I am curious if anyone has had success with tacrolimus/prograf as a treatment to help improve their lung function. High dose prednisone works well for my lungs but as we all know, is not sustainable year after year. We have tried adding Azathioprine, Cellcept and IVIG with little luck. We are now considering Tacrolimus. Forgive me for not exactly having the right language to explain but the thinking goes something like this: Azathrioprine and Cellcept target the B cells and have not been very effect for me. We could try Rituximab but that is a B cell approach too. Tacrolimus goes after the T cells. The cells gathered from my past brochiolavages have been predominately T cells. So maybe, for my particular case, we've not been able to keep things under control because we've been targeting the wrong part of the immune system. What have been your experiences with tacrolimus? I have the OJ subtype of AS causing Interstitial lung disease and 3 years into this developed weakness in my hands and forearms - was subsequently diagnosed with dermatomyositis as well.

Hi Kellb. I have not taken the tacrolimus, but have used the other meds. I was at my respirologist yesterday, andam very pleased to say my lungs are doing great!! they are up to about 80% of normal, whih is wonderful for us. I was down to 10% when diagnosed. What has been working for me is Methotrexate (30mg once per week) 25mg prednisone daily, and IVIG 3 days every 3 weeks. I am now going to try the rituximab to see if I can stop the IVIG, as although it is doing well, it does not provide me with the quality of life my neurologist would like. I do now that the prednisone is not good for long term use, however for me, I will do whatever I have to in order to have the best chance at a longer life with this. For me, the side effects of the prednisone (I have gained over 50lbs, have to watch for diabetes, etc, and have cataracts, and will surely have glaucoma as that is showing signs of starting), however this is still better then getting sicker and losing the battle. Methotrexate specifically modulates cytokine production by T cells and macrophages. I wonder if this is why it has been effective for me. It appears that this combination of medicine may target both the t cells and the b cells. I really hope they can find something that helps with your lungs. Not breathing really sucks. I will keep praying for you, and for all of us. Keep up the good fight, and God Bless. Wayne

Hi All, My husband was on Cellcept and it worked for a couple years. Then he started having a flair and was changed to Cytoxan which did not work at all. His lungs got much worse though his CK levels were normal. He was started on Prograf several weeks ago, but they are having trouble getting him up to a theraputic dose. They started him on .5 mg twice a day, he is now up to 1.5 mg twice a day. He has to have blood test twice a week to check the levels and so far his levels have been below 2.5. They want the level between 5 and 6. If he ever gets up to a theraputic level, I'll let you know how it works! Interesting about the B cells and T cells. Hubby went to UCSF last week and the doctor told us that Cytoxan and Immuran work alike and Cellcept and tacrolimus (Prograf) work alike. He felt that since Scott had success with the Cellcept, that the tacrolimus was a good choice, but was sure Immuran would not work for him since the cytoxan did not. Kelly, were you on the highest dose of cellcept? Scott's CK stayed up above 1,000 before we went to John Hopkins and they raised his does to 3 gms a day. At that time he was not having any trouble with his lungs, though his ILD was diagnosed before he had any problems with his muscles. Bev

Thank you both for responding. I do wish we could all sit down over a cup of coffee and pool our collective experiences! For the last 18 months I have been on 3000 mg of cellcept daily plus varying levels of prednisone. I managed to taper down to 8 mg of prednisone last spring but then began to develop the hand weakness and my CK levels began to rise. Like Bev's husband, I was diagnosed with ILD prior to any muscle involvement and always had normal CK results. We did catch my rising CK early (it never got about 1000) and quickly upped the prednisone to 60 mg. It slowly dropped but didn't get back to normal until after the recent rounds of IVIG (in addition to my 20 mg of pred and 3000 mg of cellcept.) Bev - Is Scott also on prednisone? My doc is recommending we increase mine to 40 mg/day while getting to the magical therapeutic level of prograf. I hope they can get Scott to the correct dosage soon. Is he being monitored by a pulmonologist or rheumatologist? My pulm doc is making the recommendation but I'm wondering who is best to monitor me. Kelly

