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Case Conference October 14 , 2009 JRA

Case Conference October 14 , 2009 JRA. Samatha Madhavarapu. 21 m/o F with limping. HPI. Intermittent limp of R leg started 6 weeks ago. Constant limp since 3 days Worse upon awakening Stiffness in R knee. Transient warmth and redness +

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Case Conference October 14 , 2009 JRA

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  1. Case ConferenceOctober 14 , 2009JRA Samatha Madhavarapu

  2. 21 m/o F with limping

  3. HPI • Intermittent limp of R leg started 6 weeks ago. • Constant limp since 3 days • Worse upon awakening • Stiffness in R knee. • Transient warmth and redness + • Not able to bear weight initially, improves over 2 hrs. • Was outdoors in Upstate at BBQ 8 weeks ago • No fever, rash, recent URI • No trauma, diarrhea,

  4. HPI Contd… • PMH: None • FH: grand father has seizure disorder. No bone/joint problems • Immunizations: UTD • NKDA • Meds: Tylenol PRN pain. • Labs done 6 weeks ago: wbc7.6, 33.9/66.3, ESR 18, xray R knee Normal.

  5. Physical Exam • Vital signs: • HEENT : Normal • Heart , Lungs ,Abdomen: Normal • Skin: No rash • R knee: decreased extension, no swelling, no redness, no warmth. • Other Jts: FROM.

  6. LABS • BMP: 136/4.9/ 101/22/12/0.4/139/10.5 • Total Protein/ Albumin: 7.5/4.7 • LFT: 0.2/01, 38/19, 244 • CBC: 7.3/ 12/36.3/392 • ESR: 15

  7. Other Labs • CRP: 0.2 • ANA negative • Lyme Ab titre: 1.2 • Anti CCP antibody: 8.3 • HLA B 27: Negative • RF: 10.0 • Ultrasound of Knees: Small R knee jt effusion

  8. DD of Arthritis/extremity pain in Children • Rheumatic & Inflammatory Diseases • Seronegative Spondyloarthropathies • Infectious Illnesses • Reactive Arthritis • Immunodeficiencies • Metabolic Disorders • Bone & Cartilage Disorders • Neoplastic Disorders • Hematologic Disorders • Pain Syndromes

  9. Juvenile Rheumatoid Arthritis • JRA most common rheumatic disease of childhood • Synovitis of peripheral joints manifested as swelling • JRA is not a single disease, but a category of diseases. • It is a diagnosis of exclusion.

  10. Criteria for Classification of JRA • Age of onset: < 16 yrs • Arthritis in > or = 1 joint • Duration of the Disease: > or = 6 weeks • Onset type is defined by type of articular involvement in the first 6 months after onset: • Poly arthritis: > or = 5 inflamed joints • Oligo arthritis: < or = 4 inflamed joints • Systemic Disease: arthritis with intermittent fever

  11. Classification of Chronic Arthritis in Children • ACR, ELAR & ILAR classification • Only ACR criteria have been statistically validated.

  12. Etiology • Unknown • Immunogenetic Susceptibility (Specific HLA subtypes) • External Triggers - Viruses( EBV, Parvo virus B19, Rubella) - Host hyperreactivity to specific self antigens(type 2 collagen) - Enhanced T-cell reactivity to bacterial /mycobacterial heat shock proteins

  13. Epidemiology • Incidence of JRA: 13.9/100,000 Sex: • Pauci and poly articular disease more in girls • Systemic onset –equal frequency in boys and girls Race: • Prevalence of JRA lower in Urban African – American compared to Caucasians • Oligo 40% newly diagnosed cases in Caucasians. • Blacks with JRA were older and less likely to test positive for ANA or to have uveitis, more likely to test positive for Ig M RF

  14. Pathogenesis • Synovitis: Villous hypertrophy & edema of subsynovial tissues. • Vascular endothelial hyperplasia • Infiltration of mononuclear and plasma cells. • Pannus formation with erosion of cartilage and bone. • Recriutment of T-cells specific to synovial non specific antigens., facilitated by specific HLA types.

  15. Clinical Features • Onset insidious or abrupt • Morning stiffness and gelling • Easy fatiguability • Joint pain and swelling, limited joint movt, mild /non erythematous.

  16. Oligoarthritis/Pauciarticular • Affects 4 or fewer joints • Typically larger joints (knees, ankles, wrists). • Starts with 1 joint • Monoarticular involvement of hip, upper extremity large joints never presenting sign in JRA. • If knee is affected-limping+, esp morning • Chronically- atrophy of extensor muscles of thigh, tight hamstrings & knee flexion contractures. • Associated with HLA-DR8

  17. Polyarthritis/Polyarticular • Minimum 5 joints should be effected. • Both large & small jts of upper and lower extremities • Resembles adult RA and HLA profile. • Associated with HLA –DR4 • Rheumatoid nodules in severe form • Micrognathia- chronic TM joint disease • C-Spine involvement- atlantoaxial subluxation

  18. Systemic Onset • Arthritis with visceral involvement • Characteristic intermittent spiking fevers to >/= 39c for >/= 2 weeks. • Febrile episode assoc with evenescent (< 1 hr) macular rash, linear or circular, salmon colored ,2-5 mm, over trunk & proximal extremities. • Koebner Phenomenon+ • Arthralgia, myalgia • Hepatosplenomegaly,Lymaphadenopathy • Serositis/pericardial effusion • Photophobia (uveitis), irregular iris due to synechiae

  19. Labs • CBC with diff:Lymphopenia,Thrombocytosis,microcytic anemia. Neutropenia is uncommon. • ESR: - Always elevated with systemic JRA. - Usually elevated with polyarticular but within reference range in pauciarticular. - When elevated, ESR helps to monitor success of medical treatment

  20. Labs contd • ANA: Positive in 40-85% with oligo/poly articular Unusual with systemic onset Titers do not correlate with disease severity. Associated with increased risk of uveitis • RF: Rare in systemic JRA. Marker of persistence of polyarticular JRA into adulthood, devpt of rheumatoid nodules and poor functioning.

