Living with Myositis

Therapy and research help patients maintain independence and quality of life

Retired commercial airline pilot William “Augie” DeAugustinis has enjoyed an active lifestyle keeping up with eight grand-daughters. The 67-year-old maintained good habits he formed as a pilot to stay in shape and eat well.
DeAugustinis began noticing some unusual changes in his strength and energy levels. He began to fall, and his hand strength decreased. Leg weakness made it hard for him to stand up after sitting. “These were early warning signs that I didn’t realize,” remembers DeAugustinis, of Gainesville, Georgia.

In 2008, a blood test found abnormally high levels of the muscle enzyme creatine kinase (CPK), showing that his muscles were breaking down. It took five physicians the next 16 months to come to an accurate diagnosis: inclusion body myositis (IBM).

Living With Myositis – JHBMC Article

Refractory Myositis: What Can Be Done?

Myositis can be difficult to treat. There are no standard treatment guidelines to follow, and the disease is heterogenous. Conventional treatment consists of steroids and an immunosuppressant such as Methotrexate, Azathioprine or Mycophenolate Mofetil.  If patients continue to be symptomatic after an adequate trial of at least 3 months on therapeutic doses of these medications, this is considered refractory disease.  Other treatment strategies that can be employed in this situation are discussed in this concise article. (Registration is Required)

Myositis Research at Johns Hopkins

Discover more about the ongoing research into the autoimmune disease, myositis, at Johns Hopkins from the co-directors of the Myositis Center, Dr. Lisa Christopher-Stine and Dr. Andrew Mammen.

AARDA Applauds Fox Sports Supports for Selecting Johns Hopkins Medicine as a 2013 Partner Charity

DETROIT, March 12, 2013 — The American Autoimmune Related Diseases Association, Inc. (AARDA) salutes FOX Sports Supports, FOX Sports Media Group’s (FSMG) charitable initiative, for naming John Hopkins Medicine (JHM) one of three 2013 partner charities, citing its work conducting extensive research into the causes of autoimmune diseases, including multiple sclerosis, lupus, arthritis and myositis, and developing new treatments to provide short and long-term relief.

Throughout the year, FSMG will focus all of its charitable resources toward JHM and the two other charities in campaigns that span all of FSMG’s sports properties, premier events and assets which started with the Daytona 500 and will culminate at the 2014 Super Bowl in New York/New Jersey.

Read the full press release here.

Follistatin Gene Transfer Trial to Begin in December 2011

ANNOUNCEMENTS: We are pleased to inform you that the Follistatin gene transfer for inclusion-body myositis has cleared its last regulatory hurdle and recruitment of patients begins this December.  The trial to be conducted at Nationwide Children’s Hospital in Columbus, OH expects to select patients in December to be enrolled, with the first patient injection to begin in January, 2012.  The group at Nationwide Children’s have worked hard to begin what we hope will be helpful to patients with sIBM to improve muscle strength.  TMA will keep in touch with the investigators regarding the progress of the trial.  Should you have any questions about the trial or patient recruitment, please contact Xiomara Rosales-Quintero at Xiomara.Rosales-Quintero@nationwidechildrens.org.

Neuronow article: Unmasking the Mystery of Myositis

Myositis is a painful, debilitating muscle disease in which the immune system attacks healthy muscle tissue. Its chief symptom is muscle weakness, but because it so closely resembles other diseases, it’s difficult to diagnose. In fact, most patients see as many as six doctors, on average, before they get a proper diagnosis.

Tamika Moore’s primary care doctor believed she had liver disease. The 26-year-old physical therapist on staff at Johns Hopkins was inexplicably weak; a blood test had showed elevated liver enzymes. A gastroenterologist scheduled her for a liver biopsy. A second blood test, though, showed abnormally high amounts of the muscle enzyme creatine kinase, an indicator of muscle disease.

Moore’s next stop? The Myositis Center where specialists in muscle disease, including neurologists, rheumatologists and a pulmonologist treat adults with all forms of the disease: polymyositis, which sometimes affects the lungs; dermatomyositis, which is often accompanied by a skin rash and is linked to a high rate of cancer; and inclusion myositis, an inherited form of the disease.

Patients undergo a battery of tests, most often electromyography (EMG) to assess the health of the nerves controlling the muscles, muscle MRI, CT scans, and pulmonary function tests. All can usually be done in one visit.

Patients are treated medically with drugs, especially corticosteroids. “A significant fraction go into remission. A second group continues treatment with some sign of disease. A third group,” says center co-director Andrew Mammen, “is difficult to help, but you keep trying.”

Center co-director Lisa Christopher-Stine is collecting clinical, serologic, imaging and pathologic information from large numbers of patients to help determine optimal treatments. “Now,” says Mammen, “we have a grab bag of medicines, but we can’t predict which patient will be most helped by which medicine.”

One of the center’s strengths is that it is jointly led by a neurologist, Mammen, and a rheumatologist, Christopher-Stine. “That’s unusual because with autoimmune disease, neurologists and rheumatologists don’t always see eye to eye,” says Mammen. “Neurologists think rheumatologists are poor at diagnosis; rheumatologists think neurologists are poor at using medicines to treat immuno-supression. Lisa and I, though, have none of those hang-ups. We’re learning from one another.”

Myositis: A Misdiagnosed Medical Mystery

Source: ABC News – Good Morning America