Background Pulmonary arterial hypertension (PAH) is a complication of Systemic Sclerosis (SSc). It is the lead cause of mortality and can affect up to 20% of SSc patients. The DETECT study developed an evidence-based screening algorithm to aid early detection and diagnosis; as well as guide management of those with SSc-PAH. Many studies, including DETECT have shown earlier detection, higher survival rates and improved outcomes in those enrolled to a screening programme. PAH is often diagnosed late, when symptoms are advanced and prognosis is poor. Therefore, screening is crucial to enhance patient safety and improve outcomes. In light of this; a retrospective audit of 29 SSc patients at a DGH in July 2015 was performed to compare adherence rates to the national BSR standards of annual pulmonary function testing (PFT) echocardiogram and DETECT criteria for PAH-screening.
Objectives 1. Evaluation of current practice against the BSR standards.
2. Evaluation of current practice against DETECT criteria
Methods Data was retrospectively collated from written medical notes, Medway and CVIS in accordance with a Microsoft Excel proforma of 30 variables, including annual echocardiography and PFT; and eight PAH-predictive variables identified by the DETECT study (FVC %predicted/DCLO %predicted, Telangiectasias, ECG Right Axis Deviation (RAD), Serum Urate, Serum Anti-Centromere Antibody (ACA) and Serum NTproBNP).
Results Adherence to the BSR standard was 75.9% over the last 18 months. Stratification showed higher rates of adherence in those with more recent diagnoses; 70%, 83.3% and 100% for 0–4years, 5–8 years and 9–12 years disease duration respectively, compared to 50% and 0% for 17–20 years and 21–24 years respectively.
DETECT scores were calculable for 2/29 patients. Serum NTproBNP, serum urate and ECG RAD are not routinely tested for or documented in SSc patients. Furthermore, in this audit, less than 50% of patients had a documented serum ACA in their notes. As a result, DETECT scores were not calculable for most patients.
Conclusions Adherence rates in the last 18 months are 75.9%. Adherence is highest in those with more recent diagnoses. Seven patients (24%) did not receive annual screening in the past 18 months. Adherence to BSR standards and patient safety could therefore be improved. As a result of this audit, a centralised patient list was uploaded to CVIS for automatic annual screening appointments.
The use of DETECT criteria is not currently applicable. However small, inexpensive changes in routine management of SSc patients would make this possibile.
Coghlan JG, Denton CP, Grunig E, Bonderman D, Distler O, Khanna D, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73(7):1340–9.
Humbert M, Yaici A, de Groote P, Montani D, Sitbon O, Launay D, et al. Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum. 2011;63(11):3522–30.
Acknowledgement Dr Lyn Williamson, Miss Colleen Wells, Mr Simon Brader, Mr Paul Charlton
Disclosure of Interest None declared