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Chapter 6 · Rheumatology
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. Fig. 6.8.5 Sagittal ( a ) and axial ( b ) T2W image MRI of a patient with SD shows Schmorl’s node seen as localized depression of the
superior end plates >3 mm in diameter due to extrusion of the nucleus pulposus into the end plates ( arrowheads )
ab
. Fig. 6.8.6 Sagittal T1W ( a ) and T2W ( b ) MRI of a patient with vertebral column osteochondrosis shows L2 limbus vertebra ( arrowheads )
and L4/L5 grade 1 (<25 %) spondylolisthesis ( arrows ). Notice the active bone marrow edema around the bone fragment of the anterior
superior end plate of L2 in ( b )
6.9 · Sjögren Syndrome (Myoepithelial Sialadenitis)
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Further Reading
Alexander CJ. Scheuermann’s disease. Skeletal Ardiol.
1977;1:209–21.
Arlet V, etal. Scheuermann’s kyphosis: surgical management.
Eur Spine J. 2005;14:817–27.
Kapetanos GA, etal. oracic cord compression caused by
disk herniation in Scheuermann’s disease. A case report
and review of the literature. Eur Spine J. 2006;15 suppl
5:S553–8.
Paajanen H, etal. Disc degeneration in Scheuermann dis-
ease. Skeletal Radiol. 1989;18:523–6.
Swischunk LE, et al. Disk degeneration in childhood:
Scheuermann’s disease, Schmorl’s nodes, and the limbus
vertebra: MRI ndings in 12 patients. Pediatr Radiol.
1998;28:334–8.
6.9 Sjögren Syndrome (Myoepithelial
Sialadenitis)
Sjögren syndrome (SS) is a chronic, systemic autoimmune
disease characterized by in ltration of the acinar cells of the
salivary and lacrimal glands by lymphocytes, causing dry eye
( xeropthalmia ), dry mouth ( xerostomia ), and in ammation
of the cornea and the conjunctiva ( keratoconjunctivitis ).
SS is classi ed as secondary ( Sicca syndrome ) when it is
associated with other connective tissue disorders (e.g., rheumatoid arthritis) and primary when it occurs without any
manifestation of other connective tissue disorders.
Patients with SS may also develop neuropsychiatric manifestations. Brain manifestations include aseptic meningoencephalitis and multiple sclerosis-like symptoms (25 % of
cases). Psychiatric manifestations include Alzheimer-type
dementia, poor attention and concentration, and memory
de cits. Rarely, neuromyelitis optica may coexist in patients
with SS.In children, although it is rare, SS is characterized by
bilateral parotid enlargement. Very rarely, SS may be associated with amyloidosis.
Laboratory investigation reveals high titer of SS-A antibodies and SS-B antibodies plus rheumatoid factor in 50 % of cases.
Signs on Sialography
On both conventional and MR-sialography, the aff ected
salivary gland shows mottled appearance with cystic
changes due to destruction of the gland parenchyma
(sialectasia). Four stages of sialectasia are classically
described:
5 Stage 1 ( punctuate ): multiple dots <1 mm in size
(
. Fig. 6.9.1 )
5 Stage 2 ( globular ): multiple dots 1–2 mm in size
5 Stage 3 ( cavitary ): multiple dots >2 mm in size
5 Stage 4 ( destructive ): multiple irregular and
widened ducts due to gland inflammation
( sialodochitis )
Criteria to Diagnose SS Include
– Symptoms and signs of ocular dryness (e.g., positive
Schrimer’s test)
– Symptoms and signs of mouth dryness
– Evidence of autoimmune disease (e.g., positive rheuma-
toid factor)
– Exclusion of lymphoma, sarcoidosis, and acquired immu-
node ciency syndrome
SS causes salivary glands in ammation ( sialadenitis ) that
leads to parenchymal destruction and salivary gland dilatation ( sialectasia ).
Patients with SS are commonly perimenopausal women
who o en develop multiple systemic manifestations that
include mouth dryness, which a ects eating, speaking, and
may lead to teeth decay; extreme fatigue occurs in 50 % of
patients, which is more troublesome than the exocrine symptoms; intermittent polyarthritis a ecting the small joints in
asymmetrical fashion; dry skin (50 %), esophageal dysmotility (up to 90 %), vaginal atrophy and dyspareunia; and interstitial nephritis. Patients with SS have risk for developing
lymphoma, and they should be closely monitored.
