Myositis and Patient Safety

So far, most of the evidence suggests that polymyositis and dermatomyositis are autoimmune disorders, illnesses in which the immune system mistakenly attacks the body's own tissues. The muscles of people with inclusion body myositis contain an abnormal protein called amyloid, but the reason it forms is unknown. This amyloid protein is similar to the protein deposited in the brains of people with Alzheimer's disease, and some experts believe that the two diseases may develop the same way. In inclusion body myositis, the muscle also contains tiny structures that resemble viral particles (called inclusion bodies), although no viral infection has been consistently identified in association with this disease. In dermatomyositis, cancer is found in approximately 10% to 20% of cases. Sometimes, the muscle problem develops first. In other cases, the cancer is detected before the myositis.

It is the disease featuring weakness and inflammation of muscles and muscle pain. The cause of much inflammatory myopathy is unknown i.e. idiopathic. The usual criteria for a diagnosis are weakness in muscles of the head, neck, trunk, upper arms or upper legs. They may be diagnosed by biopsy, MRI or electromyography. The biopsy findings of (i) muscle cell degeneration and regeneration and (ii) chronic inflammatory infiltrates in muscle cells. The goal of treatment is to improve the activities of daily living and muscle strength. The suppression of immune system activity i.e. immunosuppression is the treatment strategy. Every year between 2.18 and 7.7 people per million receive diagnosis. Around 3.2 children per million per year are diagnosed with an average age of onset of seven years. 

The following symptoms are most common: Muscle pain and tenderness, often appearing first in the hips and shoulders, Muscle weakness, Difficulty with everyday tasks such as climbing stairs or rising from a chair, General fatigue (tiredness), Difficulty speaking or swallowing, Shortness of breath or coughing, Skin rash, Joint pain and swelling, Weight loss, Sensitivity to cold in the extremities (Raynaud's phenomenon), Fever, Atrophy (loss of muscle mass).

A diagnosis of myositis usually involves: A thorough patient history and physical exam, including hands-on tests of muscle strength. Blood tests for elevated levels of muscle enzymes and/or chemical inflammation markers. Electro-diagnostic testing, this uses needles and electrodes to measure muscle and motor nerve activity. Muscle or skin biopsy, in which a tissue sample is examined under a microscope. Magnetic resonance imaging (MRI) to pinpoint areas of muscle weakness. Other tests to rule out diseases with similar symptoms.

Immune system disorders cause abnormally low activity or over activity of the immune system. In cases of immune system over activity, the body attacks and damages its own tissues (autoimmune diseases). Immune deficiency diseases decrease the body's ability to fight invaders, causing vulnerability to infections.

In response to an unknown trigger, the immune system may begin producing antibodies that instead of fighting infections, attack the body's own tissues. Treatment for autoimmune diseases generally focuses on reducing immune system activity.

The key factors responsible for the dominance in myositis market can be the presence of high geriatric population along with high demand for advanced therapeutics for effective treatment of the disorder in many regions. In some cases there is no complete cure for myositis so researchers are working to develop universal treatment that can be more effective. Studies are also being conducted to find new drugs and therapeutics. 

The global market revenue for advanced orthopaedics grew from $35.3 billion in 2013 to $36.4 billion in 2014. The global orthopaedic device market was valued at $29.2 billion in 2012 and is expected to reach $41.2 billion by 2019. The market is expected to grow at a compound annual growth rate of 4.9 % over the next five years, according to the Transparency Market Research report. The global rheumatology therapeutics market was estimated to be worth $17.1 billion in 2011, having grown at a Compound Annual Growth Rate (CAGR) of 9.4% from 2005, and is forecast to grow at a CAGR of 4.8% to $23.8 billion by 2018. Companies that produce myositis drugs: Gradalis, Inc., New Zealand Pharmaceuticals Ltd., Nobelpharma Co. Ltd., Novartis AG.,  Drugs u Bayer Healthcare, Bloomberg Medical Group, Bone SA, BSN Medical (PTY) Ltd South Africa, DJO Global, Elite Surgical, FLUOROVIZION, KARL STORZ Endoscopy (South Africa) (PTY) LTD, MEDISMEDICAL, Ortho-Xact, Professional Orthopaedics (PTY) LTD, SportsMed Ortho (Pty) Ltd,  Smith & Nephew, STRATMED, SURGITECH,  WERKOMED.

Many biotech companies are founded by scientists. But there is certainly a path, somewhat less trodden but nonetheless viable, for the inspired entrepreneur who may not be a brilliant researcher.

