Sjogren's Syndrome Support Group

Sjogren's syndrome is an autoimmune disorder in which immune cells attack and destroy the exocrine glands that produce tears and saliva. It also associated with rheumatic disorders such as rheumatoid arthritis, and it is rheumatoid factor positive in 90 percent of cases.

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Lung disease

Does anyone have lung involvement with SS? I was diagnosed by my pulmonologist with "interstitial lung disease," but the radiologist's report (ct scan) said "idiopathic pulmonary fibrosis.". I have been short of breath for about 7 years but not enough to raise a red flag--just enough to make me feel old (62 now) and out of shape. I have not searched for information about this because I know the information will only depress me, but I'm wondering if anyone else is dealing with this. I'm a pretty active person and try to work out every day, but as you know it's sometimes quite an effort to make myself get active.

Replies

DeetyB
DeetyB

There are some previous threads that I read on this site that I found very helpful. I too have lung involvement that is quite painful.
I found it interesting that Venice Williams noted her lung involvement with her Sjogrens. (For years treated her for Athsma even, steroids don't help)
My doc offers me no advice but to turn the heat up on humid days. (Which only is a killer move for my eyes)
Would love to learn of a new treatment to help with this symptom.
Tom59
Tom59

Yes, I also get lung involvement which seems to get worse as time passes. Mine comes and goes last couple days its been bad. I've worked out since a child in one fashion or another.

I always have clear lungs on my rheumy visits , which is odds. Eventually the odds will be with me. :-)

Before plaquenil I got what felt like a chest cold coming on , the fever feel , the next day or so its gone. Now it seems I just get a heavy chest.....I notice it on my walks it makes me feel like a smoker. Then a day or so later nothing I feel great again. But, it seems to be more often now.


Winter seems to make it worse for me.
barbie222
barbie222

Hi GailSK,

I've also had problems with my lungs - shortness of breath, etc....I've been on advair for a couple of years...but I MUST stop taking it asap... b/c the side effects have caused numerous health problems. I just recently found out about these, so I'm a bit upset as I write this. Fortunately, I'm seeing a new pulmonologist next week... I pray he'll be able to help me.

Initially, I refused to take any meds for my breathing issues-- shortness of breath, wheezing, etc. - which was only periodically. Eventually, it got worse, so I gave in and finally agreed to try the Advair that both my GP, and Pulmonologist presribed. I was happy at first, b/c after the 2nd dose, the severe swelling I had in my ankles and feet disappeared. This was obviously due to the 'corticosteroid' in the Advair. However, a few months later, when I saw the Rheumy for the 1st time, he told me to stop taking it, as it causes cataracts--- which was confirmd a few days later by an opthamologist. He said cataracts are highly unusual at age 52, also blaming the advair. So I stopped taking it suddenly, as my GP told me to do. I started using an inhaler...but it didn't work as well as the advair, so I began taking it again- but only on an 'as needed' basis. I regret this tremendously, b/c I've now been diagnosed with Addison's disease. This is directly due to the corticosteroid - and possibly stopping suddenly. I later found out that you must be slowly 'weaned' off this drug, b/c it can have an adverse effect the adrenal glands, can permanently damage them, possibly causing Addison's disease. Unfortunately, my GP was unaware of this. Then, yesterday my Rheumy told me I now have 'rampant osteosporosis' as per my bone density test last month. (?) This was very upsetting.... Two years ago, my bone density test was completely normal. I just read on the package "insert' "Do not take if you have osteosporosis." I intended to write a post asking if anyone else had problems with this drug... but because of this "lung disease" post - I'm telling it here.
Of course, I'm sure it's a wonderfully helpful drug for many, many people with COPD, and asthma, etc.. But medications affect us all differently. I just wanted to inform those with autoimmune disorders, osteosporosis, etc... to be very aware of the possible side effects.

Gail, sorry for straying off the topic....It's been a rough month, I guess I needed to vent a bit. I hope this didn't depress you, and I hope you're feeling better, and your breathing problems improve. Btw, have your doctors suggested an inhaler?

Take care. =).
deleted_user
deleted_user

I was wondering what symptoms go with this lung involvment??I have been experiencing weezing and shortness of breath for some time now, i also bring up (sometimes cough-up) nasty looking stuff several times a day, everyday! it is almost always blood streaked, so does anyone else have this??P.S. im not a smoker
GailSK
GailSK

I think my first symptoms about 7 years ago were not too bothersome, just something I noticed because my father had congestive heart failure and had complained of the same symptoms. I would try to take a deep breath and when I did, I had a sensation as if something was in my lungs, and I would have to cough. This would last for a day or less, and then go away. I was walking on the treadmill daily, and I would get on it during the "attacks" and I did not notice any shortness of breath. I was afraid of heart problems, so I eventually told my internist, who ordered a chest x-ray. She said it showed normal lungs with a "bit of scar tissue," nothing to be worried about. Meanwhile, I decided since it was always worse in January when many people in our area get "cedar fever" from all the cedar pollen, that it might just be drainage from an allergy. SO, I started taking Claritin D, and I would notice improvement.

