Download A Great Imitator: Imaging the Musculoskeletal Sequelae of Systemic Lupus Erythematosus

June 2015
A Great Imitator:
Imaging the Musculoskeletal Sequelae of
Systemic Lupus Erythematosus
Daniel Driscoll, Harvard Medical School Year III
Gillian Lieberman, MD
Agenda
1.  Brief Introduction to Systemic Lupus
Erythematosus
2.  Index Patient Presentation
•  Basics of SLE Arthropathy Diagnosis, Imaging and
Treatment
3.  Imaging of additional musculoskeletal sequelae
of SLE
Daniel Driscoll, HMS III
Gillian Lieberman, MD
2
Agenda
1.  Brief Introduction to Systemic Lupus
Erythematosus
2.  Index Patient Presentation
•  Basics of SLE Arthropathy Diagnosis, Imaging and
Treatment
3.  Imaging of additional musculoskeletal sequelae
of SLE
Daniel Driscoll, HMS III
Gillian Lieberman, MD
3
Introduction to Systemic Lupus Erythematosus
•  SLE is a systemic autoimmune disease
•  Epidemiology:
–  Females > Males
–  Over one million people with SLE in the
US
•  Pathogenesis:
–  There are two main mechanisms of SLE
sequelae
1. Reduced cell apoptosis
2. Self-antibody generation
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Image adapted from healthtap.com
Classic malar (“butterfly”)
rash of SLE
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Systemic Lupus Erythematosus Diagnosis Criteria
•  SLE Diagnosis requires 4 of 11 of the following American
College of Rheumatology Criteria
(Mnemonic: SOAP BRAIN MD)
– 
– 
– 
– 
– 
– 
– 
– 
– 
– 
– 
Serositis
Oral ulcers
Arthritis
Photosensitivity
Blood disorders
Renal involvement
Antinuclear antibodies
Immunologic phenomena (eg, dsDNA; anti-Smith [Sm] antibodies)
Neurologic disorder
Malar rash
Discoid rash
Daniel Driscoll, HMS III
Gillian Lieberman, MD
5
SLE is a Multisystem Disease
• 
Cardiovascular SLE sequelae
(16-44% of patients)
–  Valve disease
–  Coronary artery disease
• 
Gastrointestinal SLE sequelae
(20-40% of patients)
–  Pancreatitis
–  Colitis
• 
Neurologic and Psychiatric SLE
sequelae (10-80% of patients)
–  Seizures
–  Psychosis
• 
Respiratory SLE sequelae
–  Serositis
• 
Renal SLE sequelae
–  Lupus nephritis
Daniel Driscoll, HMS III
Gillian Lieberman, MD
• 
Musculoskeletal SLE sequelae
(90-95% of patients)
–  Joints
•  Lupus arthritis
•  Deforming Arthropathy
–  Mild Deforming Arthropathy
–  “Rhupus”
–  Jaccoud’s Arthropathy
–  Ligaments/Tendons
•  Tenosynovitis
–  Bones
•  AVN
•  Insufficiency Fracture
–  Muscles
•  Myositis
–  Fat
•  Panniculitis
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SLE is a Musculoskeletal Disease
• 
• 
Musculoskeletal Sequelae are found in 90-95%
of SLE patients
SLE affects different parts of the MSK system
–  Joints
•  Lupus arthritis
•  Deforming Arthropathy
–  Mild Deforming Arthropathy
–  “Rhupus”
–  Jaccoud’s Arthropathy
–  Ligaments/Tendons
•  Tenosynovitis
–  Bones
•  Avascular necrosis (AVN)
–  Muscles
•  Myositis
–  Fat
•  Panniculitis
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Images adapted from imgbuddy.com
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SLE Joint Sequelae (1)
There are two different types of SLE joint sequelae:
(1) SLE Arthritis and (2) Deforming arthropathy
SLE Arthritis:
•  Common in SLE patients (95%
of patients)
•  non-erosive, non-deforming
arthritis
•  moderately painful, symmetric
and migratory
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Deforming Arthropathy:
•  Less common in SLE Patients
•  Deforming but usually nonerosive process
•  Several sub-classifications
(see table on next slide for
distinguishing factors):
–  Mild Deforming Arthropathy
–  Jaccoud’s Arthropathy
–  “Rhupus”
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SLE Joint Sequelae (2)
SLE Arthritis
Mild Deforming
Arthropathy
Jaccoud's
Arthropathy
Rhupus
Erosions?