. Fig. 6.9.1 Lateral conventional sialography radiograph in a
patient with submandibular sialectasis grade 1–2 seen as diff use
multiple punctuated dots between 1 and 2 mm in diameter
Signs on CT
5 In the acute phase, the affected glands are
bilaterally enlarged with parenchymal nodules of
variable size and formation of cysts, giving a
honeycomb appearance (
. Fig. 6.9.2 ).
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Chapter 6 · Rheumatology
5 The salivary glands often show signs of fibrosis and
size shrinkage in advanced stages of the disease.
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5 The lacrimal glands might be bilaterally and
symmetrically enlarged (
. Fig. 6.9.3 ).
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. Fig. 6.9.2 Sequential postcontrast head CT images ( a ) and ( b ) of a patient with Sjögren syndrome (SS) show bilateral parotid gland
bulging and enlargement ( arrows )
a
b
. Fig. 6.9.3 Sequential non-enhanced orbital CT images of
the same patient with Sjögren syndrome (SS) show bilateral
lacrimal glands enlargement ( arrows in a and b )
Signs on Brain MRI
Brain MRI shows multifocal T2 hyperintensities located in
the subcortical and periventricular white matter
(
. Fig. 6.9.4 ), enlargement of the sulci, and ventricular
dilatation. These manifestations are often seen in 50 % of
patients with focal neurological defi cits or psychiatric
manifestations.
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. Fig. 6.9.4 Axial FLAIR brain MR illustration demonstrates
patchy areas of high T2 signal intensity lesions within the white
matter as presentation of neuro-Sjögren syndrome (SS)
Signs on Parotid MRI
5 Bilateral, almost symmetrical enlargement of the
parotid glands (
5 Low T1/high T2 multiple cysts are seen within the
parotid and lack of enhancement (“salt and
pepper” appearance) (
5 Chronic disease can lead to gland shrinkage,
microcysts formation, and calcifications.
. Fig. 6.9.5 ).
. Fig. 6.9.5 ).
a
b
. Fig. 6.9.5 Axial T1W ( a ) and T2W ( b ) images of a patient
with Sjögren syndrome (SS) show bilateral moderate parotid
glands enlargement with fi ne, small cysts formation within the
gland ( arrowheads )
Further Reading
A d žić TN, etal. Multinodular pulmonary amyloidosis in pri-
mary Sjögren’s syndrome. Eur J Int Med. 2008;19:e97–8.
Kalk WWI, etal. Parotid sialography for diagnosing Sjögren
syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol
Endod. 2002;94:131–7.
Kassan SS, etal. Clinical manifestations and early diagnosis
of Sjögren’s syndrome. Arch Intern Med. 2004;164:1275–
84.
Kobayashi I, etal. Complications of childhood Sjögren syn-
drome. Eur J Pediatr. 1996;155:890–4.
Madani G, et al. In ammatory conditions of the salivary
glands. Semin Ultrasound CT MRI. 2006;27:440–51.
Mataró M, etal. Magnetic resonance abnormalities associ-
ated with cognitive dysfunction in primary Sjögren syndrome. J Neurol. 2003;250:1070–6.
Mizuno Y, etal. Recurrent parotid gland enlargement as an
initial manifestation of Sjögren syndrome in children. Eur
J Pediatr. 1989;148:414–6.
Ohbayashi N, etal. Sjögren syndrome: comparison of assess-
ments with MR sialography and conventional sialography.
Radiology. 1998;209:683–8.
Tristano AG, etal. A case of Sjögren’s syndrome with acute
transverse myelitis and polyneuropathy in a patient free of
sicca symptoms. Clin Rheumatol. 2005;25:113–4.
Varghese JC, etal. A prospective comparative study of MR
Sialography and conventional sialography of salivary duct
disease. AJR. 1999;173:1497–503.
Yoon YH, etal. Sialectasis of Stensen’s duct: an unusual case
of recurrent check swelling. Eur Arch Otorhinolaryngol.
doi:10.1007/s00405–008–0702–0.
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Chapter 6 · Rheumatology
6.10 Behçet Disease
Diagnosis is essentially clinical, and atypical presentation
can be misleading and delay the diagnosis.
Behçet disease (BD) is a relatively rare rheumatological disease characterized by the triad of aphthous oral ulcers, genital ulcers, and ocular in ammation (uveitis). e disease is
named a er its rst describer, the Turkish dermatologist
Hulusi Behçet, in 1937.
