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■ My book on inclusion body myositis.

In 2023 I decided to write a book focussed on providing information on IBM to patients. Here is the information on the book.

Table of contents.

Link to watch a short introductory video.

Link to see the book on amazon.com

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■ 1. Overview.

▣  1.1 Introduction.

⧈  1.1.1 Key Takeaways.

⚀ Inclusion body myositis (IBM) is a devastating and progressive muscle disease that strikes later in life.

⚀ People with IBM gradually lose muscle strength and mass – the muscle shrinks and is gradually infiltrated with fat.

⚀ IBM is not responsive to treatment.

⚀ IBM is considered one of the most challenging and complex of all muscle diseases.

⚀ IBM is characterized by a pattern of weakness in the muscles in the arms and legs.
≻ As a result, everyday activities such as gripping objects, climbing stairs, and rising from a chair become increasingly difficult, and falls become common.

⚀ A high proportion of IBM patients experience dysphagia – swallowing trouble – as a result of weakness in the throat muscles.

⚀ Despite extensive research efforts, the underlying cause of IBM remains unknown.
≻ The current consensus is that multiple factors contribute to the cause, including immune, degenerative, genetic, and ageing factors.

⧈  1.1.2  The disease.

⚀ Inclusion Body Myositis (IBM) is a rare disease that affects skeletal muscles, primarily those in the arms and legs.
≻ It develops slowly over time.
≻ This chronic and progressive disease is difficult to diagnose and no effective treatment has been discovered.
≻ Generally, IBM is associated with age and tends to affect individuals over 50.
≻ The disease occurs randomly and is not inherited.
≻ In the past it was often referred to as sporadic inclusion body myositis (sIBM) – this term is no longer recommended.

⧈  1.1.3  This web page.

⚀ This webpage begins with a broad overview of IBM.
≻ 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 provide excellent descriptions.
≻ Note: this is not a disease that you can diagnose from the Internet!
≻ Section 4 includes an overview of the theories proposed to explain why IBM occurs. 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 usually 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 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.

⧈  1.1.4  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. After a four-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 this webpage. 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 that has no 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  Detailed overview.

⚀ This is an extremely rare, serious, and life-altering 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.

⚀ Over 91% of IBM patients live in their own house or apartment.

⚀ Although people may feel the disease “goes up and down,” research has shown that it does not; it slowly marches on over time.

⚀ IBM is a disease that primarily strikes and weakens the skeletal muscles.
≻ Not all muscles are impacted.
≻ The heart is not affected.
≻ It does not affect the nerves.

⚀ Over time, the disease will advance and get progressively worse.
≻ As more muscle cells are impacted, the muscle becomes weaker, and more function is lost.

⚀ As patients get older, they may need 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 people in different ways, and at different rates, so you cannot compare yourself with others.

□ The disease starts in different people at different ages.

□ The rate at which the disease progresses varies widely in patients.

□ The disease may impact men and women differently.

□ The disease may impact black patients differently.

□ 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.

□ Different muscle groups are affected at different rates and times.
≻ For example, some people first show weakness in the muscles that control the hands and arms.
≻ Some people first develop weakness in the leg muscles.
≻ Others may show weakness in swallowing as a first symptom.

⚀ The disease usually leads to severe disability of mobility and often necessitates using assistive mobility devices, for example, a wheelchair or a lift to transfer you.

⚀ A “chemical” (Anti-cN1A antibodies) strongly associated with IBM has been found in the blood.
≻ Unfortunately, this cannot be directly used for diagnosis.
≻ Twenty to sixty percent of IBM cases show Anti-cN1A antibodies.
≻ If you have the antibody, you likely have IBM; if you do not have the antibody, you could still have IBM.
≻ Also, researchers have not been able to show any practical differences between people who have these antibodies compared to people who do not.

⚀ 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.
≻ Swallowing involvement can be identified, monitored, and treated.

