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Pubblicazioni recenti - cardiac sarcoidosis
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Screening Sarcoidosis Patients for Occult Disease.
Semin Respir Crit Care Med2020 Oct;41(5):741-757. doi: 10.1055/s-0040-1709496.
Judson Marc A,
Abstract
As sarcoidosis may involve any organ, sarcoidosis patients should be evaluated for occult disease. Screening for some organ involvement may not be warranted if it is unlikely to cause symptoms, organ dysfunction, or affect clinical outcome. Even organ involvement that affects clinical outcome does not necessarily require screening if early detection fails to change the patient's quality of life or prognosis. On the other hand, early detection of some forms of sarcoidosis may improve outcomes and survival. This manuscript describes the approach to screening sarcoidosis patients for previously undetected disease. Screening for sarcoidosis should commence with a meticulous medical history and physical examination. Many sarcoidosis patients present with physical signs or symptoms of sarcoidosis that have not been recognized as manifestations of the disease. Detection of sarcoidosis in these instances depends on the clinician's familiarity with the varied clinical presentations of sarcoidosis. In addition, sarcoidosis patients may present with symptoms or signs that are not related to specific organ involvement that have been described as parasarcoidosis syndromes. It is conjectured that parasarcoidosis syndromes result from systemic release of inflammatory mediators from the sarcoidosis granuloma. Certain forms of sarcoidosis may cause permanent and serious problems that can be prevented if they are detected early in the course of their disease. These include (1) ocular involvement that may lead to permanent vision impairment; (2) vitamin D dysregulation that may lead to hypercalcemia, nephrolithiasis, and permanent kidney injury; and (3) cardiac sarcoidosis that may lead to a cardiomyopathy, ventricular arrhythmias, heart block, and sudden death. Screening for these forms of organ involvement requires detailed screening approaches.
Thieme. All rights reserved.
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Sarcoidosis-Associated Pulmonary Hypertension.
Semin Respir Crit Care Med2020 Oct;41(5):659-672. doi: 10.1055/s-0040-1713615.
Huitema Marloes P, Mathijssen Harold, Mager Johannes J, Snijder Repke J, Grutters Jan C, Post Marco C,
Abstract
Pulmonary hypertension (PH) is a well-known complication of sarcoidosis, defined by a mean pulmonary artery pressure of ?25?mm Hg. Since both PH and sarcoidosis are rare diseases, data on sarcoidosis-associated PH (SAPH) is retrieved mostly from small retrospective studies. Estimated prevalence of SAPH ranges from 3% in patients referred to a tertiary center up to 79% in patients awaiting lung transplant. Most patients with SAPH show advanced parenchymal disease as the underlying mechanism. However, some patients have disproportional elevated pulmonary artery pressure, and PH can occur in sarcoidosis patients without parenchymal disease. Other mechanisms such as vascular disease, pulmonary embolisms, postcapillary PH, extrinsic compression, and other sarcoidosis-related comorbidities might contribute to SAPH. The diagnosis of PH in sarcoidosis is challenging since symptoms and signs overlap. Suspicion can be raised based on symptoms or tests, such as pulmonary function tests, laboratory findings, electrocardiography, or chest CT. PH screening mainly relies on transthoracic echocardiography. Right heart catheterization should be considered on a case-by-case basis in patients with clinical suspicion of PH, taking into account clinical consequences. Treatment options are considered on patient level in a PH expert center, and might include oxygen therapy, immunosuppressive, or PH-specific therapy. However, qualitative evidence is scarce. Furthermore, in a subset of patients, interventional therapy or eventually lung transplant can be considered. SAPH is associated with high morbidity. Mortality is higher in sarcoidosis patients with PH compared with those without PH, and increases in patients with more advanced stages of sarcoidosis and/or PH.
Thieme. All rights reserved.
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The Burden of Neurosarcoidosis: Essential Approaches to Early Diagnosis and Treatment.
Semin Respir Crit Care Med2020 Oct;41(5):641-651. doi: 10.1055/s-0040-1710576.
