ATS Pocket Guide 2013 - (Page 22)

MONITORING THE CLINICAL COURSE OF DISEASE „ MONITORING FOR PROGRESSIVE DISEASE1 ■ Increasing respiratory symptoms, worsening pulmonary function test results, progressive fibrosis on HRCT, or acute respiratory decline, may be manifestations of disease progression. ■ In the absence of another identifiable cause, the presence of any of the following changes is consistent with progressive disease: » Progressive dyspnea (objectively assessed) » Progressive, sustained decrease from baseline in absolute FVC » Progressive, sustained decrease from baseline in absolute DLCO (corrected for hemoglobin) » Progression of fibrosis from baseline on HRCT » Acute exacerbation » Death from respiratory failure ■ Disease progression is generally monitored over periods of 3 to 6 months, but sustained changes in symptoms, physiology, and radiology over shorter periods may also identify disease progression. ■ Pulmonary function testing provides the most standardized approach to objective monitoring and quantification of disease progression. ■ Monitoring for desaturation during 6MWT is useful to assess the need for supplemental oxygen in patients with significant exercise intolerance. ■ The presence of significant emphysema impacts FVC measurement; thus changes in FVC alone may not be as reliable an indicator of disease progression in these circumstances. ■ FVC and DLCO measurements should be performed during routine monitoring in accordance with ATS/ERS standards. ■ The optimal time interval for repetition of FVC and DLCO has not been formally investigated. ■ In the presence of progressive dyspnea or other features of a more rapidly progressive course, a flexible approach to monitoring for disease progression with a lower threshold for earlier repetition of FVC and DLCO is required. „ MONITORING FOR WORSENING SYMPTOMS ■ Patients experiencing worsening respiratory symptoms require evaluation for progressive disease, assessment of oxygenation at rest and with exertion, and prompt detection of secondary complications (e.g., development of deep venous thrombosis and pulmonary embolus). ■ Some patients may also benefit from symptom-based therapies. ■ It is unclear if any of the research tools available for the quantification of dyspnea have clinical utility. 22 Guidelines for the Diagnosis and Management of Idiopathic Pulmonary Fibrosis 

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ATS Pocket Guide 2013

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