1/33. Waldenstrom's macroglobulinaemia presenting as reticulate purpura and bullae in a patient with hepatitis b virus infection.Cutaneous manifestations of Waldenstrom's macroglobulinaemia (WM) include purpura, ulcers, urticaria, leukocytoclastic vasculitis, and immunobullous dermatoses. No association has been reported previously of WM and hepatitis b virus (HBV) infection. A 40-year-old female HBV carrier was admitted to hospital because of generalized oedema, oliguria, haematuria, hypertension, fever and blood-tinged sputum. Cutaneous manifestations included generalized petechiae, palpable purpura mainly on the legs, multiple necrotic ulcerations and gangrenous changes on the toes, and necrotic, giant confluent reticulate purpura on the trunk surmounted by several tense bullae. Laboratory investigations revealed monoclonal gammopathy of IgM kappa type (6.7 g/L), membranoproliferative glomerulonephritis associated with HBV infection, Bence Jones proteinuria, and an increased number of abnormal plasmacytoid cells in the bone marrow. Pathologic examination demonstrated immune complex-mediated vasculitis with deposits of IgM in the walls of dermal vessels and secondary subepidermal bulla formation. HBV infection may have caused WM or modified the clinical course in this fatal case.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
2/33. Treatment of refractory antibody mediated autoimmune disorders with an anti-CD20 monoclonal antibody (rituximab).BACKGROUND: Rituximab, a chimeric monoclonal anti-CD20 antibody, has recently been used for the treatment of refractory antibody mediated autoimmune diseases such as immune mediated thrombocytopenia and haemolytic anaemia. patients: Because of its novel mechanism of action, rituximab was used to treat three patients with refractory systemic antibody mediated autoimmune disorders. The first patient, a 71 year old woman with idiopathic type II mixed essential cryoglobulinaemia, had both dermatological and neurological manifestations with marked renal disease attributed to her cryoglobulinaemia. Patient 2, a 73 year old woman with Goodpasture's syndrome, was refractory to conventional treatment (cyclophosphamide, prednisone, plasmapheresis). She had persistent haemoptysis and haematuria and positive antiglomerular basement membrane antibodies. The third patient, a 75 year old man with primary biliary cirrhosis, myelodysplasia, and systemic immune complex vasculitis, had progressive renal insufficiency, a macular erythematous rash, and severe thrombocytopenia. RESULTS: Treatment with rituximab resolved all clinical and laboratory manifestations in the three patients. CONCLUSIONS: Rituximab may be an important therapeutic agent for the treatment of patients refractory or intolerant to corticosteroid or cytotoxic treatment, or both.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
3/33. Necrotizing vasculitis: a circulating immune complex producing inflammatory skin lesions.A patient with acute necrotizing vasculitis is described in whom tests for circulating immune complexes were negative. The patient's serum injected intradermally produced lesions which closely resembled those occurring spontaneously. When the serum was fractionated, the skin-reactive fraction was found to be associated with IgG, but was of a higher molecular weight than normal IgG. Immunofluorescent studies showed that lesions induced by this active fraction contained IgG and complement in the epidermal basement membrane zone and within the small dermal vessels. We conclude that an immune complex of relatively low molecular weight is present in the active fractions, and capable of initiating the lesions of acute necrotizing vasculitis.- - - - - - - - - - ranking = 3keywords = vasculitis (Clic here for more details about this article) |
4/33. Immunologic considerations in renovascular hypertension.For decases certain diseases, such as glomerulonephritis, polyarteritis nodosa, scleroderma and serum sickness, have been linked with autoimmune pathogenesis. During recent years a host of additional diseases traditionally thought to have some genetic predisposition but with obscure etiology have been suspected of being autoimmune in nature. Rheumatoid arthritis, diabetes, myasthenia gravis and thyroiditis are diseases of widely divergent organ systems, yet may well have common pathways of pathology via immune complexing mechanisms. Herein we present evidence supporting the concept that renal artery stenosis (occurring primarily in association with the middle aortic syndrome or after renal transplantation) is of immune etiology. Although the specific antigenic agent is still to be defined there is growing acceptance of the theory that medium and large vessels are subject to autoimmune vasculitis in many aspects similar to the autoimmune affections of small vessels. Several cases are presented. Some of these suggest an immune reaction by the natural history but without evidence of immunochemical reactants in the involved vessels, presumably because active disease was arrested at the time to study. In other cases immunofluorescent preparations demonstrate reactants in the walls of the vessels to document the hypothesis more convincingly.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
