Korean Society of Thoracic Radiology
The Korean Radiological Society
Findings
Initial chest radiograph shows bilateral pulmonary
parenchymal infiltrates, primarily in both lower medial lung fields. Follow-up
chest radiograph after two weeks shows more aggravation of bilateral pulmonary
parenchymal infiltrates. HRCT scans after bronchoscopic biopsy show bilateral
geographic distribution of ground-glass opacity with smooth thickening
of inter- and intralobular septa. There is no cavitation, pleural effusion,
or lymphadenopathy.
Discussion
Pulmonary alveolar proteinosis(PAP) is a rare disease
that consists of the alveolar filling with eosinophilic PAS-positive proteinaceous
material, which is mostly phospholipid and protein constituents of both
surfactant and degenerated cell membranes. PAP is a non-specific tissue
response to a varity of injuries to alveolar macrophages, type„± pneumocytes,
or both. It is associated with altered immunity and exposure to dust and/or
fumes. The pathogenesis of this disease is poorly understood, and the majority
of cases are considered to be idiopathic. But proposed pathogenetic mechanism
include unbalanced turnover and defective production or clearance of surfactant.
The alveolar septa are usually normal or only slightly infiltrated, and
interstitial fibrosis is unusual. Insidious onset of nonproductive
or mild productive cough and mild dyspnea are the most common symptoms,
but hemoptysis, intermittent lower grade fever, pleuritic chest pain, weight
loss, clubbing and cyanosis have been associated in a few patients. Pulmonary
function test results typically show restrictive dysfunction with decreased
diffusing capacity. Opportunistic superinfection, particularly with Nocardia,
and pulmonary fibrosis are possible complications.
The usual radiographic manifestations are bilateral,
symmetric perihilar air-space disease in a "bat-wing distribution, simulating
severe acute pulmonary edema but with normal heart size and rare air-bronchograms.
The typical radiograph shows a bilateral, diffuse, perihilar or central,
ill defined nodular or confluent pattern, which is usually worse in the
base, but patchy disease and peripheral predominance can occur. CT can
be useful in differential diagnosis and detection of focal pneumonia. The
patterns of PAP on CT are not pathognomic, but common findings include
bilateral ground-glass opacity, smooth interlobular septal thickening in
abnormal areas, consolidation, and patchy or geographic distribution.
The diagnosis of PAP is made by biopsy, but detection of surfactant
apoprotein A in serum could allow less invasive diagnosis.
Bronchoalveolar lavage to remove the alveolar material
is an effective therapy for patients with symptomatic PAP. The radiographic
appearance after lavage have a characteristic pattern showing gradual but
incomplete clearing of the washed lung within hours to weeks of successful
treatment. Resolution is often spotty and asymmetric, and one area may
improve as another worsens. Although in some patients the disease will
resolve spontaneously or after a single lavage, most patients require chronic
treatment for months or years.
References
1. Godwin JE, Muller NL, Takasugi JE. Pulmonary alveolar proteinosis:
CT findings. Radiology 1988; 169:609-613
2. M. Elon Gale, Joel B. Karlinsky, Arthur G. Robins. Bronchopulmonary
lavage in pulmonary alveolar lavage in pulmonary alveolar proteinosis.
AJR 146:981-985, May 1986
3. Zimmer WE, Chew FS. Pulmonary alveolar proteinosis. AJR 1993;161:26