Diagnostik und Therapie der pulmonalen und kardialen Sarkoidose

Die Sarkoidose ist eine häufige seltene Erkrankung unklarer Ursache, bei der eine Vielzahl unterschiedlicher Organe in unterschiedlichem Ausmaß betroffen sein kann [1]. Das klinische Beschwerdebild, die Indikation zur medikamentösen Therapie sowie die Prognose weisen daher eine besonders hohe Variabilität auf. Dieser Beitrag fasst die korrekte Diagnosestellung, die individuelle Risikostratifizierung und die Therapie der Lungen- und Herzsarkoidose zusammen.

Abstract

Sarcoidosis occurs predominantly in younger adults and may involve multiple organ systems. Although classical features such as bihilar lymphadenopathy, low grade fever, fatigue, pulmonary opacities, ocular lesions and arthritis occur frequently it must be kept in mind that sarcoidosis is able to mimic virtually any other disease. A thorough and systematic diagnostic strategy is warranted since even the histological hallmark of non-caseating granulomas occurs in a variety of other granulomatous diseases and even malignancies. A firm diagnosis is based on a match of clinical features, imaging results and histopathological findings. The Scadding classification describes four different types, not stages, of thoracic sarcoidosis based on mediastinal lymph node and lung parenchyma involvement. Medical treatment of pulmonary sarcoidosis is indicated only in patients exhibiting progressive disease and/or organ function impairment. Cardiac sarcoidosis manifests itself by clinical signs of heart failure, impaired cardiac function and arrhythmias including ventricular tachycardia, ventricular fibrillation and AV-conduction abnormalities. Patients with symptomatic or suspected cardiac sarcoidosis require rapid and elaborate diagnostic testing including cardiac MRI and PET imaging. Referral to a specialized center should be considered to establish a firm diagnosis and to initiate medical treatment and eventual device implantation. Oral corticosteroids, as the initial medical treatment of choice, carry side effects that must be weighed carefully against clinical benefits. Immunosuppressive therapy with methotrexate, azathioprine or TNF-blockers is usually reserved for patients that are either not responsive or intolerant to systemic steroids or that require steroid maintenance therapy above the cushing threshold. Diagnosed early and treated correctly, pulmonary and cardiac sarcoidosis mostly carry a favorable prognosis.

Schlüsselwörter

Sarkoidose - Diagnostik - Therapie - Herzsarkoidose - Lungensarkoidose

Key words

sarcoidosis - diagnosis - treatment - cardiac sarcoidosis - pulmonary sarcoidosis

Publikationsverlauf

Artikel online veröffentlicht:
01. März 2021

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