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presenting as palpitations, syncope, or sudden death. Pericarditis is usually silent and
valvular involvement in scleroderma is rare. Even in the absence of symptoms or
physical findings, cardiac involvement eventually develops in the majority of patients
with JSSc. Management of cardiac dysfunction is symptomatic, including inotropic
support and afterload reduction. Extensive diuresis should be avoided because of
potential adverse effects on renal perfusion. No specific drugs are available to arrest the
progression of cardiac involvement.
Pulmonary Complications. Pulmonary involvement in JSSc may have three
manifestations: pleurisy, interstitial lung disease, or pulmonary artery fibrosis. Diffuse
interstitial lung disease is often asymptomatic. A dry cough may be the first symptom.
Early in the course of the disease, even before symptoms appear, pulmonary function
tests show a restrictive pattern and diffusion abnormalities. Later, radiographs of the
chest show increased reticulation, a so-called “honeycombed” appearance, mainly
basilar and bilateral. With progression of the disease, cough and dyspnea become
prominent.
Patients with irreversible pulmonary fibrosis and chronic respiratory failure have
diminished respiratory reserve, so they must be treated promptly and aggressively
when they contract intercurrent respiratory infections. Supplemental oxygen,
bronchodilators, and corticosteroids may be helpful.