CASE REPORT
Diagnosis and Management of Patients with Connective Tissue Disease-related Fibrosing Interstitial Lung Diseases
Bonnie Wang1, *, Vivek Nagaraja2
Article Information
Identifiers and Pagination:
Year: 2023Volume: 17
E-location ID: e187430642306150
Publisher ID: e187430642306150
DOI: 10.2174/18743064-v17-e230714-2022-26
Article History:
Received Date: 17/11/2022Revision Received Date: 11/04/2023
Acceptance Date: 29/05/2023
Electronic publication date: 15/08/2023
Collection year: 2023
open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Abstract
Background:
Fibrotic interstitial lung disease is an important driver of morbidity and mortality in patients with connective tissue diseases (CTD). Due to the lack of prospective randomized trial data in this population, practice pattern variation exists in the management of patients with CTD.
Case Presentation:
This case series describes three patients, each with a different background of autoimmunity complicated by fibrotic interstitial lung disease (ILD). We review their initial presentations, follow their disease trajectories on currently available treatments, and reference forthcoming clinical trials.
Conclusion:
Clinical impact or potential implications. Response to immunosuppression and antifibrotic therapy is variable in patients with connective tissue disease-related fibrosing interstitial lung disease. Data from prospective clinical trials and longitudinal registry studies will conceivably provide additional insight into improving care for these patients.
