Amyloid-associated cystic lung disease

Ana C. Zamora, Darin B. White, Anne-Marie Gisele Sykes, Sumedh S. Hoskote, Teng Moua, Eunhee S. Yi, Jay H Ryu

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1223-1233
Number of pages11
JournalChest
Volume149
Issue number5
DOIs
StatePublished - May 1 2016

Fingerprint

Amyloid
Collagen Diseases
Vascular Diseases
Lung
Cysts
Marginal Zone B-Cell Lymphoma
Amyloidosis
Tomography
Clinical Pathology
Lymphoproliferative Disorders
Cystic Disease Of Lung
Radiology
Demography
Pathology
Biopsy

Keywords

  • Amyloidosis
  • Connective tissue disease
  • Interstitial lung disease

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Zamora, A. C., White, D. B., Sykes, A-M. G., Hoskote, S. S., Moua, T., Yi, E. S., & Ryu, J. H. (2016). Amyloid-associated cystic lung disease. Chest, 149(5), 1223-1233. https://doi.org/10.1378/chest.15-1539

Amyloid-associated cystic lung disease. / Zamora, Ana C.; White, Darin B.; Sykes, Anne-Marie Gisele; Hoskote, Sumedh S.; Moua, Teng; Yi, Eunhee S.; Ryu, Jay H.

In: Chest, Vol. 149, No. 5, 01.05.2016, p. 1223-1233.

Research output: Contribution to journalArticle

Zamora, AC, White, DB, Sykes, A-MG, Hoskote, SS, Moua, T, Yi, ES & Ryu, JH 2016, 'Amyloid-associated cystic lung disease', Chest, vol. 149, no. 5, pp. 1223-1233. https://doi.org/10.1378/chest.15-1539
Zamora AC, White DB, Sykes A-MG, Hoskote SS, Moua T, Yi ES et al. Amyloid-associated cystic lung disease. Chest. 2016 May 1;149(5):1223-1233. https://doi.org/10.1378/chest.15-1539
Zamora, Ana C. ; White, Darin B. ; Sykes, Anne-Marie Gisele ; Hoskote, Sumedh S. ; Moua, Teng ; Yi, Eunhee S. ; Ryu, Jay H. / Amyloid-associated cystic lung disease. In: Chest. 2016 ; Vol. 149, No. 5. pp. 1223-1233.
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abstract = "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 Sj{\~A}¶gren 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.",
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AU - Yi, Eunhee S.

AU - Ryu, Jay H

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N2 - 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.

AB - 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.

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