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Chapter 6 · Rheumatology
k Signs on US
1. Ear pinna chondritis or perichondritis can be detected
by ear pinna US.The ear pinna is a highly specialized
structure that serves to collect sound and conduct it to
the middle ear. On sonograms, it is possible to distinguish two different zones of the ear pinna: an upper
region and a lower region. The anatomic difference is
mainly the presence or absence of cartilage inside the
layers. The upper region corresponds to the higher two
thirds of the ear pinna and consists of three layers:
anterior and posterior, each depicted as echoic thin
skin layers, and a middle layer containing cartilage,
which is represented as a completely hypoechoic regu-
6
lar thin band that follows the different concavities and
convexities of the ear pinna (. Fig. 6.12.4 ). The lower
structure and consists of only skin because of the
absence of cartilage in this area. The normal mean
thickness of the hypoechoic cartilage at the antihelix
boarder is 0.7–0.9mm, and the normal mean thickness
of the lobule is 6–8 mm. Ear pinna chondritis is
detected as thickened, echogenic, and beaded-shaped
cartilage. A dissecting fluid collection may be seen,
dividing the normally uniform 1-layer hypoechoic cartilage into a 2-layer structure.
2. On musculoskeletal ultrasound, the cartilage surface of
the metacarpophalangeal joint, seen as completely
hypoechoic circular layer over the metacarpal heads,
can show increased signal on power Doppler sonography, re ecting the hyperemia of chondritis
. Fig. 6.12.5 ) .
(
region is depicted by the ear lobule, which is a 1-layer
. Fig. 6.12.4 Ear helix ultrasound image of a patient with relapsing polychondritis that demonstrates the sonographic anatomy of the ear helix
with the cartilage layer seen as a hypoechoic layer ( arrowhead )
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6
. Fig. 6.12.5 Two musculoskeletal ultrasound images of the metacarpal bones of a patient with relapsing polychondritis demonstrating
increase signal of the power Doppler signal of the cartilage over the metacarpal head ( arrowhead ), which denotes infl ammation (chondritis)
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Chapter 6 · Rheumatology
Signs on CT and MRI
1. The same radiographic features of cartilage calcifi cation can be seen on CT images detected in the ear pinnae, nose,
and trachea.
2. On MRI, tracheal stenosis with circumferential increased signal intensity, and contrast enhancement is detected when
tracheal chondritis is present.
3. Brain MRI, especially angiographic time-to-fl ight (TOF) images, can show stenotic and beading of the circle of Willis if
vasculitis is present (
ab
. Fig. 6.12.6 ) .
6
. Fig. 6.12.6 Axial FLAIR-T2W MR-image ( a ) and TOF MR-image ( b ) of a patient with relapsing polychondritis; the patient presented with
severe headache for MR investigation. Although the FLAIR-T2W image shows no brain injury, the TOF image shows stenosis of the right
vertebral artery ( arrowhead )
Selected References
Ananthakrishna R, etal. Relapsing polychondritis-case series
from south India. Clin Rheumatol. 2009;28 Suppl 1:S7–
10.
Caceres M, etal. Transverse aortic arch replacement associ-
ated with MAGIC Syndrome: case report and literature
review. Ann Vasc Surg. 2006;20:395–8.
Coppola M, etal. Relapsing polychondritis: an unusual cause of
painful auricular swelling. Ann Emerg Med. 1992;21:81–5.
Irani SR, etal. Relapsing “encephalo” polychondritis. Pract
Neurol. 2006;6:372–5.
Kumakiri K, etal. A case of relapsing polychondritis preceded
by inner ear involvement. Auris Nasus Larynx. 2005;32:71–6.
Oddone M, etal. Relapsing polychondritis in childhood: a
rare observation studied by CT and MRI.Pediatr Radiol.
1992;22:537–8.
Wortsman X, etal. Sonography of the Ear Pinna. Ultrasound
Med. 2008;27:761–70.
