Arthritis & Rheumatism, Volume 60,
October 2009 Abstract Supplement

The 2009 ACR/ARHP Annual Scientific Meeting
Philadelphia October 16-21, 2009.

An On-Line Screening Tool for Cardiopulmonary Complications of Scleroderma - the Australian Scleroderma Screening Program

Proudman1,  Susanna M., Byron2,  Jill, Zochling3,  J., Youssef4,  Peter, Nash5,  P., Tymms6,  Kathleen, Chan7,  Madelynn

Royal Adelaide Hospital, Adelaide, Australia,
The Queen Elizabeth Hospital, Adelaide, Australia,
Monash Medical Centre, Melbourne, Australia
St Vincent's Hospital, Melbourne, Australia,
University of Tasmania, Hobart, Australia,
Royal Prince Alfred Hospital, Sydney, Australia,
University of Queensland, Brisbane, Australia,
Canberra Hospital, Canberra, Australia,
Royal Perth Hospital, Perth, Australia,
St George Hospital, Sydney, Australia,
Royal North Shore Hospital, Sydney, Australia,


It is recommended that patients with scleroderma (SSc) be screened annually for the serious complication of pulmonary arterial hypertension (PAH), as it is often clinically silent early in the disease and is now treatable. Compliance with evidence-based clinical guidelines (CG) in a real-life setting can be poor.


A nation-wide, multicentre on-line database of patients with SSc was established in 2007, with a computer decision support system (CDSS) for implementing screening CG for PAH. Doppler echocardiograms (ECHO) and pulmonary function tests (PFTs) are performed annually and recommendations for diagnostic tests for PAH including right heart catheter (RHC) are made by the CDSS after risk stratification according to systolic pulmonary artery pressure (sPAP), diffusing capacity (DLCOc) and symptoms. Definitions of risk categories are: high risk: ECHO sPAP > 50 mmHg; moderate risk: sPAP 40–50 mmHg; at risk: unexplained dyspnoea and/or DLCOc < 50% with FVC > 85 % despite sPAP < 40 mmHg. Detailed clinical and laboratory data are collected annually and entered on-line.


By April 2009, 714 patients (66% lSSc; 26% dSSc) had been recruited into the database from 12 centers and screened. Of these, 341 (45%) had had a second visit, 75 (10%), a third. Mean±SD age and disease duration at entry were 57.9±12.5 and 12.5±10.4 years respectively. Clinical characteristics included esophagitis (82% lSSc cf 84% dSSc), fecal incontinence (16% cf 12%), digital ulcers (24% cf 38%), joint contractures (21% cf 65%), pulmonary fibrosis (19% cf 39%), modified Rodnan score (9 cf 21) and renal crisis (1% cf 5%). 28 (4%) had had RHC-defined PAH prior to 2007. 47 (6.7%) of the remaining 686 patients had RHC-defined PAH as a result of screening (9 on exercise RHC). Of 33 patients at high risk, 22 were found to have PAH. Of 62 patients at moderate risk, 23 with symptoms were referred for RHC and 11 had PAH. Of 32 at risk patients, 10 were diagnosed with PAH (one with unrecordable ECHO sPAP). A further 5 patients had PAH due to left ventricular dysfunction. The most common reason for CG violation in the moderate risk group was underestimation of symptoms.


Not all patients with PAH have an abnormal ECHO and risk stratification which includes PFTs and symptoms, aids detection. This is the first time a CDSS has been used to facilitate compliance with CG for screening for PAH in SSc, allowing comprehensive clinical assessment and early institution of potentially beneficial therapy. This web-based database serves as a model for effective multi-centre collection of data for an uncommon rheumatic disease.

To cite this abstract, please use the following information:
Proudman, Susanna M., Byron, Jill, Zochling, J., Youssef, Peter, Nash, P., Tymms, Kathleen, et al; An On-Line Screening Tool for Cardiopulmonary Complications of Scleroderma - the Australian Scleroderma Screening Program [abstract]. Arthritis Rheum 2009;60 Suppl 10 :1711
DOI: 10.1002/art.26785

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