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1/77. Vasculitis confined to the peripheral nerve and skin: a variant of non-systemic vasculitic neuropathy.

    We describe a patient presenting with vasculitic neuropathy associated with cutaneous vasculitis in the absence of other clinical or laboratory evidence of the underlying systemic vasculitis, and showing a favourable prognosis. Although the early proposed criteria for the diagnosis of non-systemic vasculitic neuropathy (NSVN) exclude the involvement of extraneural tissues, the condition observed in our patient might represent a variant of NSVN, with the vasculitis confined to the small vessels in both the peripheral nerve and the skin.
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ranking = 1
keywords = vasculitis
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2/77. Successful treatment of hiv-related vasculitis with peripheral neuropathy with short-term steroids followed by the association of zidovudine and plasmapheresis.

    OBJECTIVE--treatment of hiv-related vasculitis, avoiding prolonged immunosuppressive therapy. DESIGN--prospective pilot study of hiv-related neurological vasculitis. patients--two hiv-infected patients with histologically proven vasculitis. INTERVENTION--short-term corticosteroid followed by zidovudine combined with plasmapheresis. MAIN OUTCOME MEASURES--clinical, biological, immunological and electromyographic evaluation. RESULTS--complete neurological recovery. CONCLUSION--excellent tolerance and efficacy of combined zidovudine and plasmapheresis therapy in peripheral neurological hiv-related vasculitis.
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ranking = 2.6666666666667
keywords = vasculitis
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3/77. Vasculitic polyradiculopathy in systemic lupus erythematosus.

    A 22 year old woman with recently diagnosed systemic lupus erythematosus presented with subacute progressive areflexic paraparesis, electrophysiologically identified as a pure axonal polyradiculopathy. sural nerve biopsy disclosed necrotising vasculitis. A striking radiological feature was marked enhancement of the cauda equina with gadolinium.
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ranking = 0.33333333333333
keywords = vasculitis
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4/77. Neurological manifestations of chronic hepatitis c.

    hepatitis c virus (HCV) infection is often associated with abnormal immunological responses. We describe four patients with vasculitic neurological signs and symptoms following HCV infection. A 56-year-old woman with HCV infection developed peripheral neuropathy characterized by asymmetric distal painful hypesthesia, dysesthesia and moderate motor weakness of the lower limbs. Serological examinations revealed cryoglobulinemia and low levels of complement c4. A biopsy of the sural nerve revealed vasculitic neuropathy. HCV infection associated immunomediated vasculitis was diagnosed. While steroid therapy was ineffective, treatment with interferon-alpha improved the neuropathy considerably without, however, eliminating HCV infection. A 62-year-old man with HCV infection developed peripheral sensory neuropathy. complement c3 was slightly diminished. Nerve biopsy revealed vasculitic neuropathy. A 71-year-old woman developed chronic symmetric sensomotor polyneuropathy. HCV hepatitis followed blood transfusions. cryoglobulins tested positive, consistent with type II cryoglobulinemia. Complements C3 and C4 were diminished. Inflammatory infiltrates in the sural nerve biopsy specimen led to the diagnosis of chronic vasculitic disorder. A 55-year-old woman with HCV infection developed vasculitis of the skin, connective tissue, visceral organs, and kidney, leading to hemodialysis. Neurologically she developed severe apathy and drowsiness, myoclonic jerks, exaggerated deep tendon reflexes, and positive pyramidal signs. magnetic resonance imaging of the brain showed diffuse increased signal abnormalities involving supra- and infratentorial white matter suggesting cerebral vasculitis. cryoglobulins were positive, complements C3 and C4 slightly diminished (54 mg/dl, 4.3 mg/dl). Supportive therapy resulted in neurological improvement. Treatment with interferon-alpha was discontinued because of agranulocytosis. In patients with peripheral neuropathy or signs of leucencephalopathy, a hepatitis c associated vasculitis should be considered in the differential diagnosis.
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ranking = 1.3333333333333
keywords = vasculitis
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5/77. Systemic lupus erythematosus demonstrating serum anti-GM1 antibody, with sudden onset of drop foot as the initial presentation.

