U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Wild type ATTR amyloidosis

MedGen UID:
87446
•Concept ID:
C0342623
•
Disease or Syndrome
Synonyms: ATTRwt amyloidosis; ATTRwt-related amyloidosis; Senile systemic amyloidosis; SSA; wild type ATTR amyloidosis; wild type ATTR-related amyloidosis; Wild type ATTR-related amyloidosis
SNOMED CT: Senile systemic amyloidosis (237877004); Wild type ATTR amyloidosis (237877004)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018018
Orphanet: ORPHA330001

Definition

A common form of systemic amyloidosis characterized by deposition of wild type transthyretin predominantly in the heart and the soft tissues (mainly the carpal tunnel region, lumbar canal and tendons). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVWild type ATTR amyloidosis

Professional guidelines

PubMed

Advancing Transthyretin Amyloidosis Drug Development in an Evolving Treatment Landscape: Amyloidosis Forum Meeting Proceedings.
Maurer MS, Soman P, Hernandez A, Garcia-Pavia P, Signorovitch J, Wei LJ, Hanna M, Ruberg FL, Kittleson M, Kazi D, Dorbala S, Hsu K, Lousada I, Adigun R, Dunnmon P, Kelly J, Gillmore J; Amyloidosis Forum Meeting Panelists
Adv Ther 2024 Jul;41(7):2723-2742. Epub 2024 Jun 4 doi: 10.1007/s12325-024-02891-0. PMID: 38833142Free PMC Article
Clinical and apparative investigation of large and small nerve fiber impairment in mixed cohort of ATTR-amyloidosis: impact on patient management and new insights in wild-type.
Papagianni A, Ihne S, Zeller D, Morbach C, Üçeyler N, Sommer C
Amyloid 2022 Mar;29(1):14-22. Epub 2021 Oct 11 doi: 10.1080/13506129.2021.1976751. PMID: 34632904
Cardiac amyloidosis: An update on pathophysiology, diagnosis, and treatment.
Siddiqi OK, Ruberg FL
Trends Cardiovasc Med 2018 Jan;28(1):10-21. Epub 2017 Jul 13 doi: 10.1016/j.tcm.2017.07.004. PMID: 28739313Free PMC Article

Recent clinical studies

Etiology

Advancing Transthyretin Amyloidosis Drug Development in an Evolving Treatment Landscape: Amyloidosis Forum Meeting Proceedings.
Maurer MS, Soman P, Hernandez A, Garcia-Pavia P, Signorovitch J, Wei LJ, Hanna M, Ruberg FL, Kittleson M, Kazi D, Dorbala S, Hsu K, Lousada I, Adigun R, Dunnmon P, Kelly J, Gillmore J; Amyloidosis Forum Meeting Panelists
Adv Ther 2024 Jul;41(7):2723-2742. Epub 2024 Jun 4 doi: 10.1007/s12325-024-02891-0. PMID: 38833142Free PMC Article
Association between spinal stenosis and wild-type ATTR amyloidosis.
Godara A, Riesenburger RI, Zhang DX, Varga C, Fogaren T, Siddiqui NS, Yu A, Wang A, Mastroianni M, Dowd R, Nail TJ, McPhail ED, Kurtin PJ, Theis JD, Toskic D, Arkun K, Pilichowska M, Kryzanski J, Patel AR, Comenzo R
Amyloid 2021 Dec;28(4):226-233. Epub 2021 Jul 15 doi: 10.1080/13506129.2021.1950681. PMID: 34263670
When and how do patients with cardiac amyloidosis die?
Escher F, Senoner M, Doerler J, Zaruba MM, Messner M, Mussner-Seeber C, Ebert M, Ensinger C, Mair A, Kroiss A, Ulmer H, Schneiderbauer-Porod S, Ebner C, Poelzl G
Clin Res Cardiol 2020 Jan;109(1):78-88. Epub 2019 May 27 doi: 10.1007/s00392-019-01490-2. PMID: 31134330Free PMC Article
Amyloidosis in Heart Failure.
Ihne S, Morbach C, Obici L, Palladini G, Störk S
Curr Heart Fail Rep 2019 Dec;16(6):285-303. doi: 10.1007/s11897-019-00446-x. PMID: 31782077
Cardiac amyloidosis: An update on pathophysiology, diagnosis, and treatment.
Siddiqi OK, Ruberg FL
Trends Cardiovasc Med 2018 Jan;28(1):10-21. Epub 2017 Jul 13 doi: 10.1016/j.tcm.2017.07.004. PMID: 28739313Free PMC Article

