Chest
Volume 110, Issue 6, December 1996, Pages 1520-1525
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Clinical Investigations: Xercise/Cleroderma
Cardiorespiratory Responses to Incremental Exercise in Patients With Systemic Sclerosis

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Patients with systemic sclerosis are known to have histologic pulmonary abnormalities despite normal chest radiograph or conventional pulmonary function or both. In an attempt to detect early features of lung involvement in progressive systemic sclerosis, we investigated patients with systemic sclerosis using cardiopulmonary exercise testing. We have studied 78 patients who fulfilled the American Rheumatism Association criteria for the classification of systemic sclerosis, and according to the classification of LeRoy, 44 had limited cutaneous systemic sclerosis and 34 had diffuse cutaneous systemic sclerosis. A significantly decreased diffusing capacity (65±3% of that predicted) was present only in the group with diffuse cutaneous systemic sclerosis. The patients with lung involvement showed a significant reduction in exercise capacity (54±3% of that predicted) and in oxygen uptake (70±3% of that predicted). Additionally, we could demonstrate an increased functional dead space ventilation (0.34±0.02) and widened alveolar-arterial oxygen difference during exercise (44±3 mm Hg). By cardiopulmonary exercise testing, 12 of the 78 patients (15%) with normal single-breath diffusing capacity for carbon monoxide had increased dead space to tidal volume ratio. Our results suggest that occult pulmonary impairment may be present in patients with normal pulmonary function and that cardiopulmonary exercise testing enables detection of such impairment. Our study results show the limitations of resting data in predicting abnormalities during exercise in patients with systemic sclerosis.

Section snippets

Study Population

Seventy-eight consecutive patients were referred to our department from the Department of Dermatology in the years 1993 and 1994 to evaluate lung involvement. All patients fulfilled the American Rheumatism Association criteria for the classification of systemic sclerosis, with or without clinical evidence of pulmonary involvement.15 There were 53 women and 25 men; the mean age was 51 years (range, 20 to 79 years) (Table 1). Twenty-one percent of the patients showed the characteristic

Pulmonary Function Tests

Pulmonary function test results and arterial blood gas values are shown in Table 2. The study group did not show a significant reduction in lung volumes or an increased bronchial obstruction. The Dco had a wide range from 38 to 132% of predicted.

When patients with ISSc and with dSSc were compared, significant differences in FVC, RV, TLC, and Dco could be demonstrated (Table 1). MEF50 and FEV1/FVC were not significantly different in the two groups. Significant differences measured in arterial

Discussion

The present study was designed to evaluate the usefulness of cardiopulmonary exercise testing in estimating disease severity and to detect possible transition from the limited to the diffuse form of systemic sclerosis. Comparing limited with diffuse systemic sclerosis, those patients with lung involvement were characterized by a significantly lower Dco, whereas an evident airways obstruction was not present in the two groups. In contrast to the study by Pistelli,20 we did not find patients

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