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Updated: Jul 18, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Progressive fibrotic interstitial lung disease.

Carlos A C Pereira¹, Soraya Cordero², Ana Carolina Resende²

¹. Programa de Assistência e Pesquisa em Doenças Pulmonares Intersticiais, Departamento de Clínica Médica, Serviço de Pneumologia, Universidade Federal de São Paulo, São Paulo (SP) Brasil.

Jornal Brasileiro De Pneumologia : Publicacao Oficial Da Sociedade Brasileira De Pneumologia E Tisilogia

|August 23, 2023

Summary

This summary is machine-generated.

Progressive fibrosing interstitial lung diseases (PF-ILDs) affect 30% of ILD patients. Antifibrotic drugs like nintedanib show promise in slowing disease progression, offering new hope for patients.

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Area of Science:

Pulmonology
Fibrotic Interstitial Lung Diseases
Pharmacology

Background:

Interstitial lung diseases (ILDs) often share mechanisms leading to progressive fibrosis (PF-ILD).
PF-ILD affects approximately 30% of ILD patients, including conditions like chronic hypersensitivity pneumonitis and idiopathic pulmonary fibrosis.
Accurate diagnosis is critical as PF-ILD progresses despite treatment.

Purpose of the Study:

To review the current understanding of progressive fibrosing interstitial lung diseases (PF-ILDs).
To evaluate the efficacy of antifibrotic drugs in managing PF-ILD.
To highlight the need for further research into risk markers and treatment responses.

Main Methods:

Review of existing literature on PF-ILD mechanisms, diagnosis, and treatment.
Analysis of clinical trial data for antifibrotic agents (nintedanib, pirfenidone) in PF-ILD patients.
Examination of regulatory approvals for nintedanib in Brazil and the US.

Main Results:

Nintedanib demonstrated a significant slowing of lung function decline (mean difference of 107.0 mL/year) compared to placebo in PF-ILD patients.
Pirfenidone has shown similar positive results in patients with unclassified ILD and other ILDs.
Regulatory agencies in the US and Brazil have approved nintedanib for PF-ILD treatment.

Conclusions:

Antifibrotic therapies, particularly nintedanib, represent a significant advancement in managing progressive fibrosing interstitial lung diseases.
Early identification of risk factors and personalized treatment strategies are crucial for improving patient outcomes.
Continued research is essential to identify predictive markers for disease progression and treatment response.