⚀ 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, monitored, and treated.

⚀ The research has generally either said that IBM is pain-free or has not addressed the issue of pain at all.
≻ However, many IBM patients report having pain, often severe pain.
≻ In a 2022 study done by Bhashyam (and others), they looked at pain in IBM and found that of “113 IBM patients, some 106 (81%) reported pain, and of this group, 88 took pain medication (83%).
≻ Of the 88, 55 (62.5%) took opioids, and 82 (93.2%) took non-opioid pain medication.”

⚀ In the past, doctors advised against exercise.
≻ Today, in the early stages after diagnosis, a supervised and individualized exercise program is recommended, focussing on unaffected muscles.

⧈  1.2.2  Impact on muscles.

⚀ Depending on how you are affected, you will face various physical challenges:

□ If the disease affects your arms, you may have problems picking up objects and using your fingers.
≻ Eventually it may become difficult to raise your arms.

□ If the disease affects your legs, you may fall frequently, have problems climbing stairs, etc.
≻ Eventually you may not be able to walk and may require the use of a wheelchair.

□ 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.

⚀ Before we go any further, it is very helpful to know these very basic terms that are used in describing the movements of the body.

□ Two very common terms describing the action of muscles in moving the body are extension and flexion (from Link ).

□ Finger flexion describes the movement of the fingers, driven by the flexor muscles located in the forearm and connected by tendons, to bend the fingers inward toward the palm, as in closing the hand and making a fist.
≻ This movement is often severely compromised by IBM.

□ A very common problem in IBM is difficulty lifting the toe when walking or stepping up.
≻ This is called dorsiflexion (from Link).
≻ This is also called “foot drop.”

⚀ This illustration shows the common muscle groups impacted (blue) and the most common and serious potential complications (orange) (after Greenberg, 2019).

⧈  1.2.3  Common questions IBM patients ask.

⧈  1.2.4  Classification of IBM.

⧈  1.2.5  Blood tests in IBM.

⧈  1.2.6  Impact on lifespan.

⚀ IBM does not directly lead to death and is not classified as a life-threatening disease.

⚀ 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).
≻ The cause of this appears to be related to the complications of IBM, primarily, respiratory issues – aspiration.
≻ Also, falls are a risk factor.

⚀ 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.

⧈  1.2.7  Brief History of IBM: A few highlights.

⧈  1.2.8  How common is IBM? / IBM and aging.

⧈  1.2.9  Genetic predisposition of IBM.

⧈  1.2.10 IBM and just getting old.

⚀ As people get older they often encounter various health issues.
≻ For example, problems with vision and hearing increase with age.
≻ Body pain in the back and neck become more common.
≻ As well, with age, depression is more common.
≻ Problems concentrating and forgetfulness may increase.
≻ Patients often ask if these types of issues are caused by their IBM.
≻ The short answer is usually no, most often they are just a sign of the normal ageing process.
≻ IBM certainly makes ageing more difficult, especially from an energy perspective.
≻ Energy levels normally decline with age and this is exaggerated by having IBM.

▣  1.3 Several videos from the Johns Hopkins Myositis Center.

⧈  1.3.1 Disease Overview.

⧈  1.3.2 Signs & Symptoms.

⧈  1.3.3 Lifestyle Options.

▣  1.4 Bill's Information pages.

⧈  1.4.1 Questions to ask your doctor. (PDF).

⧈  1.4.2 An information page to take to your family doctor. (PDF).

▣  1.5 Research reviews of IBM.

⧈  1.5.1 A comprehensive review article  –  September 2022 (open) [open means that you do not have to pay to view the article].

⧈  1.5.2 A 2022 review article from the Muscular Dystrophy Association (MDA).

⚀ As a pdf download.

⧈  1.5.3  A 2021 review article (open).

▣ 1.6  IBM may cause confusion for the doctor.

⧈ 1.6.1  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.

