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JACC. Cardiovascular Imaging Jun 2020This study aimed to compare the diagnostic and prognostic performance of native T1 mapping (T1), extracellular volume (ECV) mapping, and late gadolinium enhancement... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
This study aimed to compare the diagnostic and prognostic performance of native T1 mapping (T1), extracellular volume (ECV) mapping, and late gadolinium enhancement (LGE) imaging for evaluating cardiac amyloidosis (CA).
BACKGROUND
CA is a progressive infiltrative process in the extracellular space that is often underdiagnosed and holds a poor prognosis. Cardiac magnetic resonance (CMR) offers novel techniques for detecting and quantifying the disease burden of CA.
METHODS
We searched PubMed for published studies using native T1, ECV, or LGE to diagnose and prognosticate CA. A total of 18 diagnostic (n = 2,015) and 13 prognostic studies (n = 1,483) were included for analysis. Pooled sensitivities, specificities, diagnostic odds ratios (DORs) of all diagnostic tests were assessed by bivariate analysis. Pooled hazard ratios (HRs) for mortality for the 3 techniques were determined.
RESULTS
Bivariate comparison showed that ECV (DOR: 84.6; 95% confidence interval [CI]: 30.3 to 236.2) had a significantly higher DOR for CA than LGE (DOR: 20.1; 95% CI: 9.1 to 44.1; p = 0.03 vs. ECV). There was no significant difference between LGE and native T1 for sensitivity, specificity, and DOR. HR was significantly higher for ECV (HR: 4.27; 95% CI: 2.87 to 6.37) compared with LGE (HR: 2.60; 95% CI: 1.90 to 3.56; p = 0.03 vs. ECV) and native T1 (HR: 2.04; 95% CI: 1.24 to 3.37; p = 0.01 vs. ECV).
CONCLUSIONS
ECV demonstrates a higher diagnostic OR for assessing cardiac amyloid than LGE and a higher HR for adverse events compared with LGE and native T1. In addition, native T1 showed similar sensitivity and specificity as ECV and LGE without requiring contrast material. Although limited by study heterogeneity, this meta-analysis suggests that ECV provides high diagnostic and prognostic utility for the assessment of cardiac amyloidosis.
Topics: Adult; Aged; Amyloid Neuropathies, Familial; Cardiomyopathies; Female; Gadolinium; Humans; Immunoglobulin Light-chain Amyloidosis; Magnetic Resonance Imaging; Male; Middle Aged; Myocardium; Predictive Value of Tests; Reproducibility of Results; Stroke Volume; Ventricular Function, Left
PubMed: 32498919
DOI: 10.1016/j.jcmg.2020.03.010 -
CNS & Neurological Disorders Drug... 2021Vascular Dementia (VaD) occurs due to cerebrovascular insufficiency, which leads to decreased blood circulation to the brain, thereby resulting in mental disabilities....
Vascular Dementia (VaD) occurs due to cerebrovascular insufficiency, which leads to decreased blood circulation to the brain, thereby resulting in mental disabilities. The main causes of Vascular Cognitive Impairment (VCI) are severe hypoperfusion, stroke, hypertension, large vessel disease (cortical), small Vessel Disease (subcortical VaD), strategic infarct, hemorrhage (microbleed), Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), and Cerebral Amyloid Angiopathy (CAA), which leads to decreased cerebrovascular perfusion. Many metabolic disorders such as Diabetes Mellitus (DM), dyslipidemia, and hyperhomocysteinemia are also related to VaD. The rodent experimental models provide a better prospective for the investigation of the molecular mechanism of new drugs. A plethora of experimental models are available that mimic the pathological conditions and lead to VaD. This review article updates the current knowledge on the basis of VaD, risk factors, pathophysiology, mechanism, advantages, limitations, and the modification of various available rodent experimental models.
Topics: Alzheimer Disease; Animals; Brain; CADASIL; Cerebral Amyloid Angiopathy; Cognitive Dysfunction; Dementia, Vascular; Disease Models, Animal; Hypertension; Risk Factors; Rodentia; Stroke
PubMed: 33423653
DOI: 10.2174/1871527319666210108123438 -
Journal of Clinical Neuroscience :... Aug 2021We measured the proportion of Lewy body pathology (LB), hippocampal sclerosis (HS), and cerebral amyloid angiopathy (CAA) among community-dwelling people with and... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
We measured the proportion of Lewy body pathology (LB), hippocampal sclerosis (HS), and cerebral amyloid angiopathy (CAA) among community-dwelling people with and without dementia.
