Site presented by Bill Tillier.
☝ 4.2.5 Current IBM Research.
Link to watch a short introductory video.
Link to see the book on amazon.com
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▣ 1.1 Introduction.
⧈ 1.1.1 The disease.
⚀ Inclusion Body Myositis (IBM) is a rare disease that affects muscles, specifically those in the arms and legs, and it develops slowly over time. This chronic and progressive disease is difficult to diagnose, and although it is believed to be an autoimmune disorder, the root cause of IBM is still unknown, and no effective treatment has been discovered yet. Generally, IBM is associated with aging and tends to affect individuals over 50 years of age. The disease occurs randomly and is not inherited, and is often referred to as sporadic inclusion body myositis (sIBM).
⧈ 1.1.2 This web page.
⚀ This webpage begins with section 1, giving a broad overview of what IBM is. Section 2 presents the common complications seen as IBM progresses and some other diseases that commonly occur along with IBM. Section 3 briefly mentions diagnosis and treatment, linking to videos from Johns Hopkins that give excellent descriptions. Section 4 includes an overview of the theories proposed to explain why IBM occurs—no cause or treatment has yet been discovered. Section 4 also includes a fairly scientific overview of the latest research on IBM that is intended more for doctors than patients. Section 5 looks at the physical accommodations and adaptations often required as IBM progresses. Section 6 looks at the psychological aspects of dealing with a chronic, progressive, and debilitating illness like IBM. Finally, section 7 presents links that will take you to further explanations, discussions, and resources. There is a tremendous amount of information on this webpage and my suggestion is that you need to slowly work your way through it.
⚀ This page is a good starting point for anyone interested in IBM and contains information suitable to take to a family physician.
⚀ The site provides two levels of information:
□ Basic introductions aimed at the IBM patient, their family, and caregivers;
□ Selected research on IBM intended for physicians.
⚀ This webpage mentions some things several times, this reflects the fact that these things are important.
⧈ 1.1.3 About me.
⚀ I was a psychologist working for the government when, at age 40, I began to trip and fall. Then I noticed weakness in my hands and knees. After a three-year process of diagnosis, it was determined that I had IBM, and I retired on disability—that was in 2002. I had done a lot of research as part of my job, and when I got my diagnosis, I reviewed everything I could on IBM and created the webpage you are now reading. I try to help other patients with IBM to keep a realistic and positive outlook. This is always difficult when dealing with a chronic and debilitating disease without treatment.
▣ 1.2 Key information for new patients. If you have been diagnosed with inclusion body myositis here is what you need to know to begin with.
⧈ 1.2.1 General.
⚀ This is an extremely rare, serious, and challenging disease.
⚀ The disease strikes people later in life, usually developing after age 40. Most people develop IBM between the ages of roughly 50 and 70.
⚀ Of every hundred patients, roughly 67 will be men, and 33 will be females.
⚀ A survey done on IBM by the Yale School of Public Health found that over 91% of respondents indicated that they still lived in their own house or apartment.
⚀ Although people may feel the disease “goes up and down”, research has shown that it does not and slowly marches on over time.
⚀ A 2022 review article from MDA.
⧈ 1.2.2 Impact on muscles.
⚀ IBM is a disease that strikes and weakens primarily the skeletal muscles. Not all muscles are impacted. The heart is not affected. It does not affect the nerves.
⚀ Over time, the disease will progress and get worse and worse. As more and more muscle cells are impacted, the muscle becomes weaker and weaker, and more and more function is lost.
⚀ As patients get older, they may need more and more help to accomplish the day-to-day functions of life like getting up in the morning, showering, going to the bathroom, eating, etc.
⚀ The disease affects different people in different ways, and at different rates, so you cannot compare yourself with others.
□ Different muscle groups are affected at different rates.
□ The rate at which the disease progresses varies widely in patients.
□ The disease starts in people at different ages.
□ The disease may impact men and women differently—for some reason it is twice as common in men.
□ It is clear that severity varies widely—there are very mild cases and, as well, there are severe cases.
□ For some reason, the impact of IBM may differ based on the age when you developed symptoms.
⚀ 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 disease usually leads to severe disability and often necessitates using a wheelchair.
⚀ A common serious complication is weakness in swallowing (dysphagia) that can cause choking or aspiration (taking food into the lungs), causing pneumonia (sometimes a cause of death). Swallowing involvement is a critical factor that should be investigated after an IBM diagnosis.
⚀ Another serious possible complication is respiratory impairment caused by weakness of the diaphragm. Respiratory involvement is a critical factor that should be investigated after an IBM diagnosis. Respiratory dysfunction can be identified, treated, and monitored.
