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Pubblicazioni recenti - cardiac sarcoidosis
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Identification of a novel presumed cardiac sarcoidosis category for patients at high risk of disease.
Int J Cardiol2021 Apr;():. doi: S0167-5273(21)00658-6.
Rosenbaum Andrew N, Kolluri Nikhil, Elwazir Mohamed Y, Kapa Suraj, Abou Ezzeddine Omar F, Bois John P, Chareonthaitawee Panithaya, Schmidt Tyler J, Cooper Leslie T,
Abstract
BACKGROUND:
Histologic evidence is required for a definitive diagnosis of cardiac sarcoidosis (CS) by published guidelines; however, the sporadic nature of the disease may produce false negative biopsy results, causing CS to be underdiagnosed. We sought to establish a clinical category of CS absent histologic findings.
METHODS:
Patients evaluated for CS were stratified into 3 groups: probable CS and definite CS based on Heart Rhythm Society (HRS) criteria and presumed CS, ie, patients without any histologic evidence of sarcoidosis, but with unexplained high-grade atrioventricular block or ventricular arrhythmia and findings suggestive of CS on either cardiac magnetic resonance imaging or positron emission tomography. The primary end point was hospitalization-free and overall survival at 10?years.
RESULTS:
A total of 383 patients were included in the study: 59, definite CS; 223, probable CS; and 101, presumed CS (62, isolated CS and 39, systemic CS). Compared with patients meeting HRS criteria for CS, patients with presumed CS had lower odds of New York Heart Association class III or IV symptoms (odds ratio [OR], 0.44 [95% CI, 0.23-0.83]; P?=?.01) but greater odds of previous ventricular tachycardia (OR, 2.4 [95% CI, 1.4-4.0]; P?=?.001) or history of resuscitated sudden cardiac arrest (OR, 2.9 [95% CI, 1.0-8.6]; P?=?.05). Hospitalization-free and overall survival were similar among groups (P?=?.51 and P?=?.71, respectively).
CONCLUSIONS:
Clinical categorization of patients with presumed CS identified a high-risk cohort comparable to patients with histologic evidence of disease, although caution should be exercised in reaching this diagnosis without paying due diligence to the differential diagnosis.
Copyright © 2021. Published by Elsevier B.V.
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Cardiac sarcoidosis in the donor heart without extracardiac manifestations.
BMJ Case Rep2021 Apr;14(4):. doi: e241902.
Al-Ani Mohammad, Taha Mohamad Badie, Stewart Brian D, Graves Gabrielle S, Ahmed Mustafa M, Parker Alex M, Aranda Juan M, Vilaro Juan,
Abstract
A middle-aged woman who received heart transplantation for end-stage sarcoid cardiomyopathy developed recurrent cardiac sarcoidosis in the donor heart. She presented 5 years post-transplantation with heart block and systolic dysfunction, without extracardiac involvement. Her disease was unresponsive to corticosteroids. Routine functional imaging may help identify such recurrences.
© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.
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ATS Core Curriculum 2020. Pediatric Pulmonary Medicine.
ATS Sch2020 Dec;1(4):456-475. doi: 10.34197/ats-scholar.2020-0022RE.
Gross Jane E, McCown Michael Y, Okorie Caroline, Bishay Lara C, Dy Fei J, Rettig Jordan S, Baker Christopher D, Balmes John R, Barber Andrew T, Bose Sourav K, Casey Alicia, Hawkins Stephen M M, Kass Alexandra, Keim Garrett, Mokhallati Nadine, Montgomery Gregory, Peranteau William H, Serrano Ryan, Vece Timothy J, Yehya Nadir, Boyer Debra, Hayes Margaret M,
Abstract
The American Thoracic Society Core Curriculum updates clinicians annually in adult and pediatric pulmonary disease, medical critical care, and sleep medicine, in a 3- to 4-year recurring cycle of topics. These topics will be presented at the 2020 International Conference. Below is the pediatric pulmonary medicine core, including pediatric hypoxemic respiratory failure; modalities in noninvasive management of chronic respiratory failure in childhood; surgical and nonsurgical management of congenital lung malformations; an update on smoke inhalation lung injury; an update on vaporizers, e-cigarettes, and other electronic delivery systems; pulmonary complications of sarcoidosis; pulmonary complications of congenital heart disease; and updates on the management of congenital diaphragmatic hernia.