    In systemic lupus erythematosus (SLE), peripheral neuropathies are relatively uncommon and rarely present as the initial symptom. We herein describe a 61-year-old woman who developed a sudden onset of drop foot, which was indistinguishable from guillain-barre syndrome based on the clinical symptoms alone. antibodies against ganglioside GM1 were detected in the serum, while no antibodies to campylobacter jejuni were observed. An electrophysiological study showed axonal impairment rather than demyelination. A pathological examination of a sural nerve biopsy specimen and further laboratory examinations suggested the observed peripheral neuropathies to have arisen due to lupus vasculitis. The serological activities of SLE responded well to treatment with corticosteroids, mizoribine and immunoadsorption therapies, however, the drop foot symptoms did not change remarkably.
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ranking = 0.33333333333333
keywords = vasculitis
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6/77. Vasculitic neuropathy in association with chronic graft-versus-host disease.

    A 43-year-old woman is reported who developed acute and later chronic graft-versus-host disease following an unrelated donor bone marrow transplantation for chronic myeloid leukaemia. Four years later, she developed a sensory multiple mononeuropathy with biopsy features of chronic vasculitis. This is the first report of vasculitic neuropathy in association with graft-versus-host disease.
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ranking = 0.33333333333333
keywords = vasculitis
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7/77. Fibrosing alveolitis predating microscopic polyangiitis.

    A 65 year old male was diagnosed with "cryptogenic fibrosing alveolitis (CFA)" and treated successfully with prednisone. In the year following prednisone-tapering he presented with livedo reticularis, segmental pauci-immune glomerulonephritis and necrotizing vasculitis of the peripheral nerves, increased pulmonary fibrosis, and the presence of p-ANCA antibodies. Aggressive immunosuppressive treatment of this microscopic polyangiitis (MPA) was successful and also resulted in stabilization of the pulmonary fibrosis. This case illustrates that MPA may present itself monosymptomatic as CFA.
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ranking = 11.724320272375
keywords = angiitis, vasculitis
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8/77. Endoneurial vasculitis and tubuloreticular inclusions in peripheral nerve biopsy.

    We describe 3 patients in whom nerve biopsy revealed endothelial tubuloreticular inclusions in association with peripheral nerve endoneurial vasculitis. Two of the patients had systemic lupus erythematosus (SLE), while the third was hiv-positive. review of our biopsy material featuring the much more common finding of epineurial vasculitis failed to disclose any instances in which endothelial tubuloreticular inclusions (TRIs) were present. We conclude that TRIs and endoneurial vasculitis are closely associated. Moreover, if detected on a nerve biopsy specimen, TRIs are very suggestive of SLE or hiv infection. Finally, literature evidence is cited to suggest that an "acid-labile" alpha-interferon may be pathogenically related to the vasculitic process in these patients, perhaps through a process mediated by tumor necrosis factor.
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ranking = 2.3333333333333
keywords = vasculitis
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9/77. naproxen-induced leukocytoclastic vasculitis.

    Cutaneous reactions to non-steroidal anti-inflammatory drugs (NSAIDs) are rare in spite of their wide use. Only a few cases of hypersensitivity angiitis related to naproxen have been described. We report the case of a 62-year-old woman in whom leukocytoclastic skin vasculitis, peripheral neuropathy and nephritis developed after a short naproxen treatment, and gradually regressed after discontinuation of the drug and under glucocorticoid therapy. In the light of the relevant literature, the clinical and laboratory features of this reversible condition are described.
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ranking = 3.944864054475
keywords = angiitis, vasculitis
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10/77. Sensory ataxic dominant neuropathy associated with polyarteritis nodosa.

    A 68-year-old man with sensory ataxic dominant neuropathy associated with polyarteritis nodosa (PAN) had deep sensory disturbance with unsteady gait and absence of the achilles tendon reflex. Examination revealed weight loss, elevated CRP level, negative antineutrophil cytoplasm antibodies, decreased M-wave amplitude in the peroneal motor nerve and absence of action potentials in the sural sensory nerve. sural nerve biopsy revealed a marked loss of myelinated fibers, myelin ovoid formation and necrotizing angiitis of large epineurial arterioles. Renal biopsy revealed global and/or segmental necrotizing angiitis in glomeruli, but not in the arcuate artery. These pathological findings were distinct from those of PAN, particularly microscopic polyangiitis (MPA). Treatment with a steroid improved the deep sensory disturbance, unsteady gait, and CRP level. This case is similar to ataxic neuropathy which can arise from various disorders. This is the first report of a case of sensory ataxic dominant neuropathy associated with MPA in PAN.
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ranking = 6.8345921634249
keywords = angiitis
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