⚀ Depending on how you are affected, you will face various challenges:
□ If the disease affects your arms, you will have problems picking up objects and using your fingers.
□ If the disease affects your legs, you will have trouble falling, climbing stairs, etc.
□ If the disease affects your throat, you may have trouble swallowing.
□ If the disease affects the breathing muscles, you may have problems getting enough air, especially at night (which may cause chronically high carbon dioxide levels in the blood).
⚀ This illustration shows the common muscle groups impacted (blue) and the most common and serious potential complications (orange) (after Greenberg, 2019).
⧈ 1.2.3 Impact on lifespan.
⚀ If you have IBM, it tends to shorten your life by an average of three years compared to the population. Statistically, the median age of death is 84 compared to 87.5 in the population (Naddaf et al., 2021; Shelley et al., 2021).
□ IBM does not directly lead to death. IBM-associated death is often the result of complications, for example, choking or problems with breathing.
□ The most common causes of death in IBM patients are complications due to respiratory failure and aspiration pneumonia.
□ Ongoing monitoring and awareness of dysphagia and respiratory involvement are highly recommended.
⧈ 1.2.4 Classification of IBM.
⧈ 1.2.5 Blood tests in IBM.
⧈ 1.2.6 Possible confusion over:
⚀ 1.2.6.1 Heart attacks:
□ One of the effects of the breakdown of muscle caused by IBM is that chemicals are released in the blood. These chemicals include Cardiac Troponin T (cTnT) and creatine kinase (CK). The problem arises when a patient with IBM is given a blood test and these chemicals appear raised, this has traditionally indicated a heart attack. If the doctor does not know that IBM leads to raising these levels, it can cause confusion that the patient has had, or is having, a heart attack.
⚀ 1.2.6.2 Liver tests:
□ IBM may cause elevated levels of the liver enzymes, for example, aldolase, alanine transaminase (ALT) and aspartate transaminase (AST). These enzymes are released into the blood by damaged liver cells, so high levels of ALT and AST may be a sign of liver disease. Again, if the doctor does not know that IBM leads to raising these levels, it can cause confusion that the patient has liver issues.
⚀ 1.2.6.3 Statin-induced muscle disease:
□ A rare side effect of the use of statin medications is a muscle disease called Statin-induced immune-mediated necrotizing myopathy (IMNM), also known as reductase (anti-HMGCR) myopathy. This is an inflammatory myopathy that is not the same as IBM. However preliminary research shows that IBM may be seen in patients who take statin medications. In a study of 221 myositis patients taking statins, 66 cases were diagnosed with IBM and of these, 20 had taken a statin medication. See: Caughey (2018). Also see: 2021 open access article.
⧈ 1.2.7 Brief History of IBM: A few highlights.
⧈ 1.2.8 How common is IBM?/IBM and aging.
⧈ 1.2.9 Genetics of IBM.
⚀ 1.2.9.1 Genetic predisposition.
⚀ 1.2.9.2 Familial IBM (fIBM)
□ 1.2.9.2.1 Familial IBM refers to rare cases where (typical) IBM is seen in two or more patients within a single generation in a family.
□ 1.2.9.2.2 The symptoms and features of fIBM are very similar to those seen in IBM. The familial occurrence of such a rare disorder likely highlights the importance of genetic predisposition in the causation of IBM.
⚀ 1.2.9.3 Hereditary inclusion body myopathy (hIBM).
▣ 1.3 Common questions IBM patients ask.
▣ 1.4 Several introductory videos from the Johns Hopkins Myositis Center.
⧈ 1.4.1 Disease Overview.
⧈ 1.4.2 Signs & Symptoms.
⧈ 1.4.3 Lifestyle Options.
▣ 1.5 Bill's Information pages.
⧈ 1.5.1 An information page for patients, family and caregivers. (PDF).
⧈ 1.5.2 An information page to take to your family doctor. (PDF).
⧈ 1.5.3 Questions to ask your doctor. (PDF).
⧈ 1.5.4 An overview of several different diseases.
▣ 1.6 Recent research reviews.
⧈ 1.6.1 A comprehensive review article — September 2022 (open).
⧈ 1.6.2 A 2021 review article (open).
▣ 3.1 Diagnosis.
⧈ 3.1.1 Overview. Diagnosing muscle conditions is challenging and especially IBM, because it is quite rare, and because the symptoms it presents are often quite varied.