Voortman Mareye, Stern Barney J, Saketkoo Lesley Ann, Drent Marjolein,
Abstract
Neurosarcoidosis (NS) is an often severe, destructive manifestation with a likely under-reported prevalence of 5 to 15% of sarcoidosis cases, and in its active phase demands timely treatment intervention. Clinical signs and symptoms of NS are variable and wide-ranging, depending on anatomical involvement. Cranial nerve dysfunction, cerebrospinal parenchymal disease, aseptic meningitis, and leptomeningeal disease are the most commonly recognized manifestations. However, non-organ-specific potentially neurologically driven symptoms, such as fatigue, cognitive dysfunction, and small fiber neuropathy, appear frequently.Heterogeneous clinical presentations and absence of any single conclusive test or biomarker render NS, and sarcoidosis itself, a challenging definitive diagnosis. Clinical suspicion of NS warrants a thorough and neurologic evaluation hopefully resulting in supportive extraneural physical exam and/or tissue findings. Treatment targets the severity of the manifestation, with careful discernment of whether NS reflects active potentially reversible inflammatory granulomatous disease versus inactive postinflammatory damage whereby functional impairment is unlikely to be pharmacologically responsive. Non-organ-specific symptoms are poorly understood, challenging in deciphering reversibility and often identified too late to respond to conventional immunosuppressive/pharmacological treatment. Physical therapy, coping strategies, and stress reduction may benefit patients with all disease activity levels of NS.This publication provides an approach to screening, diagnosis, disease activity discernment, and pharmacological as well as nonpharmacological treatment interventions to reduce disability and protect health-related quality of life in NS.
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Extrapulmonary Involvement in Sarcoidosis: A Case Report.
Am J Med Case Rep2020 ;8(7):210-215.
Connelly Courtney, Hasan Abida, Chung Zachary, Mingomataj Erli, Velayudhan Vinodkumar, McFarlane Isabel M,
Abstract
Sarcoidosis is an idiopathic multisystem granulomatous disease that affects patients of all races and ethnic groups however predilection for women and African Americans is apparent. Extrapulmonary manifestations of sarcoidosis occur in up to 50% of cases. The most common sites of extrapulmonary involvement are the skin, eyes, liver, and reticuloendothelial system followed bt renal, cardiac, and neurological involvement. We present the case of a middle age man with exclusive extrapulmonary sarcoidosis affecting the renal, cardiac, hepatic, splenic and central nervous system. The patient sustained a second episode of venous thromboembolism which highlights a frequently seen complication of sarcoidosis. We discuss the proposed pathophysiology for the prothrombotic state seen in sarcoidosis and imaging modalities that can be utilized to assess extrapulmonary involvement in sarcoidosis. Lastly, sarcoidosis management is reviewed, highlighting that immunosuppressants and tumor necrosis factor inhibitors are being proposed to arrest disease progression and reduce glucocorticoid doses.
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Exercise-induced torsades de pointes as an unusual presentation of cardiac sarcoidosis: A case report and review of literature.
World J Cardiol2020 Jun;12(6):291-302. doi: 10.4330/wjc.v12.i6.291.
Ghafari Chadi, Vandergheynst Frédéric, Parent Emmanuel, Tanaka Kaoru, Carlier Stéphane,
Abstract
BACKGROUND:
Sarcoidosis is a rare multisystem disease characterized histologically by non-caseating granuloma formation in the affected organ. While cardiac sarcoidosis is found on autopsy in up to 25% of sarcoidosis cases, it is still underdiagnosed and is associated with a poor prognosis. Although the etiology of sarcoidosis remains unclear, an antigen triggered exaggerated immune response has been hypothesized. Early detection and prompt management of cardiac sarcoidosis remains pivotal.
CASE SUMMARY:
A 60-year-old female, with pulmonary sarcoidosis in remission, presented to the cardiology outpatient clinic for evaluation of weeks-long dyspnea on moderate exertion (New York Heart Association class II) that was relieved by rest. Submaximal exercise stress test showed multifocal ventricular extrasystoles, followed by a self-limiting torsades de pointes. Cardiac magnetic resonance imaging showed nondilated and normotrophic left ventricle with basoseptal and mid-septal dyskinesis. The magnetic resonance imaging-derived left ventricular ejection fraction was 45%. Delayed enhancement showed patchy transmural fibrosis of the septum and hyperenhancement of the papillary muscles, all in favor of extensive cardiac involvement of sarcoidosis. A double-chamber implantable cardiac defibrillator was implanted, and methylprednisolone (12 mg/d) and methotrexate (12.5 mg/wk) treatment was initiated. Follow-up and implantable cardiac defibrillator interrogation showed episodes of asymptomatic nonsustained ventricular tachycardia and an asymptomatic episode of nonsustained ventricular tachycardia ending by the first antitachycardia pacing run.
