Diagnostic Approach to Interstitial Lung Diseases Associated with Connective Tissue Diseases

Author:

Zamora Ana C.1,Wesselius Lewis J.1,Gotway Michael B.2,Tazelaar Henry D.3,Diaz-Arumir Alejandro1,Nagaraja Vivek4

Affiliation:

1. Division of Pulmonary and Sleep Medicine, Department of Medicine, Mayo Clinic Arizona, Scottsdale, Arizona

2. Division of Cardiothoracic Radiology, Department of Radiology, Mayo Clinic Arizona, Scottsdale, Arizona

3. Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic Arizona, Scottsdale, Arizona

4. Division of Rheumatology, Department of Medicine, Mayo Clinic Arizona, Scottsdale, Arizona

Abstract

AbstractInterstitial 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.

Publisher

Georg Thieme Verlag KG

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