Inclusion Body Myositis (IBM).

Site presented by Bill Tillier.

Introduction to this site:

This web page presents information on sporadic inclusion body myositis (sIBM) and hereditary inclusion body myopathy (hIBM). It is a good starting point for a person interested in sIBM and contains information suitable to take to a family physician. The site provides two levels of information, basic introductions and critical overview articles and also a more complex body of research / medical information, including summaries / reviews of some of the major scientific literature on sIBM. Note: some of the PDF files on this site are large and take considerable time to open.

For new patients:

⇒ IBM Overview
Peer support:⇒ Jim's group [This is not my website]
Peer support:⇒ Dagmar's page [This is not my website]
⇒ Treatment
⇒ Functional rating scale
⇒ Potential Complications
⇒ A page for your family doctor
⇒ IBM Details

Research:

Treatment of sIBM:

There is no standard course of treatment to slow or stop the progression of the disease. sIBM patients do not reliably respond to the anti-inflammatory, immunosuppressant, or immunomodulatory drugs that have been tried. Unfortunately, the side effects of all of the current medications far outweigh any benefits seen in sIBM. Management is symptomatic. Prevention of falls is an important consideration.

Summary: Presently, there is no therapy that has been reliably effective. A number of agents, including corticosteroids, cytotoxic-immunosuppressive agents, anti-TNF (tumor necrosis factor) agents, interferon beta, and intravenous gamma globulin, have been tried. In some cases, there have been modest gains, but overall there has been no dependably effective therapeutic approach to this disorder. However, it is possible that some patients who demonstrate marked signs of inflammation (such as markedly elevated enzymes or serum myoglobin and an exuberant infiltrate on biopsy) or those with rapidly progressive disease may obtain benefit from treatment. Many physicians, on initial diagnosis, will employ a course of therapy with careful ongoing assessment to determine whether there may be some benefit to be attained in individual cases.

The lack of success of measures designed to address autoimmunity has been suggested by some to indicate that immune mechanisms may not be central to disease initiation or perpetuation. This is an area requiring further investigation.

It is likely, however, that the presence of irreversible degenerative mechanisms, perhaps related at least in part to aging, as well as the late stage of illness at which many patients are first seen may be important factors in the poor response to therapy so characteristic of this disorder. This underscores the need for knowledge of the modifiable, pathological pathways in inclusion body myositis.

Beyond consideration of therapeutic measures aimed at pathogenetic mechanisms in this disorder, in the elderly patient attention should be paid to nutrition, physical therapy, and remediation of any existing comorbidities. Source: Kagen, L. J., Inclusion Body Myositis. In Kagen, L. J. (Editor), (2009). The Inflammatory Myopathies (87 - 94). New York: Humana Press.

A useful review by Wiendl (2008) can be found here

The current status of treatment for inclusion-body myositis.
Griggs RC.
Neurology. 2006 Jan 24;66(2 Suppl 1):S30-2.
From the Departments of Neurology, Medicine, Pathology and Laboratory Medicine, and Pediatrics, University of Rochester School of Medicine and Dentistry, Rochester, NY.
Abstract: There is no established treatment that improves, arrests, or slows the progression of inclusion-body myositis (IBM). Many anti-inflammatory, immunosuppressant, or immunomodulating agents have been administered to patients with IBM but the design of clinical trials was such that it can only be concluded that none produced rapid improvement. The natural history of the disease is for stabilization or improvement in a third of patients for 6 months or more. Thus some agents that did not produce dramatic benefit may have been prematurely abandoned. However, because high-dose prednisone worsens strength while decreasing inflammation but increases amyloid accumulation, alternative targets for intervention and novel treatment strategies are needed.

A good overview of treatment of sIBM is provided in Outlook Extra - Winter 2003, Treatment Issue, [excerpted here] available from the Myositis Association (www.myositis.org).

The use of vitamins and supplements in treating sIBM: Vitamins, Supplements.

A cautionary note: there is sometimes a strong tendency for both doctors and patients to want to do something to address the symptoms of a major debilitating and chronic illness like IBM. For patients, it can be very frightening and frustrating to simply "do nothing" and again there is a strong tendency to want to try to do something -- anything to try to slow down or reverse this illness. Likewise, some doctors want to provide treatment -- the issue is, what evidence is there for the basis of these treatments? Researchers sometimes are pressed to provide positive results in order to maintain their funding, for example, one recent study of IBM reported "significant" results but a careful reading indicates that of the 13 patients treated, half improved and half did not, but the criteria for success was only a 10% improvement and it is certainly not clear that such a modest improvement would translate into any clinical benefit for patients. A final note, muscle disorders are extremely difficult to research and study. Most scientific studies on treatments are difficult and limited because it seems clear that sIBM varies with one's age at onset. Its course is different if one "gets it" at 40 compared to if one "gets it" at 70. So, studies should control for this age difference and they don't (they usually lump in the 40 year old with the 70 year old). Patient reports of strength increases or decreases are generally not connected to objective scientific measurements of muscle strength and there can be strong placebo effects in the perception of improvements in strength.