CONCLUSION:
Along an extensive review of the literature, this unusual case report highlights the importance of early detection of cardiac involvement of sarcoidosis, in order to avoid potential complications and increase survival.
©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
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Biomarkers in the Diagnosis and Prognosis of Sarcoidosis: Current Use and Future Prospects.
Front Immunol2020 ;11():1443. doi: 10.3389/fimmu.2020.01443.
Kraaijvanger Raisa, Janssen Bonás Montse, Vorselaars Adriane D M, Veltkamp Marcel,
Abstract
Sarcoidosis is a heterogeneous disease in terms of presentation, duration, and severity. Due to this heterogeneity, it is difficult to align treatment decisions. Biomarkers have proved to be useful for the diagnosis and prognosis of many diseases, and over the years, many biomarkers have been proposed to facilitate diagnosis, prognosis, and treatment decisions. Unfortunately, the ideal biomarker for sarcoidosis has not yet been discovered. The most commonly used biomarkers are serum and bronchoalveolar lavage biomarkers, but these lack the necessary specificity and sensitivity. In sarcoidosis, therefore, a combination of these biomarkers is often used to establish a proper diagnosis or detect possible progression. Other potential biomarkers include imaging tools and cell signaling pathways. Fluor-18-deoxyglucose positron emission tomography and high-resolution computed tomography have been proven to be more sensitive for the diagnosis and prognosis of both pulmonary and cardiac sarcoidosis than the serum biomarkers ACE and sIL-2R. There is an upcoming role for exploration of signaling pathways in sarcoidosis pathogenesis. The JAK/STAT and mTOR pathways in particular have been investigated because of their role in granuloma formation. The activation of these signaling pathways also proved to be a specific biomarker for the prognosis of sarcoidosis. Furthermore, both imaging and cell signaling biomarkers also enable patients who might benefit from a particular type of treatment to be distinguished from those who will not. In conclusion, the diagnostic and prognostic path of sarcoidosis involves many different types of existing and new biomarker. Research addressing biomarkers and disease pathology is ongoing in order to find the ideal sensitive and specific biomarker for this disease.
Copyright © 2020 Kraaijvanger, Janssen Bonás, Vorselaars and Veltkamp.
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Recurrent Fulminant Myocarditis Accompanied by Lymphoid Follicle Formation in Myocardium.
Intern Med2020 Aug;():. doi: 10.2169/internalmedicine.5268-20.
Omatsu Takashi, Hayashi Takaharu, Ichibori Yasuhiro, Makino Nobuhiko, Hirata Akio, Yasumura Yoshio, Hirayama Atsushi, Higuchi Yoshiharu,
Abstract
A 76-year-old man developed repeated fulminant myocarditis in a short period, and immunosuppressive therapy was remarkably effective. A pathologic evaluation showed that inflammatory cells had infiltrated the myocardium. Not only invasion of inflammatory cells but also the formation of lymphoid follicle was noted. Chronic myocardial inflammation was proven, but cardiac sarcoidosis was negative according to the results of various examinations. This is the first report of recurrent autoimmune myocarditis with a lymphoid follicle in the myocardium. These findings may suggest a novel pathogenesis of myocarditis.
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Myocardial T mapping using SMART Map and MOLLI mapping in asymptomatic patients with recent extracardiac sarcoidosis.
NMR Biomed2020 Aug;():e4388. doi: 10.1002/nbm.4388.
Panovský Roman, Doubková Martina, Hole?ek Tomá?, Máchal Jan, Feitová V?ra, Masárová Lucia, Opat?il Luká?, Mojica-Pisciotti Mary Luz, Kincl Vladimír,
Abstract
INTRODUCTION:
Sarcoidosis is a systemic granulomatous disease affecting in particular the respiratory tract. Cardiac magnetic resonance (CMR), including a measurement of T relaxation time, could potentially detect early stadia of sarcoidosis of the heart. The study aims to assess T mapping in the detection of early cardiac involvement in asymptomatic patients with sarcoidosis.
