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Semin Respir Crit Care Med 2024; 45(03): 287-304
DOI: 10.1055/s-0044-1785674
Review Article

Diagnostic Approach to Interstitial Lung Diseases Associated with Connective Tissue Diseases

Ana C. Zamora
1   Division of Pulmonary and Sleep Medicine, Department of Medicine, Mayo Clinic Arizona, Scottsdale, Arizona
,
Lewis J. Wesselius
1   Division of Pulmonary and Sleep Medicine, Department of Medicine, Mayo Clinic Arizona, Scottsdale, Arizona
,
Michael B. Gotway
2   Division of Cardiothoracic Radiology, Department of Radiology, Mayo Clinic Arizona, Scottsdale, Arizona
,
Henry D. Tazelaar
3   Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic Arizona, Scottsdale, Arizona
,
Alejandro Diaz-Arumir
1   Division of Pulmonary and Sleep Medicine, Department of Medicine, Mayo Clinic Arizona, Scottsdale, Arizona
,
Vivek Nagaraja
4   Division of Rheumatology, Department of Medicine, Mayo Clinic Arizona, Scottsdale, Arizona
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Abstract

Interstitial lung disorders are a group of respiratory diseases characterized by interstitial compartment infiltration, varying degrees of infiltration, and fibrosis, with or without small airway involvement. Although some are idiopathic (e.g., idiopathic pulmonary fibrosis, idiopathic interstitial pneumonias, and sarcoidosis), the great majority have an underlying etiology, such as systemic autoimmune rheumatic disease (SARD, also called Connective Tissue Diseases or CTD), inhalational exposure to organic matter, medications, and rarely, genetic disorders. This review focuses on diagnostic approaches in interstitial lung diseases associated with SARDs. To make an accurate diagnosis, a multidisciplinary, personalized approach is required, with input from various specialties, including pulmonary, rheumatology, radiology, and pathology, to reach a consensus. In a minority of patients, a definitive diagnosis cannot be established. Their clinical presentations and prognosis can be variable even within subsets of SARDs.

Keywords

systemic autoimmune rheumatic diseases - interstitial lung diseases - screening - diagnosis - pulmonary function tests - high-resolution computed tomography - artificial intelligence


Publikationsverlauf

Artikel online veröffentlicht:
17. April 2024

© 2024. Thieme. All rights reserved.

Thieme Medical Publishers, Inc.
333 Seventh Avenue, 18th Floor, New York, NY 10001, USA

 

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