Copyright © 2020 by the American Thoracic Society.
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Elevated IL-15 concentrations in the sarcoidosis lung is independent of granuloma burden and disease phenotypes.
Am J Physiol Lung Cell Mol Physiol2021 Apr;():. doi: 10.1152/ajplung.00575.2020.
Minasyan Maksym, Sharma Lokesh, Pivarnik Taylor, Liu Wei, Adams Taylor, Bermejo Santos, Peng Xiaohua, Liu Angela, Ishikawa Genta, Perry Carrighan, Kaminski Naftali, Gulati Mridu, Herzog Erica L, Dela Cruz Charles S, Ryu Changwan,
Abstract
Sarcoidosis is a systemic granulomatous disease predominantly affecting the lungs. The mechanisms promoting disease pathogenesis and progression are unknown, although interleukin-15 (IL-15) has been associated with the immune-mediated inflammation of sarcoidosis. Because the identification of a mechanistically-based, clinically-relevant biomarker for sarcoidosis remains elusive, we hypothesized this role for IL-15. Pulmonary sarcoidosis granuloma formation was modeled using trehalose 6,6'-dimicolate (TDM), which was administered into wild-type and three lineages of mice: those over-expressing IL-15, deficient in IL-15, and deficient in IL-15 receptor alpha. The number of granulomas per lung was counted and normalized to the wild-type. IL-15 concentrations were measured in the bronchoalveolar lavage (BAL) from healthy controls and sarcoidosis subjects in our cohort, where associations between IL-15 levels and clinical manifestations were sought. Findings were validated in another independent sarcoidosis cohort. TDM administration resulted in similar granuloma numbers across all lineages of mice. IL-15 concentrations were elevated in the BAL of both human cohorts, irrespective of disease phenotypes. In exploratory analysis, an association with obesity was observed, and various other soluble mediators were identified in the BAL of both cohorts. Although IL-15 is enriched in the sarcoidosis lung, it was independent of disease pathogenesis or clinical manifestations in our mouse model and human cohorts of sarcoidosis. An association with obesity perhaps reflects the ongoing inflammatory processes of these co-morbid conditions. Our findings showed that IL-15 is redundant for disease pathogenesis and clinical progression of sarcoidosis.
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Detection of cardiac involvement in pulmonary sarcoidosis using high-resolution Holter electrocardiogram.
J Arrhythm2021 Apr;37(2):438-444. doi: 10.1002/joa3.12501.
Maru Yujin, Yodogawa Kenji, Tanaka Toru, Kashiwada Takeru, Iwasaki Yu-Ki, Azuma Arata, Shimizu Wataru,
Abstract
Background:
Early detection of cardiac involvement in patients with sarcoidosis is important but currently unresolved. The aim of this study was to elucidate the utility of frequency domain microvolt T-wave alternans (TWA), signal-averaged ECG (SAECG), and heart rate turbulence (HRT) using 24-hour Holter ECG for detecting cardiac involvement in patients with pulmonary sarcoidosis.
Methods:
This study consisted of consecutive 40 pulmonary sarcoidosis patients (11 males, 62 ± 13 years) who underwent 24-hour Holter monitoring with and without cardiac involvement. All patients underwent frequency domain TWA, SAECG, and HRT using 24-hour Holter monitoring. Patients with atrial fibrillation pacing or wide QRS electrocardiogram were excluded.
Results:
After 14 patients were excluded, a total of 26 patients (six males, 59 ± 14 years) were evaluated. Seven patients had cardiac involvement (cardiac sarcoidosis [CS] group). On the Holter SAECG, duration of low-amplitude signals <40 ?V in the terminal filtered QRS complex (LAS40) was significantly higher, and root mean square voltage of the terminal 40 ms of the filtered QRS complex (RMS40) was significantly lower in the CS group compared with the non-CS group (LAS40: 61.4 ± 35.9 vs 37.6 ± 9.2 ms; = .018, RMS40: 11.4 ± 10.3 vs 23.6 ± 13.2 ms; = .023). Prevalence of positive late potential (LP) was also significantly higher in the CS group than that in the non-CS group (85.7% vs 31.5%; = .026). The sensitivity, specificity, positive, and negative predictive values of LP for identifying patients with cardiac involvement were 85.7%, 68.4%, 50.0%, and 92.8%, respectively.
