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Chapter 6 · Rheumatology
Signs on Plain Radiographs
1. Pleura eff usion unilaterally or bilaterally can be seen in
SLE patients in 26 % of cases.
2. Cardiomegaly may be seen due to pericardial eff usion
(15 % of cases).
3 . I n shrinking lung syndrome , typically, chest radiographs
will show elevated hemidiaphragms, with blunting of
the costophrenic angles and platelike atelectasis at the
lower lobes bilaterally.
4 . Femoral head avascular necrosis is seen on radiographs
with diff erent signs depending on the stage: stage I
( normal X - rays ), stage II ( sclerotic or cystic lesions without
fracture ), stage III ( crescent sign indicative of subchondral
collapse ), and stage IV ( further progression with
osteoarthritis and acetabular changes ).
5 . Jaccoud ’ s arthropathy is seen on radiographs as ulnar
deviation, subluxation of the MCP, PIP and DIP joints,
swan-neck deformities of fi ngers, localized osteoporosis,
and absence of juxta-articular erosions (
. Fig. 6.15.3 ) .
6
. Fig. 6.15.3 Plain radiography of the hands of a patient with Jaccoud’s arthropathy that demonstrates diff use subluxation deformities
without bone erosions
Signs on PD Sonography
1. Power Doppler (PD) sonography can diff erentiate
between primary or secondary Raynaud’s phenomenon
in the fi ngers or toes. The patient is examined fi rst by PD
with the probe to gain the baseline images. The region
of interest is examined; then, the patient is asked to
place their fi ngers into cold water (7 °C) for 3 min, and
then the region of interest is reexamined again with PD
sonography. Primary Raynaud’s phenomenon will show
moderate to mark hyperemia with red-to-orange (PD)
signal in the normal temperature and then show
reduced PD signal after the cold challenge. In contrast,
secondary Raynaud’s phenomenon will almost always
show reduced hyperemia with red-to-orange (PD) signal
before and after cold challenge.
6.15 · Systemic Lupus Erythematosus
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6
Signs on CT
1. Retroperitoneal lymphadenopathy with lymph
nodes > 15 mm in diameter (64 % of cases).
2. The kidneys may be enlarged due to nephrotic
syndrome or shrunken due to long-term disease.
Spontaneous subcapsular hematomas may occur and
seen as renal masses with high attenuation values on
noncontrast-enhanced scan (38–64 HU). Predominant
tubulointerstitial lupus nephritis shows bilateral,
multiple, wedge-shaped areas or streaky zones of low
enhancement that extend from the papilla to the
renal cortex with or without slight cortical atrophy.
This CT sign is nonspecifi c to SLE predominant
tubulointerstitial lupus nephritis, but is a common
sign observed in tubulointerstitial nephritis for any
cause.
3. Hepatosplenomegaly may be seen, especially with
lymphadenopathy (11 % of cases).
4. Diff use bladder wall thickening can be seen;
however, exclusion of infectious cystitis is necessary.
5. Venous thrombosis may be seen, aff ecting the IVC or
the femoral veins.
6 . Abscesses may be seen within the pancreas (diffi cult
to diff erentiate from pseudocyst), liver, small bowel,
spleen, and kidneys. An abscess is seen as a cystic
lesion with thick wall that enhances after contrast
injection, with maybe intracystic air
( pathognomonic ).
7 . Infarctions may occur within the intestine or the
kidneys. Intestinal infarction shows bowel wall
thickening with gas within the wall (pneumatosis
intestinalis). In the kidneys, there is a wedge-shaped
area that does not enhance after contrast injection.
Diff erentiating focal pyelonephritis from renal
infarction is diffi cult since both show the same
radiological features; however, an enhancing rim
around the low-density wedge may be seen in 46 %
of renal infarctions, a feature that is not seen in
pyelonephritis.
8. Pancreatitis may be seen due to steroid treatment, a
common therapy for SLE.
9. Pulmonary manifestations include ground-glass
alveolar opacities, pleural thickening, pleural eff usion,
and/or reticular interstitial patterns. In shrinking lung
syndrome, the chest shows pleural thickening
involving the diaphragmatic pleura bilaterally with an
otherwise normal lung parenchyma.
10. In acquired Brown’s syndrome, there is thickening of
the superior oblique tendon of the aff ected eye
compared to the normal side. Signs of infl ammation
and contrast enhancement aff ecting the trochlea/
tendon complex may be seen in cases of
infl ammation or synovitis.
