Geographic Disparities in Reported US Amyloidosis Mortality From 1979 to 2015

Alexander, Kevin M.; Orav, John; Singh, Avinainder; Jacob, Sophia; Menon, Adil; Padera, Robert F.; Kijewski, Marie Foley; Liao, Ronglih; Carli, Marcelo F. Di; Laubach, Jacob P.; Falk, Rodney H.; Dorbala, Sharmila

doi:10.1001/jamacardio.2018.2093

Cited by 81 publications

(48 citation statements)

References 16 publications

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“…A higher incidence and prevalence of the disease has been reported in males as compared to females[ 12 ]. In the United States, the literature also reported a substantial increase in amyloidosis-related mortality from 1.77 to 3.96 per million between 1979 and 2015, with the highest mortality rates noted in the African-American population[ 13 ].…”

Section: Epidemiologymentioning

confidence: 99%

Gastrointestinal amyloidosis: A focused review

Dahiya¹,

Kichloo²,

Singh³

et al. 2021

WJGE

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Section: Epidemiologymentioning

confidence: 99%

Gastrointestinal amyloidosis: A focused review

Dahiya¹,

Kichloo²,

Singh³

et al. 2021

WJGE

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“…The median age at diagnosis was 76 years, and one would expect 3800 new cases in the United States each year 10 . There appear to be geographic and racial disparities in the diagnosis 11 …”

Section: Introductionmentioning

confidence: 99%

Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment

Gertz

2020

American J Hematol

112

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Disease Overview Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include heart failure with preserved ejection fraction, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and “atypical smoldering multiple myeloma or monoclonal gammopathy undetermined significance (MGUS).” Diagnosis Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple‐green birefringence is required for diagnosis. Invasive organ biopsy is not required in 85% of patients. Verification that amyloid is composed of immunoglobulin light chains is mandatory. The gold standard is laser capture mass spectroscopy. Prognosis N‐terminal pro‐brain natriuretic peptide (NT‐proBNP), serum troponin T, and difference between involved and uninvolved immunoglobulin free light chain (FLC) values are used to classify patients into four groups of similar size; median survivals are 94.1, 40.3, 14.0, and 5.8 months. Therapy All patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure. Stem cell transplant (SCT) is preferred, but only 20% of patients are eligible. Requirements for safe SCT include systolic blood pressure >90 mmHg, troponin T < 0.06 ng/mL and serum creatinine ≤1.7 mg/dL. Nontransplant candidates can be offered cyclophosphamide‐bortezomib‐dexamethasone or daratumumab‐containing regimens as it appears to be highly active in AL amyloidosis. Future Challenges Delayed diagnosis remains a major obstacle to initiating effective therapy prior to the development of end‐stage organ failure.

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“…Cardiac amyloidosis is significantly underdiagnosed, partly because of a lack of suspicion and a widespread belief that it is rare. 68 Nevertheless, wild-type transthyretin (ATTRwt) cardiac amyloidosis is common in older adults 69 and HF patients with preserved ejection fraction. 70 Recently, novel diagnostic approaches 71 and therapies 72 emerged, which can improve clinical outcomes but also demonstrate complications of longer disease durations 73 .…”

Section: Discussionmentioning

confidence: 99%

Does Ventricular Substrate Play a Role in Incident Stroke? The Atherosclerosis Risk in Communities (ARIC) Study

Johnson

Haq

Lutz

et al. 2020

Preprint

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Background: The goal of the study was to determine an association of ventricular substrate with thrombotic, cardioembolic, and hemorrhagic stroke. Methods: Participants from the Atherosclerosis Risk in Communities study with analyzable ECGs and no history of stroke were included (n=14,479; age 54±6 y; 55% female; 24% black). Ventricular substrate was characterized by cardiac memory [spatial QRS-T angle (QRS-Ta), sum absolute QRST integral (SAIQRST), spatial ventricular gradient magnitude (SVGmag)], premature ventricular contractions (PVC) and tachycardia-dependent intermittent bundle branch block (TD-IBBB) on 12-lead ECG recorded at visits 1-5. Incident strokes were adjudicated by physician reviewers. Cox proportional hazard risk models were constructed. Results: Over a median 24.5 y follow-up, there were 899 thrombotic, 400 cardioembolic, and 187 hemorrhagic strokes. After adjustment for cardiovascular disease (CVD) and its risk factors, atrial fibrillation / atrial substrate, and ECG-left ventricular hypertrophy, PVC (HR 1.72; 95%CI 1.02-2.92), QRS-Ta (HR 1.15; 95%CI 1.03-1.28), SAIQRST (HR 1.20; 95%CI 1.07-1.34) and time-updated SVGmag (HR 1.19; 95%CI 1.08-1.32) associated with cardioembolic stroke. After adjustment for CVD and its risk factors, PVC (HR 1.53; 95%CI 1.03-2.26), QRS-Ta (HR 1.08; 95%CI 1.01-1.16), SAIQRST (HR 1.07; 95%CI 1.01-1.14), and time-updated SVGmag (HR 1.11; 95%CI 1.04-1.19) associated with thrombotic stroke. In fully adjusted time-updated Cox model QRS-Ta (HR 1.20; 95%CI 1.04-1.38), SAI QRST (HR 1.23; 95%CI 1.06-1.43), SVGmag (HR 1.23; 95%CI 1.06-1.43) associated with hemorrhagic stroke, whereas TD-IBBB trended (HR 1.84; 95%CI 0.25-13.33). Conclusions: PVC burden reflected by cardiac memory is associated with ischemic stroke. Transient cardiac memory (likely through TD-IBBB) precedes hemorrhagic stroke.

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Geographic Disparities in Reported US Amyloidosis Mortality From 1979 to 2015

Cited by 81 publications

References 16 publications

Gastrointestinal amyloidosis: A focused review

Gastrointestinal amyloidosis: A focused review

Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment

Does Ventricular Substrate Play a Role in Incident Stroke? The Atherosclerosis Risk in Communities (ARIC) Study

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