Conclusion:
Holter SAECG may be useful for detecting cardiac involvement in patients with pulmonary sarcoidosis.
© 2021 The Authors. Journal of Arrhythmia published by John Wiley & Sons Australia, Ltd on behalf of the Japanese Heart Rhythm Society.
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Pulmonary sarcoidosis: from clinical features to pathology- narrative review.
Ann Palliat Med2021 Mar;10(3):3438-3444. doi: 10.21037/apm-21-344.
Li Yun, Liang Zhixin, Zheng Yiqiong, Qiao Juan, Wang Ping,
Abstract
Sarcoidosis is a multisystem disease with unknown causes. The prevalence of sarcoidosis that occurs worldwide is highly variable. It is pathologically characterized by the formation of non-necrotizing epithelioid cell granuloma, mainly affecting the respiratory tract and involving extrapulmonary organs including the skin, heart, extrathoracic lymph nodes, central nerves and eyes. A correct diagnosis of sarcoidosis depends on the clinical symptoms, imaging observations, and characteristic histopathological findings. This study aims to review the recent contributions of pulmonary sarcoidosis. It is based on a search of a published article and review on pulmonary sarcoidosis. PubMed, Embase and CNKI were searched for latest developments from 1977. The most common clinical symptoms of sarcoidosis include dry cough, dyspnea, and chest discomfort. The imaging manifestations of sarcoidosis can be divided into typical and atypical findings. Lymphadenopathy is a typical imaging manifestation of sarcoidosis. Sarcoidosis is also a highly variable multisystem disease with an unpredictable clinical course. Clinically encountered cases cover a wide range from asymptomatic patients with incidental findings in radiographic images to chronic progressive diseases. Corticosteroids are the most widely recommended as the first-line treatment for symptomatic or organ-threatening disease in sarcoidosis, but they are associated with substantial toxic effects. Meanwhile, for sarcoidosis, the follow-up is also an important part of the diagnosis and treatment. Pulmonary sarcoidosis needs further recognition. In this paper, the clinical features, imaging observations, pathology, and treatment modalities for pulmonary sarcoidosis are presented and discussed vis-à-vis the current dilemma in diagnosis and therapy.
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Early onset bioprosthetic tricuspid valve stenosis in a case with cardiac sarcoidosis: Pathological findings based on autopsy.
J Cardiol Cases2021 Apr;23(4):173-176. doi: 10.1016/j.jccase.2021.01.010.
Hirata Kazuhito, Tamaki Yusuke, Yakabi Chiaki, Ishiyama Taku, Takahashi Takanori, Wake Minoru, Tengan Toshiho, Mototake Hidemitsu,
Abstract
A 60-year-old woman with cardiac sarcoidosis developed recurrent and refractory right heart failure 26 months after tricuspid valve replacement. Echocardiography revealed thickened and immobile cusp with increased diastolic tricuspid gradient of 8-10 mmHg, consistent with bioprosthetic tricuspid stenosis (TS). Prolonged intravenous injection of dobutamine and carperitide, with intermittent intravenous furosemide, was necessary at multiple times. Despite treatment, the patient died of refractory right heart failure. The explanted tricuspid bioprosthesis on autopsy revealed marked pannus formation, resulting in stiff and immobile cusps while the same mitral bioprosthesis, which was implanted on the same day, was normal. Sarcoid granulomas were not present either in tricuspid or mitral bioprostheses. Chronic valve inflammation associated with prolonged use of intravenous drugs and multiple episodes of line-associated bacteremia may have caused early onset bioprosthetic TS. Learning objectives:1Early onset bioprosthetic tricuspid stenosis (TS) is rare.2Elevated jugular venous pulse and pan-diastolic rumble with the Rivero-Carvallo sign are keys to the diagnosis of TS which is confirmed using echocardiography.3Repeated episodes of bacteremia associated with prolonged infusion of intravenous drugs might have contributed to the development of early onset bioprosthetic TS.
© 2021 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
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Stone heart: An unusual case of heart failure with preserved ejection fraction due to massive myocardial calcification.
J Cardiol Cases2021 Apr;23(4):145-148. doi: 10.1016/j.jccase.2020.10.013.