Signs on Enteroclysis
Distal ileum infl ammation and aphthous ulceration are
seen in up to 80 % of patients with GI manifestations of BD.
Pseudopolyp formation is seen in 35 % of cases.
Clinical Criteria to Diagnose BD Require
a Combination of Three or More
of the Following Findings
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– Recurrent aphthous stomatitis (79 %). e lesions are
punched out with rolled edges.
– Recurrent genital ulcers, mainly seen on the scrotum or
labial majora, which heal by scar formation.
– Anterior or posterior uveitis, presenting with pain, blurry
vision, and redness.
– Vasculitis of cutaneous or large vessels.
– Mono- or oligoarthritis a ecting the knees in particular.
– Meningoencephalitis.
– Cutaneous hyperactivity to minor trauma.
BD lesions are characterized by chronic in ammation of
the so tissues with neutrophilic in ltration, which is characteristic of BD lesions regardless of the disease stage.
BD is a multisystemic disease with many manifestations.
Documented complications of BD are those that a ect the
gastrointestinal tract (GI), large vessels (vasculitis and thrombophlebitis), musculoskeletal system (myonecrosis and
arthritis), renal system (proteinuria and hematuria), and the
nervous system (neuro-BD).
Myonecrosis should be considered in patients with
known BD presenting with acute onset of muscle pain and
edema in the absence of signs and symptoms of infection.
Renal BD is mainly caused by secondary amyloidosis (AAtype) and glomerulonephritis.
Neuro-BD has three patterns of presentation: the rst
pattern is seen as brain parenchymal lesions presenting in
stroke-like lesions and brain stem syndrome; the second pattern is migraine headache (64 %), papilledema, and increased
intracranial hypertension; and the third pattern presents in
the form of meningitis-like disease.
e esophagus is involved in 50 % of patients usually in its
midportion. Patients with esophageal involvement o en present with substernal pain, dysphagia, and occasional hematemesis. Esophageal varices may develop when the superior vena
cava is obstructed due to thrombophlebitis ( superior vena cava
syndrome ). rombophlebitis may involve the hepatic veins
resulting in Budd – Chiari syndrome (liver congestion and cir-
rhosis due to hepatic veins out ow obstruction).
GI manifestations of BD may mimic the manifestations of
Crohn’s disease (CD) radiologically and even pathologically.
However, involvement of the rectum and anus is rare in BD;
perforation is more common in BD than CD, no cobbelstoning
in BD, and the GI disease is usually milder in BD than in CD.
Signs on MRI
5 In the muscles, myonecrosis presents with
soft-tissue inflammation in the form of soft-tissue
mass with high signal intensity on T2W images and
rim contrast enhancement on postgadolinium
injection images.
5 In the brain, multiple, round, small (>5 mm) white
matter high T2 signal intensity lesions on FLAIR
and T2W images, usually in the juxtacortical region
(
. Fig. 6.10.1 ). Brain stem lesions and atrophy can
be seen.
. Fig. 6.10.1 Axial FLAIR brain MR illustration demonstrates
the neurological fi ndings in neuro-Behçet disease
Further Reading
Akpolat T, et al. Renal Behçet’s disease: an update. Semin
Arthritis Rheum. 2008;38:241–8.
Bank I, etal. Dural sinus thrombosis in Behçet’s disease.
Arthritis Rheum. 1984;27:816–8.
6.11 · Sharp Syndrome (Mixed Connective Tissue Disease)
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Benjilali L, et al. Chylothorax and chylopericardium in a
young man with Behçet disease. Joint Bone Spine.
2008;75:740–52.
Ebert EC et al. Gastrointestinal manifestations of Behçet’s
disease. Dig Dis Sci. doi:10.1007/s10620–008–0337–4.
Hwang I, etal. Necrotizing villitis and decidual vasculitis in
placentas of mothers with Behçet disease. Human Pathol.
2009;40:135–8.
Jäger HR, etal. MRI in neuro-Behcet’s syndrome: compari-
son of conventional spin-echo and FLAIR pulse sequences.
Neuroradiology. 1990;41:750–8.
Korman U, et al. Enteroclysis ndings of intestinal Behcet
disease: a comparative study with crohn disease. Abdom
Imaging. 2003;28:308–12.
La Mantia L, etal. Headache and in ammatory disorders of
the central nervous system. Neurol Sci. 2004;25:S148–53.
