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Factors limiting exercise performance in progressive systemic sclerosis.

B Blom-Bülow, B Jonson, K Bauer

    Seminars in Arthritis and Rheumatism
    |November 1, 1983
    PubMed
    Summary
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    Systemic sclerosis (PSS) significantly limits physical performance, with cardiac and pulmonary issues, joint mobility, and muscle strength all contributing to reduced working capacity in patients. Arrhythmias during exercise are a key concern, impacting overall physical function.

    Area of Science:

    • Cardiology
    • Pulmonology
    • Rheumatology
    • Exercise Physiology

    Background:

    • Systemic sclerosis (PSS) is a complex autoimmune disease affecting multiple organ systems.
    • Physical performance limitations are common in PSS patients, impacting quality of life.
    • Understanding the specific organ systems that limit physical capacity is crucial for management.

    Purpose of the Study:

    • To evaluate the extent to which cardiac and pulmonary function, joint mobility, and muscular strength limit maximal working capacity in patients with PSS.
    • To develop a scoring model for these parameters.

    Main Methods:

    • Maximal working capacity was assessed in 22 PSS patients.
    • Cardiac function (heart rate, ECG, arrhythmias), pulmonary function (ventilation, blood gases), joint mobility, and muscular strength were evaluated.

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  • A scoring system was used to quantify limitations.

    • Main Results:

    • Average working capacity was 51% of predicted normal values.
    • High ventilation was observed during maximal workload despite normal arterial blood gases.
    • Cardiac abnormalities, including arrhythmias and ECG changes suggestive of myocardial fibrosis, were common.
    • No strong correlation was found between cardiac dysfunction and pulmonary fibrosis or musculoskeletal impairment.

    Conclusions:

    • Cardiac dysfunction, particularly arrhythmias during exercise, and pulmonary limitations significantly contribute to reduced physical capacity in PSS.
    • The scoring system indicated an equal contribution of circulatory, pulmonary, and locomotive functions to decreased working capacity.
    • Clinical attention should focus on exercise-induced arrhythmias in PSS patients, as they are often missed on resting ECGs and are linked to sudden death.