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1/252. Primary subcutaneous nocardial infection in a SLE patient.

    A patient with systemic lupus erythematosus (SLE) developed primary subcutaneous nocardiosis during steroid and cyclophosphamide therapy for diffuse proliferative glomerulonephritis. In spite of local process the patient manifested signs of general deterioration mimicking SLE exacerbation. The diagnosis was made by bacteriologic examination of the material obtained by CT guided aspiration. Surgical drainage and systemic treatment with trimethoprim/sulphamethoxazole (TMT/SMZ) 960 mg twice/d led to a clinical recovery and enabled the continuation of the steroid and cytotoxic regimen.
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2/252. Successful treatment of systemic lupus erythematosus cerebritis with intravenous immunoglobulin.

    Neuropsychiatric lupus includes extremely diverse clinical manifestations, ranging from mild cognitive dysfunction to a severe, life-threatening presentation. We report a 28-year-old patient with systemic lupus erythematosus who had persistent fever for 3 months, and developed within a few hours motor and sensory aphasia, rotator nystagmus with deviation of the eyes, and severe nuchal rigidity. An extensive series of imaging and laboratory tests were interpreted as normal, except for an elevated opening pressure at lumbar puncture, cerebrospinal fluid inflammatory findings, and asymmetrical cortical perfusion on single-photon emission computed tomography. The patient received one course of high-dose intravenous immunoglobulin (IVIg) and within 5 days her condition returned to that of 3 months before admission. The mechanisms of injury, along with the management of cerebral lupus and the mechanisms of action of IVIg, are discussed.
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3/252. Cutaneous tuberculosis mimicking cellulitis in an immunosuppressed patient.

    A 28-year-old lady suffering from systemic lupus erythomatosus (SLE) with diffuse proliferative glomerulonephritis (DPGN) and who was on oral cyclophosphamide and prednisolone presented with left lower limb 'cellulitis'. The 'cellulitis' of the left lower limb failed to respond to usual antibiotics which prompted evaluation of the clinical diagnosis. The diagnosis is made based on the presence of granulomas, multinucleated giant cells and acid fast bacilli on the skin biopsy.
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4/252. Systemic lupus erythematosus and Castleman's disease.

    Lymphadenopathy is a common finding in systemic lupus erythematosus (SLE), yet lymphoid malignancy is rare. Typically, adenopathy associated with SLE responds to glucocorticoid therapy. We evaluated a patient with a diagnosis of SLE who had progressive lymphadenopathy despite receiving aggressive corticosteroid therapy for SLE associated thrombocytopenia. Histopathology initially revealed an aggressive plasmacytosis characteristic of Castleman's disease (CD). CD, or angiofollicular hyperplasia, is a rare lymphoproliferative neoplasm that has features overlapping many autoimmune diseases. This disorder should be considered in autoimmune diseases with unremitting or progressive adenopathy.
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5/252. Systemic lupus erythematosus with a giant rectal ulcer and perforation.

    A 41-year-old man with systemic lupus erythematosus (SLE) who developed pelvic inflammation due to perforation of a giant rectal ulcer is described. The patient presented with persistent diarrhea, abdominal pain and fever without development of disease activity of SLE. Endoscopic and radiological examinations revealed a perforated giant ulcer on the posterior wall at the rectum below the peritoneal evagination. The ulcerated area was decreased after a colostomy was performed at the transverse colon to preserve anal function. The patient is currently being monitored on an outpatient basis. It should be noted that life-threatening complications such as perforated ulcer of the intestinal tract could occur without SLE disease activity.
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6/252. Long-lasting remission and successful treatment of acquired factor viii inhibitors using cyclophosphamide in a patient with systemic lupus erythematosus.

    Acquired deficiency of clotting factor viii (FVIII) is a rare bleeding diathesis seldom encountered in systemic lupus erythematosus (SLE). Reduction of FVIII activity by autoantibodies can cause potentially life-threatening situations. Herein, an SLE patient with a positive lupus anticoagulant (LAC) test who abruptly developed metrorrhagia 4 yr after diagnosis is reported. Coagulation tests revealed FVIII activity reduced to 3% and a prolonged aPTT. FVIII inhibitor(s) were found to be as high as 3.0 Bethesda Units. plasmapheresis, immunoglobulins, prednisolone and FVIII plasma concentrates induced the cessation of metrorrhagia, but the clotting tests were barely improved. One month later, extensive ecchymosis appeared and worsened, despite re-administration of the previous therapy. pulse cyclophosphamide followed by oral administration was then started with normalization of coagulation parameters and long-lasting disease remission.
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7/252. Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood.

