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Updated: Feb 19, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Acute Exacerbation in Interstitial Lung Disease.

Gabriela Leuschner¹, Jürgen Behr^1,2

¹Department of Internal Medicine V, Ludwig Maximilians University, Comprehensive Pneumology Center (CPC-M), German Center for Lung Research (DZL), Munich, Germany.

Frontiers in Medicine

|November 8, 2017

Summary

Acute exacerbations of interstitial lung disease (AE-ILD) are sudden deteriorations with poor prognosis, often triggered by infections or stress. Management typically involves corticosteroids and antibiotics, though evidence is limited.

Keywords:

acute exacerbation definition diagnosis idiopathic pulmonary fibrosis interstitial lung disease management

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Area of Science:

Pulmonology
Critical Care Medicine

Background:

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a rapid decline within a month, characterized by alveolar damage and diffuse opacities.
Acute exacerbations of interstitial lung disease (AE-ILD) share similar pathophysiology with AE-IPF and can affect all ILD patients, particularly those with usual interstitial pneumonia patterns.

Purpose of the Study:

To summarize the clinical features, diagnosis, management, and prognosis of AE-ILD.
To provide an update on current developments in the field of AE-ILD.

Main Methods:

Literature review and synthesis of existing data on AE-ILD.
Focus on clinical manifestations, diagnostic criteria, and treatment strategies.

Main Results:

AE-ILD is associated with significant alveolar damage and has a poor prognosis, with high mortality within 6-12 months.
Common triggers include infection, mechanical stress, and microaspiration.
Current management often includes high-dose corticosteroids and antibiotics, despite limited evidence-based data.

Conclusions:

AE-ILD represents a critical clinical challenge in interstitial lung disease management.
Further research is needed to establish evidence-based treatment guidelines and improve patient outcomes.