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Sarcoidosis; Cardiac Sarcoidosis; Granuloma; Inflammation; Imaging; MRI
Background: Cardiac sarcoidosis (CS) is an underdiagnosed and life-threatening condition. Histopathological diagnosis is difficult due to the risks and variable diagnostic yield of endomyocardial biopsy.
Objectives: To compare three diagnostic criteria for the diagnosis of CS before initiating immunosuppressive therapy.
Methods: A retrospective review of the Sarcoidosis database (375 patients) was performed to identify patients with CS. Demographic and clinical details were retrieved. We applied the available diagnostic criteria for the diagnosis of CS: The World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG), Heart Rhythm Society (HRS), and Japanese Ministry of Health and Welfare (JMHW) criteria.
Results: Out of 375 patients, 15 (4%) were identified with CS. The mean age was 42.8 years and 53% were female. The most common symptoms were breathlessness, palpitation, and fatigue in 80%, 53.3%, and 46.6% patients respectively. Tuberculin positivity (≥ 10mm induration) was seen in 26.6%. 80% and 53.3% of the patients had abnormal ECG and 2D-echocardiography findings, respectively. Six patients had a history of Ventricular tachycardia (40%). LV Ejection fraction was reduced in 11 subjects (73.3%). Cardiac-MRI showed late gadolinium enhancement in 53.3%. A definitive histopathological diagnosis was established in 86.6% (13/15) patients. Of the 15, all satisfied JMHW criteria and WASOG criteria (12 (80%) at least probable category, 3 (20%) possible CS), and 13 (86.6%) met HRS criteria for a diagnosis of CS.
Histopathological diagnosis can be established in a majority of patients with CS in tuberculosis endemic settings. JMHW and WASOG criteria perform equally well in TB endemic settings.
2. Fahim A, Mann JS. Pulmonary sarcoidosis: Diagnostic and treatment update. Expert Review of Respiratory Medicine. 2014;8(4):493–501.
3. Pattnaik B, Sryma PB, Mittal S, Agrawal A, Guleria R, Madan K. MicroRNAs in pulmonary sarcoidosis: A systematic review. Respiratory Investigation. 2020;(xxxx).
4. Kopriva P, Griva M, Tüdös Z. Management of cardiac sarcoidosis – A practical guide. Cor et Vasa. 2018;60(2):e155–64.
5. Sharma SK, Soneja M, Sharma A, Sharma MC, Hari S. Rare manifestations of sarcoidosis in modern era of new diagnostic tools. Indian Journal of Medical Research. 2012;135(5):621–9.
6. Gupta SK, Gupta S. Sarcoidosis in India: a review of 125 biopsy-proven cases from eastern India. Sarcoidosis. 1990 Mar;7(1):43–9.
7. Birnie DH, Nery PB, Ha AC, Beanlands RSB. Cardiac Sarcoidosis. Journal of the American College of Cardiology. 2016;68(4):411–21.
8. Nagai T, Kohsaka S, Okuda S, Anzai T, Asano K, Fukuda K. Incidence and prognostic significance of myocardial late gadolinium enhancement in patients with sarcoidosis without cardiac manifestation. Chest. 2014;146(4):1064–72.
9. Martusewicz-Boros MM, Boros PW, Wiatr E, Zych J, Piotrowska-Kownacka D, Roszkowski-Śliz K. Prevalence of cardiac sarcoidosis in white population: A case-control study: Proposal for a novel risk index based on commonly available tests. Medicine (United States). 2016;95(32):1–7.
10. Hulten E, Aslam S, Osborne M, Abbasi S, Bittencourt MS, Blankstein R. Cardiac sarcoidosis-state of the art review. Cardiovascular Diagnosis and Therapy. 2016;6(1):50–63.
11. Roth D, Kadoglou N, Leeflang M, Spijker R, Herkner H, Trivella M. Diagnostic accuracy of cardiac MRI, FDG-PET, and myocardial biopsy for the diagnosis of cardiac sarcoidosis: a protocol for a systematic review and meta-analysis. Diagnostic and Prognostic Research. 2020;4(1).
