Diagnostik und Therapie der kardialen Sarkoidose^[*]

Zusammenfassung

Während 90 % aller Sarkoidose-Patienten eine parenchymatöse Beteiligung der Lunge aufweisen, ist die kardiale Sarkoidose (in weniger als 10 % der Fälle klinisch manifest) selten. Generell können alle Strukturen des Herzens von der Granulom-Bildung betroffen sein. Meist jedoch ist das Myokard des linken Ventrikels sowie das Reizleitungssystem beeinträchtigt. Klinisch manifestiert sich die kardiale Sarkoidose als dilatative Kardiomyopathie oder in Form von Herzrhythmusstörungen wie Vorhofflimmern und/oder ventrikulärer Tachykardien. Das Spektrum reicht von der benignen, subklinischen Manifestation als Zufallsbefund bis zur lebensbedrohlichen Komplikation. Diagnostische Schritte und therapeutische Empfehlungen zur kardialen Sarkoidose liegen im deutschsprachigen Raum bisher nicht vor. Die vorliegende Arbeit ist eine Expertenstellungnahme unter dem Schirm der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin (DGP) und der Deutschen Gesellschaft für Kardiologie – Herz- und Kreislaufforschung (DGK). Die nachstehenden Empfehlungen ersetzen jedoch nicht die ärztliche Begutachtung des individuellen Patienten und Anpassung der Diagnostik und Therapie an dessen spezifische Situation.

Abstract

Sarcoidosis is a multisystemic granulomatous disorder which affects the respiratory system in the majority of the cases. Cardiac manifestations are found in up to 10 % of the affected cohort and show a large heterogeneity based on the ethnic background. Cardiac sarcoidosis are not only found in patients with rhythmogenic heart disease such as atrial and ventricular fibrillation but also in all phenotypes of cardiomyopathies. The overall morbidity and mortality caused by cardiac sarcoidois in Germany is unclear and no large prospective international studies are published on this topic. This consensus paper on diagnostic and therapeutic algorithms in cardiac sarcoidosis is based on a current literature search and forms a expert opinion statement under the hospices of the “Deutsche Gesellschaft für Pneumologie” and “Deutsche Gesellschaft für Kardiologie”. It is the rationale of this statement to offer algorithms to facilitate clinical decision-making based on the individual case.

^* Dieses Konsensuspapier erscheint zeitgleich in den Fachzeitschriften „Pneumologie“ und „Der Kardiologe“.