Lippolis Antonio, Buzzi Maria Paola, Romano Ilaria Jane, Dadone Viola, Gentile Francesco,
Abstract
We report an unusual case of heart failure due to massive myocardial calcification related to a rare combination of idiopathic mitral annular calcification, myocardial calcification of the left ventricular septum and the inferior wall without other predisposing factors, such as previous myocardial infarction, ventricular aneurysms, myocarditis, rheumatic heart disease, tuberculosis, chronic renal failure, or systemic metabolic disease (sarcoidosis or primary hyperoxaluria). The related restrictive pattern of diastolic filling of the left ventricle could explain this unusual case of heart failure with preserved ejection fraction. < The prevalence of heart failure with preserved ejection fraction has increased with an increasing prevalence of risk factors. For patients presenting with heart failure and normal left ventricular (LV) ejection fraction, many causes should be excluded. We make the argument that massive myocardial calcification related to a combination of idiopathic mitral annular calcification, myocardial calcification of the LV septum and the inferior wall without other predisposing factors may serve as an uncommon mechanism of heart failure with preserved ejection fraction.>.
© 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
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Mitral valve granulomatosis: a paradoxical reaction complicating etanercept treatment in rheumatoid arthritis. A case report.
Joint Bone Spine2021 Apr;():105183. doi: S1297-319X(21)00055-5.
Ngoufack Caroline, Semerano Luca, Podglajen Isabelle, Bruneval Patrick, Meune Christophe, Valeyre Dominique, Dhote Robin, Boissier Marie-Christophe, Saidenberg-Kermanac'h Nathalie,
Abstract
BACKGROUND:
"Sarcoidosis-like" paradoxical reactions to anti-tumor necrosis factor ? (anti-TNF?) treatment have been reported. The clinical presentations are varied, most of the time, with a relatively typical picture of mediastinopulmonary involvement. More rarely, isolated granulomatous locations from various organs are described, leading to difficulties in diagnosis.
CASE PRESENTATION:
We report a granulomatous cardiac valve location complicating etanercept treatment in a 26-years-old caucasian male with rheumatoid arthritis. The patient received leflunomide and low-dose corticosteroids, then etanercept was introduced because of persistent disease activity. He had no history of tuberculosis infection or contact, chest CT scan was normal. At 3 months, he showed complete remission. After 6 months of etanercept treatment, the patient suddenly complained of headache with scotomas of the right visual field and vertigo, without fever. Cerebral MRI revealed 3 recent infarcts. Cardiac ultrasonography revealed a mobile mass on the posterior mitral leaflet. C-reactive protein level was 8 mg/L, and all analyses were negative for an infectious agent. Leflunomide and etanercept were discontinued, and antibiotic therapy was started. Mitral valve resection and plasty were performed 2 days later. Histology of the valve revealed large non-caseating epithelioid granulomas with a suppurative-like necrotic center. After ruling out infectious endocarditis, sarcoidosis, rheumatoid valvulitis or lupus-like reaction induced by anti-TNF therapy, the diagnosis of a paradoxical reaction to etanercept was finally retained. Tocilizumab monotherapy was introduced to treat RA flare, no antibiotic preventive treatment was added. After 2 years, the patient was in remission.
CONCLUSION:
This case raises for the first time the possibility of a paradoxical adverse event with an isolated granulomatous reaction on the heart valve occurring with anti-TNF treatment, namely etanercept.
Copyright © 2021. Published by Elsevier Masson SAS.
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Update in Interstitial Lung Disease 2020.
Am J Respir Crit Care Med2021 Apr;():. doi: 10.1164/rccm.202103-0559UP.
Podolanczuk Anna J, Wong Alyson W, Saito Shigeki, Lasky Joseph A, Ryerson Christopher J, Eickelberg Oliver,
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Characteristics of patients with a diagnosis of sarcoidosis: a comparison of the 2006 and 2015 versions of diagnostic criteria for sarcoidosis in Japan.
J Rural Med2021 Apr;16(2):77-82. doi: 10.2185/jrm.2020-022.
