Incidence of light chain amyloidosis in Florence metropolitan area, Italy: a population-based study

Zampieri, Mattia; Cappelli, Francesco; Allinovi, Marco; Olivotto, Iacopo; Antonioli, Elisabetta; Tassetti, Luigi; Zocchi, Chiara; Andrei, Valentina; Mario, Carlo Di; Nozzoli, Chiara; Curciarello, Giuseppe; Ciciani, Anna Maria; Bergesio, Franco; Vannucchi, Alessandro M.; Perfetto, Federico

doi:10.1080/13506129.2021.1898365

Cited by 11 publications

(14 citation statements)

References 4 publications

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“…The prevalence of light chain (AL) amyloidosis in the United States increased significantly between 2007 and 2015, from 15.5 cases per million in 2007 to 40.5 cases in 2015 ( 8 ). More recently, the incidence of AL amyloidosis from Italy in 2021 is estimated at 9 cases per million person-years ( 9 ). From recent reports, ATTR wild type cardiac amyloidosis appears to be quite common.…”

Section: Introductionmentioning

confidence: 99%

From Clinical Clues to Final Diagnosis: The Return of Detective Work to Clinical Medicine in Cardiac Amyloidosis

Sabbour

Hasan²,

Badarin³

et al. 2021

Front. Cardiovasc. Med.

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Cardiac amyloidosis is frequently misdiagnosed, denying patients the opportunity for timely and appropriate management of the disease. The purpose of this review and case studies is to raise awareness of the diagnostic “red flags” associated with cardiac amyloidosis and the currently available non-invasive strategies for diagnosis. The review focuses on the identification of one of the two main types of cardiac amyloidosis, transthyretin amyloid cardiomyopathy, and non-invasive tools to distinguish this from light-chain amyloidosis. A diagnostic algorithm centered around the use of non-invasive imaging and laboratory analysis is presented. The algorithm generates four differential diagnoses for patients presenting with signs and symptoms consistent with cardiac amyloidosis. Case examples are presented, representing the four potential outcomes of diagnosis using the algorithm. The review provides a guide on how to recognize the often-overlooked presentations of this disease in clinical practice. Non-invasive imaging techniques and diagnostic tools that do not require the involvement of a specialty center have allowed for the improved diagnosis of cardiac amyloidosis. Timely diagnosis of this life-threatening disease is essential for optimal management and it is imperative that clinicians have a high index of suspicion for patients presenting with “red flag” symptoms.

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Section: Introductionmentioning

confidence: 99%

From Clinical Clues to Final Diagnosis: The Return of Detective Work to Clinical Medicine in Cardiac Amyloidosis

Sabbour

Hasan²,

Badarin³

et al. 2021

Front. Cardiovasc. Med.

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“…Following screening of titles and abstracts, 45 studies were quali ed for full-text evaluation. After full-text evaluation, 6 studies were retained for the nal review [11,12,13,14,15,16]. Table 1 describes the characteristics of the included studies, which were from the United States (n = 2), Argentina (n = 1), Italy (n = 1), France (n = 1), and Sweden (n = 1).…”

Section: Resultsmentioning

confidence: 99%

“…The studies involved over 200 million person-years, and included 2502 patients diagnosed with AL amyloidosis. [11,15]. Two studies relied exclusively on retrospective records from different sources, which may not be standardized in diagnostic technique or coding for documentation [13,14].…”

Section: Resultsmentioning

confidence: 99%

“…Of the 6 studies that reported an incidence rate for AL amyloidosis, 4 used biopsy specimens as part of a diagnostic criteria [11,12,15,16] whereas the rest (2/6) relied solely on medical records [13,14]. The reported incidence rates for AL amyloidosis ranged from 6.13 to 12.5 per one-million person years, and the pooled analysis showed an incidence rate of 10.48 per one-million person years (95% CI, 8.99 to 11.96; I 2 = 54%; Fig.…”

Section: The Incidence Rate Of Al Amyloidosismentioning

confidence: 99%

“…Four studies reported data by sex, each reporting a male predominance for AL amyloidosis with estimates ranging from 54-64% of cases [11,13,15,16]. Three studies reported data on age, each reporting the incidence of AL amyloidosis is higher in older age groups and rare in persons younger than 30 years old [11,13,16].…”

Section: The Incidence Rate Of Al Amyloidosismentioning

confidence: 99%

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The Incidence Rate of AL Amyloidosis: A Systematic Review and Meta-analysis

Hanover

Buchner

2022

Preprint

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Introduction: AL amyloidosis is widely regarded as a rare disease, but characterization of its epidemiology has scarcely been reported. The purpose of this systematic review is to estimate the incidence rate of AL amyloidosis and examine population differences. Methods: MEDLINE, PubMed, and Google Scholar were searched from their inception until November 13, 2021 using search terms AL amyloidosis or immunoglobulin light-chain amyloidosis or light-chain amyloidosis or primary amyloidosis and incidence or epidemiology. Random-effects meta analysis of all cohort studies reporting an incidence rates for AL amyloidosis was performed. The quality of each study was assessed using a modified Newcastle-Ottawa scale. Subgroup analysis was performed based on geographical region. Results: Six studies with data from 2502 diagnosed cases of AL amyloidosis from 5 countries were included. The pooled incidence rate for AL amyloidosis was 10.48 per one-million person years (95% CI, 8.99 to 11.96). There was moderate heterogeneity in the data, which was eliminated with a subgroup analysis according to geographical region. AL amyloidosis was found to be more common in the Americas (incidence rate 11.52 per one-million person years, 95% CI 11.04 to 12.00) than in the Europe (9.10 per one-million person years, 95% CI, 7.62 to 10.58). Conclusion: There is a low worldwide incidence rate for AL amyloidosis, supporting the characterization of AL amyloidosis as a rare disease. The incidence of AL amyloidosis appears to vary in different populations, which suggests further well-designed studies are needed to elucidate underlying etiological factors and better inform clinical suspicion for AL amyloidosis.

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Circulating biomarkers in diagnosis and management of cardiac amyloidosis: a review for internist

et al. 2022

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Cardiac amyloidosis (CA) is due to extracellular myocardial deposition of misfolded proteins resulting in severe cardiac dysfunction and death. The precursors of amyloid fibrils, able of determining a relevant cardiac infiltration, are immunoglobulin-free light chains (AL amyloidosis) and transthyretin (TTR) (both wild and mutated types). The diagnosis of amyloidosis represents a challenge for the clinician given its rarity and its protean clinical presentation, thus an early diagnosis remains a cornerstone for the prognosis of these patients, also in light of the growing available treatments. There is great interest in identifying and applying biomarkers to help diagnose, inform prognosis, guide therapy, and serve as surrogate endpoints in these patients. In AL amyloidosis, biomarkers such as free light chains, natriuretic peptides and troponins are the most extensively studied and validated; they have proved useful in risk stratification, guiding treatment choice and monitoring hematological and organ response. A similar biomarker-based prognostic score is also proposed for ATTR amyloidosis, although studies are small and need to be validated for wild-type and mutant forms. Graphical abstract

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Incidence of light chain amyloidosis in Florence metropolitan area, Italy: a population-based study

Cited by 11 publications

References 4 publications

From Clinical Clues to Final Diagnosis: The Return of Detective Work to Clinical Medicine in Cardiac Amyloidosis

From Clinical Clues to Final Diagnosis: The Return of Detective Work to Clinical Medicine in Cardiac Amyloidosis

The Incidence Rate of AL Amyloidosis: A Systematic Review and Meta-analysis

Circulating biomarkers in diagnosis and management of cardiac amyloidosis: a review for internist

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