Fornadly JA, etal. e role of MRI when relapsing polychon-
dritis is suspected but not proven. International Journal of
Pediatric Otorhinology. 1995;31:101–7.
6.13 R e fl ex Sympathetic Dystrophy
Gergely P. Relapsing polychondritis. Best Pract Res Clin
Rheumatol. 2004;18(5):723–38.
Giordano M, etal. Relapsing polychondritis with aortic arch
aneurysm and aortic arch syndrome. Rheumatol Int.
1984;4:191–3.
Hidalgo-Tenorio C, et al. Magic syndrome and true aortic
aneurysm. Clin Rheumatol. 2008;27:115–7.
e complex regional pain syndromes (CRPS I and CRPS II),
also known as re ex sympathetic dystrophy (CRPS I) and cau-
salgia (CRPS II) , are diseases characterized by discrete sensory, motor, and autonomic ndings. Nerve supply to any
limb can be divided into three main neuronal supplies: sensory, motor, and autonomic.
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6
Injury to the sensory supply results in paresthesia and
numbness, with complete loss of sensation in extreme irreversible sensory neuronal damage. Injury to the motor neuronal supply results in paraparesis or paralysis depending on
the degree of neuronal loss. In contrast to both sensory and
motor neuronal injury, autonomic injury results in CRPS
I.CRPS I is also called Sudeck ’ s atrophy and hand - shoulder
syndrome in some medical literatures.
CRPS II, or causalgia, is de ned as a limb pain that is
always preceded by a partial injury to a peripheral nerve or
one of its major branches. is syndrome is not always progressive and can persist for years without any clinical changes.
Patients with CRPS I di use pain in a limb that a ects the
autonomic and maybe the motor innervation of that limb
( commonly a ecting the upper limbs compared to the lower
limbs ).
CRPS I has a female predominance, with a normal age
distribution with a peak of 50 years of age. e disease can be
suspected and di erentiated from other causes of neuropathy
by the following typical features:
A . History of a noxious event preceding the pain : typically, the
pain is preceded by a noxious even such as minor trauma,
sprains, bone fractures, surgery ( e.g ., carpal tunnel ,
Dupuytren ’ s contracture ), and other lesions such as shoul-
der trauma, myocardial infarction, or even contralateral
stroke. However, CRPS I is idiopathic in 35 % of cases.
CRPS I can be transiently produced in healthy individu-
als by immobilizing a limb for 1 month.
B . Exaggerated pain : the pain is typically disproportionate
to the inciting event ( a small trauma not mentioned ,
followed later by severe limb pain ) and is felt deep within
the limb. Pain can be felt even due to water or air
exposure, known in the neurological literature as
allodynia , which is de ned as pain that arises due to
non-painful stimuli.
e pain is typically described as burning, throbbing,
pressing, shooting, or aching. In nearly all cases, the continuous pain is felt deeply inside the distal part of the
a ected extremity. It always shows a di use distribution
that is unrelated to territories of individual nerves.
C . Pain that does not follow a speci c dermatom : e signs
and symptoms of CRPS I are not con ned to the innervation zone of an individual nerve and show a distally generalized distribution (95 % of cases).
D . Complain that shows sensory , motor , and autonomic a ec-
tion : CRPS I pain has a triad of sensory, motor, and autonomic symptoms that are present in 90 % of cases of
CRPS I; however, there appears to be no xed combinations.
E . Limb swelling : typically, the a ected limb shows swelling
due to loss of the autonomic control of the
microvasculature of that limb causing localized vascular
shunting at the site of trauma.
F. Mirror image syndrome : in CRPS I, the pain extend along
the limb or migrates to other body parts in nearly 70 % of
patients. e pain becomes bilateral, producing a “mirror
image” of pain in up to 50 % of cases. In rare cases, the
pain can even encompass the entire body. Mirror- image
pain arises from the healthy body region contralateral to
the actual site of trauma or in ammation. Mirror-image
pain is generally characterized as mechanical allodynia.
