3. What is your Initial Impression and give your Differential Diagnosis.
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Transcript 3. What is your Initial Impression and give your Differential Diagnosis.
Pediatric Rheumatology Case
Dr. Christine Bernal
IIIB-4
PERSISTENT KNEE SWELLING IN
A LUPUS PATIENT
SALIENT FEATURES
Salient Features
Luisa, 16 y/o, female
Easy fatigability
Diagnosed with SLE at
Anemia
12
Prolonged fever
Malar rash
Photosensitivity
Hair loss
Oral ulcers
Neutropenia
Thrombocytopenia
(+) ANA
(+) anti-dsDNA
In January 2009….
Pain on the L knee with swelling after a fall
With fever and chills
Self-medicated with Ibuprofen for 2 weeks,
no improvement
PE Findings
Ill-looking
Wheelchair borne
BP: 110/70
CR: 102/min
RR: 24/min
Temp: 39.8°C
No rash or oral lesions
Regular heart rate and
rhythm
No murmur or rub
Regular heart rate and
rhythm
No murmur or rub
Clear breath sounds
Soft non-tender
abdomen, no
hepatosplenomegaly
L knee – warm tender
and swollen w/ limited
ROM
ACR CRITERIA FOR SLE
ACR Criteria for SLE
presence of four or more of the following 11 criteria, serially or
simultaneously, during any period of observation
1.
2.
3.
4.
5.
6.
7.
Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis (non-erosive)
Serositis (Pleuritis or Pericarditis)
Renal disorder
• persistent proteinuria
• > 500 mg per 24 hours (0.5 g per day) or > 3+
• cellular casts
8.
9.
Neurologic disorder
Hematologic disorder
• hemolytic anemia with reticulocytosis
• leukopenia, < 4,000 per mm3 (4.0 _ 109 per L) on two or more occasions
• lymphopenia, < 1,500 per mm3 (1.5 _ 109 per L) on two or more occasions
• thrombocytopenia, < 100 _ 103 per mm3 (100 _ 109 per L) in the absence of
offending drugs
10. Immunologic disorder
11. Antinuclear antibodies
In the patient…
Malar rash
Photosensitivity
Oral ulcers
Anemia
Thrombocytopenia
(+) ANA
(+) anti-dsDNA
L knee – warm tender and swollen w/ limited
ROM
Initial Impression and
Differential Diagnosis
What is your Initial
Impression?
Patient:
Immunocompromised
ill looking
Fever and chills
Left Knee:
+ trauma
Abrupt in onset
< 2weeks (acute)
Unilateral pain and
swelling, warm
Limited range of
motion
SEPTIC ARTHRITIS
probably bacterial
infection
SEPTIC ARTHRITIS
Occurs as a result of hematogenous seeding
of infectious organism in the synovial fluid
Consequence of inflammatory reaction
joint cartilage and synovial are damage by the
proteolytic enzymes and mechanical factors.
Common in young children
SEPTIC ARTHRITIS
Etiologic Agent:
Staphylococcus aureus (most common)
Gonococcal (sexually active)
Candida (disseminated infection)
Viral (systemic infection)
SEPTIC ARTHRITIS
Infection of joints are followed by Penetrating
injuries:
Trauma
Arthroscopy
Prosthetic Joint Surgery
Intra-articular Steroid Injection
Orthopedic Surgery
Differential Diagnosis
Juvenile Rheumatoid Arthritis
Onset < 16 y/o
Persistent arthritis in at least one joint for 6 weeks
polyarticular course and functional disability
symmetric, large and small joints
Exclusion for other diagnoses
Girls > boys
production of JRA – causes synovial
inflammation, bone erosion, fever, rash, joint
destruction; can be treated with biologic agents
Differential Diagnosis
Systemic Lupus Erythematosus
An episodic, multisystem, autoimmune disease
Widespread inflammation of blood vessels and
connective tissues
Intermittent Polyarthritis
Mild from disabling
Characterized by soft tissue swelling and
tenderness in joints of the hands, wrist, and knees
Presence of autoantibodies (hallmark of SLE)
Differential Diagnosis
Drug induced:
Glucocorticoid treatment
Can cause osteopenia and osteonecrosis
Hydrochloroquine
Can cause osteonecrosis
WORK - UPS
Culture of the synovial fluid or of synovial tissue itself is
the only definitive method of diagnosing septic arthritis.
Erythrocyte sedimentation rate (ESR) and C
reactive protein
useful to screen for infectious and rheumatic
diseases
A normal ESR value does not exclude rheumatic
disease.
Infections = increased ESR
High values persisting for more than several weeks
may necessitate further evaluation, depending on
the associated symptoms, physical findings, and
other laboratory abnormalities.
ANA test
a screening test for specific anibodies against
nuclear constituents
A positive titer (≥1 : 80) is a nonspecific reflection of
increased lymphocyte activity
RF (Rheumatoid-factor)
seropositivity may be associated with onset of
polyarticular involvement in an older child (≈8%)
and the development of rheumatoid nodules
Anti–double-stranded DNA
are more specific for lupus
often reflect the degree of serologic disease activity
Serum levels of total hemolytic complement
(CH50), C3, and C4
decreased in active disease and provide a second
measure of disease activity
Anti-Smith antibody
found specifically in patients with lupus, does not
measure disease activity
MANAGEMENT
The goals of management would include:
To treat the fever
To protect the organs by decreasing
inflammation and/or the level of autoimmune
activity in the body -- To reduce the swelling
and relieve the pain on her left knee
To reduce the swelling and
relieve the pain on her left
knee
Medical management of infective arthritis
focuses on the:
Adequate and timely drainage of the infected
synovial fluid.
Administration of appropriate antimicrobial
therapy.
Immobilization of the joint to control pain.
The empirical choice of antibiotic therapy is
based on results of the Gram stain and the
clinical picture and background of the patient.
Initial antibiotic choices must be empirical,
based on the sensitivity pattern of the
pathogens.
Because many isolates of group B streptococci
have become tolerant of penicillin, use a
combination of penicillin and gentamicin or a 2nd
or 3rd -generation cephalosporin.
Preferably, the antibiotic should be
bactericidal with some effect against the
slow-growing organisms that are protected
within a biofilm.
Rifampin fulfills these requirements. It should
never be used alone because of the rapid
development of bacterial resistance to the
drug.
Surgical Care
Surgical drainage is indicated when one or
more of the following occur:
The appropriate choice of antibiotic and
vigorous percutaneous drainage fails to clear
the infection after 5-7 days.
The infected joints are difficult to aspirate
(eg. hip), or adjacent soft tissue is infected.