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Pediatric Arthritis Syndromes
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
9. Range of movement, exact with measurement of limitationDr 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
46. Heritable disorders of collagenDr 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
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.
54. Sudden onset of a high, hectic fever (≥40°C) without an apparent source.
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
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
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
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
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
