Cardiac Sarcoidosis

Özet

Cardiac sarcoidosis (CS) is a rare but serious manifestation of systemic sarcoidosis that can lead to significant morbidity and mortality. While some patients present with conduction abnormalities, arrhythmias, or heart failure, others remain asymptomatic, making early detection difficult. Silent progression and the lack of disease-specific symptoms often result in delayed diagnosis, increasing the risk of sudden cardiac death.
Diagnosing CS remains a challenge due to its patchy myocardial involvement, which limits the sensitivity of endomyocardial biopsy. Cardiac MRI and PET-CT are valuable imaging modalities that improve diagnostic accuracy, yet they are not always definitive. A comprehensive assessment combining imaging techniques, ECG findings, and laboratory markers is often necessary to establish the diagnosis.
The early identification of CS is crucial to prevent irreversible myocardial damage and life-threatening arrhythmias. Due to its complex presentation, a multidisciplinary approach involving cardiologists, pulmonologists, radiologists, and rheumatologists is essential for optimal patient management. Improved awareness and diagnostic strategies are key to enhancing patient outcomes and reducing the burden of undiagnosed CS.

Kardiyak sarkoidoz (CS), sistemik sarkoidozun nadir ancak ciddi bir formu olup, önemli morbidite ve mortaliteye yol açabilir. Klinik seyri değişken olup, bazı hastalar iletim bozuklukları, aritmiler veya kalp yetmezliği ile başvururken, diğerleri asemptomatik kalabilir. Bu sessiz ilerleyiş ve hastalığa özgü semptomların olmaması, tanıda gecikmelere yol açarak ani kardiyak ölüm riskini artırabilir.
CS’nin teşhisi, miyokardiyal tutulumun bölgesel (parçalı) dağılımı nedeniyle zorludur ve endomiyokardiyal biyopsinin uygulanabilirliği sınırlıdır. Kardiyak MRG ve PET-CT, tanı doğruluğunu artıran önemli görüntüleme yöntemleri olsa da her zaman kesin sonuç vermeyebilir. Bu nedenle, görüntüleme teknikleri, EKG bulguları ve laboratuvar belirteçlerini içeren kapsamlı bir değerlendirme gereklidir.
CS’nin erken teşhisi, geri dönüşümsüz miyokardiyal hasarı ve yaşamı tehdit eden aritmileri önlemek için kritik öneme sahiptir. Kardiyologlar, pulmonologlar, radyologlar ve romatologları içeren multidisipliner bir yaklaşım, hastaların yönetiminde hayati rol oynar. Artan farkındalık ve gelişmiş tanı stratejileri, hasta sonuçlarını iyileştirmek ve teşhis edilmemiş CS vakalarının yükünü azaltmak açısından büyük önem taşımaktadır.

Referanslar

Sakthivel P, Bruder D. Mechanism of granuloma formation in sarcoidosis. Curr Opin Hematol. 2017 Jan;24(1):59-65. doi: 10.1097/MOH.0000000000000301. PMID: 27755127.

Hunninghake G, Costabel U. Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS), and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med. 1999 Aug;160(2):736-55. doi: 10.1164/ajrccm.160.2.ats4-99. PMID: 10430755.

Silverman KJ, Hutchins GM, Bulkley BH. Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis. Circulation. 1978 Dec;58(6):1204-11. doi: 10.1161/01.cir.58.6.1204. PMID: 709777.

Iwai K, Tachibana T, Takemura T, et al. Pathological studies on sarcoidosis autopsy. I. Epidemiological features of 320 cases in Japan. Acta Pathol Jpn. 1993 Jul-Aug;43(7-8):372-6. doi: 10.1111/j.1440-1827.1993.tb01148.x. PMID: 8372682.

Martusewicz-Boros MM, Boros PW, Wiatr E, et al. Cardiac Sarcoidosis: Is it More Common in Men? Lung. 2016 Feb;194(1):61-6. doi: 10.1007/s00408-015-9805-8. Epub 2015 Sep 28. PMID: 26411590; PMCID: PMC4740513.

