Connective tissue disease-related interstitial lung disease (CTD-ILD) and interstitial lung abnormality (ILA): Evolving concept of CT findings, pathology and management.
The connective tissue diseases (CTDs) demonstrating features of interstitial lung disease (ILD) include systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), progressive systemic sclerosis (PSS), dermatomyositis (DM) and polymyositis (PM), ankylosing spondylitis (AS), Sjogren's syndrome (SS), and mixed connective tissue disease (MCTD). In RA patients in particular, interstitial lung abnormality (ILA) (of varying degrees; severe vs. mild) is reported to occur in approximately 20-60 % of individuals and CT disease progression occurs in approximately 35-45 % of them. The ILAs have been associated with a spectrum of functional and physiologic decrement. The identification of progressive ILA may enable appropriate surveillance and the commencement of treatment with the goal of improving morbidity and mortality rates of established RA-ILD. Subpleural distribution and higher baseline ILA/ILD extent were risk factors associated with disease progression. At histopathologic analysis, connective tissue disease-related interstitial lung diseases (CTD-ILDs) are diverse and include nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), apical fibrosis, diffuse alveolar damage (DAD), and lymphoid interstitial pneumonia (LIP). Even though proportions of ILDs vary, NSIP pattern accounts for a large proportion, especially in PSS, DM/PM and MCTD, followed by UIP pattern. Evidence has been published that treatment of subclinical CT lung abnormalities showing a tendency to progress to ILD may stabilize the CT alterations. The identification of subclinical lung abnormalities can be appropriate in the management of the disease and CT appears to be the gold standard for the evaluation of lung parenchyma.
['Yoo|Hongseok|H|', 'Hino|Takuya|T|', 'Han|Joungho|J|', 'Franks|Teri J|TJ|', 'Im|Yunjoo|Y|', 'Hatabu|Hiroto|H|', 'Chung|Man Pyo|MP|', 'Lee|Kyung Soo|KS|']
Interstitial lung disease Interstitial lung abnormality Connective tissue disease 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 IPAF, Interstitial pneumonitis with autoimmune features IPF, Idiopathic pulmonary fibrosis MCTD, Mixed connective tissue disease NSIP, Nonspecific Interstitial Pneumonia OP, Organizing pneumonia PM, Polymyositis PSS, Progressive Systemic Sclerosis RA, Rheumatoid Arthritis SLE, Systemic Lupus Erythematosus SS, Sjogren’s Syndrome UCTD, Undifferentiated Connective Tissue Disease UIP, Usual Interstitial Pneumonia