Inflammatory Myopathies Information Page

Inflammatory Myopathies Information Page


What research is being done?

The National Institue of Neurological Disorders and Stroke (NINDS), along with other agencies within the National Institutes of Health (NIH), conducts and supports a wide range of research on neuromuscular disorders, including myositis and the inflammatory myopathies. The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) is the primary funding institute for these efforts.

NIH researchers are working to identify the causes of muscle weakness in order to discover effective treatments. Researchers are working to develop objective, imaging-based methods for describing the muscle damage associated with inflammatory muscle disease.  NIH-funded researchers are studying childhood-onset polymyositis and dermatomyositis to learn more about their causes, immune system changes throughout the course of the disease, and associated medical problems.  Also, NIH-funded researchers are looking for better, less invasive ways of diagnosing these disorders. Other NIH-funded researchers are studying the genetic diversity disease susceptibility in the inflammatory myopathies. 

Information from the National Library of Medicine’s MedlinePlus
Myositis

×
What research is being done?

The National Institue of Neurological Disorders and Stroke (NINDS), along with other agencies within the National Institutes of Health (NIH), conducts and supports a wide range of research on neuromuscular disorders, including myositis and the inflammatory myopathies. The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) is the primary funding institute for these efforts.

NIH researchers are working to identify the causes of muscle weakness in order to discover effective treatments. Researchers are working to develop objective, imaging-based methods for describing the muscle damage associated with inflammatory muscle disease.  NIH-funded researchers are studying childhood-onset polymyositis and dermatomyositis to learn more about their causes, immune system changes throughout the course of the disease, and associated medical problems.  Also, NIH-funded researchers are looking for better, less invasive ways of diagnosing these disorders. Other NIH-funded researchers are studying the genetic diversity disease susceptibility in the inflammatory myopathies. 

Information from the National Library of Medicine’s MedlinePlus
Myositis

The National Institue of Neurological Disorders and Stroke (NINDS), along with other agencies within the National Institutes of Health (NIH), conducts and supports a wide range of research on neuromuscular disorders, including myositis and the inflammatory myopathies. The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) is the primary funding institute for these efforts.

NIH researchers are working to identify the causes of muscle weakness in order to discover effective treatments. Researchers are working to develop objective, imaging-based methods for describing the muscle damage associated with inflammatory muscle disease.  NIH-funded researchers are studying childhood-onset polymyositis and dermatomyositis to learn more about their causes, immune system changes throughout the course of the disease, and associated medical problems.  Also, NIH-funded researchers are looking for better, less invasive ways of diagnosing these disorders. Other NIH-funded researchers are studying the genetic diversity disease susceptibility in the inflammatory myopathies. 

Information from the National Library of Medicine’s MedlinePlus
Myositis

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Definition
Definition
Treatment
Treatment
Prognosis
Prognosis
Clinical Trials
Clinical Trials
Organizations
Organizations
Publications
Publications
Definition
Definition

The inflammatory myopathies are a group of diseases, with no known cause, that involve chronic muscle inflammation accompanied by muscle weakness.  The four main types of chronic, or long-term, inflammatory myopathies are polymyositis, dermatomyositis, inclusion body myositis, and necrotizing autoimmune myopathy.  Although the cause of many inflammatory myopathies is unknown, the majority are considered to be autoimmune disorders, in which the body’s immune response system that normally defends against infection and disease attacks its own muscle fibers, blood vessels, connective tissue, organs, or joints. These rare disorders may affect both adults and children, although dermatomyositis is more common in children.  Polymyositis and dermatomyositis are more common in women than in men.  Inclusion body myositis is most common after age 50. General symptoms of chronic inflammatory myopathy include slow but progressive muscle weakness that starts in the proximal muscles—those muscles closest to the trunk of the body. Other symptoms include fatigue after walking or standing, tripping or falling, and difficulty swallowing or breathing.  Some individuals may have slight muscle pain or muscles that are tender to the touch.  Polymyositis affects skeletal muscles (involved with making movement) on both sides of the body.  Dermatomyositis is characterized by a skin rash that precedes or accompanies progressive muscle weakness.  IBM is characterized by progressive muscle weakness and wasting.  Juvenile myositis has some similarities to adult dermatomyositis and polymyositis. Symptoms of necrotizing autoimmune myopathy include weakness in both the upper and lower body, difficulty rising from low chairs, climbing stairs, or lifting objects, fatigue, weight loss, and muscle pain.

