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Journal of Clinical Medicine Sep 2023The myocardial uptake of bone-seeking tracers suspicious for transthyretin cardiac amyloidosis (ATTR-CA) can be incidentally detected in patients undergoing bone... (Review)
Review
BACKGROUND
The myocardial uptake of bone-seeking tracers suspicious for transthyretin cardiac amyloidosis (ATTR-CA) can be incidentally detected in patients undergoing bone scintigraphy for noncardiac reasons. We conducted a systematic review and meta-analysis to assess the prevalence of these scintigraphic findings.
METHODS
A comprehensive literature search was performed using two bibliographic databases (PubMed/MEDLINE and Cochrane Library), searching for articles related to the review question. Eligible articles were selected, and relevant data were extracted by two authors. The pooled prevalence of incidental findings suspicious for ATTR-CA among patients undergoing bone scintigraphy was calculated on a per-patient-based analysis using a random-effects model. The pooled measure was provided with 95% confidence interval (95% CI) values.
RESULTS
Among 219 records, 11 articles were selected for the systematic review and 10 for the meta-analysis. The pooled prevalence of incidental findings suspicious for ATTR-CA was 1.1% (95% CI: 0.7-1.4%) with heterogeneity due to the characteristics of the included studies, patients, and index tests. These findings are more prevalent in older men.
CONCLUSIONS
The prevalence of incidental findings of ATTR-CA among patients undergoing bone scintigraphy is low but not negligible. Nuclear medicine physicians should suggest, in the scintigraphic report, further clinical investigations when these findings are detected. Prospective studies are warranted.
PubMed: 37685765
DOI: 10.3390/jcm12175698 -
European Journal of Nuclear Medicine... May 2024
PubMed: 37606860
DOI: 10.1007/s00259-023-06408-9 -
EJHaem Aug 2023Long-term follow-up of multiple myeloma (MM) clinical trials are needed to assess long-term outcomes. We aimed to investigate the length of follow-up of all phase III MM...
Long-term follow-up of multiple myeloma (MM) clinical trials are needed to assess long-term outcomes. We aimed to investigate the length of follow-up of all phase III MM clinical trials. Median follow-up duration of clinical trials of newly diagnosed MM was longer when compared to relapsed/refractory MM clinical trials (42.7 vs. 20.5 months, respectively). The follow-up duration of phase III clinical trials in MM is relatively short when compared to the improved outcomes in the current era. Efforts should be made to facilitate long-term clinical trials follow-up and/or publication of results of updated results.
PubMed: 37601879
DOI: 10.1002/jha2.680 -
European Journal of Nuclear Medicine... Dec 2023Transthyretin (ATTR) amyloidosis is a progressive protein misfolding disease with frequent cardiac involvement. This review aims to determine the value of PET in... (Review)
Review
PURPOSE
Transthyretin (ATTR) amyloidosis is a progressive protein misfolding disease with frequent cardiac involvement. This review aims to determine the value of PET in diagnosis, assessment of disease progression or treatment response and its relation to clinical outcome in follow-up of ATTR amyloid cardiomyopathy (ATTR-CM) patients.
METHODS
Medline, Cochrane Library, Embase and Web of Science databases were searched, from the earliest date available until December 2022, for studies investigating the use of PET in ATTR-CM patients. Studies containing original data were included, except for case reports. Risk of bias was assessed by QUADAS-2.
RESULTS
Twenty-one studies were included in this systematic review, investigating five different tracers: carbon-11 Pittsburgh compound B ([C]PIB), fluorine-18 Florbetaben ([F]FBB), fluorine-18 Florbetapir ([F]FBP), fluorine-18 Flutemetamol ([F]FMM) and fluorine-18 Sodium Fluoride (Na[F]F). In total 211 ATTR amyloidosis patients were included. A majority of studies concluded that [C]PIB, [F]FBP and Na[F]F can distinguish ATTR amyloidosis patients from controls, and that [C]PIB and Na[F]F, but not [F]FBP, can distinguish ATTR-CM patients from patients with cardiac light chain amyloidosis. Evidence on the performance of [F]FBB and [F]FMM was contradictory. No studies on the use of PET in follow-up were found.
