Updated: 9/22/2017

Juvenile Idiopathic Arthritis

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Snapshot
  • A 9-year-old girl presents to his pediatrican's office by his mother due to fever and joint pain. The mother reports that she has been having knee and wrist pain for approximately 7 weeks. Her pain is worse in the morning and is associated with swelling of the affected joint. She develops a fever daily that later subsides. On physical exam, there is tenderness to palpation of the affected joint. Laboratory studies are significant for an elevated ANA, ESR, and CRP. She is negative for rheumatoid factor.
Introduction
  • Clinical definition
    • a broad term that encompasses different types of pediatric chronic arthritis that is characterized by joint pain and inflammation
      • 1 or more joints are involved for at least 6 weeks in patients 16 years of age
      • subtypes
        • polyarticular juvenile idiopathic arthritis (JIA)
          • ≥ 5 joints involved
        • pauciarticular JIA
          • 1-4 joints involved
        • systemic JIA
          • visceral involvement
  • Epidemiology
    • incidence
      • the most common chronic rheumatologic disorder in children
    • demographics
      • more frequently with European ancestry
  • Pathogenesis
    • not well understood but may involve both genetic and environmental factors
      • the humoral and cell-mediated immune system are involved
        • T-cells release tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), and IL-1
        • the humoral immune system is involved by increasing antinuclear antibody (ANA) production, immunoglobulins, and circulating immune complexes
  • Prognosis
    • postitive
      • improved with advances in treatment
    • negative
      • early hip or wrist involvement
      • presence of rheumatoid factor
Presentation
  • Symptoms
    • fever
      • quotidian pattern
        • fevers occurring once or twice per day that eventually subside
    • morning stiffness and joint pain
      • joint stiffness and pain can also be noted with prolonged periods of inactivity (e.g., sitting)
    • visual changes
      • can be suggestive of a uveitis
  • Physical exam
    • joint involvement
      • swelling
      • limited range of motion
      • tenderness to palpation
    • uveitis
    • visceral involvement in cases of systemic JIA such as
      • evanescent and salmon-colored macular rash
      • hepatosplenomegaly
      • serositis
        • e.g., pleural and pericardial effusion
Studies
  • Labs
    • c-reactive protein (CRP) and erythrocyte sedimentation rate (ESR)
      • typically in systemic and polyarticular JIA
    • positive ANA
      • in most cases
  • Diagnostic criteria
    • a patient < 16 years of age with 1 or more joints involved for at least 6 weeks
Differential
  • Reactive arthritis
  • Rheumatoid arthritis
  • Systemic lupus erythematosus
  • Leukemia
  • Pediatric sarcoidosis
Treatment
  • Conservative
    • period ophthalmologic exams
      • indication
        • for monitoring of eye involvement (e.g., uveitis)
  • Medical
    • nonsteroidal antiinflammatory drugs (NSAIDs)
      • indication
        • for symptomatic relief
    • disease-modifying antirheumatic drugs (DMARDs)
      • indication
        • for controlling inflammatory process
      • medication
        • methotrexate
        • etanercept
        • adalimumab
    • intra-articular glucocorticoids
      • indication
        • effective in managing inflamed joint
Complications
  • Infection
  • Macrophage-activation syndrome
  • Pericarditis
  • Hemolytic anemia
  • Enarteritis

 

 

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Questions (1)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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