Progressive Systemic Sclerosis

Percentage of Patients with Progressive Systemic Sclerosis who Obtain Yearly Echocardiograms and Pulmonary Function Testing (2015-2019)

Percentage of Patients with PSS who Obtain Yearly Echocardiograms and Pulmonary Function Testing (N=798)

2015-2019

  1. Khanna 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.

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. 798 unique patients with PSS were seen between 2015-2019. The percentage of patients who had PFTs in the year of the clinic visit plus the previous year ranged from 62.7% to 70.5% between 2015 and 2019. The percentage of patients who had echocardiograms in the year of the clinic visit plus the previous year ranged from 71.9% to 76.5% between 2015 and 2019. 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%). The great majority of patients had a PFT or echocardiogram performed at least every 2-years.