Identification Of Role For Proteins In Children's Muscle Disease Could Open Up New Treatment Options

Main Category: Arthritis / Rheumatology
Also Included In: Pediatrics / Children's Health
Article Date: 26 Jun 2006 - 21:00 PDT

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A study presented by Mrs. Elisabeth Elst today shows for the first time that a protein - heat shock protein 60 (HSP60) - that is present in chronic inflammations, triggers a response by T-cells (a type of white blood cells that plays a part in the body's own immune response) in children with juvenile dermatomyositis (JDM).

The specific response has earlier been observed in juvenile idiopathic arthritis, but to date, little is known about the role of HSP60 in inflammatory myositis. Inflammatory myositis (IM) is the name given to a group of diseases that cause inflammation in the muscles of the body, which is mediated by the immune system of the body. The main symptoms are pain and weakness and can cause patient disability because of damage to the muscles. The main types of IM are dermatomyositis and polymyositis.

For children, the conditions of myositis are complex and are characterized by muscle damage due to an inflammatory process of the blood vessels that lie under the skin and muscles. Some of the symptoms include skin changes around the eyelids and over the knuckles and finger joints, as well as weakness in muscles, mainly affecting the large muscles around the hips and shoulders resulting in increased difficulty with walking, climbing stairs, getting up from the floor and lifting the arms. The children also often become uncharacteristically miserable and fractious and they may complain of tummy pain.

Heat shock proteins (HSP) are a group of proteins whose expression is increased when the cells are exposed to elevated temperatures. Production of high levels of heat shock proteins can also be triggered by exposure to different kinds of environmental stress conditions, such as infection, inflammation, exposure of the cell to toxins (e.g. ethanol, arsenic, and ultraviolet light), or water deprivation.

Mrs. Elst, pediatric immunologist at the University Medical Centre Utrecht, said, "We have shown for the first time that HSP60 plays an active part in the control of the inflammatory process in JDM. Thus, therapy aimed at the expansion of T-cells with regulatory capacities reacting to HSP60 could contribute to disease remission in patients with JDM. This conclusion opens up new perspectives for the understanding and approach for antigens in immunotherapy."

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Abstract number: OP0060

About EULAR

* The European League Against Rheumatism (EULAR) is the organization which represents the patient, health professional and scientific societies of rheumatology of all the European nations.
* The aims of EULAR are to reduce the burden of rheumatic diseases on the individual and society and to improve the treatment, prevention and rehabilitation of musculoskeletal diseases. To this end, EULAR fosters excellence in education and research in the field of rheumatology. It promotes the translation of research advances into daily care and fights for the recognition of the needs of people with musculoskeletal diseases by the governing bodies in Europe.
* Diseases of bones and joints, such as rheumatoid arthritis and osteoarthritis cause disability in 4 - 5 % of the adult population and are predicted to rise as people live longer.
* As new treatments emerge and cellular mechanisms are discovered, the 7th Annual European Congress of Rheumatology in Amsterdam (EULAR 2006) brings together more than 10,000 experts - scientists, clinicians, healthcare workers, pharmaceutical companies and patients - to share their knowledge in a global endeavour to challenge the pain and disability caused by musculo-skeletal disorders.
* To find out more information about the activities of EULAR, visit: http://www.eular.org/.

Contact: Mia Gannedahl
European League Against Rheumatism

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