Cases reported "polyarteritis nodosa"

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1/606. Acute sensorimotor polyneuropathy with tonic pupils and an abduction deficit: an unusual presentation of polyarteritis nodosa.

    A tonic pupil may occur in isolation or as part of a systemic disorder. We report a patient who developed tonic pupils and an abduction deficit in the setting of polyarteritis nodosa. The combination of a tonic pupil and an abduction deficit should suggest the possibility of a vasculopathic disorder, because the ciliary ganglion and lateral rectus muscle are both supplied by the lateral muscular artery. Widespread small artery and arteriolar narrowing and occlusion are the hallmarks of polyarteritis nodosa. Unusual ischemic syndromes may occur, such as this rare combination of neuro-ophthalmic signs, by involvement of both the nutrient artery and its collaterals. We are unaware of other reports of neuropathic tonic pupils in association with polyarteritis nodosa. ( info)

2/606. Pauci-immune rapidly progressive glomerulonephritis after nephrectomy in a renal donor.

    Idiopathic rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic syndrome in which glomerular damage is accompanied by a rapid and progressive decline in renal function, usually resulting in irreversible renal failure in weeks or months. We report the occurrence of pauci-immune RPGN, more specifically microscopic polyarteritis nodosa (PAN), in a 60-year-old woman 15 months after donor nephrectomy, and 3 months after documentation of intact, residual renal function. The transplanted kidney continues to function well in the recipient, 6 years posttransplantation, and 4.5 years beyond destruction of the donor's contralateral kidney by RPGN. The donor underwent cadaveric renal transplantation after 2 years on dialysis, and at the 3-year mark has intact renal function. These intriguing observations strongly argue that host environmental factors, rather than intrarenal factors, play a major causative role in the pathogenesis of RPGN. ( info)

3/606. Fatal polyarteritis nodosa with massive mesenteric necrosis in a child.

    polyarteritis nodosa (PAN) is a rare vasculitic syndrome in childhood. There are few reported cases of ischaemic necrosis of the intestine and even fewer survivors in adults. We report the case of a 10-year-old boy with PAN and an acute abdomen that required operative intervention. Evidence was found of mesenteric arteritis with large ischaemic segments resulting in infarction and perforation. ( info)

4/606. Cutaneous polyarteritis nodosa: a case report and literature review.

    Cutaneous polyarteritis nodosa (CPN) is an uncommon form of vasculitis. It exists as a separate entity, though bearing similar name with polyarteritis nodosa (PAN) which is an aggressive systemic vasculitis with multi-organ involvement. CPN runs a chronic but benign course. Its aetiology is unknown and it usually presents with painful nodules on the legs with mild constitutional symptoms, and extracutaneous features of arthralgias, arthritis, neuropathy and myopathy. No mortality has been reported thus far. It is therefore important to distinguish CPN apart from PAN. Symptomatic treatment with judicial use of systemic steroids and anti-inflammatory agents will suffice in most cases. ( info)

5/606. Acute pancreatitis as a complication of polyarteritis nodosa.

    CONCLUSIONS: polyarteritis nodosa (PAN) must be considered as one of the rare causes of "idiopathic" acute necrotizing pancreatitis. BACKGROUND: PAN is characterized by panmural inflammation of arterioles causing arteriolar ectasia, aneurysm formation, and thrombosis, resulting in organ ischemia. methods: We report a case of necrotizing pancreatitis associated with segmental necrosis of the liver and spleen due to polyarteritis nodosa. RESULTS: Five previously reported cases of documented acute pancreatitis secondary to PAN have been identified from the English literature. The mechanism through which pancreatic ischemia results in acute pancreatitis is unknown. Although limited pancreatic infarction is common in PAN, necrotizing pancreatitis is rare, and the poor overall prognosis of PAN is owing largely to other organ complications. ( info)

6/606. Isolated periarteritis nodosa of the spermatic cord presenting as a scrotal mass: report of a case.

    We report a 57-year-old man who visited a local hospital because of a fist-sized swelling and pain in his right scrotum. Surgically resected tissue revealed necrotizing arteritis in a small spermatic artery. As systemic symptoms for angiitis were not present, this is thought to be the second case of isolated angiitis occurring in the spermatic cord. ( info)

7/606. Primary polyarteritis nodosa presenting as acute symmetric quadriplegia.

    We report a case of peripheral neuropathy presenting as acute symmetric areflexic quadriplegia in the setting of a well-defined clinical, histopathologic, and angiographic diagnosis of classic polyarteritis nodosa. While it is usually easy to recognize the typical clinical presentation of necrotizing angiopathy-induced peripheral neuropathy as a mononeuritis multiplex or a distal polyneuropathy in association with a collagen vascular disease, clinicians must be equally sensitive to a number of more challenging possibilities. Acute quadriplegia similar to that seen in guillain-barre syndrome can be secondary to primary classic polyarteritis nodosa and the former may be the chief or even the sole manifestation of the latter. ( info)

8/606. Renal hemorrhage in polyarteritis nodosa: diagnosis and management.

    We report a patient with biopsy-proven polyarteritis nodosa with massive hemorrhage from presumed interlobar artery rupture. The use of interventional radiology techniques such as embolization are discussed in the acute management of such patients. Embolization by interventional radiology should be considered a useful alternative to surgery in these patients. The utility of angiography as a diagnostic and therapeutic tool is demonstrated in this case. ( info)

9/606. Multiple intracranial aneurysms in polyarteritis nodosa: MRI and angiography.

    In polyarteritis nodosa involvement of the central nervous system is infrequent; small cerebral infarcts are the most common neuroradiological finding. We report a 10-year-old boy with polyarteritis nodosa and intracranial haemorrhage. MRI showed an intracerebral haemorrhage. angiography revealed two peripheral aneurysms in the posterior cerebral circulation, previously reported on only two occasions, and multiple microaneurysms in the hepatic, renal, mesenteric and even the lumbar arteries. ( info)

10/606. Familial occurrence of systemic vasculitis and rapidly progressive glomerulonephritis.

    Two familial clusters of systemic vasculitis are described. In one family, microscopic polyangiitis and rapidly progressive glomerulonephritis occurred in HLA-identical siblings; in the second family, 3 second- and fourth-degree related members were affected by Wegener's granulomatosis. Published clusters of systemic vasculitides and Goodpasture's syndrome are reviewed, and, together with the observed families, the evidence for genetic susceptibility and a causative role of environmental factors for these diseases with special emphasis on the HLA system is discussed. ( info)
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