Kandolin R, Lehtonen J, Airaksinen J, et al. Cardiac sarcoidosis: epidemiology, characteristics, and outcome over 25 years in a nationwide study. Circulation. 2015 Feb 17;131(7):624-32. doi: 10.1161/CIRCULATIONAHA.114.011522. Epub 2014 Dec 19. PMID: 25527698.

Berlin M, Fogdell-Hahn A, Olerup O, et al. HLA-DR predicts the prognosis in Scandinavian patients with pulmonary sarcoidosis. Am J Respir Crit Care Med. 1997 Nov;156(5):1601-5. doi: 10.1164/ajrccm.156.5.9704069. PMID: 9372682.

Ezzie ME, Crouser ED. Considering an infectious etiology of sarcoidosis. Clin Dermatol. May-Jun 2007;25(3):259-66. doi: 10.1016/j.clindermatol.2007.03.003

Barnard J, Rose C, Newman L, et al. ; ACCESS Research Group. Job and industry classifications associated with sarcoidosis in A Case-Control Etiologic Study of Sarcoidosis (ACCESS). J Occup Environ Med. 2005 Mar;47(3):226-34. doi: 10.1097/01.jom.0000155711.88781.91. PMID: 15761318.

Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007 Nov 22;357(21):2153-65. doi: 10.1056/NEJMra071714. PMID: 18032765.

Agostini C, Adami F, Semenzato G. New pathogenetic insights into the sarcoid granuloma. Curr Opin Rheumatol. 2000 Jan;12(1):71-6. doi: 10.1097/00002281-200001000-00012. PMID: 10647958.

Liu Y, Qiu L, Wang Y, et al. The Circulating Treg/Th17 Cell Ratio Is Correlated with Relapse and Treatment Response in Pulmonary Sarcoidosis Patients after Corticosteroid Withdrawal. PLoS One. 2016 Feb 4;11(2):e0148207. doi: 10.1371/journal.pone.0148207. PMID: 26845566; PMCID: PMC4742270.

Nunes H, Freynet O , Naggara N, et al.; Valeyre, D. Cardiac Sarcoidosis. Semin. Respir. Crit. Care Med. 2010, 31, 428–441. doi: 10.1055/s-0030-1262211

Viles-Gonzalez JF, Pastori L, Fischer A, et al. Supraventricular arrhythmias in patients with cardiac sarcoidosis prevalence, predictors, and clinical implications. Chest. 2013 Apr;143(4):1085-1090. doi: 10.1378/chest.11-3214. PMID: 23667912.

Gilotra NA, Griffin JM, Pavlovic N, et al. Sarcoidosis-Related Cardiomyopathy: Current Knowledge, Challenges, and Future Perspectives State-of-the-Art Review. J Card Fail. 2022 Jan;28(1):113-132. doi: 10.1016/j.cardfail.2021.06.016. Epub 2021 Jul 11. PMID: 34260889; PMCID: PMC8748280.

Kim JS, Judson MA, Donnino R, et al. Cardiac sarcoidosis. Am Heart J. 2009 Jan;157(1):9-21. doi: 10.1016/j.ahj.2008.09.009. Epub 2008 Nov 12. PMID: 19081391.

From AM, Maleszewski JJ, Rihal CS. Current status of endomyocardial biopsy. Mayo Clin Proc. 2011 Nov;86(11):1095-102. doi: 10.4065/mcp.2011.0296. PMID: 22033254; PMCID: PMC3203000.

Freeman AM, Curran-Everett D, Weinberger HD, et al. Predictors of cardiac sarcoidosis using commonly available cardiac studies.Am J Cardiol. 2013; 112:280–285. doi: 10.1016/j.amjcard.2013.03.027.

Padala SK, Peaslee S, Sidhu MS, et al. Impact of early initiation of corticosteroid therapy on cardiac function and rhythm in patients with cardiac sarcoidosis. Int J Cardiol. 2017 Jan 15;227:565-570. doi: 10.1016/j.ijcard.2016.10.101. Epub 2016 Nov 2. PMID: 27836297.

