Despite the statistical limitations imposed by the small sample-size, our findings highlight the negative impact of psychiatric comorbidity on health and psychosocial function.

Clinically-relevant OC syndromes start early and are associated with substantial distress, treatment-seeking activity and in the case of OCD, functional disability.

Our findings suggest a lack of diagnostic stability over the long-term and a high chance of eventual remission, albeit often after several years of illness, for obsessive-compulsive syndromes including OCD. However, roughly one-third of OCD cases do not remit by 50 years of age.

Health-related quality of life (HRQoL) scores assess symptom burden in pulmonary arterial hypertension (PAH) but data regarding their role in prognostication and risk stratification are limited. We assessed these relationships using the emPHasis-10 HRQoL measure.1745 patients with idiopathic or connective tissue disease-associated PAH who had completed emPHasis-10 questionnaires between 2014–17 at 6 UK referral centres were identified. Correlations with exercise capacity and WHO functional class (FC) were assessed, and exploratory risk stratification thresholds were tested.Moderate correlations were seen between emPHasis-10 scores and 6-minute walk distance (r=−0.546), incremental shuttle walking distance (r=−0.504) and WHO FC (r=0.497; p all <0.0001). Distribution of emPHasis-10 differed significantly between each WHO FC (p all <0.0001). At multivariate analysis, emPHasis-10, but not WHO FC, was an independent predictor of mortality. In a risk stratification approach, scores of 0–16, 17–33 and 34–50 identified incident patients with one-year mortality of 5%, 10% and 23%, respectively. Survival of patients in WHO FC III could be further stratified using an emPHasis-10 score ≥34 (p<0.01). At follow-up, patients with improved emPHasis-10 had improved exercise capacity (p<0.0001), and patients who transitioned risk groups demonstrated similar survival to patients originally in those risk groups.The emPHasis-10 score is an independent prognostic marker in patients with idiopathic or connective tissue disease-associated PAH. It has utility in risk stratification in addition to currently used parameters. Improvement in emPHasis-10 score is associated with improved exercise capacity.

Please cite this article as: Lewis RA, Armstrong I, Bergbaum C, et al. EmPHasis-10 healthrelated quality of life score predicts outcomes in patients with idiopathic and connective tissue disease-associated pulmonary arterial hypertension: results from a UK multi-centre study. Eur Respir J 2020; in press (

Pulmonary veno-occlusive disease (PVOD) is classified into idiopathic PVOD, heritable PVOD, and PVOD associated with other conditions: most often connective tissue disease (CTD) and sarcoidosis. In patients with PVOD and a CTD, immunosuppressive therapies can improve symptoms, gas transfer, imaging and haemodynamics [1,2]. Despite evidence for inflammation in PVOD pathophysiology [3,4], including capillary immune complex and complement deposition [5], the role for immunosuppression in idiopathic or heritable forms of PVOD is uncertain. We present three patients with heritable and idiopathic PVOD who received immunosuppression for suspected immune dysregulation.

No abstract