Systemic lupus erythematosus CT

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Pulmonary

Pulmonary hypertension (right ventricular prominence, or loud P2)

• ECG-gated CT pulmonary angiography shows:

• • Right ventricular hypertrophy: defined as wall thickness of >4 mm
  • Straightening or bowing (towards the left ventricle) of the interventricular septum
  • Right ventricular dilatation (a right ventricle–to–left ventricle diameter ratio of more than 1:1 at the midventricular level on axial images)
  • Decreased right ventricular ejection fraction
  • Ancillary features
    • dilatation of the inferior vena cava and hepatic veins
    • pericardial effusion

• Enlarged pulmonary trunk (measured at pulmonary artery bifurcation on an axial slice vertical to its long axis)

• Enlarged pulmonary arteries
• Mural calcification in central pulmonary arteries
• Centrilobular ground-glass nodules
• Neovascularity: tiny serpiginous intrapulmonary vessels that often emerge from centrilobular arterioles but do not conform to usual pulmonary arterial anatomy

Pulmonary fibrosis

• Honeycombing: Fibrotic cystic changes
• Traction bronchiectasis: Dilatation of bronchi and bronchioles within fibrotic lung tissue
• Lung architectural distortion
• Reticulation
• Interlobular septal thickening

Shrinking lung

• Reduced lung volumes with diaphragmatic elevation +/- occasional basal atelectasis but without any major parenchymal lung or pleural disease 
• Pulmonary infarction 
• Wedge-shaped (less often rounded) juxtapleural opacification (Hampton hump) without air bronchograms 
• Consolidation with an specific pattern called "bubbly consolidation" that is the co-existing non-infarcted lung parenchyma side-by-side with infarcted lung in the same lobule 
• Cavitation

Pneumonitis

unilateral or bilateral patchy and focal consolidation typically in the lung bases

accompanying pleural effusion may be present

Cardiac

Pericarditis

Noncalcified pericardial thickening with pericardial effusion is suggestive of acute pericarditis

Cardiomyopathy (ventricular dysfunction)

Valvular disease (diastolic murmur, or systolic murmur >3/6)

Pericarditis

Abnormal thickening and enhancement of the pericardium as well as a pericardial effusion in contrast-enhanced chest CT

neurology

CT scans are useful for detecting structural and focal abnormalities (such as infarcts/hypodense areas, hemorrhage, tumors, cerebral calcification, abscess, and basilar meningitis) [38]. Brain atrophy has been noted in some patients; this finding has been thought by some (but disputed by others) to reflect the effects of steroid therapy [6] or age [4]. We have seen brain atrophy out of proportion to a patient's age, and prior to steroid therapy.

Abdominal computed tomography (CT) scan fi ndings compatible with mesenteric vasculitis include prominence of mesenteric vessels with a comb-like appearance supplying dilated bowel loops, small bowel thickening and ascites. Excessive fatty infi ltration (steatosis) in liver/

Gastrointestinal

Pancreatitis

• Peripancreatic edema
• Phlegmon formation
• Mesenteric fatty infiltration around the pancreas
• Glandular enlargement

Bowel ischemia due to mesentric vascuitis

• Ascites
• Dilated bowel
• Mural thickening
• Abnormal wall enhancement
• Mesentric vessel engorgement