CRPS I clinically can be divided into three stages: acute
phase, which is marked by pain, edema, warm skin, and
increased sweating; dystrophic phase, which is marked by
cold, dry skin, and trophic changes; and atrophic phase,
which is marked by atrophied skeletal muscles and bones,
joint contractures, progressive loss of function, and persistent pain.
Signs on Plain Radiographs
Plain radiographs often show a diff use and spotty distal
distribution of demineralization (osteopenia) of small
bones with periarticular dominance at the longer bones
(
. Fig. 6.13.1 ). These radiologic fi ndings ( which are called
Sudeck ’ s atrophy ) are generally not evident until the
syndrome has been established for several months. The
pathophysiological explanation for the bone
demineralization is due to the vascular shunting that
causes autonomic bone marrow edema and
infl ammation, which in the end will boost the
osteoclastic activity over the osteoblastic one. It should
be remembered that the radiographic manifestations of
CRPS I means that the patient has been suff ering for
years without proper therapy.
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Chapter 6 · Rheumatology
6
. Fig. 6.13.1 Plain radiograph of both hands of a patient with CRPS I that shows right-sided, bone osteopenia compared to the left hand
that aff ects the metacarpal heads ( arrow ) and the carpal bones ( arrowhead ), with old fracture of the distal radius
Signs on Doppler Sonography
1. In the acute phase, arterial Doppler of the arteries in the
aff ected limb above the area of pain (or injury) shows
mono- to biphasic waveform spectrum, compatible with
the arterial shunting which occurs due to autonomic
disturbance (
artery on the contralateral arm or leg shows normal
triphasic waveform spectrum. After treatment, the
. Fig. 6.13.2 ). In comparison, the same
mono- to biphasic waveform spectrum can return to
normal (triphasic).
2. On musculoskeletal US, the aff ected nerve will show a
hypo echoic texture (
show a hyper echoic texture due to the fatty nature of
the myelin sheath. The hypoechoic texture seen in
neuropathies is suggested by the medical literature to
be seen due to lipid peroxidation.
. Fig. 6.13.3 ); a normal nerve will
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. Fig. 6.13.2 Doppler sonographic images of a 53-year-old female patient presented with 3 months history of left-sided pain and
allodynia due to CRPS I. Doppler sonography of the deep palmar arch revealed a classical sign of mild “arteriovenous shunting” in Doppler
sonography, detected as a mixture wave pattern that merges the arterial and venous waves together ( upper image ). In comparison, the
right-sided Doppler wave sonography ( lower image ) shows biphasic Doppler waveform, not the normal peripheral, arterial triphasic
pattern, which denotes a mild arterial shunting present, but not as severe as the left hand (mirror syndrome)
6
. Fig. 6.13.3 Nerve ultrasound image of a 47-year-old female patient presented with CRPS I for 1-month duration. The median nerve
ultrasound showed hypoechoic texture ( arrow ), while her husband’s median nerve shows normal iso- to hyperechoic texture ( arrowhead )
Signs on MRI
The distal bones of the limb aff ected by CRPS I can show nonspecifi c, patchy bone marrow edema; any bone can be aff ected.
In the medical literature, CRPS I commonly aff ects the hip joint in pregnant women, a condition known as hip bone marrow
edema syndrome , a variant of CRPS I.
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Chapter 6 · Rheumatology
. Fig. 6.14.1 ). Due to the absence of speci c laboratory
Selected References
Bennett DS, etal. Complex regional pain syndromes (re ex
sympathetic dystrophy and causalgia) and spinal cord
stimulation. American Academy of Pain Medicine.
(
serological tests for this condition, radiological investigations are important to assist in investigating the clinical
diagnostic criteria.
2006;7:S64–96.
Unusual Presentations of Polymyalgia Rheumatica
Crozier F, etal. Magnetic resonance imaging in re ex sympa-
thetic dystrophy syndrome of the foot. Joint Bone Spine.
2003;70:503–8.