Diagnosis

Association between spinal stenosis and wild-type ATTR amyloidosis.
Godara A, Riesenburger RI, Zhang DX, Varga C, Fogaren T, Siddiqui NS, Yu A, Wang A, Mastroianni M, Dowd R, Nail TJ, McPhail ED, Kurtin PJ, Theis JD, Toskic D, Arkun K, Pilichowska M, Kryzanski J, Patel AR, Comenzo R
Amyloid 2021 Dec;28(4):226-233. Epub 2021 Jul 15 doi: 10.1080/13506129.2021.1950681. PMID: 34263670
Amyloidosis in Heart Failure.
Ihne S, Morbach C, Obici L, Palladini G, Störk S
Curr Heart Fail Rep 2019 Dec;16(6):285-303. doi: 10.1007/s11897-019-00446-x. PMID: 31782077
Transthyretin Amyloid Cardiomyopathy: JACC State-of-the-Art Review.
Ruberg FL, Grogan M, Hanna M, Kelly JW, Maurer MS
J Am Coll Cardiol 2019 Jun 11;73(22):2872-2891. doi: 10.1016/j.jacc.2019.04.003. PMID: 31171094Free PMC Article
Cardiac amyloidosis: An update on pathophysiology, diagnosis, and treatment.
Siddiqi OK, Ruberg FL
Trends Cardiovasc Med 2018 Jan;28(1):10-21. Epub 2017 Jul 13 doi: 10.1016/j.tcm.2017.07.004. PMID: 28739313Free PMC Article
Transthyretin (ATTR) amyloidosis: clinical spectrum, molecular pathogenesis and disease-modifying treatments.
Sekijima Y
J Neurol Neurosurg Psychiatry 2015 Sep;86(9):1036-43. Epub 2015 Jan 20 doi: 10.1136/jnnp-2014-308724. PMID: 25604431

Therapy

Advancing Transthyretin Amyloidosis Drug Development in an Evolving Treatment Landscape: Amyloidosis Forum Meeting Proceedings.
Maurer MS, Soman P, Hernandez A, Garcia-Pavia P, Signorovitch J, Wei LJ, Hanna M, Ruberg FL, Kittleson M, Kazi D, Dorbala S, Hsu K, Lousada I, Adigun R, Dunnmon P, Kelly J, Gillmore J; Amyloidosis Forum Meeting Panelists
Adv Ther 2024 Jul;41(7):2723-2742. Epub 2024 Jun 4 doi: 10.1007/s12325-024-02891-0. PMID: 38833142Free PMC Article
Tafamidis: a selective transthyretin stabilizer to treat wild-type ATTR amyloidosis and hereditary ATTR amyloidosis with cardiomyopathy.
Paton DM
Drugs Today (Barc) 2019 Dec;55(12):727-734. doi: 10.1358/dot.2019.55.12.3078389. PMID: 31942875
First nationwide survey on systemic wild-type ATTR amyloidosis in Japan.
Sekijima Y, Yazaki M, Ueda M, Koike H, Yamada M, Ando Y
Amyloid 2018 Mar;25(1):8-10. Epub 2017 Nov 28 doi: 10.1080/13506129.2017.1409706. PMID: 29182024
Safety and efficacy of a TTR specific antisense oligonucleotide in patients with transthyretin amyloid cardiomyopathy.
Benson MD, Dasgupta NR, Rissing SM, Smith J, Feigenbaum H
Amyloid 2017 Dec;24(4):219-225. Epub 2017 Sep 14 doi: 10.1080/13506129.2017.1374946. PMID: 28906150
Transthyretin (ATTR) amyloidosis: clinical spectrum, molecular pathogenesis and disease-modifying treatments.
Sekijima Y
J Neurol Neurosurg Psychiatry 2015 Sep;86(9):1036-43. Epub 2015 Jan 20 doi: 10.1136/jnnp-2014-308724. PMID: 25604431