• Literatur

• 1 Hiraga H, Hiroe M, Iwai K. Guideline for diagnosis of cardiac sarcoidosis: Study report on diffuse pulmonary diseases (in Japanese). Tokyo: The Japanese Ministry of Health and Welfare; 1993: 23-24
• 2 Soejima K, Yada H. The work-up and management of patients with apparent or subclinical cardiac sarcoidosis: with emphasis on the associated heart rhythm abnormalities. J Cardiovasc Eletrophysiol 2009; 20: 578-583
• 3 Pabst S, Skowasch D, Grohé C. Sarkoidose. Pneumologie 2012; 66: 96-109
• 4 Pabst S, Tuleta I, Grohé C. Kardiale Sarkoidose. Kardiologe 2008; 2: 299-311
• 5 Schulte W, Costabel U, Kirsten D. Kardiale Sarkoidose. Pneumologie 2003; 57: 752-760
• 6 Pietinalho A, Hiraga Y, Hosoda Y et al. The frequency of sarcoidosis in Finland and Hokkaido, Japan: a comparative epidemiological study. Sarcoidosis Diff Vasc 1995; 12: 61-67
• 7 Deubelbeiss U, Gemperli A, Schindler C et al. Prevalence of sarcoidosis in Switzerland is associated with environmental factors. Eur Respir J 2010; 35: 1088-1097
• 8 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; 43: 372-376
• 9 Reid JD. Sarcoidosis in coroner’s autopsies: a critical evaluation of diagnosis and prevalence from Cuyahoga County, Ohio. Sarcoidosis Vasc Diffuse Lung Dis 1998; 15: 44-51
• 10 Pollak B. Epidemiology of sarcoidosis in Canada. Acta Med Scand Suppl 1964; 425: 145
• 11 Kitaichi M. Prevalence of sarcoidosis around the world. Sarcoidosis Vasc Diffuse Lung Dis 1998; 15: 16-18
• 12 Rybicki BA, Major M, Popovich Jr J et al. Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol 1997; 145: 234-241
• 13 Roberts WC, McAllister Jr HS, Ferrans VJ et al. Sarcoidosis of the heart. A clinicopathologic study of 35 necropsy patients and review of 78 previously described necropsy patients. Am J Med 1977; 63: 86-108
• 14 Kim JS, Judson MA, Donnino R et al. Cardiac sarcoidosis. Am Heart J 2009; 157: 9-21
• 15 Youssef G, Beanlands RSB, Birnie DH et al. Cardiac sarcoidosis: applications of imaging in diagnosis and directing treatment. Heart 2011; 97: 2078-2087
• 16 Shigemitsu H, Nagai S, Sharma OP. Pulmonary hypertension and granulomatous vasculitis in sarcoidosis. Curr Opin Pulm Med 2007; 13: 434-438
• 17 Chapelon-Abric C, de Zuttere D, Duhaut P et al. Cardiac sarcoidosis: a retrospective study of 41 cases. Medicine (Baltimore) 2004; 83: 315-334
• 18 Fleming HA, Baily SM. Sarcoid heart disease. J R Coll Physicians London 1981; 15: 245-246
• 19 Matsui Y, Iwai K, Tachibana T et al. Clinicopathological study of fatal myocardial sarcoidosis. Ann New York Acad Sci 1976; 278: 455-469
• 20 Smedema JP, Snoep G, van Kroonenburgh MP et al. Cardiac involvement in patients with pulmonary sarcoidosis at two university medical centers in the Netherlands. Chest 2005; 128: 30-35
• 21 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
• 22 Pabst S, Hammerstingl C, Grau N et al. Pulmonary Arterial Hypertension in Patients with Sarcoidosis: The Pulsar Single Center Experience. Adv Exp Med Biol 2013; 755: 299-305
• 23 Iwai K, Sekiguti M, Hosoda Y et al. Racial difference in cardiac sarcoidosis incidence observed at autopsy. Sarcoidosis Diff. Vasc 1994; 11: 26-31
• 24 Judson MA, Baughman RP, Teirstein AS et al. Defining organ involvement in sarcoidosis: the ACCESS proposed instrument. ACCESS Research Group. A case control etiologic study of sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 1999; 16: 75-86
• 25 Kandolin R, Lehtonen J, Graner M et al. Diagnosing isolated cardiac sarcoidosis. J Intern Med 2011; 270: 461-468
• 26 Sulica R, Teirstein AS, Kakarla S et al. Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension. Chest 2005; 128: 1483-1489
• 27 Baughman RP, Judson MA, Teirstein A et al. Presenting characteristics as predictors of duration of treatment in sarcoidosis. QJM 2006; 99: 307-315
• 28 Handa T, Nagai S, Miki S et al. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest 2006; 129: 1246-1252
• 29 Bourbonnais JM, Samavati L. Clinical predictors of pulmonary hypertension in sarcoidosis. Eur Respir J 2008; 32: 296-302
• 30 Baughman RP, Engel PJ, Taylor L et al. Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest 2010; 138: 1078-1085
• 31 Nunes H, Humbert M, Capron F et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax 2006; 61: 68-74
