1. PERSISTENT KNEE SWELLING IN A LUPUS PATIENT
Pediatric Rheumatology Case
Dr. Christine Bernal
IIIB-4
PERSISTENT KNEE SWELLING IN A LUPUS PATIENT

2. Salient Features

3. 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

4. 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

5. 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

6. ACR Criteria for SLE

7. ACR Criteria for SLE
presence of four or more of the following 11 criteria, serially or simultaneously, during any period of observation
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
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
Immunologic disorder
Antinuclear antibodies

8. In the patient… Malar rash Photosensitivity Oral ulcers Anemia
Thrombocytopenia
(+) ANA
(+) anti-dsDNA
L knee – warm tender and swollen w/ limited ROM

9. Initial Impression and Differential Diagnosis

10. What is your Initial Impression?
Patient:
Immunocompromised
ill looking
Fever and chills
SEPTIC ARTHRITIS probably bacterial infection
Left Knee:
+ trauma
Abrupt in onset
< 2weeks (acute)
Unilateral pain and swelling, warm
Limited range of motion

11. 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

12. SEPTIC ARTHRITIS Etiologic Agent: Staphylococcus aureus (most common)
Gonococcal (sexually active)
Candida (disseminated infection)
Viral (systemic infection)

13. SEPTIC ARTHRITIS
Infection of joints are followed by Penetrating injuries:
Trauma
Arthroscopy
Prosthetic Joint Surgery
Intra-articular Steroid Injection
Orthopedic Surgery

14. 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

15. 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)

16. Differential Diagnosis
Drug induced:
Glucocorticoid treatment
Can cause osteopenia and osteonecrosis
Hydrochloroquine
Can cause osteonecrosis

17. WORK - ups

18. Erythrocyte sedimentation rate (ESR) and C reactive protein
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.

19. RF (Rheumatoid-factor)
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

20. 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

21. MANAGEMENT

22. 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

23. 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.

24. 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.

25. 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.

26. 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.