CONCLUSION
[C]PIB, Na[F]F and [F]FBP can be used to diagnose cardiac amyloidosis, although [F]FBP may not be suitable for the distinction of different types of amyloid cardiomyopathy. No studies on PET in the follow-up of ATTR amyloidosis patients were found. Future research should focus on the use of these PET tracers in the follow-up of ATTR amyloidosis patients.
Topics: Humans; Prealbumin; Follow-Up Studies; Amyloidosis; Positron-Emission Tomography; Cardiomyopathies
PubMed: 37561144
DOI: 10.1007/s00259-023-06381-3 -
Medicina Oral, Patologia Oral Y Cirugia... Jul 2023Amyloidosis is a disease characterized by the progressive deposition of abnormal proteins that can occur in any organ. In the oral cavity, the tongue is the most common...
BACKGROUND
Amyloidosis is a disease characterized by the progressive deposition of abnormal proteins that can occur in any organ. In the oral cavity, the tongue is the most common affected site, usually causing macroglossia. Biopsy is essential for the diagnosis and the occurrence of its systemic form is mandatory to be investigated. This systematic review evaluated the existing information in the literature on Amyloidosis in the oral cavity to allow a more comprehensive and updated analysis of its clinicopathological characteristics, as well as to explore the main forms of treatment and prognostic factors.
MATERIAL AND METHODS
Electronic searches were undertaken in five databases supplemented by manual scrutiny.
RESULTS
A total of 111 studies were included with 158 individuals.
CONCLUSIONS
The disease had a higher prevalence in women, the tongue was the most affected site, as well as the systemic form of the disease. The worst prognosis was for cases of systemic amyloidosis associated with multiple myeloma.
Topics: Humans; Female; Amyloidosis; Macroglossia; Multiple Myeloma; Tongue Diseases; Tongue
PubMed: 37330968
DOI: 10.4317/medoral.25761 -
Translational Stroke Research Jun 2024In intracerebral hemorrhage (ICH) with pathology-proven etiology, we performed a systematic review and meta-analysis to elucidate the association between cerebral... (Meta-Analysis)
Meta-Analysis
In intracerebral hemorrhage (ICH) with pathology-proven etiology, we performed a systematic review and meta-analysis to elucidate the association between cerebral amyloid angiopathy (CAA) and arteriolosclerosis, and directly compared MRI and pathological changes of markers of cerebral small vessel disease (CSVD). Studies enrolling primary ICH who had received an etiological diagnosis through biopsy or autopsy were searched using Ovid MEDLINE, PubMed, and Web of Science from inception to June 8, 2022. We extracted pathological changes of CSVD for each patient whenever available. Patients were grouped into CAA + arteriolosclerosis, strict CAA, and strict arteriolosclerosis subgroups. Of 4155 studies identified, 28 studies with 456 ICH patients were included. The frequency of lobar ICH (p<0.001) and total microbleed number (p=0.015) differed among patients with CAA + arteriolosclerosis, strict CAA, and strict arteriolosclerosis. Concerning pathology, severe CAA was associated with arteriolosclerosis (OR 6.067, 95% CI 1.107-33.238, p=0.038), although this association was not statistically significant after adjusting for age and sex. Additionally, the total microbleed number (median 15 vs. 0, p=0.006) was higher in ICH patients with CAA evidence than those without CAA. The pathology of CSVD imaging markers was mostly investigated in CAA-ICH. There was inconsistency concerning CAA severity surrounding microbleeds. Small diffusion-weighted imaging lesions could be matched to acute microinfarct histopathologically. Studies that directly correlated MRI and pathology of lacunes, enlarged perivascular spaces, and atrophy were scarce. Arteriolosclerosis might be associated with severe CAA. The pathological changes of CSVD markers by ICH etiology are needed to be investigated further.