Bev, Would you mind giving me the name of your husband's doctor at UCSF? I'd love to be able ask my doc to consult with them since our cases have similarities. Kelly

Hi Kelly, Scott is on 40 mg of Prednisone. He has actually been followed at Stanford for the past several months, but before that was at UCSF and we ate ready to turn his care back over to UCSF. But for now, It's Stanford that is trying to get the blood levels up to the therapeutic range. He is followed at Stanford by a pulmonologist, a physicians assistant in Pulmonology and a rheumatologist. Scott was hospitalized twice at Stanford, once the end of December and again the end of January. That was while he was on Cytoxan. He was also on 20 of pred. His lung function was deteriorating and the pulmonologist at Stanfprd did not want to raise his pred dose because they thought he had an infection. I believe he had more inflammation in his lungs which made him more susceptible to infection and felt like they were not acting quickly enough to help him. Finally the beginning of Febuary, with much convincing, the physicians assistant raised his dose of pred and put him on the program. He is much improved on the higher dose of pred but the pulmonologist is wanting Scott to start tapering. I said no, not until you get his steroid sparing medication, Prograf to a therapeutic level. Then I called UCSF and insisted on seeing either Dr. Collard (the head of the ILD program) or Dr Woltors, who Scott saw, instead of another doctor that Scott was assigned to when Dr Ryerson left. I really liked Dr Wolters, who explained lots of things that I did not know. We are going to change Scott's care back to UCSF. But Dr. Wolters wanted Stanford to monitor the Prograf levels until there therapeutic. The rheumatologist that he's seen we're Dr. Chaganti at UCSF and Dr. Chung at Stanford. They are both young women with young children and don't have many office hours. Sorry this is so long. Bev

One more thing Kelly. I was an ICU nurse for many years. I'd rather have Scott monitored by a Pulmonologist then a Rheumotolpgist. The muscle disease can make you very miserable, but the lung disease can kill you.

Bev, Thank you for every word of your post. It is hard enough to go through it as the patient but sometimes I feel it is so much harder on our spouses. I am so glad Scott has such a fierce advocate in you. I personally believe the only reason I am still alive is because I have fought hard to get doctors to listen to me - to trust that I know my body and when I say my lung function is going south that we have to act quickly and strongly. I know they're incredibly worried about the high dose prednisone - the risks are real - but it has saved me several times now. Time is lung function and we can't waste it. Most of my care is lead by Dr. Sherbin at Legacy Health in Portland but I have also been up to University of Washington Medical Specialty clinic to see Dr. Raghu, another pulmonologist, a few times. UWMC evaluated me for lung transplant but luckily I am still functioning well enough. Sherbin has also consulted with Dr. Danoff at Johns Hopkins on my case. I periodically see a rheumatologist but since my lungs are the main issue, the pulmonary folks are in charge. Boy you two have been through the wringer the past few months. It's so much sometimes. I hope you have an army of support holding you all through this time.

Hi Kelly, We are blessed to have great friends and wonderful family and are grateful for the support. Scott has never had bad enough lung function that they thought he might need a transplant, but it is a Physicans Assisant on the Lung Transplant Team at Stanford who is adjusting his Prograf, as the pulmonologist and rheumatologist are not very familiar with it. But it has been several weeks and the levels are still below 2.5, which is the lowest the lab test reads. Scott was on Cellcept before and Dr. Wolters at the Universtiy of San Francisco explained that even though Scott had a flair on the Cellcept that it did not mean it had quit working or that it wouldn't work again, he said that sometimes the disease just flairs and it over rides the medicine and if the Predinsone is increased for a period of time that the Cellcept could still work. That was the first time that was explained to me. I don't know what it is about some doctors that don't explain things. Their thinking may be right, but if they don't explain it in a way the patient and family can understand, which was why we changed from UCSF to Stanford. The Pulmonologist at UCSF that we had before was probably doing the right things, but either he wasn't good at explaining why, didn't want to take the time to explain things or he thought we were too stupid to get it. Dr. Wolters also explained why people with pulmonary fibrosis cough. He explained that the lungs get stiff with the scar tissue and the oxygen can't get into the aveoli where the oxgen and C02 are exchanged, and coughing is the bodies way of loosening up the stiff lungs so the oxygen can get into the aveoli. That is why people with pulmonary fibrosis cough more in the mornings when they wake up. I'm not explaining it as well as he did, but it made sense. I think patients need this kind of information and need to know they are not alone. That is why this group is so important.

