Zusammenfassung
Während 90 % aller Sarkoidosepatienten 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 Granulombildung betroffen sein. Meist jedoch sind 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 ventrikulären 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 less than 10 % of the affected cohorts and show a large heterogeneity based on the ethnic background. Cardiac sarcoidosis is not only found in patients with rhythmogenic heart disease, such as atrial and ventricular fibrillation but also in all phenotypes of cardiomyopathy. The overall morbidity and mortality caused by cardiac sarcoidosis in Germany is unclear and no large prospective international studies have been published on this topic. This consensus paper on diagnostic and therapeutic algorithms for cardiac sarcoidosis is based on a current literature search and forms an expert opinion statement under the auspices of the German Respiratory Society and the German Cardiac Society. The rationale of this statement is to provide algorithms to facilitate clinical decision-making based on the individual case situation.
Literatur
Hiraga H, Hiroe M, Iwai K (1993) Guideline for diagnosis of cardiac sarcoidosis: study report on diffuse pulmonary diseases (in Japanese). The Japanese Ministry of Health and Welfare, Tokyo, S 23–24
Soejima K, Yada H (2009) The work-up and management of patients with apparent or subclinical cardiac sarcoidosis: with emphasis on the associated heart rhythm abnormalities. J Cardiovasc Electrophysiol 20:578–583
Pabst S, Skowasch D, Grohé C (2012) Sarkoidose. Pneumologie 66:96–109
Pabst S, Tuleta I, Grohé C (2008) Kardiale Sarkoidose. Kardiologe 2:299–311
Schulte W, Costabel U, Kirsten D (2003) Kardiale Sarkoidose. Pneumologie 57:752–760
Pietinalho A, Hiraga Y, Hosoda Y et al (1995) The frequency of sarcoidosis in Finland and Hokkaido, Japan: a comparative epidemiological study. Sarcoidosis 12:61–67
Deubelbeiss U, Gemperli A, Schindler C et al (2010) Prevalence of sarcoidosis in Switzerland is associated with environmental factors. Eur Respir J 35:1088–1097
Iwai K, Tachibana T, Takemura T et al (1993) Pathological studies on sarcoidosis autopsy. I. Epidemiological features of 320 cases in Japan. Acta Pathol Jpn 43:372–376
Reid JD (1998) Sarcoidosis in coroner’s autopsies: a critical evaluation of diagnosis and prevalence from Cuyahoga County, Ohio. Sarcoidosis Vasc Diffuse Lung Dis 15:44–51
Pollak B (1964) Epidemiology of sarcoidosis in Canada. Acta Med Scand Suppl 425:145
Kitaichi M (1998) Prevalence of sarcoidosis around the world. Sarcoidosis Vasc Diffuse Lung Dis 15:16–18
Rybicki BA, Major M, Popovich J Jr et al (1997) Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol 145:234–241
Roberts WC, McAllister HS Jr, Ferrans VJ et al (1977) Sarcoidosis of the heart. A clinicopathologic study of 35 necropsy patients and review of 78 previously described necropsy patients. Am J Med 63:86–108
Kim JS, Judson MA, Donnino R et al (2009) Cardiac sarcoidosis. Am Heart J 157:9–21
Youssef G, Beanlands RSB, Birnie DH, Nery PB (2011) Cardiac sarcoidosis: applications of imaging in diagnosis and directing treatment. Heart 97:2078–2087
Shigemitsu H, Nagai S, Sharma OP (2007) Pulmonary hypertension and granulomatous vasculitis in sarcoidosis. Curr Opin Pulm Med 13:434–438
Chapelon-Abric C, Zuttere D de, Duhaut P et al (2004) Cardiac sarcoidosis: a retrospective study of 41 cases. Medicine (Baltimore) 83:315–334
Fleming HA, Baily SM (1981) Sarcoid heart disease. J R Coll Physicians Lond 15:245–246
