The American College of Rheumatology provided guidelines for detection of pulmonary hypertension (PH) in connective tissue diseases, including progressive systemic sclerosis (PSS).¹ The key recommendation stated that all patients with PSS should be screened for PH with pulmonary function tests (PFTs), including single-breath diffusing capacity for carbon monoxide; transthoracic echocardiogram; and measurement of N-terminal pro-brain natriuretic peptide (NT-proBNP), performed annually. 764 unique patients with PSS were seen between 2016-2020. The percentage of patients who had PFTs in the year of the clinic visit plus the previous year ranged from 61.2% to 70.8% between 2016 and 2020. The percentage of patients who had echocardiograms in the year of the clinic visit plus the previous year ranged from 71.0% to 76.7% between 2016 and 2020. A chart review of 20 patients from 2019 who did not obtain a PFT in the last 2-years showed that 10 patients (50%) had PFTs performed outside Cleveland Clinic. A chart review of 20 patients who did not obtain an echocardiogram in the previous 2-years showed that 11 patients echocardiograms performed outside Cleveland Clinic (55%).
¹Khanna D, Gladue H, Channick R, Chung L, Distler O, Furst DE, Hachulla E, Humbert M, Langleben D, Mathai SC, Saggar R, Visovatti S, Altorok N, Townsend W, FitzGerald J, McLaughlin VV; Scleroderma Foundation and Pulmonary Hypertension Association. Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension. Arthritis Rheum. 2013 Dec;65(12):3194-3201.