An unusual presentation of classic polyarteritis nodosa in a child

Abstract

Classic polyarteritis nodosa (c-PAN) is a rare disease in adults and extremely rare in children. We report a 3-year-old girl with c-PAN who presented with disturbances of consciousness and hypertension. Cranial tomography showed a subarachnoid hemorrhage. Subsequent magnetic resonance imaging and magnetic resonance angiography demonstrated subarachnoid hemorrhage and acute ischemic lesions. Renal angiography revealed bilateral multiple aneurysms. Due to her constitutional symptoms and hypertension and radiological findings she was diagnosed as having c-PAN. She was successfully treated with hydralazine followed by angiotensin-converting enzyme inhibitor, calcium channel blocker, intravenous pulse methylprednisolone, and subsequently oral prednisolone and oral cyclophosphamide. To our knowledge this is the youngest patient with c-PAN presenting with subarachnoid hemorrhage. Malign hypertension at this young age deserves a meticulous investigation of the vascular origin. Furthermore, treatment with pulse methyl prednisolone followed by oral prednisolone and oral cyclophosphamide is a successful modality of treatment in such a life-threatening presentation of c-PAN in childhood.

Polyarteritis Nodosa Associated with Essential Mixed Cryglobulinemia Revealed by Subarachnoid Hemorrhage

“…Only a few similar cases have been described (5). In the present patient, as in two previously reported cases, no micro-aneurysm was detected (6,7). The subarachnoid hemorrhage likely resulted from blood vessel weakening due to vasculitis, although a small, thrombosed, or spinal artery aneurysm could not be definitely excluded.…”

Mycophenolate mofetil treatment in two children with severe polyarteritis nodosa refractory to immunosuppressant drugs

“…The proper diagnosis was delayed as the initial neurological disease was not regarded as brain ischemia and cutaneous features in the boy were misdiagnosed as HSP. In several cases, the disease may be fatal as visceral involvement is aggressive [10][11][12][13][14]. Recently, tumor necrosis factor alpha blockade has been proposed in patients refractory to other therapies [15].…”

Hypertensive crisis, hepatitis B virus and polyarteritis nodosa in a child

“…The blood pressure was controlled by ACEinhibitors, emphasizing the importance of the renin-angiotensin system in the physiopathology of hypertension in PAN [9]. However the need for multiple medications is frequent in order to control PAN-related hypertension [10].…”