Pediatric Rheumatology CaseDr. Christine BernalIIIB-4

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

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 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

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?

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

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

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

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

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.