METHODS
We searched for community-based cohorts with postmortem brain autopsy until 1 January 2020. We calculated the summary risk difference and 95% confidence interval (95% CI) using a random-effects model in R.
RESULTS
We found 12 articles, comprising 2197 demented and 2104 non-demented participants. LB, HS, CAA were prevalent lesions among community-dwelling elderly (15%, 10%, and 24%, respectively). These significantly increased the risk of dementia (LB: risk difference 38%, 95% CI 20-56%, HS: 34%, 24-44%, CAA: 19%, 3-34%). 20% of cases with neocortical LB, 17% with bilateral HS, and 42% with moderate/severe CAA pathology remained non-demented by death.
DISCUSSION
LB or HS or CAA are common neuropathologies among community-dwelling elderly. Although these lesions independently are associated with dementia, many remain non-demented, by death.
Topics: Aged; Aged, 80 and over; Cerebral Amyloid Angiopathy; Dementia; Female; Hippocampus; Humans; Independent Living; Lewy Bodies; Male; Prevalence; Sclerosis
PubMed: 34275535
DOI: 10.1016/j.jocn.2021.05.044 -
Journal de Mycologie Medicale Dec 2019Secondary amyloidosis results from the deposition of abnormally folded proteins in body organs due to chronic inflammatory disorders. Kidneys are the most commonly...
Secondary amyloidosis results from the deposition of abnormally folded proteins in body organs due to chronic inflammatory disorders. Kidneys are the most commonly affected organ and manifest as nephrotic syndrome with or without renal failure. Chronic pulmonary aspergillosis (CPA) is a chronic infection of lung parenchyma affecting those with an underlying structural lung disease. Herein, we present a case of CPA where the initial manifestation was that of nephrotic syndrome due to renal amyloidosis. We also perform a systematic review for studies describing secondary amyloidosis due to CPA.
Topics: Adult; Amyloidosis; Chronic Disease; Female; Humans; Kidney; Nephrotic Syndrome; Pulmonary Aspergillosis
PubMed: 31570305
DOI: 10.1016/j.mycmed.2019.100898 -
Rheumatology International Jul 2024We aimed to review the literature on the clinical presentation, renal pathology, treatment, and outcome of renal manifestations in adult-onset Still's disease (AOSD).
OBJECTIVE
We aimed to review the literature on the clinical presentation, renal pathology, treatment, and outcome of renal manifestations in adult-onset Still's disease (AOSD).
METHODS
We used PRISMA guidelines for our systematic review and included all English-language original articles from inception till September 15, 2023, on AOSD and kidney involvement in any form. Data on patient demographics, diagnostic criteria, clinical presentation, renal pathology, treatment employed including dialysis, outcome, cause of death were collected and analyzed.
RESULTS
The median age at the diagnosis of renal issues was 37, with a higher prevalence among females (58.1%). Among the cases, 28 experienced renal problems after being diagnosed with AOSD, 12 had simultaneous diagnoses of renal issues and AOSD, and in 4 cases, renal problems appeared before AOSD diagnosis. Out of the 44 cases, 36 underwent renal biopsy, revealing various pathology findings including AA amyloidosis (25%), collapsing glomerulopathy (11.4%), thrombotic microangiopathy (TMA) (11.4%), IgA nephropathy (9.1%), minimal change disease (6.8%), and others. Some cases were clinically diagnosed with TMA, proximal tubular dysfunction, or macrophage activation syndrome-related acute kidney injury. Treatment approaches varied, but glucocorticoids were commonly used. Renal involvement was associated with increased mortality and morbidity, with 6 out of 44 patients passing away, 4 progressing to end-stage renal disease (ESRD), and data on 2 cases' outcomes not available.
CONCLUSION
Renal manifestations in AOSD are diverse but rarely studied owing to the rarity of the disease. Studies with larger data would be essential to study further on the pathogenesis and implications.
Topics: Humans; Still's Disease, Adult-Onset; Kidney Diseases; Adult; Nephrosis, Lipoid; Kidney; Thrombotic Microangiopathies; Female; Amyloidosis; Glomerulonephritis, IGA; Glucocorticoids
PubMed: 38625385
DOI: 10.1007/s00296-024-05578-5 -
Autoimmunity Reviews Feb 2020To evaluate the prevalence, clinical presentation, serological and morphological features of, and therapeutic options for Interstitial Lung Disease (ILD) in primary...
Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sjӧgren's syndrome: A systematic review from the Italian Society of Rheumatology.
OBJECTIVE
To evaluate the prevalence, clinical presentation, serological and morphological features of, and therapeutic options for Interstitial Lung Disease (ILD) in primary Sjögren's Syndrome (pSS).
METHODS
Pubmed was searched between February 1996 and December 2018 using a combination of MESH terms related to pSS and ILD. Selected works were subjected to blind evaluation by two authors and a senior author in case of disagreement. The work followed PRISMA guidelines and was registered on PROSPERO (CRD42018118669).
RESULTS
About 20% of pSS patients have ILD, with a 5-y survival of 84% and a need for supplemental oxygen in the 11-33% range. A significant proportion of ILD patients are seronegative without sicca syndrome. ILD seems to be associated with higher levels of Lactic Dehydrogenases and positivity for Anti-Ro52k. The prevalent pattern in High Resolution Computed Tomography is Nonspecific Interstitial Pneumonia (NSIP), but all other patterns can be present. No difference in mortality was found between patients with NSIP and Usual Interstitial Pneumonia patterns. Amyloidosis and primary lung lymphoma can be observed in about 10% of pSS patients.
CONCLUSION
The recognition of pSS underlying an ILD can be challenging in seronegative patients with no or mild sicca symptoms. A complete diagnostic assessment, including minor salivary glands and, in some cases, lung biopsy, should be performed on all patients at risk. A better recognition of the clinical or serological markers of ILD progression in these patients is warranted to drive the physicians to an early diagnosis and an effective treatment.
Topics: Humans; Italy; Lung Diseases, Interstitial; Prognosis; Rheumatology; Sjogren's Syndrome; Societies, Medical
PubMed: 31843713
DOI: 10.1016/j.autrev.2019.102447 -
World Neurosurgery Jun 2020Management of cerebral amyloidomas has lacked consensus owing to their rarity. We present a case and review 39 reported cases of amyloidomas in the literature, comparing...
BACKGROUND
Management of cerebral amyloidomas has lacked consensus owing to their rarity. We present a case and review 39 reported cases of amyloidomas in the literature, comparing their initial presentation, imaging characteristics, treatment, and progression.
CASE DESCRIPTION
A 56-year-old woman presented with a history of intractable headaches and progressive speech difficulty. Imaging demonstrated a 1.7 × 2.6 cm left frontal subcortical hemorrhagic mass with an associated developmental venous anomaly, characteristics initially suggestive of a cavernous malformation. Diffusion tractography imaging was employed to avoid interference with the intact arcuate fasciculus and corticospinal tracts to minimize neurologic deficits.
CONCLUSIONS
To our knowledge, this is the first documented case of the use of diffusion tractography in preoperative planning for cerebral amyloidoma resection. Our systematic review of 39 reported cases of amyloidomas in the literature provided insight regarding how these rare lesions have manifested and progressed and further understanding of current theories regarding their etiology and pathophysiology. Cerebral amyloidomas are rare, localized, proteinaceous aggregates with variable presentation and prognosis and no apparent relationship to systemic amyloidosis. Biopsy may be useful in determination of progression. Diffusion tractography is a valuable tool for minimizing complications associated with resection.
Topics: Amyloidosis; Brain Diseases; Female; Frontal Lobe; Humans; Middle Aged; Minimally Invasive Surgical Procedures; Neurosurgical Procedures
PubMed: 32084617
DOI: 10.1016/j.wneu.2020.02.072 -
Neurology Dec 2019To assess the association of cortical superficial siderosis (cSS) presence and extent with future bleeding risk in cerebral amyloid angiopathy (CAA). (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To assess the association of cortical superficial siderosis (cSS) presence and extent with future bleeding risk in cerebral amyloid angiopathy (CAA).
METHODS
This was a meta-analysis of clinical cohorts of symptomatic patients with CAA who had T2*-MRI at baseline and clinical follow-up for future intracerebral hemorrhage (ICH). We pooled data in a 2-stage meta-analysis using random effects models. Covariate-adjusted hazard ratios (adjHR) from multivariable Cox proportional hazard models were used.
