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Weekly Chest CasesArchive of Old Cases

Case No : 81 Date 1999-05-15

  • Courtesy of Jae-Woo Song, MD / Seoul City Boramae Hospital, Seoul, Korea
  • Age/Sex 66 / M
  • Chief Complaintdry cough & progressive exertional dyspnea for one month with rapid progression during last several days, previously healthy, no hystory of DM or tuberculosis, no fever, no sign of edema WBC 9600 Arterial Blood Gas Analysis: pH 7.39, PaCO2 39mmHg, PaO2 50mmHg, HCO3- 23meq/L Pulmonary Function Test: FEV1 1.41(50%), FVC 1.64(40%), DLCO 30%
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Chest PA 1 (on admission)

Diagnosis With Brief Discussion

Diagnosis
Acute Interstitial Pneumonia
Radiologic Findings
Initial chest radiograph (on admission) shows bilateral ill defined ground glass opacity with some peripheral predonimancy. One day after admission, chest radiograph shows rapid progression of bilateral air-space opacification. HRCT scans show bilateral ground glass opacity with some foci of air-space consolidation. There is no zonal predominance in distribution.
Brief Review
Acute interstitial pneumonia is a fulminant disease of unknown etiology that usually occurs in a previously healthy person and produces histologic findings of diffuse alveolar damage. There is often a prodromal illness associated with symptoms of a viral upper respiratory infection, followed by rapidly increasing dyspnea and respiratory failure. AIP was described in 1986 by Katzenstein et al. in eight patients. The patients presented with symptoms of acute respiratory failure and required mechanical ventilation within 1 to 2 weeks from onset of symptom. Seven of the eight patients died within six months, and one recovered. The pathologic abnormalities consisted of thickening of the alveolar walls due to edema, inflammatory cells, and active fibroblast proliferation but little mature collagen deposition. There was extensive alveolar damage with hyaline membrane formation. They emphasized that this condition, which they called acute interstitial pneumonia, is clinically and pathologically distinct from UIP and DIP, which accounts for the majority of cases of idiopathic pulmonary fibrosis. because the acute presentation and the histologic features are identical with those of ARDS, AIP has also been referred to as idiopathic ARDS. Primack et al. reviewed the radiographic and HRCT findings in nine patients with acute interstitial pneumonia. Bilateral, symmetric areas of ground glass opacity were present on HRCT in all nine patients. The areas of groundless opacity involved all lung zones to a similar extent in 7 patients and had an upper lung zone predominance in the other 2 patients. In six patients, the areas of groundless opacity had a patchy distribution with focal areas of sparing, giving a geographic appearance, and 3 patients had diffuse involvement. In none of the cases did the areas of groundless opacity involve mainly the central or subpleural regions. Bilateral areas of air-space consolidation were identified on HRCT in 6 of nine cases. The consolidation had a predominantly basilar distribution in 3 patients, a diffuse distribution in 2, and an upper lung zone predominance in one. Subpleural honeycombing was also seen at HRCT in 3 patients. The areas of honeycombing involved less than 10% of the lung parenchyma.
References
1. Katzenstein ALA, Myers JL, Mazur MT. Acute interstitial pneumonia: a clinicopathologic, ultrastructural, and cell kinetic study. Am J Surg Pathol 1986;10:256-257
2. Primack SL, Hartman TE, Ikezoe J, et al. acute interstitial pneumonia: radiographic and CT findings in nine patients. Radiology 1993;188:817-820
Keywords
Lung, Idiopathic interstitial pneumonia, AIP, IIP,

No. of Applicants : 23

▶ Correct Answer : 18/23,  78.3%
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▶ Semi-Correct Answer : 2/23,  8.7%
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