| Full text | |
| Author(s): |
Fernando Henrique Carlos de Souza
[1]
;
Marcela Gran Pina Cruellas
[2]
;
Mauricio Levy-Neto
[1]
;
Samuel Katsuyuki Shinjo
[1]
Total Authors: 4
|
| Affiliation: | [1] Univ Sao Paulo, Fac Med, Sch Med, Rheumatol Serv, Clin Hosp, Sao Paulo - Brazil
[2] Univ Sao Paulo, Fac Med, Div Rheumatol, Sao Paulo - Brazil
Total Affiliations: 2
|
| Document type: | Journal article |
| Source: | REVISTA BRASILEIRA DE REUMATOLOGIA; v. 53, n. 4, p. 352-357, 2013-08-00. |
| Abstract | |
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. (AU) | |
| FAPESP's process: | 11/12700-1 - Profile and prevalence of myositis-specific and myositis-associated autoantibodies in Brazilian population with dermatomyositis/polymyositis |
| Grantee: | Samuel Katsuyuki Shinjo |
| Support Opportunities: | Regular Research Grants |
| FAPESP's process: | 12/03399-9 - Profile and prevalence of myositis-specific autoantibodies and myositis-associated in the Brazilian population with Dermatomyositis/Polymyositis |
| Grantee: | Marcela Gran Pina Cruellas |
| Support Opportunities: | Scholarships in Brazil - Scientific Initiation |