RESULTS
We included data from 6 eligible studies (n = 1,239). cSS pooled prevalence was 34% (95% confidence interval [CI] 26%-41%; 87.94%; < 0.001): focal cSS prevalence was 14% (95% CI 12%-16%; 6.75%; = 0.37), and disseminated cSS prevalence was 20% (95% CI 13%-26%; 90.39%; < 0.001). During a mean follow-up of 3.1 years (range 1-4 years), 162/1,239 patients experienced a symptomatic ICH-pooled incidence rate 6.9% per year (95% CI 3.9%-9.8% per year; 83%; < 0.001). ICH incidence rates per year according to cSS status were 3.9% (95% CI 1.7%-6.1%; 70%; = 0.018) for patients without cSS, 11.1% (95% CI 7%-15.2%; 56.8%; = 0.074) for cSS presence, 9.1% (95% CI 5.5%-12.8%; 0%; = 0.994) for focal cSS, and 12.5% (95% CI 5.3%-19.7%; 73.2%; = 0.011) for disseminated cSS. In adjusted pooled analysis, any cSS presence was independently associated with increased future ICH risk (adjHR 2.14; 95% CI 1.19-3.85; < 0.0001). Focal cSS was linked with ICH risk (adjHR 2.11; 95% CI 1.31-2.41; = 0.002), while disseminated cSS conferred the strongest bleeding risk (adjHR 4.28; 95% CI 2.91-6.30; < 0.0001).
CONCLUSION
In patients with CAA, cSS presence and extent are the most important MRI prognostic risk factors for future ICH, likely useful in treatment planning.
CLASSIFICATION OF EVIDENCE
This study provides Class III evidence that in symptomatic CAA survivors with baseline T2*-MRI, cSS (particularly if disseminated, i.e., affecting >3 sulci) increases the risk of future ICH.
Topics: Aged; Brain Diseases; Cerebral Amyloid Angiopathy; Cerebral Cortex; Cerebral Hemorrhage; Female; Hemosiderosis; Humans; Male; Middle Aged; Prevalence; Risk Factors
PubMed: 31732564
DOI: 10.1212/WNL.0000000000008590 -
International Journal of Stroke :... Oct 2019We aimed to investigate cortical superficial siderosis as an MRI predictor of lobar intracerebral hemorrhage (ICH) recurrence risk in cerebral amyloid angiopathy (CAA),... (Meta-Analysis)
Meta-Analysis
BACKGROUND
We aimed to investigate cortical superficial siderosis as an MRI predictor of lobar intracerebral hemorrhage (ICH) recurrence risk in cerebral amyloid angiopathy (CAA), in a large prospective MRI cohort and a systematic review.
METHODS
We analyzed a single-center MRI prospective cohort of consecutive CAA-related ICH survivors. Using Kaplan-Meier and Cox regression analyses, we investigated cortical superficial siderosis and ICH risk, adjusting for known confounders. We pooled data with eligible published cohorts in a two-stage meta-analysis using random effects models. Covariate-adjusted hazard rations (adj-HR) from pre-specified multivariable Cox proportional hazard models were used.
RESULTS
The cohort included 240 CAA-ICH survivors (cortical superficial siderosis prevalence: 36%). During a median follow-up of 2.6 years (IQR: 0.9-5.1 years) recurrent ICH occurred in 58 patients (24%). In prespecified multivariable Cox regression models, cortical superficial siderosis presence and disseminated cortical superficial siderosis were independent predictors of increased symptomatic ICH risk at follow-up (HR: 2.26; 95% CI: 1.31-3.87, p = 0.003 and HR: 3.59; 95% CI: 1.96-6.57, p < 0.0001, respectively). Three cohorts including 443 CAA-ICH patients in total were eligible for meta-analysis. During a mean follow-up of 2.5 years (range: 2-3 years) 92 patients experienced recurrent ICH (pooled risk ratio: 6.9% per year, 95% CI: 4.2%-9.7% per year). In adjusted pooled analysis, any cortical superficial siderosis and disseminated cortical superficial siderosis were the only independent predictors associated with increased lobar ICH recurrence risk (adj-HR: 2.4; 95% CI: 1.5-3.7; p < 0.0001, and adj-HR: 4.4; 95% CI: 2-9.9; p < 0.0001, respectively).
CONCLUSIONS
In CAA-ICH patients, cortical superficial siderosis presence and extent are the most important MRI prognostic risk factors for lobar ICH recurrence. These results can help guide clinical decision making in patients with CAA.
Topics: Aged; Cerebral Amyloid Angiopathy; Cerebral Hemorrhage; Cohort Studies; Female; Humans; Magnetic Resonance Imaging; Male; Neuroimaging; Recurrence; Risk Factors
PubMed: 30785378
DOI: 10.1177/1747493019830065