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Paediatrics: Polyarteritis nodosa

Rare, medium-vessel necrotizing vasculitis with aneurysm formation.

Polyarteritis nodosa

 

Rare, medium-vessel     necrotizing   vasculitis    with   aneurysm    formation.

Male:female ratio 2:1.

 

Clinical

 

Antecedent systemic illness with unexplained fevers, abdominal pains, and arthralgia of up to 1yr.

Testicular pain in males (often mistaken for torsion).

Vasculitis or purpuric skin rash.

Arthritis (30%) large joints: exquisite bony tenderness from peripheral vasculitis and periosteal new bone formation.

Renal: hypertension; haematuria, proteinuria; renal failure; intrarenal aneurysms.

GI involvement (50%): abdominal pain; pancreatitis; bowel infarction.

CNS: mononeuritis multiplex; peripheral neuropathy; fits; hemiplegia.

 

Investigations

 

FBC; LFTs may be elevated.

 

Renal function urinalysis for active sediment.

 

MRI; MR angiography to reveal multiple aneurysms.

 

Histology: panarteritis with fibrinoid necrosis, thrombosis, infarction, weakening of artery walls, and aneurysms. Segmental lesions at bifurcations of small- and medium-vessel walls.

 

Course and treatment 

Without treatment may be fatal. Often under recognized and undertreated in children. If treated promptly, with pulsed cyclophosphamide and high dose steroids, prognosis improved.

 

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Paediatrics: Bones and joints : Paediatrics: Polyarteritis nodosa |

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Paediatrics: Bones and joints


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