Chest
Clinical InvestigationsAcute Exacerbation in Idiopathic Pulmonary Fibrosis: Analysis of Clinical and Pathologic Findings in Three Cases
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)
- et al.
Diagnosis of nosocomial bacterial pneumonia in intubated patients undergoing ventilation: comparison of the usefulness of bronchoalveolar lavage and the protected specimen brush
Am J Med
(1988) - et al.
Hamman-Rich syndrome revisited
Mayo Clin Proc
(1990) - et al.
Fibroproliferative phase of ARDS: clinical findings and effects of corticosteroids
Chest
(1991) - et al.
7S collagen in BAL fluid of patients with adult respiratory distress syndrome
Chest
(1992) Pathogenesis of “fibrosis” in interstitial pneumonia
Hum Pathol
(1985)- et al.
Epithelial necrosis and alveolar collapse in the pathogenesis of usual interstitial pneumonia
Chest
(1988) Idiopathic pulmonary fibrosis: clinical, histologic, radiographic, scintigraphic, cytologic and biochemical aspects
Ann Intern Med
(1976)- et al.
Natural history and treated course of usual and desquamative interstitial pneumonia
N Engl J Med
(1978) - et al.
Cryptogenic fibrosing alveolitis: clinical features and their influence on survival
Thorax
(1980) Interstitial diseases
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Manuscript received June 30; revision accepted October 5.