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Pediatric Arthritis Syndromes

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  • he common underlying manifestation of this group of illnesses is the presence of chronic synovitis, or inflammation of the joint synovium. The synovium becomes thickened and hypervascular with infiltration by lymphocytes, which also can be found in the synovial fluid along with inflammatory cytokines. The inflammation leads to the production and release of tissue proteases and collagenases, which if left untreated can lead to tissue destruction, particularly of the articular cartilage and eventually the underlying bony structures.
  • Cervical lymphadenopathy is found in 70% of children and should be greater than 1.5 cm in diameter for the purposes of diagnosis. , which can vary in appearance, occurs in 80% of children with KD and may be particularly accentuated in the inguinal area and on the chest.
  • which usually appear in the subacute and convalescent phases, and pose the highest risk of sudden death. Risk factors for development of coronary artery aneurysms include prolonged fever, prolonged elevation of inflammatory parameters such as the ESR, age younger than 1 year, and male gender. Body_ID: P088005 page 430 page 431 Body_ID: P0431
  • Transcript

    • 1. Pediatric Arthritis Syndromes
      Dr Abdullah Aburiziza
      MD, FAAP, FAAAAI
      Assistant professor
      Pediatric Allergy/Clinical Immunology
      Um AlQura University
      Makkah
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 2. Outlines
      Approach to Arthritis
      Juvenile Rheumatoid (Idiopathic) Arthritis
      Kawasaki Disease
      Henoch-Schönlein Purpura
      Juvenile Dermatomyositis
      SLE
      Growing pain
      Hypermobility Syndrome
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 3. Approach to Arthritis
      Ask about the following:
      -Presenting Complain ( reason for admission)
      -HPI
      Joints Symptoms, problem joints , morning stiffness, pain, tenderness, swelling, limitation of movement, splints, orthoses, trauma…
      General health, fever, pallor, weight loss
      Skin rash, color, distribution, size site itchiness fading, bleeding, secretion..ect
      GI, IBD
    • 4. Eyes, redness, secretions, disturbed vision, cataract
      CNS, seizure, personality changes, drowsiness, headache..
      Drugs side effects, steroid, poor height gain, weight gain …
      Level of function, walking, eating, writing, dressing, wheelchair, walker…
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 5. PMH
      Initial diagnosis, when, presentation, investigation, trigger, hospitalizations, sequence of joints involvement, complications( disease or medications)
      Management: physiotherapy, sports, drugs, side effect, diet
      Social History: Child, Parents, Sibling, Social support, Financial
      Family History, Arthritis, IBD, psoriasis, uveitis, thyroid disease…
    • 6. At the completion of your history, you should have a clear impression of the patient’s:
      Current function
      Current and past treatment modalities
      Cooping with the disease
    • 7. Examination
      Vitals
      Anthropometric measurement
      Rash
      Joint Examination, # of joints
      • Inspection, scars, fullness, dilated vessels, deformity, muscle wasting,
      • 8. Palpation. temperature, effusion, tenderness
      • 9. Range of movement, exact with measurement of limitation
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 10. Investigations
      CBC
      XRAY
      ESR, CRP.
      ASO titer
      ANA
      Anti DNA
      RF
      Echo
      HLA B27
      Biopsy
      …………
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 11. Juvenile Rheumatoid (Idiopathic) Arthritis
      Autoimmune disease of unknown etiology.
      Inflammation of the joint synovium.
      The disease has two peaks, one at ages of 1 to 3 years and another peak at ages 8 to 12 years.
      Girls are affected more commonly than boys
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 12. The diagnosis of JRA is established by the presence of arthritis, the duration of the disease for at least 6 weeks, and the exclusion of other possible diagnoses.
