High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease.
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.


BACKGROUND
Clinicaltrials.gov Identifier: NCT00004563.
Goldin
Jonathan G
JG
Department of Radiological Sciences, University of California at Los Angeles, Los Angeles, CA. Electronic address: jgoldin@mednet.ucla.edu.
Lynch
David A
DA
Department of Radiology, National Jewish Medical and Research Center, Denver, CO.
Strollo
Diane C
DC
Department of Radiology, University of Pittsburgh Medical Center, Pittsburgh, PA.
Suh
Robert D
RD
Department of Radiological Sciences, University of California at Los Angeles, Los Angeles, CA.
Schraufnagel
Dean E
DE
Department of Pulmonary, Critical Care and Sleep Medicine, University of Illinois at Chicago, Chicago, IL.
Clements
Philip J
PJ
Department of Rheumatology, University of California at Los Angeles, Los Angeles, CA.
Elashoff
Robert M
RM
Department of Biomathematics, University of California at Los Angeles, Los Angeles, CA.
Furst
Daniel E
DE
Department of Rheumatology, University of California at Los Angeles, Los Angeles, CA.
Vasunilashorn
Sarinnapha
S
Department of Radiological Sciences, University of California at Los Angeles, Los Angeles, CA.
McNitt-Gray
Michael F
MF
Department of Radiological Sciences, University of California at Los Angeles, Los Angeles, CA.
Brown
Mathew S
MS
Department of Radiological Sciences, University of California at Los Angeles, Los Angeles, CA.
Roth
Michael D
MD
Department of Pulmonary and Critical Care Medicine, University of California at Los Angeles, Los Angeles, CA.
Tashkin
Donald P
DP
Department of Pulmonary and Critical Care Medicine, University of California at Los Angeles, Los Angeles, CA.
D000328:Adult; D000368:Aged; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011653:Pulmonary Diffusing Capacity; D045743:Scleroderma, Diffuse / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology; D045745:Scleroderma, Limited / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology; D013147:Spirometry; D014057:Tomography, X-Ray Computed; D014109:Total Lung Capacity
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide