Interstitielle Lungenerkrankungen (ILD) können im Rahmen rheumatologischer Systemerkrankungen auftreten und müssen bei Verdacht durch detaillierte Diagnostik detektiert werden. Besonders kritisch ist das Auftreten von akuten Exazerbationen, die während eines Progresses der ILD auftreten und mit einer erheblichen Mortalität verbunden sind. Eine interdisziplinär entwickelte Leitlinie gibt Handlungsempfehlungen für die IDL-Diagnostik.
Abstract
Interstitial lung diseases (ILD) are etiologically heterogeneous with unknown and known causes like rheumatologic systemic diseases differing in their therapeutic and prognostic consequences. In consensus between pulmonologists, rheumatologists, radiologists, and pathologists, we developed practical instructions for ILD diagnosis in rheumatologic systemic diseases, in particular because ILD can present in early stages of rheumatic systemic diseases. ILD diagnosis is based on clinical assessment results including a detailed medical history, physical examination, focused laboratory tests, radiology with a high-resolution computed tomography, lung function, and histopathology also to differentiate it from cardiac and infection associated lung diseases. The ILD diagnosis is made in a multidisciplinary discussion leading to therapeutic and prognostic consequences. The occurrence of acute exacerbations is especially critical. They are often the causes for ILD progression and are associated with considerable mortality.
Schlüsselwörter
interstitielle Lungenerkrankung - rheumatologische Systemerkrankung - diagnostische Handlungsempfehlung - Lungenfibrose - diffuse Lungenparenchym-Erkrankung
Key words
interstitial lung disease - rheumatoid systemic disease - diagnostic recommendation - lung fibrosis - diffuse parenchymal lung disease