Oktay Kaçmaz R, etal. Ocular in ammation in Behçet dis-
ease: incidence of ocular complications and loss of visual
acuity. Am J Opthalmol. 2008;146:828–36.
Stubbs AY, et al. Myonecrosis in Behcet’s disease. Skeletal
Radiol. 2008;37:357–60.
6.11 Sharp Syndrome (Mixed Connective
Tissue Disease)
Mixed connective tissue disorder (MCTD) is a rare disease
characterized by a combination of clinical features similar to
systemic lupus erythematosus (SLE), scleroderma, and polymyositis and unusually high titers of antibody to RNasesensitive ribonucleoprotein complex (RNP).
MCTD patients have mixed features of rheumatological
symptoms that do not match a certain rheumatological category. For example, polyarthritis, myositis, and hypergammaglobulinemia are more common in MCTD than in
scleroderma. Also, polyarthritis and hypergammaglobulinemia are more frequently found in MCTD than in polymyositis. Esophageal hypermotility, pulmonary hypertension,
and absence of renal disease are typical features of
MCTD.Moreover, the detection of serological antibodies to
RNP is very speci c for MCTD and very rarely found in
other rheumatic or connective tissue disorders.
Patients with MCTD may show neurological signs like
seizures, psychosis, headache, gait disturbance, and polyneuritis. Trigeminal neuralgia has been reported in patients with
MCTD and seen frequently when the patient has more symptoms toward scleroderma. In contrast, MCTD with more
symptoms toward SLE shows neurological symptoms like
gait ataxia, transverse myelitis, and optic neuropathy. MCTD
is one of the rare causes of “treatable dementia.”
MCTD should be considered in a patient when his/her
symptoms and signs cannot be con ned to a sole rheumatological disease.
Signs on Radiograph and CT
Chest radiograph or CT may show signs of pulmonary
hypertension (e.g., prominent pulmonary trunk)
(
. Fig. 6.11.1 ).
. Fig. 6.11.1 Axial postcontrast CT angiography of the
pulmonary vessels in 32-year-old woman with Sharp’s syndrome
shows mildly dilated pulmonary trunk (28 mm) due to newly
developed pulmonary hypertension ( arrowhead )
Signs on Brain MRI
5 Optic or trigeminal neuritis is seen as contrast
enhancement of the nerve after gadolinium
injection due to the hyperemia and inflammation
(the normal nerve does not enhance after contrast
injection).
5 Transverse myelitis shows enlargement of the cord
with diffuse hyperintense signal of the spinal cord
on T2W images in the affected segment
(
. Fig. 6.11.2 ). The affected segment enhances in
an inhomogeneous pattern after contrast
administration. Cord atrophy occurs in chronic
cases. On axial segments, the high signal on T2W
images occurs on both sides of the cord, giving a
“snake-eye appearance.”
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Chapter 6 · Rheumatology
Relapsing
Polychondritis
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. Fig. 6.11.2 Sagittal T1W postcontrast cervical spine MR
illustration demonstrates transverse myelitis as thickened spinal
cord with
Further Reading
Colombo A, etal. Mixed connective tissue disease (Sharp
syndrome): description of two cases. Ital J Neurol Sci.
1983;2:203–5.
Malaviya AN, etal. Sharp’s syndrome (mixed connective tis-
sue disease) with extensive in ammatory panniculitis
complicated with pyoderma gangrenosum–a case report.
J Indian Rheumatol Assoc. 2003;11:45–50.
Matsui H, etal. Encephalopathy and sever neuropathy due to
probable systemic vasculitis as an initial manifestation of
mixed connective tissue disease. Neurol India. 2006;54:83–5.
6.12 Relapsing Polychondritis
Relapsing polychondritis (RP) is a rare, multisystemic, autoimmune disease characterized by recurrent, episodic in ammation and destruction of cartilaginous tissues.
e peak age for disease onset is the h decade. RP is
associated with HLA class II DR4 and is frequently associated with autoimmune, rheumatologic, or hematologic disorders (>30 %). Examples of diseases associated with RP
include diabetes mellitus, Crohn’s disease, rheumatoid arthritis, and lymphoma.
