Chest
Clinical InvestigationsPatterns of Pulmonary Involvement in Systemic Amyloidosis
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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Cited by (123)
The spectrum of pulmonary amyloidosis
2023, Respiratory MedicinePulmonary amyloidosis as the presenting finding in a patient with multiple myeloma
2022, Respiratory Medicine Case ReportsCitation Excerpt :Although pulmonary involvement in systemic amyloidoses is relatively common, progressive subacute dyspnea and hypoxemia, as seen in this case, are rare as the primary presenting symptoms. Often, the diagnosis of pulmonary amyloidosis is made at autopsy in patients who otherwise did not exhibit respiratory symptoms [5]. The uniquely severe clinical presentation of this patient is likely explained by the unusual diffuse tricompartmental nature of amyloid involvement.
Emphysematous Lung Lesions Caused by Perivascular and Alveolar–Septal Deposition of Amyloid Light-Chain Amyloidosis
2021, ChestCitation Excerpt :Although cystic lesions have been described with AL amyloidosis involving the lung parenchyma, we are only aware of one previous report of emphysematous findings with this disorder.8 Although factors that influence the pattern of pulmonary amyloid deposition remain unclear, CT image findings reflect the histopathologic patterns of deposition.4 For example, histopathologic amyloidomas correlate with parenchymal nodules, whereas interstitial deposition correlates with reticulonodular opacities, interlobular septal thickening, and honeycombing.6,7
Pulmonary amyloidosis mimicking interstitial lung disease and malignancy - A case series with a review of a pulmonary patterns
2021, Respiratory Medicine Case ReportsCitation Excerpt :CT imaging shows bronchial wall thickening, associated with calcifications, stenosis of bronchi lumen with mural nodulation, which can lead to athelectasis and post-obstructive air trapping. The differential diagnosis vary from calcifying neoplasm to granulomatous infectious (tuberculosis, histoplasmosis) and noninfectious (granulomatosis with polyangiitis, sarcoidosis, inflammatory bowel disease) diseases [6,13]. Our female patient (Case 1.)
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.