Topics: Humans; Cerebral Hemorrhage; Cerebral Small Vessel Diseases; Cerebral Amyloid Angiopathy; Arteriolosclerosis
PubMed: 37280502
DOI: 10.1007/s12975-023-01154-4 -
ESC Heart Failure Aug 2023The clinical value of cardiopulmonary exercise testing (CPET) in cardiac amyloidosis (CA) is uncertain. Due to the growing prevalence of the disease and the current... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The clinical value of cardiopulmonary exercise testing (CPET) in cardiac amyloidosis (CA) is uncertain. Due to the growing prevalence of the disease and the current availability of disease-modifying drugs, prognostic stratification is becoming fundamental to optimizing the cost-effectiveness of treatment, patient phenotyping, follow-up, and management. Peak VO and VE/VCO slope are currently the most studied CPET variables in clinical settings, and both demonstrate substantial, independent prognostic value in several cardiovascular diseases. We aim to study the association of peak VO and VE/VCO slope with prognosis in patients with CA.
METHODS AND RESULTS
We performed a systematic review and searched for clinical studies performing CPET for prognostication in patients with transthyretin-CA and light-chain-CA. Studies reporting hazard ratio (HR) for mortality and peak VO or VE/VCO slope were further selected for quantitative analysis. HRs were pooled using a random-effect model. Five studies were selected for qualitative and three for quantitative analysis. A total of 233 patients were included in the meta-analysis. Mean peak VO resulted consistently depressed, and VE/VCO slope was increased. Our pooled analysis showed peak VO (pooled HR 0.89, 95% CI 0.84-0.94) and VE/VCO slope (pooled HR 1.04, 95% CI 1.01-1.07) were significantly associated with the risk of death in CA patients, with no significant statistical heterogeneity for both analyses.
CONCLUSIONS
CPET is a valuable tool for prognostic stratification in CA, identifying patients at increased risk of death. Large prospective clinical trials are needed to confirm this exploratory finding.
Topics: Humans; Exercise Test; Prospective Studies; Oxygen Consumption; Prognosis; Amyloidosis; Cardiomyopathies
PubMed: 37264762
DOI: 10.1002/ehf2.14406 -
Frontiers in Cardiovascular Medicine 2023Cardiac amyloidosis (CA) is primarily a restrictive cardiomyopathy in which the impairment of diastolic function is dominant. Despite this, the left ventricular ejection...
BACKGROUND
Cardiac amyloidosis (CA) is primarily a restrictive cardiomyopathy in which the impairment of diastolic function is dominant. Despite this, the left ventricular ejection fraction (LVEF) may be depressed in the late stage of the disease, but it poorly predicts prognosis in the earlier phases and does not represent well the pathophysiology of CA. Many echocardiographic parameters resulted important diagnostic and prognostic tools in patients with CA. Stroke volume (SV) and myocardial contraction fraction (MCF) may be obtained both with echocardiography and cardiac magnetic resonance (MRI). They reflect many factors intrinsically related to the pathophysiology of CA and are therefore potentially associated with symptoms and prognosis in CA.
OBJECTIVES
To collect and summarize the current evidence on SV and MCF and their clinical and prognostic role in transthyretin (TTR-CA).
METHODS AND RESULTS
We performed a systematic review following the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. We searched the literature database for studies focusing on SV and MCF in patients with TTR-CA. We analysed the following databases: PUBMED, Cochrane Library, EMBASE, and Web of Science database. Fourteen studies were included in the review. Both SV and MCF have important prognostic implications and are related to mortality. Furthermore, SV is more related to symptoms than LVEF and predicts tolerability of beta-blocker therapy in TTR-CA. Finally, SV showed to be an excellent measure to suggest the presence of TTR-CA in patients with severe aortic stenosis.
CONCLUSION
Stroke volume and MCF are very informative parameters that should be routinely assessed during the standard echocardiographic examination of all patients with TTR-CA. They carry a prognostic role while being associated with patients' symptoms.
SYSTEMATIC REVIEW REGISTRATION
https://doi.org/10.17605/OSF.IO/ME7DS.
