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1University of California at Los Angeles, Department of Radiological Sciences, Los Angeles, California 2National Jewish Medical and Research Center, Department of Radiology, Denver, Colorado 3University of Pittsburgh Medical Center, Department of Radiology, Pittsburgh, Pennsylvania 4University of Illinois at Chicago, Department of Pulmonary, Critical Care and Sleep Medicine Chicago, Illinois 5University of California at Los Angeles, Department of Rheumatology, Los Angeles, California 6University of California at Los Angeles, Department of Biomathematics, Los Angeles, California 7University of California at Los Angeles, Department of Pulmonary and Critical Care Medicine, Los Angeles, California
jgoldin{at}mednet.ucla.edu
Abstract
Background Lung disease has become the leading cause of mortality and morbidity in scleroderma patients. The frequency, nature, and progression of interstitial lung disease on high-resolution computed tomography (HRCT) in patients with diffuse (dcSSc) versus limited scleroderma (lcSSc) has not been well characterized.
MethodsBaseline HRCT images of 162 participants randomized into a NIH-funded clinical trial were compared to clinical features, pulmonary function test measures, and bronchoalveolar lavage (BAL) cellularity. The extent and distribution of interstitial lung disease HRCT findings–including pure ground glass opacity (pGGO), fibrosis (PF), and honeycomb cysts (HC)–were recorded in upper, middle, and lower lung zones on baseline and follow-up CT studies.
Results HRCT findings included 92.9% PF, 49.4% pGGO, and 37.2% HC. There was a significantly higher HC incidence in three zones lcSSc versus dcSSc patients (p=0.034, p=0,048, p= 0.0007). The HRCT PF extent was significantly negatively correlated with forced vital capacity (r=–0.22), carbon monoxide diffusing capacity (r=–0.44), and total lung capacity (r=–0.36). A positive correlation was found between pGGO and increased number of BAL acute inflammatory cells (r=0.28). In the placebo group, disease progression was assessed as 30% in upper and middle, and 45% in lower zones. No difference in progression rate was seen between lcSSc and dcSSc.
Conclusions PF and GGO were the most common HRCT findings in symptomatic scleroderma patients. HC was seen in more than one third of cases, being more common in lcSSc versus dcSSc. There was no relationship between progression and baseline PF extent or lcSSc versus dcSSc.
Key Words: Scleroderma Lung disease Computed tomography
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