Matsui Y, Iwai K, Tachibana T et al (1976) Clinicopathological study of fatal myocardial sarcoidosis. Ann N Y Acad Sci 278:455–469
Smedema JP, Snoep G, Kroonenburgh MP van et al (2005) Cardiac involvement in patients with pulmonary sarcoidosis at two university medical centers in the Netherlands. Chest 128:30–35
Yazaki Y, Isobe M, Hiroe M et al (2001) Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone. Am J Cardiol 88:1006–1010
Pabst S, Hammerstingl C, Grau N et al (2013) Pulmonary arterial hypertension in patients with sarcoidosis: the Pulsar single center experience. Adv Exp Med Biol 755:299–305
Iwai K, Sekiguti M, Hosoda Y et al (1994) Racial difference in cardiac sarcoidosis incidence observed at autopsy. Sarcoidosis 11:26–31
Judson MA, Baughman RP, Teirstein AS et al (1999) Defining organ involvement in sarcoidosis: the ACCESS proposed instrument. ACCESS Research Group. A case control etiologic study of sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 16:75–86
Kandolin R, Lehtonen J, Graner M et al (2011) Diagnosing isolated cardiac sarcoidosis. J Intern Med 270:461–468
Sulica R, Teirstein AS, Kakarla S et al (2005) Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension. Chest 128:1483–1489
Baughman RP, Judson MA, Teirstein A et al (2006) Presenting characteristics as predictors of duration of treatment in sarcoidosis. QJM 99:307–315
Handa T, Nagai S, Miki S et al (2006) Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest 129:1246–1252
Bourbonnais JM, Samavati L (2008) Clinical predictors of pulmonary hypertension in sarcoidosis. Eur Respir J 32:296–302
Baughman RP, Engel PJ, Taylor L, Lower EE (2010) Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest 138:1078–1085
Nunes H, Humbert M, Capron F et al (2006) Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax 61:68–74
Shlobin OA, Nathan SD (2012) Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation. Eur Respir J 39:1520–1533
Galiè N, Hoeper MM, Humbert M et al (2009) 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 30:2493–537
Gideon NM, Mannino DM (1996) Sarcoidosis mortality in the United States 1979–1991: an analysis of multiple-cause mortality data. Am J Med 100:423–427
Baughman RP, Winget DB, Bowen EH, Lower EE (1997) Predicting respiratory failure in sarcoidosis patients. Sarcoidosis Vasc Diffuse Lung Dis 14:154–158
Kandolin R, Lehtonen J, Kupari M (2011) Cardiac sarcoidosis and giant cell myocarditis as causes of atrioventricular block in young and middle-aged adults. Circ Arrhythm Electrophysiol 4:303–309
Patel MR, Cawley PJ, Heitner JF et al (2009) Detection of myocardial damage in patients with sarcoidosis. Circulation 120:1979–1977
Schuller JL, Olson MD, Zipse MM (2011) Electrocardiographic characteristics in patients with pulmonary sarcoidosis indicating cardiac involvement. J Cardiovasc Electrophysiol 22:1243–1248
Das MK, Kahn B, Jacob S et al (2006) Significance of a fragmented QRS complex versus a Q wave in patients with coronary artery disease. Circulation 113:2495–2501
Silverman KJ, Hutchins GM, Bulkley BH (1978) Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis. Circulation 58:1204–1211
Burstow DJ, Tajik AJ, Bailey KR et al (1989) Two-dimensional echocardiographic findings in systemic sarcoidosis. Am J Cardiol 63:478–482
Mehta D, Lubitz SA, Frankel Z et al (2008) Cardiac involvement in patients with sarcoidosis: diagnostic and prognostic value of outpatient testing. Chest 133:1426–1435
Fahy GJ, Marwick T, McCreery CJ et al (1996) Doppler echocardiographic detection of left ventricular diastolic dysfunction in patients with pulmonary sarcoidosis. Chest 109:62–66
