Inclusion Body Myositis (IBM) is a sporadic and progressive muscle disorder & autoimmune myopathy affecting men and women, usually over 45 years old. This condition involves the gradual weakening and wasting of muscles in the legs and arms, leading to difficulty performing everyday tasks like walking, lifting, and gripping. IBM can be sporadic or familial (genetic causes) ^[1].

Genetic inclusion-body myopathy can be inherited in either a recessive or dominant pattern. Dominant IBM genetic disorders require only one genetic flaw to show themselves. Recessive IBM disorders, however, require that both parents pass on a flaw in the same gene before their offspring can show signs of the disease. Unfortunately, there is no cure for Genetic IBM, but researchers hope gene therapies will eventually be able to cure the disease. For patients with Sporadic inclusion body myositis (sIBM), Currently, early diagnosis and proper management are the best options for patients. They can help slow down the progression of the disease and improve the quality of life for those affected ^[2].

Recognizing the symptoms of IBM can be challenging, as they typically develop slowly over time and can mimic other muscle disorders. Some common signs of IBM include:

• Weakness in the hands and fingers,
• Difficulty swallowing
• Muscle pain
• Stiffness
• Peripheral neuropathy and loss of sensation in the limbs.

Understanding Inclusion Body Myositis (IBM)

Inclusion Body Myositis (IBM) can affect the body in multiple ways. Myositis is a general term referring to inflammation of the muscles. In muscle cells, abnormal entities known as inclusion bodies may be observed during muscle biopsies of patients suffering from IBM. The existence of these inclusion bodies distinguishes this form of myositis from comparable conditions, like multiple sclerosis, Broca’s Aphasia, polymyositis, and dermatomyositis.

Recognizing IBM Symptoms

For many patients, the exact cause of IBM is often unknown and classified as Idiopathic; Doctors believe IBM is related to an abnormal immune response that attacks and damages muscle tissues. Spotting the telltale signs of IBM can also be difficult, but there are specific indicators that can help with diagnosis. One of the earliest symptoms is muscle weakness in the quadriceps, which can cause difficulty standing up from a seated position or climbing stairs. Over time, weakness in other muscles, such as the finger flexors, wrist extensors, and ankle dorsiflexion, may also develop. In addition, IBM can cause muscle wasting, particularly in the forearms and quadriceps, and difficulty swallowing or speaking due to weakness in the throat muscles^[3].

Other common symptoms of IBM include muscle pain, stiffness, and fatigue. These symptoms can be exacerbated by exercise and may be more noticeable after periods of inactivity. Some people with IBM also experience a loss of sensation or numbness in their fingers or toes. It is important to note that while these symptoms may point to IBM, they are not definitive, and a proper diagnosis requires a combination of clinical examination, blood tests, and muscle biopsy.

Investigating the causes of IBM

Patients can better understand IBM by exploring the various factors that can contribute to its pathogenesis. IBM is believed to be an autoimmune disorder, meaning the patient’s immune system mistakenly attacks its cells and tissues. Aside from autoimmune factors, age and genetics may also play a role in the development of IBM. IBM typically affects people over 45 years old, which is more common in men than women. Additionally, some studies have found that specific genetic mutations may increase a person’s risk of developing IBM. However, more research is needed to determine the genetic factors contributing to this condition’s development. Understanding the causes of IBM is crucial in developing effective treatments and prevention strategies for this debilitating condition.

Diagnosing IBM Disorder

To accurately diagnose IBM, your doctor may recommend blood tests, biopsies, and radiological evaluations to assess a patient’s muscle strength, range of motion, and overall physical abilities. One standard test used to diagnose IBM is a muscle or tissue biopsy, in which a tiny sample of muscle tissue is surgically removed and examined under a microscope. This can help determine if there are any abnormal protein deposits or inclusion bodies in the muscle tissue, which is a hallmark of IBM.