Sakamoto Noritaka, Sawahata Michiru, Yamanouchi Yoshitaka, Konno Satoshi, Shijubo Noriharu, Yamaguchi Tetsuo, Nakamura Yosikazu, Suzuki Takuji, Hagiwara Koichi, Bando Masashi,
Abstract
Histological verification of epithelioid cell granuloma is important in diagnosing sarcoidosis; tissue sampling is a worldwide requirement. In 2006, to reduce medical expenses and avoid invasive procedures, diagnostic criteria without histological verification were permitted by the Japanese government. In 2015, new diagnostic criteria, allowed clinical diagnoses based on only respiratory, ocular, and cardiac systems with at least a two-system involvement, increasing the need to sample tissue from clinically unevaluable organs in suspected sarcoidosis. This study aimed to compare the characteristics of patients who were diagnosed with sarcoidosis according to the 2006 and 2015 criteria. Using the 2015 version, we re-evaluated the characteristics of 264 patients with diagnosed or suspected sarcoidosis according to the 2006 criteria, at Jichi Medical University Hospital between 2004 and 2012 (clinical diagnosis, 84; histological diagnosis, 117; suspected sarcoidosis 63). Thirty-nine patients were diagnosed with suspected sarcoidosis due to the absence of at least a two-system involvement; two patients had insufficient laboratory data suggestive of sarcoidosis. Six patients moved from suspected sarcoidosis to a histological diagnosis because of a greater leniency in the criteria for supportive findings. The 2015 diagnostic criteria excluded patients with organ involvement without a requirement for systemic steroids from the clinical diagnosis group. A case of schwannoma, erroneously placed in the clinical diagnosis group by the 2006 criteria, was reclassified according to the 2015 criteria. The 2015 version is preferable for clinically diagnosing sarcoidosis, even without histological specimens, and provides guidance for indications for systemic treatment.
©2021 The Japanese Association of Rural Medicine.
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Sudden Cardiac Arrest in a Patient With Sarcoidosis and Familial Hypercholesterolemia.
Cureus2021 Mar;13(3):e13649. doi: 10.7759/cureus.13649.
Sugimoto Hiroshi, Takeuchi Mariko, Taniguchi Yayoi, Sato Junya,
Abstract
A 32-year-old Japanese man experienced out-of-hospital cardiac arrest. On arrival, computed tomography (CT) showed ground-glass opacity in the right lung. Emergency coronary angiography revealed triple vessel disease, then he underwent percutaneous coronary intervention. We also diagnosed him with heterozygous familial hypercholesterolemia and administered rosuvastatin and evolocumab. His clinical course was uncomplicated, and he was discharged on the 21st day of admission. Follow-up CT performed two years later revealed multiple areas of consolidation with sarcoid galaxy sign and mediastinal lymphadenopathy. We diagnosed him with pulmonary sarcoidosis by histopathological evaluation of the biopsied specimen via endobronchial ultrasound-guided transbronchial fine-needle aspiration of enlarged subcarinal lymph nodes. After we administered oral prednisolone with a gradual taper, his CT findings improved.
Copyright © 2021, Sugimoto et al.
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[Sinus node dysfunction associated with cardiac sarcoidosis: A case report].
Rev Med Interne2021 Apr;():. doi: S0248-8663(21)00057-6.
Azoulay L-D, Perrot D, Sharifzadehgan A, Mousseaux E, Marijon E, Lavergne T,
Abstract
INTRODUCTION:
Sarcoidosis is a multisystemic granulomatous disease of unknown cause occurring in young adults. Cardiac sarcoidosis patients are at increased risk for atrioventricular blocks and ventricular arrhythmias. Sinus node dysfunction is scarcely reported.
OBSERVATION:
We report a case of cardiac sarcoidosis revealed by a sinus node dysfunction and focus on cardiac and thoracic imaging to guide diagnosis.
CONCLUSION:
Sinus node dysfunction may be the first manifestation of cardiac sarcoidosis. In unexplained sinus node dysfunction in young patients, advanced cardiac imaging is a key to cardiac sarcoidosis diagnostic. Early recognition of cardiac sarcoidosis enables to start immunosuppressive treatment and discuss implantable cardioverter defibrillator implantation.
Copyright © 2021 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.
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Incidence and Predictors of Sudden Cardiac Arrest in Sarcoidosis: A Nationwide Analysis.
JACC Clin Electrophysiol2021 Mar;():. doi: S2405-500X(21)00124-9.
