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Sarkoidose

Sarcoidosis

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Zusammenfassung

Bei der Sarkoidose, einer granulomatösen Systemerkrankung unklarer Ätiologie, steht die pulmonale Beteiligung zahlenmäßig im Vordergrund, wenn auch prinzipiell alle Organe befallen sein können. Die Diagnose ergibt sich aus dem typischen klinisch-radiologischen Bild und dem histologischen Nachweis epitheloidzelliger Granulome, worauf nur bei der akuten Verlaufsform des Löfgren-Syndroms verzichtet werden sollte. Die Prognose ist mit einer hohen Rate von Spontanremissionen insgesamt gut und hängt von den Organkomplikationen ab. Am häufigsten wird, neben dem Befall von Lunge oder mediastinalen Lymphknoten, klinisch ein Haut- oder Augenbefall gesehen; eine kardiale oder zentralnervöse Manifestation ist von kritischer Bedeutung. Wenn die Entscheidung zur Einleitung einer Therapie gefällt wird, werden zunächst Kortikosteroide eingesetzt. Andere Immunsuppressiva dienen zum Einsparen der Steroiddosis. Bei eher seltenen therapierefraktären Fällen konnten gute Effekte mit TNF-α-Blockern erzielt werden. Eine pneumologische Verlaufskontrolle sollte bei Sarkoidosepatienten angestrebt werden.

Abstract

The most common manifestations of sarcoidosis, a systemic granulomatous disease of unknown etiology, involve the lungs but all organs may be affected. For diagnosis the typical clinical radiological picture should be supported by the histological confirmation of granuloma but this is not necessary for acute sarcoidosis (Löfgren’s syndrome). The long-term prognosis of sarcoidosis is generally good and depends on the various organ manifestations. The lungs and mediastinal lymph nodes, followed by the skin and eyes are most commonly affected but cardiac or central nervous sarcoidosis is a critical organ involvement. The cornerstone of treatment is corticosteroids but other immunosuppressants can be used to decrease the minimal required dosage of steroids. In rarely seen treatment refractory patients TNF-alpha inhibitors may be effective. Continuous monitoring by a pneumologist during the course of the disease is recommended.

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Interessenkonflikt

Der korrespondierende Autor weist für sich und seine Koautoren auf folgende Beziehungen hin: T.E. Wessendorf gibt keine Interessenkonflikte an. U. Costabel hat als Berater und Prüfarzt und F. Bonella als Prüfarzt an einer Therapiestudie zur Sarkoidose der Firma Centocor teilgenommen.

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Authors and Affiliations

  1. Abteilung Pneumologie & Allergologie, Ruhrlandklinik, Westdeutsches Lungenzentrum, Universitätsklinikum Essen, Universität Duisburg - Essen, Tüschener Weg 40, 45239, Essen, Deutschland

    T.E. Wessendorf, F. Bonella & U. Costabel

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  1. T.E. Wessendorf
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  2. F. Bonella
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  3. U. Costabel
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Correspondence to T.E. Wessendorf.

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Wessendorf, T., Bonella, F. & Costabel, U. Sarkoidose. Pneumologe 10, 137–152 (2013). https://doi.org/10.1007/s10405-012-0665-0

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  • DOI: https://doi.org/10.1007/s10405-012-0665-0

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