Fahy GJ, Marwick T, McCreery CJ et al. Doppler echocardiographic detection of left ventricular diastolic dysfunction in patients with pulmonary sarcoidosis. Chest. 1996 Jan;109(1):62-6. doi: 10.1378/chest.109.1.62. PMID: 8549220.

Lehtonen J, Uusitalo V, Pöyhönen P, et al. Cardiac sarcoidosis: phenotypes, diagnosis, treatment, and prognosis. Eur Heart J. 2023 May 1;44(17):1495-1510. doi: 10.1093/eurheartj/ehad067. PMID: 36924191.

Dweck MR, Abgral R, Trivieri MG, et al. Hybrid Magnetic Resonance Imaging and Positron Emission Tomography With Fluorodeoxyglucose to Diagnose Active Cardiac Sarcoidosis. JACC Cardiovasc Imaging. 2018 Jan;11(1):94-107. doi: 10.1016/j.jcmg.2017.02.021. Epub 2017 Jun 14. PMID: 28624396; PMCID: PMC5995315.

Cheong BY, Muthupillai R, Nemeth M, et al. The utility of delayed-enhancement magnetic resonance imaging for identifying nonischemic myocardial fibrosis in asymptomatic patients with biopsy-proven systemic sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2009 Jul;26(1):39-46. PMID: 19960787.

Youssef G, Leung E, Mylonas I, et al. The use of 18F-FDG PET in the diagnosis of cardiac sarcoidosis: a systematic review and metaanalysis including the Ontario experience. J Nucl Med. 2012 Feb;53(2):241-8. doi: 10.2967/jnumed.111.090662. Epub 2012 Jan 6. PMID: 22228794..

Vita T, Okada DR, Veillet-Chowdhury M,et al. Complementary Value of Cardiac Magnetic Resonance Imaging and Positron Emission Tomography/Computed Tomography in the Assessment of Cardiac Sarcoidosis. Circ Cardiovasc Imaging. 2018 Jan;11(1):e007030. doi: 10.1161/CIRCIMAGING.117.007030. PMID: 29335272; PMCID: PMC6381829.

Birnie D, Beanlands RSB, Nery P, et al. Cardiac Sarcoidosis multi-center randomized controlled trial (CHASM CS- RCT). Am Heart J. 2020 Feb;220:246-252. doi: 10.1016/j.ahj.2019.10.003. Epub 2019 Oct 20. PMID: 31911261; PMCID: PMC7367280.

Slart, R. H., Glaudemans, A. W., Lancellotti, P., and Writing group; Docu ment reading group; EACVI Reviewers: This document was reviewed by members of the EACVI Scientific Documents Committee for 2014- 2016 and 2016-2018. (2017) A joint procedural position statement on imaging in cardiac sarcoidosis: from the Cardiovascular and Inflammation and Infection Committees of the European Association of Nuclear Medicine, the European Association of Cardiovascular Imaging, and the American Society of Nuclear Cardiology. European Heart Journal of Cardiovascular Imaging 18, 1073-1089.

Terasaki, F., Azuma, A., Anzai, T., Ishizaka, N., Ishida, Y., Isobe, M., Inomata, T., Ishibashi-Ueda, H., Eishi, Y., Kitakaze, M., et al. (2019) JCS 2016 Guideline on diagnosis and treatment of cardiac sarcoidosis ― Digest version ―. Circulation Journal 83, 2329-2388.

Birnie, DH, Sauer, WH, Bogun, F, Cooper, JM, Culver, DA, Duvernoy, CS, et al. HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis. Heart Rhythm. (2014) 11:1305–23. doi: 10.1016/j. hrthm.2014.03.043

Judson, MA, Costabel, U, Drent, M, Wells, A, Maier, L, Koth, L, et al. The WASOG sarcoidosis organ assessment instrument: an update of a previous clinical tool. Sarcoidosis Vasc Diffuse Lung Dis. (2014) 31:19–

Bussinguer M, Danielian A, Sharma OP. Cardiac sarcoidosis: Diagnosis and management. Curr Treat Options Cardiovasc Med 2012; 14: 652–664. PMID: 22983661

Yazaki Y, Isobe M, Hiroe M, et al. Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone. Am J Cardiol 2001; 88: 1006–1010

Sadek MM, Yung D, Birnie DH, Beanlands RS, Nery PB. Corticosteroid therapy for cardiac sarcoidosis: a systematic review. Can J Cardiol. 2013;29:1034–41.