5/33. Systemic hypersensitivity vasculitis associated with bronchiectasis.Systemic hypersensitivity vasculitis developed in a 53-year-old man during acute exacerbation of bronchiectasis infected with pseudomonas aeruginosa. High grade fever, mononeuropathy multiplex, cutaneous vasculitis, and biopsy specimen-proved mesangioproliferative glomerulonephritis with crescent formation and leukocytoclastic vasculitis associated with circulating immune complex occurred. Corticosteroid and cyclophosphamide therapy was effective for vasculitis and bronchiectasis.- - - - - - - - - - ranking = 4keywords = vasculitis (Clic here for more details about this article) |
6/33. skin lesions in viral hepatitis: histologic and immunofluorescent findings.A variety of skin rashes are knowned to occur as a part of the serum sickness-like prodrome of acute viral hepatitis which is thought to be due to immune complex deposition. We report the histologic and immunofluorescent findings in the skin and the seroloigc abnormalities in a patient with both erythematous maculopapular and purpuric rashes. We found circulating hepatitis B surface antigen (HBsAg), hypocomplementemia and cultaneous vasculitis associated with deposition of immunoglobulin and complement in the skin. We could not demonstrate intradermal deposition of HBsAg, but the findings are consistent with the immune complex hypothesis.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
7/33. Vascular disease in infective endocarditis. Report of immune-mediated events in skin and brain.A patient with subacute bacterial endocarditis, who had a prosthetic mitral valve, exhibited hypocomplementemia, mixed (IgG, IgM) cryoglobulinemia, and widespread dermal vasculitis with IgM deposited at the dermoepidermal junction. Postmortem findings included immune-complex glomerulonephritis and deposits of IgG in the choroid plexus. These findings are consistent with a generalized vasculitis, which may be mediated by circulating immune complexes; basement membrane localization of immune complexes to the skin and choroid plexus appears not to be specific for systemic lupus erythematosus, as has been previously thought, but may represent the general phenomenon of immune-mediated pathogenesis.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
8/33. Immune complex vasculitis as a cause of ascites and pleural effusions in systemic lupus erythematosus.A patient is described with systemic lupus erythematosus and large painless ascites and pleural effusions. Pleural and peritoneal fluid complement levels were depressed, and dna binding was elevated in the presence of normal serum values. Immunoglobulin and complement deposits were demonstrated in vessels of the pleura, peritoneum, and skin, along with histologic evidence of vasculitis. The relation of the ascites and pleural effusions to the presence of widespread vasculitis and local immune complex formation is discussed. These complications responded poorly to corticosteroid therapy but slowly resolved following the addition of an immunosuppressive agent.- - - - - - - - - - ranking = 3keywords = vasculitis (Clic here for more details about this article) |
9/33. Acute massive pulmonary hemorrhage in systemic lupus erythematosus.Acute massive pulmonary hemorrhage is described as part of the clinical picture of SLE. Seven patients had sudden onset of high fever, dyspnea, tachycardia, and cough with blood-tinged sputum that within hours progressed to massive hemoptysis and death. There were no vasculitis or other inflammatory lung changes found at autopsy. Only one of the seven reported cases survived when treated with 2 g of intravenous hydrocortisone daily. The mechanism of this pulmonary bleeding is unknown but seems to be an immune complex mediated phenomenon.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
10/33. Immunoelectronmicroscopic examination of early lesions in histamine induced immune complex vasculitis in man.In 4 cases of allergic vasculitis circulating immune complexes (IC) were demonstrated. Spontaneous and histamine induced vascular changes were studied by immunofluorescence microscopy. The early events in IC vasculitis were investigated at the ultrastructural level by immunoelectronmicroscopy using the peroxidase-antiperoxidase multistep technique. Our findings support the concept that human IC vasculitis is triggered by the deposition of circulating IC in the walls of postcapillary venules between endothelial cells, pericytes and the layers of the basal lamina. Tissue destruction is only secondary due to local complement activation and the release of lysosomal enzymes from chemotactically attracted leukocytes.- - - - - - - - - - ranking = 3.5keywords = vasculitis (Clic here for more details about this article) |
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