Sarkoidose

Die Sarkoidose ist eine granulomatöse Systemerkrankung und befällt meist die Lunge und thorakale Lymphknoten. Die Diagnose wird anhand des typischen klinisch-radiologischen Erscheinungsbilds und der Histologie gestellt. Die Therapie wird aufgrund einer funktionellen Einschränkung, organbezogener oder systemischer Symptomatik und lebensbedrohlicher Organmanifestationen indiziert. Bisher gibt es keine zugelassene medikamentöse Therapie für die Sarkoidose. Die Gabe von Immunsuppressiva wie Glukokortikosteroide und ggf. als Add-on oder sequenziell Methotrexat, Azathioprin oder Mycophenolat-Mofetil wird von der aktuell publizierten internationalen Leitlinie empfohlen.

Abstract

Sarcoidosis is a granulomatous systemic disease of unknown etiology most commonly affecting the lungs and thoracic lymph nodes. The diagnosis is based on typical clinical radiologic appearance and histology with evidence of noncaseating epithelioid cell granulomas without central necrosis. In the acute form, Löfgren's syndrome, histologic confirmation may not be necessary. Approximately half of patients may develop a chronic form, and extrathoracic organ involvement should be investigated during the course. Indications for therapy are based on functional limitations, marked organ-related or systemic symptoms, and life-threatening organ manifestations (cardiac, central nervous system, renal, and ocular sarcoidosis). To date, there is no approved drug therapy for sarcoidosis. Administration of immunosuppressants such as glucocorticosteroids and as add-on or sequential, methotrexate, azathioprine or mycophenolate mofetil is recommended in the currently published international guideline.

Kernaussagen

• Die Sarkoidose ist eine granulomatöse Systemerkrankung unklarer Genese, die am häufigsten Lymphknoten und Lunge betrifft.

• Eine pneumologische Mitbetreuung sollte bei häufiger pulmonaler Manifestation erfolgen.

• Das Erythema nodosum stellt eine vaskulitische und keine granulomatöse Läsion dar, eine histologische Sicherung ist daher nicht zu empfehlen.

• In der BAL kann eine Lymphozytose mit einem erhöhten CD4-/CD8-Quotienten nachweisbar sein, sie sollte jedoch nicht als Verlaufskontrolle eingesetzt werden.

• Ein symptomarmer Patient mit fehlender lungenfunktioneller Einschränkung muss nicht therapiert werden.

• Die initiale Therapie besteht aus Glukokortikosteroiden und kann bei Therapieversagen mit Immunsuppressiva ergänzt werden.

• Verlaufskontrollen erfolgen zu Beginn alle 3–6 Monate.

Schlüsselwörter

pulmonale Sarkoidose - granulomatöse Entzündung - Glukokortikosteroide - Immunsuppression - Fibrosierung

Keywords

pulmonary sarcoidosis - granulomatous inflammation - glucocorticoids - immunosuppression - fibrosis

Publication History

Article published online:
22 April 2022

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