Clinical Trials for sIBM

You can find information on the latest clinical trials on sIBM by going to this website and entering inclusion body myositis on the search line: NIH Clinical Trials: http://clinicaltrials.gov/

Web sites for neuromuscular disorders:

The Myositis Association (formerly The Myositis Association of America (MAA)) http://www.myositis.org
TMA publishes an excellent newsletter called OUTLOOK.

Muscular Dystrophy Canada / Dystrophie musculaire Canada. http://www.muscle.ca/

The Muscular Dystrophy Campaign (England): http://www.muscular-dystrophy.org/

Support groups for myositis:

The [USA] Myositis Support Group: http://www.myositissupportgroup.org
Their general sIBM discussion group is at: http://www.myositissupportgroup.org/IBM/

Homepage of Dagmar Slaven, an IBM patient. http://www.myomusings.com/

The UK Myositis Support Group: http://www.myositis.org.uk/

Web sites for IBM Information:

The Muscular Dystrophy Association of America recently put IBM under its list of covered disorders.
They are at: http://www.mdausa.org
For their page on IBM, see: http://www.mda.org/disease/ibm.html

The IBM Research Project. A research group, including Steven A. Greenberg, MD and Anthony A. Amato, MD, dedicated to understanding and finding treatment for sporadic inclusion body myositis (s-IBM) and other inflammatory myopathies. This is a multidisciplinary group, part of the Brigham and Women's Hospital, Department of Neurology, Division of Neuromuscular Disease, Harvard Medical School; the Children's Hospital Informatics Program; and the Harvard-MIT Division of Health Sciences and Technology. http://www.s-ibm.org/

ganfyd.org The free medical knowledge base that anyone can read and any registered medical practitioner may edit.

IBM overview: http://www.orpha.net/data/patho/GB/uk-IBM.html

The Muscular Dystrophy Campaign (England) IBM overview:

National Institute of Neurological Disorders and Stroke (NINDS) site on IBM:. http://www.ninds.nih.gov/health_and_medical/disorders/inclusion_doc.htm

Selected Medical Related and Research Sites:

American Medical Association: http://www.ama-assn.org/

American Academy of Family Physicians: http://www.aafp.org/

Pub Med: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi The comprehensive site for most medical research done in the world.

Medline Plus http://medlineplus.gov/ Look up anything to do with health care on this site from, prescription drugs to local resources to symptoms and diseases.

RxList http://www.rxlist.com/script/main/hp.asp RxList is "the Internet drug index," and you search by prescriptions dispensed, names searched or just by letter.

Google Directory - Health and Medicine http://www.google.com/Top/Health/Medicine/Reference/ Categories and individual web pages are listed on this Google reference site. Browse topics like health news, history of medicine, medical dictionaries or patient education.

Patient Care http://www.cumc.columbia.edu/health/index.html Columbia University Medical Center lists a number of patient resources, including tools for finding a doctor, dentist and hospital.

MediLexicon http://www.medilexicon.com/ At MediLexicon, you can use the medical dictionary search, hospital search, medical abbreviations search or read all the latest medical news.

InteliHealth http://www.intelihealth.com/IH/ihtIH This reference site has an Ask the Expert section, as well as a database full of information for diseases and conditions, from asthma to digestive issues to weight management to STDs.

Healthfinder http://www.healthfinder.gov/ This government site features a Drug Interaction Checker, a Health Library and consumer guides.

The Merck Manual http://www.merck.com/mmpe/index.html Search this online medical library for diseases and conditions and drug products.

Other Web sites:

The CIHR, the IMHA and the KETF

How to Use pdf files:

Note: some of the PDF files on this site are large and take considerable time to open. Pdf files are like a photocopy of an article. To use them you need to install a pdf reader (many computers already have one installed). If you click on a pdf file and you have a reader, it will open automatically. If you need to install a reader, it is easy. You can get a free reader download at:

http://www.adobe.com/products/acrobat/readstep2.html

Disclaimer:

I am not a medical Doctor and this information is not intended to be read as medical advice nor is it a substitute for medical advice. Please consult your Physician if you have medical concerns. I have done my best to offer a layman's interpretation of this material. Any opinions offered are personal.

Contact:

For comments or improvements, please contact Bill at e-mail: btillier@shaw.ca

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Page Created: April 06, 2001.

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