Cases reported "Polyarteritis Nodosa"

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1/34. polyarteritis nodosa mimicking prostatic cancer.

    We describe a 72-year-old man with prostate enlargement, prostate-specific antigen level of 35 ng/dl, mild polyarthritis, and constitutional symptoms. Prostatic ultrasonography suggested neoplasm; however, transrectal biopsy revealed findings consistent with polyarteritis nodosa (PAN). The patient went on to develop leg paresthesia and dysesthesia, increased serum creatinine, and systemic hypertension. steroids and intravenous cyclophosphamide were administered, followed by improvement. Our case emphasizes the protean onset of PAN, and provides a new differential diagnosis of prostatic diseases related to elevated prostate-specific antigen.
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2/34. Successful treatment of hepatitis b virus associated polyarteritis nodosa with a combination of prednisolone, alpha-interferon and lamivudine.

    Therapy of hepatitis b virus (HBV)-associated poly-arteritis nodosa is still evolving. Here we report a successful treatment with a short-term steroid administration in combination with a-interferon and lamivudine and a complete sequence analysis of the HBV genome. A 58-year-old man presented with the symptoms of mononeuritis multiplex associated in time with the onset of highly replicative hepatitis B. polyarteritis nodosa was confirmed by biopsy. During an initial course with alpha-interferon and prednisolone no clinical improvement or hepatitis b virus seroconversion was observed. After addition of lamivudine to the protocol with fast tapering of prednisolone, HBV dna fell to undetectable levels within 1 month and liver transaminases normalized. After 6 months of treatment HBeAg seroconversion took place, followed by HBsAg seroconversion 2 months later. Clinical symptoms of polyarteritis improved. No relapse of polyarteritis or hepatitis B was seen during the follow up of 9 months. Complete sequence analysis of the HBV genome revealed 6 nucleotide mutations but none in a relevant antigenic epitope. The present protocol of short-term prednisolone administration combined with alpha-interferon and lamivudine was effective for the treatment of HBV-related polyarteritis nodosa and may be a promising new therapeutic approach.
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3/34. Hepatitis B-related polyarteritis nodosa complicated by pulmonary hemorrhage.

    A 34-year-old man presented with fever, weight loss, paresthesia, abdominal pain, and hypertension. He had hepatitis B antigenemia, with negative antineutrophil cytoplasmic antibody, antinuclear antibody, and antiglomerular basement membrane serology results. Renal arteriography showed multiple intrarenal microaneurysms. In spite of therapy with antiviral agents (lamivudine, famciclovir), prednisone, cyclophosphamide, and plasmapheresis, renal function deteriorated. He later developed rapidly progressive dyspnea and hemoptysis. Diffuse alveolar hemorrhage was confirmed by bronchoscopy. He died of respiratory failure. The cause of pulmonary hemorrhage in this case of polyarteritis nodosa is unclear, but may include underlying capillaritis, cocaine-induced pulmonary hemorrhage, or recurrent attacks of malignant hypertension.
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4/34. HBV-related cutaneous periarteritis nodosa in a patient 16 years after renal transplantation: efficacy of lamivudine.

    Cutaneous periarteritis nodosa (PAN) is a clinical feature characterized by chronic, benign course; its pathogenesis is unknown. In patients submitted to renal transplantation cutaneous PAN is a rare complication. We report a case of cutaneous PAN associated with the reappearance of hepatitis B antigen 16 years after kidney transplantation. A 44-year-old man underwent successful renal transplantation in June 1980. In December 1996 he presented multiple painful erythematous subcutaneous nodules on both legs. skin lesion biopsy showed the presence of cutaneous PAN. Six months later laboratory data demonstrated the presence of HbsAg. HBeAg, HBcAb and detectable HBV-dna serum by polymerase-chain-reaction (PCR) assay. Anti-HBs and anti-HBe proved negative. In July 1998 the laboratory tests showed an important increase of HBV-dna (5.1 billion by Branched dna), and so lamivudine (100 mg/day) was introduced. HBV-dna became undetectable by PCR after 3 months of therapy. Seven months later a new skin biopsy was performed. The typical signs of PAN were no longer evident. As HBV infecion was demonstrated six months after the clinical appearance of the PAN, in a patient who was believed to be immune to the virus, it is possible that, in the early stages, the hepatitis B antigen title was methodologically indeterminable, but sufficient to form circulating immune complexes responsible for vasculitis primer. lamivudine therapy resulted efficacious in favouring the regression of cutaneous PAN, but its long-term efficacy requires further evaluation as regards potential selection of drug resistant hepatitis b virus (HBV) mutants during treatment.
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5/34. Limited polyarteritis nodosa of the male and female reproductive systems: diagnostic and therapeutic approach.

