Chest
Original Research: Diffuse Lung DiseaseAmyloid-associated Cystic Lung Disease
Section snippets
Study Population and Data Collection
Following approval by the Mayo Foundation Institutional Review Board in Rochester, Minnesota (10-006289), we reviewed the medical records of subjects with biopsy-proven pulmonary amyloidosis who had cystic lung disease at HRCT encountered at Mayo Clinic during a 14-year period (1997-2010). Age, sex, clinical presentation, smoking status, PFT, autoimmune serology test, HRCT, lung biopsy, and protein immunoelectrophoresis results were extracted from the medical record. Pathology reports were
Clinical and Laboratory Features
Of 187 subjects identified as having biopsy-proven pulmonary amyloidosis during the period of 1997 through 2010, 21 subjects (11.2%) manifested cystic lung disease and included 13 women (62%). The median age of the subjects was 61 years (range, 26-91). Eight subjects (38%) had a smoking history (Table 2). Fourteen received a diagnosis at surgical lung biopsy, and the remaining seven at transbronchial biopsy. None of the subjects who received a diagnosis at surgical lung biopsy had undergone
Discussion
In this study, we found amyloid-associated cystic lung disease to be characterized by multiple small to moderate-size, thin-walled, predominantly round or lobulated cysts that were either evenly distributed in the craniocaudal dimension or lower lung predominant. The presence of peribronchovascular and subpleural cysts was typical. Nodules, some of which were calcified, accompanied the cysts in most cases. To our knowledge, our study is the first to compare characteristics of cystic amyloid
Conclusions
Amyloid-associated cystic lung disease is a rare entity, usually associated with AL (lambda or kappa) amyloidosis and can occur in subjects with or without underlying CVD (especially Sjögren syndrome). Cystic lesions in the lung are usually numerous (≥10), are often peribronchovascular and/or subpleural, and are frequently associated with nodular lesions that are often calcified.
Acknowledgments
Author contributions: A. C. Z. contributed to conception and design of the study; data acquisition, analysis, and interpretation; and writing the final manuscript; she is the guarantor of the manuscript and takes responsibility for the integrity of the data and the accuracy of the data analysis and served as principal author.
D. B. W. contributed to conception and design of the study; data acquisition, analysis, and interpretation and reading of the HRCT scans; writing or revising the final
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2022, Radiologic Clinics of North AmericaCitation Excerpt :CT typically reveals well-defined 2- to 4-mm micronodules with reticular opacities, interlobar septal thickening, and confluent consolidations with basal and peripheral predominance.43 Pulmonary cysts, when they occur, are often multiple and subpleural or perivascular in distribution48 (Fig. 8). Although cancers associated with cystic spaces do not represent DCLD, it is important to be aware of this entity, as delayed diagnosis may lead to increased mortality.
FUNDING/SUPPORT: The authors have reported to CHEST that no funding was received for this study.