SESSION TYPE: Pleural Cases I
PRESENTED ON: Tuesday, October 23, 2012 at 11:15 AM - 12:30 PM
INTRODUCTION: Pulmonary amyloidosis is commonly seen in primary systemic (AL) amyloidosis and presents as nodular opacities, septal thickening or less commonly persistent pleural effusions. This is a case of amyloidosis that presented as calcified pleural plaques mimicking asbestos related pleuropulmonary disease.
CASE PRESENTATION: A 76 year old male nephrologist, ex-smoker with history of COPD, OSA on CPAP and hypothyroidism presented to the emergency department complaining of acute onset of shortness of breath that began just prior to arrival. The patient was found to be in moderate distress, afebrile, hypotensive, hypoxemic, tachypneic, and bradycardic. EKG showed a junctional rhythm at 35 beats per minute. An intravenous pacemaker was placed. Laboratory data was significant for mild leukocytosis, mild troponin elevation and normal thyroid function testing. Chest roentgenogram showed bilateral airspace disease, pleural thickening and a small right pleural effusion. CT chest showed interstitial septal thickening, new bilateral pleural effusions, dependent areas of dense ground glass and increase in size of calcified pleural plaques as compared to previous CT scan 9 performed months earlier. Broad spectrum antibiotics were continued and a permanent pacemaker was placed. Official echocardiogram showed a normal LV ejection fraction, but was suggestive of cardiac amyloidosis. A fat pad biopsy was planned. The patient initially improved, but over the course of the next few days developed acute delirium. On hospital day 6 the patient was found unresponsive. ACLS was performed unsuccessfully and the patient expired. The histopathology of the cardiac muscle, lung parenchyma and of the pleural plaques obtained at autopsy showed diffuse eosinophilic infiltration that positively stained with congo red. These findings are consistent with cardiac and pleural and pulmonary parenchymal amyloidosis.
DISCUSSION: This case represents an extremely rare finding of a patient with pleuropulmonary amyloidosis and a radiographic presentation consistent with asbestos exposure. This patient was a nephrologist who was known to have calcified pleural plaques that were unexplained as the patient had no known asbestos exposure. When amyloid is associated with the pleura it has been reported in association with persistent pleural effusions often in the setting of cardiac amyloidosis resulting in heart failure. The largest series that examined pleural disease did so in the setting of AL amyloidosis. In this series 5% of the patients were found to have pleural effusions. Microscopic examination showed amyloid deposition in the pleura, but no calcified pleural plaques were reported.
CONCLUSIONS: This case identifies amyloidosis as a possible etiology for pleural plaques in a patient without asbestos exposure.
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DISCLOSURE: The following authors have nothing to disclose: Joseph Gorga, Guy Aristide, Michael Bergman
No Product/Research Disclosure InformationSUNY Downstate Medical Center, Brooklyn, NY