⚀ This webpage will not focus on diagnosis. Diagnosis of IBM is a long, complicated process usually involving seeing several doctors and having numerous tests done. It is counterproductive to try to diagnose yourself using web pages or information from the Internet. Diagnosis is a job for medical specialists.
⚀ Because the condition comes on very slowly, it may take years to notice that you are ill and seek help. You and those around you may attribute the symptoms to simply “getting old”.
⚀ The diagnosis of this disease is challenging, and many patients describe going to many doctors. On average, it takes five years to receive a diagnosis.
⚀ Doctors often first mistake inclusion body myositis for a different disease called polymyositis, or, often, Amyotrophic lateral sclerosis (ALS).
⚀ After going through the process myself and talking to other patients, the best advice I can give is that you must be patient and keep advocating for yourself. Diagnosis is not “black-and-white,” and it is important not to give up as you move through the process. Be clear in your descriptions of your symptoms, keep good records and remember that a correct diagnosis is essential as it maps out what to expect in the future.
⚀ More information on the diagnosis of IBM can be found here: Johns Hopkins Medicine
⧈ 3.1.2 Diagnosis video from the Johns Hopkins Myositis Center.
▣ 3.2 Treatment.
⧈ 3.2.1 Overview.
⚀ Based upon research, no treatment is recognized as effective for IBM (as of 2023).
⚀ Based upon their experience and opinions, doctors may try medications with IBM patients however, this is a clinical judgment where any possible benefits must be weighed against potential side effects.
⚀ Some doctors prescribe steroids (prednisone) even though research has demonstrated it does not help and may even harm IBM patients.
⚀ The most effective thing you can do is watch for, and manage, complications and prevent injuries due to falls.
⚀ Exercise programs specifically developed for each patient are now being recommended.
⚀ Many companies sell stem cells, different diets, or supplements, but no research shows these are of any help.
⚀ The best approach is to manage the complications that may arise from the disease. Evaluation of swallowing and respiration are critical, ongoing issues. Prevention of falls is an important consideration.
⚀ Standard of care for most patients with IBM involves strictly nonpharmacological management, including emotional support, physical therapy, education on fall precautions and exercise.” From: Greenberg, 2019.
⚀ Currently, no evidence is available to support any specific treatment in clinical practice. To date, there are no effective or approved treatment options for inclusion body myositis” From: Hanna et al., 2019.
⚀ A cautionary note. There is a strong tendency for both doctors and patients to “want to do something”—anything—to try to slow down or reverse the symptoms of a major debilitating and chronic illness like IBM. For patients, it can be very frightening and frustrating to simply “do nothing.” Caution must be used when no significant benefits of treatment can be demonstrated and when treatments all have significant potential side effects.
⚀ A recent (2021) review article on treatment.
□ Summary: Thus far, no treatment for IBM has demonstrated a therapeutic effect.
□ Reference: Snedden, A. M., Lilleker, J. B., & Chinoy, H. (2021). In pursuit of an effective treatment: The past, present and future of clinical trials in inclusion body myositis. Current Treatment Options in Rheumatology. Link.
⚀ Also see: Needham, M., & Mastaglia, F. L. (2016). Sporadic inclusion body myositis: A review of recent clinical advances and current approaches to diagnosis and treatment. Clinical Neurophysiology, 127(3), 1764-1773. Link.
⧈ 3.2.2 Exercise in inclusion body myositis.
⚀ Also see: Exercise in Place—Myositis Support and Understanding (MSU)
⧈ 3.2.3 Treatment video from the Johns Hopkins Myositis Center.
▣ 4.1 Causes.
⧈ 4.1.1 After nearly 50 years of research, the basic causes of IBM remain unknown. There is no general consensus within the research community concerning the basic causes of inclusion body myositis.
⚀ It appears that multiple factors work together to cause IBM (it is a “multifactorial disorder”). It is also very complicated to determine if an effect causes the disease or, if the effect is caused by the disease.
⚀ IBM appears to be an age-related, autoimmune disease characterized by cytotoxic T cells, but the exact cause of the disorder is unknown. IBM is a complex disorder believed to be influenced by a combination of genetic, immunological, and environmental factors. Based on: Link.
⚀ Researchers have identified two distinct processes — one autoimmune and one degenerative — that occur in individuals with IBM. Many scientists support the view that it appears likely that autoimmunity drives the disease and accounts for the degenerative-pathological changes seen in IBM skeletal muscle.
⚀ Other researchers believe that IBM is primarily a degenerative muscle disorder and not an inflammatory one.
⚀ Further research discoveries will be necessary to achieve a better understanding of the root causes of IBM.
