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You are here: Home / Articles / Autoantibodies in Autoimmune Myopathy

Autoantibodies in Autoimmune Myopathy

September 18, 2017 • By Ruth Jessen Hickman, MD

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Dr. Aggarwal advises, “It’s very important physicians screen for interstitial lung disease in these patients, because 70–80% may have or develop interstitial lung disease, and they need to be managed early.”

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Anti-Mi-2 Autoantibody
First described in 1976, the anti-Mi-2 autoantibody became the first serological marker closely linked to DM, occurring in 10 to 30% of DM patients. Researchers later learned that this antibody is directed against the chromatin-remodeling complex, Mi-2/NuRD.1

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These patients tend to have classic skin eruptions alongside mild to moderate myositis and less systemic disease. “Patients with anti-Mi-2 autoantibodies have a milder phenotype, which means these patients in most cases get better more easily and much faster,” explains Dr. Aggarwal. “Generally, you [don’t] need big guns, in the form of second- or third-line immunosuppression, for these patients. A little steroid taper with or without methotrexate or azathioprine may get them significantly better.”

Anti-MDA5 (CADM-140)
For patients with significant skin disease and lung disease, but little weakness, Dr. Gunawardena suggests an anti-MDA5 test. This autoantibody targets MDA5, a cytoplasmic sensor that triggers antiviral responses. It may be particularly prevalent in patients of Asian descent.2,3

Dr. Aggarwal

Dr. Aggarwal

“These patients have generally poor prognosis, especially if they have interstitial lung disease—seen in half of the cases in the U.S.,” explains Dr. Aggarwal. “Their interstitial lung disease can be of a rapidly progressing nature, so a physician needs to act fast when they recognize a patient with interstitial lung disease and anti-MDA5 antibody. Otherwise, 50% of these patients with interstitial lung disease may die within three to six months,” he continues. “In patients with these antibodies, the skin rash tends to be more severe and the outcomes poorer if it’s not treated aggressively.”

In addition to providing information about initial prognosis, some autoantibodies may be able to play a role in monitoring disease activity. For example, high levels of anti-MDA5 despite treatment are a sign of refractory lung disease and increased mortality risk.9

The Boards of EULAR & the ACR recently approved this new set of classification criteria for idiopathic inflammatory myopathies. … The new myositis classification guidelines include various clinical characteristics, muscle biopsy findings & autoantibody results. … Having myositis autoantibodies gives half the points needed to achieve diagnosis of IIM under the new classification criteria.

Anti-TIF1-γ (p155/140) Autoantibodies
The autoantibody anti-TIF1-γ is a particularly important one to consider when assessing risk for cancer-related myositis, which is more common in patients with DM than PM. Patients with these auto­antibodies often have significant disease-resistant skin disease. The autoantibody targets proteins in the human transcription intermediary factor 1 (TIF1) family.2,3

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Filed Under: Conditions, Systemic Inflammatory Syndromes Tagged With: autoantibodies, Autoimmune disease, Classification, connective tissue disease, dermatomyositis, Diagnosis, idiopathic inflammatory myopathies, myositis, patient care, polymyositis, prognosis, Research, rheumatologist, rheumatology, Systemic sclerosis, Test, TreatmentIssue: September 2017

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About Ruth Jessen Hickman, MD

Ruth Jessen Hickman, MD, was born and raised in eastern Kentucky, where she first cultivated her love of literature, writing and personal narratives. She attended Kenyon college, where she received a Bachelor of Arts in philosophy, summa cum laude. She worked with individuals with psychiatric conditions and later in a neuroscience lab at the University of Illinois, Chicago, before graduating from Indiana University Medical School in 2011. Instead of pursuing clinical medicine, Ruth opted to build on her strength of clearly explaining medical topics though a career as a freelance medical writer, writing both for lay people and for health professionals. She writes across the biomedical sciences, but holds strong interests in rheumatology, neurology, autoimmune diseases, genetics, and the intersection of broader social, cultural and emotional contexts with biomedical topics. Ruth now lives in Bloomington, Ind., with her husband, son and cat. She can be contacted via her website at ruthjessenhickman.com.

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