Hi all - I was wondering if anyone can give me input as to the extent of their lung disease,, i.e. as shown on CAT scan, etc. and what type of pulmonary function deficiency you may have associated with it. My husband was diagnosed with UIP on CAT scan and has 40% decreased lung function at this point. Is any treatment that you have had helping with progression? Thanks for your response. D.

Hi Doreen, I know how frustrating and scary all of this can be. First everyone's disease is different, and this is what makes it so hard to treat. I have been as low as 36% lung capacity, and now I am at about 42% thats been the best its bee in two years. The doctors tell me I have nonspecific interstitial pneumonitis ( NSIP ). I have had many different treatments some have helped a little at first but stopped working for me. Right now my lungs are stable, and my treatments are targeted for my other symptoms. Here is a web site that might help: http://radiology.rsna.org/content/221/3/583.full Good Luck, Cathy

Hi Doreen I've always been confused by the diagnosis I have for my lungs: Organising Fibrosing Pneumonia. My TLCO (think this is the transfer rate of oxygen into the blood) was 42% when I was first unwell one year ago, and last week it was up to 56% My myositis doctor told me that my lungs won't improve, or only very tiny amounts that won't be noticeable. I have noticed that my shortness of breath is worse in the mornings and then improves over the day, and it's really my muscle weakness that is holding me back and slowing me down now rather than breathlessness, so there has definately been improvement. My respiratory doctors say chest xrays show a small improvement. I was diagnosed via bronchoscopy. I had 6 iv cycles of cyclophosphamide (one per month) with high dose oral steroids, the iv gave disappointing results for me, and then I started Azathioprine and have been gradually tapering the steroids down; we are still waiting to see if this therapy is working as my CK levels have been going back up recently. I have been noticing more muscle pain as the steroids are reduced rather than lung problems. As Cathy said, we are all so different in the way this disease presents itself, that it is hard to generalise. It's very frustrating that there isn't a known therapy that will help us all, the meds and doses have to be tailored to the individual. Take care Alison

My lung disease was first diagnosed as Cryptogenic Organizing Pneumonia. I was treated with high dose steriods (Prednisone 60 mg) for 18 months. Initially it helped a great deal and my lungs were clear. Since Oct. of 2009 my lungs have been filled with "ground glass opacities." This hase been causing shortness of breath. I just returned from seeing several new doctors at the Myositis Center at Johns Hopkins University in Baltimore, Maryland, USA. These doctors are AMAZING! All they deal with are people with myositis related disaeases and those who are Jo-1 positive. If you can get there I highly encourage you to go. I am currently on Cellcept (3000 mg) and Prednisone (10 mg). As a result of my trip to Johns Hopkins (I live in Florida) I will be starting IVIG (immunoglobulin infusions) and transitioning to imuran. The doctors at Johns Hopkins were very encouraging and were quite certain that I could be in full remission in under a year's time! Check their website: http://www.hopkinsmedicine.org/myositis/ This place is the gold standard for our diseases throughout the world.

Thanks to all of you for responding - It certainly does prove what Cathy and Alison said about how individuals present with this disease in so many different ways!! Sfabela (sorry I don't know your name) that is so great that you found John Hopkins (I have also heard very good things about them). We live in New york so it isn't that far away. I am so glad they have a good plan for you. It it so important to have confidence in your doctor's. I think one of the big differences about the lung part of the disease is that with NSIP and I guess COP there is inflammation in the lungs showing as "ground glass opacities". In my husband's case, with UIP there is little or no inflammation and there is already honeycombing which is not reversible with treatment. He is currently being treated with IV Cytoxan to possibly help put off further progression of the lung disease and stablize his pulmonary function at this current decreased level. Alison - my husband was diagnosed with dermatomyositis several years ago and like you his CPK levels were elevated but they are not elevated anymore. His rheumatologist told us that autoimmune disease can morph and you can actually end up with another autoimmune disease at some point. Besides the antiJo being positive and the ANA, he also tests positive for rheumatoid arthritis and Sjorgens now. The dermatomyositis was in remission, so to speak, for about a year but then the joints became more and more painful over the past year with the start of a dry cough and mild hoarseness. I have been reading so much on this disease as prior to this I never knew about it. It is helpful that we are able to share here about our experiences. Thanks again and lets keep in touch!! Regards, Dee

