Management of scleroderma in a New Zealand tertiary rheumatology centre: emphasis on pulmonary complications

To determine the current local practice of managing scleroderma (SSc) patients, in particular screening of SSc-related lung disease in a tertiary rheumatology centre.

SSc patients were identified from our inpatient (July 1999 till June 2004) and outpatient (January 2002 till June 2004) databases. Patient demographics and relevant investigations performed to monitor for pulmonary, renal, and cardiac complications related to SSc were sought from computerised clinical and laboratory records.

Nine of the 39 (23%) limited SSc (lcSSc) patients and 5 of the 10 (50%) diffuse SSc (dcSSc) patients had lung involvement. A higher proportion of diffuse SSc patients had investigations for SSc lung disease compared to the lcSSc group (90% vs 67% had pulmonary function tests and 70% vs 56% had high resolution chest CT scans respectively). About half of the patients in both groups had echocardiographs (50% lcSSc vs 46% dcSSc) for assessment of pulmonary arterial hypertension.

SSc lung disease has been generally poorly screened in our cohort of patients with SSc. Limited SSc patients were not screened as rigorously as dcSSc patients for SSc lung disease. In large part, this was because of the lack of availability of treatments in New Zealand when the lung disease was identified. The generation of a standardised screening and monitoring protocol may help identify patients with progressive lung disease so that early treatment could be considered as this becomes more readily available.