Oyen WJG, etal. Re ex sympathetic dystrophy of the hand:
an excessive in ammatory response? Pain. 1993;55:151–7.
Pekindil G, etal. Doppler sonographic assessment of post-
traumatic re ex sympathetic dystrophy. J Ultrasound
6
Med. 2003;22:395–402.
z
1 . Peripheral joint synovitis : a ecting typically the shoulder
and the hip joints, symmetrically or asymmetrically.
2 . Sternoclavicular synovitis : the sternoclavicular joint is not
usually a ected in in ammatory diseases, but it is
sometimes involved in polymyalgia rheumatica.
3 . Distal swelling with pitting edema : another unusual
syndrome is known as RS3PE ( remitting seronegative
symmetrical synovitis with pitting edema ).
4 . Aortic dissection : due to giant cell aortitis.
6.14 Polymyalgia Rheumatica
Polymyalgia rheumatica (PR) is an in ammatory condition
with unknown origin characterized by morning sti ness and
Polymyalgia rheumatica
aching sensation in the cervical region, shoulder, and pelvic
girdles. It has been considered in the past as a variant manifestation of giant cell arteritis, until it has been rede ned by
Bird etal. (1979) as a separate rheumatic entity. Although
PMR is considered a disease of unknown origin, it has been
found to have a close concurrence with some infections like
Mycoplasma pneumonia , Chlamydia pneumonia , and parvo-
virus B19.
e diagnostic criteria of PR include ( according to Healey ;
1984 ):
(a) Bilateral pain persisting for at least one month and
involving one of the following areas: neck, shoulders,
and pelvic girdles.
(b) Morning sti ness lasting more than one hour.
(c) Rapid response to low-dose steroid (10–15mg in the
morning).
(d) Absence of other diseases explains the current
symptoms.
(e) Age more than 50 years.
(f) Erythrocytes sedimentation rate and C-reactive protein
serum levels are raised.
(g) Ultrasonographic features of bursitis and/or synovitis in
the shoulder and/or hip joints.
Patients with PR present with bilateral discomfort in the
upper limbs that interfere with the daily activities. e pain
in the shoulder and pelvic girdles o en radiates to the
elbows and the knees. Symmetric peripheral arthritis a ecting the knees and wrists, carpal tunnel syndrome, and pitting edema of the dorsum of the hands may be seen
. Fig. 6.14.1 An illustration that demonstrates the body’s
geographic distribution of polymyalgia rheumatica arthropathy
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Signs on US
Shoulder US shows hip eff usion (68 %), shoulder subacromial bursa fl uid collection (96 %), and biceps tendon tenosynovitis
with peritendinous fl uid collection (Figs.
6.14.2 and 6.14.3 ), all attributed to bursitis and/or synovitis of these joints
6
a
. Fig. 6.14.2 Musculoskeletal sonographic images of the right shoulder of a patient with polymyalgia rheumatic showing fl uid
collection around the long head of biceps tendon ( arrowhead in a ), associated with hyperemia seen as increased power Doppler signal
( arrow in b ); the fi ndings refl ects tenosynovitis
b
Signs on MRI
1. Shoulder MRI may show subdeltoid and subacromial
bursitis, and biceps tendon tenosynovitis.
Tenosynovitis is seen as an enlarged tendon with free
fl uid surrounding it due to infl ammation and edema.
2. Subacromial bursitis is seen as free fl uid located
above the supraspinatus tendon with intact
supraspinatus tendon (no signs of tears or
tendinosis).
3. Hands and wrists tenosynovitis may be seen as
abnormal tendon signal intensity on T2W images
with surrounding free fl uid.
4. A patient with chest pain should be investigated for
aortic dissection.
. Fig. 6.14.3 Musculoskeletal, panoramic sonographic
images of the same patient in Fig. 6.14.2 showing fl uid
collection around the iliopsoas tendon at the right hip
( arrowhead ), refl ecting iliopsoas bursitis
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Selected References
Mandell B. Polymyalgia rheumatic: clinical presentation is
key diagnosis and treatment. Cleve Clin J Med.
