Pediatric emergency medicine trisk 2950 2950

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