© 2008 American Thoracic Society
An Algorithm to Tackle Acute Exacerbations in Idiopathic Pulmonary FibrosisTo the Editor:The pulmonary perspective by Dr. Collard and colleagues (1) provides an overview of the current understanding about acute exacerbations in idiopathic pulmonary fibrosis (IPF). To tackle this challenging problem, we developed an algorithm (Figure 1). As a first step, we test for the presence of D-dimer and clinical probability of pulmonary embolism. If the test is positive, a specific computed tomography (CT) scan is performed. The CT scan is an accurate modality (2) with which to detect pulmonary embolism; moreover, images can be compared with previous CT scans to evaluate reticular shadowing, honeycombing, and ground-glass appearances. Echocardiography is performed to rule out left heart failure. At the same time, (increasing) pulmonary hypertension can be ruled out. If diffusion capacity is greater than 30%, and if hypoxemia can be corrected to a PO2 of 75 mm Hg with supplemental oxygen (3), then bronchoalveolar lavage (BAL) is performed to rule out infection (bacterial, viral, opportunistic infections, and fungi).
BAL must be performed soon after admission, and broad-spectrum antibiotics are started immediately after bronchoscopy. We usually start broad-spectrum antibiotics (such as piperacillin-tazobactam or third-generation cephalosporins intravenously). However, atypical bacilli (such as Legionella and Mycoplasma) must be covered by adding quinolones. As most of these patients with IPF are immunocompromised, Pneumocystis jiroveci pneumonia needs to be covered empirically with sulphametoxazole. If infection cannot be proven, corticosteroids are added to the treatment in a dose of 500–1,000 mg for 3 consecutive days. Antiviral agents should be considered when herpesvirus or cytomegalovirus (CMV) is found in BAL, and antifungal agents should be initiated especially when Aspergillus is found in an immunocompromised patient or if the Aspergillus antigen test in BAL is clearly positive (4). If there is no effect from these therapies, and in the absence of generalized infection, lung transplantation should be considered as a treatment for acute exacerbation in IPF. It is the only treatment that improves survival in patients with interstitial lung disease (5). The patient can only be put on the high-urgency list when a transplant workup has been completed before the acute exacerbation. This is another strong reason to refer patients with IPF in an early stage to a transplant center for transplant evaluation, as an acute exacerbation is not related to pulmonary function tests (1). In conclusion, Collard and coworkers' article gives an excellent overview of the challenges in acute exacerbations of IPF. We need carefully chosen protocols to guide us in this challenge. We hope that the algorithm we propose will help physicians in dealing with this problem.
University Hospital Gasthuisberg FOOTNOTES
Conflict of Interest Statement: W.A.W. has attended an expert meeting organized by Actelion and received REFERENCES
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1,000 as a consultancy fee. M.T. is a member of Intermune's steering committee (INSPIRE Study). L.J.D. has no financial relationship with a commercial entity that has an interest in the subject of this manuscript. G.M.V. has no financial relationship with a commercial entity that has an interest in the subject of this manuscript.