Anti-Jo-1 antisynthetase syndrome.
OBJECTIVE
Given a lack of population-based studies, we report an epidemiological-clinic study of anti-Jo-1 antisynthetase syndrome (ASS).


METHODS
To study a retrospective cohort of a single-center from 1980 to 2010. Clinical-laboratory and demographic data were obtained from medical files. All patients fulfilled the Bohan and Peter criteria (1975) and presented anti-Jo-1, articular, muscle and lung involvement. Eighteen patients with anti-Jo-1 ASS were analyzed.


RESULTS
The mean age at disease onset was 39.9 ± 15.7 years and average disease duration was 9.7 ± 7.0 years. All subjects were white, and 94.4% were female. Constitutional symptoms occurred in 50 % of cases. There was cutaneous and gastrointestinal tract involvement in 66.6% and 55.6% of cases, respectively. No cases manifested neurologic or cardiac involvement. Half of the patients showed incipient pneumopathy, ground-glass opacities and basal pulmonary fibrosis. There was one case of tuberculosis, three of herpes zoster and one of non-Hodgkin lymphoma. One death occurred due to sepsis shock (hospital bronchopneumonia). All patients received prednisone (1mg/kg/day) and 12 (66.7%) participants received methyl prednisolone pulse therapy (1g/day, 3 days). Various immunosuppressants were used as corticosteroid tapers, depending on tolerance, side effects and/or refractoriness. Although disease relapse (clinical and/or laboratory) occurred in 87.5% of cases, 12 out of 16 patients (75%) were in disease remission at study endpoint.


CONCLUSIONS
In the present study, almost all patients were white females and the disease relapse rate was high.
Division of Rheumatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. samuel.shinjo@gmail.com
Samuel Katsuyuki
SK
Shinjo
Mauricio
M
Levy-Neto
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D005260:Female; D006643:Histidine-tRNA Ligase / Q000276:immunology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000276:immunology; D012189:Retrospective Studies; D055815:Young Adult
D006643:Histidine-tRNA Ligase