Chest
Volume 103, Issue 6, June 1993, Pages 1808-1812
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Clinical Investigations
Acute Exacerbation in Idiopathic Pulmonary Fibrosis: Analysis of Clinical and Pathologic Findings in Three Cases

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We treated three patients with idiopathic pulmonary fibrosis who had an acute clinical exacerbation. We analyzed their clinical, radiographic, therapeutic, and pathologic findings. Their initial symptoms were influenza-like illness or cough with fever, and all had leukocytosis and elevation of C-reactive protein. Infectious events were ruled out by extensive bacteriologic and serologic examination. The patients' lung injury scores progressed rapidly to severe lung injury compatible with adult respiratory distress syndrome. Findings from bronchoalveolar lavage fluid showed marked neutrophilia and elevation of albumin concentrations. All patients showed various degrees of improvement following corticosteroid therapy. Histologic findings from open lung biopsy specimens showed both usual interstitial pneumonia (UIP) and organizing acute lung injury pattern. Whether these two forms of interstitial pneumonia (UIP and acute lung injury pattern) are variants of one disease or are unrelated and also the effectiveness of corticosteroid therapy on such conditions remain to be determined by further studies.

(Chest 1993; 103:1808-12)

Section snippets

Subjects

The subjects were three patients with chronic interstitial pneumonia. Their clinical features before the acute clinical exacerbation were as follows: They all had an insidious onset of breathlessness with exercise and a nonproductive cough. Their physical examinations revealed inspiratory fine crackles (Velcro rales) at the lung bases. Clubbing of the digits was found in two of the patients. Their previous chest x-ray films taken at the time of the initial symptoms (2 years, 18 months, and 6

Clinical Course and Data on Acute Exacerbation

A summary of the clinical data, including course, is shown in Table 1. Patients' (all male) ages ranged from 55 to 68 years (mean, 61 years). Initial symptoms of acute exacerbation began with influenza-like illness or cough with fever. Within a few weeks (except for patient 3, whose symptoms continued for 20 days and whose condition deteriorated rapidly during admission), the patients' conditions worsened, and their lung injury score (LIS) reached the state of SLI, the severity of which is

DISCUSSION

The clinical courses of the three patients were characterized by a rapid exacerbation of respiratory failure with newly developing diffuse pulmonary infiltrates on their chest x-ray films. Their LIS reached to the state of SLI, the severity of which is compatible with ARDS as described by Murray et al.7 Because all three patients' conditions deteriorated within a few weeks, a diagnosis of acute interstitial pneumonia (AIP) (synonymous with Hamman-Rich syndrome)9, 10 could be given; however,

ACKNOWLEDGMENT

We thank Dr. Thomas V. Colby (Division of Pathology, Mayo Clinic, Rochester, Minn) for the pathologic evaluation and informative comments. We also thank Mr. M. Bodman, language consultant of our department, for reading the previous draft and making suggestions on language and style.

REFERENCES (21)

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Manuscript received June 30; revision accepted October 5.

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