Other tests that may be used to diagnose IBM include electromyography (EMG), which measures the electrical activity in your muscles, and magnetic resonance imaging (MRI), which can help identify any muscle inflammation or damage. Blood tests may also rule out other conditions that can cause muscle weakness, such as thyroid disorders or other autoimmune diseases such as ankylosing spondylitis, lupus, rheumatoid arthritis, connective tissue disease, type 1 diabetes, Sjogren’s or Hashimoto’s disease,   Using a combination of these tests and evaluations, a primary care doctor can make an accurate diagnosis and develop an effective treatment strategy to manage the negative symptoms and improve patient quality of life.

Traditional Treatment Options for IBM

Once diagnosed with IBM, managing the condition with various treatment options can significantly improve your quality of life. Although there is no cure for genetic IBM, there are ways to slow down the rate of progression of the disease and alleviate symptoms. Currently, some of the most effective strategies use a combination of isolated mesenchymal stem cells, physical therapy, and lifestyle changes that can effectively manage IBM.

So far, clinical trials have not shown many clear benefits for any specific drug treatments of inclusion body myositis. For that reason, many experts do not prescribe medications for the inclusion of body myositis except in certain limited situations.   Traditional medication options for IBM include:

• Immunosuppressive medications (help reduce inflammation and slow down muscle damage)
• Corticosteroids
• Physical therapy and rehab can also help improve muscle strength and mobility and prevent muscle atrophy.
• Lifestyle changes such as a healthy diet and regular exercise can also help manage symptoms and improve symptoms related to IBM and autoimmune myopathies

Other Biological treatments include:

• TNF inhibitors
• Abatacept offers a selective co-stimulation inhibitor that inhibits the binding of CD28 expressed on effector T cells, reducing T-cell activations.
• Tocilizumab
• Alemtuzumab
• Anakinra
• MEDI-545 (anti-IFNα antibody)

Stem Cell Treatment for Inclusion Body Myositis

Inclusion Body Myositis involves both inflammatory and degenerative characteristics, making it an ideal candidate for cellular therapy. Clinical trials for IBM have shown that satellite cell (SC) proliferation is normal for patients with IBM patients. Usually, their overall cell differentiation ability is impaired, thus leading to muscle failure. In extremely resistant myositis cases,  a combination treatment using myeloablative conditioning with cyclophosphamide. Treatment with cyclophosphamide and total body irradiation, followed by reinfusion of cord tissue stem cells and granulocyte colony-stimulating factors, helped improve muscle strength, with no trace of myositis seen on an MRI 6 months after treatment. This outcome remained stable for over three years. Other patients with progressive IBM demonstrated significant improvements and sustained remission after modified stem cell treatment without the need for adding toxic treatment requirements for patients with severe, resistant diagnoses.

Can stem cells cure myositis?

Mesenchymal stem cells and immunomodulation can help suppress various immune cell activities and have minimal immunogenicity risk. The Regeneration Center offers both autologous and allogeneic mesenchymal stem-cell transplantation for patients with non-genetic sporadic cases of IBM.

The ability of stem cells to renew themselves throughout our lives has proven beneficial for many body tissues. However, it’s believed that skeletal muscles, which comprise our body structure, only renew a few times under specific conditions. As we age, the body handles increased demands by growing bigger (hypertrophy), not by making more cells (hyperplasia). This could be why muscle stem cells can’t renew as much as other stem cells, like those in peripheral blood^[4].

Using isolated MSC cells with cultured muscle stem cells and growth factors offers patients a targeted way to treat muscle diseases over weeks using multiple cycles to initiate the repair process. When muscle stem cells get used up, the muscle is gradually replaced by fat and connective tissue. In some cases, The Regeneration Center also uses fibro-adipogenic progenitor cells due to their vital role in muscle repair. The isolated progenitor cells and growth factors help activate satellite cells even though they produce fat and scar tissue. Dealing with scar tissue (fibrosis) is vital to any treatment strategy. From our research, we’ve learned that treatments must start as early as possible or are unlikely to be effective, particularly in cases with significant fibrosis^[5].

