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1/5. Anti-MPO-ANCA-positive microscopic polyangiitis following subacute bacterial endocarditis.

    Although infectious agents such as staphylococcus aureus have been implicated in the pathogenesis of Wegener's granulomatosis, the role of bacterial infections in the pathogenesis of other types of small-vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA) is less clear. We describe a patient who developed a non-granulomatous necrotising small vessel vasculitis and perinuclear ANCA (p-ANCA) directed against myeloperoxidase (MPO) after recurrent episodes of bacterial endocarditis due to Staph. aureus. Although cytoplasmic ANCA (c-ANCA) directed against proteinase 3 have been reported in single patients with bacterial endocarditis, to our knowledge this patient is the first reported case of an anti-MPO-ANCA positive systemic vasculitis following bacterial endocarditis.
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2/5. Henoch-Schonlein purpura secondary to subacute bacterial endocarditis.

    Henoch-Schonlein purpura (HSP), a systemic, small-vessel vasculitic syndrome, is characterized by a nonthrombocytopenic purpuric rash, arthralgia, abdominal pain, and nephritis. These signs and symptoms may occur in any order, and not all are necessary for the diagnosis. Although most common in 4- to 7-year-olds, HSP is well documented in adults and is often preceded by a history of mucosal-based infections, especially of the upper respiratory tract. We report a case of HSP that occurred coincident with the onset of subacute bacterial endocarditis (SBE) in an otherwise healtny 41-year-old white woman. The patient presented with a purpuric rash and arthralgia and was found to have left-sided streptococcal SBE. She subsequently developed abdominal pain and immune complex glomerulonephritis. The bacterial endocarditis was treated with antibiotics and mitral valve replacement, followed by a spontaneous resolution of the associated signs and symptoms of HSP.
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3/5. cerebral infarction due to systemic necrotizing vasculitis in a patient with rheumatic heart disease, subacute bacterial endocarditis and status epilepticus.

    Systemic necrotizing vasculitis involving cerebral blood vessels is described in a 30-year-old man with rheumatic heart disease and subacute bacterial endocarditis. Fever, anaemia, splenomegaly and positive blood cultures for gram-negative bacteria were found on admission. The fever resolved with antibiotic therapy on the third hospital day but he then developed hemiplegia and multifocal seizures. The seizures progressed to uncontrollable status epilepticus accompanied by congestive heart failure and the patient died 20 d after admission. At autopsy, exudative and necrotizing vasculitis involving medium- to small-sized arteries was seen in the brain, the heart and the skeletal muscles. Rheumatic myocarditis and endocarditis and old rheumatic mitral valve deformities were also present. In addition, verrucous endocarditis in the mitral valve and Lohlein's focal glomerulonephritis were noted. We discuss the possible mechanism of the systemic necrotizing vasculitis in relation to rheumatic fever.
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4/5. A case report of the immunodysplasia syndrome and heavy chain disease associated with subacute bacterial endocarditis.

    A 36-year-old man was admitted to Saitama Medical School Hospital, because of a remittent fever which had continued for approximately 6 months, hepatosplenomegaly and lymphadenopathy. He had direct Coombs' test positive auto-immune hemolytic anemia associted with subacute bacterial endocarditis (SBE). The lymphnode demonstrated focal diffuse proliferation of immunoblasts and arborizing vessels with a few small germinal centers, which resembled histological features of the immunoblastic lymphadenopathy. The immunochemical analysis revealed the presence of free IgG Fc fragments in serum. From the above results the patient was diagnosed as immunodysplasia syndrome (IDS) and heavy chain disease (HCD) associated with SBE. It was suggested that the chronic antigenic stimulation due to SBE might have some role in the mechanism of the development of the IDS and HCD in our patient.
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5/5. Subacute bacterial endocarditis presenting with necrotic skin lesions.

    Classical lesions associated with subacute bacterial endocarditis include petechiae, splinter hemorrhages, Osler's modes and Janaway lesions. We describe a patient with subacute bacterial endocarditis, whose sole cutaneous findings were bullous necrotic lesions of the lower extremities. The superficial vessels were occluded by acidophilic material and coccal bacteria were identified in the vessels. Immunofluorescent studies revealed Igm and C3 in the vessel walls.
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