 

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Definition

The inflammatory myopathies are a group of diseases, with no known cause, that involve chronic muscle inflammation accompanied by muscle weakness.  The four main types of chronic, or long-term, inflammatory myopathies are polymyositis, dermatomyositis, inclusion body myositis, and necrotizing autoimmune myopathy.  Although the cause of many inflammatory myopathies is unknown, the majority are considered to be autoimmune disorders, in which the body’s immune response system that normally defends against infection and disease attacks its own muscle fibers, blood vessels, connective tissue, organs, or joints. These rare disorders may affect both adults and children, although dermatomyositis is more common in children.  Polymyositis and dermatomyositis are more common in women than in men.  Inclusion body myositis is most common after age 50. General symptoms of chronic inflammatory myopathy include slow but progressive muscle weakness that starts in the proximal muscles—those muscles closest to the trunk of the body. Other symptoms include fatigue after walking or standing, tripping or falling, and difficulty swallowing or breathing.  Some individuals may have slight muscle pain or muscles that are tender to the touch.  Polymyositis affects skeletal muscles (involved with making movement) on both sides of the body.  Dermatomyositis is characterized by a skin rash that precedes or accompanies progressive muscle weakness.  IBM is characterized by progressive muscle weakness and wasting.  Juvenile myositis has some similarities to adult dermatomyositis and polymyositis. Symptoms of necrotizing autoimmune myopathy include weakness in both the upper and lower body, difficulty rising from low chairs, climbing stairs, or lifting objects, fatigue, weight loss, and muscle pain.

 

Treatment
Treatment

The chronic inflammatory myopathies can’t be cured in most adults but many of the symptoms can be treated.  Options include medication, physical therapy, and rest.  Polymyositis, dermatomyositis, and necrotizing autoimmune nmyopathy are first treated with high doses of corticosteroid drugs such as prednisone. Immunosuppressant drug may reduce inflammation in individuals who do not respond well to prednisone. Injections of adrenocorticotropic hormone gel may be another option for people who do not respond to or cannot tolerate other drug treatment options. Physical therapy is usually recommended to prevent muscle atrophy as well as to maintain muscle strength and range of motion. Some individuals may use a topical cream to treat skin problems associated with the disorder. IBM has no standard course of treatment. 

×
Treatment

The chronic inflammatory myopathies can’t be cured in most adults but many of the symptoms can be treated.  Options include medication, physical therapy, and rest.  Polymyositis, dermatomyositis, and necrotizing autoimmune nmyopathy are first treated with high doses of corticosteroid drugs such as prednisone. Immunosuppressant drug may reduce inflammation in individuals who do not respond well to prednisone. Injections of adrenocorticotropic hormone gel may be another option for people who do not respond to or cannot tolerate other drug treatment options. Physical therapy is usually recommended to prevent muscle atrophy as well as to maintain muscle strength and range of motion. Some individuals may use a topical cream to treat skin problems associated with the disorder. IBM has no standard course of treatment. 

Definition
Definition

The inflammatory myopathies are a group of diseases, with no known cause, that involve chronic muscle inflammation accompanied by muscle weakness.  The four main types of chronic, or long-term, inflammatory myopathies are polymyositis, dermatomyositis, inclusion body myositis, and necrotizing autoimmune myopathy.  Although the cause of many inflammatory myopathies is unknown, the majority are considered to be autoimmune disorders, in which the body’s immune response system that normally defends against infection and disease attacks its own muscle fibers, blood vessels, connective tissue, organs, or joints. These rare disorders may affect both adults and children, although dermatomyositis is more common in children.  Polymyositis and dermatomyositis are more common in women than in men.  Inclusion body myositis is most common after age 50. General symptoms of chronic inflammatory myopathy include slow but progressive muscle weakness that starts in the proximal muscles—those muscles closest to the trunk of the body. Other symptoms include fatigue after walking or standing, tripping or falling, and difficulty swallowing or breathing.  Some individuals may have slight muscle pain or muscles that are tender to the touch.  Polymyositis affects skeletal muscles (involved with making movement) on both sides of the body.  Dermatomyositis is characterized by a skin rash that precedes or accompanies progressive muscle weakness.  IBM is characterized by progressive muscle weakness and wasting.  Juvenile myositis has some similarities to adult dermatomyositis and polymyositis. Symptoms of necrotizing autoimmune myopathy include weakness in both the upper and lower body, difficulty rising from low chairs, climbing stairs, or lifting objects, fatigue, weight loss, and muscle pain.

