Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose: Half of all polymyositis (PM)/dermatomyositis (DM) patients suffer from muscle weakness after initial treatment. Therefore, many patients with PM/DM have trouble with daily living even after disease activity has been adequately controlled. However, details regarding the clinical factors that are associated with the disability of physical function after treatment in PM/DM remain unclear. The aim of our study was to clarify the clinical manifestations and myositis-specific autoantibodies (MSA) that are associated with the severity of physical dysfunction after treatments in PM/DM.
Methods: In the present study, Seventy seven patients who were diagnosed with PM, DM or clinically amyopathic DM (CADM) were retrospectively enrolled. They were admitted to our hospital from December 1991 to February 2013 because of initial treatment for PM/DM/CADM. Diagnoses were made based on the criteria of Bohan and Peter or those of Sontheimer. We obtained clinical data from their medical records, including age of disease onset, gender, and disease duration. We also obtained laboratory data prior to initial treatment, such as CK, LD, antinuclear antibodies and MSA, the content of treatment, and the presence of relapse. We evaluated the physical dysfunction of each patient after treatment from August to October 2013 using the Japanese version of the Health Assessment Questionnaire Disability Index (J-HAQ-DI). We retrospectively analyzed the clinical data prior to the initial treatment as predictors for the severity of physical dysfunction after treatment.
Results: Of the 77 patients with PM/DM/CADM, the median age of disease onset was 46 years old, and 79% of the patients were female. The numbers of PM, DM, and CADM cases were 40, 30, and 7, respectively. Anti-aminoacyl-tRNA synthetase antibody (anti-ARS), anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5), and anti-signal recognition particle antibody (anti-SRP) were identified in 25, 7, and 9 patients, respectively. Anti-Mi-2, anti-TIF1-γ, and anti-NXP-2 were also identified in a few patients. The median dose of prednisolone (PSL) at the initial treatment was 50 mg/day. The median of J-HAQ-DI score after treatment was 0.125 (range 0-2.75). In a multivariate analysis, the age of disease onset (t value=4.72, p<0.0001), female status (t=2.80, p<0.01), and the initial doses of PSL (t=1.83, p=0.073) were associated with the severity of the J-HAQ-DI score after treatment. The serum levels of CK prior to treatment, the presence of mPSL pulse therapy, and the relapse of the diseases were not associated with the severity of the J-HAQ-DI scores after treatment. From the viewpoint of MSA, anti-SRP positivity was associated with more severe physical dysfunction after treatment than other MSAs. However, anti-MDA-5-positivity was associated with better physical function after treatment than other MSAs.
Conclusion: The age of disease onset, gender, and the initial dose of PSL were significant factors associated with the severity of physical dysfunction after treatment in PM/DM. The clarification of MSA is useful for predicting the prognosis of physical function after treatment in PM/DM.
Disclosure:
H. Kawasumi,
None;
T. Gono,
None;
Y. Kawaguchi,
None;
Y. Katsumata,
None;
H. Ichida,
None;
A. Tochimoto,
None;
M. Hanaoka,
None;
Y. Okamoto,
None;
S. Kataoka,
None;
H. Yamanaka,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/analysis-of-clinical-manifestations-and-myositis-specific-autoantibodies-associated-with-severity-of-physical-dysfunction-after-treatment-for-polymyositis-and-dermatomyositis/