Chest
Volume 134, Issue 2, August 2008, Pages 358-367
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Original Research
High-Resolution CT
High-Resolution CT Scan Findings in Patients With Symptomatic Scleroderma-Related Interstitial Lung Disease

https://doi.org/10.1378/chest.07-2444Get rights and content

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.

Section snippets

Patients

The SLS was a prospective, randomized, double-blinded, controlled clinical trial that evaluated the effectiveness of oral cyclophosphamide vs placebo therapy in the treatment of active, symptomatic pulmonary alveolitis inflammation secondary to systemic sclerosis.12 Institutional board approval was obtained at all 13 clinical centers; informed consent and Health Insurance Portability and Accountability Act compliance were obtained from all participants recruited over a period of 3 to 4 years.

Patient Characteristics

Of the 267 subjects screened for eligibility, 162 were SLS randomized, including 114 female subjects and 46 male subjects. The mean (± SD) age was 51 ± 12.3 years; 40% of subjects (65 of 162 subjects) had lcSSc, and 60% of subjects (97 of 162 subjects) had dcSSc (Table 2). A total of 79 subjects were randomized to the placebo control group. Of the 105 screened-eligible subjects who failed randomization (for a variety of reasons), 68 underwent HRCT scanning and 53 underwent BAL. Figure 5

Discussion

This study confirms that thoracic HRCT scanning plays a central role in the detection and characterization of lung involvement in SSc patients.1, 3, 8, 9, 10, 24, 25 The reported hallmark features5, 26, 27, 28, 29 of SSc-related lung involvement are similar to those of idiopathic NSIP. This association has also been reported5, 6, 30 in radiology-pathology correlations in surgical biopsy specimens. Several studies11, 31, 32, 33, 34, 35 have summarized the results of SSc CT findings (Table 7).

In

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    This study was funded by National Institutes of Health (grant UO1 HL 60748) and research grants U01 HL60587-01A1 and R01 HL072424, from the National Heart, Lung, and Blood Institute, Bethesda, MD.

    The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

    Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).

    A list of SLS participants is given in the Appendix.

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