No
No
No
Yes
Deforming?
No
Yes, mild
Yes, severe
Yes
~90%
~5%
10-35%
3-5%
Limited
Yes
Yes
Yes
Percentage of
SLE patients
Role for
imaging?
Daniel Driscoll, HMS III
Gillian Lieberman, MD
9
Agenda
1.  Brief Introduction to Systemic Lupus
Erythematosus
2.  Index Patient Presentation
•  Basics of SLE Arthropathy Diagnosis, Imaging and
Treatment
3.  Imaging of additional musculoskeletal sequelae
of SLE
Daniel Driscoll, HMS III
Gillian Lieberman, MD
10
Our Patient:
•  A 48 year old woman presents with
one year history of pain and stiffness
in digits 2, 3, 4 and 5 on her right
hand.
•  What is the appropriate imaging?
Daniel Driscoll, HMS III
Gillian Lieberman, MD
11
ACR Appropriateness Criteria
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Image from American College of Radiology
12
Hand Anatomy
Bones
Joints
Distal Phalanges
Intermediate
Phalanges
Proximal Phalanges
Metacarpals
Distal interphalangeal
joints (DIP)
Proximal
Interphalangeal joints (PIP)
Metacarpophalangeal
joints (MCP)
Carpometacarpal joints
(CMC)
Carpals
Carpal Ulnar Joints
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Images adapted From Wikimedia Commons
13
Our Patient’s Radiographs (Right Hand)
Frontal
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Lateral
Images from PACS, BIDMC
Courtesy of Dr. Jim Wu
Oblique
14
Our Patient’s Radiographic Findings
Frontal
Ulnar deviation and MCP
subluxation
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Lateral
Boutonnière
Deformity
Images from PACS, BIDMC
Courtesy of Dr. Jim Wu
Oblique
*NO erosions
noted*
15
Initial Differential Diagnosis for our patient:
Arthritis with Multiple Subluxations and/or Ulnar Deviation
• 
• 
• 
• 
• 
• 
• 
• 
• 
Rheumatoid Arthritis
Ehlers-Danlos Syndrome
Jaccoud’s arthritis (post-rheumatic fever)
Juvenile chronic arthritis
Systemic Lupus Erythematosus
Mixed Connective Tissues
Neuropathic arthropathy with or without
destruction
Other advanced arthritis
Psoriatic Arthritis
Daniel Driscoll, HMS III
Gillian Lieberman, MD
From Reder and Felson’s Gamuts in Radiology, D-227 p. 295
16
More information about our patient:
•  Chief Complaint: A 48 year old woman with long-standing
history of SLE presents with one year history of inability to
extend digits 2-5 on right hand.
•  HPI:
–  Denies tingling or numbness
–  Patient is able to place joints in place manually
•  Physical Exam
–  On exam, deformities are passively correctable
•  PMH and Labs:
– 
– 
– 
– 
Positive ANA and anti-Sm antibody
Negative Anti-U1RNP Antibody
Negative Rheumatoid Factor and Anti-CCP Antibody
No history of rheumatic fever
Daniel Driscoll, HMS III
Gillian Lieberman, MD
17
Refined Differential Diagnosis for our patient
After learning more about our patient, we can dismiss nearly
all potential diagnoses
• 
• 
• 
• 
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• 
• 
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Systemic Lupus Erythematosus
Rheumatoid Arthritis
Ehlers-Danlos Syndrome
Post-rheumatic fever Jaccoud’s arthritis
Juvenile chronic arthritis
Mixed Connective Tissue Disease
Neuropathic arthropathy with or without destruction
Psoriatic Arthritis
Daniel Driscoll, HMS III
Gillian Lieberman, MD
From Reder and Felson’s Gamuts in Radiology D-227 p. 295
18
SLE Arthropathy Diagnosis Algorithm
Start HERE
MCP Finger
deviation/subluxation
No
IMAGING:
Ultrasound or MRI
IMAGING:
RADIOGRAPHY
Erosions?
Erosions?