METHODS:
One hundred and twenty patients with extracardiac sarcoidosis and without any heart disease history were included. One hundred and thirteen of them underwent a CMR examination. The mean time from the diagnosis of sarcoidosis was 0.8 (0.2-3.3) years. Cine images for the assessment of left ventricular (LV) functional parameters and pre- and post-contrast saturation method using adaptive recovery times for cardiac T mapping (SMART Map) and modified Look-Locker inversion recovery (MOLLI) images were acquired for the assessment of native T relaxation time and extracellular volume (ECV). The measured parameters were compared between sarcoidosis patients and 22 controls.
RESULTS:
The sarcoidosis patients had normal global and regional systolic LV function-LV ejection fraction 65 ± 5% versus 66 ± 7% (p NS). The mean native T relaxation times were not prolonged-1465 ± 93 ms versus 1480 ± 88 ms (p NS) measured by SMART Map and 1317 ± 60 ms versus 1313 ± 83 ms (p NS) measured using a MOLLI sequence. Similarly, the mean ECV values did not increase-16.9 ± 3.9% versus 17.9 ± 3.7% (p NS) measured by SMART Map and 30.9 ± 2.9% versus 31.6 ± 8.3% (p NS) measured using a MOLLI sequence.
CONCLUSION:
Myocardial native T relaxation times were not prolonged and ECV was not increased in asymptomatic patients with extracardiac sarcoidosis.
© 2020 John Wiley & Sons, Ltd.
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Features and clinical impact of extra-cardiac lesions with F-fluorodeoxyglucose positron emission tomography in patients with suspected cardiac sarcoidosis.
Int J Cardiol Heart Vasc2020 Oct;30():100587. doi: 10.1016/j.ijcha.2020.100587.
Higashi Haruhiko, Inaba Shinji, Iio Chiharuko, Inoue Katsuji, Ogimoto Akiyoshi, Miyagawa Masao, Mochizuki Teruhito, Ikeda Shuntaro, Yamaguchi Osamu,
Abstract
Background:
Sarcoidosis is a systemic inflammatory disorder and can often affect any other organs beyond the heart. Whole-body F-fluorodeoxyglucose positron emission tomography (FDG-PET) is used to detect not only cardiac but also extra-cardiac involvement of sarcoidosis. However, the features and clinical impact of extra-cardiac lesions have not yet been fully elucidated. Therefore, this study aimed to clarify these using FDG-PET.
Methods and results:
We enrolled 120 consecutive patients with abnormal findings clinically suggesting cardiac sarcoidosis who underwent whole-body FDG-PET. In this study, a patient with suspected cardiac sarcoidosis was defined as one having both clinically suspected findings and FDG-PET positive cardiac uptake. Subsequently, a total of 36 patients with suspected cardiac sarcoidosis were found and analyzed. Extra-cardiac involvement was detected in 35 lesions of 14 patients (39% per patient). In particular, the extra-cardiac lesions were widely distributed throughout the body, and mediastinal/hilar lymph node involvement was most commonly observed. In most of the patients (93% per patient, 13/14), the extra-cardiac lesions were localized in the regions that were considered more accessible with less risk of complication compared with endomyocardial biopsy (EMB). Based on the FDG-PET findings, 8 patients underwent extra-cardiac biopsy without complication, and its diagnostic sensitivity for histological sarcoidosis was high (75%, 6/8). Moreover, FDG-PET-guided extra-cardiac biopsy could confirm histological sarcoidosis in 4 lesions that EMB failed to prove.
Conclusions:
Extra-cardiac involvement in patients with suspected cardiac sarcoidosis was relatively high. FDG-PET-guided extra-cardiac biopsy may be safe and useful for the imaging based diagnosis of cardiac sarcoidosis.
© 2020 The Authors.
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Lower left ventricular ejection fraction and higher serum angiotensin-converting enzyme activity are associated with histopathological diagnosis by endomyocardial biopsy in patients with cardiac sarcoidosis.
Int J Cardiol2020 Jul;():. doi: S0167-5273(20)33482-3.
