Connective tissue disease-related interstitial lung disease (CTD-ILD) and interstitial lung abnormality (ILA): Evolving concept of CT findings, pathology and management. (2022)

• Record Type: Journal Article
• Authors: Yoo, Hongseok; Hino, Takuya; Hwang, Jiwon; Franks, Teri J.; Han, Joungho; Im, Yunjoo; Lee, Ho Yun; Chung, Man Pyo; Hatabu, Hiroto; Lee, Kyung Soo
• Is Part Of: European journal of radiology open. Volume 9(2022)
• Publication Date: 2022
• Subjects: CTD Connective tissue disease -- CTD-ILD Connective tissue disease-related interstitial lung disease -- DM Dermatomyositis -- IIP Idiopathic interstitial pneumonia -- ILA Interstitial lung abnormality -- ILD Interstitial lung disease -- IPF Idiopathic pulmonary fibrosis -- IPAF Interstitial pneumonitis with autoimmune features -- LIP Lymphoid interstitial pneumonia -- MCTD Mixed connective tissue disease -- NSIP Nonspecific interstitial pneumonia -- OP Organizing pneumonia -- PM Polymyositis -- RA Rheumatoid arthritis -- SLE Systemic lupus erythematosus -- SS Sjogren's syndrome -- SSc Systemic sclerosis -- UCTD Undifferentiated connective tissue disease -- UIP Usual interstitial pneumonia; Connective tissue disease -- Interstitial lung abnormality -- Interstitial lung disease; Medical radiology -- Periodicals; 616.075705
• DOI: 10.1016/j.ejro.2022.100419
• Languages: English
• ISSNs: 2352-0477

Abstract

Abstract: Connective tissue diseases (CTDs) demonstrating features of interstitial lung disease (ILD) include systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (SSc), dermatomyositis (DM) and polymyositis (PM), ankylosing spondylitis (AS), Sjogren syndrome (SS), and mixed connective tissue disease (MCTD). On histopathology of lung biopsy in CTD-related ILDs (CTD-ILDs), multi-compartment involvement is an important clue, and when present, should bring CTD to the top of the list of etiologic differential diagnoses. Diverse histologic patterns including nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia, apical fibrosis, diffuse alveolar damage, and lymphoid interstitial pneumonia can be seen on histology in patients with CTD-ILDs. Although proportions of ILDs vary, the NSIP pattern accounts for a large proportion, especially in SSc, DM and/or PM and MCTD, followed by the UIP pattern. In RA patients, interstitial lung abnormality (ILA) is reported to occur in approximately 20–60% of individuals of which 35–45% will have progression of the CT abnormality. Subpleural distribution and greater baseline ILA involvement are risk factors associated with disease progression. Asymptomatic CTD-ILDs or ILA patients with normal lung function and without evidence of disease progression can be followed without treatment. Immunosuppressive or antifibrotic agents for symptomatic and/or fibrosing CTD-ILDs can be used in patients who require treatment.