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2017 ; 4
(ä): 176
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Acute Exacerbation in Interstitial Lung Disease
#MMPMID29109947
Leuschner G
; Behr J
Front Med (Lausanne)
2017[]; 4
(ä): 176
PMID29109947
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Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) has been defined as
an acute, clinically significant deterioration that develops within less than
1?month without obvious clinical cause like fluid overload, left heart failure,
or pulmonary embolism. Pathophysiologically, damage of the alveoli is the
predominant feature of AE-IPF which manifests histopathologically as diffuse
alveolar damage and radiologically as diffuse, bilateral ground-glass
opacification on high-resolution computed tomography. A growing body of
literature now focuses on acute exacerbations of interstitial lung disease
(AE-ILD) other than idiopathic pulmonary fibrosis. Based on a shared
pathophysiology it is generally accepted that AE-ILD can affect all patients with
interstitial lung disease (ILD) but apparently occurs more frequently in patients
with an underlying usual interstitial pneumonia pattern. The etiology of AE-ILD
is not fully understood, but there are distinct risk factors and triggers like
infection, mechanical stress, and microaspiration. In general, AE-ILD has a poor
prognosis and is associated with a high mortality within 6-12?months. Although
there is a lack of evidence based data, in clinical practice, AE-ILD is often
treated with a high dose corticosteroid therapy and antibiotics. This article
aims to provide a summary of the clinical features, diagnosis, management, and
prognosis of AE-ILD as well as an update on the current developments in the
field.
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