Hi Sfabela I also am being treated at the Myositis clinic at Hopkins. The doctors are very caring and compasionate. I feel fortunate to be able to have these doctors working on my case. I wish you the best of luck on your IVIG treatment. keep us posted on how you are doing. Take care Cathy

It is astonishing how different each of our cases are. Still, I find it very helpful to know each story and treatment paths. Last July I tested positive for the OJ subtype of Antisynthetase Syndrome, which is AS in absence of myositis. In my case, the onset was rapid - I went from a mild respiratory virus to short of breath to barely 33% lung capacity in 8 weeks. My lungs were 2/3rds full of immune system cells and had that ground glass look. My diagnosis was Interstitial Lung Disease precipitated by Antisynthetase Syndrome. I went on high dose prednisone and 4 months into treatment added Imuran. Over the last 13 months my lungs have slowly cleared and my last chest CT showed only the lower lobes still 'ground glass' The parts of my lungs that were previously saturated in immune system cells don't appear to have a honey combed or scarred look. I am back into the mid-70's percent of lung capacity and my lung diffusion is in the mid-50's. I guess we'll have to see if time brings about more healing and I also regain that efficiency/diffusion component. Another thing that has been helpful to me is going to pulmonary rehab. It was vital in helping learn to negotiate the world with my limited lung capacity as well as regaining strength lost while so gravely ill and also from the high dose prednisone. Cathy - thanks for posting that link to Hopkins. I had forgotten about their clinic and am at the point where some research and guidance would be helpful in my ongoing medical care. I am glad to have found you all and hope we continue to find treatment options for each other. Kelly

I was just diagnosed with this disease and my TLC (total lung compacity) was at 70% I go back to my rhuematologist and pulmonologist in October and get new PFT's done. It was done a little bit. I was put on Myfortic at that time. I had just been on 60 mg of Prednisone and now I will be going down to 10 mg on Wednesday. I am on 720 mg twice a day of the Myfortic. I also have to take an antibiotic for the prevention of infection. I have had two CT scans and a broncoscopy. My last CT scan was the one that showed my lungs were getting worse even though I had stopped the Methotrexate because of my allergy to it. I wish all of us the best of luck with this disease. Jen

Jen - I'm glad you're holding on at 70%. Hopefully the meds will slow it down and you'll regain some lung capacity and function. A note about your meds: if you've been on prednisone for a while I'd highly recommend a gradual taper from 60 to 10. When I had been on 40 mg/day for 5 months, the docs suggested we go to 30 mg/day - I did the next day as they said. And it plunged me into about 3-4 weeks of body aches, exhaustion, muscle weakness and jumpiness, black black moods, etc. My body was going through withdrawals. Each time I've dropped my dosage, like clockwork I' m tired on day 3, have muscle problems days 4-7 and have a hard time with exhaustion and moods. Now I only taper 1 mg at a time (for about 10 days, then do the next drop) so to minimize the withdrawal effects and so I can still function with my kids. My body just doesn't seem to handle it otherwise. I also take Bactrim (antibiotic to prevent pneumonia) and in addition to various vitamins and supplements take 600 mg daily of a supplement called NAC that my pulmonologist recommended. It's being used in conjunction with various pharmaceuticals to treat patients with pulmonary fibrosis. Hang in there and keep us posted on how you're doing. Kelly

I am going to check out the Hopkins website as soon as I sign out Thank You

Hi: I'm so glad I joined this group. In such a short time I'm learning so much.It will been over 3 years now since I found out I had A.S.S.. I have had all the tests and surgeries. And I feel pretty good. One of the problems I have had is not knowing anyone who has this condition. I never really understood what the doctors were saying and I guess I was afraid to ask very many questions. Now thru reading your stories it has helped me realize that I'm not going crazy. When people look at me they really don't think anything is wrong with me. It even took my partner Fred a while. (I believe he thought I was just checking out on life) My lungs are very good now. At first I was at 43% (TLC) now I'm in the 70's. My diffusion is still at 51 but the doctor doesnot believe that will change. I started at 110 ml of prednisone. now I'm at 5 and I'll be at 4 next month on the first. I still take the Imuran at 150 ml. And I had all the months on Cytoxan like most of you. Has anyone been able to cut back on the imuran? What I have read on the drug store leaflet is Imuran should not be taked if you have had Cytoxan treatment. My doctor just tells me Imuran is not harmful that even pregnant women have taken it. My hope is that I can cut back. But I still have active RA and myositis. Thanks again, Erin