Narasimhan Bharat, Patel Neel, Ho Kam, Amgai Birendra, Okada David R, Bandyopadhyay Dhrubajyoti, Krittanawong Chayakrit, Wu Lingling, Bhatia Kirtipal, Shah Rushil, Correa Ashish, Mehta Davendra,
Abstract
OBJECTIVES:
This study sought to identify electrocardiographic (ECG) and clinical predictors of sudden cardiac arrest (SCA) in sarcoidosis.
BACKGROUND:
Sudden cardiac death (SCD) is the leading cause of death in cardiac sarcoidosis (CS) and may be the earliest manifestation of disease. Widespread or repeated advanced imaging is a challenging solution to this problem. ECG is an affordable and widely accessible modality that could help guide diagnostic approaches and risk stratification.
METHODS:
Data were obtained from the National Inpatient Sample (2005-2017) using International Classification of Diseases, Ninth Revision and 10th Revision, Clinical Modification. The primary outcome was to identify predictors of SCA, whereas predictors of SCA in young individuals and those with normal ventricular function served as secondary measures. Furthermore, temporal trends in sarcoidosis as well as SCA were also analyzed. Logistic regression analysis was used to calculate odds ratios, following which a multivariable regression was used to adjust for potential confounders.
RESULTS:
Electrocardiographic markers of AV node dysfunction or bundle branch block are associated with substantially increased risk of SCA in a limited proportion of patients (8.6%). This association is also observed among younger patients (<40 years) and those with normal ventricular function.
CONCLUSIONS:
ECG evidence of AV nodal dysfunction or distal conduction disease should raise suspicion for cardiac involvement in patients with sarcoidosis and are associated with increased risk of SCA. ECG markers could help identify patients who would benefit from advanced imaging. The sensitivity of ECGs is, however, limited and presence of a normal ECG does not reflect a low risk of SCA.
Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
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Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis.
Cells2021 Mar;10(4):. doi: 766.
Sève Pascal, Pacheco Yves, Durupt François, Jamilloux Yvan, Gerfaud-Valentin Mathieu, Isaac Sylvie, Boussel Loïc, Calender Alain, Androdias Géraldine, Valeyre Dominique, El Jammal Thomas,
Abstract
Sarcoidosis is a multi-system disease of unknown etiology characterized by the formation of granulomas in various organs. It affects people of all ethnic backgrounds and occurs at any time of life but is more frequent in African Americans and Scandinavians and in adults between 30 and 50 years of age. Sarcoidosis can affect any organ with a frequency varying according to ethnicity, sex and age. Intrathoracic involvement occurs in 90% of patients with symmetrical bilateral hilar adenopathy and/or diffuse lung micronodules, mainly along the lymphatic structures which are the most affected system. Among extrapulmonary manifestations, skin lesions, uveitis, liver or splenic involvement, peripheral and abdominal lymphadenopathy and peripheral arthritis are the most frequent with a prevalence of 25-50%. Finally, cardiac and neurological manifestations which can be the initial manifestation of sarcoidosis, as can be bilateral parotitis, nasosinusal or laryngeal signs, hypercalcemia and renal dysfunction, affect less than 10% of patients. The diagnosis is not standardized but is based on three major criteria: a compatible clinical and/or radiological presentation, the histological evidence of non-necrotizing granulomatous inflammation in one or more tissues and the exclusion of alternative causes of granulomatous disease. Certain clinical features are considered to be highly specific of the disease (e.g., Löfgren's syndrome, lupus pernio, Heerfordt's syndrome) and do not require histological confirmation. New diagnostic guidelines were recently published. Specific clinical criteria have been developed for the diagnosis of cardiac, neurological and ocular sarcoidosis. This article focuses on the clinical presentation and the common differentials that need to be considered when appropriate.
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Elevated Serum Amyloid a Levels Are not Specific for Sarcoidosis but Associate with a Fibrotic Pulmonary Phenotype.
Cells2021 Mar;10(3):. doi: 585.
