Clinical spectrum of males with primary antiphospholipid syndrome and systemic lupus erythematosus: a comparative study of 73 patients

The objective of this study was to compare the clinical findings, laboratory data, functional outcome and chronic damage in male patients with primary antiphospholipid syndrome (PAPS) and systemic lupus erythematosus (SLE). We studied 29 male patients with PAPS and 44 with SLE. Clinical findings, laboratory data, lupus damage index (SLICC/ACR DI), and functional outcome in PAPS, were analysed in each group. The mean age at diagnosis was 29.8 +/- 10.4 years in patients with PAPS and 26 +/- 10.1 years in SLE patients. The duration of disease was 4.5 +/- 2.6 versus 5.2 +/- 3.8 years in patients with PAPS and SLE, respectively (P = NS). In patients with PAPS the most frequent clinical manifestations were venous thrombosis, thrombocytopenia, and pulmonary thromboembolism. Patients with SLE had joint, skin and renal involvement more frequently than those with PAPS (P = 0.0001). All PAPS patients had anticardiolipin antibodies (aCL), and 14 patients (48%) had lupus anticoagulant (LA). All SLE patients had antinuclear antibodies (ANAs). Anti-dsDNA antibodies were positive in 39% of SLE patients. Five patients died: one with 'catastrophic' APS and four with SLE. SLICC/ACR-DI score in SLE patients was 1.9 (SD = 1). In PAPS patients poor functional outcome was due to myocardial infarction, pulmonary thromboembolism, stroke and mesenteric thrombosis. Lupus nephritis was the principal organ damage in SLE. In conclusion, in male patients with PAPS and SLE, the clinical manifestations were significantly different. Arterial thrombosis was the major cause of functional impairment and permanent organ damage in PAPS. Renal involvement was the major cause of chronic damage in SLE.     

系统性红斑狼疮合并Evans综合征临床特点

目的总结系统性红斑狼疮(systemic lupus erythematosus,SLE)合并Evans综合征患者的临床特点。方法回顾性分析北京协和医院2004年1月至2012年7月SLE合并Evans患者的临床表现及实验室特点及治疗和预后。结果 SLE并发Evans综合征患者22例,占同期SLE住院患者3400例的0.65%。其中男3例,女19例,平均35.1岁(16~53岁)。22例患者中以血液系统受累[特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)或自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)]为首发表现的11例(50%),确诊SLE后诊断Evans综合征者6例,二者同时诊断的5例。SLE并发Evans综合征时,患者往往有多系统受累,表现为肾脏受累13例(59.1%),皮肤黏膜受累、关节炎各9例(40.9%),神经系统受累4例(18.2%),胃肠道、肺部受累各2例等。Evans综合征多发生于SLE活动期,患者平均狼疮活动指数评分(11.45±7.6)分(3~30分)。伴发其他结缔组织病5例(22.7%)。经激素联合免疫抑制剂治疗后,20例好转,2例无效者应用利妥昔单抗后好转。结论 SLE合并Evans综合征罕见,发生于SLE多系统受累及活动期。部分患者以ITP或AIHA为SLE首发表现,应及时筛查多种自身抗体,并定期随访密切观察,以期早期诊治。     

Impact of C1q deficiency on the severity and outcome of childhood systemic lupus erythematosus

Objective: To delineate the clinical and laboratory features of systemic lupus erythematosus (SLE) in C1q‐deficient Saudi children and to compare them with sporadic SLE patients with respect to their clinical and laboratory features and disease outcome. Methods: The C1q‐deficient SLE patient group comprised 14 patients, while the comparative group comprised 11 patients selected by systemic sampling from our pediatric lupus clinic database. The two groups were compared with respect to: demographic, clinical and laboratory variables and outcome. Results: The C1q‐deficient SLE patients had an earlier age of onset of disease (P = 0.003); 43% had familial SLE and none of the comparative group had family histories of SLE. The two groups were comparable with respect to gender, disease duration and follow‐up. Scarring alopecia, discoid rash and nail changes were more frequent in the C1q‐deficient SLE patient group. However, there were no significant differences. The mean white blood cell count was lower (P = 0.04) and the level of anti‐Sm and anti‐phospholipid antibodies were higher (P = 0.04) in the C1q‐deficient SLE patients. Other variables did not show significant differences. Two patients from the C1q‐deficient SLE patient group died due to infection. All patients from the control group are alive. Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index mean was higher in the C1q‐deficient SLE patients group but was not statistically significant. Conclusion: C1q‐deficient SLE patients tend to be younger and more likely to have familial disease with severe cutaneous manifestations. The mortality among them is more frequent, which may reflect disease severity.     