• 32 Shlobin OA, Nathan SD. Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation. Eur Respir J 2012; 39: 1520-1533
• 33 Galiè N, Hoeper MM, Humbert M et al. ESC Committee for Practice Guidelines (CPG). Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009; 30: 2493-537
• 34 Gideon NM, Mannino DM. Sarcoidosis mortality in the United States 1979-1991: an analysis of multiple-cause mortality data. Am J Med 1996; 100: 423-427
• 35 Baughman RP, Winget DB, Bowen EH et al. Predicting respiratory failure in sarcoidosis patients. Sarcoidosis Vasc Diffuse Lung Dis 1997; 14: 154-158
• 36 Kandolin R, Lehtonen J, Kupari M. Cardiac sarcoidosis and giant cell myocarditis as causes of atrioventricular block in young and middle-aged adults. Circ Arrhythm Electrophysiol 2011; 4: 303-309
• 37 Patel MR, Cawley PJ, Heitner JF et al. Detection of myocardial damage in patients with sarcoidosis. Circulation 2009; 120: 1979-1977
• 38 Schuller JL, Olson MD, Zipse MM. Electrocardiographic characteristics in patients with pulmonary sarcoidosis indicating cardiac involvement. J Cardiovasc Electrophysiol 2011; 22: 1243-1248
• 39 Das MK, Kahn B, Jacob S et al. Significance of a Fragmented QRS Complex Versus a Q Wave in Patients With Coronary Artery Disease. Circulation 2006; 113: 2495-2501
• 40 Silverman KJ, Hutchins GM, Bulkley BH. Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis. Circulation 1978; 58: 1204-1211
• 41 Burstow DJ, Tajik AJ, Bailey KR et al. Two-dimensional echocardiographic findings in systemic sarcoidosis. Am J Cardiol 1989; 63: 478-482
• 42 Mehta D, Lubitz SA, Frankel Z et al. Cardiac involvement in patients with sarcoidosis: diagnostic and prognostic value of outpatient testing. Chest 2008; 133: 1426-1435
• 43 Fahy GJ, Marwick T, McCreery CJ et al. Doppler echocardiographic detection of left ventricular diastolic dysfunction in patients with pulmonary sarcoidosis. Chest 1996; 109: 62-66
• 44 Schulz-Menger J, Wassmuth R, Abdel-Aty H et al. Patterns of myocardial inflammation and scarring in sarcoidosis as assessed by cardiovascular magnetic resonance. Heart 2006; 92: 399-400
• 45 Cummings KW, Bhalla S, Javidan-Nejad C et al. A pattern-based approach to assessment of delayed enhancement in nonischemic cardiomyopathy at MR imaging. Radiographics 2009; 29: 89-103
• 46 Ichinose A, Otani H, Oikawa M et al. MRI of cardiac sarcoidosis: basal and subepicardial localization of myocardial lesions and their effect on left ventricular function. AJR Am J Roentgenol 2008; 191: 862-869
• 47 Smedema JP, Snoep G, van Kroonenburgh MP et al. Evaluation of the accuracy of gadolinium-enhanced cardiovascular magnetic resonance in the diagnosis of cardiac sarcoidosis. J Am Coll Cardiol 2005; 45: 1683-1690
• 48 Ohira H, Tsujino I, Ishimaru S et al. Myocardial imaging with 18F-fluoro-2-deoxyglucose positron emission tomography and magnetic resonance imaging in sarcoidosis. Eur J Nucl Med Mol Imaging 2008; 35: 933-941
• 49 Pellegrino D, Bonab AA, Dragotakes SC et al. Inflammation and infection: imaging properties of 18F-FDG-labeled white blood cells versus 18F-FDG. J Nucl Med 2005; 46: 1522-1530
• 50 Tellier P, Valeyre D, Nitenberg A et al. Cardiac sarcoidosis: reversion of myocardial perfusion abnormalities by dipyridamole. Eur J Nucl Med 1985; 11: 201-204
• 51 Tellier P, Paycha F, Antony I et al. Reversibility by dipyridamole of thallium-201 myocardial scan defects in patients with sarcoidosis. Am J Med 1988; 85: 189-193
• 52 Hirose Y, Ishida Y, Hayashida K et al. Myocardial involvement in patients with sarcoidosis. An analysis of 75 patients. Clin Nucl Med 1994; 9: 522-526
• 53 Mana J. Nuclear imaging. 67Gallium, 201thallium, 18F-labeled fluoro-2-deoxy-D-glucose positron emission tomography. Clin Chest Med 1997; 18: 799-811
• 54 Okayama K, Kurata C, Tawarahara K et al. Diagnostic and prognostic value of myocardial scintigraphy with thallium-201 and gallium-67 in cardiac sarcoidosis. Chest 1995; 107: 330-334
• 55 Le Guludec D, Menad F, Faraggi M et al. Myocardial sarcoidosis. Clinical value of technetium-99m sestamibi tomoscintigraphy. Chest 1994; 106: 1675-1682
• 56 Okumura W, Iwasaki T, Toyama T et al. Usefulness of fasting 18F-FDG PET in identification of cardiac sarcoidosis. J Nucl Med 2004; 45: 1989-1998