Schulz-Menger J, Wassmuth R, Abdel-Aty H et al (2006) Patterns of myocardial inflammation and scarring in sarcoidosis as assessed by cardiovascular magnetic resonance. Heart 92:399–400
Cummings KW, Bhalla S, Javidan-Nejad C et al (2009) A pattern-based approach to assessment of delayed enhancement in nonischemic cardiomyopathy at MR imaging. Radiographics 29:89–103
Ichinose A, Otani H, Oikawa M et al (2008) MRI of cardiac sarcoidosis: basal and subepicardial localization of myocardial lesions and their effect on left ventricular function. AJR Am J Roentgenol 191:862–869
Smedema JP, Snoep G, Kroonenburgh MP van et al (2005) Evaluation of the accuracy of gadolinium-enhanced cardiovascular magnetic resonance in the diagnosis of cardiac sarcoidosis. J Am Coll Cardiol 45:1683–1690
Ohira H, Tsujino I, Ishimaru S et al (2008) Myocardial imaging with 18F-fluoro-2-deoxyglucose positron emission tomography and magnetic resonance imaging in sarcoidosis. Eur J Nucl Med Mol Imaging 35:933–941
Pellegrino D, Bonab AA, Dragotakes SC et al (2005) Inflammation and infection: imaging properties of 18F-FDG-labeled white blood cells versus 18F-FDG. J Nucl Med 46:1522–1530
Tellier P, Valeyre D, Nitenberg A et al (1985) Cardiac sarcoidosis: reversion of myocardial perfusion abnormalities by dipyridamole. Eur J Nucl Med 11:201–204
Tellier P, Paycha F, Antony I et al (1988) Reversibility by dipyridamole of thallium-201 myocardial scan defects in patients with sarcoidosis. Am J Med 85:189–193
Hirose Y, Ishida Y, Hayashida K et al (1994) Myocardial involvement in patients with sarcoidosis. An analysis of 75 patients. Clin Nucl Med 9:522–526
Mana J (1997) Nuclear imaging. 67Gallium, 201thallium, 18F-labeled fluoro-2-deoxy-D-glucose positron emission tomography. Clin Chest Med 18:799–811
Okayama K, Kurata C, Tawarahara K et al (1995) Diagnostic and prognostic value of myocardial scintigraphy with thallium-201 and gallium-67 in cardiac sarcoidosis. Chest 107:330–334
Le Guludec D, Menad F, Faraggi M et al (1994) Myocardial sarcoidosis. Clinical value of technetium-99m sestamibi tomoscintigraphy. Chest 106:1675–1682
Okumura W, Iwasaki T, Toyama T et al (2004) Usefulness of fasting 18F-FDG PET in identification of cardiac sarcoidosis. J Nucl Med 45:1989–1998
Ishimaru S, Tsujino I, Takei T et al (2005) Focal uptake on 18F-fluoro-2-deoxyglucose positron emission tomography images indicates cardiac involvement of sarcoidosis. Eur Heart J 26:1538–1543
Tadamura E, Yamamuro M, Kubo S et al (2005) Effectiveness of delayed enhanced MRI for identification of cardiac sarcoidosis: comparison with radionuclide imaging. AJR Am J Roentgenol 185:110–115
Yamagishi H, Shirai N, Takagi M et al (2003) Identification of cardiac sarcoidosis with (13)N- NH(3)/(18)F-FDG PET. J Nucl Med 44:1030–1036
Ardehali H, Howard DL, Hariri A et al (2005) A positive endomyocardial biopsy result for sarcoid is associated with poor prognosis in patients with initially unexplained cardiomyopathy. Am Heart J 150:459–463
Cooper LT, Baughman KL, Feldman AM et al (2007) 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 50:1914–1931
Aizer A, Stern EH, Gomes JA et al (2005) Usefulness of programmed ventricular stimulation in predicting future arrhythmic events in patients with cardiac sarcoidosis. Am J Cardiol 96:276–282
Mehta D, Mori N, Goldbarg S et al (2011) Primary prevention of sudden cardiac death in silent cardiac sarcoidosis: role of programmed ventricular stimulation. Circ Arrhythm Electrophysiol 4:43–48
Date T, Shinozaki T, Yamakawa M et al (2007) Elevated plasma brain natriuretic peptide level in cardiac sarcoidosis patients with preserved ejection fraction. Cardiology 107:277–280
