CONNECTIVE TISSUE DISEASE
SLE
• Joint space narrowing is uncommon in
systemic lupus erythematosus
A. TRUE
B. FALSE
ANSWER
• Joint space narrowing is uncommon in systemic
lupus erythematosus
A. TRUE
B. FALSE
- Joint space narrowing is uncommon, and when present
is likely due to disuse atrophy or pressure from an
adjacent subluxed bone. Altered stresses across the joint
may also cause a "hook erosion" at the metacarpal
heads due to capsular stress, mimicing findings of
rheumatoid arthritis. This is more often observed on the
radial side.
• Patients with SLE are at increased risk for
insufficiency fractures, possibly due to :
A.
B.
C.
D.
Disuse demineratization
Osteopenia secondary to steroid therapy
Normal or abnormal forces on weakened bone
All of the above
ANSWER
• Patients with SLE are at increased risk for
insufficiency fractures, possibly due to :
A.
B.
C.
D.
Disuse demineratization
Osteopenia secondary to steroid therapy
Normal or abnormal forces on weakened bone
All of the above
• Distribution of radiographic abnormalities in
the hand of patients with lupus arthropathy
A. MCP and PIP joints of all digits
B. MCP and IP joints of the ulnar digits, particularly
the 4th and 5th fingers
C. MCP and IP joints of all the digits; prominent
abnormalities of the thumb
D. All of the above
ANSWER
• Distribution of radiographic abnormalities in
the hand of patients with lupus arthropathy
A. MCP and PIP joints of all digits (RHEUMATOID
ARTHRITIS)
B. MCP and IP joints of the ulnar digits, particularly
the 4th and 5th fingers (CLASSIC JACCOUD’S
ARTHROPATHY)
C. MCP and IP joints of all the digits; prominent
abnormalities of the thumb
D. All of the above
CASE 1:
• 27 year old female with
SLE, on steroid therapy
• (+) left hip pain
ANSWER: AVASCULAR NECROSIS OF
THE FEMORAL HEAD
•
•
•
•
•
Osteonecrosis or AVN occurs in 5%–
50% of SLE patients
Mainly affects weight-bearing
The femoral head is most commonly
affected, followed by the humeral
head, femoral condyle, and tibial
plateau
Radiographs are usually normal in
early AVN, and late changes of bone
sclerosis indicate the presence of
irreversible articular damage
With radiography, a grading system is
used to denote the severity of AVN
according to the sclerosis, flattening
of the articular surface, and joint
space abnormalities
– stage 0 (clinically suspected AVN) to
stage V (obvious joint space narrowing
and articular surface disruption)
CASE 2
• Same patient
• (+) ankle pain
• No history of trauma
ANSWER: INSUFFICIENCY FRACTURE
DISTAL FIBULAR DIAPHYSIS
•
•
•
•
Normal stress on abnormal bone can
cause insufficiency fractures
In patients with SLE, the
pathogenesis of insufficiency
fractures is unclear but may be
related to deconditioning,
accelerated bone loss due to steroid
therapy, or both
MR imaging may depict early or
subtle insufficiency fractures, which
may be occult on radiographs due to
severe osteoporosis
At T2-weighted MR imaging,
insufficiency fractures appear as
areas of high signal intensity due to
bone marrow edema in characteristic
stress locations
CASE 3
• SLE patient
• Leg pain
• (+) fever
ANSWER: OSTEOMYELITIS
• Dermatologic factors,
corticosteroids, and vasculopathy
predispose patients with SLE to
septic arthritis and osteomyelitis
• Because of steroid therapy,
infection can be masked, and
chronic indolent disease is often
seen
• Organisms: S aureus, gramnegative bacilli, and M
tuberculosis
• Radiographic : progressive bone
destruction and cartilage loss,
periostitis, and joint effusion
CASE 4
ANSWER: MULTIPLE BONE INFARCTS
Anteroposterior radiograph of the left
knee shows sclerosis in the distal femur
and proximal tibia.
Sagittal T1-weighted MR image shows foci of
isointense signal encircled by a low-signalintensity rim in the distal femur and proximal
tibia (arrows). The hypointense rim represents
reparative granulation tissue surrounding
infarcted bone.
SCLERODERMA
Patient with scleroderma.
ANSWER: ACRO-OSTEOLYSIS
•
Refers to terminal tuft bony erosions.
It is associated with a heterogeneous
group of pathological entities
– primary acroosteolysis - Hajdu-Cheney
syndrome
– psoriatic arthritis
– hyperparathyroidism
– polyvinyl chloride exposure
– insensitivity to pain
– ergot poisoning
– thermal injury
– extreme cold : frost bite
– extreme heat : burns
– leprosy
– juvenile chronic arthritis (JCA/JIA)
– dermatomyositis
– vascular occlusion
– Raynaud disease
– Scleroderma
• Alterations at the distal IP joints
are usually confined to regional
or periarticular osteoporosis and
swelling and thickening of the
adjacent soft tissue, without
evidence of joint space narrowing
or osseous erosions
• However, joint manifestations in
scleroderma closely resemble
those of RA, with osteoporosis,
joint space narrowing and
osseous erosions
• In some patients with
scleroderma, alterations occur at
the DIP, articulations that are not
commonly involved in RA.
• Scleroderma-like syndrome with eosinophilia,
hypergammaglobulinemia, but without
systemic or vascular involvement
A.
B.
C.
D.
SCLERODERMA ADULTORUM
SHULMAN’S SYNDROME
THIBIERGE-WISSENBACH SYNDROME
CREST SYNDROME
ANSWER
• Scleroderma-like syndrome with eosinophilia,
hypergammaglobulinemia, but without systemic or
vascular involvement
A. SCLERODERMA ADULTORUM – benign, self limited
condition unrelated to scleroderma that occurs after
infection; characterized by non-pitting edema of the skin
and spontaneous resolution within a few months
B. SHULMAN’S SYNDROME
C. THIBIERGE-WISSENBACH SYNDROME – combination of
calcinosis and digital ischemia
D. CREST SYNDROME – subcutaneous calcinosis, reynaud’s
phenomenon, esophageal abnormalities, sclerodactyly,
telangirctasia
• Chemical that can induce cutaneous
abnormalities simulating those of scleroderma
A.
B.
C.
D.
VINYL CHLORIDE
PENTAZOCINE
BLEOMYCIN
ALL OF THE ABOVE
ANSWER
• Chemical that can induce cutaneous
abnormalities simulating those of scleroderma
A. VINYL CHLORIDE
B. PENTAZOCINE
C. BLEOMYCIN
D. ALL OF THE ABOVE
– Other chemical agents: solvents, paraffin, silicone
implants, coccaine, rapeseed oil
• Relatively specific dental sign of scleroderma
A.
B.
C.
D.
THICKENING OF PERIODONTAL MEMBRANE
THICKENING OF ENAMEL AND DENTIN
THICKENING OF DENTIN AND GINGIVA
THICKENING OF GINGIVA AND ENAMEL
ANSWER
• Relatively specific dental sign of scleroderma
A.
B.
C.
D.
THICKENING OF PERIODONTAL MEMBRANE
THICKENING OF ENAMEL AND DENTIN
THICKENING OF DENTIN AND GINGIVA
THICKENING OF GINGIVA AND ENAMEL
• Sites of osteolysis in scleroderma except
A.
B.
C.
D.
PHALANGES OF THE HAND AND FOOT
CARPAL BONES
PROXIMAL POSTIONS OF RADIUS AND ULNA
MANDIBLE
ANSWER
• Sites of osteolysis in scleroderma except
A. PHALANGES OF THE HAND AND FOOT
B. CARPAL BONES
C. PROXIMAL POSTIONS OF RADIUS AND ULNA
(DISTAL)
D. MANDIBLE
- OTHER SITES: RIBS, CLAVICLE, HUMERUS,
ACROMION, CERVICAL SPINE
RHEUMATIC FEVER
• Patient with rheumatic
fever
• A deforming non
erosive arthropathy
characterised by ulnar
deviation of the second
to 5th fingers with MCP
subluxation.
• DIAGNOSIS?
ANSWER: JACCOUD’S ARTHROPATHY
•
hand x rays typically shows
marked ulnar subluxation
and deviation at the MCP
joints
• absence of erosions is a
notable feature, although
occasionally hook erosions
may be observed, which are
similar to those seen in SLE
and AS sevidence of muscle
(soft tissue) atrophy also
may be present
• Diseases that may lead to deforming nonerosive arthropathy
A.
B.
C.
D.
E.
F.
G.
RHEUMATIC FEVER
SLE
RHEUMATOID ARTHRITIS
AGAMMAGLOBULINEMIA
A, B & C
ALL OF THE ABOVE
NONE OF THE ABOVE
ANSWER
• Diseases that may lead to deforming non-erosive
arthropathy
A. RHEUMATIC FEVER
B. SLE
C. RHEUMATOID ARTHRITIS
D. AGAMMAGLOBULINEMIA
E. A, B & C
F. ALL OF THE ABOVE
G. NONE OF THE ABOVE
- ALSO EHLERS-DANLOS SYNDROME
• CLINICAL MANIFESTATIONS NECESSARY FOR
DIAGNOSIS OF JACCOUD’S ARTHROPATHY
EXCEPT:
A. HX OF RECURRENT ATTACKS OF ACUTE
RHEUMATIC FEVER
B. IMMEDIATE RECOVERY AFTER JOINT
INFLAMMATION
C. ELICITATION OF TENDON CREPITUS
D. JOINT DISEASE THAT IS GENERAKKY
ASYMPTOMATIC
ANSWER
• CLINICAL MANIFESTATIONS NECESSARY FOR
DIAGNOSIS OF JACCOUD’S ARTHROPATHY EXCEPT:
A. HX OF RECURRENT ATTACKS OF ACUTE RHEUMATIC
FEVER
B. IMMEDIATE RECOVERY AFTER JOINT INFLAMMATION
(DELAYED RECOVERY; WITH SUBSEQUENT DEFORMITY
OF THE MCP JOINT)
C. ELICITATION OF TENDON CREPITUS
D. JOINT DISEASE THAT IS GENERAKKY ASYMPTOMATIC
- ALSO: CHARACTERISTIC ARTICULAR DEFORMITY
CONSISTING OF FLEXION AND ULNAR DEVIATION AT THE
MCP, PARTICULARLY IN THE 4TH AND 5TH DIGITS