Conducted in seven reference or competence centres in north-eastern France, the TYPASS retrospective study included 132 adults with antisynthetase syndrome and interstitial lung disease:
- 39% of them had rapidly progressive lung disease,
- the three factors identified as being associated with this rapid progression were the presence of fever, organised pneumonia or pleural effusion,
- in addition, the TYPASS study identified two relevant prognostic profiles for patients,
- the first corresponds to a severe inflammatory phenotype (fever, pleuropericarditis) with a poor prognosis (55% mortality), more often associated with advanced age and rapidly progressive interstitial lung disease (68%),
- the second, with a better prognosis, is associated with a more systemic phenotype, rapid progression of pneumonia, which is rarer (18% of cases), and more frequent anti-Jo-1 autoantibodies.
These parameters could help to optimise monitoring and treatment as soon as the diagnosis is made.