Organ	Disease	Description	CT	MRI	SONO
Gastrointestinal system	Dysphagia	Barium swallow/esophagography Oesophageal stricture Peptic strictures that appear as smooth, tapered narrowing in the distal esophagus Esophageal dilatation
	Intestinal pseudo-obstruction	dilated bowel loops with or without the presence of fluid levels Erect chest radiographs for perforation evaluating	dilated bowel loops with or without the presence of fluid levels a distinct transition point where bowel calibre changes from normal to abnormal dilated bowel loops proximal to the transition point small bowel >3.5 cm large bowel >5 cm collapsed or normal calibre bowel distal to the transitional point bowel wall thickening Obstruction: pneumoperitoneum indicating perforation bowel ischaemia
	Hepatitis		Hepatic granulomas Nonspecific, ranging from normal to hepatomegaly and cirrhosis. Discrete, sharply defined nodular lesions within the liver	nodules ranging around 0.5-4.5 cm in diameter  T2: nonspecific, increased periportal oedema 4 MRCP: primary sclerosing cholangitis (PSC) should be excluded
	Acute pancreatitis		Abnormalities that may be seen in the pancreas include: typical findings focal or diffuse parenchymal enlargement changes in density because of oedema indistinct pancreatic margins owing to inflammation surrounding retroperitoneal fat stranding liquefactive necrosis of pancreatic parenchyma lack of parenchymal enhancement often multifocal infected necrosis difficult to distinguish from aseptic liquefactive necrosis the presence of gas is helpful FNA helpful abscess formation circumscribed fluid collection little or no necrotic tissues (thus distinguishing it from infected necrosis) haemorrhage high-attenuation fluid in the retroperitoneum or peripancreatic tissues	Contrast-enhanced MR is equivalent to CT in the assessment of pancreatitis. Abnormalities that may be seen in the pancreas include: typical findings focal or diffuse parenchymal enlargement changes in density because of oedema indistinct pancreatic margins owing to inflammation surrounding retroperitoneal fat stranding liquefactive necrosis of pancreatic parenchyma lack of parenchymal enhancement often multifocal infected necrosis difficult to distinguish from aseptic liquefactive necrosis the presence of gas is helpful FNA helpful abscess formation circumscribed fluid collection little or no necrotic tissues (thus distinguishing it from infected necrosis) haemorrhage high-attenuation fluid in the retroperitoneum or peripancreatic tissues	to identify gallstones as a possible cause diagnosis of vascular complications, e.g. thrombosis identify areas of necrosis which appear as hypoechoic regions
	Aotpsplenectomy	If heavily calcified, the splenic remnant may be visible in the left upper quadrant.	CT easily identifies the abnormally small and irregular splenic remnant, which is usually calcified.		Ultrasound will either not be able to demonstrate a spleen at all, or identify a small irregular and shadowing nodule in the splenic bed.
	Enteritis	The main feature of enteritis is small bowel wall thickening. Low density submucosal edema can usually be differentiated from higher density mural haemorrhage or infiltration. Dilatation or strictures may or may not be present, the later if chronic.
	Mesenteric vasculitis		The comb sign refers to the hypervascular appearance of the mesentery  This forms linear densities on the mesenteric side of the affected segments of small bowel, which give the appearance of the teeth of a comb.	The comb sign refers to the hypervascular appearance of the mesentery  This forms linear densities on the mesenteric side of the affected segments of small bowel, which give the appearance of the teeth of a comb.
	Acute cholecystitis		gallbladder distension gallbladder wall thickening mural or mucosal hyperenhancement pericholecystic fluid and inflammatory fat stranding enhancement of the adjacent liver parenchyma due to reactive hyperaemia tensile gallbladder fundus sign 7 fundus bulging the anterior abdominal wall	MR cholangiopancreatography (MRCP) may show an impacted stone in the gallbladder neck or cystic duct as a rounded filling defect.	gallbladder wall thickening (>3 mm) and pericholecystic fluid  Positive Murphy sign gallbladder distension
Pulmonary involvement	Pleural effusion	A lateral decubitus film can visualise small amounts of fluid layering against the dependent parietal pleura. PA and AP CXR: blunting of the costophrenic angle blunting of the cardiophrenic angle fluid within the horizontal or oblique fissures mediastinal shifts with large amounts of fluid	May be associated with thickening of the pleura Fluid density		echo-free space between the visceral and parietal pleura
	Respiratory muscle dysfunction	elevated hemidiaphragms at chest radiography linear atelectasis and an ill-defined juxtadiaphragmatic areas of increased opacity  Wiedemann HP, Matthay RA. Pulmonary manifestations of collagen vascular diseases.Clin Chest Med 1989; 10:677-696
	Acute pneumonitis	A rare and fulminant form of diffuse lung injury that generally occurs in previously healthy individuals and has a rapid onset with fever, cough, and shortness of breath.
	Pulmonary hemorrhage	Patchy bilateral and acinar areas of increased opacity, predominantly in the lower lungs
	Pulmonary hypertension	Elevated cardiac apex due to right ventricular hypertrophy enlarged right atrium prominent pulmonary outflow tract enlarged pulmonary arteries pruning of peripheral pulmonary vessels
