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1/152. Recurrent miscarriage, congenital heart block and systemic lupus erythematosus.

    We report the obstetric history of a woman, who between 15 spontaneous abortions, gave birth to a child with congenital heart block. She later developed systemic lupus erythematosus, had antibodies to SS-A/Ro and SS-B/La but was repeatedly negative for antiphospholipid antibodies.
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keywords = block
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2/152. 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 = 0.0087038582267958
keywords = nerve
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3/152. November 1998--70 year old woman with SLE, paraproteinemia and polyneuropathy.

    A 70 year old woman developed a sensory-motor polyneuropathy 12 years after almost continuous treatment with hydrochloroquine for SLE. sural nerve biopsy disclosed abundant lipid inclusions in schwann cells and vessels against a background of axonal degeneration. After cessation of therapy the patient improved moderately. This case underscores the usefulness of nerve biopsy in the study of management of patients with peripheral neuropathy associated with systemic illnesses.
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ranking = 0.017407716453592
keywords = nerve
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4/152. Optic neuropathy in systemic lupus erythematosus and antiphospholipid syndrome (APS): clinical features, pathogenesis, review of the literature and proposed ophthalmological criteria for APS diagnosis.

    Optic neuropathy is a well-known ocular manifestation occurring in patients with systemic lupus erythematosus (SLE), and it remains one of the major causes of blindness in these patients. We report data from six SLE patients with optic neuropathy, one of whom was considered to have antiphospholipid syndrome (APS). This patient had monolateral optic neuropathy, whereas the other five SLE patients had bilateral optic nerve disease. We believe that the monolateral occurrence of optic neuropathy in our patient can be considered as a 'focal' neurological disease due to a thrombotic event involving the ciliary vasculature. Conversely, bilateral optic nerve damage in SLE could be considered to be a 'general' neurological disease due to different immunological mechanisms, such as vasculitis. Additionally, the literature on SLE patients affected by optic neuropathy is reviewed to evaluate the major clinical features, particularly neurological features. In reviewing the literature, it appears that bilateral optic neuropathy in SLE occurs more frequently than monolateral optic neuropathy, and the main neurological manifestation seen in these patients is transverse myelitis, particularly in SLE patients with bilateral optic nerve disease. Finally, we propose a clinico-ophthalmological spectrum of APS and outline the ocular clinical manifestations that can be considered as diagnostic for the syndrome.
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ranking = 0.026111574680387
keywords = nerve
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5/152. 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 = 0.0087038582267958
keywords = nerve
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6/152. 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 = 0.060927007587571
keywords = nerve
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7/152. Extensive lymphadenopathy as the first clinical manifestation in systemic lupus erythematosus.

    Lymphadenopathy (LAP) is a frequent sign of systemic lupus erythematosus (SLE). The lymph nodes are usually soft, nontender, varying in size from 0.5 cm to several centimeters. However, generalized LAP in SLE is extremely rare, while to our knowledge, the presentation of extensive LAP with blocks of retrosternal, mesenteric, and retroperitoneal nodes has not been previously reported as the first clinical manifestation of the disease. In this report we describe a patient with extensive LAP and active SLE and we point out that generalized LAP should be included among the clinical findings indicating disease activity in SLE patients.
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8/152. hydrops fetalis with complete heart block secondary to congenital lupus: report of one case.

    Neonatal lupus erythematosus is an uncommon syndrome characterized by a congenital heart block and/or cutaneous lesion. We report a male newborn with neonatal lupus erythematosus presenting with complete heart block, cutaneous lesions, and hydrops. Transplacental passage of anti-SSA/Ro and anti-SSB/La antibodies were positive. Under the regimens of steroid for maternal systemic lupus erythematosus, perinatal anticongestive agents, and postnatal ventricular pacing with inotropic therapy, the infant died at the age of two days. The prognosis of hydrops fetalis secondary to neonatal lupus with complete heart block is usually fatal.
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9/152. A case report on the perinatal management of a 30-week preterm baby with congenital complete heart block.

    INTRODUCTION: Congenital complete heart block is an uncommon condition in the newborn, but is known to occur with maternal systemic lupus erythematosus. CLINICAL PICTURE: This paper presents one such baby with complete heart block who was born premature (after a gestation of 30 weeks) and weighing 759 g. TREATMENT: Continuous isoprnaline infusion was initially used to support the baby while her other neonatal problems were treated. A Medtronics VV1 pacemaker was subsequently inserted to maintain a heart rate that would be more physiologically acceptable for the patient. OUTCOME: This baby is currently thriving well, having been followed up for one year. CONCLUSIONS: The management issues, encompassing maternal and neonatal problems, and a review of current literature on this condition are discussed.
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10/152. Complete heart block in an adult with systemic lupus erythematosus and recent onset of hydroxychloroquine therapy.

    Complete heart block (CHB) is a rare complication of systemic lupus erythematosus (SLE), mainly seen during an acute flare-up of the disease or after high-dose long-term treatment with antimalarial drugs, although anti-Ro and anti-RNP antibodies have also been implied by some authors. A 40-y-old woman developed CHB in the context of an acute flare-up of SLE, first diagnosed three years ago, having recently commenced hydroxychloroquine (HCQ) treatment. Anti-Ro and anti-RNP antibodies were also positive. No features of myocarditis were found. A temporary pacemaker was required and complete resolution was achieved on steroid therapy with withdrawal of antimalarial therapy. The characteristics of previous cases are well publicised and discussion focuses on the possible aetiology and pathogenesis of the present case.
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