    OBJECTIVE: To determine the association between childhood-onset thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE). methods: The charts of all 5 patients diagnosed with idiopathic TTP at the Hospital for Sick Children (HSC) in Toronto from 1975 to 1998, and all cases of childhood-onset TTP (ages 6-20 years) reported in the literature over the same period were reviewed. Fourteen of the 44 patients identified in the literature were excluded from the analysis for lack of clinical and laboratory information. The remaining 35 patients were grouped into either an SLE/TTP group or a TTP only group, according to the presence or absence of the American College of rheumatology (ACR) classification criteria for SLE. The groups were compared for differences in clinical or laboratory features. RESULTS: The clinical presentation and initial disease course of pediatric patients with TTP were similar to those observed in adults. Of the 35 patients with childhood-onset TTP included in this review, 9 (26%) fulfilled > or = 4 ACR criteria for SLE and 8 (23%) were found to have incipient SLE. Of the 5 patients initially diagnosed with idiopathic TTP at the HSC, 3 were diagnosed with SLE within 3 years, and the other 2 patients fulfilled 3 ACR classification criteria for SLE within 4 years of disease onset. The clinical syndrome of pediatric TTP presenting with proteinuria, especially with high-grade proteinuria, was significantly associated with the development or coexistence of childhood-onset SLE. CONCLUSION: TTP in childhood is a rare, but life-threatening, disease. Unlike in adults, TTP in childhood is commonly associated with SLE. High-grade proteinuria at diagnosis of TTP is the best predictor for the presence or subsequent development of SLE.
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8/252. calciphylaxis precipitated by ultraviolet light in a patient with end-stage renal disease secondary to systemic lupus erythematosus.

    calciphylaxis is a rare and severe calcification syndrome described mainly in patients with end-stage renal disease (ESRD) undergoing dialysis or with a renal transplant. This life-threatening condition is characterized by the abrupt onset of painful ischemic skin ulcers and necrosis. Secondary local and systemic infection may supervene and, without timely and appropriate interventions, calciphylaxis may be fatal. A precipitant or challenging agent is believed to be necessary to initiate the process. We describe a case of a woman with ESRD receiving continuous ambulatory peritoneal dialysis who developed calciphylaxis in the setting of severe hyperparathyroidism after receiving UV photoradiation therapy. We suggest that the UV light served as the challenging agent in the precipitation of this devastating condition.
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9/252. Massive gastrointestinal hemorrhage in systemic lupus erythematosus: successful treatment with corticosteroid pulse therapy.

    Although mesenteric vasculitis due to systemic lupus erythematosus (SLE) is relatively uncommon, it is the most dangerous manifestation associated with high mortality. We describe the case of a SLE patient with life-threatening gastrointestinal hemorrhage due to mesenteric vasculitis in whom methylprednisolone pulse therapy was quite effective in controlling the hemorrhage and resulted in a satisfactory long term outcome. A 47-yr-old woman presenting with high fever, rash, and melena was diagnosed with SLE from positive antinuclear antibodies, anti-dsDNA, and low complement titers. Although fever and rash subsided with administration of prednisolone, massive hematemesis appeared with melena. endoscopy demonstrated bleeding ulceration of the antrum, which was intractable despite intensive antiulcer therapy and transfusion. Surgical exploration revealed ileal penetration, and multiple bleeding ulcerations were observed over the resected ileum as well as the antral ulceration. However, bleeding persisted after surgery and surgical findings prompted us to select methylprednisolone pulse. Hemorrhage responded promptly to the therapy, and the patient has remained well since then for >10 yr. Our report indicates that corticosteroid pulse may serve as one of the therapeutic options for SLE with massive hemorrhage due to widespread mesenteric vasculitis.
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10/252. Daily psychosocial stressors interfere with the dynamics of urine neopterin in a patient with systemic lupus erythematosus: an integrative single-case study.

    OBJECTIVE: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by flare-ups, the causes of which are not known. In accordance with new concepts in stress research, this study investigated whether daily psychosocial stressors interfere with immunological processes in SLE. Because such processes are unique to each individual, single-case design using time-series analysis (Box and Jenkins) was applied. methods: A 40-year-old woman with SLE (last flare-up September 1995) was interviewed initially to determine major life events and difficulties (using the life Events and Difficulties Schedule) in the previous 2 years. She was then observed for 63 days. urine neopterin, an immunological parameter demonstrated to parallel disease activity in SLE patients, was measured in daily overnight urine. Daily incidents were identified weekly by the Incidents and Hassles Inventory and independently rated. Intervening factors, including infections, medication, and lifestyle, were controlled. RESULTS: Retrospectively, data obtained from the life Events and Difficulties Schedule indicated that major life events and difficulties had preceded the patient's last flare-up in 1995. Although there were no clinical signs of SLE during this prospective study of 63 days, cross-correlational analyses revealed that "moderately" stressful incidents associated with higher levels of emotional irritation (lag 0: 0.271, p < .05) predicted an increase in urine neopterin the following day (lag 1: 0.441, p < .05). Moreover, a 7-day cyclicity in neopterin levels that corresponded to the weekly examinations and interviews was found. CONCLUSIONS: This study showed a causal relationship between psychosocial stressors and urine neopterin concentrations that may be related to SLE disease activity. Furthermore, the workability of an integrative approach using single-case design and time-series analysis in psychoneuroimmunology was demonstrated for the first time.
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