12. Bansal R, Parakh N, Naik N, Jagia P, Gulati G, Juneja R, et al. Acute arrhythmia or ventricular dysfunction - when is it sarcoid? Indian perspective. Journal of the Practice of Cardiovascular Sciences. 2015;1(3):252.
13. Neto MLR, Jellis CL, Joyce E, Callahan TD, Hachamovitch R, Culver DA. Update in Cardiac Sarcoidosis. Annals of the American Thoracic Society. 2019;16(11):1341–50.
14. Terasaki F, Yoshinaga K. New Guidelines for Diagnosis of Cardiac Sarcoidosis in Japan. Annals of Nuclear Cardiology. 2017;3(1):42–5.
15. Kouranos V, Hansell DM, Sharma R, Wells AU. Advances in imaging of cardiopulmonary involvement in sarcoidosis. Current Opinion in Pulmonary Medicine. 2015;21(5):538–45.
16. Mehta D, Lubitz SA, Frankel Z, Wisnivesky JP, Einstein AJ, Goldman M, et al. Cardiac involvement in patients with sarcoidosis: Diagnostic and prognostic value of outpatient testing. Chest. 2008;133(6):1426–35.
17. Birnie DH. Cardiac Sarcoidosis. Semin Respir Crit Care Med. 2020 Jun 27;s-0040-1712535.
18. Bera D, Shanthi Naidu K, Kaur Saggu D, Yalagudri S, Kishor Kadel J, Sarkar R, et al. Serum angiotensin converting enzyme, Erythrocyte sedimentation rate and high sensitive-C reactive protein levels in diagnosis of cardiac sarcoidosis- where do we stand? Indian Pacing Electrophysiol J. 2020 May 11;
19. Birnie DH, Chb MB. Cardiac Sarcoidosis. 2020;
20. Thachil A, Christopher J, Sastry BKS, Reddy KN, Tourani VK, Hassan A, et al. Monomorphic ventricular tachycardia and mediastinal adenopathy due to granulomatous infiltration in patients with preserved ventricular function. J Am Coll Cardiol. 2011 Jun 28;58(1):48–55.
21. Madan K, Mohan A, Ayub II, Jain D, Hadda V, Khilnani GC, et al. Initial Experience With Endobronchial Ultrasound-guided. 2014;21(3):208–14.
22. Madan K, Dhungana A, Mohan A, Hadda V, Jain D, Arava S, et al. Conventional Transbronchial Needle Aspiration Versus Endobronchial Ultrasound-guided Transbronchial Needle Aspiration, With or Without Rapid On-Site Evaluation, for the Diagnosis of Sarcoidosis: A Randomized Controlled Trial. J Bronchology Interv Pulmonol. 2017 Jan;24(1):48–58.
23. Cooper LT, Baughman KL, Feldman AM, Frustaci A, Jessup M, Kuhl U, et al. The role of endomyocardial biopsy in the management of cardiovascular disease: A scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology. Circulation. 2007;116(19):2216–33.
24. Ribeiro Neto ML, Jellis C, Hachamovitch R, Wimer A, Highland KB, Sahoo D, et al. Performance of diagnostic criteria in patients clinically judged to have cardiac sarcoidosis: Is it time to regroup? American Heart Journal. 2020;223(Table I):106–9.
25. Sarda Y, Neto MLR, Jellis C, Hachamovitch R, Culver DA, Medicine I. Diagnosis of Cardiac Sarcoidosis : A Cautionary Note. 2020;
26. Kaur D, Roukoz H, Shah M, Yalagudri S, Pandurangi U, Chennapragada S, et al. Impact of the inflammation on the outcomes of catheter ablation of drug-refractory ventricular tachycardia in cardiac sarcoidosis. J Cardiovasc Electrophysiol. 2020 Mar;31(3):612–20.