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High-Resolution CT Scan Findings in Patients With Symptomatic Scleroderma-Related Interstitial Lung Disease^*

Jonathan G. Goldin, MB ChB, PhD; David A. Lynch, MD; Diane C. Strollo, MD; Robert D. Suh, MD; Dean E. Schraufnagel, MD, FCCP; Philip J. Clements, MD, MPH; Robert M. Elashoff, PhD; Daniel E. Furst, MD; Sarinnapha Vasunilashorn, BS; Michael F. McNitt-Gray, PhD; Mathew S. Brown, PhD; Michael D. Roth, MD, FCCP; Donald P. Tashkin, MD, FCCP; for the Scleroderma Lung Study Research Group

^* From the Departments of Radiological Sciences (Drs. Goldin, Suh, Vasunilashorn, McNitt-Gray, and Brown), Rheumatology (Drs. Clements and Furst), Biomathematics (Dr. Elashoff), and Pulmonary and Critical Care Medicine (Drs. Roth and Tashkin), University of California at Los Angeles, Los Angeles, CA; the Department of Radiology (Dr. Lynch), National Jewish Medical and Research Center, Denver, CO; the Department of Radiology (Dr. Strollo), University of Pittsburgh Medical Center, Pittsburgh, PA; and the Department of Pulmonary, Critical Care and Sleep Medicine (Dr. Schraufnagel), University of Illinois at Chicago, Chicago, IL. A list of SLS participants is given in the ^Appendix.

Correspondence to: Jonathan Goldin, MD, PhD, David Geffen School of Medicine at UCLA, Department of Radiology, 10833 Le Conte Ave, B2-165 CHS, Los Angeles, CA 90095-1721; e-mail: jgoldin{at}mednet.ucla.edu

Abstract

Background: Lung disease has become the leading cause of mortality and morbidity in scleroderma (SSc) patients. The frequency, nature, and progression of interstitial lung disease seen on high-resolution CT (HRCT) scans in patients with diffuse SSc (dcSSc) compared with those with limited SSc (lcSSc) has not been well characterized.

Methods: Baseline HRCT scan images of 162 participants randomized into a National Institutes of Health-funded clinical trial were compared to clinical features, pulmonary function test measures, and BAL fluid cellularity. The extent and distribution of interstitial lung disease HRCT findings, including pure ground-glass opacity (pGGO), pulmonary fibrosis (PF), and honeycomb cysts (HCs), were recorded in the upper, middle, and lower lung zones on baseline and follow-up CT scan studies.

Results: HRCT scan findings included 92.9% PF, 49.4% pGGO, and 37.2% HCs. There was a significantly higher incidence of HCs in the three zones in lcSSc patients compared to dcSSc patients (p = 0.034, p = 0.048, and p = 0.0007, respectively). The extent of PF seen on HRCT scans was significantly negatively correlated with FVC (r = – 0.22), diffusing capacity of the lung for carbon monoxide (r = – 0.44), and total lung capacity (r = – 0.36). A positive correlation was found between pGGO and the increased number of acute inflammatory cells found in BAL fluid (r = 0.28). In the placebo group, disease progression was assessed as 30% in the upper and middle lung zones, and 45% in the lower lung zones. No difference in the progression rate was seen between lcSSc and dcSSc patients.

Conclusions: PF and GGO were the most common HRCT scan findings in symptomatic SSc patients. HCs were seen in more than one third of cases, being more common in lcSSc vs dcSSc. There was no relationship between progression and baseline PF extent or lcSSc vs dcSSc.

Trial registration: Clinicaltrials.gov Identifier: NCT00004563 [ClinicalTrials.gov] .

Key Words: CT scan • lung disease • scleroderma