Hiramitsu S, Morimoto S, Uemura A, et al. National survey on status of steroid therapy for cardiac sarcoidosis in Japan. Sarcoidosis Vasc Diffuse Lung Dis 2005; 22: 210–213.

Baughman RP, Valeyre D, Korsten P, Mathioudakis AG, Wuyts WA, Wells A, Rottoli P, Nunes H, Lower EE, Judson MA, Israel-Biet D, Grutters JC, Drent M, Culver DA, Bonella F, Antoniou K, Martone F, Quadder B, Spitzer G, Nagavci B, Tonia T, Rigau D, Ou Baughman RP, Valeyre D, Korsten P, Mathioudakis AG, Wuyts WA, Wells A, Rottoli P, Nunes H, Lower EE, Judson MA, Israel-Biet D, Grutters JC, Drent M, Culver DA, Bonella F, Antoniou K, Martone F, Quadder B, Spitzer G, Nagavci B, Tonia T, Rigau D, Ouellette DR. ERS clinical practice guidelines on treatment of sarcoidosis. Eur Respir J. 2021 Dec 16;58(6):2004079. doi: 10.1183/13993003.04079-2020. PMID: 34140301

Nagai S, Yokomatsu T, Tanizawa K, et al. Treatment with methotrexate and low-dose corticosteroids in sarcoidosis patients with cardiac lesions. Intern Med 2014; 53: 2761. PMID: 25447669)

Barnabe C, McMeekin J, Howarth A, et al. Successful treatment of cardiac sarcoidosis with infliximab. J Rheumatol 2008; 35: 1686–1687. PMID: 18671332).

Tsutsui H, Isobe M, Ito H, et al. Japanese Circulation Society and the Japanese Heart Failure Society Joint Working Group. JCS 2017/JHFS 2017 Guideline on Diagnosis and Treatment of Acute and Chronic Heart Failure - Digest Version. Circ J. 2019 Sep 25;83(10):2084-2184. doi: 10.1253/circj.CJ-19-0342. Epub 2019 Sep 10. PMID: 31511439.

Nordenswan HK, Lehtonen J, Ekström K, et al. Outcome of Cardiac Sarcoidosis Presenting With High-Grade Atrioventricular Block. Circ Arrhythm Electrophysiol. 2018 Aug;11(8):e006145. doi: 10.1161/CIRCEP.117.006145. PMID: 30354309.

Kumar S, Barbhaiya C, Nagashima K, et al. Ventricular tachycardia in cardiac sarcoidosis: characterization of ventricular substrate and outcomes of catheter ablation. Circ Arrhythm Electrophysiol. 2015 Feb;8(1):87-93. doi: 10.1161/CIRCEP.114.002145. Epub 2014 Dec 19. PMID: 25527825.

Muser D, Santangeli P, Liang JJ, et al. Characterization of the Electroanatomic Substrate in Cardiac Sarcoidosis: Correlation With Imaging Findings of Scar and Inflammation. JACC Clin Electrophysiol. 2018 Mar;4(3):291-303. doi: 10.1016/j.jacep.2017.09.175. Epub 2017 Nov 29. PMID: 30089553.

Papageorgiou N, Providência R, Bronis K, et al. Catheter ablation for ventricular tachycardia in patients with cardiac sarcoidosis: a systematic review. Europace. 2018 Apr 1;20(4):682-691. doi: 10.1093/europace/eux077. PMID: 28444174.

Sayfalar

253-260

Gelecek

23 Haziran 2025

Lisans

Lisans