In the meantime, my exercise regimen became sporadic because I had a serious ankle problem and 2 bunions removed. I then noticed that I had shortness of breath when walking up stairs. I figured I was simply out of shape and just needed to exercise. Then I started getting repeated sinus infections. Finally, I had sinus surgery. Sometime during all this, i noticed that my mouth was becoming dry but was told it was my age. Then, one dental visit in 2009, my cleaning took 45 minutes, so I decided something was wrong. Hence the diagnosis of Sjogren's, then GI problems, then constant coughing, yeast infections, dry eyes, worsening cataracts, tinnitus, corneal dystrophy, and the host of other problems. I now go to a rheumatologist who is a Sjogren's expert. He ordered a chest x-ray which showed somewhat the same "scar tissue" the earlier one had. He sent me straight to a pulmonologist who ordered a ct scan and then diagnosed "interstitial lung disease."

So far no medicines have been prescribed, but I now have to go in for breathing tests every 6 months and will have to have a ct scan if they show any worsening of my "air exchange" problems. Then, if the ct scan shows worse problems, I have to have a lung biopsy. In the meantime, I can no longer tolerate the Claritin D because it is so drying, but I take Mucinex (extra strength, not "DM") twice a day to keep the coughing to a minimum. I also seem to breathe more comfortably when I take it.

I went to Crested Butte in Colorado in August and was miserable at that elevation. I have noticed that I'm better if I walk daily and for some reason, weight training helps. However, I'm depressed that this is an illness that is listed as "cause of death" for so many people.

Thank you, Barbie, for your information on the Advair. I seem to be susceptible to side effects, so I will beware of that one. I'm so sorry about the problems you're having. Again, this all just points out the need for more doctors to understand this disease. I'm very thankful to have my new rheumatologist who seems to be so in tune with the condition. Thanks to everyone else who replied also. Somehow is is a comfort to know I'm not alone. And I learn things--For example, I didn't know Venus Williams had lung issues too. I thought it was just fatigue!

And Tom, my pulmonary doc says the only way this can be diagnosed is through breathing tests (no fun!) and a CT scan. My lungs sound clear, too, and apparently the x-ray results look like those of most other people my age.
barbie222
barbie222

cherokee, I also have wheezing and shortness of breath, which, as Gail said, is improved with walking. Also I "bring up" phlegm, but it's not bloody - that is very worrisome, and should be checked. Perhaps your dr. could order a test so it can be examined by a lab.
Yes, Sjogren's is systemic unfortunately, and can affect all our organs.

Best of luck...Blessings to you all. :)
deleted_user
deleted_user

Tough road ahead. Seven years ago I was diagnosed with emphysema and a ct scan showed tumors in my lower left lobe. I never smoked but I've had Sjogrens since 1982. I declined a biopsy so my pulmonary doc ordered ct scans every six months until last spring he convinced me to have a biopsy. Came back positive with Non Hodgkins lymphoma. From May to August i went thru chemo and the results were negative although the vasculitis in my legs which caused a lot of leg pain went away. I now have a new rumy doc along with a new oncologist and I start new chemo treatments next week. They think the tumors are caused by the Sjogrens but can be knocked out by the chemo (Ritoxin). Sounds dreadful but all in all Life is pretty good. 30 years with Sjogrens has been trying but there are a lot of people worse off than me.
deleted_user
deleted_user

For anyone who may have heard that Interstitial Lung Disease or Pulmonary Fibrosis could be their diagnosis, please feel free to call us at the Coalition for Pulmonary Fibrosis. We can alert you to the methods by which this diagnosis is made, steer you to special centers, and generally be helpful. This is a difficult diagnosis and we are anxious to be sure you have all the information you need and excellent care. Mishka Michon, CEO, CPF
JoanneHG
JoanneHG