⧈ 4.1.2 Theories of what may cause IBM: Detail.
▣ 4.2 Research.
⧈ 4.2.1 General Muscle Research.
⚀ Muscles are very complex systems and research into their basic structures and functions continues.
⚀ The causes of some muscle diseases (like IBM) are unknown and in turn, few specific treatments are available. Until more specific treatments are developed, researchers are looking into trying to develop general approaches to enhance muscle function.
⚀ If researchers could boost muscle function it might be possible to offset the effects of the different muscle diseases. Even a small increase in function could be significant to the patient having one of these illnesses.
⚀ The basic goal is to have the body produce more muscle than normal. The underlying muscle disease will not be treated, but with more muscle being produced, the overall impact of the disease may be reduced. Even small gains might be significant for the affected patient.
⚀ An example of this approach is a drug called Bimagrumab. This drug has been used to try to produce more muscle in various conditions. A report says it's safe to use but did not appear to work in IBM: See: (Hanna et al., 2019).
⧈ 4.2.2 IBM Research.
⚀ Research specifically focused on IBM attempts to understand what causes the disease and how it unfolds.
⚀ Most research on the treatment of IBM has looked at using existing medications and examining their impact on IBM. Generally speaking, no medication has shown significant improvements in IBM patients (see above).
⚀ Summary: IBM is a very complex and challenging disease to research. Much has been learned about the disease, but frustratingly, more remains unknown. The understanding of IBM and its causes is a very slowly evolving phenomenon.
At this stage, IBM research is primarily focused on finding the cause and
“inventing” a treatment that will help. Research also looks at trying to apply existing medications to see if they will help patients with IBM. I believe that this quotation from 2017 remains a good summary of the situation: “For the past two decades, the field of IBM research has been split with some researchers suggesting that IBM pathogenesis begins with inflammation leading to myodegeneration and others favouring a primary degenerative myopathy stimulating autoimmunity. Now with emerging therapies aimed at targeting muscle degeneration and other therapies focused on immune modulation, it is essential to understand the connection between these two pathologies. A siloed approach that ignores one or the other will not advance future therapeutics. Instead, additive therapies or dual acting therapies that focus on both aspects of disease pathogenesis will likely need to be employed.”
From: Link.
⧈ 4.2.3 Current IBM Research.
⧈ 4.2.4 Past research.
⧈ 4.2.5 Functional assessment of IBM.
▣ 7.1 A key resource: Myositis Support and Understanding (MSU)
▣ 7.2 A key resource: The Myositis Association.
▣ 7.3 A brochure from the Myositis Association of America. (PDF).
▣ 7.4 Selections from Outlook (TMA).
▣ 7.5 IBM in Images.
▣ 7.6 Other Relevant Webpages:
⧈ 7.6.1 Web sites for IBM Information:
⚀ Muscular Dystrophy Association (USA). LINK.
⚀ Cure IBM is dedicated to inclusion body myositis awareness, education, and research. LINK.
⚀ National Organization for Rare Disorders (NORD) LINK.
⚀ Peter Frampton. LINK.
⚀ IBM overview: LINK.
⚀ Muscular Dystrophy UK IBM overview:
IBM Alert Card: LINK.
⚀ National Institute of Neurological Disorders and Stroke (NINDS) site on IBM: LINK.
⚀ Washington University School of Medicine in St. Louis, MO, IBM Specialty Clinic devoted to inclusion body myositis. LINK.
⚀ Wikipedia entry: LINK.
⚀ IBM Facebook pages:
□ IBM Facebook Page #1 (open):
□ IBM Facebook Page #2 (membership):
⚀ Clinical Trials for IBM:
□ You can find information on the latest clinical trials on IBM by going to this website and entering inclusion body myositis on the search line: NIH Clinical Trials: http://clinicaltrials.gov/
⚀ Yale IBM Registry: Yale IBM Registry.
⚀ Jerry King's youtube IBM channel. (This is not my website).
⚀ Seven warning signs of bogus science. (This is not my website).
▣ Website Miscellaneous:
⧈ 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. You need to install a PDF reader (many computers already have one installed). If you click on a PDF file and 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://get.adobe.com/reader/
⧈ Mission Statement:
⚀ There are many excellent websites that present information on IBM. However, I was struck by how much of the information was very technical. Therefore, the primary mission of this site is to provide accurate and understandable information to help the reader make sense of, and cope with IBM.
⧈ 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. Thank you.
⧈ Contact: For comments or improvements, please contact Bill at bill.tillier@gmail.com
⧈ Search by JRank
⧈ Page Created: April 06, 2001.
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