U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Pulmonary amyloidosis

MedGen UID:
675153
•Concept ID:
C0700376
•
Disease or Syndrome
Synonym: pulmonary amyloidosis
SNOMED CT: Pulmonary amyloidosis (196135008)
 
HPO: HP:0033247
Monarch Initiative: MONDO:0800127

Definition

Extracellular deposition in lung tissue of a proteinaceous material that, when stained with Congo red, demonstrates apple-green birefringence under polarized light and that has a distinct color when stained with sulfated Alcian blue. Viewed with electron microscopy, the amyloid deposits are seen to be composed of a beta-sheet fibrillar material. These nonbranching fibrils have a diameter of 7.5 to 10 nm and are the result of protein misfolding. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPulmonary amyloidosis

Professional guidelines

PubMed

Incidental Colonic Masses, Cytomegalovirus Infection, and Amyloidosis in a Treatment-Naive Chronic Lymphocytic Leukemia (CLL) Patient without GI Symptoms.
Elzamly S, Joldoshova A, Taha H, Shitawi M, Ghosh A, Everett J
Ann Clin Lab Sci 2022 Nov;52(6):1002-1006. PMID: 36564073
Local vs. systemic pulmonary amyloidosis-impact on diagnostics and clinical management.
Baumgart JV, Stuhlmann-Laeisz C, Hegenbart U, Nattenmüller J, Schönland S, Krüger S, Behrens HM, Röcken C
Virchows Arch 2018 Nov;473(5):627-637. Epub 2018 Aug 22 doi: 10.1007/s00428-018-2442-x. PMID: 30136180
Amyloid-associated cystic lung disease in primary Sjögren's syndrome.
Baqir M, Kluka EM, Aubry MC, Hartman TE, Yi ES, Bauer PR, Ryu JH
Respir Med 2013 Apr;107(4):616-21. Epub 2013 Feb 10 doi: 10.1016/j.rmed.2013.01.005. PMID: 23402779

Recent clinical studies

Etiology

Pulmonary storage.
Šterclová M
Vnitr Lek 2022 Winter;68(8):525-531. doi: 10.36290/vnl.2022.111. PMID: 36575071
Clinical, radiologic, and pathologic features and outcomes of pulmonary transthyretin amyloidosis.
Eggleston RH, Hartman TE, Walkoff LA, Yi ES, Ryu JH, Baqir M
Respir Med 2022 Apr;194:106761. Epub 2022 Feb 8 doi: 10.1016/j.rmed.2022.106761. PMID: 35217402
Amyloidosis of the Lung.
Khoor A, Colby TV
Arch Pathol Lab Med 2017 Feb;141(2):247-254. doi: 10.5858/arpa.2016-0102-RA. PMID: 28134587
Amyloid-associated Cystic Lung Disease.
Zamora AC, White DB, Sykes AM, Hoskote SS, Moua T, Yi ES, Ryu JH
Chest 2016 May;149(5):1223-33. Epub 2016 Jan 12 doi: 10.1378/chest.15-1539. PMID: 26513525
Senile pulmonary amyloidosis.
Kunze WP
Pathol Res Pract 1979 Aug;164(4):413-22. doi: 10.1016/S0344-0338(79)80058-8. PMID: 514902

Diagnosis

Pulmonary storage.
Šterclová M
Vnitr Lek 2022 Winter;68(8):525-531. doi: 10.36290/vnl.2022.111. PMID: 36575071
Clinical, radiologic, and pathologic features and outcomes of pulmonary transthyretin amyloidosis.
Eggleston RH, Hartman TE, Walkoff LA, Yi ES, Ryu JH, Baqir M
Respir Med 2022 Apr;194:106761. Epub 2022 Feb 8 doi: 10.1016/j.rmed.2022.106761. PMID: 35217402
Amyloidosis of the Lung.
Khoor A, Colby TV
Arch Pathol Lab Med 2017 Feb;141(2):247-254. doi: 10.5858/arpa.2016-0102-RA. PMID: 28134587
Radiographic and Pathologic Manifestations of Uncommon and Rare Pulmonary Lesions.
Pfeifer K, Mian A, Adebowale A, Alomari A, Kalra V, Krejci E, Shin MS
Can Assoc Radiol J 2016 May;67(2):179-89. Epub 2015 Dec 11 doi: 10.1016/j.carj.2015.07.004. PMID: 26690551
Senile pulmonary amyloidosis.
Kunze WP
Pathol Res Pract 1979 Aug;164(4):413-22. doi: 10.1016/S0344-0338(79)80058-8. PMID: 514902

