Polymyositis and mito??

[kass]

Dear Doctor,
33yo healthy female till April of this year. Psoriasis(05/01)/arthritis(06/01)/partial lung collapse(07/01)/leg contractures and weakness(07/01)/dx'd with Psoriatic Arthritis(08/01).
On August 9 admitted to hospital: CK 1200, CK-MB 55, Myoglobin 811, myocarditis, elevated liver enzymes, enlarged liver, clotting problems, lower body weakness, chest pain,  shortness of breath. There 9 days for IV fluids and muscle biopsy. On discharge numbers were CK 1100, CK-MB 48, Myoglobin 711, ALT and AST elevated. Started on Prednisone, Lasix and Potassium. Muscle biopsy showed inflammatory myopathy, most likely Polymyositis, but signs of Dermatomyositis also. Readmitted August 27 because of tachycardia, chest pain...numbers CK 900, CK-MB 55, Myoglobin 811 (this was 13 days into Prednisone, 10 @ 60mgs, 3 @ 55mgs). Cardiac catheter normal. Kept me 2 days for fluids. Numbers at release (CK 800, CK-MB 45, Myoglobin 715).

Questions:
1. Is elevated myoglobin at these numbers (or at all) associated with PM/DM a rare finding?
2. My research indicates that the myoglobin numbers generally fall quickly and yet mine are staying very elevated. How much at risk am I of kidney problems with the numbers remaining this high? Is this indicative that there is still major muscle breakdown happening?
3. Would this be considered Rhabdomyolysis?
4. My case is being referred to as "severe" via pathology...what does this mean for prognosis?
5. I have two children diagnosed by Dr. Cohen with Mitochondrial Disease. What are the chances that my issues are not somehow related?

Thanks so much

[CCF-Neuro-M.D.-JT]

You have a lot of legitimate concerns regarding all that's happened to you in such a short time. However, I think it's best that you sit down with your doctors and talk about what's going on face to face. I can understand how lab numbers can be frightening when they're persistently abnormal, especially when you don't completely understand their clinical significance. This is why you should talk to the physicians who know you and your case, so that they could explain in detail what everything means in each particular setting. There are treatments out there for your condition, so hang in there.

[kass]

Thanks so much for the response. I had a ton more I wanted to share but was limited by the text limit and didn't want to overwhelm.

Thanks for helping to clarify some things for me. I do appreciate the inability to comment specifically on the case, so am grateful for just some additional guidance in the technical aspects of things.

I have searched high and low and talked with as many other PM/DM patients I can find and had yet to find anything other than a clinical correlation between it and the myoglobin....in other words, I could find where its said that it CAN happen, but no case histories or others with the same finding. I am assuming its atleast a rare finding, would that be right?? Does it mean anything significant?? It happens to be one of the findings that has me the most concerned right now and not being able to find any information that gives me some real insight into its clinical significance, both just being present, as well as being elevated for so long, is distressing.

There was evidence of necrosis on the muscle biopsy, specifically it says: "The fiber size variability is accompanied by a spectrum of acute myopathic changes, including myofiber necrosis, myophagocytosis, and regenerative change. No fibrinoid necrosis is present, however." For our conversation here, I guess what I am trying to understand is what the differences are between myoglobin being elevated in the blood, myoglobinuria and rhabdomyolysis?? Where one turns into the next, and in my case, where an elevated myoglobin would become rhabdo??

I appreciate a great deal your comments here. I do intend to likely get in to see Dr. Cohen myself when we return with the children early next year. In the meantime, I'll be seeing the local MDA neuromuscular doctors here this coming week and am hoping to feel like we are following the treatment protocol best fitted to my case. I am unfortunatly continuing to lose strength and abilities right now, so feel like we are atleast currently not working with one that is appropriate.

Thanks again so much for your response, and for any further information you can provide.

[CCF-Neuro-M.D.-JT]

I'm sorry to hear about all of your recent medical problems. You've given me some lab numbers and bit of clinical info, but without personally reviewing your records and examining you, I can't give you accurate medical information regarding your particular case. ALso, every lab is different and I don't know what the normal ranges are at your particular lab. However, I'll do the best I can to answer your list of questions:
1.No, both polymyositis and dermatomyositis are associated with elevated levels of myoglobin.
2.The rate of myoglobin numbers coming down depends on the clinical setting. In heart attacks, they come down relatively quickly, but not always so in muscle diseases. Your levels of myoglobin should start to come down when there is adequate treatment of the underlying disease and normal kidney function. Low potassium can also cause high numbers. Persistently high numbers can cause renal problems, but the entire clinical picture has to be taken into account to predict your risk.
3.I cannot diagnose rhabdo without clinically examining you or looking at your urine with special studies. Also, the biopsy often is able to pick up muscle necrosis on pathology if there is rhabdo, but not always.
4.Severe disease requires more aggressive treatment with immunosuppressants such as steroids if you indeed have ONLY inflammatory myositis and nothing else.You may also need chemo agents to syppress your immune system even further.  Only time will tell how you will eventually respond to these treatments.
5.With all of your other problems:liver, heart, clottin system, respiratory in addition to your muscles, I am concerned about mitochondrial disease. This could certainly explain all of your multi-system organ disease. If Dr. Cohen already sees your kids, it may be of benefit to schedule an appointment with him for yourself as he is the CCF expert on mitochondrial diseases.  If he thinks it's just an inflammatory myopathy, Dr. Shields and Dr. Levins in the neuromuscular dept are outstanding physicians also. Best of luck.