The Regeneration Center Difference

Total Number of MSC+ Cell Sessions Needed:  For most patients with IBM, we will need multiple Infusions of isolated and expanded mesenchymal cells, which can be cultured from autologous sources of bone marrow (BMMSC) or allogeneic sources of cells (UC-MSC) and Neural Stem cells (MSC–NSC). These stem cells, and our proprietary combination of growth factors are joined to enhance the therapeutic potential of the treatment. The growth factors we isolate and culture from MSC Cells are naturally occurring proteins in cells that stimulate cell growth, proliferation, healing, and differentiation and are combined with stem cells to support brain repair and regeneration. Some of the growth factors we isolate for IBM patients include Vascular Endothelial Growth Factor (VEGF), Fibroblast Growth Factors (FGFs), Insulin-like Growth Factor 1 (IGF-1), Glial Cell Line-Derived Neurotrophic Factor (GDNF)Brain-Derived Neurotrophic Factor (BDNF), Nerve Growth Factor (NGF) and Epidermal Growth Factor (EGF).

Delivery Methods of MSC+ and Neural Stem Cells: The methods of delivery and cell infusions will vary based on patient needs. Stem cells can be administered using a combination of Intravenous infusions, stereotactic-guided delivery, inhalation therapy via micro-nebulized mesenchymal cells, Intrathecal infusions, and fluoroscopic guided cell delivery (in a hospital setting only). All intrathecal injections at The Regeneration Center are done by a board-certified neurosurgeon and are needed to bypass the blood-brain barrier.

Rehabilitation Post Treatment: Physical Rehabilitation in Bangkok is optional but highly recommended. Complete physical rehab services post-therapy can be provided upon request for 2-3 hours per day and up to 5 days per week.

Total Treatment Time Required: It is estimated to be around two weeks (depending on the type and seriousness of the condition). Medical and travel visas for extended accommodations at a hotel or apartment for the patient and family can also be provided upon request.

New IBM Disease Treatment Guidelines 2024

The cost of inclusion in body myositis treatment will vary depending on the degree of severity. Our medical team will need to evaluate the patients using current medical records. Evaluations can be done in person or online (submitting documents online). After review, a detailed treatment plan will be provided that will include the specifics such as the total number of nights required and total medical costs for treatment of connective tissue disease with stem cells. Please prepare all current medical records and contact us today to begin the evaluation process.

Published Clinical Citations

[1] ^Naddaf E, Barohn RJ, Dimachkie MM. Inclusion Body Myositis: Update on Pathogenesis and Treatment. Neurotherapeutics. 2018 Oct;15(4):995-1005. doi: 10.1007/s13311-018-0658-8. PMID: 30136253; PMCID: PMC6277289.

[2] ^ Sanmaneechai O, Swenson A, Gerke AK, Moore SA, Shy ME. Inclusion body myositis and sarcoid myopathy: coincidental occurrence or associated diseases. Neuromuscul Disord. 2015 Apr;25(4):297-300. doi: 10.1016/j.nmd.2014.12.005. Epub 2014 Dec 19. PMID: 25599912.

[3] ^ Schmidt K, Schmidt J. Inclusion body myositis: advancements in diagnosis, pathomechanisms, and treatment. Curr Opin Rheumatol. 2017 Nov;29(6):632-638. doi: 10.1097/BOR.0000000000000436. PMID: 28832349.

[4] ^ Lloyd TE. Novel therapeutic approaches for inclusion body myositis. Curr Opin Rheumatol. 2010 Nov;22(6):658-64. doi: 10.1097/BOR.0b013e32833f0f4a. PMID: 20827206; PMCID: PMC4365473.

[5] ^ Tedesco FS, Dellavalle A, Diaz-Manera J, Messina G, Cossu G. Repairing skeletal muscle: regenerative potential of skeletal muscle stem cells. J Clin Invest. 2010 Jan;120(1):11-9. doi: 10.1172/JCI40373. PMID: 20051632; PMCID: PMC2798695.