系统性红斑狼疮合并血栓性血小板减少性紫癜14例临床分析

 目的 探讨系统性红斑狼疮（SLE）合并血栓性血小板减少性紫癜 (TTP) 的临床特点、诊断及治疗。方法 回顾性分析14例SLE合并TTP者的临床表现、实验室检查结果、诊治和转归。结果 14例SLE合并TTP者中,男4例,女10例,中位年龄23（17～69）岁。5例以SLE首发,9例两者同时发病。所有患者均有血小板减少、微血管病性溶血性贫血,发热、神经系统异常、肾功能损害的发生比例分别为12/14、11/14、11/14,其中8例患者具有上述五联征。糖皮质激素（单用或联合静脉注射人免疫球蛋白和环磷酰胺）治疗6例,糖皮质激素联合血浆置换8例,有效率分别为2/6、6/8。8例患者存活,6例死亡。对6例存活者进行了随访,均无复发。结论 SLE与TTP临床表现相似,多次重复血涂片检查对早期明确诊断SLE合并TTP十分重要。SLE合并TTP者肾功能损害程度比单纯TTP或SLE者更为严重,早期诊断并及时应用糖皮质激素联合血浆置换有助于改善患者的预后。     

Pattern of neuropsychiatric manifestations and outcome in juvenile systemic lupus erythematosus

The aim of this study was to study the neuropsychiatric (NP) manifestations, diagnostic evaluation, treatment and outcome in juvenile systemic lupus erythematosus (SLE). We reviewed the charts of all children with SLE and evidence of NP manifestations as defined by the presence of at least one of the following: headache, cerebrovascular accident (CVA), chorea, seizure, papilledema, and psychiatric or spinal cord manifestations. Out of 90 children with SLE, 20 (16 female) had NP manifestations. The mean age at onset was 8.8 years. The mean period between onset of SLE and NP manifestations was 10.2 months. NP manifestations were the presenting feature in 3 patients. Eleven patients had headache, 10 had psychiatric manifestations, 10 had seizure and 6 had CVA. Coma was seen in 5 patients, chorea in 4, transverse myelitis in 2 and papilledema in 2. Anticardolipin antibodies were high in 12 patients. Five patients had an abnormal CSF study. Nine patients had EEG abnormalities and 13 showed MRI abnormalities. All patients received oral prednisone and 17 were treated with IVMP and immunosuppressive therapy (cyclophosphamide or azathioprine); 85% of our patients recovered completely, but 15% had persistent NP sequelae; 10% died from severe infection. In conclusion, NP involvement in juvenile SLE is common. However, early diagnosis and early treatment with adjunctive intravenous pulse cyclophosphamide may improve the outcome.Abbreviations CVA Cerebrovascular accident - IVMP Intravenous methylprednisolone - NP Neuropsychiatric - SLE Systemic lupus erythematosus     

The spectrum of pericardial tamponade in systemic lupus erythematosus. Report of ten patients.

