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Antisynthetase syndrome

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Your Question

I have been recently diagnosed with antisynthetase syndrome, acute interstitial lung disease, muscle weakness, skin involvement, scleroderma, and dermatomyositis. Could you please provide me with information on antisynthetase syndrome?  I am also interested in learning about prognosis, treatment, and clinical trials.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is antisynthetase syndrome?

Antisynthetase syndrome is a chronic autoimmune disease. It is a subgroup of the idiopathic (unknown cause) inflammatory muscle diseases (myopathies) and is characterized by myositis, interstitial lung disease, arthritisRaynaud's phenomenon and the finding of autoantibodies to “aminoacyl transfer RNA synthetases” (anti-ARS) in a blood exam. The exact cause is not known. The diagnosis is based on the clinical features and is confirmed in the presence of positive serologic testing for anti-ARS antibodies (anti-Jo-1, anti-PL-12, anti-PL-7, anti-OJ; anti-KS). The treatment is done with oral corticosteroids (prednisone) and other immunosuppressive agents.[1][2][3]
Last updated: 4/28/2015

What symptoms are associated with antisynthetase syndrome?

Symptoms of antisynthetase syndrome vary from person to person and may include [4]:

Last updated: 5/15/2008

What causes antisynthetase syndrome?

The cause of antisynthetase syndrome is unknown; however, the production of a group 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. The amicoacyl-tRNA synthetases are a family of enzymes which play a vital role in protein synthesis in the body. The autoantibody more commonly associated with antisynthetase syndrome is anti-Jo-1.  [1][4] Their exact role in causation of antisynthetase syndrome is not yet known, but viruses have been implicated.  The fact that the onset of symptoms of antisynthetase syndrome typically occur during winter provides indirect evidence that infectious agents may play a role. [4]
Last updated: 5/15/2008

What prognosis is associated with antisynthetase syndrome?

The severity and type of lung involvement typically determines the outcome of the disease. Interstitial lung disease may follow either a progressive or nonprogressive course, with the progressive course marked by cloudy spots in the lungs on CT scan and the presence of neutrophilia in the bronchoalveolar lavage fluid (a diagnostic procedure of washing a sample of cells and secretions from the alveolar and bronchial airspaces of the lung). [5]
Last updated: 5/15/2008

What treatment is available for antisynthetase syndrome?

Corticosteroids are typically the first-line of treatment with the goal of driving the muscle enzymes down to normal or near-normal. Steroid therapy is usually tapered over several months.  Other immunosuppressives may be used in cases where there are skin manifestations of dermatomyositis and where interstitial lung disease is unresponsive to less-aggressive therapies. [1][5] Depending on the presentation and severity, drug treatment used may include azathioprine, methotrexate, cyclophosphamide, cyclosporine, tacrolimus, mycophenolate mofetil, or rituximab. Skin symptoms have been treated effectively with hydroxychloroquine. In patients with associated respiratory problems, the response to immunosuppressive treatment is generally good. Intravenous immunoglobulin (IVIg) have been used to treat the myositis observed in antisynthetase syndrome. In addition, physical therapy is generally recommended.[5]
Last updated: 4/28/2015

Are there any clinical trials for antisynthetase syndrome?

Currently, there are no clinical trials specifically for antisynthetase syndrome listed through  However, there are studies investigating some of the symptoms associated with antisynthetase syndrome such as myositis, interstitial lung disease, and scleroderma.   Click on the following link to read about these studies.

You can search for other trials by visiting their Web site and entering a disease or condition in their search box.  Additionally, you can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling the toll-free number listed below to speak with a specialist, who can help you determine if there are any clinical trials for which an individual with antisynthetase syndrome may be eligible.   If you are located outside the United States, and would like to be contacted via telephone, you will need to 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
Toll-free: 800-411-1222
Fax: 301-480-9793
Web site:

To find helpful general information on clinical trials at the following Web page.

A tutorial about clinical trials can be found at the following link from the National Library of Medicine:

Resources on many charitable or special-fare flights to research and treatment sites and low-cost hospitality accommodations for outpatients and family members, as well as ambulance services, are listed on the Web site of the Office of Rare Diseases (ORD), part of the National Institutes of Health.

Last updated: 5/15/2008

See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.