MCTD Support Group

Mixed connective tissue disease (MCTD) or Sharp's syndrome is a human autoimmune disease in which the immune system attacks the body. MCTD combines features of polymyositis, systemic lupus erythematosus, and systemic scleroderma and is thus considered an overlap syndrome. MCTD commonly causes joint pain/swelling, Raynaud phenomenon, muscle inflammation, and scarring of...

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MCTD and PAh

dawnc4t
dawnc4t
Saw the chest doc today and he tells me I now have PAH .
I was wondering if others with MCTD had this also .I was surprised he could diagnose it without me having the right hand cath which he is organising he showed me a scan which showed a big difference in size of one artery to another he had told me previously he thought I had borderline pah I guess I will find out more after these other tests . It surprised me as for years I had reduced lung function on my tests . I'm waiting to hear from the specialist heart unit and I'm taking things as they come . Just frustrating how it just suddenly got worse with breathing and exhaustion coughing now this . Thanks for listening All this site has been great support .
brunob
brunob
I have pulmonary fibrosis from mctd and I am now borderline for PAH. I've been on oxygen for about 6 years now and my lung disease is starting to progress. My docs are looking at doing another round of Rituxan to see if it can put things back into a remission of sorts.
dawnc4t
dawnc4t
Thanks for the reply it feels a lonely place I have not heard from anyone else with PAH to compare treatments with or get an insight to what to expect . I thought MCTD sjogrens and limited systemic sclerosis were enough to deal with but this new diagnosis I'm finding hard to absorb . I keep thinking the doctor has made a mistake after all we are all fallible . I haven't even had the right catheter test yet. I
am wondering if it's my fault it has progressed at a couple of stages I felt depressed with it all and stopped the azathioprine and plaquenil .
bettyeldridge
bettyeldridge
My echo just showed mild pulmonary hypertension. I am undergoing a lot of tests. My mom had MCTD and developed lung issues and she passed last year. My new pulmonary md is working hard to get all test done and see if treatment is needed yet. I am worried but there are more treatments now out there for it.
dawnc4t
dawnc4t
Hi Betty your right there are a lot of new medications in the pipeline . I
Feel more positive today I have my lung function test apt come through for Monday I am just waiting on the cardiologist apt. I have read the echo are not reliable and the right cath is the gold standard test . I worry that things are progressing fast as I am getting dull chest pain , which tends to come on if I do too much .
Hi brunobob thanks for your reply at what point did they decide to put you on the oxygen? I want them to help me with medication oxygen anything but just more and more tests love to you all and hope you guys are feeling better xxx
brunob
brunob
because I have pulmonary fibrosis from the scleroderma, I've been on oxygen for about 5 years..I just had an exacerbation of the fibrosis and have had to increase my O2 and it's now that they say there may be pulmonary hypertension to go along with all the other stuff. I will be going and having all the testing done to assess if I am ready to be listed for a lung transplant. I've been very stable for about 7 years, so all in all I can't complain, but it's still a very scary thing to go through. Good luck with your PFT.they always give me a headache!
dawnc4t
dawnc4t
I didn't make it for the pft appt . I went to the doctors to tell her about the dull chest pain over the weekend and she did an ecg which showed t wave inversions I believe. They called an ambulance and here I am nearly 2 weeks later having had a RHC . LHC and angiogram. They confirmed I have PHA with a mean pressure of 30 . I am waiting to be moved to the heart specialist hospital on Weds where they will start treatment . I apparently also have left heart problems with lesions and narrowing but too dangerous to put a stent in . I think as a heads up to others I would say never ignore what you think is a little dull ache in your chest go get it checked .