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OP0038 Myositis autoantibodies outperform clinical subgroup classification in predicting muscle weakness in myositis patients
  1. I Pinal-Fernandez1,
  2. W Huang1,
  3. M Casal-Dominguez1,
  4. E Tiniakou2,
  5. C Parks1,
  6. K Pak1,
  7. J Albayda2,
  8. JJ Paik2,
  9. SK Danoff3,
  10. L Christopher-Stine2,
  11. AL Mammen1
  1. 1Muscle Disease Unit, NIAMS/NIH, Bethesda
  2. 2Rheumatology
  3. 3Pulmonology, Johns Hopkins University, Baltimore, United States

Abstract

Background Myositis patients may be classified as belonging to one of four clinical groups: dermatomyositis (DM), polymyositis (PM), clinically amyopathic dermatomyositis (CADM) or necrotizing myositis (NM). Alternatively, myositis patients may be classified according to myositis autoantibody status.

Objectives The aim of this study was to determine whether clinical groups or myositis autoantibodies provide better prognostic categories with regard to muscle involvement in these patients.

Methods All Johns Hopkins Myositis Center patients from 2002 to 2015 with a myositis specific autoantibody confirmed by two different immunologic techniques were included. Autoantibody groups accounting for less than 2% of the final sample size were excluded. Strength (analyzed as the average of deltoid and hip flexor strength using Kendall's scale) and log transformed CK levels were compared between the different autoantibody groups using multilevel regression models adjusted for age, time from disease onset, sex, race and treatments. Models with different combinations of key variables were compared using the likelihood ratio test to ascertain if autoantibody groups and clinical subgroups provided the same amount of information regarding muscle weakness and CK levels over time.

Results 483 patients with 4181 visits were included and 10 different autoantibody groups were identified. Muscle weakness and CK levels followed a gradient among both antibody and clinical groups. Anti-SRP patients had the greatest weakness, followed by anti-HMGCR, anti-Mi2 and anti-NXP2, and then anti-Jo1. CK levels were highest in anti-HMGCR patients, followed by anti-SRP, anti-PL7, anti-Jo1 and anti-Mi2. Interestingly, strength and CK levels were dissociated in two groups: anti-NXP2 patients had significant weakness with low CK levels and anti-PL7 patients were relatively strong despite high CK levels. Multilevel regression models showed autoantibody groups explained the strength and the CK variability better than the clinical groups (AIC difference>20). Indeed, adding clinical groups to a model using only autoantibodies did not improve the model's ability to predict strength (p=0.2) and only mildly improved its ability to predict CK (p=0.01). In comparison, adding the autoantibodies to a model using the clinical groups resulted in a marked improvement in predicting both CK and strength (both p<0.001).

Conclusions In patients with myositis, autoantibody status predicts strength and CK levels better than clinical grouping.

Disclosure of Interest None declared

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