Komoriyama Hirokazu, Omote Kazunori, Nagai Toshiyuki, Kato Yoshiya, Nagano Nobutaka, Koyanagawa Kazuhiro, Kamiya Kiwamu, Konishi Takao, Sato Takuma, Kobayashi Yuta, Tsujinaga Shingo, Iwano Hiroyuki, Kusano Kengo, Yasuda Satoshi, Ogawa Hisao, Ishibashi-Ueda Hatsue, Anzai Toshihisa,
Abstract
BACKGROUND:
The histopathological diagnosis of cardiac sarcoidosis (CS) is challenging because of sampling error in endomyocardial biopsy (EMB) and the determinants of positive EMB are unclear. Reduced left ventricular ejection fraction (LVEF) is a simple parameter of the extent of myocardial damage, and higher serum angiotensin-converting enzyme (ACE) activity would indicate the spread of disease activity in CS patients. Thus, we sought to examine whether these parameters are related to the histopathological diagnosis of CS by EMB.
METHODS:
A total of 94 consecutive clinically diagnosed CS patients between August 1986 and March 2019 who were admitted to two academic hospitals were examined. We determined EMB as positive if non-caseating epithelioid granulomas were confirmed in the myocardial tissue. Patients were divided into two groups according to positive (n?=?37) and negative (n?=?57) EMB. We assessed the relationship between LVEF, serum ACE activity and positive EMB.
RESULTS:
Multivariable analysis revealed that both LVEF and serum ACE were independently associated with positive EMB (OR 0.83, 95% CI 0.70-0.99; OR 1.39, 95% CI 1.02-1.90, respectively). Moreover, patients with both lower LVEF (<37%, median) and higher ACE activity (?13.5?IU/L, median) had the highest frequency of positive EMB (p?=?.003). The combination of lower LVEF and higher serum ACE showed better specificity (91.2%) and positive predictive value (73.7%) than either LVEF or serum ACE alone for positive EMB.
CONCLUSIONS:
Lower LVEF and higher serum ACE activity were associated with positive EMB, suggesting that these parameters might be useful for predicting positive EMB in CS patients.
Copyright © 2020. Published by Elsevier B.V.
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Indirect pathological indicators for cardiac sarcoidosis on endomyocardial biopsy.
J Pathol Transl Med2020 Jul;():. doi: 10.4132/jptm.2020.06.10.
Cha Myung-Jin, Seo Jeong-Wook, Oh Seil, Park Eun-Ah, Lee Sang-Han, Kim Moon Young, Park Jae-Young,
Abstract
Background:
The definitive pathologic diagnosis of cardiac sarcoidosis requires observation of a granuloma in the myocardial tissue. It is common, however, to receive a "negative" report for a clinically probable case. We would like to advise pathologists and clinicians on how to interpret "negative" biopsies.
Methods:
Our study samples were 27 endomyocardial biopsies from 25 patients, three cardiac transplantation and an autopsied heart with suspected cardiac sarcoidosis. Pathologic, radiologic, and clinical features were compared.
Results:
The presence of micro-granulomas or increased histiocytic infiltration was always (6/6 or 100%) associated with fatty infiltration and confluent fibrosis, and they showed radiological features of sarcoidosis. Three of five cases (60%) with fatty change and confluent fibrosis were probable for cardiac sarcoidosis on radiology. When either confluent fibrosis or fatty change was present, one-third (3/9) were radiologically probable for cardiac sarcoidosis. We interpreted cases with micro-granuloma as positive for cardiac sarcoidosis (five of 25, 20%). Cases with both confluent fibrosis and fatty change were interpreted as probable for cardiac sarcoidosis (seven of 25, 28%). Another 13 cases, including eight cases with either confluent fibrosis or fatty change, were interpreted as low probability based on endomyocardial biopsy.
Conclusion:
The presence of micro-granuloma could be an evidence for positive diagnosis of cardiac sarcoidosis. Presence of both confluent fibrosis and fatty change is necessary for probable cardiac sarcoidosis in the absence of granuloma. Either of confluent fibrosis or fatty change may be an indirect pathological evidence but they are interpreted as nonspecific findings.
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Non-specific orbital inflammation: Current understanding and unmet needs.
Prog Retin Eye Res2020 Jul;():100885. doi: S1350-9462(20)30057-4.