Prognosis

Advancing Transthyretin Amyloidosis Drug Development in an Evolving Treatment Landscape: Amyloidosis Forum Meeting Proceedings.
Maurer MS, Soman P, Hernandez A, Garcia-Pavia P, Signorovitch J, Wei LJ, Hanna M, Ruberg FL, Kittleson M, Kazi D, Dorbala S, Hsu K, Lousada I, Adigun R, Dunnmon P, Kelly J, Gillmore J; Amyloidosis Forum Meeting Panelists
Adv Ther 2024 Jul;41(7):2723-2742. Epub 2024 Jun 4 doi: 10.1007/s12325-024-02891-0. PMID: 38833142Free PMC Article
When and how do patients with cardiac amyloidosis die?
Escher F, Senoner M, Doerler J, Zaruba MM, Messner M, Mussner-Seeber C, Ebert M, Ensinger C, Mair A, Kroiss A, Ulmer H, Schneiderbauer-Porod S, Ebner C, Poelzl G
Clin Res Cardiol 2020 Jan;109(1):78-88. Epub 2019 May 27 doi: 10.1007/s00392-019-01490-2. PMID: 31134330Free PMC Article
Wild-type ATTR amyloidosis may be associated with unexpected death among the elderly.
Shiozaki T, Sato N, Hayashi T, Kobayashi K, Asamura H
Leg Med (Tokyo) 2019 Nov;41:101634. Epub 2019 Oct 31 doi: 10.1016/j.legalmed.2019.101634. PMID: 31731233
Transthyretin Amyloid Cardiomyopathy: JACC State-of-the-Art Review.
Ruberg FL, Grogan M, Hanna M, Kelly JW, Maurer MS
J Am Coll Cardiol 2019 Jun 11;73(22):2872-2891. doi: 10.1016/j.jacc.2019.04.003. PMID: 31171094Free PMC Article
Cardiac amyloidosis: An update on pathophysiology, diagnosis, and treatment.
Siddiqi OK, Ruberg FL
Trends Cardiovasc Med 2018 Jan;28(1):10-21. Epub 2017 Jul 13 doi: 10.1016/j.tcm.2017.07.004. PMID: 28739313Free PMC Article

Clinical prediction guides

Thermal evaporation as sample preparation for silver-assisted laser desorption/ionization mass spectrometry imaging of cholesterol in amyloid tissues.
Strnad Š, Vrkoslav V, Mengr A, Fabián O, Rybáček J, Kubánek M, Melenovský V, Maletínská L, Cvačka J
Analyst 2024 May 28;149(11):3152-3160. doi: 10.1039/d4an00181h. PMID: 38630503
Wild-type Transthyretin Amyloidosis with Diffuse Alveolar-septal Amyloidosis Diagnosed by a Transbronchial Lung Biopsy.
Ishida M, Enomoto M, Hata T, Tanaka T, Sakaguchi C, Tamiya N, Tsuchiya M, Nagasaka Y
Intern Med 2022;61(14):2203-2207. Epub 2022 Jul 15 doi: 10.2169/internalmedicine.8521-21. PMID: 35850989Free PMC Article
Cardiac amyloidosis: An update on pathophysiology, diagnosis, and treatment.
Siddiqi OK, Ruberg FL
Trends Cardiovasc Med 2018 Jan;28(1):10-21. Epub 2017 Jul 13 doi: 10.1016/j.tcm.2017.07.004. PMID: 28739313Free PMC Article

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.

    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...