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Updated: Feb 4, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Radiological images of interstitial pneumonia in mixed connective tissue disease compared with scleroderma and
Yumie Yamanaka1, Tomohisa Baba1, Eri Hagiwara1
1Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan.
Objective:
Little has been reported on the radiological and pathological findings of interstitial pneumonia in mixed connective tissue disease (MCTD). There may be possible difference in treatment response and prognosis between the imaging patterns of systemic sclerosis (SSc)-like and polymyositis/dermatomyositis (PM/DM)-like. The purpose of this study was to examine whether the radiological images of interstitial pneumonia in MCTD presented SSc-like or PM/DM-like pattern, and to assess whether the imaging patterns corresponded to clinical and pathological features.
Materials And Methods:
This retrospective study included 29 patients with interstitial pneumonia who underwent surgical lung biopsy; 10 with SSc, 10 with PM/DM, and 9 with MCTD. High resolution computed tomography (HRCT) images were classified as SSc, PM/DM, or the other pattern by two radiologists independently without clinical information. The pathology of the lung specimens from MCTD patients were evaluated and compared with the imaging pattern.
Results:
The concordance rate between clinical diagnosis and radiological pattern was 100% in SSc patients, and 80% in PM/DM patients. Among patients with MCTD, imaging patterns were classified as SSc pattern in 4 (MCTD-SSc), PM/DM pattern in 4 (MCTD-PM/DM) and other in one. The imaging patterns did not always correlate with the clinical findings in MCTD patients. Pathologically, plasma cell infiltration and organizing pneumonia were relatively more frequent in MCTD-PM/DM, and smooth muscle hyperplasia was relatively more frequent in MCTD-SSc.
Conclusion:
HRCT images in MCTD patients can be classified as SSc pattern or PM/DM pattern. MCTD-SSc and MCTD-PM/DM were corresponded to similar pathological findings of SSc and PM/DM.
Insights
Interstitial pneumonia in mixed connective tissue disease (MCTD) shows radiological patterns similar to systemic sclerosis (SSc) or polymyositis/dermatomyositis (PM/DM). These imaging patterns correlate with distinct pathological findings, aiding in diagnosis and prognosis.
Area of Science:
- Radiology
- Pathology
- Rheumatology
Background:
- Interstitial pneumonia is a known complication in connective tissue diseases.
- Radiological and pathological findings of interstitial pneumonia in mixed connective tissue disease (MCTD) are not well-characterized.
- Distinct imaging patterns may influence treatment and prognosis in MCTD-related interstitial pneumonia.
Purpose of the Study:
- To determine if interstitial pneumonia in MCTD presents with systemic sclerosis (SSc)-like or polymyositis/dermatomyositis (PM/DM)-like radiological patterns.
- To assess the correlation between these imaging patterns and clinical/pathological features in MCTD patients.
Main Methods:
- Retrospective analysis of 29 patients with interstitial pneumonia who underwent surgical lung biopsy (10 SSc, 10 PM/DM, 9 MCTD).
- High-resolution computed tomography (HRCT) images were independently classified by radiologists into SSc, PM/DM, or other patterns.
- Pathological evaluation of lung specimens from MCTD patients was compared with imaging patterns.
Main Results:
- Radiological patterns showed 100% concordance with clinical diagnosis in SSc and 80% in PM/DM.
- In MCTD, 4 patients showed an SSc pattern (MCTD-SSc), 4 showed a PM/DM pattern (MCTD-PM/DM), and 1 showed another pattern.
- Imaging patterns in MCTD did not consistently correlate with clinical findings; pathological differences included more plasma cell infiltration in MCTD-PM/DM and smooth muscle hyperplasia in MCTD-SSc.
Conclusions:
- High-resolution computed tomography (HRCT) can classify interstitial pneumonia in MCTD into SSc or PM/DM patterns.
- The MCTD-SSc and MCTD-PM/DM subgroups exhibit pathological findings similar to those seen in SSc and PM/DM, respectively.
- Radiological classification aids in understanding the pathological basis of interstitial pneumonia in MCTD.
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