- Design
- International multicentre registry, observational
- Population
- 1,868 patients with anti-synthetase syndrome and 2,043 autoimmune controls
- Primary outcome
- Prevalence of pulmonary hypertension and all-cause mortality
- Effect
- Prevalence 8.9%; mortality HR 2.52 (1.41-4.53)
A worldwide physician-reported registry (Rheumatology, 1 October 2026) compared 1,868 patients with anti-synthetase syndrome against 2,043 controls with other autoimmune diseases from 103 centres.
Pulmonary hypertension was present in 8.9% with anti-synthetase syndrome, more than in other myositis (3%), rheumatoid arthritis (4%) or interstitial pneumonia with autoimmune features (3%), and not clearly different from systemic sclerosis (13%) or lupus (11%). Five-year survival was 94% overall but 88.3% in those with pulmonary hypertension, which was independently associated with death (HR 2.52, 95% CI 1.41-4.53) after adjustment for age, sex and interstitial lung disease.
Screening for pulmonary hypertension is routine in systemic sclerosis but not in anti-synthetase syndrome, where attention usually falls on the lungs' parenchyma. These data suggest the pulmonary vasculature deserves the same attention, particularly when breathlessness is out of step with the lung disease.
- Consider echocardiography in anti-synthetase syndrome, especially with unexplained breathlessness
- Suspect pulmonary hypertension when DLCO falls out of proportion to lung volumes
- Check NT-proBNP as part of review where pulmonary hypertension is possible
- Refer suspected cases for right heart catheterisation to confirm
Why it matters
A complication screened for in scleroderma is being missed in another disease where it is nearly as common.
Don't overread it
Registry data without uniform diagnostic criteria for pulmonary hypertension.
The statistics, in plain English
An HR of 2.52 means about two and a half times the risk of death, with an interval (1.41-4.53) well above 1. Registry diagnoses were physician-reported and not all confirmed by catheterisation, so prevalence may be over- or under-estimated.
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