2004;71(6):489–95.
Salvarani C, et al. Polymyalgia rheumatica and giant-cell
arteritis. N Engl J Med. 2002;347:261–71.
Salvarani C, etal. Polymyalgia rheumatica. Best Pract Res
Clin Rheumatol. 2004;18(5):705–22.
Soriano A, etal. Polymyalgia rheumatica in 2011. Best Pract
Res Clin Rheumatol. 2012;26(1):91–104.
6
6.15 Systemic Lupus Erythematosus
Systemic lupus erythematosus (SLE) is a chronic, in ammatory, autoimmune systemic disorder of unknown origin
characterized by the formation of autoantibodies that attack
multiple organs. SLE has a female predominance, with periods of ares and remissions. SLE antibodies can be categorized into categories:
1 . Antibodies against nuclei ( antinuclear antibodies ): anti-
DNA histone, anti-double-stranded DNA (dsDNA), and
anti-single-stranded DNA antibodies
2 . Antibodies against cytoplasmic component s:
mitochondrial and microsomal antibodies
3 . Organ-speci c antibodies : e.g., antithyroid antibodies
4 . Others : rheumatoid factor (50 % of cases), cryoglobulins,
and antiphospholipid antibodies
3 . Renal SLE : these are ranges from mild asymptomatic
proteinuria to rapidly progressing glomerulonephritis
associated with end-stage renal disease. e main injury
of the kidneys in SLE is related to glomerular lesions
with or without injury to the tubules and interstitium.
Rarely, isolated tubulointerstitial changes can be
encountered in the presence of minimal glomerular
abnormalities in SLE or so-called predominant
tubulointerstitial lupus nephritis.
4 . Neuropsychiatric manifestations of SLE : these
manifestations include headaches, dementia, seizures
(focal or di use), and psychoses.
5 . Pulmonary SLE : this includes unilateral or bilateral pleural
e usions, reticular interstitial lung disease, pleural
thickening, alveolar lung disease, and shrinking lung
syndrome. Shrinking lung syndrome (SLS) is a rare
complication of SLE with unknown origin characterized by
unexplained progressive dyspnea, pleuritic chest pain,
fever, dry cough, small lung volumes, elevation of the
diaphragm, and restrictive physiology on pulmonary
function tests. It is suggested that SLS is caused by myositis
of the diaphragm, phrenic nerve paresis, restrictive rib cage
abnormality of unknown pathology, or pleural adhesions.
6 . Other systemic manifestations of SLE : fever and anemia.
ere is increased frequency of midtrimester abortions
(15 %), prematurity (20 %), and stillbirth (10 %) in
pregnant women with SLE ares.
7 . Skeletal rheumatological manifestations of SLE include:
SLE pathology is characterized by widespread of vasculi-
tis, a ecting capillaries, arterioles, and venules. SLE can be
localized to the skin without systemic manifestations ( chronic
cutaneous lupus ), self-limiting due to certain medications
( drug - induced lupus ), and a ecting neonates due to maternal
anti-Rho antibodies crossing the placenta ( neonatal lupus ).
SLE can be triggered by endogenous factors (e.g., sex hormones) or exogenous factors (e.g., sunlight exposure). Drugs
that induce lupus-like reaction include alpha interferon and
hydralazine.
Serological characteristics of SLE include high serum levels of antinuclear antibody (ANA), anti-double-strand DNA
(anti-dsDNA), rheumatoid factor, and antiphospholipid
antibodies. Patients with SLE have ANA-positive results in
95 % of cases. However, positive ANA results are not speci c
to SLE and can be seen in normal individuals > 65 years of
age (15 %) at low titers, patients with Sjögren’s syndrome,
patients with scleroderma, and patients with autoimmune
thyroid disease.
