1/39. Acquired bernard-soulier syndrome: a case with necrotizing vasculitis and thrombosis.We describe a patient with positive antinuclear antibodies, polyclonal gammopathy and high level of circulating immunocomplexes, resulting in vascular purpura. In addition, the patient had a slightly prolonged bleeding time and an isolated defect of ristocetin-induced platelet aggregation (RIPA) in platelet-rich plasma (PRP). The patient's plasma also inhibited RIPA in normal PRP and in normal platelet suspension. The activity and multimeric structure of plasmatic von willebrand factor showed no alteration. We could demonstrate an autoantibody against platelet membrane glycoprotein (GP) Ib, using an ELISA-type assay. These data suggest an acquired bernard-soulier syndrome. We suggest that the patient had an immunocomplex-mediated leukocytoclastic vasculitis accompanied by production of antinuclear autoantibodies as well as the presence of an autoantibody against GPIb. The titer of the anti-GPIb antibody, however, was too low to induce significant platelet-type bleeding tendency, only laboratory alterations were found. Moreover, in a later stage of her disease, she developed a severe necrotizing vasculitis which was followed by a deep venous thrombosis.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/39. polyarteritis nodosa involving the hepatobiliary system in an eight-year-old girl with a previous diagnosis of familial mediterranean fever.polyarteritis nodosa (PAN) is a vasculitis of small- and medium-sized muscular arteries with deposition of immune complex in the vessel wall. Although gastrointestinal involvement is common, the symptomatic involvement of the hepatobiliary system is rare. An eight-year old female patient with a previous diagnosis of familial mediterranean fever (FMF) was hospitalized for right upper quadrant pain and fever. The thickened gall bladder wall by ultrasonography, called for exploration. Histopathological evaluations of the liver biopsy and gall bladder revealed PAN. Corticosteroid therapy was initiated and the patient recovered fully. This case represents one of the rarest forms of PAN in childhood.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
3/39. 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.- - - - - - - - - - ranking = 0.11830710291535keywords = neoplasm (Clic here for more details about this article) |
4/39. Successful combination therapy of polyarteritis nodosa associated with a pre-core promoter mutant hepatitis b virus infection.BACKGROUND/AIMS: To describe the clinical and virological evolution of a polyarteritis nodosa (PAN) case associated with a hepatitis b virus (HBV) pre-core promoter mutant infection that was successfully treated with plasma exchanges, corticosteroids, and interferon alpha (IFN-alpha). methods: Viral markers were used, including HBV DNA quantified by the branched DNA assay and detected by PCR, the HBV genome sequence, pre-S1Ag and anti-HBC IgM which were studied throughout the treatment period and the entire follow-up in the serum, while the presence of virus in extrahepatic sites was detected by immuno-staining. RESULTS: The patient was infected with a typical pre-core promoter mutant harboring four point mutations. Pre-S1Ag was cleared rapidly from serum, most likely via the formation of immune complexes since HBV DNA declined more progressively. Viral infection was then cleared after a second episode of hepatocyte lysis. This was accompanied by a recovery from all clinical manifestations. CONCLUSIONS: The favorable treatment outcome observed in this first case of pre-core promoter HBV mutant associated PAN underlines that combination therapy based on IFN-alpha can clear pre-core promoter HBV infection and cure PAN. It also provides new insight in the pathogenesis of HBV associated PAN.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
5/39. life-threatening complications of hepatitis b virus-related polyarteritis nodosa developing despite interferon-alpha2b therapy: successful treatment with a combination of interferon, lamivudine, plasma exchanges and steroids.polyarteritis nodosa (PAN) is a rare vasculitis associated with hepatitis b virus (HBV) infection in a significant proportion of cases. When used to treat HBV-related PAN, immunosuppressive agents may enhance viral replication and relaspes are frequent. In recent years the use of antiviral drugs has been proposed. We report the case of a patient with HBV-related PAN who, despite 6 weeks of interferon-alpha2b (IFN-alpha2b) monotherapy, developed life-threatening complications with bowel perforation. He was thereafter successfully treated with a combination of IFN-alpha2b, lamivudine, plasma exchanges and short-term steroids. In contrast to IFN-alpha2b, lamivudine is effective in rapidly suppressing viral replication. This may be valuable in the treatment of HBV-related PAN by contributing to a faster diminution of circulating immune complex levels. This case report highlights the importance of aggressive combined therapy in patients with HBV-related PAN.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