 

Treatment
Treatment

The chronic inflammatory myopathies can’t be cured in most adults but many of the symptoms can be treated.  Options include medication, physical therapy, and rest.  Polymyositis, dermatomyositis, and necrotizing autoimmune nmyopathy are first treated with high doses of corticosteroid drugs such as prednisone. Immunosuppressant drug may reduce inflammation in individuals who do not respond well to prednisone. Injections of adrenocorticotropic hormone gel may be another option for people who do not respond to or cannot tolerate other drug treatment options. Physical therapy is usually recommended to prevent muscle atrophy as well as to maintain muscle strength and range of motion. Some individuals may use a topical cream to treat skin problems associated with the disorder. IBM has no standard course of treatment. 

Prognosis
Prognosis

Most cases of dermatomyositis respond to therapy. Approximately one-third of individuals with juvenile-onset dermatomyositis recover from their illness, one-third have a relapsing-remitting course of disease, and the other third have a more chronic course of illness. The prognosis for polymyositis varies.  Most individuals respond fairly well to therapy, but some people have a more severe disease that does not respond adequately to therapies and may have significant disability.  IBM is generally resistant to all therapies and its rate of progression appears to be unaffected by currently available treatments.  Necrotizing autoimmune myopathy generally responds well to long-term combination immunosuppressive therapies.

 

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Most cases of dermatomyositis respond to therapy. Approximately one-third of individuals with juvenile-onset dermatomyositis recover from their illness, one-third have a relapsing-remitting course of disease, and the other third have a more chronic course of illness. The prognosis for polymyositis varies.  Most individuals respond fairly well to therapy, but some people have a more severe disease that does not respond adequately to therapies and may have significant disability.  IBM is generally resistant to all therapies and its rate of progression appears to be unaffected by currently available treatments.  Necrotizing autoimmune myopathy generally responds well to long-term combination immunosuppressive therapies.

 

Prognosis
Prognosis

Most cases of dermatomyositis respond to therapy. Approximately one-third of individuals with juvenile-onset dermatomyositis recover from their illness, one-third have a relapsing-remitting course of disease, and the other third have a more chronic course of illness. The prognosis for polymyositis varies.  Most individuals respond fairly well to therapy, but some people have a more severe disease that does not respond adequately to therapies and may have significant disability.  IBM is generally resistant to all therapies and its rate of progression appears to be unaffected by currently available treatments.  Necrotizing autoimmune myopathy generally responds well to long-term combination immunosuppressive therapies.

 

Definition

The inflammatory myopathies are a group of diseases, with no known cause, that involve chronic muscle inflammation accompanied by muscle weakness.  The four main types of chronic, or long-term, inflammatory myopathies are polymyositis, dermatomyositis, inclusion body myositis, and necrotizing autoimmune myopathy.  Although the cause of many inflammatory myopathies is unknown, the majority are considered to be autoimmune disorders, in which the body’s immune response system that normally defends against infection and disease attacks its own muscle fibers, blood vessels, connective tissue, organs, or joints. These rare disorders may affect both adults and children, although dermatomyositis is more common in children.  Polymyositis and dermatomyositis are more common in women than in men.  Inclusion body myositis is most common after age 50. General symptoms of chronic inflammatory myopathy include slow but progressive muscle weakness that starts in the proximal muscles—those muscles closest to the trunk of the body. Other symptoms include fatigue after walking or standing, tripping or falling, and difficulty swallowing or breathing.  Some individuals may have slight muscle pain or muscles that are tender to the touch.  Polymyositis affects skeletal muscles (involved with making movement) on both sides of the body.  Dermatomyositis is characterized by a skin rash that precedes or accompanies progressive muscle weakness.  IBM is characterized by progressive muscle weakness and wasting.  Juvenile myositis has some similarities to adult dermatomyositis and polymyositis. Symptoms of necrotizing autoimmune myopathy include weakness in both the upper and lower body, difficulty rising from low chairs, climbing stairs, or lifting objects, fatigue, weight loss, and muscle pain.