Manifestations of SLE include ( diagnosis is con rmed by
ful lling at least four systemic manifestations with positive
serological tests ):
1 . Cutaneous SLE : this includes malar rash, photosensitivity,
and discoid lupus
2 . Cardiopulmonary SLE : this is seen mostly in the form of
pleuritis, pericarditis, bilateral pleural e usion, and
uncommonly noninfective (Libman–Sacks) endocarditis.
I . Lupus arthritis : arthritis a ects 69–95 % of SLE
patients in the form of symmetric, nonerosive
polyarthritis that preferentially involves the small
joints over the large joints, although any joint may be
a ected. e most common joints a ected are the
hand joints including the metacarpal phalangeal
(MCP), proximal interphalangeal (PIP), and distal
interphalangeal (DIP) as well as the knees (Fig. 6.15.1 ).
Shoulders, ankles, and elbows are less commonly
a ected but can be involved. Swelling of the joints or
synovial proliferation can be present, although the
swelling is o en not as prominent as it is with
rheumatoid arthritis (RA). Other signs include
morning sti ness, arthralgia, and joint erythema.
II. Sacroiliitis : although sacroiliitis is typically thought of
as a manifestation of the seronegative
spondyloarthropathies, it has been reported in SLE in
up to 50 % of cases.
III. Atlantoaxial subluxation : it is seen in 8.5 % of SLE
patients, especially patients with Jaccoud’s
arthropathy.
IV. Deforming arthropathy ( Jaccoud ’ s arthritis ): this is an
uncommon form of deforming arthritis characterized
by ligament laxity, joints deformity, which may be
associated with joints erosions (
3–13 % of SLE patients and is associated with high
serum titers of anti-RNP antibodies. Jaccoud’s
. Fig. 6.15.2 ). It a ects
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6
SLE arthritis
Jaccoud’s arthropathy
. Fig. 6.15.1 An illustration that demonstrates the body’s
geographic distribution of SLE arthropathy
arthropathy is not speci c for SLE and can be seen on
other conditions including scleroderma and
dermatomyositis.
V. Erosive arthritis ( rhupus ): SLE arthritis is typically
nonerosive. However, erosive SLE arthritis can be
seen in cases of SLE/RA overlap, also known as
(rhupus). Rhupus has an incidence of 0.01–2 % of SLE
patients and has been associated with high serum
titers of anti-CCP and anti-RA33 antibodies in some
studies.
VI. Synovitis and tenosynovitis : they are seen in up to 44 %
of SLE patients typically symmetrical in distribution.
Tendon rupture is a rare complication of SLE. e
tendons that are more commonly involved include the
Achilles, patellar, infrapatellar tendons, and tendons
in the hand.
VII. Avascular necrosis and bone infarction : it is commonly
seen in SLE patients who show high serum titers of
antiphospholipid antibodies, especially in the femoral
head.
VIII. Others : periarticular osteoporosis, acral sclerosis,
so -tissue calci cation (calcinosis), and cystic bone
lesions.
. Fig. 6.15.2 An illustration that demonstrates the body’s
geographic distribution of Jaccoud’s arthropathy
D i ff erential Diagnoses and Related Diseases
k Brown’s Syndrome
Brown’s syndrome is a disease characterized by intermittent
diplopia due to congenital or acquired motility impairment
of the superior oblique muscle. e disease is believed to be
secondary to restriction of the superior oblique muscle in
the trochlea/tendon complex, causing tethering of the muscle when the eye is adducted. Acquired Brown syndrome
can be caused by paranasal sinuses infections, trauma,
orbital in ammation, rheumatoid arthritis stenosing synovitis, SLE, scleroderma, and rarely psoriasis. Acquired
Brown syndrome usually responds to corticosteroids therapy, especially when in ammation or systemic connective
tissue disorders are the cause. Patients are presenting with
intermittent diplopia when gazing upward, and the disease
is characterized by inability to actively or passively elevate
the a ected eye in full adduction. Other features include
widened palpebral ssure on adduction and divergence on
midline eye elevation.
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