Pictorial review of intrathoracic manifestations of progressive systemic sclerosis

Abstract

Intra-thoracic manifestations of progressive systemic sclerosis (PSS) are not well known particularly the imaging features, which forms the basis of accurate and timely diagnosis. The aim of this study is to familiarize the physicians and radiologists with these features. The diagnosis can remain elusive because of the non-specific nature of symptoms which mimic many common conditions. Thus, the diagnosis of PSS can be missed leading to continuous morbidity if the correct imaging is not pursued. The authors examined the records of rheumatology patient referrals of over a 5 year period. A hundred and seventy patients with systemic sclerosis and mixed connective tissue disorders were chosen for detailed study of the imaging available, which form the basis of this review. The images included conventional chest radiographs, digital radiographs computed radiography (CT) and high resolution computed tomography (HRCT). Where applicable computed pulmonary angiography (CTPA) and radionuclide scans were also interrogated.

Keywords: Imaging; mixed connective tissue disorders; progressive systemic sclerosis; systemic sclerosis.

Figure 1

PSS is considered limited when involvement is restricted to the distal extremities and face. The limited form is associated with a lower risk of visceral involvement; however, pulmonary hypertension is more common

Figure 2

46 year old male with PSS. (a and b) CXR shows volume loss and basal reticular changes suggestive of interstitial lung disease. (c) Axial CT scan shows basal reticular changes, ground glass opacification and traction bronchiectasis in a pattern that is suggestive of NSIP (nonspecific interstitial pneumonia). Note the dilated lower esophagus suggestive of dysmobilty. (d) Prone high resolution CT shows persistent ground glass opacities, reticular changes and traction bronchiectasis, confirming the presence of fibrosis. (e) Coronal CT scan shows the basal distribution of fibrotic changes in PSS

Figure 3

55 year old female with PSS. (a) Axial supine HRCT shows areas of honeycombing and traction bronchiectasis in the left upper lobe in a patient with proved PSS. (b) Prone CT in the same patient confirms the persistence of the fibrotic lesions

Figure 4

48-year-old female with PSS. (a) Axial CT scan shows the presence of volume loss and patchy areas of ground glass opacities in the lower lobes. There is also esophageal dilation, indicating dysmotility. (b) Coronal CT scan showsscattered areas of ground glass opacities associated with volume loss and traction bronchiectasis in a patient with scleroderma. (c) Axial CT scan with soft tissue window shows esophageal dilation. (d) Axial CT scan at a higher level and in soft tissue window show dilated pulmonary arteries, due to pulmonary hypertension

Figure 5

58-year-old male with PSC. (a) Chest radiograph shows volume loss of lungs and bilateral diffuse, predominantly peripheral and basilar areas of reticular opacities indicative of fibrosis. (b) Axial CT scan shows basal reticular changes in a peripheral distribution along with traction bronchiectasis and architectural distortion. There is also esophageal dilation (c) Coronal CT shows basal and peripheral ground glass opacities, reticular changes, architectural distortion and tractionbronchiectasis. (d) Esophagogram shows dilated esophagus due to dysmotility

Figure 6

A PA chest radiograph and axial CT scans in 51-year old male with PSS showing basal fibrotic changes (CXR) a dilated thickened lower esophagus (red arrow), patchy subpleural ground glass, mild bronchial wall thickening, cystsand an air bronchogram (yellow)

Figure 7

(a) A PA chest radiograph on 57-year old lady with PSS showing incidental dextrocardia. (b) Axial CT scans showing bilateral reticular basal shadowing due to pulmonary fibrosis

Figure 8

Pulmonary hypertension in patient with PSS. (a) Black blood, T2 weighted MRI, image shows dilated pulmonary arteries. (b) Axial steady state free precession (SSFP) image shows dilated pulmonary arteries due to pulmonary arterial hypertension

Figure 9

PA chest radiograph on a 67-year female with PSS showing minor bronchial wall thickening at the lung bases also shown on axial CT scans, also note the minor lung fibrosis, and fluid within the lower esophagus

Figure 10

PA chest radiograph and axial CT scan of a 52-year old male patient with PSS that presented with breathlessness, showing extensive pleural thickening. An old unrelated rib fracture is noted on the right. Also note coarse subpleural fibrosis

Figure 11

HRCT of 53 year old man, with PSS showing bilateral basal pleural thickening and minor bronchiectasis. Also note the ground glass appearance and air within the esophagus due to gastroesophageal reflux

Figure 12

Axial CT showing a soft tissue density mass in posterior right lower dorsal paravertebral gutter in a patient with known PSS. Note subpleural fibrosis, subpleural cysts/bullae and minor bronchiectasis. Biopsy revealed an adenocarcinoma

Figure 13

Axial CT, at the level of the pulmonary arteries on a patient with known PSS showing extensive mediastinal lymphadenopathy. Other causes of adenopathy were excluded on mediastinoscopy and biopsy. Note the thickened and dilated esophagus.