Hi, I also have interstitial lung disease. It was first diagnosed about a year ago and I was told that I had lost 30 percent of my lung capacity (which feels like a lot more than it sounds). I was very lucky in that my family doctor is an expert in her particular field and is very well connected - hence, when she suspected SS she got me in to the top rheumy in town (who used to be head rheumy at the main hospital) who also got me in as a patient as the head rheumy of the univeristy hospital. When they realized that there were lung complications they got me in as a patient of both the head of the pulmonary unit at the main hospital and the head of the university teaching hospital (and they used my case as a case study). I'm letting you know all of this as I am about to tell you what they told me and what treatment they used. They all worked as a close team in determining how to treat both the SS and the lung disease. First the lung doctors asked me not to research on the internet as the disease itself can be serious but this is because they often do not know how to stop it. In my situation, as it was caused by my SS, it is easier to treat. They decided to undertake aggressive treatment for both my lungs and SS. They put me on prednisone (40mg) and CellCept (2000mg) for a little less than a year before my lungs "normalized". I went for constant lung tests throughout this to ensure that things were improving. The side effects of such a high does of prednisone was not fun but it was more important to get my lung situation under control. A few months ago they started reducing the prednisone and we'll continue this followed by a reduction of CellCept on a step-by-step basis testing how I react regularly. Originally, they gave no guarantees that I would regain any of the 30 percent loss from scar tissue but it seems that my lungs are getting better and better and they expect the remaining scar tissue to be minimal. I still get short of breath, cough more than normal and have had to change hiking etc to flatter ground and lower altitutes (although the SS has changed this part of my life as well so I've had to cut out a lot of sports). With respect to regular exercise, I walk, do yoga, meditation along with breathing exercises and use the eliptical. My ability to keep up an exercise programme waivers but my doctors are constantly supporting me by reminding me how important it is but also ensuring I cut myself some slack and listen to my body. Based on my experience, it is really important to get a good lung doctor who understands SS and is ready to work with your rheumy (who should also understand SS very well). I wish you all the best. I am sure your lungs will get better but it takes some time (patience is not my virtue so this part was tough for me). Take care and keep your spirits up.
GailSK
GailSK

Thank you so much for your input, Joanne. I am similarly lucky in that I have a rheumatologist who is particularly knowledgeable about SS (writes articles for Sjogren's newsletters), and he has a pulmonologist to whom he refers. I feel very good about her skill and knowledge and have been very impressed by her knowledge of the effects of SS on the lungs, but there is another pulmonary group here in my city that is part of a university teaching/research facility that I am going to check out. My situation is not as critical as yours, and my second CT showed no progress of the disease, so I'm playing the waiting game--frequent breathing tests and recheck appointments followed by another CT if the results of the tests worsen, then by a biopsy if the results of that show further damage.
Right now, my breathing is difficult at times, but I have noticed that if I take Mucinex every morning, I feel I have much greater lung capacity and cough much less. I hope I don't have to go the prednisone route since I remember my grandmother had such trouble with that, but I have been told that is the most likely treatment. In the meantime, I'm saving your information to share with the doctors. Again, thank you so much!
JoanneHG
JoanneHG

We so seldom send each other good news on this site so I thought I would share some great news I received about my lung disease. It has not only stopped progressing but the scans show that my tissue has regenerated - something my lung doctors, my rheumy and the CT scan doctors have never seen before. They are hopeful that I will ultimately end up with no permanently damaged lung tissue (although there is still inflammation so I'll be on cellcept for another year or so but I'm getting off the Prednisone). My progress was so good that I got the thumbs up to go to Tibet (a long time dream of mine which I wanted to do while my lungs are ok - just in case the disease comes back). It was not an easy trip but it was AMAZING!!!
deleted_user
deleted_user

hey Joanne, thanks for sharing your treatment regimine. Have you been given any pft's since the beginning of your treatment regime and now and has there been improvement? Also, did you do a CT scan of your lungs before and now and do you see less damage now or is it the same? I am getting ready to start treatment at NIH and prednisone alone has not worked on me, but there has been talk about adding or trying cellcept. Anyhow, I would love to know if your pfts showed improvement and your CT's showed improvement since you started your regime. My main probem is with gas exchange. I tested in the 70's 2 years ago, and now I am in the 50's so that has all the doctors very concerned to try and find something that works very soon. My gas exchange seems to be dropping about 8% each year. Not good..
JoanneHG
JoanneHG

