Clinical study
Type and distribution of pulmonary parenchymal and vascular amyloid: Correlation with cardiac amyloidosis

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Abstract

The spectrum of amyloidosis was studied in 223 patients examined at autopsy at The Johns Hopkins Hospital since 1889. Of these patients, pulmonary involvement with amyloid was found in 68 patients including 31 with senile cardiac amyloidosis, 23 with primary amyloidosis, eight with myeloma associated amyloidosis, two with familial amyloidosis with polyneuropathy, three with isolated nodular pulmonary parenchymal amyloidosis and only one patient with secondary amyloidosis. The degree of pulmonary involvement ranged from either focal parenchymal or vascular amyloid to severe diffuse parenchymal and vascular amyloid. In general, cardiac amyloid involvement tended to parallel the pulmonary involvement and usually was more severe. Correlations between pulmonary amyloidosis and cardiac amyloidosis were statistically significant. Patients with primary amyloidosis, senile cardiac amyloidosis, myeloma associated amyloidosis, nodular pulmonary amyloidosis and familial amyloidosis with polyneuropathy had potassium permanganate-resistant amyloid. These findings suggest that the amyloid in these types of amyloidosis, in which pulmonary involvement is frequent, is a protein of either immunoglobulin origin or of a similar structure.

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      Although systemic ATTR primarily involves heart and peripheral nerve tissue, many other tissue types are affected including the pulmonary parenchyma and vasculature [7]. Multiple studies have described the spectrum of pulmonary manifestations in patients with amyloid light chain (AL) amyloidosis, but very little is known about lung involvement in ATTR outside of individual case reports and autopsies, and the antemortem diagnosis of pulmonary ATTR is increasing with more widespread use of chest imaging and lung biopsy [8–11]. The aim of this retrospective single-center study was to investigate the clinical, pathologic, and radiologic manifestations of pulmonary ATTR.

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    This study was supported by Grants HL-14153 and P50-HL-17655-04 from The National Institutes of Health, Public Health Service, Department of Health, Education and Welfare.

    1

    From the Departments of Pathology, Oncology and Medicine, The Johns Hopkins Medical Institutions, Baltimore, Maryland.

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