Therapy

Atypical primary pulmonary amyloidosis: A rare case report.
Peng X, Wang X, Luo D, Zuo W, Yao H, Zhang W
Medicine (Baltimore) 2020 Jun 26;99(26):e20828. doi: 10.1097/MD.0000000000020828. PMID: 32590770Free PMC Article
Pulmonary and Bronchiolar Involvement in Sjogren's Syndrome.
Chung A, Wilgus ML, Fishbein G, Lynch JP 3rd
Semin Respir Crit Care Med 2019 Apr;40(2):235-254. Epub 2019 May 28 doi: 10.1055/s-0039-1688448. PMID: 31137063
Pulmonary amyloidosis in Sjogren's syndrome: a case report and systematic review of the literature.
Rajagopala S, Singh N, Gupta K, Gupta D
Respirology 2010 Jul;15(5):860-6. Epub 2010 Jun 4 doi: 10.1111/j.1440-1843.2010.01772.x. PMID: 20546191
Aging and transthyretin-related amyloidosis: pathologic examinations in pulmonary amyloidosis.
Ueda M, Ando Y, Haraoka K, Katsuragi S, Terasaki Y, Sugimoto M, Sun X, Uchino M
Amyloid 2006 Mar;13(1):24-30. doi: 10.1080/13506120500537194. PMID: 16690497
Amyloidosis and lymphoproliferative disease in Sjögren syndrome: thin-section computed tomography findings and histopathologic comparisons.
Jeong YJ, Lee KS, Chung MP, Han J, Chung MJ, Kim KI, Seo JB, Franquet T
J Comput Assist Tomogr 2004 Nov-Dec;28(6):776-81. doi: 10.1097/00004728-200411000-00008. PMID: 15538150

Prognosis

Pulmonary storage.
Šterclová M
Vnitr Lek 2022 Winter;68(8):525-531. doi: 10.36290/vnl.2022.111. PMID: 36575071
Clinical, radiologic, and pathologic features and outcomes of pulmonary transthyretin amyloidosis.
Eggleston RH, Hartman TE, Walkoff LA, Yi ES, Ryu JH, Baqir M
Respir Med 2022 Apr;194:106761. Epub 2022 Feb 8 doi: 10.1016/j.rmed.2022.106761. PMID: 35217402
Localised forms of pulmonary amyloidosis.
Mnacakanová E, Henzlová L, Flodrová P, Pika T
Vnitr Lek 2021 Winter;67(6):357-364. PMID: 35459379
Local vs. systemic pulmonary amyloidosis-impact on diagnostics and clinical management.
Baumgart JV, Stuhlmann-Laeisz C, Hegenbart U, Nattenmüller J, Schönland S, Krüger S, Behrens HM, Röcken C
Virchows Arch 2018 Nov;473(5):627-637. Epub 2018 Aug 22 doi: 10.1007/s00428-018-2442-x. PMID: 30136180
The lung in amyloidosis.
Milani P, Basset M, Russo F, Foli A, Palladini G, Merlini G
Eur Respir Rev 2017 Sep 30;26(145) Epub 2017 Sep 6 doi: 10.1183/16000617.0046-2017. PMID: 28877975Free PMC Article

Clinical prediction guides

The "Wagshurst study": p.Val40Ile transthyretin gene variant causes late-onset cardiomyopathy.
Bauer R, Dikow N, Brauer A, Kreuter M, Buss S, Evers C, Röcken C, Schnabel PA, Hinderhofer K, Ehlermann P, Katus HA, Kristen AV
Amyloid 2014 Dec;21(4):267-75. Epub 2014 Oct 7 doi: 10.3109/13506129.2014.967846. PMID: 25291558
External beam radiation therapy is safe and effective in treating primary pulmonary amyloidosis.
Ren S, Ren G
Respir Med 2012 Jul;106(7):1063-9. Epub 2012 Mar 8 doi: 10.1016/j.rmed.2012.02.011. PMID: 22405606
Pulmonary amyloidosis in Sjogren's syndrome: a case report and systematic review of the literature.
Rajagopala S, Singh N, Gupta K, Gupta D
Respirology 2010 Jul;15(5):860-6. Epub 2010 Jun 4 doi: 10.1111/j.1440-1843.2010.01772.x. PMID: 20546191
Amyloidosis and lymphoproliferative disease in Sjögren syndrome: thin-section computed tomography findings and histopathologic comparisons.
Jeong YJ, Lee KS, Chung MP, Han J, Chung MJ, Kim KI, Seo JB, Franquet T
J Comput Assist Tomogr 2004 Nov-Dec;28(6):776-81. doi: 10.1097/00004728-200411000-00008. PMID: 15538150
Lung pathology in chronic hemodialysis patients.
Fairshter RD, Vaziri ND, Mirahmadi MK
Int J Artif Organs 1982 Mar;5(2):97-100. PMID: 7095887

Recent systematic reviews

Diffuse parenchymal pulmonary amyloidosis associated with multiple myeloma: a case report and systematic review of the literature.
Liu Y, Jin Z, Zhang H, Zhang Y, Shi M, Meng F, Sun Q, Cai H
BMC Cancer 2018 Aug 8;18(1):802. doi: 10.1186/s12885-018-4565-5. PMID: 30089469Free PMC Article
Pulmonary amyloidosis in Sjogren's syndrome: a case report and systematic review of the literature.
Rajagopala S, Singh N, Gupta K, Gupta D
Respirology 2010 Jul;15(5):860-6. Epub 2010 Jun 4 doi: 10.1111/j.1440-1843.2010.01772.x. PMID: 20546191

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...