Cardiac sarcoidosis mimics cardiac amyloidosis in an elderly patient.
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ABSTRACT:
SUBMITTER: Kafil TS
PROVIDER: S-EPMC9731992 | biostudies-literature | 2022 Dec
REPOSITORIES: biostudies-literature
Publications
Cardiac sarcoidosis mimics cardiac amyloidosis in an elderly patient.
Kafil Tahir S TS Jellis Christine L CL Hoda Raza S RS Griffin Brian P BP Cremer Paul C PC
European heart journal. Case reports 20221128 12
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Project description:ObjectiveThe manifestations of sarcoidosis differ by ethnicity and region. However, the few studies that have focused on elderly sarcoidosis are only from Western countries. Therefore, we investigated elderly sarcoidosis in Japan.MethodsWe retrospectively reviewed the records of adult patients (≥18 years old) who were diagnosed with sarcoidosis from 1 April 2006 to 31 March 2020. The diagnosis was pathologically confirmed in all patients. We compared the clinical features of elderly (diagnosed at ≥65 years old) and non-elderly (diagnosed at <65 years old) patients.ResultsThirty-five (33%) of 106 patients were elderly. The elderly group had significantly more comorbidities than the non-elderly group (median [range], 1 [0-4] vs. 0 [0-5]). The biopsy site at diagnosis included significantly more extrathoracic sites in the elderly than non-elderly group (57.1% vs. 33.8%). The elderly group had significantly more muscle lesions than the non-elderly group at the time of diagnosis (11.4% vs. 1.4%) and at any time during follow-up (17.1% vs. 1.4%).ConclusionIn Japan, elderly patients with sarcoidosis might have more muscle involvement and comorbidities than younger patients. Because comorbidities might affect the prognosis of elderly sarcoidosis, further study is needed to clarify the effect of comorbidities on elderly sarcoidosis.
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Project description:PURPOSE OF REVIEW:In this state-of-the-art review, we highlight our current understanding of diagnosis, assessment, and management of cardiac sarcoidosis (CS), focusing on recently published data and expert consensus statement guidelines. RECENT FINDINGS:Academic interest in cardiac sarcoidosis research has increased over the past decade along with increased clinical awareness among clinicians. In 2014, the Heart Rhythm Society published the first expert consensus statement on diagnosing and managing arrhythmias associated with CS. Cardiac magnetic resonance has emerged as a valuable tool both for diagnosing CS and predicting risk of life-threatening ventricular arrhythmias based on burden of late gadolinium enhancement. Cardiac fluorodeoxyglucose-positron emission tomography now plays a role in diagnosis, risk stratification, and assessing response to immunosuppressive therapy. Collaborative, multidisciplinary research efforts are needed to further our understanding of this rare, complex disease. Two large multicenter prospective registries-the international Cardiac Sarcoidosis Consortium and the Canadian Cardiac Sarcoidosis Research Group-are enrolling patients to help provide insights into the natural history of the disease and current treatment strategies. Future research should focus on randomized controlled trials comparing different treatment strategies and identifying and testing novel therapeutic agents.
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Project description:ObjectiveAdvancements in diagnostics and treatment options for cardiac amyloidosis have improved patient outcomes, yet few patient education materials exist to help patients understand the disease and diagnosis process. We sought to develop and evaluate a set of plain language, patient-centered infographics describing the condition and common diagnostic tests.MethodsUsing health literacy best practices, we developed 7 infographics which were further revised based on multilevel stakeholder feedback. To evaluate the materials, we recruited 100 patients from healthcare settings in Chicago, IL; participants completed a web-assisted interview during which they were randomized 1:1 to first view either our infographics or a standard material. Participants completed a knowledge assessment on their assigned material and subsequently reported impressions of both materials.ResultsNo differences were found between study arms in knowledge. The infographics took significantly less time to read and were more highly rated by participants in terms of appearance and understandability. Over two-thirds of participants preferred the infographics to the standard.ConclusionsThe infographics created may improve the learning process about a complex condition and diagnosis process unknown to most adults.InnovationThese infographics are the first of their kind for cardiac amyloidosis and were created using health literacy best practices.
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Project description:Cardiac AL amyloidosis is a medical emergency causing rapid deterioration of cardiac function; however, it remains to be a diagnostic challenge especially when presenting with unusual symptoms and clinical findings. We present case of a 44-year-old patient with typical angina, persistently elevated troponin and normal epicardial coronary arteries. He was initially treated for myocarditis due to chest pain with troponin rise. However, CMR finding of subendocardial enhancement, increased native T1 values as well as extensive diffuse FDG uptake on PET-CT also suggested inflammatory cardiac conditions. Rapid decline in LV function and clinical deterioration led to further investigations including serum free light chains and bone marrow biopsy which confirmed systemic AL amyloidosis. Although the pathophysiology of unusual FDG PET-CT findings remains unknown, marked myocardial FDG uptake might have been caused by various features that were associated with AL amyloidosis including myocardial cell toxicity/inflammation or microvascular dysfunction. Awareness of these features specific to AL amyloidosis among physicians and description of associated cardiac FDG uptake findings has a potential to aid early diagnosis.
