Pulmonary hypertension associated with antisynthetase syndrome: insights from the ASPIRE registry

Objectives To assess the characteristics and outcomes in patients with Pulmonary Hypertension (PH) associated with Antisynthetase syndrome (ASyS). Methods Data for 88 ASyS patients assessed between 2000 and 2022 were obtained from the ASPIRE (Assessing the Spectrum of PH identified at a Referral centre) registry. Patients with pre-capillary PH were matched for age, sex and severity of PH with systemic sclerosis controls on a 1:1 basis. Results 64 patients had pre-capillary PH, 9 PH associated with left heart disease and 15 had no/unclassified-PH. Jo-1 was the most common antibody and was present more frequently in pre-capillary PH. Most patients had interstitial lung disease; nonspecific interstitial pneumonia was most common. Jo-1 antibodies were associated with an increased risk of PH and of more severe lung disease. Patients with pre-capillary PH had significantly worse spirometry, diffusing capacity of the lungs for carbon monoxide (DLco) and a trend towards more extensive interstitial lung disease than patients with no/unclassified-PH. In patients with ASyS-PH, higher age and increased pulmonary vascular resistance were independent predictors of mortality. 10-year survival was significantly worse in patients with pre-capillary PH than no/unclassified-PH. Despite less favourable spirometry, 10-year survival was significantly better in ASyS-PH than in systemic sclerosis-associated PH. Conclusion Most patients with ASyS-PH have parenchymal lung disease. Age and pulmonary vascular resistance are important prognostic predictors. Survival in ASyS-PH is superior to that of systemic sclerosis-associated pre-capillary PH.