JAI > 5
Yes
Diagnosis: “Rhupus”
No
*JAI = Jaccoud
Arthropathy
Index
Yes
Diagnosis: Jaccoud’s
Arthropathy
Daniel Driscoll, HMS III
Gillian Lieberman, MD
JAI < or = 5
Adapted from Messuti et al. 2014
Diagnosis: Mild
Deforming Arthropathy
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Classic Deformities of Jaccoud’s Arthropathy
“Z” Deformity
Ulnar Deviation
Boutonniere
Deformity
Swan Neck
Deformity
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Images from PACS, BIDMC and
adapted from www.kleisertherapy.com
20
Jaccoud Arthropathy Index
Jaccoud Arthropathy Index (JAI)
(greater than 5 points is diagnostic for Jaccoud Arthropathy)
Deformity
Ulnar Drift
Swan Neck Deformities
Boutonniere Deformities
Z deformity
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Affected Fingers
<=50%
>50%
<=50%
>50%
<=50%
>50%
1
2
Adapted from Messuti et al. 2014
Points
2
3
2
3
1
2
2
3
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Which deforming arthropathy does our patient have?
1.  Ulnar deviation of digits 2,
3, 4 and 5
2.  No degenerative changes
or erosions
3.  JAI < 5
Diagnosis: Mild Deforming
Arthropathy
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Image from PACS, BIDMC
Courtesy of Dr. Jim Wu
22
Arthroplasty for SLE Deforming Arthropathy
Our patient received MCP joint arthroplasty in digits 2-5
as definitive treatment for her deforming arthropathy
Frontal
Oblique
Lateral
Radiolucent implants
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Images from PACS, BIDMC
Courtesy of Dr. Jim Wu
23
Companion Patient 1: Potential Rhupus Patient
Frontal
Oblique
MCP Joint subluxation Subchondral erosions
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Images from PACS, BIDMC
Courtesy of Dr. Jim Wu
Lateral
Boutonnière Deformity
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Companion Patients 2 and 3
Ultrasound can be used to show tenosynovitis, joint erosion, or joint effusion in SLE patients
Patient 2. SLE patient with
tenosynovitis of third finger flexor
tendon showing positive power
Doppler signal
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Patient 3. SLE patient who is
ANA positive with erosion and
large effusion of the second MCP
joint
Images and descriptions from Ball EM, Bell AL.
Rheumatology. 2012.
25
Agenda
1.  Brief Introduction to Systemic Lupus
Erythematosus
2.  Index Patient Presentation
•  Basics of SLE Arthropathy Diagnosis, Imaging and
Treatment
3.  Imaging of additional musculoskeletal sequelae
of SLE
Daniel Driscoll, HMS III
Gillian Lieberman, MD
26
Avascular Necrosis
Epidemiology: 13% of SLE
patients have AVN
Late AVN lesions:
•  detectable by Radiograph
•  Serpiginous sclerosis
Early AVN lesions:
•  detectable by MRI
•  Serpiginous sclerosis
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Frontal Radiograph
Coronal MRI DWI
Images and descriptions from Goh YP, Naidoo P, Ngian GS.
Clin Radiol. 2013.
27
Myositis
•  Definition: Inflammation of muscle tissue
•  Epidemiology: 8% of SLE patients
•  T2 MRI with fat suppression findings: linear areas of high
signal intensity
Axial
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Coronal
Images from Goh YP, Naidoo P, Ngian GS. Clin Radiol. 2013.
28
Tenosynovitis
Ultrasound
•  Definition: Inflammation of synovium
surrounding tendon
•  Epidemiology: 10-44% of SLE patients
Axial and Coronal MRI T1
Daniel Driscoll, HMS III
Gillian Lieberman, MD
•  Ultrasound findings: Fluid collection
around the tendon and within its sheath
•  MRI findings: hyperintensity
Images from Goh YP, Naidoo P, Ngian GS. Clin Radiol. 2013.
29
Panniculitis
•  Definition: Inflammation
of subcutaneous fat
tissue
•  Epidemiology: <1% of
SLE patients
Axial STIR
Axial T1 FS
Imaging findings:
•  MRI STIR (post contrast)
•  hyperintensity
•  MR T1 FS (post contrast)
•  hyperintensity
Coronal STIR
Daniel Driscoll, HMS III
Gillian Lieberman, MD
Images from PACS, BIDMC
Courtesy of Dr. Jim Wu
Coronal T1 FS
30
Summary
1.  SLE is an autoimmune disease with multiple
potential effects on the body and
musculoskeletal system
2.  Radiographs and ultrasound, along with clinical
history, are efficacious in the diagnosis of SLE
deforming arthropathy
3.  Additional musculoskeletal sequelae of SLE can
be imaged with different modalities, with MRI
being efficacious for detection of inflammation
Daniel Driscoll, HMS III
Gillian Lieberman, MD
31
Acknowledgements
Jim Wu, MD
Michael Acord, MD
Gillian Lieberman, MD
32
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