Hi Erin - I am glad we found each other also - I just wanted to add something to your question on Imuran - I believe that it is like Cellcept - an anti-rejection drug/autoimmune suppressant. They wanted to put my husband on Cellcept first but decided on IV Cytoxan as a more aggressive first treatment because of his significant lung damage and then going with the oral Cellcept. I heard that Cellcept is a very safe drug and you can stay on it for a long period of time as they give it to people who have had transplants and they need to be on it forever. So maybe this is the same with Imuran. Regards, Dee.

Hi Dee: Thanks for the in put. I have my apointment next month with my doctor. So I will follow up on the Cellcept. Erin

Kelly - Your description of withdrawal from higher Prednisone dosage is right on target: "body aches, exhaustion, muscle weakness and jumpiness, black black moods, etc." It really is great to hear other people suffering from the same things, just to have the chance to recognize that these things are real and predictable, instead of random and crazy. So, thanks. I've experienced just the same set of plagues, going down from 60 (in July) to 50 and 40 and 30 and now 25 mg. (this week) of Prednisone. What I'm hoping is that the IVIG (I've just had one four-day infusion series) can eventually substitute for most of my Prednisone. But who knows? By the way, I'm "anti-OJ" too, as my doctor (and the blood test lab, taking three weeks to provide results) described it, so we're kind of rare within rare within rare, I think. I wonder what features of myositis the two of us share, which are strictly due to that 'OJ'? You would think there'd be something, right? But the rarity -- my rheumatologist estimated maybe four such instances in all of Manhattan Island right now -- means that very, very little is actually known, I guess. *sigh* Greg

Hi Greg, Sounds like they are stepping you down very quickly on the prednisone! The prednisone shuts down your adrenal glands and should be stepped down very slowly. as I have said in other posts in different topics I was on prednisone at 60 mg for 18 months. Everytime they tried to step me down when I got below 20mg it was awful and when I got to 5mg I could barely function. I was being stepped down at 5mg per month. Right now I am on 10mg with 3000mg of cellcept, and start IVIG next week. I hve read a couple studies from Italy where patients on IVIG and cellcept achieved total remission in six months! That is what I am hoping for. Since you are in New York I encourage you to go to the Myositis Center at Johns Hopkins Bayview Medical Center in Baltimore. Dr. Lisa Christopher Stine (rheumatology) and Dr. Andrew Mannen (neurology) review all the cases. I saw Dr. Sonya Danoff (pulmonologist) and Dr. Thein Nguyen (neurologist). They were AMAZING!!!! You can make an appointment online and they will check your insurance for you. It really is worth the trip. I live in Florida and no one near me had any idea of what was happening to me. Luckily I was referred to a great rheumatologist in Miami who took one look at me and said you need to go to Johns Hopkins ASAP. I am also very fortunate to have superior insurance and a sister who is a nurse practicioner who goes to every appointment with me. Go to Baltimore you will not regret it.

Greg - I would love to know if your rheumatologist has any referrals In Oregon or Washington! I have only been treated by pulmonologists so far but the next step for making decisions about long term care will need to involve a rheumatologist. I am desperate to find one who has seen ASS, especially the OJ subtype. I do not have any other autoimmune disorders nor do I have any typical ASS symptoms/signs other than losing 70% of my lung capacity (and that's enough!) So for me it has all about lungs and tons of side effects from the treatment. I did have lots of joint pain as I was first getting sick and then for most of the last year had incredible muscle weakness - so I was very worried about myositis. But as we have slowly lowered the prednisone much of my muscle strength has come back. (weakness is another side effect of prednisone) Still don't have much muscle endurance (due to prednisone) but I am working with my PT at Pulmonary rehab to increase that. I am intensely curious and hopeful about the treatment you are trying. Please continue to let me know how it's going. And I'm with the others on the lowering of prednisone... clearly the docs have never been on long term, high dose prednisone when they suggest lowering dosages by 5 or 10 mg drops. It's too much for our precariously balanced systems and can cause so many other problems. I can only tolerate dropping 1 mg at a time and no closer than 2-3 weeks together. Kelly

I started on 60 mg of Prednisone and am tapering from 20 to to mg this week. I don't know what I will find out it October when I go back to my rheumatologist and pulmonologist. I also have more PFT's. I go the University of Wisconsin Hospitals and Clinics for my treatments. I can't wait to get off or to as low a dose of prednisone. I have had trouble sleeping and I have gained 10 pounds since May. I had recently gotten down to where I should be at for my weight. I wish we had a way of connecting all of our secialist doctors together so that they can compare treatment options.