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Project description:Background and aimSarcoidosis is a granulomatous disease. Malignant tumors are accompanied rarely by granuloma reactions that mimic metastasis.MethodsWe present the case of a patient with possible advanced lung cancer with metastases to the mediastinal lymph nodes and bilateral ilia.ResultsIlium biopsy revealed the presence of sarcoid-like reaction. Bronchoscopy and endobronchial ultrasound revealed an adenocarcinoma in the right upper lung lobe, with a negative mediastinal lymph node. The correct staging of lung cancer was achieved through pathological examination of the surgically removed lung tissues. Two years later, the lung cancer metastasized, and the patient underwent systemic treatment.ConclusionsCoexistent sarcoid-like reaction may mimic metastatic lung cancer. A multidisciplinary approach and sequential diagnostic biopsies can prevent unnecessary surgery or inadequate treatment by distinguishing between coexistent sarcoidosis and metastatic lung cancer.
| S-EPMC11008325 | biostudies-literature
Project description:BackgroundSarcoidosis is a multi-organ granulomatous disease of unknown aetiology. Adverse outcome related with cardiac involvement, makes early diagnosis of cardiac sarcoidosis crucial.Case summaryIn a 55-year-old man presenting with recurrent pulmonary infections, computed tomography (CT) showed several enlarged mediastinal lymph nodes and no lung pathology. Subsequent mediastinoscopy revealed the diagnosis of sarcoidosis. Further screening for organ involvement showed multifocal cardiac involvement both on cardiac magnetic resonance (CMR) and 18-F-fluorodeoxyglucose-positron emission tomography-computed tomography (18F-FDG PET-CT). Because of the lack of functional deterioration and clinical symptoms, no steroid treatment was initiated and regular follow-up of cardiac abnormalities was performed by CMR. Unremarkable progression of cardiac involvement during the first 2 years of follow-up turned into a dramatic involvement after 4 years, with the increase in the number and size of lesions at late gadolinium enhancement (LGE) CMR. Late gadolinium enhancement areas matched the regions of strongly increased 18F-FDG uptake. For the first time, the patient started complaining on shortness of breath, electrocardiography showed an atrioventricular block Grade 1. Cardiac biomarkers and cardiac function were still preserved. Steroid treatment was started. Although an electrophysiology study was negative, Holter monitoring showed ventricular arrhythmia. Cardioverter-defibrillator was implanted.DiscussionThis case shows the progression of cardiac sarcoidosis on CMR in an asymptomatic untreated patient over a 4-year period, and rises the awareness of possible severe cardiac damage even in the absence of clinical signs of cardiac involvement. Combination of PET and CMR is appealing to better understand the evolution of cardiac sarcoidosis and may help in the management of such patients.
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Project description: Not available
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Project description:Transthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized cause of heart failure (HF) and mortality worldwide. Advances in non-invasive diagnosis, coupled with the development of effective treatments, have shifted ATTR-CA from a rare and untreatable disease to a relatively prevalent condition that clinicians should consider on a daily basis. Amyloid fibril formation results from age-related failure of homoeostatic mechanisms in wild-type ATTR (ATTRwt) amyloidosis (non-hereditary form) or destabilizing mutations in variant ATTR (ATTRv) amyloidosis (hereditary form). Longitudinal large-scale studies in the United States suggest an incidence of cardiac amyloidosis in the contemporary era of 17 per 100 000, which has increased from a previous estimate of 0.5 per 100 000, which was almost certainly due to misdiagnosis and underestimated. The presence and degree of cardiac involvement is the leading cause of mortality both in ATTRwt and ATTRv amyloidosis, and can be identified in up to 15% of patients hospitalized for HF with preserved ejection fraction. Associated features, such as carpal tunnel syndrome, can preceed by several years the development of symptomatic HF and may serve as early disease markers. Echocardiography and cardiac magnetic resonance raise suspicion of disease and might offer markers of treatment response at a myocardial level, such as extracellular volume quantification. Radionuclide scintigraphy with 'bone' tracers coupled with biochemical tests may differentiate ATTR from light chain amyloidosis. Therapies able to slow or halt ATTR-CA progression and increase survival are now available. In this evolving scenario, early disease recognition is paramount to derive the greatest benefit from treatment.
| S-EPMC9897687 | biostudies-literature
Project description:BackgroundInfiltrative diseases (IDs), including amyloidosis, sarcoidosis, and hemochromatosis, are characterized by abnormal cellular infiltration in multiple organs, including the heart. The prognosis of percutaneous coronary intervention (PCI) patients with underlying IDs has not been well-studied. We evaluated the prevalence of IDs in patients undergoing PCI and their association with post-PCI outcomes.MethodsThe National Inpatient Sample (NIS) 2016-2020 database was used to identify PCI patients with ICD-10 codes for a retrospective analysis. PCI patients were then divided into those with and without underlying IDs, which included amyloidosis, sarcoidosis, and hemochromatosis. Multivariable logistic regression was performed for composite post-PCI outcomes analyses.ResultsAmong 2,360,860 patients admitted to undergo PCI, 7855 patients had underlying IDs. The highest prevalence was observed for sarcoidosis (0.2%) followed by hemochromatosis (0.07%) and amyloidosis (0.04%). Underlying amyloidosis was associated with worse composite post-PCI outcomes (odds ratio [OR], 1.6; 95% CI, 1.1-2.44; P = .02), including higher in-hospital mortality (OR, 1.9; 95% CI, 1.1-3.4; P = .04), higher risk of intra/post-PCI stroke (OR, 4.0; 95% CI, 1.1-16.0; P = .04), but not major bleeding (OR, 2.2; 95% CI, 0.97-5.03; P = .058). In contrast, underlying sarcoidosis (OR, 1.1; 95% CI, 0.87-1.41; P = .4), and hemochromatosis (OR, 1.18; 95% CI, 0.77-1.8; P = .44) were not associated with composite post-PCI outcomes. Amyloidosis patients undergoing PCI also had higher hospitalization charges ($212,123 vs $141,137; P = .03) and longer length of stay (8.2 vs 3.9 days; P < .001).ConclusionsUnderlying amyloidosis was associated with worse post-PCI outcomes including higher in-hospital mortality, intra/post-PCI stroke, and socioeconomic burden. A multidisciplinary approach and future studies are needed to investigate the screening and treatment strategies in these patients.
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