Signs on MRI
1. Cortical hyperintensity lesions in the brain MRI may be
seen on T2W and DW images due to thrombotic
cerebral infarction. Multiples stroke episodes can be
seen in SLE due to hypercoagulability especially when
SLE is associated with positive anticardiolipin
antibodies or due to embolic events related to
Libman–Sacks endocarditis (
2 . Acute lupus encephalopathy is seen as reversible lacy
areas of hyperintensity in cortical gray matter and
subcortical white matter, particularly in occipital,
temporal, and parietal lobes on T2 and FLAIR images.
Long-standing neuro-SLE cerebral disease can show
poroencephalic cysts formation due to old brain
infarctions, diff use brain atrophy, and/or intracranial
hemorrhages due to SLE vasculitis aff ecting the circle
of Willis (
3. Cerebral calcinosis ( Fahr ’ s disease ) has been reported to
occur uncommonly as a severe, rare manifestation of
neuropsychiatric SLE. Fahr’s disease is seen as extensive
calcifi cation in the thalamus, putamen, caudate
nucleus, white matter, and posterior gray matter.
4. In lupus arthritis, the MRI fi ndings in the hand,
including tenosynovitis, were nearly
indistinguishable from early RA except that RA
patients had more bone marrow edema of the MCP
joints and more abnormalities of the right fourth
extensor tendon.
. Figs. 6.15.6 and 6.15.7 ).
. Figs. 6.15.4 and 6.15.5 ).
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Chapter 6 · Rheumatology
6
. Fig. 6.15.4 Axial and coronal T2W-MR images of a patient with neuro-SLE show infarction of the left hemisphere seen as hyperintense
cortices at the parietotemporal regions ( arrowhead )
. Fig. 6.15.5 Axial and coronal FLAIR-T2W-MR images of a patient with neuro-SLE show ischemic, vasculitis insults to the brain seen as
diff use lesions aff ecting both hemispheres in the white matter’s U-fi bers and even including the right thalamus
6.15 · Systemic Lupus Erythematosus
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6
. Fig. 6.15.6 Axial gradient-T*MR images of a patient with neuro-SLE show multiple microbleedings (arrowheads) detected as
hypointense foci in the occipital lobes bilaterally
Selected References
. Fig. 6.15.7 Time-to-fl ight (TOF) MR images of the same
patient in Fig. 6.15.6 showing left-sided beading of the internal
carotid artery (M2–M3) segments due to long-standing
neurovasculitis ( arrowheads )
Gezer A, etal. Bilateral acquired Brown syndrome in sys-
temic scleroderma. J AAPOS. 2005;9:195–7.
Grossman JM, et al. Lupus arthritis. Best Pract Res Clin
Rheumatol. 2009;23:495–506.
Heiberg E, etal. Body computed tomography ndings in sys-
temic lupus erythematosus. Journal of Computed
Tomography. 1988;12:68–74.
Kakati S, etal. A clinical study of pulmonary manifestations
in systemic lupus erythematosus with special reference to
CT ndings. Indian Journal of Rheumatology.
2007;2(4):133–6.
Kamishima T, etal. Predominant tubulointerstitial nephritis
in a patient with systemic lupus erythematosus with an
emphasis on CT and MR imaging ndings. European
Journal of Radiology Extra. 2009;72:e87–90.
Lee SI, etal. e usefulness of power Doppler sonography in
di erentiating primary from secondary Raynaud’s phenomenon. Clin Rheumatol. 2006;25:814–8.
Mafee MF, etal. Computed tomography in the evaluation of
Brown syndrome of the superior oblique tendon sheath.
Radiology. 1985;154:691–5.
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Chapter 6 · Rheumatology
Rodnan GP, etal. Systemic lupus erythematosus. Dis Mon.
1964;10(8):1–38.
Sibbitt Jr WL, etal. Magnetic resonance imaging and brain
histopathology in neuropsychiatric systemic lupus erythematosus. Semin Arthritis Rheum. 2010;40:32–52.
Sp T, etal. Association of the shrinking lung syndrome in sys-
temic lupus erythematosus with pleurisy: A systematic
review. Semin Arthritis Rheum. 2008;39:30–7.
Wilson ME, et al. Brown’s syndrome. Surv Ophthalmol.
1989;34:153–72.