OBJECTIVE. To describe the range of clinical manifestations and the outcome of pericardial tamponade in patients with systemic lupus erythematosus (SLE). METHODS. Patients with pericarditis and with pericardial tamponade were identified from our computerized database of 395 SLE patients. Medical records were reviewed to establish activity of SLE at the time of tamponade, as well as clinical and laboratory features, treatment, and outcome of the tamponade. RESULTS. Pericarditis occurred in 75 patients (19%), with 11 episodes of tamponade in 10 of them (13%; 2.5% of entire series). Tamponade was the initial manifestation of SLE in 4 patients. Seven episodes occurred during active lupus, with nephritis present in 6. Signs of venous congestion, including ascites and facial or peripheral edema, were the most common manifestation of tamponade. Pericardial fluid was exudative, and pericardial tissue demonstrated a range of findings including fibrinous and fibrotic changes, acute and chronic inflammatory infiltrates, and vascular proliferation. Tamponade was fatal in 1 patient, and 2 patients each had recurrent effusions and pericardial thickening. CONCLUSION. Pericardial tamponade may occur at any point in the course of SLE, and should be considered in patients with unexplained signs of venous congestion. The differential diagnosis includes active SLE, uremia, and infection. Treatment with high-dose steroids and either pericardiocentesis or placement of a pericardial window is indicated, but recurrent effusions or pericardial thickening may develop.     

Protein-losing enteropathy in systemic lupus erythematosus: analysis of the clinical features of fifteen patients.

OBJECTIVE: Protein-losing enteropathy (PLE) is an unusual manifestation of systemic lupus erythematosus (SLE), so its clinical manifestations and management are not well understood. In this study, we try to characterize the basic clinical features and the management of PLE by retrospectively analyzing the clinical data of 15 PLE patients and hope this study can improve the awareness of PLE in lupus patients with severe hypoalbuminemia that could not be explained by other causes. METHODS: The clinical data of 15 SLE patients with PLE hospitalized during November 2001 and April 2006 in Peking Union Medical College Hospital were retrospectively reviewed. The PLE was diagnosed by Tc-99m albumin scintigraphy (99mTc-HAS). The clinical characteristics, laboratory tests, response to treatment, and the outcome were studied. RESULTS: The mean age of PLE onset was 40.1 +/- 15.4 years (19-71 years). Twelve were female and 3 were male. 53.3% (8 of 15) patients had PLE as the initial presentation of SLE. All patients had different degree of peripheral pitting edema. Eleven had ascites, 9 had pleural effusion, and 7 had pericardial effusion. Only 6 patients presented with abdominal pain and diarrhea. Positive antinuclear antibodies (HEP-2) with a speckled pattern were found in all patients, but the antidsDNA antibody was negative in most cases. All patients had marked hypoalbuminemia, 80% had hypocomplementemia, 66.7% had hyperlipoproteinemia, and 40% had hypocalcemia. The liver function tests and the prothrombin time were in normal ranges. The 24-hours urine protein was less than 0.5 g in 60% (9 of 15) and more than 1.0 g in 20% (3 of 15) patients who were renal biopsied but only found to have very mild pathologic changes. Gastrointestinal endoscopy examination discovered generalized edema in the intestinal wall whereas the biopsy showed chronic inflammation only. Most cases had good response to corticosteroid and immunosuppressive therapies. The serum albumin level improved evidently in all patients after treatment and normal scintigraphic finding was found in 9 patients. CONCLUSION: PLE can be the initial presentation of SLE or can develop a very long time after the diagnosis of SLE. The prominent clinical presentations are caused by hypoalbuminemia. 99mTc-HAS is useful not only for the diagnosis of PLE but is also helpful for monitoring the efficacy of treatment. When a SLE patient presents with evident hypoalbuminemia without evidence of other causes, PLE should be considered. Early diagnosis and treatment may improve the prognosis.     

肝豆状核变性并发红斑狼疮4例及文献复习

目的 分析肝豆状核变性(Wilson's disease,WD)并发红斑狼疮患者的临床特点.方法 收集北京协和医院2013年1月至2020年1月WD并发红斑狼疮患者的临床资料,回顾性分析及总结临床表现、实验室检查、诊治及转归并复习相关文献.结果 WD并发红斑狼疮患者4例,其中2例为青霉胺诱导的药物性红斑狼疮(drug ...     