PubMed: 36776259
DOI: 10.3389/fcvm.2023.1085824 -
Cerebral Circulation - Cognition and... 2022The term Cerebral Amyloid Angiopathy (CAA) refers to a group of neurovascular disorders characterized by amyloid deposition within the walls of leptomeningeal and...
The term Cerebral Amyloid Angiopathy (CAA) refers to a group of neurovascular disorders characterized by amyloid deposition within the walls of leptomeningeal and cortical blood vessels of the brain, with specific predilection for arterioles, and (less often) capillaries and veins. Most CAA cases in the general population are sporadic in nature, and represent primarily an age-related condition affecting individuals in the fifth decade of life and beyond. Sporadic CAA is caused by deposition of amyloid-β (Aβ), originating from proteolytic cleavage of the Amyloid Precursor Protein (APP), within the walls of cerebral small caliber vessels. However, hereditary forms of CAA have also been described, generally presenting as rare familial disorder with monogenic (predominantly autosomal dominant) inheritance patterns. Hereditary CAA forms tend to affect younger individuals, and their course and clinical progression is more severe. Studies to date primarily focused on the vascular manifestations of sporadic and hereditary CAA, chiefly symptomatic lobar Intracerebral Hemorrhage (ICH). However, in the past decade sporadic CAA has also been consistently linked to progressive neurocognitive, neurobehavioral, and neuropsychiatric symptoms. This systematic review focuses on the genetics, pathogenesis, neuroimaging, neuropathology, and clinical manifestations of hereditary CAA with specific emphasis on previously overlooked cognitive, behavioral, and psychiatric symptoms.
PubMed: 36324420
DOI: 10.1016/j.cccb.2022.100124 -
Annals of Medicine Dec 2022Almost always, Congo red-stained amyloid between polariser and analyser is said to show "green birefringence" or "apple-green birefringence". In 2010, we found that not...
BACKGROUND
Almost always, Congo red-stained amyloid between polariser and analyser is said to show "green birefringence" or "apple-green birefringence". In 2010, we found that not all published images showed green, and not all that did showed only green. This systematic review of more recent papers was to find if there had been any improvement in the accuracy of reporting.
MATERIALS AND METHODS
MEDLINE was searched on 15 March 2021 for papers published between 2010 and 2020 inclusive mentioning amyloid and Congo red. These were examined for descriptions of colours, which were compared with images. Papers were searched for mentions of anomalous colours, errors in physical optics, and misquotation of references about polarisation.
RESULTS
In 374 papers, there were 444 descriptions of colours, with 511 images in 257 papers. The commonest descriptions were apple-green, 249444 (56%), and green, 105444 (24%). The description agreed with colours seen in 116/511 images (23%) (previously 64/191, 34%). Green was seen in 342511 images (67%) (previously 159/191, 83%), but not in 169511 (33%), although each image was reported to show green. Green alone was seen in 103511 images (20%) (previously 59/191, 31%), and was combined with at least one other colour in 239511 (47%). Ten papers included the term anomalous. Eight papers incorrectly said that there was green dichroism, three incorrectly used the term green metachromasia, and two incorrectly mentioned green fluorescence. Twenty-seven papers misquoted references.
CONCLUSIONS
There is widespread and increasing inaccuracy of reporting of colours seen in Congo red-stained amyloid. People persist in saying "green birefringence" or "apple-green birefringence", even when no green is seen, or there are also other colours. Few appear to appreciate that the other colours are genuine, respectable, and helpful, the physical optical principles that explain the colours are now understood, and the best expression to use is anomalous colours.KEY MESSAGE"Green birefringence" and "apple-green birefringence" are inappropriate terms to describe the findings in amyloid stained with Congo red and examined between crossed polariser and analyser, because green is not always seen, and even when it is, other colours are commonly seen as well. The proportions of colour images showing any green and green alone, and the proportion of descriptions that agreed with illustrated colours, significantly decreased in 2010-2020 compared with earlier. The most appropriate and scientific description of the findings is anomalous colours.
Topics: Amyloid; Amyloidosis; Birefringence; Congo Red; Humans; Staining and Labeling
PubMed: 36120888
DOI: 10.1080/07853890.2022.2123558