Kelly and Cats_36 - My rheumatologist, who prescribed the Prednisone, and started me at 60 mg. daily in early July, was extremely hesitant to go the "steroid" route and did so only after myositis began to appear so likely as a diagnosis that he would proceed. Muscle weakness and weight loss were the really alarming symptoms, accelerating through May and June, after an April and May preoccupied with what had seemed pneumonia, and brought me a pulmonologist referral. Both the pulmonologist -- Dr. Brian D. Gelbman -- and the rheumatologist to whom he referred me -- Dr. Stephen J. DiMartino at Hospital for Special Surgery -- have been exceptionally kind, helpful, imaginative, and attentive. CT scanning has been a feature of both their approaches, and they are still collaborating on my case. In fact I see Dr. Gelbman Friday (in two days), to go over the results of abdominal, pelvic and chest imagery developed at Hospital for Special Surgery last week, with contrast agents (swallowed and intravenously delivered). I don't know what the report on that imagery contains, but he said we would have a chance to compare the imagery to CT-scans he had ordered over the last few months. I won't be seeing Dr. DiMartino till after my second round of IVIG -- the consultation is set up for Sept. 24 -- but he's reachable at (212) 774-7016, a line often answered by his office assistant, Devon. Dr. DiMartino is an MD-PhD, by the way, whose "retired" mentor, Dr. Kagan, still teaches there at Hospital for Special Surgery, itself connected to the Weill Cornell School of Medicine, a unit of New York Presbyterian Hospital. I would be very surprised if Dr. DiMartino's office were unable to offer you West Coast referrals -- especially since almost any researcher would probably love to get her hands on an "anti-OJ"! Good luck, keep me informed... Greg

to Cats36 and all - my husband will also be going for complete pulmonary function testing in October after he receives his 3rd IV Cytoxan treatment. I am curious if there will be any changes. He is on a very low 10 mg. dose of prednisone which his rheumatologist just recently put him on to take the edge off his RA symptoms. He was always hesitant in the past about taking this at all!! I have noticed that even on this low dose, he has gained some weight (I certainly do not mention that to him though). I heard that when you reach about 10 mg. and you want to taper further down from the 10 mg., you have to withdraw very slowly - even only 1 mg. at a time. I thought all who were diagnosed with antisynthetase had positive anti Jo1? Greg - I am very interested to know what your CAT scan shows. Please keep us informed. When my husband was first diagnosed with dermatomyositis several years ago, he went to the Hospital for Special Surgery - I don't recall the name of the doctor. His rheumatologist at the time wanted him to get a second opinion there. Regards to all, Dee.

Dee - What Kelly and I seem to have is anti-OJ, not the more "common" anti-JO. Unless that was a typo from Kelly. But I don't think so. What I understand is that there are some dozen distinct serological markers for anti-synthetase syndrome, the "rarest" of which is anti-OJ. And even more confusing, the "anti-synthetase" diagnosis does not seem to be restricted to polymyositis, or to dermatomyositis, or to Inclusion Body Myositis -- it would appear to cross those otherwise significant boundaries. As I get the picture, without having done much research, the syndrome involves a symptomatology of muscle weakness, lung involvement, swallowing difficulty (certainly in my case), and risk of several other specific ailments and disabilities, mixed and matched over time in individual patients. All this while the immune system functions quite well in many areas where it is not impaired at all, such as responding to pneumonia vaccine and flu shots -- both of which I've received in the last month. The CT-scan imagery (and report) I'll be going over on Friday was developed as part of cancer screening, itself indicated as pertinent to polymyositis sufferers in somewhat greater proportion than to "normal" population members. Or so they tell me. Sleeplessness was a huge part of my reaction to the higher dose Prednisone, and has gratefully left me since I got down below 50 mg. I was getting two or three hours a night on 60; now, on 25, I routinely sleep seven hours or so, sometimes longer. It's made a huge difference in my mood and my general sense of well being. Greg