. Fig. 6.12.1 An illustration that demonstrates the body’s
geographic distribution of relapsing polyarthritis arthropathy
Patients with RP present with recurrent episodes of
in ammation involving the elastic cartilage of the nose, and
ears, hyaline cartilage of the peripheral joints, brocartilage
of the vertebrae, tracheobronchial cartilage, and proteoglycanrich structures like the blood vessels, eyes, and inner ear.
Symptoms include fever, asymmetric polyarthritis, weight
loss, and deformity of the ears and nose. Painful swelling of
the cartilaginous part of the ears with violaceous discoloration is its characteristic. Hearing impairment may occur if
the external auditory canal is closed due to serous otitis
media or due to vasculitis of the internal auditory artery.
Arthritis in RP is usually asymmetric (
. Fig. 6.12.1 ),
migratory, seronegative, and nonerosive, a ecting most commonly the metacarpophalangeal, PIP, wrists, and knees with
or without synovitis. In short, any joint with cartilage is
a ected or vulnerable. Symmetric involvement may be seen,
sometimes mimicking rheumatoid arthritis.
Up to 25 % of patients present with respiratory
symptoms and symptoms due to laryngeal or tracheal cartilage in ammation like hoarseness of voice, persistent
cough, stridor, and dyspnea. Mortality due to recurrent
6.12 · Relapsing Polychondritis
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respiratory infections or tracheal collapse ranges between
10 and 50 %.
Nasal chondritis is seen in up to 50 % of cases and may
lead to saddle nose deformity. Symptoms of nasal chondritis
include nasal pain, swelling, nasal stu ness, rhinorrhea, and
maybe epistaxis. Dermatological manifestations are seen in
15–20 % of cases and resemble urticaria.
Ocular manifestations include recurrent scleritis, keratitis, and/or conjunctivitis. Cardiovascular manifestations
include aortic aneurysm, aortic valve rupture, pericarditis,
and complete heart block. Glomerulonephritis with proteinuria may occur.
Laboratory investigations are nonspeci c and include
elevated levels of C-reactive protein and ESR.Rheumatoid
factor and ANA are negative unless RP coexist with SLE or
rheumatoid arthritis. Antibodies against collagen type II, IX,
and XI as well as matrilin I can be found.
Neurorelapsing polychondritis occurs as a rare manifestation of relapsing polychondritis and is characterized by
uctuating headache, confusion, gait deterioration and
ataxia, memory loss, personality change, and paranoia.
Neurological manifestations occur in up to 5 % of patients.
e main pathological features on brain biopsy have been
vasculitis and parenchymal in ammation. Other neurological
manifestations include meningoencephalitis, cranial nerves
palsies, seizures, ataxias, isolated cerebellar syndrome, and
stroke-like episodes, all of which can be attributed to cerebral
vasculitis. Uncommonly, limbic encephalitis may occur with
neurorelapsing polychondritis.
Diagnostic Criteria for Relapsing
Polychondritis
1. Recurrent chondritis of both auricles
2. Nonerosive seronegative in ammatory polychondritis
3 . I n ammation of ocular structures ( uveitis , keratitis ,
scleritis , and / or episcleritis )
4. Chondritis of the respiratory tract involving laryngeal or
tracheal cartilage
5. Cochlear and/or vestibular damage causing sensorineural
hearing loss, tinnitus, and/or vertigo
Diagnosis is established if (1) the patient has three of the
above clinical criteria, (2) the patient has one criteria with
cartilage biopsy conformation, or (3) chondritis at two or
more separate anatomical locations with respond to steroid
therapy.
D i ff erential Diagnoses and Related Diseases
MAGIC Syndrome
z
MAGIC syndrome is a disease characterized by M outh A nd
G enital ulcers with I n amed C artilage. MAGIC syndrome is
simply a combination of both relapsing polychondritis and
Behcet’s disease (. Fig. 6.12.2 ).
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Chapter 6 · Rheumatology
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. Fig. 6.12.2 Multiple images of a 43-years-old female patient with MAGIC syndrome that shows the classical clinical signs of ear pinna redness
( arrowhead ) and mouth ulcers seen in the tongue ( arrow )
Signs on Radiographs
On plain radiography, patients with relapsing
polychondritis present with calcifi ed ear pinna and/or
nasal cartilage (pathognomonic) (
. Fig. 6.12.3 ).
6.12 · Relapsing Polychondritis
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. Fig. 6.12.3 Plain radiographs of the nose and the ears of a patient with relapsing polychondritis shows the classical feat of calcifi ed ear
pinnae bilaterally and the nasal cartilage
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