Extrapulmonale Sarkoidose: Befall des Gastrointestinaltraktes – Fallbericht und Literaturrecherche

Zusammenfassung

Bei der Sarkoidose handelt es sich um eine Systemerkrankung, welche histologisch durch nicht verkäsende Granulome charakterisiert ist. In > 90 % der Fälle manifestiert sie sich in der Lunge, kann aber auch jedes andere Organ betreffen. Extrapulmonale Manifestationen finden sich in Lymphknoten, Haut, dem Nervensystem, Herz, Augen sowie dem Knochenmark, der Milz oder dem Gastrointestinaltrakt mit Leber und Pankreas. Ein Befall des Gastrointestinaltraktes ist selten, kann in diesem jedoch jedes Organ betreffen und findet sich am häufigsten im Magen. Trotz des insgesamt seltenen Auftretens der gastrointestinalen Sarkoidose kann diese in Abhängigkeit der betroffenen Organe und der Ausprägung klinisch relevante Auswirkungen haben, sodass eine entsprechende zeitgerechte Diagnosestellung und Therapie von großer Wichtigkeit ist. Das medikamentöse Therapiekonzept besteht in einer immunsuppressiven Therapie, wobei Kortikosteroide initial das Mittel der Wahl darstellen. Die notwendige Therapiedauer und die Langzeitprognose der gastrointestinalen Sarkoidose werden kontrovers diskutiert. In der Literatur gibt es zu diesem Krankheitsbild nur wenige evidenzbasierte Daten; randomisierte Studien fehlen vollständig. Ziel dieser Arbeit ist es, einen Überblick über die aktuellen Datenlage zu diesem Thema zu geben.

Abstract

Sarcoidosis is a rare systemic disease characterized by noncaseating granulomas in affected organs. With > 90 % of all cases lung involvement is the most frequent site of manifestation of sarcoidosis, nevertheless multiple other organs can be affected. Extrapulmonary manifestations are lymph nodes, skin, nervous system, heart, eyes, bone marrow, spleen or gastrointestinal tract including liver and pancreas. Involvement of the gastrointestinal tract is rare, it can affect the whole intestine and is most frequently found in the stomach. Despite its infrequent manifestation, gastrointestinal sarcoidosis can have a significant clinical impact depending on the organ involved, so that early diagnosis is warranted. Medical treatment mainly consists of immunosuppressive therapy, most frequently with corticosteroids being the first treatment of choice. Dosage and duration of therapy is not well established yet. In the literature very limited data are available on this topic with randomized trials missing completely. The aim of this paper is to give a summary of the available data to this date.

Schlüsselwörter

extrapulmonale Sarkoidose - gastrointestinale Beteiligung - Immuntherapie

Key words

extrapulmonary sarcoidosis - gastrointestinal involvement - immunotherapy

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