Orthopaedic & Rheumatologic Institute Outcomes

The American College of Rheumatology provided guidelines for detection of pulmonary hypertension (PH) in connective tissue diseases, including systemic sclerosis (SSc).¹ The key recommendation stated that all patients with SSc should be screened for PH by pulmonary function tests (PFTs) with single-breath diffusing capacity for carbon monoxide, transthoracic echocardiogram, and measurement of N-terminal pro–brain natriuretic peptide (NT-proBNP) . In patients with SSc, transthoracic echocardiogram and PFTs should be performed annually. The full screening panel (transthoracic echocardiogram, PFTs, and measurement of NT-proBNP) should be performed as soon as any new signs or symptoms are present. 862 unique patients with SSc were seen between 2018-2022. The percentage of patients who had PFTs in the year of the clinic visit plus the previous year ranged from 67.1% to 72.2% between 2018 and 2022. The percentage of patients who had echocardiograms in the year of the clinic visit plus the previous year ranged from 75.6% to 80.2% between 2018 and 2022. A chart review of 25 patients who did not obtain a yearly echo showed that 44% of patients had an echocardiogram performed outside Cleveland Clinic. A chart review of 25 patients who did not obtain yearly PFTs showed that 16% of patients had PFTs performed outside Cleveland Clinic.

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.