Clinical and electrophysiological characteristics of peripheral neuropathy in Cuban systemic lupus erythematosus patients

BackgroundPeripheral neuropathy (PN) is one of the most heterogeneous and poorly understood or characterized manifestations in systemic lupus erythematosus (SLE). The aim of this study was to describe the clinical and electrophysiological features, and neuropathic disease associations, in Cuban SLE patients.Patients and methodsOne hundred and two consecutive SLE patients admitted to the Psychoneuroimmunology service at the National Institute of Nephrology were included in the study. Patients with other disorders known to cause neuropathy were excluded. Demographic, clinical and laboratory data were obtained using a pre-defined questionnaire. Nerve conduction studies were carried out in both upper and lower limbs. Neuropathy was defined as the presence of clinical symptoms and/or signs and bilateral abnormal nerve conduction study parameters.ResultsThe 102 patients were 99 females and 3 males with mean age of 46 ± 12 years and disease duration 8 ± 9 years. PN was found in 55/102 (53.9%) patients; 48 (87.3%) had clinical peripheral neuropathy manifestations and 7 (12.7%) were asymptomatic. Nerve conduction studies suggested asymmetric axonal-demyelination neuropathy pattern. Mixed sensory-motor neuropathy was the most common involvement in 23(41.8%) cases. The most frequent pattern was polyneuropathy. Compared to those without neuropathy, SLE-related polyneuropathy patients were significantly older, but had no other significant associations with demographic, disease duration or serological/immunological data.ConclusionPeripheral nervous system manifestations are common in SLE; may be related to an increased susceptibility of peripheral nerves to effects of aging. Nerve conduction studies are recommended, therefore, for inclusion in the follow-up of SLE patients especially in the older population.     

Clinical features and outcome of neuropsychiatric lupus in Chinese: analysis of 240 hospitalized patients

Neuropsychiatric (NP) events are severe manifestations of systemic lupus erythematosus (SLE) and relate to poor outcome. The aims of this study are to investigate the NP manifestations of SLE and to identify the predictive factors for clinical outcome. There was a retrospective review of 240 hospital patients with primary NP events of SLE (NPSLE) from 1990 to 2004. Neuropsychiatric manifestations, SLE disease activity index (SLEDAI) score, System lupus International Collaborating Clinic/American College of Rheumatology Damage Index (SLICC/ACR-DI) score, magnetic resonance imaging (MRI) findings, treatment and mortality rate were included for analysis. From this group of patients, 15 NP syndromes were identified. The most frequent manifestation was headache, followed by seizure. The mean SLEDAI and SLICC/ACR-DI scores were 19.9 +/- 6.9 and 3.5 +/- 1.6, respectively. Abnormal MRI features were found in 67% (61/91) patients. At least one intrathecal (IT) injection of methotrexate (MTX) plus dexamethasone (DXM) was administered to 109 (45.4%) patients. High dose (1 g) intravenous methylprednisolone pulse therapy (IVMP) was administered to 167 (69.5%) patients. Multifactor analysis revealed that high SLICC/ACR-DI scores and sets of concurrent NP symptoms were independently associated with poor outcome, whereas pulse IVMP and IT injection of MTX plus DXM were protective factors against poor outcome. From our data, NPSLE is heterogeneous and is usually associated with high disease activity and organ damage scores. High SLICC/ACR-DI score and having more than two sets of NP symptoms are the predictors for poor outcome, whereas pulse IVMP and IT injection of MTX plus DXM can improve the prognosis.     

The spectrum of pericardial tamponade in systemic lupus erythematosus: Report of ten patients

Objective. To describe the range of clinical manifestations and the outcome of pericardial tamponade in patients with systemic lupus erythematosus (SLE). Methods. Patients with pericarditis and with pericardial tamponade were identified from our computerized database of 395 SLE patients. Medical records were reviewed to establish activity of SLE at the time of tamponade, as well as clinical and laboratory features, treatment, and outcome of the tamponade. Results. Pericarditis occurred in 75 patients (19%), with 11 episodes of tamponade in 10 of them (13%; 2.5% of entire series). Tamponade was the initial manifestation of SLE in 4 patients. Seven episodes occurred during active lupus, with nephritis present in 6. Signs of venous congestion, including ascites and facial or peripheral edema, were the most common manifestation of tamponade. Pericardial fluid was exudative, and pericardial tissue demonstrated a range of findings including fibrinous and fibrotic changes, acute and chronic inflammatory infiltrates, and vascular proliferation. Tamponade was fatal in 1 patient, and 2 patients each had recurrent effusions and pericardial thickening. Conclusion. Pericardial tamponade may occur at any point in the course of SLE, and should be considered in patients with unexplained signs of venous congestion. The differential diagnosis includes active SLE, uremia, and infection. Treatment with high-dose steroids and either pericardiocentesis or placement of a pericardial window is indicated, but recurrent effusions or pericardial thickening may develop.     

Severe clinical course of systemic lupus erythematosus in the first year of life

Systemic lupus erythematosus (SLE) very rarely occurs before the age of 5. Herein we describe the clinical features of infantile SLE (iSLE) with onset during the first year of life. The clinical and laboratory characteristics of iSLE patients followed at the Department of Pediatrics of Padua were analyzed. They were combined with those collected from the literature by performing a systematic literature search on PubMed using the following keywords: SLE, infant, laboratory, therapy, and outcome. A total of 13 patients with iSLE, 2 from our Institution and 11 from the literature, are included in this review. Seven (53.8%) were females and 6 were males (46.2%). The age at disease onset ranged from 6 weeks to 11 months. In comparison with juvenile systemic lupus erythematosus (jSLE), iSLE showed a higher prevalence of positive family history for autoimmune diseases, systemic symptoms at presentation, internal organs involvement, and shorter time between symptoms onset and diagnosis. Anemia and thrombocytopenia were present in the majority of the patients at diagnosis, whereas leukopenia was rarely observed. The overall prognosis in iSLE was very poor: 5/13 infants died between 2 and 31 months after the onset, and 5/13 had severe disease course with residual organ damage. SLE can start as early as during the first year of life and is more severe than in the later age groups.     

系统性红斑狼疮合并巨细胞病毒感染121例临床分析

目的 探讨系统性红斑狼疮(SLE)合并巨细胞病毒(CMV)感染的临床表现.方法 分析SLE合并CMV感染患者的相关临床资料,进行统计分析.结果 2221例SLE住院患者中,合并CMV活动性感染121例,发生率为5.4%.发热98例占81%,其中80例(66.1%)以发热为唯一表现,肝功能损害8例、呼吸道症状5例、血液系统异常4例、心肌炎和脑病各1例,分别占6.6%、4.1%、3.3%、0.8%、0.8%;另22例(18.2%)不明原因原发病活动而经检查发现CMV感染.75.2%患者SLE疾病活动指数达10分以上.121例中,4例死亡,6例放弃治疗.其余患者CMV感染均于治疗2～4周[(15±6)d]得到控制.结论 CMV感染是SLE的常见合并症,临床表现无特征性,对不明原因发热者应及时做CMV的相关检查以利诊断.     

系统性红斑狼疮合并巨细胞病毒感染121例临床分析

目的 探讨系统性红斑狼疮(SLE)合并巨细胞病毒(CMV)感染的临床表现.方法 分析SLE合并CMV感染患者的相关临床资料,进行统计分析.结果 2221例SLE住院患者中,合并CMV活动性感染121例,发生率为5.4%.发热98例占81%,其中80例(66.1%)以发热为唯一表现,肝功能损害8例、呼吸道症状5例、血液系统异常4例、心肌炎和脑病各1例,分别占6.6%、4.1%、3.3%、0.8%、0.8%;另22例(18.2%)不明原因原发病活动而经检查发现CMV感染.75.2%患者SLE疾病活动指数达10分以上.121例中,4例死亡,6例放弃治疗.其余患者CMV感染均于治疗2～4周[(15±6)d]得到控制.结论 CMV感染是SLE的常见合并症,临床表现无特征性,对不明原因发热者应及时做CMV的相关检查以利诊断.

Abstract：

Objective To investigate the clinical manifestations of cytomegalovirus (CMV) infection in patients with systemic lupus erythematosus (SLE). Methods Data of the consecutive cases of SLE complicated with active CMV infection including clinical manifestations, SLEDAI score, dosage of corticosteroid and immunosuppressants used for treatment,radiological and laboratory examinations were collected and analyzed.Results Among 2221 consecutive patients of SLE, 5.4%(121 cases) were diagnosed to be complicated with active CMV infection. Fever was the most common symptom, followed by serious liver function damage,respiratory symptoms,hematological abnormalities, myocarditis, and encephalopathy, accounted for 81%(98cases), 6.6%(8 cases), 4.1%(5 cases), 3.3%(4 cases), 0.8%(1 case), and 0.8%(1 case)respectively; in addition, 22 (18.2%) cases had no symptom. SLEDAI was higher than 15 in 47.1% cases, and 10-14 in 28.1% cases. 81% of patients were treated with corticosteroid, and 55.4% were treated with immunosuppressants. Ganciclovir was given once the diagnosis of active CMV infection was established. In most of the patients, active CMV infection had been controlled within 14-28 days, except 4 died and 6 gave up the therapy. Conclusion SLE with active CMV infection is common,especially in patients who are treated with corticosteroid and/or immunosuppressants. Clinical manifestations of SLE complicated with active CMV infection are generally nonspecific.In patients with unexplained fever,or liver damage,or lung disease,or active SLE patients who have no symptom but are refractory to the treatment, CMV infection should be suspected and the relevant laboratory tests should be ordered for early diagnosis and treatment.     

Less disease severity and favorable prognosis are associated with postmenopausal systemic lupus erythematosus patients

The aim of this study is to characterize the clinical manifestations of postmenopausal systemic lupus erythematosus (SLE) patients. Of the 699 SLE inpatients, 20 postmenopausal and 70 menstruous SLE patients were evaluated and compared for the clinical manifestations. The mean age of onset was 55.05 years (range from 42 to 66) with a peak of 50-60 in postmenopausal lupus patients. The average time from SLE onset to diagnosis was 2.18 years. Arthritis was the most frequent initial manifestation in the postmenopausal group. Other common clinical manifestations and laboratory abnormalities include lassitude, fever, alopecia, malar rash, cardiac impairment and weight loss, and elevated ESR, decreased C3, ANA >/= 1:80, hypergammaglobulinemia and anti-RNP antibody positive. Compared with menstruous lupus patients, postmenopausal patients were more likely to have weight loss (P < 0.01), myalgia and myasthenia (P < 0.01), and less likely to have malar rash (P < 0.05), renal involvement (P < 0.01), leukocytopenia (P < 0.05) and positive ANA (P < 0.01). Thus, less disease severity and favorable prognosis were associated with postmenopausal SLE patients. Misdiagnosis and missed diagnosis were easy to make with their non-specific symptoms with fewer features suggestive of diagnosis.     

<Emphasis Type="Italic">Listeria monocytogenes</Emphasis> infection in patients with systemic lupus erythematosus

Listeria monocytogenes infection (LMI) is a rare complication in systemic lupus erythematosus (SLE) patients, and it is associated with nonspecific clinical manifestations and is often mistaken with SLE flares. Several cases of LMI in SLE patients have been reported, with high mortality rates. This article describes five new cases of LMI in patient with SLE in a cohort of 174 patients (2.8%). All patients were women, with a mean age of 19.4 years (range, 5–29 years). Mean duration of SLE before clinical LMI was 2.8 years (range, 2–4 years). Recurrent infection was not evidenced. At the time of LMI, all patients had an inactive disease, receiving steroids and immunosuppressive treatment. Clinical picture of meningitis was present in two patients. All patients were treated with ampicillin, with resolution of clinical manifestations without sequels. In order to eliminate the intracellular forms of L. monocytogenes, trimethoprim–sulfamethoxazole was initiated, and an allergic skin reaction was presented in all but one patient. Our report highlights the unspecific clinical manifestations of LMI and these characteristics are initially challenging and may be interpreted as lupus flares. An accurate diagnosis and an early antibiotic treatment are essential to improve the outcome in these patients.     

系统性红斑狼疮相关性眼部损害

目的 了解系统性红斑狼疮(SLE)相关性眼部损害的发生率、表现形式以及与SLE疾病活动性、其他系统损害和自身抗体的相关性.方法 回顾性分析我科2008年1月至12月期间住院SLE患者152例,其中有眼部损害者34例为实验组,另选取其中无眼部损害的SLE 48例为对照组,详细记录患者主要临床症状、器官受累情况、眼部体征、外周血自身抗体以及SLE疾病活动指数(SLEDAI).结果 152例患者中34例出现眼部损害,发生率22.4%.其中女性31例,平均年龄35.6岁,平均SLEDAI评分(18±7)分.16例(47%)患者眼部损害出现在SLE病程1年以内.最常见的眼部表现是眼底病变23例(68%),其中13例视网膜病变(11例双眼,2例单眼),9例视网膜血管病变(7例双眼微血管病变,2例单眼视网膜中央动静脉血管栓塞),1例双眼脉络膜萎缩,其他眼部表现包括8例Schirmer's试验异常(6例双眼,2例单眼),1例视神经病变(单眼),4例视野缺损(2例双眼,2例单眼),1例巩膜炎(双眼),1例虹膜睫状体炎(单眼),1例青光眼(单眼),4例角膜炎(3例双眼,1例单眼),3例结膜炎(2例双眼,1例单眼),1例睑缘炎(双眼).实验组与对照组比较,前者皮疹发生率更高,其他系统损害、SLEDAI及自身抗体检测差异均无统计学意义.免疫抑制剂治疗后27例(79%)眼部症状或体征好转.结论 SLE患者眼部病变并不少见,其表现形式多种多样,重者视力下降甚至失明.SLE相关性眼部损害可出现在疾病早期,甚至先于疾病出现,免疫抑制剂治疗可能有效改善眼部症状.     

Clinical and serological spectrum of systemic lupus erythematosus in greek children

Summary In the present study 19 Greek Caucasian children with systemic lupus erythematosus (SLE), onset before the age of 16, were followed up for 1–12 years (mean 5.6 yrs.). Diagnosis was determined early in 14 patients and delayed by 2 to 6 years in 5. The clinical manifestations and laboratory findings did not differ significantly from those reported in adults with lupus. The major organ system involvement at onset and early course were skin and joints (80%) followed by kidneys (42%). During the course of the disease 26% of the children developed central nervous system (CNS) involvement. All the patients were treated with steroids and/or cytotoxic drugs in severe uncontrolled progressive disease. At the mean 5.6 years follow-up most patients were in remission on small doses of steroids; one patient still presents signs of active lupus nephritis and one patient died from sepsis. All the patients with CNS involvement recovered without permanent CNS residue.     

The prevalence, onset, and clinical significance of antiphospholipid antibodies prior to diagnosis of systemic lupus erythematosus

OBJECTIVE: To determine whether antiphospholipid antibodies (aPL) occur before the diagnosis of systemic lupus erythematosus (SLE) and before initial clotting events, and whether their presence early in the disease course influences clinical outcome. METHODS: Serum samples obtained from 130 lupus patients before and after SLE diagnosis were screened for IgG and IgM aPL using an anticardiolipin (aCL) enzyme-linked immunosorbent assay. Medical records of all patients were carefully reviewed for data on the time of onset of SLE features meeting clinical criteria and on disease manifestations. RESULTS: Twenty-four patients (18.5%) were positive for IgG and/or IgM aCL prior to SLE diagnosis. Anticardiolipin antibodies appeared from 7.6 years prior to SLE diagnosis to within the same month as SLE diagnosis, with a mean onset occurring 3.0 years before SLE diagnosis. Additionally, aCL presence early in the disease process seemed to predict a more severe clinical outcome; these patients eventually met an average of 6.1 of the 11 classification criteria for SLE, compared with 4.9 criteria for other patients (P < 0.001). The early aCL-positive population also had more frequent renal disease, central nervous system disease, thrombocytopenia, and clotting events. In this population, aCL preceded initial thrombotic events by a mean of 3.1 years. CONCLUSION: Anticardiolipin antibodies in SLE patients tend to precede initial clotting events by several years. Furthermore, the presence of early, prediagnosis aPL seems to herald a more varied, severe clinical course with earlier onset in patients with SLE.