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
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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
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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
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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…
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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.