Inclusion Body Myositis (IBM).
Site presented by Bill Tillier.
|FOR NEW PATIENTS|
|INFORMATION ON IBM Johns Hopkins 2012|
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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..
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- Inclusion body myositis is a disease affecting muscle cells.
- As more and more cells are affected, the muscle becomes weaker and weaker.
- Not all muscles are affected and not all muscles are affected at the same rate.
- When you get it, exactly how it affects you, and how fast it progresses are all very variable between different people.
- In most cases, the muscles of the arms and legs are affected first and weakness in the hands and tripping are common first symptoms.
- The illness is age related; it usually occurs after age 40 and becomes more common in people as they age.
- Muscles naturally shrink and become weaker with age; this complicates research into diseases that impact on muscle strength.
- Research shows that in IBM patients, muscle power is lost at a rate of approximately 1% per month (this is only a rough guideline, a great deal of muscle loss has occurred before symptoms come to our awareness and before the diagnosis is made).
- The illness can be difficult to diagnose and is often mistaken for another similar illness called polymyositis.
- Today, researchers do not understand what causes it.
- No effective treatment has been developed yet.
- The most effective coping strategy is watching for and managing complications and the prevention of injuries due to falls.
- The illness can cause severe disability and often necessitates use of a wheelchair.
- A common serious complication is weakness in swallowing that can cause choking or aspiration (taking food into the lungs) causing pneumonia (sometimes a cause of death).
- Another serious but less common complication is respiratory impairment caused by weakness of the diaphragm (sometimes a cause of death).
- It does not appear that the heart muscle is affected.
- The illness does not appear to be directly genetic however, a complex pattern of genetics may be involved in creating either a predisposition or in protecting against the illness.
- The illness is considered a type of muscular dystrophy.
- Generally speaking, there are three main components to muscle activity; the nerves that carry signals from the brain, the junction between the nerves and the muscle, and finally, the muscles themselves.
- sIBM affects the muscles cells (properly called myocytes and also called muscle fibers).
- One of the major components of muscles are proteins and, as muscles are worked, protein is continually damaged, recycled and reproduced.
- Muscles are considered a complex tissue and how they operate is not yet fully understood.
- Here is a recent diagram illustrating how muscles contract.
- Theories of sIBM
- There are two major theories about how muscles are affected by sIBM, corresponding to the two major findings in sIBM; the finding of protein abnormalities and the finding of a strong immune response.
- One theory suggests that protein abnormalities develop that harm the muscle cells.
- One theory suggests that the immune system is involved, perhaps in causing the original muscle cell damage or, alternately, in response to damage done by protein abnormalities.
- Research continues to try to understand which of these two aspects may come first and how these two aspects may be related.
- Of course, it is always possible that some other factor, yet to be discovered, is the primary cause.
- Until the basic causes are discovered, a specific treatment regime will be a major challenge to develop.
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When faced with a progressive disabling disease that has no effective and reliable treatment options, day to day management becomes critically important to avoid complications. Management involves two critical components; awareness and prevention. We need to be aware of the possible consequences of inclusion body myositis and be able to proactively prevent complications. Simple and consistent practices can prevent many of the complications that can threaten one's life.
- The muscles in the legs are often affected and this creates a major vulnerability to tripping and falling. Prevention of falls is critical.
- A common complication is weakness in swallowing and one must be vigilant to prevent choking.
- Individuals with IBM whose mobility is restricted need to be aware of complications related to this, including the development of edema and the possibility of developing pressure sores.
- Potential impacts on respiration (diaphragm weakness) are less common but serious. Awareness of respiratory shallowness, especially while sleeping, is important, leading to assessment and management.
- We must prevent iatrogenic complications. This means complications introduced by efforts to treat the illness. In this case, the most common problem is the harm caused by prednisone. This medication is often prescribed although it has not demonstrated any positive effect and many people needlessly suffer its complications.
The gentlemen in these pictures have not had proper management (with the use of pressure stockings) to prevent edema and they now face potentially serious complications.
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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
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- • Jump to 2013
- • All Research
- • Major review articles
- • Research Topics
- • Milestones in IBM research.
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- • Clinical trials
- • Web resources
- • Practical coping aspects
- • More coping Information
- • Using pdf files
- • Disclaimer
- • Contact
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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
Treatment for inclusion-body
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.
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.
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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/.
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Web sites for neuromuscular disorders:
The Myositis Association (formerly The Myositis Association of America (MAA))
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:
Homepage of Dagmar Slaven, an IBM patient. http://www.myomusings.com/
The UK Myositis Support Group: http://www.myositis.org.uk/
Dermatomyositis and assisted living: http://www.assisted-living-directory.com/content/dermatomyositis-assisted-living.cfm
Web sites for IBM Information:
The Inclusion Body Myositis Foundation (IBMF)
Steven A. Greenberg, MD, Director
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.mdausa.org/disease/inclusion-body-myositis
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/
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:
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.
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:
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.Return to Top
For comments or improvements, please contact Bill at e-mail: firstname.lastname@example.org
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Page Created: April 06, 2001.