Each year, over a million companies are started in the US with about 5–10% (57,793 in 1998) of them classified as high technology companies1. Many nascent entrepreneurs exist around us, and thinking about starting a new venture is not an uncommon activity—in fact at any given point in time, over 4% of the working age population might be thinking of an idea for a new venture2. Not surprisingly, turning ideas into business ventures is tricky: only about 10% of budding entrepreneurs have a new firm in place within 12–18 months3; the other 90% either fail to define a sound business model that can drive their business idea forward to a new venture or realize their idea was flawed.

A key ingredient in successful entrepreneurship is self-knowledge. If entrepreneurs know their limitations and strengths, they may be able to avoid some common pitfalls in starting up a biotech company.

Researchers are making some progress in understanding what happens in myositis and what goes wrong with the immune system. Currently they believe that the muscle fibres in some people with myositis are damaged by a group of cells called lymphocytes which make up part of the immune system. Researchers also are studying the possibility that certain autoantibodies may travel through the blood and cause damage to the muscle. Other researchers are studying how many different forms of myositis exist and how they are different from one another in their cause and treatment. Musculoskeletal system disorder is another of its kind. Other disorders caused by it also include arthritis. They not only cause internal damages but also cause different skin infections. As scientists get closer to understanding the process leading to muscle damage in the different forms of myositis they can hope to find new ways of stopping the harmful actions. With the scientific progress that has been made in recent years people with myositis have reason to share that hope. Research holds the key to better ways to prevent diagnose and control myositis.

Rheumatologists and orthopaedic doctors treating these conditions work to minimize pain and discomfort in the joints. Treatment focuses on improving quality of life and getting patients back to the activities they enjoy. While non-operative solutions, such as physical therapy, steroid injections into the joint, and lifestyle modification, can be effective in treating painful musculoskeletal conditions, surgery is sometimes necessary to alleviate rheumatic symptoms. Prior to beginning a treatment for a condition such as osteoarthritis or rheumatoid arthritis, it is best to speak with an orthopaedic doctor or rheumatologist to ensure the best treatment path possible. When a rheumatologist feels that treatment will no longer alleviate the pain and disability

Therapies for accelerating muscle regeneration, primarily through inhibition of

myostatin, have shown promise in the laboratory and are now entering clinical trials.

Recent studies have implicated autophagy, a key cellular process involved in clearance of ubiquitinated aggregates, in the pathogenesis of familial and sporadic inclusion body myositis (sIBM). IBM has joined a growing list of diseases known as TDP-43 proteinopathies, in which this protein becomes mislocalized to the cytoplasm; however, it is unclear whether these protein aggregates or others are pathogenic in this disease. New discoveries of biomarkers in sIBM and new insights into the pathogenesis of familial IBM are opening novel therapeutic pathways for these disorders. In particular, drugs that stimulate autophagy, already in development for cancer and neurodegenerative diseases, are candidates for clinical trials. These disease-specific therapies combined with novel therapies to accelerate muscle regeneration hold promise for future therapy for this devastating disease.

Pain is a global phenomenon in which it has been estimated that 20% of adults and 8% of children suffer from at any one time of whom 10% suffer chronic pain. Pain is the leading reason for primary care consultations (80%) and musculoskeletal pain is the commonest reason for work absenteeism. Pain is complex and there are aspects that require better recognition and understanding in order to improve its treatment and management.
Pain has been defined as ‘An unpleasant sensory and emotional experience associated with actual or potential tissue damage, or described in terms of such damage’. Pain is generally relieved soon after it is perceived. However, for some the sensation persists long after the normal physiological processes of healing have occurred, leading to persistent daily pain which affect a person’s ability to function and to engage socially and/or emotionally and impacts on their quality of life. Pain is not always linked to pathology and as professionals we may not be able to identify its cause. Never the less in such cases it is important to acknowledge that pain reported by the sufferer will have physiological and psychological aspects in addition
to factors that contribute to persistence. The management of pain can involve pharmacological, surgical or non-pharmacological procedures. Successful interventions are dependent on identifying the causes of the pain and recognising
its multi-systemic nature. To address the multi-systemic aspects requires an inter-professional holistic approach involving the expertise of different medical specialists in collaboration with the pain sufferer. Pain sufferers have also requested education about their pain experience but this can only happen if professionals are able to
adequately communicate their understanding of pain.
 

People with myositis may have significant functional limitations and disability despite appropriate pharmacologic treatment to reduce inflammatory damage to their muscles. The impairments associated with myositis often result in a loss of independent ambulation, difficulty rising from a chair, an inability to negotiate stairs, and diminished health-related quality of life. Therefore, meaningful outcomes measures and effective therapeutic exercise remain important in the medical management of the disease.