Monitoring
Monitoring of the clinical course in patients is recommended every 3 to 6 months for those with early disease and less frequently (6-12 months) for those with longstanding stable disease. Yearly pulmonary function tests (spirometry, lung volumes, and diffusing capacity measurement) and echocardiograms are performed to evaluate for pulmonary artery hypertension and interstitial lung disease. Level of evidence for these recommendations is expert opinion, although a review published in 2007 has proposed similar guidelines.[71]
Other testing is performed as necessary, and may be specific to the individual treatments being used. LFTs, CBC, and kidney function require monitoring while on immunomodulatory agents. WBC count should be monitored 2 weeks postinfusion of cyclophosphamide, and urinalysis is performed during cyclophosphamide therapy to monitor for hemorrhagic cystitis.
Prednisone should be avoided at doses higher than 10 mg/day due to increased risk for precipitating renal crisis. If this therapy must be given, it should be tapered as rapidly as possible, and BP requires close monitoring.
When vasodilators are used for the treatment of Raynaud phenomenon or digital ulceration, it is necessary to monitor BP for hypotension. LFTs must be monitored frequently throughout treatment with some endothelin-1 receptor antagonists (e.g., bosentan). Continuous telemetry monitoring is necessary during the titration of an intravenous infusion of epoprostenol for Raynaud/digital ulcers.
If metoclopramide or another promotility agent is given for severe symptoms due to gastroparesis, it should be used with extreme caution due to the risk of tardive dyskinesia. Its use should be limited to as brief a period of time as necessary
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