6
Pulmonology
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7.1 Pleural Diseases – 275
Pleural Eff usion – 275
Pneumothorax – 278
Pleural Calcifi cation – 280
7.2 Alveolar Lung Diseases – 282
Types of Alveolar Lung Diseases – 282
How to Diff erentiate Between Cardiogenic Edema from ARDS
on Plain Chest Radiographs? – 284
Types of Pneumonias – 284
7.3 Atelectasis (Lung Collapse) – 287
Types of Pulmonary Atelectases – 287
273
7
7.4 Sarcoidosis – 290
Pulmonary Sarcoidosis – 291
Hepatic, Splenic, and Gastric Sarcoidosis – 292
Dermatological Sarcoidosis – 292
Cardiac Sarcoidosis – 293
Neurosarcoid – 293
Musculoskeletal Sarcoidosis – 295
Head and Neck Sarcoidosis – 295
Genitourinary Sarcoidosis – 296
7.5 Emphysema – 297
7.6 Idiopathic Interstitial Pneumonias – 300
Idiopathic Pulmonary Fibrosis – 300
Nonspecifi c Interstitial Pneumonia – 302
Cryptogenic Organizing Pneumonia – 302
Respiratory Bronchiolitis-Associated Interstitial Lung Disease – 303
Desquamative Interstitial Pneumonia – 303
Lymphoid Interstitial Pneumonia – 303
Acute Interstitial Pneumonia (Hamman–Rich Syndrome) – 304
© Springer International Publishing Switzerland 2017
J.A. Al-Tubaikh, Internal Medicine, DOI 10.1007/978-3-319-39747-4_7
7.7 Histiocytoses – 304
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Langerhans Cell Histiocytosis – 305
Infection-Associated Hemophagocytic Syndrome – 307
Omenn Syndrome – 307
Chédiak–Higashi Disease – 307
Diff erential Diagnoses and Related Diseases – 307
Rosai–Dorfman’s Disease (Sinus Histiocytosis) – 308
Xanthoma Disseminatum (Montgomery Syndrome) – 308
Erdheim–Chester Disease (Lipogranulomatosis) – 309
7.8 Hemoptysis – 310
Bronchopulmonary Sequestration – 311
Anomalous Systemic Artery Supplying Normal Lung Parenchyma – 312
Pulmonary Vasculitis – 312
Cardiac Bronchus – 314
Dieulafoy Disease – 314
7.9 Cystic Fibrosis (Mucoviscidosis) – 315
Pulmonary Manifestations of Cystic Fibrosis – 315
Nasal and Sinus Manifestations of Cystic Fibrosis – 316
Gastrointestinal (GI) Manifestations of Cystic Fibrosis – 317
Genitourinary Manifestations of Cystic Fibrosis – 319
Musculoskeletal Manifestations of Cystic Fibrosis – 319
7.10 Sleep Apnea Syndromes – 321
Obstructive Sleep Apnea Syndrome – 321
Upper Airway Resistance Syndrome – 324
Central Alveolar Apnea Syndrome (Ondine’s Curse) – 324
Cheyne–Stokes Respiration – 325
Uncommon and Rare Causes of Sleep Apnea – 325
7.1 · Pleural Diseases
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7.1 Pleural Diseases
e pleura are composed of two layers, parietal and visceral
layers, separated by a pleural space. e parietal pleuron is
supplied by systemic vessels and drains into the right atrium
via the azygos, hemiazygos, and internal mammary veins.
e visceral pleuron is supplied by bronchial and pulmonary
vessels and drains into the pulmonary veins.
e pleural space normally contains interstitial uid
(1–5mL) that is cleared by the parietal pleural lymphatic vessels. ere is no direct communication between the visceral
pleura lymphatics and the pleural space.
e pleura appear normally on radiographs only when
the X-ray beam is tangentially set on the lm. On radiographs, the pleura appear as ssures and junctional lines.
Fissures are made up of two layers of visceral pleura. e normal parietal pleuron is never visualized on posteroanterior
(PA) radiographs.
Di erent pathological conditions a ecting the pleura can
be diagnosed with con dence by PA chest radiographs alone.
is topic discusses the main pathological pleural conditions
with their typical radiologic manifestations.
Pleural Eff usion
Pleural e usion is a condition characterized by abnormal
uid collection between the parietal and visceral pleura
(excess pleural space uid). e pleural uid can be water
(edematous e usion), blood (hemothorax), pus (empyema),
tumor cells (malignant pleural e usion), or lymph
(chylothorax).
Pathologically, pleural e usion is divided into serous or
exudative according to the protein content a er lab analysis.
Serous plural e usion contains little protein content (<2.5g/
dL) and usually arises due to systemic disease like cardiac
failure, nephrotic syndrome, or liver failure. Exudative pleural
e usion contains high protein count (>2.5g/dL) and usually
arises due to in ammatory or infectious process like tuberculosis, malignancy, and acute pancreatitis.
Disruption of the thoracic duct due to lymphoma or a
tumor can cause lymphatic blockage and leakage into the
pleural space causing chylothorax. Malignant e usion typically results from metastasizing of the malignant cells into
the pleural cavity via the parietal pleura lymphatics, and it is
o en massive.
