Paediatric class presentation

1. CHILDHOOD POLYARTERITIS NODOSA
(CPAN)
УЗЕЛКОВЫЙ ПОЛИАРТЕРИИТ
• rare and often fatal disease that affects
small and medium arteries
• Previously known as infantile polyarteritis
nodosa (IPAN)

2. Definition andClassification
acute, subacute or chronic disease, which is
based on loss of peripheral and visceral
arteries, mainly small and medium caliber
Classification:
Polyarteritis nodosa (classic).
Polyarteritis lung with lesions (Churg-Strauss
syndrome).
Juvenile polyarteritis.

4. EPIDEMIOLOGY
 Asian descent appear to have a
disproportionately high incidence of
vasculitis.
 It occurs in children of all ages, juvenile
polyarteritis - more common in girls.

5. ClinicalPicture
 All variants: Fever, pain in the joints, muscles,
hypersensitivity, weight loss
 Classic: Renal Hypertension, multiple mononeuritis,
cerebral vascular crises, coronaritis, bowel necrosis
 Juvenile polyarteristis: Nodules, livedo, skin and mucous
membrane necrosis, distal gangrene
 Churg- Strauss Syndrome (синдромЧерджа-Стросс):
Bronchial asthma or recurrent history of obstructive
syndrome, hypereosinophilic syndrome in combination
with any of the symptoms stated above in other
polyarteritis

7. Livedo

8. Distal
Gangrene

9. LaboratoryDiagnosis
 CBC: Leucocytosis, neutrophilia,
hyperesinophilia (>20-30%), increase ESR
 Hypergammaglobulinemia
 HBV markers present (symptoms absent)

10. Imaging
 Bilateral axillary aneurysm
 Ateriogram: multiple aneurysm
 Histopathology+ biopsy- necrotising
aneurysm
 Ateriography: Renal aneurysm
 Other Dx: ECHO, MRI, CT, EMG, EEG

11. Histopathology
 necrotizing arteritis with a mixed
cellular infiltrate with few eosinophils
Thrombosed cystic artery

12. Complication
 Stroke
 cerebral edema
 myocardial infarction
 renal failure
 Peritonitis
 respiratory failure
 infection foci of necrosis

13. Treatment
 Surgery- develop complication
 Medication:
a) Prednisolone (1-2mg/kg) for 4-6 weeks
b) Cyclophosphamide (immunosuppressant): 2-3 mg / kg
daily or 10-15 mg / kg 1 time per month for at least one
year.
c) Severe hypertension: cyclophosphamide used alone or
its combination with a short course of prednisolone at
low doses (less than 0.5 mg / kg)
d) After glucocorticoid- hormone therapy for at least 2
yrs
e) NSAID- Phenylbutazone
f) Improve circulation: antiplatelet, anticoagulation,
angioprotector