Beijer Els, Roodenburg-Benschop Claudia, Schimmelpennink Milou C, Grutters Jan C, Meek Bob, Veltkamp Marcel,
Abstract
Elevated Serum Amyloid A (SAA) levels have been found in several inflammatory diseases, including sarcoidosis. SAA is suggested to be involved in sarcoidosis pathogenesis by involvement in granuloma formation and maintenance. We hypothesized that SAA serum levels would be higher in sarcoidosis compared to other non-infectious granulomatous and non-granulomatous diseases. SAA levels were measured in serum from sarcoidosis, Hypersensitivity pneumonitis (HP), and (eosinophilic) granulomatosis with polyangiitis ((E)GPA) patients. Idiopathic pulmonary fibrosis (IPF) patients were included as non-granulomatous disease group. SAA levels of patients with sarcoidosis (31.0 µg/mL), HP (23.4 µg/mL), (E)GPA (36.9 µg/mL), and IPF (22.1 µg/mL) were all higher than SAA levels of healthy controls (10.1 µg/mL). SAA levels did not differ between the diagnostic groups. When SAA serum levels were analyzed in sarcoidosis subgroups, fibrotic sarcoidosis patients showed higher SAA levels than sarcoidosis patients without fibrosis (47.8 µg/mL vs. 29.4 µg/mL, = 0.005). To conclude, the observation that fibrotic sarcoidosis patients have higher SAA levels, together with our finding that SAA levels were also increased in IPF patients, suggests that SAA may next to granulomatous processes also reflect the process of fibrogenesis. Further studies should clarify the exact role of SAA in fibrosis and the underlying mechanisms involved.
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Cardiac magnetic resonance imaging-negative cardiac sarcoidosis.
HeartRhythm Case Rep2021 Mar;7(3):139-143. doi: 10.1016/j.hrcr.2020.11.021.
Yung See-Yue Arthur, Ho James Chung-Man, Yeung Maximus C F, Chan Carmen, Siu Chung-Wah,
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Cardiac MRI and FDG PET in Cardiac Sarcoidosis: Competitors or Collaborators?
Radiol Cardiothorac Imaging -
Active Cardiac Sarcoidosis Imitating Cardiac Metastases.
Radiol Cardiothorac Imaging2020 Dec;2(6):e200310. doi: 10.1148/ryct.2020200310.
Yamasaki Yuzo, Yoshikawa Mayumi, Sagiyama Koji, Kamitani Takeshi,
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Utility of FDG PET and Cardiac MRI in Diagnosis and Monitoring of Immunosuppressive Treatment in Cardiac Sarcoidosis.
Radiol Cardiothorac Imaging2020 Aug;2(4):e190140. doi: 10.1148/ryct.2020190140.
Coulden Richard A, Sonnex Emer P, Abele Jonathan T, Crean Andrew M,
Abstract
Purpose:
To compare the contributions of cardiac MRI and PET in the diagnosis and management of cardiac sarcoidosis (CS), with particular reference to quantitative measures.
Materials and Methods:
This is a retrospective, observational study of 31 patients (mean age, 45.7 years) with proven extracardiac sarcoidosis and possible CS who were investigated with fluorine 18 fluorodeoxyglucose (FDG) PET/CT and cardiac MRI. Patients were treated at physicians' discretion with repeat combined imaging after an interval of 102-770 days (median, 228 days).
Results:
Significant myocardial FDG uptake was shown on visit 1 (myocardial maximum standardized uptake value [SUV] > 3.6) in 17 of 22 patients who were subsequently treated. Myocardial SUV decreased at follow-up (6.5 to 4.0; < .01) and was matched by significant decreases in FDG-avid lung and mediastinal node disease. A volumetric measure of myocardium above a threshold SUV (cardiac metabolic volume) decreased from a mean of 42.5 to a mean of 4.1 ( < .001). This was associated with significant improvement in the left ventricular ejection fraction (LVEF) (45.8 increasing to 50.9; < .031). There was no change in volume of late gadolinium enhancement at treatment. Patients who were untreated showed no change in any FDG PET or cardiac MRI parameter.
Conclusion:
Myocardial FDG uptake in patients suspected of having CS is presumed to represent active inflammation. When treated with corticosteroids, this resolved or regressed at follow-up, with an improvement in LVEF and FDG-avid thoracic disease. Patients who were untreated showed no change in any parameter. Quantification of FDG-avid myocardium using cardiac metabolic volume is proposed as a useful objective measure for assessing response to therapy.©?RSNA, 2020See also commentary by Gutberlet in this issue.
2020 by the Radiological Society of North America, Inc.
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