      Children must be younger than 16 years old at time of onset of disease; the diagnosis of JRA does not change when the child becomes an adult.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 13. CLINICAL PRESENTATION
      Table 89-1. Features of Juvenile Rheumatoid Arthritis Subgroups
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 14. Pauciarticular Juvenile Rheumatoid Arthritis
      Fewer than five joints within the first 6 months from diagnosis.
      This is the most common form of JRA, 50% of cases.
      Peak at ages 1 to 3 years and another broader peak at ages 8 to 12 years.
      Medium-sized to large joints, (knee)
      NO evidence of systemic inflammation (fever, weight loss, or failure to thrive) or any laboratory evidence of systemic inflammation (elevated WBC count or ESR).
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 15. Polyarticular Juvenile Rheumatoid Arthritis
      Five or more joints within the first 6 months of diagnosis
      40% of cases.
      can affect any joint, but typically involves the small joints of the hands, feet, ankles, wrists, and knees.
      Can present with evidence of systemic inflammation, including malaise, low-grade fever, growth retardation, anemia of chronic disease, and elevated markers of inflammation.
      Polyarticular JRA can present at any age, although there is a peak in early childhood( RF –ve).
      There is a second peak in adolescence( RF +ve)
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 16. Systemic-Onset Juvenile Rheumatoid Arthritis
      Typical recurring spiking fever, usually once or twice a day, which can occur for several weeks to months.
      Rash, typically morbilliform and salmon colored, evanescent and occur only at times of high fever.
      Serositis, such as pleuritis and pericarditis, occurs in half of children.
      Hepatosplenomegaly occurring in 70% of children.
      Significant constitutional symptoms, including malaise and failure to thrive.
      Elevated ESR, CRP, WBC count, and platelet counts and anemia.
      Arthritis of JRA follows the systemic inflammation by 6 weeks to 6 months.
      The arthritis is typically polyarticular ,extensive and resistant to treatment, with risk for long-term disability.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 17. Connective Tissue Diseases
      • Juvenile rheumatoid arthritis
      • 18. Systemic lupus erythematosus
      • 19. Juvenile dermatomyositis
      • 20. Scleroderma with arthritis
      Seronegative Spondyloarthropathies
      • Seronegative arthropathy syndrome
      • 21. Juvenile ankylosing spondylitis
      • 22. Psoriatic arthritis
      • 23. Reiter syndrome
      • 24. Inflammatory bowel disease
      Infectious Arthritis
      • Bacterial/ Viral / Fungal arthritis
      • 25. Lyme disease
      Reactive Arthritis
      • Poststreptococcal arthritis
      • 26. Rheumatic fever
      • 27. Toxic synovitis
      • 28. Henoch-Schönlein purpura
      • 29. Orthopedic Disorders
      • 30. Traumatic arthritis
      • 31. Legg-Calvé-Perthes disease
      • 32. Slipped capital femoral epiphysis
      • 33. Osteochondritis dissecans
      • 34. Chondromalacia patellae
      • 35. Musculoskeletal Pain Syndromes
      • 36. Growing pains
      • 37. Hypermobility syndromes
      • 38. Myofascial pain syndromes/fibromyalgia
      • 39. Reflex sympathetic dystrophy
      • 40. Hematologic/Oncologic Disorders
      • 41. Leukemia, Lymphoma, Sickle cell disease, Thalassemia, Malignant and benign tumors of bone, cartilage, or synovium
      • 42. Metastatic bone disease
      • 43. Miscellaneous
      • 44. Rickets/metabolic bone disease
      • 45. Lysosomal storage diseases
      • 46. Heritable disorders of collagen
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 47. Investigations
      CBC-Diff
      ESR
      CRP
      ASO
      ANA, Anti-DNA,RF.
      X-Ray
      Arthrocenthesis
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 48. TREATMENT
      NSAIDs
      Systemic Steroid
      Intraarticular Steroid injection
      Second-line medications, such as hydroxychloroquine , sulfasalazine, Methotrexate.