    BACKGROUND: polyarteritis nodosa (PAN) is a multisystem necrotising small and medium sized vasculitis that when left untreated carries a grave prognosis, with a five year survival of 10-15%. Prolonged immunosuppressive treatment with cyclophosphamide and steroids leads to high remission rates while carrying the risk of life threatening complications. The diagnostic and therapeutic approach for patients with isolated genital tract PAN is not well defined. OBJECTIVE: To present the management and follow up of two patients with limited PAN localised to the male and female reproductive system. case reports: A 26 year old man presented with an "acute scrotum". He was afebrile and had no other sign or symptom. Laboratory tests, including complete blood count, erythrocyte sedimentation rate, liver and renal function tests, C reactive protein, antinuclear antibody, cryoglobulins, complement levels, antineutrophil cytoplasmic antibodies, and hepatitis B surface antigen, were all normal. His left testis was excised. Histopathology disclosed PAN of medium sized arteries with testicular infarction but no signs of torsion or infection. The other patient was a 51 year old woman who had had a total hysterectomy for a uterine myoma; incidentally PAN of the uterus and fallopian tubes was discovered. Neither patient received any immunosuppressive treatment after surgical removal of the affected organ. On prolonged follow up (clinical and laboratory evaluation) both patients are healthy with no sign of local recurrence or systemic PAN.
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6/34. 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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7/34. polyarteritis nodosa with aortic dissection: necrotizing vasculitis of the vasa vasorum.

    A 59-year-old woman was admitted to our hospital with acute onset of chest pain. She had experienced high fever, weight loss, polyarthralgia, myalgia, polyneuropathy and hallucinations for 3 years before admission. The diagnosis of polyarteritis nodosa with hepatitis B surface antigenemia was made by muscle biopsy and serological examinations, and administration of prednisolone induced remission of all the manifestations. After developing the acute attack of severe chest pain, she died suddenly. At autopsy, a DeBakey type 1 aortic dissection was found and the immediate cause of death was found to be cardiac tamponade secondary to rupture of the aortic dissection. Microscopically, necrotizing inflammatory lesions were present in the medium sized vascular arteries throughout her body. Furthermore, necrotizing vasculitis was also found in the vasa vasorum of the adventitia and media of the thoracic aorta. The dissecting lesion was seen in the outer layer of the media. Our results suggest that spontaneous dissection of the aorta may be attributed to necrotizing vasculitis of the vasa vasorum.
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8/34. Treatment of hepatitis b virus-related polyarteritis nodosa: two case reports and a review of the literature.

    A substantial number of cases of polyarteritis nodosa (PAN) are related to hepatitis b virus (HBV) infection. Different treatment strategies are reported in the literature. The aim of this study was to review 15 years of literature (1988-2002) to determine the optimal treatment for HBV-related PAN at present, and to discuss the indications and mechanism of action of corticosteroids in HBV-related PAN, as many physicians are reluctant to use these in the presence of HBV infection. The first patient stopped his initial treatment, relapsed and died of cerebral infarction. The second case illustrates the favorable outcome with the standard treatment: corticosteroids, lamivudine and plasma exchanges. If adequate follow-up is possible, antiviral agents as well as corticosteroids are indicated in HBV-related PAN. Corticosteroids diminish inflammation and corticosteroid withdrawal induces an alanine aminotransferase (ALT) rebound in patients with a low baseline ALT level. antiviral agents are essential, as they reduce the production of HBV antigens and help to achieve hepatitis B early antigen (HBeAg) seroconversion. Plasma exchanges reduce the level of circulating immune complexes and are included in the treatment protocol of all recent studies. However, their effect has not been evaluated in controlled trials. We concluded that if adequate follow-up is possible, antiviral agents as well as corticosteroids are indicated in HBV-related PAN.
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9/34. vasculitis with hepatitis B antigenemia: long-term observation in nine patients.

    The development of generalized necrotizing vasculitis in association with hepatitis B antigenemia is the first example in man of a chronic rheumatic disease presumably caused by a viral infection. This report reviews the experience in nine biopsy-proven cases of hepatitis B-associated necrotizing vasculitis followed for up to six years. The natural history of the disease is emphasized and the manifestations of patients with vasculitis who carry hepatitis B antigen are compared with those of vasculitis patients who are antigen negative.
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10/34. Immune complexes in hepatitis B antigen-associated periarteritis nodosa. Detection by antibody-dependent cell-mediated cytotoxicity and the Raji cell assay.

    A subpopulation of peripheral blood lymphocytes with the ability to lyse target cells coated with specific antibody (antibody-dependent cell-mediated cytotoxicity, ADCC) was serially studied in a patient with hepatitis B antigen-associated periarteritis nodosa. The effector lymphocytes possess FC and complement receptors but do not require complement for functional activity. We found that the patient's ADCC was decreased during periods of disease activity and was almost normal during remission. The patient's serum could block ADCC in normal lymphocytes, and the blocking ability correlated with the concentration of immune complexes as determined by the Raji cell assay (a radioimmunoassay using complement receptors on human cultured lymphoblastoid cells). The concentration of immune complexes and the ADCC blocking ability of ther serum both correlated with disease activity. serum from five other patients with active vasculitis was found to contain significant amounts of immune complexes and was able to block normal ADCC. It appears that the ADCC assay can be used to detect the presence of circulating immune complexes and to monitor disease activity in periarteritis nodosa.
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