Lee Min Joung, Planck Stephen R, Choi Dongseok, Harrington Christina A, Wilson David J, Dailey Roger A, Ng John D, Steele Eric A, Hamilton Bronwyn E, Khwarg Sang In, Rosenbaum James T,
Abstract
Non-specific orbital inflammation (NSOI) is a noninfectious inflammatory condition of the orbit. Although it is generally considered the most common diagnosis derived from an orbital biopsy, it is a diagnosis of exclusion, meaning that the diagnosis requires exclusion of a systemic process or another identifiable etiology of orbital inflammation. The clinical diagnosis of NSOI is ill-defined, but it is typically characterized by acute orbital signs and symptoms, including pain, proptosis, periorbital edema, chemosis, diplopia, and less commonly visual disturbance. NSOI poses a diagnostic and therapeutic challenge: The clinical presentations and histological findings are heterogeneous, and there are no specific diagnostic criteria or treatment guidelines. The etiology and pathogenesis of NSOI are poorly understood. Here we recapitulate our current clinical understanding of NSOI, with an emphasis on the most recent findings on clinical characteristics, imaging findings, and treatment outcomes. Furthermore, gene expression profiling of NSOI and its implications are presented and discussed.
Copyright © 2020. Published by Elsevier Ltd.
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Treatment with corticosteroids is associated with an increase in ventricular arrhythmia burden in patients with clinically manifest cardiac sarcoidosis: Insights from implantable cardioverter-defibrillator diagnostics.
J Cardiovasc Electrophysiol2020 Jul;():. doi: 10.1111/jce.14689.
Medor Maria C, Spence Stewart, Nery Pablo B, Beanlands Rob, Promislow Steven, Juneau Daniel, de Kemp Rob, Ha Andrew C, Rivard Lena, Gula Lorne, Birnie David H,
Abstract
INTRODUCTION:
We sought to explore the relationship between ventricular tachycardia (VT) and premature ventricular complex (PVC) burden (from implantable cardioverter-defibrillator diagnostics), before and during corticosteroid use in patients with newly diagnosed clinically manifest cardiac sarcoidosis (CS).
METHODS:
A single-centre, prospective cohort study was performed in consecutive patients who met all of the following criteria: (1) presentation with clinically manifest CS, (2) abnormal myocardial fluoro-deoxyglucose (FDG) uptake on positron emission tomography scan, (3) plan for implantation with implantable cardioverter-defibrillator device that reports accurate PVC count, (4) plan to initiate corticosteroids after the device healing period. Data were collected during each device interrogation visit for all patients in the study. For each inter-visit period the total number of episodes of VT-sustained and nonsustained, and the number of PVCs was obtained. Each inter-visit period was classified into one of the following three periods: (1) New diagnosis of treatment-naive active disease without corticosteroids during the period. (2) Known treatment-naive active disease with corticosteroids initiated during the inter-visit period. (3) On corticosteroid therapy during the entire period.
RESULTS:
A total of 20 patients with a mean age of 59.7?±?7.7 years were recruited and 82 inter-visit periods were analyzed. All patients were corticosteroid responders based on FDG uptake. The maximum left ventricular standardized uptake value was 11.14?±?5.19 before corticosteroid initiation and 4.07?±?0.88 after (p?
CONCLUSION:
This study demonstrated, on average, a threefold increase in daily PVC count in clinically manifest CS patients during treatment with corticosteroids compared to pretreatment. There was also a significant increase in episodes of NSVT. Clinicians and patients with active manifest CS should be aware that corticosteroids are unlikely to lead to a reduction in the burdens of PVC and NSVT.
© 2020 Wiley Periodicals LLC.
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Role of Cardiac Computed Tomography for Etiology Evaluation of Newly Diagnosed Heart Failure with Reduced Ejection Fraction.
J Clin Med2020 Jul;9(7):. doi: E2270.