Figure 14

Axial CT scans, on another male patient with known PSS showing mediastinal lymphadenopathy (red arrow). Note the dilated esophagus

Figure 15

Cardiac involvement in 53 year old female with PSS. (a) Short-axis view of delayed enhancement MRI shows patchy areas of linear mid myocardial enhancement (straight arrows) in the mid ventricular septum and the lateral wall. There is also a moderate sized circumferential pericardial effusion (curved arrow). (b) 3 chamber delayed enhancement MRI shows patchy areas of linear mid myocardial enhancement (straight arrows) and a circumferential pericardial effusion (curved arrow)

Figure 16

Pericarditis in 42-year-old male patient with PSS. (a) 4 chamber black blood T1 weighted image shows thickening of pericardium, more prominently surrounding the lateral wall of the left ventricle (arrow), consistent with pericarditis. (b) 4-chamber delayed enhancement image shows circumferential delayed enhancement of the pericardium, consistent with pericardial inflammation

Figure 17

Pericardial effusion in 49-year-old male with PSS. (a) 4 chamber blackblood double inversion recovery sequence areas of loculated pericardial effusion (straight arrows) surrounding the heart. (b) Short axis STIR image in the same patient shows heterogeneous high signal in the pericardial fluid (straight arrow). There is also myocardial edema (arrowheads) in the ventricle indicating myocardial involvement

Figure 18

CT images showing major but non-occlusive pulmonary embolism in a patient with known PSS. Figure a and b are axial and coronal CT scans showing multiple pulmonary emboli (red arrows). Figures c and d are from the same patient showing pulmonary emboli associated with pulmonary fibrosis

Figure 19

(a) PA chest radiographs of a patient with known PSS that presented with a weight loss of 3.2 kg and left shoulder pain. The two radiographs are 6 months apart. Figure b show a suggestion of a mass lesion at the rightcardiophrenic angle (red arrow)

Figure 20

a and b are from the same patient as in the previous two figures confirming a mass at the right cardiophrenic angle associated with pulmonary fibrosis and destruction of the left scapular blade (red arrow) highly suggestive of a metastatic deposit. The patient is awaiting a biopsy for tissue typing

Figure 21

Coronal images of a patient with MCTD and peripheral neuropathy. This patient had paradoxical movement of the diaphragm on fluoroscopy and non-existent thoracic excursion in keeping with neuropathy. Note the extensive pulmonary fibrosis, multiple cysts/bullae, pleural thickening and the elevated right hemidiaphragm

Figure 22

(a) Axial CT scan shows basal fibrotic changes (curved arrows) and a welldefined mass in the anterior aspect of the left upper lobe (straight arrow). (b) FDG-PET image shows high uptake in the lesion in the left upper lobe (straight arrow), indicative of malignancy. There is also high uptake in left hilar lymph node (curved arrow), indicative of metastasis. (c) Fused PET-CT image shows high uptake in the tumor and left hilar lymph node. (d) Fused PET-CT image shows high uptake in the lymph node. The tumor was biopsy proven to be moderately differentiated adenocarcinoma

Figure 23

Pre and post contrast axial CT scans showing the right posterior gutter mass and mediastinal lymphadenopathy. Note the dilated esophagus (the same patient as in the previous figure). Also see the following three figures showing PET/CT images

Figure 24

PET/CT showing biopsy proved, adenocarcinoma, associated with PSS. Note the intense radionuclide uptake within the tumor and mediastinal lymph nodes and a tiny metastatic deposit within segment 2 of the liver. The images are from the same patient as in the previous two figures. Also note the dilated and thickened lower esophagus

Figure 25

PET/CT coronal and axial images showing biopsy proved, adenocarcinoma, associated with PSS. Note the intense radionuclide uptake within the tumor and mediastinal lymph nodes and a tiny metastatic deposit within segment 2 of the liver. The images are from the same patient as in the previous three figures. Also note the dilated and thickened lower esophagus

Figure 26

PET/CT axial images showing biopsy proved adenocarcinoma, associated with PSS. Note the intense radionuclide uptake within metastatic mediastinal lymph nodes. The images are from the same patient as in the previous four figures