J Rheum Dis.  2024 Jan;31(1):3-14. 10.4078/jrd.2023.0042.

Radiologic approach and progressive exploration of connective tissue disease-related interstitial lung disease: meeting the curiosity of rheumatologists

• Jeon H
• Nam BD
• Yoon CH
• Kim HS

• KMID: 2549631
• DOI: http://doi.org/10.4078/jrd.2023.0042

Abstract

Interstitial lung disease (ILD) is often observed in connective tissue diseases (CTDs), frequently in rheumatoid arthritis, systemic sclerosis, primary Sjögren’s syndrome, and inflammatory myositis. Early detection of ILDs secondary to rheumatic diseases is important as timely initiation of proper management affects the prognosis. Among many imaging modalities, high-resuloution computed tomography (HRCT) serves the gold standard for finding early lung inflammatory and fibrotic changes as well as monitoring afterwards because of its superior spatial resolution. Additionally, lung ultrasound (LUS) and magnetic resonance imaging (MRI) are the rising free-radiation imaging tools that can get images of lungs of CTD-ILD. In this review article, we present the subtypes of ILD images found in each CTD acquired by HRCT as well as some images taken by LUS and MRI with comparative HRCT scans. It is expected that this discussion would be helpful in discussing recent advances in imaging modalities for CTDILD and raising critical points for diagnosis and tracing of the images from the perspective of rheumatologists.

Keyword

Interstitial lung disease; Connective tissue diseases; Pulmonary fibrosis; Rheumatology

Figure

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  Figure 1 Effect of being in the supine position on high-resuloution computed tomography imaging. In the supine position, the effect of gravity on lung aeration and distribution of pulmonary abnormalities can lead to underestimation of lung abnormalities. (A) Supine expiratory scans may show increased opacity in dependent lung regions, limiting the assessment of interstitial abnormalities. (B) The prone scan demonstrates a recovery of aerated lung parenchyma in the lower lobes with a small area of residual consolidation. Subpleural reticulations are well delineated, suggesting early interstitial lung fibrosis (arrows).

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  Figure 2 Honeycombing distribution showing the usual interstitial pneumonia (UIP) pattern. The image illustrates a predominant distribution of honeycombing with traction in the basal and subpleural regions. The presence of cystic spaces resembling honeycombs, primarily located in the lower regions of the lungs and adjacent to the pleural surfaces, indicates fibrotic changes characteristic of the UIP pattern. (A) Axial view, (B) coronal view.

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  Figure 3 High-resuloution computed tomography (HRCT) in a rheumatoid arthritis (RA) patient. This image shows HRCT findings in a 60-year-old female patient with RA. The presence of honeycombing and traction bronchiectasis with upper to middle lung distribution is highly suggestive of connective tissue diseases-associated interstitial lung disease. (A) Axial view, (B) coronal view.

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  Figure 4 High-resuloution computed tomography (HRCT) of a systemic sclerosis patient. This image depicts HRCT findings in a 44-year-old female patient with systemic sclerosis. Peripheral and lower lung predominant reticulation, ground glass opacity, and traction bronchiolectasis with architectural distortion are observed. Immediate subpleural sparing is well visualized. A coronal reformatted scan highlights the presence of traction bronchiolectasis. In addition, esophageal dilation is noted in the upper thoracic esophagus. These findings are consistent with thoracic involvement in systemic sclerosis, with little evidence of honeycombing. (A) Coronal view, (B, C) axial view.

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  Figure 5 High-resuloution computed tomography (HRCT) of a patient with primary Sjögren’s syndrome (pSS). HRCT findings in a 61-year-old female patient with pSS and biopsy-proven lymphocytic interstitial pneumonia. (A) The image reveals subpleural or peribronchial air space consolidation with ground glass opacity, along with multiple noncalcific nodules in both lungs. (B) Nodular or subpleural consolidations with septal thickening and traction bronchiolectasis are observed in the lower lobes.

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  Figure 6 High-resuloution computed tomography (HRCT) of a patient with polymyositis (PM). HRCT findings in a 67-year-old male patient diagnosed with PM. The image shows areas of ground glass opacity and reticulations with peribronchovascular and lower lung predominance. The HRCT pattern suggests a differential diagnosis of nonspecific interstitial pneumonia and organizing pneumonia patterns. (A) Axial view, (B) coronal view.

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  Figure 7 Subdivision of chest areas and scanning views in a lung ultrasound. (A) The image illustrates the subdivision of chest areas for basic scanning views in lung ultrasound. (B, C) A complete lung ultrasound examination includes transverse and longitudinal scans through the anterior, lateral, and posterior lungs. AAL: anterior axillary line, PAL: posterior axillary line, PSL: parasternal line [29,34,37].

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  Figure 8 Characteristic findings in interstitial lung disease (ILD) detected by lung ultrasound. Lung ultrasound can reveal characteristic findings in ILD. However, the definitive diagnosis of ILD typically requires a combination of clinical assessment, imaging studies (such as high-resuloution computed tomography [HRCT]), and sometimes a lung biopsy. (A) Traction bronchiectasis and parenchymal changes of upper lung in HRCT (arrows). (B) Corresponding changes of lung ultrasound presented by B-lines (arrows).

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  Figure 9 Characteristic findings in interstitial lung disease (ILD) detected by lung magnetic resonance imaging (MRI). In ILD, lung MRI can reveal several characteristic findings. (A) Single-shot fast spin echo T2-weighted MRI demonstrates high signal intensity in the area of lung fibrosis (arrows). (B) Fat-saturated nonenhanced T1-weighted MRI shows suspicious reticulations in both dorsal lungs. (C, D) Ultra-short echo-time sequence images provide more delineated reticulations and a microcystic appearance (traction bronchiolectasis and honeycombing cysts) in both lower lobes (arrows). (E, F) Comparative high-resuloution computed tomography scans corresponding to (C) and (D).

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