This conference abstract on A local audit of pulmonary hypertension screening in systemic sclerosis in the north western region, written by Tang E.Y.; Knight S.
Citation: Rheumatology (United Kingdom), April 2014, vol./is. 53/(i118), 1462-0324 (April 2014)
Abstract: Background: Scleroderma, both limited and diffuse, can be associated with pulmonary hypertension. This can be life threatening. Licensed treatments are now available. In the North Western region, this rare condition is diagnosed and managed in conjunction with the Sheffield Pulmonary Hypertension Unit. This unit has developed national guidelines for screening for pulmonary hypertension in this patient group. This retrospective audit was to assess how well we comply with the guidelines and whether steps need to be taken to improve compliance.
Methods: Patients identified from Medisave programme by searching scleroderma, SSc, CREST. Proforma was designed based on the Sheffield guidelines.
Both e-clinic letters and clinic notes were reviewed. Exclusion criteria
included patients who were undergoing investigations or who had unconfirmed diagnosis.
Results: 25 patients were identified. Three patients were excluded due to unconfirmed
diagnosis. 19 patients had limited disease and 3 patients had diffuse disease. Patients
ranged from 32 to 81 years. Approximately two-thirds of patients had annual
echocardiogram. Three-quarter of patients had annual diffusing capacity (DLCO)
monitored. For those who did not have annual echocardiogram and DLCO monitoring,
additional of 12-month window period was given in case of waiting list or appointment
cancellation due to unforeseen circumstances. None of the patients had follow-up
echocardiogram or DLCO within this 12-month period. As we are aware, systolic
pulmonary artery pressure directly correlates with the development of pulmonary
hypertension. 15 patients had low probability of pulmonary hypertension development, 4
had intermediate probability and 3 had high probability. In the guideline, it is
recommended that for patients with intermediate probability of pulmonary hypertension
development, specialists should be referred if DLCO <50% or if DLCO >50% with
symptom of breathlessness. All patients with high probability should be referred to
specialists. This audit demonstrated that all patients with intermediate probability had
DLCO >50% and none of them were symptomatic. Three patients had high probability
and all were referred to Sheffield. 2 out 3 patients were diagnosed with pulmonary
hypertension following review by the specialist, which represented 10% of the patients
audited. All patients attended annual clinic review but not all had annual echocardiogram
and annual DLCO arranged. However, for those who have had annual investigations,
there was 100% adherence to the Sheffield guideline. Conclusion: SSc patients are at risk
of developing pulmonary hypertension, although rare, it is life-threatening if left
untreated. All rheumatology units should be made aware of the national guidelines of
pulmonary hypertension screening in SSc. Annual clinic review checklist for patients
with SSc should be created to ensure guidelines adherence. Following this audit,
departmental and regional education was carried out to increase awareness of the
guidelines. Re-audit in 12 months has been planned.
Publication Type: Journal: Conference Abstract