⧈  1.6.2  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 muscle 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.

⧈  1.6.3 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 some patients who take / have taken 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) (pdf). Also see: 2021 open access article.

▣  1.7  Hereditary inclusion body myopathy (hIBM) (old terminology).

▣  1.8  Familial IBM (fIBM) (old terminology).

⧈  Familial IBM (fIBM) is a term that has been used to refer to rare cases where IBM is seen in two or more patients within a single generation in a family.
≻ The symptoms and features of fIBM are the same as those seen in IBM. The familial occurrence of such a rare disorder likely highlights the importance of genetic predisposition in the causation of IBM.

▣  1.9  Terminology.

⧈ The terms “Sporadic IBM,” “Familial IBM,” and “Hereditary IB Myopathy” are considered misleading and are no longer recommended.
≻ Originally, the disease was called sporadic inclusion body myositis (sIBM). It is now recommended to call it inclusion body myositis (IBM).
≻ It has been common to refer to familial inclusion body myositis (fIBM). Although IBM may have genetic predispositions, it does not appear to be genetic. It is now recommended to refer to these cases as simply inclusion body myositis (IBM).
≻ As different conditions were being described, several very rare genetic diseases were called hereditary inclusion body myopathy (hIBM).
≻ This has been very confusing, and today, these genetic disorders should be referred to by their specific names – they do not belong under the IBM label (see Lilleker et al. (2024) for recommendations).

▣  1.10 Several diseases we often hear about.

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■ 2. Complications / Comorbidities.  

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■ 3. Diagnosis and Treatment:

▣  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.

⚀ Revised diagnostic criteria 2024.

⚀ 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 currently recognized as effective for IBM.

⚀ 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.
≻ 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 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.

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■ 4. Causes and Research.

▣  4.1 Causes.
≻The science behind the possible causes of IBM is extremely complex and very challenging to put into understandable, everyday language.
≻ It’s very important to me to be careful not to create any confusion or misunderstandings.
≻ There is no consensus among researchers as to the cause of IBM.
≻ Not knowing the cause makes it more challenging for researchers to develop a treatment and it is very frustrating for patients with IBM – we want to know.
≻ We can say that there are several major abnormalities seen in IBM – there are autoimmune issues, problems seen in mitochondria, and degenerative aspects.
≻ The question is how these abnormalities relate to a possible cause, and the answer is that they don’t know yet.

▣  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.
≻ The underlying muscle disease will not be treated, but with more muscle being produced, the overall impact of the disease may be reduced.
≻ Even a small increase in function could be significant to the patient having one of these illnesses.
≻ 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 “creating” 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  Functional assessment of IBM.

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■ 5. Physical adaptation.

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■ 6. Psychological aspects.   

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■ 7. Further resources.

▣  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:

⚀   MYOCAN Myositis Canada.

⚀   Muscular Dystrophy Canada (MDC).

⚀   Myositis Support and Understanding.

⚀   Muscular Dystrophy Association (USA).

⚀   THE MYOSITIS ASSOCIATION (TMA).

⚀   Cure IBM is dedicated to inclusion body myositis awareness, education, and research.
≻ It is run by a doctor, an ophthalmologist, Kevin Dooley, who has been diagnosed with IBM.

⚀   National Organization for Rare Disorders (NORD).

⚀   Peter Frampton.

⚀  Muscular Dystrophy UK IBM overview:
IBM Alert Card: LINK to pdf.

⚀   National Institute of Neurological Disorders and Stroke (NINDS) site on IBM.

⚀   Washington University School of Medicine in St. Louis, MO, IBM Specialty Clinic devoted to inclusion body myositis run by Dr. Conrad “Chris” Weihl.

⚀   Wikipedia entry.

⚀  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.

⚀   Webpage credibility.

⚀   Jerry King's youtube IBM channel. (This is not my website).

⚀   Seven warning signs of bogus science. (This is not my website).

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