Lungenbeteiligung bei systemischer Sklerodermie

Zusammenfassung

Unter dem Oberbegriff Sklerodermie werden heterogene Autoimmunerkrankungen zusammengefasst, die verdickte, sklerotische Hautläsionen gemein haben. Die Sklerodermie zählt zum Formenkreis der Kollagenosen. Die systemische Form der Sklerodermie wird als Progressive Systemische Sklerose (PSS) oder systemische Sklerodermie (SSc) bezeichnet. Die Lungenbeteiligung bei SSc in Form von interstitieller Lungenerkrankung (ILD) und pulmonal-arterieller Hypertonie (PAH) ist eine der häufigsten Manifestationen der Erkrankung. Das Ziel dieser Arbeit besteht darin, einen Überblick über die Formen des Lungenbefalls bei SSc mit besonderem Augenmerk auf die interstitiellen Lungenerkrankungen zu geben – unter Berücksichtigung der bisher identifizierten pathogenetischen Mechanismen und der Therapieoptionen.

Abstract

Scleroderma is a generic term for autoimmunological diseases having thickened sclerotic skin lesions in common. Scleroderma belongs to a group of connective tissue diseases. The systemic form of scleroderma is called progressive systemic sclerosis (PSS) or systemic sclerosis (SSc). Lung impairments, namely interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), are one of the most common manifestations in SSc. This article summarises the forms of lung impairment in SSc with special emphasis on interstitial lung diseases and draws attention to the so far identified pathogenetic mechanisms and the presently accepted therapeutic options.

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