Im Dong Jin, Youn Jong-Chan, Lee Hye-Jeong, Nam Kyungsun, Suh Young Joo, Hong Yoo Jin, Hur Jin, Kim Young Jin, Choi Byoung Wook, Kang Seok-Min,
Abstract
Delayed-enhanced dual-energy computed tomography (DECT) can evaluate the extent and degree of myocardial fibrosis while coronary CT angiography (CCTA) is a widely accepted coronary artery evaluation method. We sought to describe the role of combined cardiac CT for the evaluation of underlying etiology in patients with newly diagnosed heart failure with reduced ejection fraction (HFrEF). Sixty-three consecutive patients (31 men, 63 ± 16 years) with newly diagnosed HFrEF were enrolled in this prospective study. Coronary artery disease and myocardial fibrosis were evaluated on CCTA and DECT, respectively, and the tentative underlying etiologies of heart failure (HF) were determined with combinations of findings from both CTs. Concordance between tentative etiologies from cardiac CT and final etiologies from clinical decisions within a 2-year follow-up was assessed. Eighteen patients were diagnosed with ischemic HF on initial cardiac CT, and the final diagnosis was not changed. Another 45 patients with nonischemic HF included tentative etiologies of dilated cardiomyopathy ( = 32, 71.1%), sarcoidosis or myocarditis ( = 8, 17.8%), amyloidosis ( = 2, 4.4%), noncompaction ( = 2, 4.4%) and arrhythmogenic right ventricular cardiomyopathy ( = 1, 2.2%). Five nonischemic HF patients showed different etiologies between initial cardiac CT and clinical decisions. The concordance between cardiac CT and clinical decisions was 92.1%. A high degree of concordance was achieved between tentative etiologies from cardiac CT and final diagnoses from clinical decisions. Combined cardiac CT is a feasible, safe and effective imaging tool for the initial evaluation of newly diagnosed HFrEF patients.
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Clinical evaluation of infiltrative cardiomyopathies resulting in heart failure with preserved ejection fraction.
Rev Cardiovasc Med2020 Jun;21(2):181-190. doi: 10.31083/j.rcm.2020.02.65.
Madan Nidhi, Kalra Dinesh,
Abstract
Heart failure with preserved ejection fraction is a very common clinical problem. Its prevalence is increasing with aging of the population. A diverse group of risk factors and etiologies comprise the HFpEF syndrome. No specific therapies have been shown to improve survival for the vast majority of HFpEF cases. Restrictive cardiomyopathies account for a significant portion of HFpEF patients and are characterized by diastolic dysfunction due to infiltration of the myocardium or ventricular hypertrophy. Many of these restrictive diseases occur in the context of myocardial infiltration by other substances such as amyloid, iron or glycogen or endomyocardial fibrosis. These infiltrative diseases usually have important clues in the clinical picture and on cardiac imaging that may allow differentiation from the usual HFpEF phenotype (that is commonly seen in the older, hypertensive patient). Noninvasive diagnosis has replaced endomyocardial biopsy for most instances in the workup of these conditions. Early recognition is important to institute specific therapies and to improve prognosis. In this review, we describe 4 major infiltrative cardiomyopathies (Cardiac Amyloidosis, Sarcoidosis, Hemochromatosis and Fabry disease), and their key imaging features.
© 2020 Madan and Kalra Published by IMR press.
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The need for a holistic approach for SSc-ILD - achievements and ambiguity in a devastating disease.
Respir Res2020 Jul;21(1):197. doi: 10.1186/s12931-020-01459-0.
Hoffmann-Vold Anna-Maria, Allanore Yannick, Bendstrup Elisabeth, Bruni Cosimo, Distler Oliver, Maher Toby M, Wijsenbeek Marlies, Kreuter Michael,
Abstract
Systemic sclerosis (SSc) is a multi-organ autoimmune disease with complex interactions between immune-mediated inflammatory processes and vascular pathology leading to small vessel obliteration, promoting uncontrolled fibrosis of skin and internal organs. Interstitial lung disease (ILD) is a common but highly variable manifestation of SSc and is associated with high morbidity and mortality. Treatment approaches have focused on immunosuppressive therapies, which have shown some efficacy on lung function. Recently, a large phase 3 trial showed that treatment with nintedanib was associated with a reduction in lung function decline. None of the conducted randomized clinical trials have so far shown convincing efficacy on other outcome measures including quality of life determined by patient reported outcomes. Little evidence is available for non-pharmacological treatment and supportive care specifically for SSc-ILD patients, including pulmonary rehabilitation, supplemental oxygen, symptom relief and adequate information. Improved management of SSc-ILD patients based on a holistic approach is necessary to support patients in maintaining as much quality of life as possible throughout the disease course and to improve long-term outcomes.
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Current treatment of sarcoidosis.
Curr Opin Pulm Med2020 Sep;26(5):591-597. doi: 10.1097/MCP.0000000000000720.