	Pulmonary emboli	Fleishner sign: enlarged pulmonary artery (20%) Hampton hump: peripheral wedge of airspace opacity and implies lung infarction (20%) Westermark sign: regional oligaemia and highest positive predictive value (10%) pleural effusion (35%) knuckle sign 11 Palla's sign17: enlarged right descending pulmonary artery	filling defects within the pulmonary vasculature with acute pulmonary emboli vascular CT signs include direct pulmonary artery signs complete obstruction partial obstruction eccentric thrombus calcified thrombus - calcific pulmonary emboli pulmonary arterial bands/pulmonary arterial webs 1,4-5 post stenotic dilatation signs related to pulmonary hypertension enlargement of main pulmonary arteries the peripheral pulmonary arteries in affected segments may be narrowed ref required pulmonary arterial calcification tortuous pulmonary vessels right ventricular enlargement/hypertrophy signs of systemic collateral supply enlargement of bronchial and nonbronchial systemic arteries Signs of chronic obstruction: webs or bands, intimal irregularities 3/ abrupt narrowing or complete obstruction of the pulmonary arteries 3 / “pouching defects” which are defined as chronic thromboembolism organised in a concave shape that “points” toward the vessel lumen parenchymal signs (often non-specific on their own): scars mosaic perfusion pattern focal ground-glass opacities bronchial anomalies The central filling defect from the thrombus is surrounded by a thin rim of contrast, appearing like the popular sweet, the polo mint
	Shrinking lung syndrome	small but clear lungs with diaphragmatic elevation basal atelectasis	reduced lung volumes with diaphragmatic elevation +/- occasional basal atelectasis but without any major parenchymal lung or pleural disease
Cardiac involvement	Cardiomegaly	Cardiac enlargement
	Mitral stenosis	cardiomegaly double right heart border (enlarged left atrium and normal right atrium) prominent left atrial appendage splaying of the subcarinal angle (>120 degrees)	valve thickening or leaflet fixation	mitral leaflet thickening reduced diastolic opening abnormal valve motion toward the left ventricular outflow tract
	Mitral regurgitation	frontal projection left atrial enlargement convexity or straightening of the left atrial appendage just below the main pulmonary artery (along left heart border) double density sign: the right side of the enlarged left atrium pushes into the adjacent lung and creates an addition contour superimposed over the right heart elevation of the left main bronchus and splaying of the carina upper zone venous enlargement due to pulmonary venous hypertension left ventricular enlargement is also eventually present due to volume overload
	Acute pericarditis		enhancement of the thickened pericardium generally indicates inflammation	The normal pericardial thickness is considered 2 mm while a thickness of over 4 mm suggests a pericarditis
	Pericardial effuson	globular enlargement of the cardiac shadow giving a water bottle configuration Lateral CXR may show a vertical opaque line (pericardial fluid) separating a vertical lucent line directly behind sternum (epicardial fat) anteriorly from a similar lucent vertical lucent line (pericardial fat) posteriorly; this is known as the Oreo cookie sign	Fluid density material is seen surrounding the heart	Fluid density material is seen surrounding the heart	Echocardiography is the method of choice to confirm the diagnosis, estimate the volume of fluid and most importantly assess the haemodynamic impact of the effusion
	Myocarditis			regional or global wall motion abnormalities are common, but nonspecific (biventricular wall motion abnormality, however, is the main predictor of death or transplantation) pericardial effusion is reported in ~45% (range 32-57%) of patients with myocarditis regional vasodilatation and increased blood volume due to the inflammation in myocarditis causes early postcontrast enhancement
	Coronary artery disease		coronary CT angiography (cCTA) can show the amount of stenosis
Neurological involvement	Cognitive dysfunction	The mental status of SLE patients can be temporarily affected by multiple, transient metabolic and systemic processes
	Stroke		Early sign: a hyperdense segment of a vessel, representing direct visualisation of the intravascular thrombus / embolus and as such is visible immediately Early hyperacute: loss of grey-white matter differentiation, and hypoattenuation of deep nuclei cortical hypodensity with associated parenchymal swelling with resultant gyral effacement elevation of the attenuation of the cortex. This is known as the CT fogging phenomenon a region of low density with negative mass effect. Cortical mineralisation can also sometimes be seen appearing hyperdense.	the affected parenchyma appears normal on other sequences, although changes in flow will be detected (occlusion on MRA) and the thromboembolism may be detected (e.g. on SWI). Slow or stagnant flow in vessels may also be detected as a loss of normal flow void and high signal  after 6 hours, high T2 signal will be detected
	Neuropathies			Optic neuritis: Typically findings are most easily identified in the retrobulbar intra-orbital segment of the optic nerve, which appears swollen, with high T2 signal. High T2 signal persists and may be permanent; chronically the nerve will appear atrophied rather than swollen. Contrast enhancement of the nerve, best seen with fat-suppressed T1 coronal images, is seen in >90% of patients if scanned within 20 days of visual loss
	Autoimmune encephalitis			mesial temporal lobes and limbic systems, typically manifested by cortical thickening and increased T2/FLAIR signal intensity of these regions. Bilateral involvement is most common (60%), although often asymmetric Patchy areas of enhancement
	Raynaud phenomen			contrast-enhanced MR angiography may also reveal characteristic narrowing and tapering of digital vessels	Doppler sonography: flow volume and vessel size irregularities
	Myositis			Intramuscular oedema (increased high T2/STIR signal)
Musculoskeletal involvement	Arthritis	Mostly symmetrical and non-erosive Arthralgias Effusions Decreased range of motion of both small and large joints Morning stiffness
	Osteonecrosis (Avascular necrosis)	Most common in the femoral head Can involve humeral head, tibial plateau, and scaphoid navicular Usually bilateral and is often asymptomatic Glucocorticoids treatment is associated with the greatest risk of developing the disease
	Subcutaneous nodules	In association with active disease
	Osteoporosis	Mostly due to glucocorticoid usage Loss of height Sudden back pain