Vasculitis syndromes

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Kawasaki Syndrome(Mucocutaneous lymph node syndrome)
Diagnostic criteria:
 Fever for > 5 days plus 4 of the following:
 Bilateral bulbar conjunctival injection
 Mucous membrane changes (injected pharynx, cracked lips, strawberry tongue)
 Extremity changes (edema, erythema, or desquamation of hands or feet)
 Polymorphous rash
 Cervical lymphadenopathy (at least one >1.5 cm)

Clinical manifestions
Arthritis and arthralgia
  Present in 7-25% of patients
  Involves large or small joints
Urethritis
  Causes sterile pyuria
CNS involvement
  Aseptic meningitis, facial nerve palsy, hearing loss
GI symptoms
  Abdominal pain, diarrhea, vomiting, hepatitis
Cardiac involvement
Coronary artery aneurysms, myocardial dysfunction

Henoch-Schonlein Purpura
Most common systemic vasculitis in children.
Immune mediated
 Deposition of IgA immune complexes.
Often a self-limited disease.
Occurs more often in fall, winter, and spring.
 Rare in the summer.
About 50% of cases are preceded by URI’s.
 Streptococcus is often implicated.
 Vaccines, insect bites, viruses have also been reported as triggers.

Classic tetrad
Palpable purpura (100%)
In absence of thrombocytopenia or coagulopathy
Arthritis or arthralgia (75%)
Abdominal pain (50%)
Renal disease (21-50%)

Behçet Disease
Very rare systemic vasculitis of unknown etiology.
Affects blood vessels of all sizes.
 Both arteries and veins
Clinical manifestations are similar in children and adults.
Characterized by recurrent, painful ulcers of the mouth and skin and uveitis.

Clinical Presentation
Apthous stomatitis
Genital ulcerations
Uveitis
GI symptoms (due to ulcers)
Arthritis
Lab findings
 Normal ANA and RF
 ESR/CRP may be elevated

Scleroderma
Juvenile Systemic Sclerosis
Characterized by symmetrical fibrous thickening of skin and various internal organs.
 Esophagus and GI tract, heart, lungs, kidneys
Clinical presentation
 Skin changes – edema then tightening, thinning, atrophy
 Raynaud’s – 70% at presentation
 Arthritis, arthralgia
 Muscle weakness/pain
 CREST syndrome
Pulmonary fibrosis and pulmonary hypertension are major causes of morbidity in children.

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