Chest
Volume 74, Issue 5, November 1978, Pages 543-547
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Clinical Investigations
Patterns of Pulmonary Involvement in Systemic Amyloidosis

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The clinical and histopathologic features of pulmonary amyloidosis were reviewed in 22 patients with systemic amyloidosis who came to autopsy. Eleven of 12 patients (92 percent) with primary amyloidosis had prominent interalveolar amyloid deposits. Symptoms attributable to these deposits were found in four cases (33 percent), while severe lung involvement was the apparent cause of death in one. Extensive deposition was noted in all three cases of amyloidosis associated with multiple myeloma or Waldenstrom's macroglobulinemia. Five of seven patients (71 percent) with secondary amyloidosis showed histologic lung involvement, which was perivascular or tracheobronchial in location, but not associated with symptoms. Histologic lung involvement is frequent in all forms of amyloidosis and lung tissue obtained from any patient with unexplained interstitial or reticular-alveolar pulmonary disease should be stained with Congo-red and viewed for green birefringence under polarizing microscopy for the presence of amyloid.

Section snippets

Patients and Methods

During the period 1960–1975 a series of patients with systemic amyloidosis were referred to one of us (ASC) for consultation and evaluation and prospectively followed thereafter. The majority of these patients were referred from numerous locations in the United States and abroad while others were diagnosed in the Thorndike Clinical Research Center at Boston City Hospital or at the Boston University Medical Center. At the time of initial consultation, they demonstrated a wide spectrum of

Primary Amyloidosis

There were 12 patients (55 percent) with primary amyloidosis, seven men and five women (Table 1). Their ages ranged from 29–67 (mean 50) years. A smoking history was not obtained from any of these patients. Eleven were initially seen because of complaints unrelated to the lung. Four of the 12 complained of cough and dyspnea which could not be explained on the basis of cardiac involvement (as determined by clinical, electrocardiographic or roentgenologic criteria). One patient (case 11)

Discussion

Although conclusions concerning the overall incidence of pulmonary amyloidosis and its clinical significance based on the findings of autopsy-derived data have their limitations, the extent of amyloid deposition in our series of patients allows some inferences regarding its possible clinical importance.

The prevalence of pulmonary deposition in patients with primary systemic amyloidosis based on histologic studies ranges from 36 percent7 to 90 percent8 but clinical correlative data are lacking.

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These investigations have been supported by grants from the United States Public Health Service, National Institute of Arthritis, Metabolic and Digestive Diseases (AM-04599 and AM-07014), from the General Clinical Research Centers Branch of the Division of Research Resources, National Institutes of Health (RR-533), from the Massachusetts Chapter of the Arthritis Foundation and from the Arthritis Foundation.

Manuscript received February 7; revision accepted May 19.

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