6/39. polyarteritis nodosa and mixed cryoglobulinaemia related to hepatitis B and C virus coinfection.OBJECTIVE: To determine the responsibility of hepatitis b virus (HBV) and hepatitis c virus (HCV) and therapeutic implications in a patient who developed systemic vasculitis. CASE REPORT: The case of a 38 year old woman who had a past history of addiction to intravenous drugs and developed systemic vasculitis after infection by HBV and HCV is described. The clinical and laboratory findings substantiated not only the diagnosis of polyarteritis nodosa (PAN) but also that of mixed cryoglobulinaemia with a monoclonal IgMkappa component. CONCLUSION: Because cryoglobulins are rarely found in HBV related PAN but often associated with HCV infection, and in light of the histological findings, cryoglobulinaemia was interpreted as being secondary to HCV infection. This example of a highly complex situation emphasises the need to gather all relevant clinical, biological, histological, and complementary data so that the best treatment for overlapping of distinct vasculitides can be selected.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
7/39. 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.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
8/39. Isolated testicular polyarteritis nodosa mimicking testicular neoplasm-long-term follow-up.polyarteritis nodosa is a systemic vasculitis characterized by segmental necrotizing lesions of medium and small-size arteries. Rarely, the inflammatory process is isolated and involves a single organ without systemic manifestations. We describe a patient with isolated polyarteritis nodosa of the testis who presented with a testicular mass mimicking primary testicular tumor. The postoperative laboratory evaluation was negative. Long-term follow-up, without systemic treatment, showed no evidence of recurrence in the remainder of the testis or development of systemic vasculitis.- - - - - - - - - - ranking = 0.47322841166139keywords = neoplasm (Clic here for more details about this article) |
9/39. 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.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
10/39. polyarteritis nodosa associated with hepatitis b virus infection. The role of antiviral treatment and mutations in the hepatitis b virus genome.polyarteritis nodosa (PAN) is a systemic inflammatory disease causing vasculitis of medium sized and small arteries. Circulating immune complexes containing viral proteins have been implicated in the pathogenesis of hepatitis b virus (HBV) related PAN and several immunosuppressive and antiviral regimens have been used with varying success. In our hospital seven HBV positive patients with a confirmed diagnosis of PAN could be identified between 1984 and 2001. Most patients had an acute HBV infection and all patients were treated with prednisone. A combination of prednisone and antiviral therapy with alpha-interferon (IFN) was used only in the last four patients. HBV DNA was isolated from serum samples obtained before treatment from the four IFN treated patients and amplified by using the polymerase chain reaction technique. None of the patients without, but two of four with antiviral therapy exhibited HBsAg seroconversion. In three out of four patients HBV DNA decreased rapidly after starting IFN therapy. Clinical remission of PAN was observed in three of the four treated patients, but in none of the three patients who were not receiving antiviral medication. Analysis of the HBV genome revealed no mutations that could be associated with PAN. In one patient a stop codon in the pre-core region and a double mutation A1762T-G1764A were found during antiviral therapy. We did not find HBV heterogeneity predisposing to the development of PAN. In our group of patients it appeared that clinical remission of PAN was primarily related to spontaneous or therapy induced loss of HBV dna replication. The combined administration of a priming steroid course and IFN appears to be an improvement over prednisone monotherapy and should be considered for every patient with HBV related PAN. The efficacy of new generation nucleoside analogues should be further elucidated in future studies.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
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