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The hidden threat of indifference: Why you should know about IBM?
In the elderly, intramuscular body myositis (IBM) is the most common inflammatory muscle disease. The disease is characterized by slowly progressive muscle weakness and wasting, primarily affecting the proximal and distal muscles of the arms and legs, especially the finger flexors and knee extensors. Despite its rarity, with an incidence ranging from 1 to 71 per million people, the condition is still often misdiagnosed as other illnesses, leaving many patients with a heavy burden in their quest for appropriate treatment.
Symptoms of corpuscular myositis usually develop gradually and asymmetrically, and especially in the early stages, can cause patients to fall.
IBM is often confused with hereditary microsomal myopathy (hIBM). The "M" in IBM stands for "myositis" while the "M" in hIBM stands for "myopathy." This means that there are two parallel processes in IBM: one autoimmune and the other degenerative. The muscles are invaded by immune cells and inflammation occurs. Degenerative changes in muscle fibers are manifested by abnormal protein deposition and vesicle formation.
Symptoms and effects
IBM patients typically experience slowly progressive, increasing muscle weakness, with common early symptoms including frequent falls, difficulty climbing stairs, and limited finger dexterity. Some patients may be unable to maintain basic activities of daily life and eventually need to rely on assistive devices to move around. Although IBM does not significantly shorten life expectancy, the risk of death related to malnutrition or respiratory failure remains. As the disease progresses, many patients experience significant limitations in their mobility within 5 to 10 years.
The exact cause of the disease is still unclear, but some studies suggest that IBM may be related to an unidentified virus or autoimmune disorder.
Causes and theories
There is no definitive answer to the cause of IBM, but two main theories have been proposed. The first hypothesis is that an inflammatory response caused by some unknown trigger may be the primary cause, with degenerative changes in muscle fibers being a secondary consequence. The second view is that IBM is actually an age-related degenerative disease, and the accumulation of abnormal proteins is closely related to the aging of muscle fibers.
Diagnosis and differential diagnosis
Diagnosis of IBM usually relies on a muscle biopsy and blood tests to check creatine kinase (CK) levels. Misdiagnosis often occurred in the early stages, especially when IBM was mistaken for polymyositis. When treatments such as steroids are ineffective, a diagnosis of IBM may be made.
Therapeutic management and supportive care
As of 2019, there are no effective treatment options that can slow or stop the progression of IBM. The vast majority of management is supportive care, focusing on preventing falls and improving the patient's quality of life. In addition, physical therapy is recommended to assist patients in developing appropriate home exercise programs and mobility training.
Socio-cultural influence
In the media, attention to corpuscular myositis is gradually increasing. For example, musician Peter Fretton was diagnosed with the disease in 2019, which also increased the attention of the disease. In the movie "Father Stu", the protagonist is also a disabled priest, and his character's background involves this rare disease.
Conclusion
Whether in the medical field or in society in general, the awareness of corpuscular myositis in the muscle body still needs to be strengthened. This disease is hidden in the shadows of illness, and patients often encounter misunderstanding and neglect. Why don’t we pay enough attention to this hidden threat? Should we reassess the status and importance of this disease in public health?