      Physiotherapy and occupational therapy
      Ophthalmology referral.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 49. Complications
      Loss of function of an involved joint secondary to joint contractures or bony fusion or loss of joint space.
      Uveitis, which if left untreated can lead to serious visual loss or blindness.
      Systemic-onset disease, a positive RF, poor response to therapy, and the presence of erosions on x-ray all predict a poorer prognosis.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 50. Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 51. Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 52. Kawasaki Disease
      KD is a vasculitis of unknown etiology that is characterized by multisystem involvement and inflammation of small to medium-sized arteries with resulting aneurysm formation.
      KD most commonly occurs in children younger than age 5, with a peak between ages 2 and 3 years, and is rare in children older than age 7.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 53. CLINICAL MANIFESTATIONS
      Acute Phase :
      • Lasts 1 to 2 weeks.
      • 54. Sudden onset of a high, hectic fever (≥40°C) without an apparent source.
      • 55. Conjunctival erythema, bilateral and nonsuppurative.
      • 56. Mucosal changes, dry, cracked lips and a strawberry tongue
      • 57. Cervical lymphadenopathy.
      • 58. Swelling of the hands and feet.
      • 59. A Polymorphous rash, primarily truncal rash.
      • 60. Extreme irritability is prominent, especially in infants.
      • 61. Abdominal pain
      • 62. Arthritis, particularly of medium-sized to large joints.
      • 63. Carditis in the acute phase may be manifested by tachycardia, shortness of breath, or overt congestive heart failure.
      • 64. Giant coronary artery aneurysms.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 65. CLINICAL MANIFESTATIONS
      Subacute Phase
      • Lasts until the fourth week
      • 66. Gradual resolution of fever and other symptoms.
      • 67. Desquamation of the skin, particularly of the fingers and toes.
      • 68. The platelet count, increases to a significant degree (often >1 million/mm3).
      Convalescent Phase
      • Begins with the disappearance of clinical symptoms and continues until the ESR returns to normal
      • 69. 6 to 8 weeks after the onset of illness.
      • 70. Beau lines of the fingernails.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 71. Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 72. Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 73. Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 74. Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 75. Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 76. Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 77. Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 78. Investigations
      CBC-Diff
      ESR
      CRP
      Echocardiogram
      LP
      Coronary angiography
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 79. Criteria for Diagnosis of Kawasaki Disease
      Fever of ≥5 days' duration associated with at least 4* of the following 5 changes:
      Bilateral nonsuppurative conjunctivitis
       One of more changes of the mucous membranes of the upper respiratory tract, including pharyngeal injection, dry fissured lips, injected lips, and "strawberry" tongue
      One or more changes of the extremities, including peripheral erythema, peripheral edema, periungual desquamation, and generalized desquamation
      Polymorphous rash, primarily truncal
      Cervical lymphadenopathy >1.5 cm in diameter
      Disease cannot be explained by some other known disease process
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 80. TREATMENT
      IV immunoglobulin (IVIG) (2 g/kg over 12 hours)
      Aspirin, anti-inflammatory doses (80 to 100 mg/kg/day divided every 6 hours)
      Antithrombotic doses (3 to 5 mg/kg/day as a single dose).
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 81. Henoch-Schönlein Purpura
      HSP is a vasculitis of unknown etiology characterized by inflammation of small blood vessels with associated leukocytic infiltration of tissue, hemorrhage, and ischemia.
      The immune complexes associated with HSP are predominantly composed of IgA.
      It occurs in children age 3 to 15 years, although it has been described in adults.
      HSP is more common in boys than girls .
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 82. CLINICAL MANIFESTATIONS
      Palpable purpura, caused by small vessel inflammation in the skin leading to extravasation of blood into the surrounding tissues.
      Classically found in dependent areas, below the waist on the buttocks and lower extremities.
      Begin as small macules or urticarial lesions, but rapidly progresses to purpura with areas of ecchymosis.
