Poor Long-term Clinical Outcomes & Health-Related Quality of Life in Dermatomyositis & Polymyositis

Dermatomyositis (DM) and polymyositis (PM) are rare autoimmune inflammatory myopathies characterized by muscle weakness and multiple extra-muscular manifestations that have detrimental impact on patients’ lives. Despite various therapies used in clinical practice, a large proportion of patients do not achieve sustainable remission. There is a need to better understand the clinical and humanistic burden of DM and PM. This presentation is to review current data on the long-term disease outcomes and the true impact of DM and PM on patient’s health-related quality of life.
This presentation is sponsored by Kezar Life Sciences and presented by Dr. Rohit Aggarwal, MD, the Co-Director of the Myositis Center of University Pittsburgh. Follow Dr. Aggarwal on TH-cam ‪@Myositis101‬
View the slides and additional resources about this webinar on our website at understandingmyositis.org/poo...

[@iloveshaakah]

Excellent ! I will watch this again and again.❤

[@swfloridagal]

Thank you Dr Aggarwal and MSU for helping us living with DM and ILD and Lupus and AsyS for all you do to support us! I am getting Benlysta IV and IVIG monthly and take hydroxychloroquine and plaquenil. I've learned so much from listening to you. Thank you!

[@angeliathao96]

Deepest gratitude for this presentation. Updated information in regards to this rare autoimmune disease is empowering and helping build trust in conventional medicine. I am 3 months living with DM and I am experiencing muscle pain, muscle weakness, fatigue, brain fog, dysphagia, clinical rashes, and currently systemic itchiness. Currently only on prednisone treatment for one week now and it has helped relived swelling and some muscle pain. Looking forward to remission and god bless to all.

[@Lost-In-Blank]

48:00 As a patient who has also worked at insurance companies and governments in my career, early use of IVIG saves money if it allows the patient to continue working. The cost of all these drugs is nothing compared to society's cost of supporting people who have been disabled by inadequate treatments.
In my case, I think it was that the science wasn't there yet, the necessary drugs didn't exist and the clinical trials had not been run in time for me back in 2006.
But now, denying people with on-going active DM affecting their muscles IVIG because their disease is "not advanced enough" is just plain stupid. The first choice should be to keep patients healthy and productive.
Waiting until the patient is too damaged to ever do meaningful work again is wasting taxpayers and insurance company dollars. Fix the patient up and get us back to work while that is still possible.
The prime objective of these expensive drugs should never be to allow lengthier stays in nursing homes, no matter what the short-sighted bureaucratic medical professionals that work for Canadian medicare and world-wide private insurance companies think.
I believe, the only valid medical reason to not give an effective drug to a patient is that the drug would be too dangerous to be worth the risk in that patient. And that happens with the drugs we take for DM/PM/NDM. So many have black box warnings and so many are only allowed to be prescribed by specialist physicians experienced with the specific drug.
That is my opinion. I'm not a medical professional, but whether money is best spent on using drugs to keep people healthy, productive and contributing to society (and taxes) or to warehouse them in nursing homes is a political discussion anyways. If our doctors say the drugs are safe and likely effective for us, I say the correct political decision is use them early to keep us productive.

[@Lost-In-Blank]

Thanks you MSU and Dr Aggarwal!
And they Myositis 101 videos are excellent.

[@heatherr1141]

Thanks for recording this. I have seen a lot of Dr Aggerwal not sure how to spell that but alot just f his videos and learned alot. Thank you

[@clivenayler6689]

Really useful presentation. Now 3 years with DM/ILD/Adrenal Supression in and still learning! Is it possible to get a copy of the slides as a pdf?

[@Lost-In-Blank]

I have had anti-HMGCR necrotizing myopathy (NAM) since 2006 at age 52. Finally, after all these years I was able to get into drug-induced remission for over 6 months now by adding Xeljanz to my IVIG, tacrolimus, and prednisone regime. My pred is now tapered down to 5 mg/day.
I had tried prednisone plus at different times all of the following: methotrexate, azathioprine, methotrexate+azathioprine, mycophenolate, cyclosporine C. And then prednisone+IVIG plus: cyclophosphamide, leflunomide, rituximab, and tacrolimus. These were sometimes able to get my CK down from the 2,000 to 10,000 range to under 350 for a while, and then it would go back up.
The Xeljanz showed definite improvement within 2 months and my CK dropped to 172 (which is the upper reference range) by the third month. I'm still on IVIG at 2g/kg ideal body weight and 1mg tacrolimus twice daily.
IF you have anti-HMGCR myopathy, my recommendation as a fellow patient with no medical training is that you ask your specialist about IVIG and if IVIG alone is not enough, IVIG + Xeljanz. Feel free to take along a printout of this comment.
Xeljanz is currently very expensive, almost as expensive as IVIG. Your insurance company, even Canadian medicare, will want you to have tried numerous other drugs first. But if none of those got you into remission, ask if adding Xeljanz to IVIG will work for you, at least for a 3 month trial. Pfizer provided samples to my doctor so he could trial me for 3 months before seeking approval from medicare here.
Again, I'm just a fellow patient and I have no medical training. I'm retired and do not own any shares of any company.

[@Lost-In-Blank]

Ironic that the same Pfizer that makes Xeljanz also makes the Lipitor that caused my very rare case of life ruining anti-HMGCR myopathy. My opinion based on my experience.

[@Lost-In-Blank]

I had to ask my rheumy to get my autoantibodies tested. These tests didn't exist when I was first diagnosed, and even now they are still a bit expensive. They key to eventually getting him to agree was pointing out that now the autoantibody we have can influence the choice of drugs. So it would influence what drugs we would try next in an attempt to get me into remission.
I asked nicely every 2 years until eventually the price of tests came down and their usefulness increased enough that he agreed.
I've asked newly diagnosed patients here, and autoantibody testing is still not something automatically done in my part of Canada. You have to ask about it and make a case to justify it.

[@lesleygraham5927]

I have refractive illness, and I have had Ritux treatment I've been doing so well, I was diagnosed with ILD 8 years after my initial illness, I have non-statin induced NAM which was initially 11 years extremely aggressive, I didn't get ILD until 2017, as I got a bad round of asthma, they did some scans and saw the start of the ILD..

[@mahmamaa]

I have DM and no muscle weakness, but I have pain in all my joints from time to time plus Gottron's papules

[@Lost-In-Blank]

50:30 Has Dr Aggarwal had any DM or PM patients with fibromyalgia-type pain which respond to fibromyalgia-type treatments but not narcotics? Like Cymbalta, etc?

[@beverleysprague9163]

I was the first juvenile to get DM and 2nd person diagnosed. I am now 69 and I have another muscle disease that I am the first and only person with this disease. Could that be a fall back of JDM