Sellarés Jacobo, Francesqui Joel, Llabres Marta, Hernandez-Gonzalez Fernanda, Baughman Robert P,
Abstract
PURPOSE OF REVIEW:
Sarcoidosis is a granulomatous systemic disease of unknown cause where the lung is the most frequently affected organ. Therapeutic management of the disease is challenging as clinical presentation and prognosis are very heterogeneous. In the present review, we will summarize the main advances in sarcoidosis therapy.
RECENT FINDINGS:
Current sarcoidosis therapies are categorized in three lines: glucocorticoids (first line), immunosuppressants (second line), and biologics (third line). Recent glucocorticoid studies have reported that efficacy could be similar with high and low doses, but with an increase in side effects with higher doses. In immunosuppressants, recent publications in mycophenolate and repository corticotropin injection (RCI) have added more information in their use and efficacy. Finally, new evidence has been published in the use of antitumor necrosis factor (anti-TNF?) agents in refractory cardiac sarcoidosis and neurosarcoidosis.
SUMMARY:
Sarcoidosis therapy is constantly evolving, and new drugs have been added to the recommended treatments. However, extensive clinical trials are still necessary to optimize the current recommendations.
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Experimental models of sarcoidosis: where are we now?
Curr Opin Pulm Med2020 Sep;26(5):554-561. doi: 10.1097/MCP.0000000000000708.
Jeny Florence, Grutters Jan C,
Abstract
PURPOSE OF REVIEW:
Sarcoidosis remains a mysterious disease that presents many challenges both in pathogenetic understanding and in the management of patients. This review presents experimental models for sarcoidosis developed since 2016 and discusses their strengths and weaknesses and how they have contributed to the understanding and therapeutic approaches in this disease. In addition, future directions are proposed to overcome the limitations of current models.
RECENT FINDINGS:
New cellular models using infectious antigen as trigger, and transgenic models in mice have recently been developed to study signaling pathways potentially implicated in the pathogenesis of sarcoidosis.
SUMMARY:
No model fully reproduces sarcoidosis, but most of them generate data supporting key concepts and some open up therapeutic perspectives, like mTOR inhibition or IL-1? blocking. However, there are still many limitations to these models, largely related to the complexity of sarcoidosis, which might be overcome with new technologies, such as mathematical modeling.
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Management of Immunosuppression During Severe COVID-19 Infection in a Patient With Pulmonary and Cardiac Sarcoidosis.
J Clin Rheumatol2020 Jul;():. doi: 10.1097/RHU.0000000000001557.
Padala Sandeep A, Medepalli Vidya M, Mohammed Azeem, Vakiti Anusha, Elam Rachel, Gupta Sandeep J,
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Coronavirus Disease 2019 Suspicion: A Case Report Regarding a Male Emergency Medical Service Pilot With Newly Diagnosed Sarcoidosis.
Air Med J;39(4):296-297. doi: S1067-991X(20)30085-7.
Momenzadeh Mahmud, Shahali Hamze, Farahani Azade Amirabadi,
Abstract
A 38-year-old emergency medical service Bell 214 male pilot with a dry cough, fever, anorexia, fatigue, and sweating for the past 3 days; an oral temperature of 38°C; blood pressure of 105/65 mm Hg; heart rate of 94 beats/min; respiratory rate of 21 breaths/min; and pulse oximetry of 93% on room air was suspicious for coronavirus disease 2019. Surprisingly, reverse transcription polymerase chain reaction was negative, but bilateral hilar adenopathy was reported in his chest radiography as a new challenge. The pathologic report of the adenopathy biopsy was noncaseating sarcoid-type granulomas. Serologic tests showed a serum angiotensin-converting enzyme level of 58 nmol/mL/min. The bronchoalveolar lavage fluid CD4/CD8 ratio was 3.68. The bronchoalveolar lavage findings provided an accurate sarcoidosis diagnosis, and a high-resolution computed tomographic scan revealed stage 1 pulmonary involvement. Because of the pulmonary involvement, clinical manifestations, use of inhaled fluticasone, and need for longer and accurate follow-up and to protect against coronavirus disease 2019, he has been temporarily suspended until the final assignment.
Copyright © 2020. Published by Elsevier Inc.
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