      Edema of the calves and dorsum of the feet and the scalp and scrotum or labia.
      HSP occasionally is associated with encephalopathy, pancreatitis, and orchitis.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 83. Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 84. CLINICAL MANIFESTATIONS
      Arthritis occurs in 80% of patients in any joint, > the lower extremities, most commonly the ankles and knees.
      GI involvement typically presents as mild to moderate crampy abdominal pain.
      Less commonly, significant abdominal distention, bloody diarrhea, intussusception, or abdominal perforation occurs and requires emergent intervention.
      1/3 renal involvement, which can occur as an acute or chronic event.
      Acute glomerulonephritis manifested by hematuria, hypertension, or acute renal failure can occur, although in most cases renal involvement is mild.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 85. Investigations
      CBC-Diff (Platelet Count)
      ESR
      CRP
      Urinalysis for evidence of hematuria, and serum BUN and creatinine
      Stool analysis.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 86. Criteria for Diagnosis of Henoch-Schönlein Purpura
      Palpable purpura: Raised, palpable hemorrhagic skin lesions in the absence of thrombocytopenia.
      Bowel angina: Diffuse abdominal pain or the diagnosis of bowel ischemia.
      Diagnostic biopsy: Histologic changes showing granulocytes in the walls of arterioles or venules.
      Pediatric age group: Age ≤20 years at onset of symptoms.
      *The diagnosis of Henoch-Schönlein purpura is based on the presence of two of four criteria.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 87. TREATMENT
      supportive.
      NSAIDs
      Systemic corticosteroids reserved for children with gastrointestinal disease or renal involvement.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 88. Complications
      Recurrence
      Renal
      GI
      Scrotal
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 89. Juvenile Dermatomyositis
      Vasculitis affecting small vessels of skeletal muscle.
      Present in a slow, progressive fashion with insidious onset of fatigue, malaise, and progressive muscle weakness.
      Affects the proximal muscles, particularly the hip and shoulder girdles, and the abdominal and neck muscles.
      Children have difficulty climbing steps, getting out of chairs, and getting off the floor.
      The patient may have a positive Gower sign.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 90. (needing to lean on legs while getting up from the ground)
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 91. Heliotrope discoloration
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 92. Gottron papules,
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 93. Investigations
      ↑ serum muscle enzymes, aspartate aminotransferase, alanine aminotransferase, creatine phosphokinase, aldolase, and lactate dehydrogenase.
      Electromyography
      Muscle biopsy
      MRI is a noninvasive means of showing muscle inflammation.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 94. Criteria for Diagnosis of Juvenile Dermatomyositis
      Rash typical of dermatomyositis
      Symmetric proximal muscle weakness
      Elevated muscle enzymes (SGOT, SGPT, LDH, CPK, and aldolase)
      EMG abnormalities typical of dermatomyositis (fasciculations, needle insertion irritability, and high-frequency discharges)
      Positive muscle biopsy specimen with chronic inflammation
      *To make definitive diagnosis of dermatomyositis, 4 of 5 criteria are required.
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 95. Treatment
      Systemic corticosteroids
      In severe or refractory cases, it may be necessary to use cyclosporine or cyclophosphamide.
      Calcinosis.
      Complications
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 96. Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 97. Case#1
      2 years old boy, presented to the clinic with 3 days history of limping.
      Approach
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 98. History
      HPI, onset, duration, severity, aggravating factors, reliving factors.
      Associated symptoms, fever, rash, swelling, weight loss, GI symptoms, bleeding, visual symptoms, morning stiffness.
      Trauma
      PMH, chronic or recent illness, anemia, blood transfusion.
      Birth Hx
      Social, abuse, recent travel, raw milk
      FH
      Developmental Hx
      Medications
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology
    • 99. Physical Examination
      Investigations
      Dr Abdullah Aburiziza,MD, FAAP, FAAAAI, Assistant Professor, Allergy/Immunology