  21. Total protein and albumin: levels are often decreased during active disease • ALT test: to exclude hepatitis (viral or autoimmune) prior to starting NSAIDS • U/A: to r/o infection (trigger of JRA or transient postinfectious arthritis) and nephritis (seen in pts with SLE)

  22. Imaging X-ray: • When 1 jt is affected , to r/o osteomyelitis or septic arthritis. • Soft tissue swelling, regional osteoporosis, osteopenia, sub chondral bone erosions, narrowing of cartilage spaces, fusion of nueral arches. MRI • synovial inflammation, early minimal changes seen Echo cardiography: Serositis DEXA: osteopenia

  23. X rays

  24. Management • Multidisciplinary Team for care of children • Core Team: • Parents and Child • Pediatric Rheumatologist • Pediatrician • Nurse • Social Worker • Physical Therapist • Occupational Therapist • Nutritionist • Ophthalmologist

  25. Main goal: maximize daily functioning , minimize drug toxicity • Key predictor of long term outcome is early diagnosis and referral to rheumatology team. • Diet: Include 3 servings of calcium rich foods • Activity: more active, better the prognosis

  26. NSAIDS • Used to treat all subtypes of JRA (40-60% children show improvement). • Mean duration for anti-inflammatory effect in JRA- 30 days • Most with pauci and few with poly respond to NSAID alone . • Rofecoxib , celecoxib (selective Cox-2 inhibitors) ~ similar to naproxen effectiveness • Adverse : nausea, decreased appetite, abd pain. Less gastritis.

  27. Methotrexate • Safest, most efficacious, least toxic of of 2nd line agents for JRA. • Used in 60% patients with poly JRA • Inhibits DHFR, purine synthesis. • Pts unresponsive to PO MTX benefit from SC or IM administration. • Well tolerated in children. • Pts who respond well have improved growth, functionality, radiographic improvement.

  28. Glucocorticoids • For overwhelmingly inflammatory or systemic illness. • Bridge therapy for those who did not respond to conventional therapy • Ocular control of uveitis (drops or injections) • Intra articular use :initial therapy in pts with only 1 or 2 joint involvement • Improvement in symptoms in 2-3 days, which last for at least 6 mo in 60% and 1yr in 45%

  29. Anti TNF alpha • Etanercept: Only one approved for children • Fusion protein with TNF receptor monomer fused to Fc portion of Ig G1. • Administered SC twice weekly used in active polyarticular JRA who fail MXT therapy. • JRA assoc-chronic uveitis that is inadequately responsive to steroid therapy. • Not to be used with h/o chronic infections • R/o TB before starting rx

  30. Sulphasalazine • Improved joint inflammation & labs compared to placebo. • GI irritation and rash. • Steven johnson in pts with active systemic JRA. • CI in porphyria and G6PD deficiency

  31. Systemic JRA

  32. Prognostic features • Child with oligo: esp. girls, onset< 6yrs age –chronic uveitis risk • Polyarticular: RF, rheumatoid nodules • Systemic onset: number of joints involved, duration of inflammation, severity of arthritis. • Limb length discrepancy, contractures, • Disability continues into adulthood in 20% • Chronic pain syndromes. • Psychological complications.

  33. LIVING WITH RHEUMATOID ARTHRITIS • http://www.youtube.com/watch?v=NqyB-cTxvs8

  34. A 16-month-old boy is brought to your clinic because his mother says he is "walking funny" today. She states that he has been walking for 4 months and is very active, but she is unaware of any trauma or falls. She denies fever or other symptoms. He appears well and has normal vital signs. Physical examination reveals mild tenderness to palpation over the medial aspect of the lower leg just above the ankle. There is no overlying bruising, erythema, or edema, and you can elicit full range of motion in the hips, knees, and ankles.Of the following, the MOST likely diagnosis isa. Aneurysmal Bone Cystb. Ankle Sprainc. Fractured. Osteomyelitise. Transient Synovitis

  35. An 11-year-old girl presents 2 weeks after an office visit for a presumed viral illness characterized by fever, malaise, and flushing of the cheeks. Today, her mother notes that she no longer has a fever, but she complains of pain in her knees and elbows. On physical examination, the left knee is slightly swollen and warm but not erythematous. The girl reports pain on movement of both elbows, but there are no physical findings on examination of the elbows or other joints. The remainder of the physical examination findings are normal, except for an oral temperature of 100.6°F (38.1°C). Results of laboratory studies include a white blood cell count of 8.9x103/mcL (8.9x109/L) with 40% polymorphonuclear leukocytes, 45% lymphocytes, and 15% monocytes; hemoglobin of 11.0 g/dL (110.0 g/L); platelet count of 472.0x103/mcL (472.0x109/L); and erythrocyte sedimentation rate of 20 mm/hr.

  36. Of the following, the MOST likely pathogen to cause this child's joint complaints is a. Borrelia burgdorferib. Coxsackievirusc. group A beta-hemolytic streptococcid. influenza A viruse. parvovirus B19

  37. AcknowledgementDr. Jillian ParekhDr. Karen Sawitz

  38. Questions???

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