For almost 2 years I had routine PFTs - breathing and oxygen level tests, x-rays and CT scans on my lungs. I was first told that I had lost 30% of my lung capacity from fibroids and scar tissue and that the best we could hope for was to maybe get 5% capacity back. They treated the diseases aggressively (I was on 40mg Prednisone and 2000mg Cellcept for quite while). As my lungs improved they started to lower the Prednisone as it was also causing some pretty awful side effects (but was doing the job on my lungs). In addition to this treatment I did acupuncture to help my body get rid of the toxins of the medicines, gentle yoga every day (which was not easy at first) and I learnt proper meditation and good breathing techniques to strengthen my lungs. For a long time I couldn't walk and talk, had to limit any my activities (they essentially didn't want me doing any cardio or exercise), I couldn't even walk up one flight of stairs, carry a heavy briefcase for a short distance and I had to rewrite any oral presentations I was giving as I could not talk for long periods at once. I telling you all this just to say that it was a long and difficult road but all our efforts (the medicines and the additional stuff) paid off. As I mentioned in my earlier message my last set of PFTs showed that my lungs have now normalized and that there is no longer any scar tissue or fibroids. There is still some inflammation in my lungs and they remain weak as I need to now start strengthening them through cardio activity, hence, while I'm diminishing the Prednisone I will have to stay on the cellcept for anther year or so (just to make sure it's completely healed). The PFTs, x-rays and scans were critical to knowing which drugs were working best (I was using something else before the cellcept) and whether I was making progress. I am still scheduled to continue all these tests and anticipate that they will do so on a regular basis until I've got a final "all clear" sign off. Hope this is helpful. Cheers, Joanne
GailSK
GailSK

Thank you, Joanne, for the good news! I've been going for breathing tests and CT scans, and my last breathing test showed a little decline. My doctor is telling me to come back in December for a CT and one more breathing test, but she wants to do a lung biopsy if there is a continuing decline. Did you have a biopsy? I hear it is a hospital procedure with a 5-day stay in the hospital. I just had a dental implant, and that alone has made me feel miserable, so I'm terrified to go have that done.
deleted_user
deleted_user

Hey JoanneHG and GailSK,
I guess misery loves company. Thanks for sharing your situations. I had no symptoms of lung disease and it was only found out when they did a CT for an ovarian cyst that they caught the bottom of my lungs and found I had cysts all over them. I went to a LAM conference shortly after that thinking I could possibly have LAM because of the cysts all over my lungs. At the conference I found out there is a lung disease called sjogrens cystic lung disease. I have been connected up with a few other women that have sjogrens and cysts all over their lungs. My pft's were in the normal range when I went for my first pft's in early 2010. After about 1 1/2 years and my pft's continuing to decline, I finally did a robotic lung biospy because they just did not have the answers about what was going on and I wasn't respondind to prednisone. Althought they could not see inflamation on my CT scans, they were, with the biopsy, able to find that I had inflamation I shouldn't have there. At the same time they checked for mold, cancer and other things which all came out negative. I had my biopsy material sent to NIH, Johns Hopkins, Sloan Kettering, and Mayo. No one has seen in anyone else what I have but they have seen something similar in other sjogrens patients. I do have some signs of light chain disease of the lungs, but there was not enough of these damaged cells to classify me and treat me for that. I will tell you that when they did my lung biopsy, they fractured a couple of ribs which they told me sometimes happens. My pain after the biopsy was good while I had the morphine pump, but once I got off that, I was in a lot of pain for quite a few months. I kept trying to do my physical therapy to get better, but that seem to make my pain worse. My Johns Hopkins doctor finally told me just to take it easy and let myself heal, and she was right. After I rested for a while, I started to heal. It was later that I found out I had these fractured ribs, and in retrospect, I think that why I was hurting so much after I would exercise and do my physical therapy with the blowing tube (don't remember the correct name for this, sorry).

Anyhow, my pft's continue to fall and I am getting less and less able to do things. I still am strong. Used to teach tennis before I got feeling so crumby and feeling so worn out from doing practically nothing. But I am getting out of breath now just from walking which is a very bad sign. Again, my gas exchange (DLCO) seems to be what is dropping so fast. I have emailed my NIH doctor about JoanneHG having positive results with cellcept so I am hoping that can be part of their plan for trying to treat me. The prednisone seemed to make my lungs feel worse though. Not thrilled about doing that again (they want to put me on 60mg--did 40mg last time I did it) but the cellcept I have heard doctors talk about and my doctor in cincinnati has mentioned that is what he thinks they should try on me since I didn't get anything much out of the prednison. Hope this is helpful on the lung biopsy. Also, I did take some pain killers a few weeks before the lung biopsy to try and prevent the chronic pain you can get from having a lung biopsy. I have to say that I have soreness, but not chronic pain from it with it now being about 1 1/2 years later. So if you decide to do the lung biopsy, you might talk to your doctor about doing this. At the LAM conference I went to, I met lots of LAM patients that had had lung biopsies and had very bad chronic pain. ALso, while I was at the conference, I sat in on a seminar on lung biopsies and that is where I was clued in on taking the pain meds before the surgery as well as afterwards. They find out that patients that did this were less likely to suffer chronic pain afterwards. And another patient I met while at the LAM conference had a lung biopsy just before I did, and she did the pain meds before and suffers from no pain whatsoever from the operation. So that is something to think about.