Chest
Volume 149, Issue 5, May 2016, Pages 1223-1233
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Original Research: Diffuse Lung Disease
Amyloid-associated Cystic Lung Disease

https://doi.org/10.1378/chest.15-1539Get rights and content

Background

Amyloid-associated cystic lung disease is rare. It can be associated with collagen vascular disease (CVD). We aimed to describe the clinical, radiology, and pathology findings of this entity.

Methods

We reviewed the records of subjects having biopsy-proven pulmonary amyloidosis with cystic lung disease demonstrated at high-resolution computed tomography (HRCT). Demographic characteristics, association with CVD and lymphoproliferative disorders, pulmonary function, and pathology results were reviewed. HRCT appearance was analyzed for number, size, distribution, and morphology of cysts and nodules.

Results

Twenty-one subjects (13 female, eight male; median age, 61 years) with cystic pulmonary amyloidosis were identified. The most common pulmonary function patterns were normal (42%) and obstructive (32%). The most common associated CVD was Sjögren syndrome (10 of 12). Nine subjects had no CVD. Cysts tended to be multiple (≥ 10 in 14 of 21, 67%), round (21 of 21, 100%), or lobulated (20 of 21, 95%); thin-walled (< 2 mm in 17 of 21, 81%); and of small (< 1 cm in 21 of 21, 100%) to moderate (1-2 cm in 17 of 21, 81%) size. Peribronchovascular (19 of 21, 90%) and subpleural (19 of 21, 90%) cysts were typically present. Seventeen (81%) subjects had lung nodules, which tended to be numerous (≥ 10 in 10 of 17, 59%; 4-9 in six of 17, 35%). At least one calcified nodule was present in 14 of 17 subjects (82%). Pulmonary mucosa-associated lymphoid tissue lymphoma (MALToma) was diagnosed in seven subjects (33%).

Conclusions

Amyloid-associated cystic lung disease can occur with or without underlying CVD. Cystic lesions in the lung are commonly numerous, often are peribronchovascular or subpleural, and are frequently associated with nodular lesions that are often calcified. MALToma was a relatively frequent association.

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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    FUNDING/SUPPORT: The authors have reported to CHEST that no funding was received for this study.

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