rheumy would treat you without a formal biopsy for confirmation, but you should check.
Many have limited vasculitis, limited to one organ and usually get treated with immunosuppressants that are not as toxic as Rituxan and Cytoxan.
Personally, I would never let a dr do a lung biopsy for confirmation of vasculitis due to the damage that's caused from such an invasive procedure.
Lung biopsies are rarely conclusive.
Don't let this go too long.

[Rose]

Thank you, everyone!
T Especially the solitary mass....I can only find anecdotal evidence of that with Wegener's. It seems lung involvement presents as multiple nodules not solitary, large masses.
I am seeing another rheumy tomorrow morning for second opinion and I am still waiting to see a pulmonologist.
I am not in complete denial, I know something is wrong. Some days I can't even get out of bed.
<1:20 titer Neg:<1:20 N

Hi
I would just like to comment that whilst multiple masses in the lung are common with WG, solitary masses in the lung can and do occur with WG being the underlying cause. There was someone here a few months ago with a lung cancer diagnosis (a solitary mass) and was told the same thing. Other symptoms he was experiencing were indicative of Wegeners and many of us on the forum suspected that WG was what was going on and not lung cancer and this turned out to be so. Sorry I cant remember the name of the poster but maybe he will see this and comment or you could put lung cancer (many of us had that initial diagnosis) in search and it may come up. Good luck!

Rose

I should have mentioned that I have been told on 3 different occasions that i have lung cancer. 1) Initial diagnosis March 2008 2) Again when my new Rhumy sent me for a check up Xray about a year after diagnosis in 2009 and the report came back that a mass was seen. I had an MRI scan a few days later which confirmed lung cancer. She made an urgent appointment with my Pulmonologist. Previous xrays and scans were reviewed and I was told it was scar tissue. 3) 2013. After a scan of kidneys, lung cancer (single mass) was seen in lungs. This time the Pulmonologist was worried as there were some changes but it was determined after reviews of previous scans and xrays by senior radiologists that it was scar tissue again.

[mishb]

Hey Newbie, welcome to this amazing forum.

I'm sorry I'm late in saying hello - I have many many threads to catch up on.

I remember those roving pains very well - 3 days on with one limb and then 2 days off with no pain, then 3 days on with a totally different limb/joint and then 2 days free again. Crazy.
Yours certainly sound like WG

Best of wishes and good luck tomorrow

[XnCelt]

So, Rose your masses started to shrink within days?...how was that confirmed? Did you have a repeat CT scan that quickly? Drs at the UPenn GPA Center told me that it takes a couple of months for the Rituxan to work...the prednisone eliminated my symptoms overnite but that was more likely due to its anti-inflammatory affect. Hopefully the immusonsuppesing properties of predisone have also slowed the progression of the disease.

Roger/XnCelt/Phillie

[Rose]

HI

It wasn't a repeat CT scan. It was just a plain old chest x ray. I had chest x rays on admission (full of golf sized masses and I had no symptoms) and then a CT scan of lungs followed by a kidney biopsy. When the result of that confirmed WG treatment began. I had though developed a pneumothorax caused by the fine needle lung biopsy and so they were keeping an eye on that. The day after the completion of solumedrol 1000mg x3 days and just before commencing IV cyclophosphamide an x ray was taken to check on the pneumothorax and a delighted doctor came to my room to show me the x-rays and how the masses had shrunk. I obviously was not in remission after 4 days but the masses had definitely shrunk and that was with the very high dose prednisone over 3 days alone. IV cyclophosphamide commenced the following day. The masses in my lungs continued to improve. I am though left with scar tissue. The next scan I had after diagnosis was about a year later when I had a follow up chest x ray and lung cancer was Reported to have been found but it turned out to be scar tissue.

yes I believe it does take a few months to knock the immune system into submission with RTX /Cyclophosphamide and it was obviously the prednisone in such high doses that reduced inflammation in just few days and as you say, slowed the progression of the disease until the other chemo drugs, in my case Cyclophosphamide IV, kicked in.

Rose

[newbie]

Hey there! Thought I would check in. I'm on week 5 of treatment. I had a little setback in the form of sinus infection. My rhuemy prescribed antibiotic and while on it, I caught a virus that was making its rounds through the family. I've kicked them both now and am feeling better. Back to the clear, sticky stuff from nose and chest. Yay? Lol
I am up to 7 pills at 2.5 mg of MTX. I max out at 8 this coming sunday. I think I actually feel a little better today. My upper gums on the left side seem to have gone numb....which is ok as it has reduced the facial pain on that side. It's like someone snuck in and gave me a shot of novacaine. Weird, but it seems weird is the new normal so...
Thank you all for everything you share on t his site. I have learned so much from all of you.....and I've much more to learn.
Blessings to all!

[mrtmeo]

That is great news newbie!
Keep us posted.