CT Morphologic Characteristics and Variant Patterns of Interstitial Pulmonary Fibrosis in Systemic Lupus Erythematosus

PURPOSE: To assess CT features of pulmonary fibrosis in patients with systemic lupus erythematosus (SLE) and to assess the presence of several distinctive patterns of fibrosis associated with connective tissue disease. MATERIALS AND METHODS: A cross-sectional retrospective analysis was performed. An institutional clinical database was queried for the years of 2005–2015 to identify CT examination reports of patients with SLE and fibrotic lung disease, which yielded 50 patients (median age, 49 years; age range, 22–71 years; 46 women). CT examination reports were scored by two subspecialty thoracic radiologists using a standard multilevel semiquantitative system. Readers noted the presence or absence of several recently described CT signs of variant patterns of fibrosis in connective tissue disease (the “anterior upper lobe,” “straight-edge,” and “exuberant honeycombing” signs), as well as two other morphologic characteristics (an “island-like” appearance of areas of well-defined fibrosis with angular margins surrounded by normal lung and confluent regions of lucent lung destruction). RESULTS: The most common CT patterns were characterized as either fibrotic nonspecific interstitial pneumonia (38%, 19 of 50) or variant fibrosis (44%, 22 of 50). CT signs of variant fibrosis were identified by both readers in up to 62% of patients, with good κ agreement (0.44–0.64); the island-like sign (62%) and anterior upper lobe sign (52%) were most commonly observed. Pulmonary function test results showed correlations with several imaging findings but did not show correlations with CT signs of variant fibrosis. CONCLUSION: When present, pulmonary fibrosis in SLE often has a distinctive appearance and may also manifest as several variant fibrotic patterns. Keywords: CT, Lung © RSNA, 2021 See also the commentary by White in this issue.