Anti-synthetase syndrome: anti-PL-7, anti-PL-12 and anti-EJ

作者：Carlos de Souza Fernando Henrique; Pina Cruellas Marcela Gran; Levy Neto Mauricio; Shinjo Samuel Katsuyuki^*

来源：Revista Brasileira de Reumatologia, 2013, 53(4): 352-357.

• Aminoacyl-tRNA synthetases
• Inflammatory myopathy
• Myositis
• Antisynthetase syndrome

Objectives: Due to the scarcity of studies in the literature, we conducted an analysis of a series of patients with the anti-PL-7, PL-12 and EJ types of antisynthetase syndrome (ASS).
Methods: We conducted a retrospective cohort study of 20 patients with ASS (8 with anti-PL-7, 6 with PL-12, 6 with EJ) monitored in our department between 1982 and 2012.
Results: The mean patient age at disease onset was 38.5 +/- 12.9 years, and the disease duration was 4.5 +/- 6.4 years. Of all the patients, 70% were white and 85% were female. Constitutional symptoms occurred in 90% of cases. All patients presented objective muscle weakness in the limbs; in addition, 30% were bedridden and 65% demonstrated high dysphagia at diagnosis. Joint and pulmonary involvement and Raynaud's phenomenon occurred in 50%, 40% and 65% of cases, respectively, with more than half of the patients presenting incipient pneumopathy, ground-glass opacity and/or pulmonary fibrosis. There were no cases of neurological and/or cardiac involvement. All patients received prednisone or other immunosuppressants depending on tolerance, side effects and/or disease refractoriness. Importantly, patients with the anti-EJ type of ASS demonstrated higher rates of recurrence. Two patients died during follow-up, and 1 patient had breast cancer at the time of diagnosis.
Conclusions: ASS (anti-PL-7, PL-12 and EJ) was found to predominantly affect white women. Although the autoantibodies described in the present study are more related to pulmonary than joint involvement, our patients showed a significant percentage of both types of involvement and a high percentage of myopathy. We also observed a low mortality rate.

• 出版日期2013-8