Yasutake H, Seino Y, Kashiwagi M et al (2005) Detection of cardiac sarcoidosis using cardiac markers and myocardial integrated backscatter. Int J Cardiol 102:259–268
Baughman RP, Culver DA, Judson MA (2011) A concise review of pulmonary sarcoidosis. Am J Respir Crit Care Med 183:573–581
o A (1999) 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 160:736–755
Grutters JC, Bosch JM van den (2006) Corticosteroid treatment in sarcoidosis. Eur Respir J 28:627–636
Kato Y, Morimoto S, Uemura A et al (2003) Efficacy of corticosteroids in sarcoidosis presenting with atrioventricular block. Sarcoidosis Vasc Diffuse Lung Dis 20:133–137
Chiu CZ, Nakatani S, Zhang G et al (2005) Prevention of left ventricular remodeling by long-term corticosteroid therapy in patients with cardiac sarcoidosis. Am J Cardiol 95:143–146
Banba K, Kusano KF, Nakamuro K et al (2007) Relationship between arrhythmogenesis and disease activity in cardiac sarcoidosis. Heart Rhythm 4:1292–1299
Jefic D, Joel B, Good E et al (2009) Role of radiofrequency catheter ablation of ventricular tachycardia in cardiac sarcoidosis: report from a multicenter registry. Heart Rhythm 6:189–195
Yodogawa K, Seino Y, Ohara T et al (2011) Effect of corticosteroid therapy on ventricular arrhythmias in patients with cardiac sarcoidosis. Ann Noninvasive Electrocardiol 16:140–147
Epstein AE, Dimarco JP, Ellenbogen KA et al (2008) ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: executive summary. Heart Rhythm 5:934–955
Oni AA, Hershberger RE, Norman DJ et al (1992) Recurrence of sarcoidosis in a cardiac allograft: control with augmented corticosteroids. J Heart Lung Transplant 11:367–369
Viles-Gonzalez JF, Pastori L, Fischer A et al (2013) Supraventricular arrhythmias in patients with cardiac sarcoidosis prevalence, predictors, and clinical implications. Chest 4:1085–1090
Soejima K, Yada HJ (2009) The work-up and management of patients with apparent or subclinical cardiac sarcoidosis: with emphasis on the associated heart rhythm abnormalities. J Cardiovasc Electrophysiol 20(5):578–583
Kato Y, Morimoto S, Uemura A et al (2003) Efficacy of corticosteroids in sarcoidosis presenting with atrioventricular block. Sarcoidosis Vasc Diffuse Lung Dis 20(2):133–137
Yazaki Y, Isobe M, Hiroe M et al (2001) Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone. Am J Cardiol 88(9):1006–1010
Iannuzzi MC, Fontana JR (2011) Sarcoidosis: clinical presentation, immunopathogenesis, and therapeutics. JAMA 305:391–299
Jefic D et al (2009) Role of radiofrequency catheter ablation of ventricular tachycardia in cardiac sarcoidosis: report from a multicenter registry. Heart Rhythm 6:189–195
Einhaltung ethischer Richtlinien
Interessenkonflikt. U. Costabel, D. Skowasch, S. Pabst, S. Störk, C. Tschöpe, M. Allewelt, H. Worth, J. Müller-Quernheim J und C. Grohé geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.
Author information
Authors and Affiliations
Corresponding author
Additional information
Dieser Beitrag wurde in den Zeitschriften Der Kardiologe und Pneumologie zeitgleich publiziert.
Rights and permissions
About this article
Cite this article
Costabel, U., Skowasch, D., Pabst, S. et al. Konsensuspapier der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin (DGP) und der Deutschen Gesellschaft für Kardiologie – Herz und Kreislaufforschung (DGK) zur Diagnostik und Therapie der kardialen Sarkoidose. Kardiologe 8, 13–25 (2014). https://doi.org/10.1007/s12181-013-0550-z
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12181-013-0550-z