interstitial lung disease

[suzan54]

I have undifferentiated connective tissue disease, Sjogren's and fibromyalgia.  My HRCT shows ground glass opaques and several small nodules, one possibly calcified. My DLCO is 77% (it is decreasing), and I cough and am short of breath on exertion. I am told I will need to go on a more aggressive form of therapy to try and preserve my lung function.  Presently I am on Plaquenil.  Cellcept or Immuran have been suggested.  My question is how do they know when the time is right to initiate this treatment?  How dangerous are these class of drugs?  Will I have to be cautious in public?  I also had breast cancer with a mastectomy for Stage I, high grade with necrosis, 7 months ago.  Thank you.

[National Jewish Health]

I am very sorry to hear of your health problems.  I believe that you will find the following, from Firestein’s:  Kelley’s Textbook of Rheumatology the 8th Edition, of interest, although much of it may already be familiar to you.
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UNDIFFERENTIATED CONNECTIVE TISSUE DISEASE
Rheumatologists frequently see patients who present with a weakly positive ANA test and nonspecific symptoms such as arthralgias, fatigue, and cold sensitivity. The critical question in such patients is whether they will develop a connective tissue disease or whether they have fibromyalgia.[50] The answer to this question is not always straightforward, because fibromyalgia is not a diagnosis of exclusion,[51] and it is a common comorbidity with well-defined connective tissue diseases.[52] In the early stages of a connective tissue disease, there may be only one or two suspicious clinical and laboratory features, and a definitive diagnosis cannot always be made. In such cases, a working diagnosis of UCTD may be appropriate. [1] [53] Most patients with this diagnosis have Raynaud's phenomenon with or without an unexplained polyarthralgia and a positive ANA test.[54] A 5-year follow-up study of 665 patients with UCTD reported that only 34% developed a well-defined connective tissue disease: RA, 13.1%; Sjögren's, 6.8%; SLE, 4.2%; MCTD, 4.0%; scleroderma, 2.8%; systemic vasculitis, 3.3%; and PM-DM, 0.5%.[2] The highest probability of evolution into a well-defined connective tissue disease was within the first 2 years after onset of symptoms; a complete remission of symptoms occurred in 12.3%. Similar findings have been reported in other series.
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You ask, “how do they know when the time is right to initiate this treatment?”  I believe the answer to this would be, when there is evidence of progression of the disease, as with your lung signs and symptoms and pulmonary function tests (PFTs), or signs or symptoms of other organs, and/or when the disease is no longer “undifferentiated”.

I have had no personal experience with the medicines you mention but they carry the risk of opportunistic infection and an increased risk of lymphoma and skin cancer, albeit still small at 1% or so, in addition to the common side effects of pain, fever, and headache.  The risks and benefits of these medicines should best be discussed with an experienced rheumatologist, one quite familiar with these medicines.

I very much doubt that you will have to be cautious in public, except to the extent of avoidance of individuals you know to have acute infectious disease, especially respiratory disease.

The best advice I can give you is that you work with an empathetic, experienced rheumatologic who you trust and with whom you have good rapport.

Good luck.

[Reeda22]

Hello

I am sorry to hear all that you have...My sister has Lupus and they have mentioned connective tissue disease but really are not sure as she had her Gallbladder removed 17 years ago and the Doctor who did the surgery shot more clips then needed, so she has many clips that are just floating and they dont know if this is causing some problems.. Do you have Lupus, usually Sjogren's is secondary to Lupus....My sister is on Plaquenil for some time now and she is a beautician who has not had to make any changes in public but it is always a precautionary for her NOT to be around ANYONE who is sick.. Not sure about the other meds you are on but whatever you do just be sure to wash your hands ( a lot) and if you know someone is sick try to stay away or ware a mask if you have to be around them.  

I hope this helps a little, I know being sick can be so frustrating especially with these medications that supress your immune system.  Can be a scary thing..I know she cant just stop the meds either, she needs to ween herself off when its time so just something else you should know...If you have not been diagnosed with Lupus I would have them check again.  I know there is no exact test but I believe SSA? SSN? may be the test they should do...I was diagnosed with FMS and they prescribed Lyrica but for me it made it soo much worse but it may help you...I have other issues that could have caused the Lyrica not to work..I wish you the best and pray for your health..Stay strong and NEVER own your disease...and do your research as we are best as our own Doctors.  If you are ever prescribed a medication you are not comfortable taking, dont.  The great thing about the internet is there is so much information with the touch of your keypad...1 more thing, you could call a pharmacist and ask them about any meds you are on and about the ones they are talking about...I call mine all the time before taking anything, they know more about medication than anyone!!