title: Senile systemic amyloidosis: clinical features at presentation and outcome.
abstract: BACKGROUND
  Cardiac amyloidosis is a fatal disease whose prognosis and treatment rely on identification of the amyloid type. In our aging population transthyretin amyloidosis (ATTRwt) is common and must be differentiated from other amyloid types. We report the clinical presentation, natural history, and prognostic features of ATTRwt compared with cardiac-isolated AL amyloidosis and calculate the probability of disease diagnosis of ATTRwt from baseline factors.
  
  
  RESULTS
  All patients with biopsy-proven ATTRwt (102 cases) and isolated cardiac AL (36 cases) seen from 2002 to 2011 at the UK National Amyloidosis Center were included. Median survival from the onset of symptoms was 6.07 years in the ATTRwt group and 1.7 years in the AL group. Positive troponin, a pacemaker, and increasing New York Heart Association (NYHA) class were associated with worse survival in ATTRwt patients on univariate analysis. All patients with isolated cardiac AL and 24.1% of patients with ATTRwt had evidence of a plasma cell dyscrasia. Older age and lower N-terminal pro-B-type natriuretic peptide (NT pro-BNP) were factors significantly associated with ATTRwt. Patients aged 70 years and younger with an NT pro-BNP <183 pmol/L were more likely to have ATTRwt, as were patients older than 70 years with an NT pro-BNP <1420 pmol/L.
  
  
  CONCLUSIONS
  Factors at baseline associated with a worse outcome in ATTRwt are positive troponin T, a pacemaker, and NYHA class IV symptoms. The age of the patient at diagnosis and NT pro-BNP level can aid in distinguishing ATTRwt from AL amyloidosis.
authors.affiliation: National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK. j.pinney@ucl.ac.uk
authors.forename: Jennifer H
authors.initials: JH
authors.lastname: Pinney
authors.forename: Carol J
authors.initials: CJ
authors.lastname: Whelan
authors.forename: Aviva
authors.initials: A
authors.lastname: Petrie
authors.forename: Jason
authors.initials: J
authors.lastname: Dungu
authors.forename: Sanjay M
authors.initials: SM
authors.lastname: Banypersad
authors.forename: Prayman
authors.initials: P
authors.lastname: Sattianayagam
authors.forename: Ashutosh
authors.initials: A
authors.lastname: Wechalekar
authors.forename: Simon D J
authors.initials: SD
authors.lastname: Gibbs
authors.forename: Christopher P
authors.initials: CP
authors.lastname: Venner
authors.forename: Nancy
authors.initials: N
authors.lastname: Wassef
authors.forename: Carolyn A
authors.initials: CA
authors.lastname: McCarthy
authors.forename: Janet A
authors.initials: JA
authors.lastname: Gilbertson
authors.forename: Dorota
authors.initials: D
authors.lastname: Rowczenio
authors.forename: Philip N
authors.initials: PN
authors.lastname: Hawkins
authors.forename: Julian D
authors.initials: JD
authors.lastname: Gillmore
authors.forename: Helen J
authors.initials: HJ
authors.lastname: Lachmann
mesh_terms: D000368:Aged; D000682:Amyloid / Q000145:classification* / Q000235:genetics; D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D001706:Biopsy; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality; D018450:Disease Progression; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism* / Q000473:pathology; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D011228:Prealbumin; D011379:Prognosis; D012017:Referral and Consultation / Q000706:statistics & numerical data
chemical_list: D000682:Amyloid; D010446:Peptide Fragments; D011228:Prealbumin; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain