系统性红斑狼疮合并可逆性脑白质后部综合征1例报道

目的 探讨系统性红斑狼疮（SLE）合并可逆性脑后部白质综合征（RPLS）的诱发因素、临床特点及诊治要点,以提高临床医生对该病的认识,降低误诊率。方法 对近期我科收治的1例SLE合并RPLS的临床资料进行了深入分析,并复习相关文献。结果 患者原发病诊断明确,入院后双手颤抖伴癫痫失神发作,予以大剂量激素联合他克莫司治疗及抗癫痫处理后效果不佳,停用他克莫司及积极控制血压等处理后,未再出现类似症状,头颅MRI提示病变范围较前明显缩小,综合考虑诊断为SLE合并RPLS。结论 SLE合并RPLS极易与神经精神狼疮、高血压脑病等疾病混淆,临床应提高对该病的认识和警惕,避免误诊误治。     

可逆性大脑后部白质脑病综合征16例的临床分析

目的探讨可逆性大脑后部白质脑病综合征的病因和临床特点,寻找早期诊断和治疗方法。方法选取该院2010．02～2013-02收治的16例可逆性大脑后部白质脑病综合征患者,回顾性分析其病因、临床表现特点、治疗及预后。结果16例患者中男6例,女10例。病因：高血压脑病7例（其中合并肾功能不全4例）,子痫4例,多发性大动脉炎2例,血栓性血小板减少性紫癜1例,系统性红斑狼疮1例,右颈内动脉内膜剥脱术后1例。临床表现以头痛、谵妄、意识障碍、癫痫发作性抽搐、视幻觉为主。头颅MRI检查可见大脑半球后部白质为主的T1加权呈短信号及12加权呈长信号的病灶,常双侧对称。经及时适当的脱水、降颅压,改善循环和高压氧等治疗,全组16例中,症状与体征消失者14例（87．5％）,明显好转者2例（12．5％）。结论可逆性大脑后部白质脑病综合征是一种具有自身病因、临床表现特点的临床综合征,经及时有效治疗,大多预后较好。     

Acute pan-dysautonomia as well as central nervous system involvement and peripheral neuropathies in a patient with systemic lupus erythematosus

A 32-year-old woman was diagnosed with leucopenia in 2002, being antinuclear antibody, anti-DNA antibody, and antiphospholipid antibody positive, and she was administered low-dose aspirin. In July 2006, she was admitted to our hospital because of pyrexia and abdominal pain. Examination revealed paralytic ileus, absence of the pupillary light reflex, dyshidrosis and anuresis. In addition, with high-level interleukin-6 in cerebrospinal fluid, the sensory nerve conduction velocity was derivation impotence. She was subsequently diagnosed with systemic lupus erythematosus (SLE) with central nervous system involvement, peripheral neuropathy as well as acute pan-dysautonomia. After pulse corticosteroid therapy, paralytic ileus was improved, however, the urination disorder persisted, and syncope due to orthostatic hypotension became marked. Plasma exchange and a second course of pulse corticosteroid therapy were performed, and were ineffective, whereas intravenous cyclophosphamide was effective. This patient is a rare case of central nervous system, peripheral neuropathy as well as acute pan-dysautonomia with SLE.     

Acute pan-dysautonomia as well as central nervous system involvement and peripheral neuropathies in a patient with systemic lupus erythematosus

Abstract

A 32-year-old woman was diagnosed with leucopenia in 2002, being antinuclear antibody, anti-DNA antibody, and antiphospholipid antibody positive, and she was administered low-dose aspirin. In July 2006, she was admitted to our hospital because of pyrexia and abdominal pain. Examination revealed paralytic ileus, absence of the pupillary light reflex, dyshidrosis and anuresis. In addition, with high-level interleukin-6 in cerebrospinal fluid, the sensory nerve conduction velocity was derivation impotence. She was subsequently diagnosed with systemic lupus erythematosus (SLE) with central nervous system involvement, peripheral neuropathy as well as acute pan-dysautonomia. After pulse corticosteroid therapy, paralytic ileus was improved, however, the urination disorder persisted, and syncope due to orthostatic hypotension became marked. Plasma exchange and a second course of pulse corticosteroid therapy were performed, and were ineffective, whereas intravenous cyclophosphamide was effective. This patient is a rare case of central nervous system, peripheral neuropathy as well as acute pan-dysautonomia with SLE.     

Thyroid hormones and thyroid-stimulating hormone in Egyptian patients with systemic lupus erythematosus: correlation between secondary hypothyroidism and neuropsychiatric systemic lupus erythematosus syndromes

 The purpose of this study was to determine the serum levels of thyroid hormones and thyroid-stimulating hormone (TSH), in addition to antithyroglobulin and antimicrosomal antibodies and to investigate the correlation between these hormones and various disease manifestations among Egyptian patients with systemic lupus erythematosus (SLE). A group of 45 patients with SLE (43 women and 2 men with a mean age of 27.57 ± 9.89 years) underment assessment of their thyroid hormones. Antithyroglobulin and antimicrosomal antibodies were assessed in 27 patients. Various disease manifestations were evaluated. A group of 20 normal female volunteers were involved as controls. The mean serum free triiodothyronine (FT₃) levels in all patients were significantly lower than in controls (1.89 ± 1.14 vs. 3.15 ± 0.93 pg/ml; P < 0.05). Patients with a history of intravenous pulsed cyclophosphamide therapy showed significantly decreased levels of FT₃ compared to those in other patients (1.17 ± 0.5 vs. 2.05 ± 0.95 pg/ml; P = 0.04). The mean serum free thyroxine (FT₄) levels in all patients were significantly less than in the control group (1.24 ± 1.22 vs. 1.4 ± 0.3 mg/dl; P < 0.001). Of the 45 patients, 2 (4.4%) were considered to have primary hypothyroidism. Five of six patients (83.3%) with decreased FT₄ levels developed fibromyalgia compared to 7 of 39 (17.9%) patients with normal T₄ (P = 0.003). The mean serum TSH levels in all patients were significantly higher than in the controls (4.82 ± 22.2 vs. 2.65 ± 1.18 μIU/ml; P < 0.001). Six patients with decreased TSH levels were considered to have secondary hypothyroidism (13.3%); one of them showed decreased T₃ and T₄, two had decreased T₄ only, and the other three were euthyroid. Comparing patients with and without secondary hypothyroidism, showed acute confusion in four (66.7%) in the former group versus four (10.3%) in the latter group (P = 0.006), anxiety in four (66.7%) in the former group versus six (15.4%) in the latter group (P = 0.016), and cognitive disorders in five (83.3%) in the former group versus nine (23.1%) in the latter group (P = 0.008). This study demonstrated evidence of secondary as well as primary hypothyroidism in SLE patients and revealed a close association between thyroid hormones or TSH and some organ involvement in SLE. Received: August 7, 2001 / Accepted: May 8, 2002 Present address: 453 Al-Ahram Street, Al-Ahram, Giza, Egypt Tel./Fax +202-5870668 e-mail: rughe@rusys.eg.net Correspondence to:A.A. Shahin     

Systemic lupus erythematosus complicated with posterior reversible encephalopathy syndrome and intracranial vasculopathy

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic condition characterized by reversible vasogenic edema on neuroimaging. It is associated with various neurological manifestations, including headaches, vomiting, seizures, visual loss, altered mental status and focal neurological deficits. PRES mainly occurs in the setting of eclampsia, hypertension, uremia, malignancy, transplantation, autoimmune diseases and/or use of immunosuppressive drugs. This syndrome has been described in patients with systemic lupus erythematosus (SLE). PRES is a potentially reversible clinical-radiological entity; however, it can be complicated with vasculopathy, infarction or hemorrhage. Vasculopathy has been demonstrated to be a common finding in patients with SLE. We report the case of a woman with lupus nephritis and PRES whose diffuse vasculopathy was present on initial neuroimaging. Subsequent brain computed tomography scan demonstrated interval development of intraparenchymal hemorrhage and subarachnoid hemorrhage. To our knowledge, this unique brain image pattern has not been reported in SLE patients.     

Reversible posterior leukoencephalopathy in patients with systemic lupus erythematosus

BACKGROUND: The development of central nervous system (CNS) symptoms in patients with preexisting systemic lupus erythematosus (SLE) evokes a wide differential diagnosis. Reversible posterior leukoencephalopathy (RPLE) is a rapidly evolving neurologic syndrome with characteristic clinical and radiographic features. Conditions commonly associated with RPLE include hypertensive encephalopathy, eclampsia, immunosuppressive drugs, and inflammatory disorders. OBJECTIVES: To describe our experience with RPLE in patients with concomitant SLE and review the literature. METHODS: The details of 5 novel cases and a MEDLINE review of the literature concerning the development of RPLE in association with SLE are presented. RESULTS: All cases included patients with SLE who developed the acute onset of headache, altered mental status, visual changes, and seizures. Neuroimaging demonstrated posterior white matter edema involving the parietal, temporal, and occipital lobes. Complete clinical and radiographic recovery occurred with prompt antihypertensive treatment and supportive care. Literature review identified 16 additional cases of RPLE occurring in patients with active SLE; the majority of these reports was similar in presentation and outcome to our experience. CONCLUSIONS: It is likely that the clinical manifestations and neuroimages in these lupus patients were the result of the RPLE syndrome. Fortunately, this cause of "secondary" CNS symptoms in patients with SLE is readily reversible when diagnosed early and treated with blood pressure control and supportive care.     

Pathogenesis of neuropsychiatric systemic lupus erythematosus and potential biomarkers

Abstract

Systemic lupus erythematosus is a chronic, multisystemic, autoimmune disease that may involve the central, peripheral, and autonomic nervous systems and can present with a wide variety of neurological and psychiatric manifestations. In this article, we review the recent literature pertaining to the pathogenesis of neuropsychiatric systemic lupus erythematosus (NPSLE). We searched the PUBMED database with no chronological constraints using the following terms: “neuropsychiatric systemic lupus erythematosus” cross-referenced with the terms “pathogenesis” and “biomarkers” for full-text articles in English. The etiology of NPSLE is as yet unknown, though numerous autoantibodies and cytokines have been suggested as possible mediators. Of the numerous autoantibodies and biomarkers examined, anti-phospholipid, anti-ribosomal P, anti-neuronal, anti-glial fibrillary acidic protein (GFAP), anti-endothelial cell, anti-N-methyl-d-aspartate (NMDA), microtubule-associated protein 2 (MAP-2), and matrix metalloproteinase-9 (MMP-9) appear to be elevated in patients with NPSLE. Cytokines that may be involved in the pathology of NPSLE include interleukin (IL)-2, IL-6, IL-8, IL-10, tumor necrosis factor (TNF)-α, and interferons (IFN)-α and -γ. With continued advances in immunological research, new insights into the pathophysiologic mechanisms of NPSLE may lead to the development of biomarkers and new treatment strategies.     

Clinical and immunological features of systemic lupus erythematosus complicated by Jaccoud's arthropathy

This work was undertaken to evaluate clinical and immunological features in patients with systemic lupus erythematosus (SLE) complicated by Jaccoud's arthropathy. Patients diagnosed with SLE between 1985 and 1999, and who met the criteria of Villiaumey et al., were checked for Jaccoud's arthropathy. Clinical features were retrospectively analysed for patients with both diseases. Sjögren's syndrome and human leukocyte antigens (HLA) in these patients were evaluated. Jaccoud's arthropathy was found in 15 (4.4%) of 340 patients with SLE. The mean age at the time of SLE diagnosis was significantly higher in these patients than in our control SLE patients, which was 51.2 ± 13.0 years (n = 15) and 29.6 ± 13.0 years (n = 222) (p = 2.1 × 10^?8). Sjögren's syndrome was diagnosed according to the European Community Study Group's criteria in 10 (91%) of 11 patients examined. The incidence of HLA-A11 (5/9, 55%) and -B61(40) (5/9, 55%) in patients with Jaccoud's arthropathy was higher in the Japanese population (A11, 17.4%, p < 0.05; B61, 17.5%, p < 0.057. Jaccoud's arthropathy in patients with SLE is associated with Sjögren's syndrome, elderly SLE, HLA-A11, and HLA-B61. These clinical features might be characteristic of patients with Jaccoud's arthropathy and SLE.     

Long-term remission in three patients with childhood-onset systemic lupus erythematosus

Abstract

Three cases of childhood-onset systemic lupus erythematosus (childhood SLE) with long-term remission are reported. These cases were not complicated with collagen diseases and had no SLE disease activity index scores either 3 or 5 years after onset. We suggest that some patients with childhood SLE may be able to abandon the maintenance therapy, and that careful observation is needed for each individual case. Uniform remission criteria based on clinical trials are needed.     

Long-term remission in three patients with childhood-onset systemic lupus erythematosus

Three cases of childhood-onset systemic lupus erythematosus (childhood SLE) with long-term remission are reported. These cases were not complicated with collagen diseases and had no SLE disease activity index scores either 3 or 5 years after onset. We suggest that some patients with childhood SLE may be able to abandon the maintenance therapy, and that careful observation is needed for each individual case. Uniform remission criteria based on clinical trials are needed.     

Early morning neutropenia in a patient with systemic lupus erythematosus

Abstract

An autopsy case of an 11-year-old boy with polyarteritis nodosa is described in which the onset of the disease was associated with the presence of hepatitis B (HB) antigens (Ag) in the cytoplasm and nuclei of hepatocytes as detected by immunohistological methods. Deposits of HBsAg, HBeAg, IgG, IgM, C3, and C1q were demonstrated in systemic vascular lesions. It is considered that the arteritis was due to deposition in the arteries of immune complexes formed by HBAg and HB antibodies.     

可逆性后部白质脑病综合征的临床和影像学特征:9例回顾性病例系列研究

目的 探讨可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)的临床和影像学特征.方法 回顾性分析9例RPLS患者的临床和影像学资料.结果 继发于妊娠高血压综合征4例(44%),短肠综合征1例(11%),急性淋巴细胞白血病1例(11%)...     

Catastrophic transverse myelitis in a patient with systemic lupus erythematosus

A 24-year-old Japanese woman was admitted to our hospital suffering from high fever and progressive paralysis in both legs. Magnetic resonance imaging of the spinal cord showed high-intensity signals from C5 to Th4 and from Th7 to L1 on T2-weighted images. The patient was diagnosed as having acute transverse myelitis , which was a complication of systemic lupus erythematosus based on the serological findings. Despite aggressive immunosuppressive treatments including corticosteroid pulse therapy, plasmapheresis, and intravenous cyclophosphamide, the paralysis of her lower extremities did not improve. In the catastrophic type of lupus-associated TM, which develops extensively and longitudinally along the spinal cord, the prognosis still seems to be poor despite intensive treatments.     

Acute respiratory distress syndrome due to systemic lupus erythematosus with hemophagocytic syndrome: an autopsy report

This report concerns a patient with systemic lupus erythematosus (SLE) who died of acute respiratory distress syndrome (ARDS) 1 day after the onset of pulmonary symptoms. Autopsy demonstrated severe hemophagocytosis in the bone marrow and histopathology indicating a marked increase in vascular permeability in both lungs and kidneys. In this patient, active SLE and associated hemophagocytic syndrome may have induced an increase in the production of inflammatory cytokines, which immediately induced ARDS. Since fatal ARDS can occur as a life-threatening complication of SLE, careful observation is necessary, particularly when there are clinical findings suggestive of associated hemophagocytic syndrome.     

系统性红斑狼疮脑病36例临床分析

目的研究系统性红斑狼疮脑病患者临床表现特点。方法分析、归纳2000-01-01~2004-10-10中日友好医院神经内科36例系统性红斑狼疮脑病患者症状、体征、辅助检查。结果36例系统性红斑狼疮脑病患者主诉症状9类,其中头痛、意识障碍、肢体无力居发生率前3位;发现体征13类,其中病理反射、意识障碍、肢体瘫痪居前3位。结论(1)系统性红斑狼疮脑病是活动性系统性红斑狼疮的表现之一。(2)系统性红斑狼疮患者无论病史长短,均可发生系统性红斑狼疮脑病。(3)系统性红斑狼疮脑病表现复杂多样。     

A case of systemic lupus erythematosus associated with trigger wrist

A 54-year-old woman complained of a painful, swollen, and clicking left wrist for 1 year. She had an 8-year history of systemic lupus erythematosus (SLE), treated with oral prednisolone. Flexion and extension of all fingers were difficult to initiate on wrist extension, and movement was accompanied by a palpable click at the wrist. Magnetic resonance imaging (MRI) and tenography revealed the expanded sheath of the flexor tendons and well-defined round free bodies known as rice bodies. Synovectomy and excision of rice bodies resulted in complete disappearance of swelling and triggering of the wrist. Received: August 23, 2000 / Accepted: July 6, 2001     

Successful plasmapheresis in alveolar hemorrhage associated with systemic lupus erythematosus

Abstract

The case of an 18-year-old Japanese man with systemic lupus erythematosus (SLE) complicated by alveolar hemorrhage is described. The patient presented with fever, butterfly rash, and polyarthralgia, and was diagnosed with SLE. He suddenly developed alveolar hemorrhage during steroid pulse therapy. Treatment with plasmapheresis was initiated, with prompt clearing of the chest radiograph. This experience suggests that the prompt initiation of plasmapheresis should be considered for SLE patients with life-threating alveolar hemorrhage resistant to conventional immunosuppressive therapies.