Classification of Childhood Vasculitis
1. Large vessel vasculitis
• Takayasu arteritis
2. Medium vessel vasculitis
• Childhood polyarteritis nodosa
• Cutaneous polyarteritis nodosa
• Kawasaki disease
3. Small vessel vasculitis
A) Granulomatous
- Granulomatous polyangiitis (Wegener)
- Eosinophilic granulomatous polyangiitis (Churg-Strauss)
B) Non-granulomatous
- Microscopic polyangiitis
- Henoch-Schonlein purpura
- Isolated cutaneous leukocytoclastic vasculitis
- Hypocomplementemic urticarial vasculitis
4. Other vasculitides
• Behcet 's disease
• Infection (including hepatitis B-associated PAN), malignancy, drug-induced vasculitides
• Vasculitis associated with connective tissue disease
• Cogan syndrome
• Unclassified
Henoch-Schonlein Purpurasi (HSP)
• It is the most common vasculitis in childhood.
• It is more common in males and is most common between the ages of 3 and 10, usually after a viral URI in winter and spring.
• Non-thrombocytopenic purpura, arthritis/arthralgia, abdominal pain, gastrointestinal bleeding and glomerulonephritis are characteristic findings.
• It has repetition feature.
Pathology
• Neutrophil and monocyte infiltration in the vessel wall, IgA deposition in immunofluorescence, swelling in endothelial cells and leukocytoclasis (breakdown of neutrophil cell nuclei) around small vessels (arteriole, venule, small artery and vein) are seen (leukocytoclastic vasculitis).
Clinic:
Skin rashes (100%):
• It is the most common clinical finding in HSP.
• Petechiae, ecchymosis or palpable purpura (especially in the gluteal region and extensor surfaces)
• Subcutaneous edema (25-35%) (back of hands and feet, periorbital region, scrotum)
Arthralgia, arthritis (50-70%)
• Oligoarticular, mostly involving the lower extremities.
• It usually heals within two weeks without leaving any deformity.
Gastrointestinal symptoms (50-85%)
• It is the most common internal organ involvement in HSP.
• Abdominal pain, vomiting, diarrhea
• Hematemesis (less than 10%)
• Occult blood positivity in stool (50%)
• Invagination, mesenteric ischemia, perforation
Renal involvement (30%)
• It is the most serious organ involvement and the most common cause of mortality in HSP.
• Similar to IgA nephropathy.
• The most common finding is microscopic hematuria.
• Acute or chronic kidney failure (2-5%)
• Nephritic and/or nephrotic syndrome
• Since kidney involvement can develop after 1-6 months, the patient should be followed up with a urinalysis once a week during the active disease period and monthly thereafter.
Other findings:
• CNS vasculitis findings (convulsion, paresis, bleeding and coma)
• Orchitis, testicular torsion, carditis, pulmonary hypertension and inflammatory eye disease
Laboratory Findings
• The acute phase response is often high (CRP, ESR).
• IgA is high in the blood in 40-50% of the cases.
• ANA, ANCA and RF are negative.
• Barium graphy is used in the diagnosis and treatment of intussusception.
Treatment:
• Non-steroidal anti-inflammatory drugs are used for arthralgia and myalgia.
• Steroid therapy is used for severe kidney and gastrointestinal disease. Although this treatment relieves abdominal and joint pain, it does not affect the prognosis of the disease and does not prevent kidney failure.
• 15-60% of children with HSP experience a relapse within 4-6 months.