I am starting the tacrolimus this week. Will stay at 20 mg of prednisone and drop the cellcept after two days of overlap with tacrolimus. Will do weekly blood work. They want me to do the bloodwork 12 hrs after evening dose and just before I take morning dose to see what the levels are at their low point between doses. Curious to see how long it takes to get to "therapeutic" levels. I'll let you know how goes.

Hi Kelly, What dose are they starting you on? Scott has been on 2 mg twice a day for weeks, and his labs still don't show a theraputic level. Now the PA at Stanford is saying she doesn't think the lab here is running it right and wants him to go to Stanford to have it done...which is a 6 hour round trip, twice a week. So he just has not been having labs done at all for about 3 weeks. He has put in several calls to Stanford and either has not gotten any call backs or they tell him they'll have the PA call him then she doesn't. I guess I need to call myself, but it is frustrating and time consuming. Bev

I am starting on .5 mg twice a day for the first 4 days, then it will be adjusted after blood work results. (Go in for that tomorrow) It sounds like he's on 4 times the dose I'm starting with. There has got to be a lab facility near you that can properly run the tests. That is ridiculous and counter productive to being well to travel so far for weekly blood work. These are the things that make me crazy about our healthcare system and detract from the small bit of energy we have each day. I hope they figure it out soon with the least amount of aggravation.

Hi Kellyb I was diagnosed with Antisynthetase Syndrome with Jo-1 antibody on 9 July 2013. I'm told I have type 1 respiratory failure, hypoxemia, interstitial lung disease, pulmonary fibrosis, cryptogenic organising pneumonia, myositis, Raynaud's phenomenon and low vitamin d. I also have as a result of medication fluid and high cholesterol. It seems I have less of a muscle problem as since starting on prednisone I have had no pain at all and regained full movement in hands, knees and back. My CK enzyme level was 673. I have serious problems with my lungs. I was heartened by weiner66's post of improving from 10-80%. I had been on 75mg prednisone for 12 days while going through tests and waiting for diagnosis. On diagnosis I was started on 5mg tacrolimus twice a day, in the hope that I can get off prednisone. When I left hospital on 12 July they reduced the prednisone to 50mg, then to 37.5mg from 19 July, 25 mg from 25 July. If I'm feeling OK I will be reduced to 12.5mg on 8 August. I had my tacrolimus levels checked and am responding well. I was at 12. The ideal range is between 5 and 20. I currently have 34% lung function. I am on oxygen 24/7. When I left hospital I was on 4.5% and have been able to reduce it to 3%. I'm told the 'crackle' noise in my lungs has improved, but there hasn't been any noticeable improvement in the X-rays, although I'm due to have more done soon. I'm still processing and trying to learn what I can about AS. I am in Australia.

