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Serial pulmonary function in systemic sclerosis

P D Schneider, R A Wise, M C Hochberg

The American Journal of Medicine

|September 1, 1982

Summary

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Systemic sclerosis patients show a rapid decline in vital lung capacity, more than three times the normal rate. Diffusing capacity remained stable, indicating a progressive restrictive lung defect over time.

Area of Science:

Pulmonary Medicine
Rheumatology
Systemic Autoimmune Diseases

Background:

Pulmonary involvement in systemic sclerosis (SSc) is a significant complication.
The natural history and progression of lung function decline in SSc are not well-characterized.

Purpose of the Study:

To longitudinally assess the changes in pulmonary function over time in a cohort of patients with systemic sclerosis.
To quantify the rate of decline in vital capacity and diffusing capacity in SSc patients.

Main Methods:

Serial pulmonary function tests were conducted on a cohort of patients with systemic sclerosis.
Data were analyzed to determine the mean annual rate of change in vital capacity and diffusing capacity.
Comparison was made to age- and sex-matched normal reference populations.

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Main Results:

Patients with systemic sclerosis experienced a mean vital capacity loss of 0.10 liters/year, over three times the expected rate.
The mean rate of loss for diffusing capacity was similar to that of a normal population.
An increase in the percent forced vital capacity expired in the first second (0.53%/year) indicated a progressive restrictive ventilatory defect.
Smokers showed a trend towards a greater vital capacity loss (p=0.069).

Conclusions:

Systemic sclerosis is associated with a progressive restrictive ventilatory defect, primarily characterized by accelerated vital capacity loss.
While diffusing capacity may remain relatively stable, the decline in vital capacity highlights the significant pulmonary impact of SSc.
Individual variability exists, but the overall trend suggests a slow progression of lung impairment in SSc patients.