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Pulmonale Beteiligung bei Kollagenosen

Pulmonary manifestations of connective tissue diseases

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Zusammenfassung

Systemische Autoimmunerkrankungen sind für rund 25% aller Todesfälle infolge interstitieller Lungenerkrankungen verantwortlich. Daher bestimmt eine frühe Identifikation der Patienten mit einer pulmonalen Manifestation das Management. Detektion, differenzialdiagnostische Einordnung und Staging der pulmologischen Befundmuster basieren maßgeblich auf der hochauflösenden Computertomographie (HR-CT). Die größte differenzialdiagnostische Herausforderung sind interstitielle Parenchymmanifestationen, die sich mit radiologisch-histopathologischen Phänotypen der interstitiellen Pneumonien präsentieren. Die häufigste interstitielle Reaktionsform der Kollagenosen ist das NSIP („nonspecific interstitial pneumonia“)-Muster. Bei der rheumatoiden Arthritis (RA) ist ein UIP („usual interstitial pneumonia“)-Pattern dominant. Die uncharakteristischen Reaktionen an Atemwegen und Pleura können symptomführend sein oder Begleitbefunde darstellen. Eine gefürchtete Komplikation ist die pulmonale Hypertonie. HR-CT-morphologisch vergleichbare medikamentenassoziierte Veränderungen sind nur im zeitlichen und klinischen Kontext zu differenzieren.

Abstract

Systemic autoimmune diseases are responsible for about 25% of all deaths due to interstitial lung disease; therefore, an early identification of patients with pulmonary manifestation changes the management. Detection, differential diagnostic classification and staging of the pneumological pattern of findings are largely based on high-resolution computed tomography (HR-CT). The main differential diagnostic challenges are interstitial manifestations which present with radiological-histopathological phenotypes of interstitial pneumonia. The most common form of interstitial pulmonary reaction form of connective tissue diseases is the nonspecific interstitial pneumonia (NSIP) pattern. In rheumatoid arthritis, a usual interstitial pneumonia (UIP) pattern is dominant. Uncharacteristic reactions of airways and pleura can be the leading symptom or present as accompanying findings. A serious complication is pulmonary hypertension. Drug-induced lung lesions can present with similar HR-CT morphology as connective tissue diseases and can only be differentiated in the temporal and clinical context.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. B. Rehbock hat Honorare für Vorträge und/oder Beratertätigkeit von AstraZeneca, Bayer, Berlin-Chemie, Boehringer Ingelheim, Chiesi, InterMune und Novartis erhalten.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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  1. Praxis für Diagnostische Radiologie – Spezialgebiet Lunge, Bismarckstr. 45–47, 10627, Berlin, Deutschland

    B. Rehbock

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Rehbock, B. Pulmonale Beteiligung bei Kollagenosen. Radiologe 55, 241–256 (2015). https://doi.org/10.1007/s00117-014-2789-1

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