• 57 Ishimaru S, Tsujino I, Takei T et al. Focal uptake on 18F-fluoro-2-deoxyglucose positron emission tomography images indicates cardiac involvement of sarcoidosis. Eur Heart J 2005; 26: 1538-1543
• 58 Tadamura E, Yamamuro M, Kubo S et al. Effectiveness of delayed enhanced MRI for identification of cardiac sarcoidosis: comparison with radionuclide imaging. AJR Am J Roentgenol 2005; 185: 110-115
• 59 Yamagishi H, Shirai N, Takagi M et al. Identification of cardiac sarcoidosis with (13)N-NH(3)/(18)F-FDG PET. J Nucl Med 2003; 44: 1030-1036
• 60 Ardehali H, Howard DL, Hariri A et al. A positive endomyocardial biopsy result for sarcoid is associated with poor prognosis in patients with initially unexplained cardiomyopathy. Am Heart J 2005; 150: 459-463
• 61 Cooper LT, Baughman KL, Feldman AM 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. Endorsed by the Heart Failure Society of America and the Heart Failure Association of the European Society of Cardiology. J Am Coll Cardiol 2007; 50: 1914-1931
• 62 Aizer A, Stern EH, Gomes JA et al. Usefulness of programmed ventricular stimulation in predicting future arrhythmic events in patients with cardiac sarcoidosis. Am J Cardiol 2005; 96: 276-282
• 63 Mehta D, Mori N, Goldbarg S et al. Primary prevention of sudden cardiac death in silent cardiac sarcoidosis: role of programmed ventricular stimulation. Circ Arrhythm Electrophysiol 2011; 4: 43-48
• 64 Date T, Shinozaki T, Yamakawa M et al. Elevated plasma brain natriuretic peptide level in cardiac sarcoidosis patients with preserved ejection fraction. Cardiology 2007; 107: 277-280
• 65 Yasutake H, Seino Y, Kashiwagi M et al. Detection of cardiac sarcoidosis using cardiac markers and myocardial integrated backscatter. Int J Cardiol 2005; 102: 259-268
• 66 Baughman RP, Culver DA, Judson MA. A concise review of pulmonary sarcoidosis. Am J Respir Crit Care Med 2011; 183: 573-581
• 67 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; 160: 736-755
• 68 Grutters JC, van den Bosch JM. Corticosteroid treatment in sarcoidosis. Eur Respir J 2006; 28: 627-636
• 69 Kato Y, Morimoto S, Uemura A et al. Efficacy of corticosteroids in sarcoidosis presenting with atrioventricular block. Sarcoidosis Vasc Diffuse Lung Dis 2003; 20: 133-137
• 70 Chiu CZ, Nakatani S, Zhang G et al. Prevention of left ventricular remodeling by long-term corticosteroid therapy in patients with cardiac sarcoidosis. Am J Cardiol 2005; 95: 143-146
• 71 Banba K, Kusano KF, Nakamuro K et al. Relationship between arrhythmogenesis and disease activity in cardiac sarcoidosis. Heart Rhythm 2007; 4: 1292-1299
• 72 Jefic D, Joel B, Good E et al. Role of radiofrequency catheter ablation of ventricular tachycardia in cardiac sarcoidosis: report from a multicenter registry. Heart Rhyth 2009; 6: 189-195
• 73 Yodogawa K, Seino Y, Ohara T et al. Effect of Corticosteroid Therapy on Ventricular Arrhythmias in Patients with Cardiac Sarcoidosis. Ann Noninvasive Electrocardiol 2011; 16: 140-147
• 74 Epstein AE, Dimarco JP, Ellenbogen KA et al. ACC/AHA/HRS 2008 guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: executive summary. Heart Rhythm 2008; 5: 934-955
• 75 Oni AA, Hershberger RE, Norman DJ et al. Recurrence of sarcoidosis in a cardiac allograft: control with augmented corticosteroids. J Heart Lung Transplan 1992; 11: 367-369
• 76 Viles-Gonzalez JF, Pastori L, Fischer A et al. Supraventricular arrhythmias in patients with cardiac sarcoidosis prevalence, predictors, and clinical implications. Chest 2013; 4: 1085-1090
• 77 Soejima K, Yada H. The work-up and management of patients with apparent or subclinical cardiac sarcoidosis: with emphasis on the associated heart rhythm abnormalities. J Cardiovasc Electrophysiol 2009; 20: 578-588
• 78 Kato Y, Morimoto S, Uemura A et al. Efficacy of corticosteroids in sarcoidosis presenting with atrioventricular block. Sarcoidosis Vasc Diffuse Lung Dis 2003; 20: 133-137
• 79 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
• 80 Iannuzzi MC, Fontana JR. Sarcoidosis: clinical presentation, immunopathogenesis, and therapeutics. JAMA 2011; 305: 391-299
• 81 Jefic D et al. Role of radiofrequency catheter ablation of ventricular tachycardia in cardiac sarcoidosis: report from a multicenter registry. Heart Rhythm 2009; 6: 189-195