BarbieRose- wow you have been through so much in the past few months. Your diagnosis experience is similar to mine (July 2009) and I was also down to 33% lung function upon discharge. I can tell you lungs can and do recover but it can take a long while. When you get a little more stable, ask about pulmonary rehab. Essentially it's exercise guided by a respiratory/physical therapist but it's tailored to helping you with your overall strength and how to do so given reduced lung function. My body sure doesn't move in this world the way it used to but I'm still moving and that's what counts in my book. I used to be tethered to 50 feet of oxygen tubing at all times. Tripping over it nearly got me in more trouble than the AS! But now I only use oxygen when out of the house. I do have to go slow up the stairs and catch my breath but I recover in 60 seconds and then continue my puttering. Don't worry about how high your oxygen setting is. You can't overdose or become dependent on oxygen. As my respiratory therapist says, whatever setting helps you feel like you have enough air is the right setting. I have a small finger oximeter I use to periodically check my oxygen saturation levels if I'm having trouble figuring out if I'm short of breath due to tiredness or low O2. Also, if you're on O2 24/7 your nasal passages can get dry and painful from always being blasted with air. A few squirts of saline nasal spray or gel will work wonders. I hope the tacrolimus works well for you. And to be on lower doses of prednisone is always helpful for overall sanity. Keep us posted on how things progress. My best to you. Kelly

OMG... Barbie-Rose...You have certainly been introduced to this crappy disease in typical fashion. Seems to be a whole lot of trial, definately some error, and a whole ton of hope. I am finally tapering my prednisone down. At 12.5 mg/day now.... There is definately hope for the lungs. What worked for me to get me to this point was regular infusions of IVIG, high doses of prednisone, and my Methotrexate (like tacrolimus). I am now also getting Rituxan, and am doing much better. I actually went to the cabin this weekend and was able to have a wonderful time, even if I do get "worn out" easier then before.Over all, knowing what can happen, I am pretty happy with my progress. Give it time...nothing really happens overnight with this, except for getting worse...lol Getting better takes time, plenty of it...but it does get better. This is a great place to come if you have any questions or just need to vent. We all understand what you are going through. God Bless, and Keep up the Good Fight Wayne

Thanks Kelly and Wayne It's good to hear some positive things, all I read at the moment is pretty dire and negative. I rather hear real stories:)

I remember reading the few things I could find about Antisynthetase when I was first diagnosed (and so debilitated) and they were bleak. Right then and there I knew that to survive this was going to require finding other patients and doing lots of my own research. Not easy to do without a medical background and when you're so sick but we've muddled through so far. I try not to look at the statistics in the medical articles and if I do, my goal is to always to end up in the more positive side of the numbers! Things have progressed tremendously just in the last four years since I was diagnosed. I think there's good reason for us to be positive and feel hopeful. Right now life as you know it has been suddenly taken away. It's an awful thing to go through. But in a little bit you will improve and rebuild your life in a new way. Be gentle with your self as you go through this journey.

A testimony (from France) of a positive story with ASS (hoping it will last !). My ASS was discovered exactly 3 years ago. I was in bad shape at that time (but never had oxygen assistance) : instersticial pneumonia, joints arthrosis, skin problems, Raynaud syndrom, muscles weakness. I 've spent most of the summer in the hospital. The first treatment was not very efficient (corticoïde +immurel) and lead to med intoxications. Things were getting worse. Doctors've changed Immurel to Cellcept and decided Rituximab injections. It works extremely well. Within 3 months, I was able to live normally again and it lasts until today. No symptom from ASS but still under med treatment. My only trouble relates to probable side effects of cortisone. As far as I know, a lot of progress have been done recently to treat or at least control this disease. Hope that BarbieRose will tell us about her progress too.

Hello to all.....Just finished a Bone Marrow Drive for a 20year old that's is worse shape then me with her Sickle Cell.....Far as myself I'm Keeping up the good fight...On 80 prednisone.,dapsone once a day.2,000 cellcept when it don't send me to the bathroom and now Infusions with benlysta-belimumab this is my first month and they wanted me to do 3treatments in this month then once a month after....Just started having weakness in my legs and hands not everyday but it is noticeable ...On the 15 of this month not sure what its called but going to get my heart check because of pain....Have not been able to go down on prednisone because of the coughing comes back so intense it makes my heart an neck hurts ...I went to John Hopkins my experience was not good so I will leave that alone. Other wise than that I created a job for myself open a taxi business so I can drive ...The only time I need my oxgen is when walking so I feel good about that.....The best to all Live Life as well as you can ...and have a little fun while you doing it!