Bronchopleural stula is a condition characterized by
opening of a bronchus into the pleural space. It can develop
occasionally following thoracic surgery, infection, medical
intervention, or malignancy. Bronchopleural stula is seen in
2–3 % of postpneumonectomy cases.
Signs on Chest Radiographs
5 Obliteration of the lateral costophrenic angle with
a meniscus like arc at the interface between the
fluid and the chest wall in PA radiographs
(Meniscus sign) (
5 Obliteration of the posterior costophrenic angle in
lateral radiographs (
sensitive to plural effusion collection due to
gravity effect. Up to 50 mL of fluid is necessary to
obliterate the posterior costophrenic angle, and
. Fig. 7.1.1 ).
. Fig. 7.1.1 ). This angle is more
a
. Fig. 7.1.1 Posteroanterior ( a ) and lateral ( b ) chest radiographs in two diff erent patients with pleural eff usion show meniscus sign with
right pleural eff usion obliterating the lateral costophrenic angle ( arrowhead ) in ( a ) and pleural eff usion obliterating the posterior
costophrenic angle in ( b ) ( arrow )
b
Chapter 7 · Pulmonology
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200 mL is necessary to obliterate the lateral
costophrenic angle.
5 Subpulmonic pleural effusion ( SPE ) is a pleural
effusion that occurs below the lungs at the
diaphragmatic surface. SPE does not obliterate the
costophrenic angle, but it distorts the shape of the
diaphragmatic dome, giving the impression of
raised hemidiaphragm. You can suspect SPE in the
left lung when the space between the gastric
bubble and the lower lung margins increases up to
3 cm instead of usual few millimeters. Beside the
raised hemidiaphragm, the lung appears to end
early on PA radiographs (
5 Encysted ( loculated ) pleural effusion is a localized
7
encysted fluid at the fissures between lobes of the
lung. It occurs usually at the right lung’s minor
fissure, and it has biconvex contour mimicking a
mass (
. Fig. 7.1.3 ). Very rarely, a benign form of
mesothelioma can grow along the major or minor
fissures mimicking encysted pleural effusion, a
condition known as pseudotumor .
5 Parapneumonic effusion is an effusion that
develops adjacent to pneumonias (empyema).
Almost 30 % of patients with pneumonia develop
pleural effusion and usually resolve with antibiotic
therapy.
5 Mediastinal pleural effusion is a fluid collection
around the mediastinum. It is an unusual
. Fig. 7.1.2 ).
condition, and when it occurs, it forms
silhouette sign along the mediastinal borders
causing mediastinal widening. Silhouette sign
is a term used to describe any opacity within the
chest radiograph that obliterates a mediastinal
border.
. Fig. 7.1.2 Posteroanterior chest radiograph of a patient
with right subpulmonic pleural eff usion ( SPE ) shows raised
hemidiaphragm, and the lung seems to end early ( arrowhead )
a
. Fig. 7.1.3 Posteroanterior ( a ) and lateral ( b ) chest radiographs show right-sided encysted pleural eff usion ( arrowheads )
b
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Signs on US
Pleural eff usion appears as anechoic or hypoechoic
collection that lies between the echogenic line of the
visceral pleura and lung (
. Fig. 7.1.4 ).
. Fig. 7.1.4 Transverse ultrasound image shows right-sided
pleural eff usion ( arrowhead ). The diaphragm can be visualized
as a hyperechoic line separating the right lung base from the
liver ( arrow )
a
b
Signs on CT
5 Serous pleural effusion is visualized as a crescent
peripheral area with CT water density. Exudative
effusion can be hyperdense.
5 Empyema characteristically demonstrates
thickened parietal/visceral pleura (e.g., > 2 mm)
with effusion in between (split pleura sign)
(
. Fig. 7.1.5 ). Enhancement of both pleura occurs
in 80–100 % cases after contrast injection. Multiple
gas pockets within the empyema may be seen.
5 Bronchopleural fistula occurs when a bronchus
opens into the pleural space due to lung
parenchymal destruction (e.g., pneumonia with
empyema formation). It is seen as pleural effusion
with air–fluid level on radiographs or HRCT
(
. Fig. 7.1.6 ) .
. Fig. 7.1.5 Posteroanterior chest radiograph ( a ) and axial
chest CT ( b ) of a patient with huge left-sided empyema show
split pleura sign in ( b ), with thickened, enhanced pleura with
eff usion in between ( arrowheads )
. Fig. 7.1.6 Axial chest CT shows huge right
bronchopulmonary fi stula
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