Diagnostik und Therapie der kardialen Sarkoidose^[*]

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, wie akuelle Kohortenstudien zeigen. Diagnostische Schritte und therapeutische Empfehlungen zur kardialen Sarkoidose sollten daher einem einheitlichen Standard unterliegen. Die vorliegende Arbeit ist die 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. Symptomatic 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 remains unclear and large prospective international observational studies.underline the importance of this disease entity. 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.

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

^# N. Marx hat an diesem Beitrag für die Kommission für Klinische Kardiovaskuläre Medizin der DGK mitgewirkt.

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