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Abstract Number: 2222

Predictors of Myositis Treatments Received and Associated Treatment Responses in Myovision, a National Myositis Patient Registry

Abdullah Faiq1, Payam Noroozi Farhadi1, Jesse Wilkerson2, Nastaran Bayat1, Anna Jansen3, Kathryn Rose4, Lukasz Itert5, Anne Johnson6, Christine Parks7, Edward H. Giannini8, Hermine I. Brunner9, Bob Goldberg10, Richard Morris4, Frederick W. Miller1 and Lisa G. Rider1, 1Environmental Autoimmunity Group, NIEHS, NIH, Bethesda, MD, 2Social and Scientific Systems, Inc., Research Triangle Park, NC, 3Environmental Autoimmunity Group, NIEHS / EAG, Bethesda, MD, 4Social and Scientific Systems, Inc., Durham, NC, 5Division of Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 6Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 7Epidemiology Branch, NIEHS, NIH, Research Triangle Park, NC, 8Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 9PRCSG, Cincinnati, OH, 10The Myositis Association, Alexandria, VA

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: juvenile dermatomyositis, Myositis, Polymyositis/dermatomyositis (PM/DM), registry and treatment

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Session Information

Title: Muscle Biology, Myositis and Myopathies: Immunological Aspects of Inflammatory Myopathy

Session Type: Abstract Submissions (ACR)

Background/Purpose: Little is known about medications received for myositis and patients’ responses to therapies. We present information on self-reported myositis therapy use and responses from a national patient registry.

Methods: MYOVISION consists of 1796 patients who met probable or definite Bohan and Peter criteria for DM/PM (708 DM, 483 PM, 139 JDM) or possible Griggs criteria for IBM (466 IBM) with a median diagnosis date of March 2002. Enrolled patients were queried about myositis treatments received and treatment effectiveness. Logistic regression modeling, using a backwards elimination approach, was used to determine demographic and clinical covariates; a significance level of <0.1 was required to retain variables in the model. 

Results: Most DM, PM and JDM patients reported receiving prednisone (96-98%) and methotrexate (MTX) (70-84%); these treatments were reported less commonly in IBM patients (54% and 28%, p<0.0001 respectively). Use of azathioprine (41%, 47%) and rituximab (ritux) (14%, 16%) were reported more frequently in DM and PM, in contrast to IBM and JDM (11%, 15%, p<0.012 and 9%, 10% p<0.007, respectively). JDM patients reported receiving hydroxychloroquine (60%), IV methylprednisolone (54%), IVIG (48%), and cyclosporine (19%) more frequently than other subgroups (2-10% p<0.021 for all). Overall, ritux was the most common biologic therapy (13%), and anti-TNFs were received by 10% of patients.  Factors associated with MTX treatment among DM, PM and IBM patients included younger age, geographic region, absence of lung disease, and type of treating physician (rheumatologist) (p=0.022 - < 0.0001). Younger age, SES, and being treated by a neurologist were factors associated with receipt of IVIG in DM and PM, and presence of dysphagia, fever, and lung disease were additional factors for DM.

Overall, prednisone was reported to be the most helpful medication (39% p<0.007), followed by IVIG (35%, p<0.005), mycophenolate mofetil (31%) and ritux (24%, p<0.029). Some patients (17%) did not find any treatments helpful, and 46% of these had IBM.  Absence of dysphagia in DM (OR 0.66), presence of fever in IBM (OR 5.66), and fewer myositis therapies (OR 0.68 – 0.76) were factors associated with a response to prednisone in DM, PM and JDM. Absence of an overlapping autoimmune disease in PM (OR 0.35) and fewer myositis therapies in DM (OR 0.67) were associated with response to IVIG. IBM patients reported physical therapy as the most effective treatment (38%). Older age (OR 1.03), overlapping autoimmune diseases (OR 2.6), absence of fever (OR 0.13), lung disease (OR 2.1) and receipt of fewer myositis therapies (OR 0.48) predicted a positive response to physical therapy in IBM.

Conclusion: Prednisone and MTX are the most frequently prescribed medications in DM, PM, IBM and JDM. Patients vary substantially in their assessment of the effectiveness of these and other treatment approaches. Demographics, clinical features and the specialty of the treating physician appear to influence which therapies are received by myositis patients and the perception of their effectiveness. In the absence of controlled clinical trials, prospective registries of inception cohorts may aid in identifying effective therapies in rare disorders.


Disclosure:

A. Faiq,
None;

P. Noroozi Farhadi,
None;

J. Wilkerson,
None;

N. Bayat,
None;

A. Jansen,
None;

K. Rose,
None;

L. Itert,
None;

A. Johnson,
None;

C. Parks,
None;

E. H. Giannini,
None;

H. I. Brunner,

Janssen R and D, LLC,

2,

The Myositis Association and NIEHS, NIH,

9;

B. Goldberg,

CDC grant,

2;

R. Morris,
None;

F. W. Miller,
None;

L. G. Rider,
None.

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