The following information may help to address your question:
What is antisynthetase syndrome?
is a chronic autoimmune condition
that affects the muscles and various other parts of the body. The signs and symptoms can vary but may include muscle inflammation (myositis), polyarthritis (inflammation of many joints), interstitial lung disease
, thickening and cracking of the hands,
and Raynaud phenomenon
. The exact underlying cause is unknown; however, the production of autoantibodies (antibodies
that attack normal cells
) that attack certain enzymes
in the body called 'aminoacyl-tRNA
synthetases' appears to be linked to the cause of the syndrome. These autoantibodies may arise after viral infections, or patients may have a genetic predisposition
. Treatment is based on the signs and symptoms present in each person but may include corticosteroids
, immunosuppressive medications, and/or physical therapy
Last updated: 3/10/2017
What are the signs and symptoms of antisynthetase syndrome?
The signs and symptoms of antisynthetase syndrome vary but may include:
- Loss of appetite
- Weight loss
- Muscle inflammation (myositis)
- Inflammation of multiple joints (polyarthritis)
- Interstitial lung disease (ILD) causing shortness of breath, coughing, and/or dysphagia
- Mechanic's hands (thickened skin of tips and margins of the fingers)
- Raynaud phenomenon
Some studies suggest that affected people may be at an increased risk for various types of cancer, as well. Some symptoms of the disease seem to vary according to the autoantibody involved in the disease. Myopathy occurs more often in patients with anti-Jo-1 or anti-PL-7; anti-Jo-1 is related to severe arthritis and "mechanic's hand", while anti-PL-12 with higher rates of Raynaud phenomenon; and anti-PL-7, anti-PL-12, anti-KS, and anti-OJ with cases of ILD.
Last updated: 4/17/2017
What causes antisynthetase syndrome?
The underlying cause of antisynthetase syndrome is currently unknown. However, it is considered an autoimmune disease
. Autoimmune disorders occur when the body's immune system
attacks and destroys healthy body tissue
by mistake. In antisynthetase syndrome, specifically, the production of autoantibodies (antibodies
that attack normal cells instead of disease-causing agents) that recognize and attack certain enzymes
in the body called 'aminoacyl-tRNA synthetases' appears to be linked to the cause of the syndrome. Aminoacyl-tRNA synthetases are involved in protein
production within the body. These autoantibodies seem to appear after certain viral infections, drug exposure or in some people who already have a genetic predisposition. The exact role of autoantibodies in causing antisynthetase syndrome is not yet understood.
Aminoacyl-tRNA synthase (ARS) autoantibodies associated with ASS include anti-Jo1 (anti-histidyl), anti-EJ (anti-glycyl), anti-OJ (anti-isoleucyl), anti-PL7 (anti-threonyl), anti-PL12 (anti-alanyl), anti-SC (anti-lysil), anti-KS (anti-asparaginyl), anti-JS (anti-glutaminyl), anti-Ha or anti-YRS (anti-threonyl), anti-tryptophanyl, and anti-Zo (anti-phenylalanyl) autoantibodies, with anti-Jo1 being the most common.
Last updated: 4/17/2017
What is the long-term outlook for people with antisynthetase syndrome?
The long-term outlook (prognosis
) for people with antisynthetase syndrome varies based on the severity of the condition and the signs and symptoms present. Although the condition is considered chronic
and often requires long-term treatment, those with muscle involvement as the only symptom are generally very responsive to treatment with corticosteroids
and/or immunosuppressive medications. When the lungs are affected, the severity and type of lung condition generally determines the prognosis.
For example, patients with a progressive course of interstitial lung disease
generally have a worse prognosis than those with a nonprogressive course, because respiratory failure is the main cause of death. However, in most cases the interstitial lung disease is nonprogressive.
Several studies have shown that the following factors may be associated with a worse prognosis:
- Older age at onset (greater than 60 years)
- Severity and extension of lung disease: The more severe and extensive lung involvement the worse the prognosis
- Presence of malignancy (cancer)
- Delay in diagnosis and treatment: Prognosis is better when the patients are treated early
- Having a negative Jo1 antibody test: Several studies have shown that Jo1 status seems to be associated with prognosis, suggesting that non-Jo1 patients (patients who have other anti-ARS antibodies, that are non-Jo1) have worse survival rates than Jo1 patients.
Last updated: 4/17/2017
What treatment is available for antisynthetase syndrome?
are typically the first-line of treatment and may be required for several months or years. These medications are often given orally; however, in severe cases, intravenous methylprednisolone
may be prescribe initially. Immunosuppressive medications may also be recommended, especially in people with severe muscle weakness or symptomatic interstitial lung disease
According to recent studies, Rituximab
is the medication option when patients with lung disease do not respond well to other treatments. Physical therapy
is often necessary to improve weakness, reduce further muscle wasting from disuse, and prevent muscle contractures
Last updated: 5/26/2016
Are there any clinical trials for antisynthetase syndrome?
The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov
to provide patients, family members, and members of the public with current information on clinical research studies. Currently, several clinical trials
are identified as enrolling individuals with antisynthetase syndrome. To find these trials, click on the link above. After you click on a study, review its "eligibility" criteria to determine its appropriateness. Use the study’s contact information to learn more. Check this site often for regular updates.
You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling 1-800-411-1222 to speak with a specialist, who can help you determine if you are eligible for any clinical trials. If you are located outside the United States, and would like to be contacted via telephone, you will need to contact PRPL and provide your telephone number in full, including area code and international dialing prefix.
Patient Recruitment and Public Liaison Office
NIH Clinical Center
Bethesda, Maryland 20892-2655
Web site: http://clinicalcenter.nih.gov
You can find information about participating in a clinical trial as well as learn about resources for travel and lodging assistance, through the Get Involved in Research
section of our Web site.
Last updated: 8/10/2015
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