Cases reported "Polyarteritis Nodosa"

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1/14. 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.
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2/14. Post-infantile giant cell hepatitis associated with autoimmune hepatitis and polyarteritis nodosa.

    We report the case of a patient with corticosteroid-responsive giant cell hepatitis associated with typical manifestations and changes of polyarteritis nodosa from the kidney and central nervous system. Initially, the patient presented with transient right hemiparesis, followed by spontaneous remission without any abnormalities on computed tomography scan, magnetic resonance imaging and cerebrospinal fluid examination. A few months later he was admitted to our clinic because of icterus, peripheral oedema and abdominal distension. He was found to have clinical signs of active cirrhosis. Serological tests for hepatitis B, C and hiv virus were negative. serum ceruloplasmin. a1-AT and ferritin levels were within normal limits. Antinuclear antibodies were positive (1: 160). Liver biopsy showed micronodular cirrhosis with many eosinophils in the portal tracts and giant hepatocytes with multiple nucleoli in the lobule. Fulfilling the diagnostic criteria for autoimmune hepatitis, he was started on treatment with prednisolone and azathioprine, resulting in both clinical and biochemical responses. Four years later he presented with severe pain at the right costovertebral angle. ultrasonography revealed a haematoma at the right kidney, and selective angiography of the abdominal aorta, renal arteries and hepatic artery documented microaneurysms in both kidney and liver arteries. Because of severe haemorrhage, right nephrectomy was performed. histology of kidney specimen showed characteristic lesions of polyarteritis nodosa. Several months later, while on treatment with prednisolone and cyclophosphamide, the patient experienced a fatal episode of brain haemorrhage. An association between autoimmune hepatitis, polyarteritis nodosa and postinfantile giant cell hepatitis has not been reported previously.
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3/14. Hepatitis B associated fulminant polyarteritis nodosa: successful treatment with pulse cyclophosphamide, prednisolone and lamivudine following emergency surgery.

    For hepatitis b virus associated polyarteritis nodosa, alpha interferon and plasma exchanges have been proposed to be the first-line treatment. We report a case of hepatitis B surface antigen (HBsAg)-positive fulminant polyarteritis nodosa with predominant gastrointestinal involvement who showed good response to pulse cyclophosphamide, prednisolone, and lamivudine therapy. The patient, a 22-year-old man, presented with a short history of epigastric pain. Initial upper gastrointestinal endoscopy revealed gastritis and duodenal erosions. His pain did not respond to H2-receptor antagonists. He had slightly impaired liver function tests, and was HBsAg and hepatitis B e antigen (HBeAg) positive. Around 3 weeks after initial presentation, he developed massive gastrointestinal haemorrhage requiring resuscitation and emergency laparotomy. Microscopic examination of the resection specimens revealed necrotizing vasculitis of small and medium-sized arteries in the submucosa compatible with polyarteritis nodosa. The patient was treated with pulse cyclophosphamide and prednisolone, with lamivudine being added when he showed an acute rise in liver enzymes. He subsequently developed HBeAg seroconversion, and remained well 18 months after cessation of all immunosuppressives. We believe that the efficacy of pulse cyclophosphamide, prednisolone, and lamivudine in the treatment of hepatitis b virus associated polyarteritis nodosa, especially in comparison with interferon and plasma exchanges, deserves further evaluation.
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4/14. Dorsal thalamic haemorrhage complicating polyarteritis nodosa: a clinico-pathologic case report.

    Intracerebral haemorrhage is a rare complication of polyarteritis nodosa (PAN). We present the clinico-pathologic findings of a 62 year-old patient with systemic involvement by PAN, who developed temporary decrease of consciousness and a mild left hemisyndrome due to a small right dorsal thalamic haemorrhage. No clear cause for the haemorrhage was found on postmortem examination.
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5/14. Microscopic polyarteritis presenting with chest infections and acute appendicitis.

    We describe a 38-year-old male with antineutrophil cytoplasmic auto-antibody (ANCA) positive microscopic polyarteritis who presented with recurrent chest infections, lung haemorrhage, renal insufficiency and acute appendicitis. appendectomy was followed by resolution of abdominal symptoms and the surgical specimen revealed vasculitis of the serosal vessels. A renal biopsy was performed because of impaired renal function and this revealed focal necrotising glomerulonephritis with absence of immune deposits. Chest infections were treated with antibiotics resulting in partial clinical response, but pulmonary symptoms relapsed and a complete resolution was achieved only after plasma exchange and the administration of cyclophosphamide. Our observation emphasises the protean manifestations of microscopic polyarteritis and the relationship between ANCA and disease activity.
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6/14. Necrotising arteritis in amoebic colitis.

    Massive intestinal haemorrhage rarely occurs in amoebic colitis. We report a case of caecal amoebic ulcer in a 61 year old diabetic male who presented with massive lower intestinal haemorrhage requiring blood transfusion and emergency surgical intervention. Histologically, trophozoites of entamoeba histolytica were seen invading the wall of the submucosal arteries, causing necrotising arteritis. rupture of a necrosed artery probably caused massive haemorrhage.
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7/14. polyarteritis nodosa complicated by intrahepatic-perihepatic hemorrhage and acute appendicitis: successful treatment with cyclophosphamide and corticosteroids.

    polyarteritis nodosa (PAN) is a systemic vasculitic disease characterised by necrotising inflammation of small and medium-sized arteries. Abdominal complications due to PAN are rare, but the death rate for these cases is high. We describe a serious case of PAN that involved intrahepatic-perihepatic haemorrhage and acute appendicitis. Exploratory laparotomy and appendectomy were performed, and the patient was successfully treated with cyclophosphamide and corticosteroids.
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8/14. Microscopic polyarteritis: a forgotten aetiology of haemoptysis and rapidly progressive glomerulonephritis.

    A 76 year old white woman died from massive pulmonary haemorrhage nine days after she was admitted to Parkland Memorial Hospital for evaluation of haemoptysis and rapidly progressive glomerulonephritis. The differential diagnosis of haemoptysis with rapidly progressive glomerulonephritis is presented with particular emphasis on Wegener's granulomatosis and microscopic polyarteritis. Necropsy showed a small vessel necrotising vasculitis associated with a focal segmental necrotising glomerulonephritis consistent with microscopic polyarteritis.
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9/14. Spontaneous bilateral rupture of kidneys in a patient with polyarteritis nodosa. A case report.

    A previously well 42-year-old man presented with a three-hour history of pain in the right flank of acute onset. At laparotomy he was found to have a ruptured right kidney which was treated by nephrectomy. Eight days later he developed similar symptoms on the left: at operation 21 of blood were drained and nephrostomy and catheterisation carried out. He recovered after a complicated postoperative course, and histological examination of the removed kidney, and biopsy specimens, showed classic polyarteritis nodosa. This is a rare cause of spontaneous rupture of the kidney but must be considered whenever a patient presents with renal haemorrhage of unknown cause.
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10/14. Perirenal and renal subcapsular haematoma as presenting symptoms of polyarteritis nodosa.

    Two young men, were hospitalized due to acute massive blood loss with left abdominal flank pain. In both cases renal angiography showed signs of a haemorrhagic event in the left kidney, perirenal in one and subcapsular in the other. Microaneurysms indicated a diagnosis of polyarteritis nodosa, supported by renal biopsy in one case. Renal haemorrhage is an infrequent presentation of polyarteritis nodosa. Furthermore, one patient suffered also from familial mediterranean fever, and is the fifth reported case with this combination of diseases.
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