 

Treatment

The chronic inflammatory myopathies can’t be cured in most adults but many of the symptoms can be treated.  Options include medication, physical therapy, and rest.  Polymyositis, dermatomyositis, and necrotizing autoimmune nmyopathy are first treated with high doses of corticosteroid drugs such as prednisone. Immunosuppressant drug may reduce inflammation in individuals who do not respond well to prednisone. Injections of adrenocorticotropic hormone gel may be another option for people who do not respond to or cannot tolerate other drug treatment options. Physical therapy is usually recommended to prevent muscle atrophy as well as to maintain muscle strength and range of motion. Some individuals may use a topical cream to treat skin problems associated with the disorder. IBM has no standard course of treatment. 

Prognosis

Most cases of dermatomyositis respond to therapy. Approximately one-third of individuals with juvenile-onset dermatomyositis recover from their illness, one-third have a relapsing-remitting course of disease, and the other third have a more chronic course of illness. The prognosis for polymyositis varies.  Most individuals respond fairly well to therapy, but some people have a more severe disease that does not respond adequately to therapies and may have significant disability.  IBM is generally resistant to all therapies and its rate of progression appears to be unaffected by currently available treatments.  Necrotizing autoimmune myopathy generally responds well to long-term combination immunosuppressive therapies.

 

What research is being done?

The National Institue of Neurological Disorders and Stroke (NINDS), along with other agencies within the National Institutes of Health (NIH), conducts and supports a wide range of research on neuromuscular disorders, including myositis and the inflammatory myopathies. The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) is the primary funding institute for these efforts.

NIH researchers are working to identify the causes of muscle weakness in order to discover effective treatments. Researchers are working to develop objective, imaging-based methods for describing the muscle damage associated with inflammatory muscle disease.  NIH-funded researchers are studying childhood-onset polymyositis and dermatomyositis to learn more about their causes, immune system changes throughout the course of the disease, and associated medical problems.  Also, NIH-funded researchers are looking for better, less invasive ways of diagnosing these disorders. Other NIH-funded researchers are studying the genetic diversity disease susceptibility in the inflammatory myopathies. 

Information from the National Library of Medicine’s MedlinePlus
Myositis

Patient Organizations
American Autoimmune Related Diseases Association
22100 Gratiot Avenue
Eastpointe
MI
Eastpointe, MI 48021-2227
Tel: 586-776-3900; 800-598-4668
Arthritis Foundation
P.O. Box 7669
Atlanta
GA
Atlanta, GA 30357
Tel: 800-283-7800; 404-872-7100; 404-965-7888
Muscular Dystrophy Association
National Office - 222 S. Riverside Plaza
Suite 1500
Chicago
IL
Chicago, IL 60606
Tel: 800-572-1717
Myositis Association
1737 King Street
Suite 600
Alexandria
VA
Alexandria, VA 22314
Tel: 703-299-4850; 800-821-7356
National Institute of Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse
1 AMS Circle
Bethesda
MD
Bethesda, MD 20892-3675
Tel: 877-22-NIAMS (226-4267); 301-565-2966 (TTY)
National Institute of Environmental Health Sciences (NIEHS)
National Institutes of Health, DHHS
111 T.W. Alexander Drive
Research Triangle Park
NC
Research Triangle Park, NC 27709
Tel: 919-541-3345
Publications

Polymyositis information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Inclusion Body Myositis (Inflammatory Myopathy) information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Dermatomyositis information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Patient Organizations