pediatric rheumatology case dr. christine bernal iiib-4
TRANSCRIPT
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Pediatric Rheumatology CaseDr. Christine BernalIIIB-4
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Salient Features
Luisa, 16 y/o, female
Diagnosed with SLE at 12
Prolonged fever Malar rash Photosensitivity Hair loss Oral ulcers
Easy fatigability Anemia Neutropenia Thrombocytopenia (+) ANA (+) anti-dsDNA
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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
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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
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ACR Criteria for SLE presence of four or more of the following 11 criteria, serially or
simultaneously, during any period of observation
1. Malar rash2. Discoid rash
3. Photosensitivity
4. Oral ulcers
5. Arthritis (non-erosive)
6. Serositis (Pleuritis or Pericarditis)
7. Renal disorder • persistent proteinuria• > 500 mg per 24 hours (0.5 g per day) or > 3+ • cellular casts
8. Neurologic disorder
9. 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
• Immunologic disorder
• Antinuclear antibodies
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In the patient…
Malar rash Photosensitivity Oral ulcers Anemia Thrombocytopenia (+) ANA (+) anti-dsDNA L knee – warm tender and swollen w/
limited ROM
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Initial Impression and Differential Diagnosis
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What is your Initial Impression?
SEPTIC ARTHRITIS probably bacterial infection
Left Knee: + trauma
Abrupt in onset< 2weeks (acute)
Unilateral pain and swelling,
warmLimited range of
motion
Patient:Immunocompromi
sedill looking
Fever and chills
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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
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SEPTIC ARTHRITIS
Etiologic Agent: Staphylococcus aureus (most common) Gonococcal (sexually active) Candida (disseminated infection) Viral (systemic infection)
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SEPTIC ARTHRITIS
Infection of joints are followed by Penetrating injuries: Trauma Arthroscopy Prosthetic Joint Surgery Intra-articular Steroid Injection Orthopedic Surgery
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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
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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)
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Differential Diagnosis
Drug induced: Glucocorticoid treatment
Can cause osteopenia and osteonecrosis Hydrochloroquine
Can cause osteonecrosis
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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.
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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
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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
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The goals of management would include:
To treat the feverTo 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
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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.
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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.
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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.
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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.