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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.