Clinical aspects of systemic lupus erythematosus in childhood

Systemic lupus erythematosus (SLE) is an uncommon childhood illness that is characterized by the formation of autoantibodies and immune complexes, which mediate inflammatory responses in multiple organ systems. Children who develop SLE are frequently very ill at the time of presentation and need careful evaluation to determine which organ systems are involved and how severely, meticulous control of medications to suppress active disease, and close monitoring to avoid complications from both the disease and its treatment.     

小儿系统性红斑狼疮的特点

目的了解小儿系统性红斑狼疮(SLE)的特点。 方法对85例SLE儿的临床资料进行分析。 结果85例中起病年龄10a以上者66例(77．6％),男女比为16．1,12例(14．1％)家族中有结缔组织病史。最常见的表现是抗核抗体(ANA)阳性(91．8％)、血沉增快(90．6％)、肾脏受累(82．3％)、发热(82．3％)、低补体血症(81．2％)、蝶形红斑(69．4％)、关节症状(62．3％)及血液系统损害(62．3％)。18例(21．2％)起病时表现为单一系统损害。6例(7．0％)肾损害始终为唯一临床表现。经激素联合免疫抑制剂治疗,随访0．5～23a,临床缓解或病情波动者49例,无1例进入慢性肾功能衰竭,死亡9例,失访27例。 结论本病临床表现多样,肾损害发生率高,早期正规治疗疗效显著。     

Clinical systemic lupus erythematosus with dense deposit disease

A 12-year-old girl with signs and symptoms of lupus erythematosus and nephritis had, by renal biopsy, glomerular capillary loop deposits identical in location and composition to those described in children with the dense deposit lesion of membranoproliferative glomerulonephritis, Type II. Following of prednisone therapy, serologic and clinical abnormalities resolved. The patient has experienced excellent growth and no recurrence of disease while receiving alternate-day prednisone therapy. The dense deposit lesion has not previously reported in patients having signs and symptoms of lupus glomerulonephritis.     

儿童狼疮性脑病临床特征

目的探讨儿童狼疮性脑病(NPSLE)的临床特点、辅助检查及治疗。方法对11例NPSLE患儿的临床资料进行分析。结果11例NPSLE患儿均有不同程度的头痛症状,意识障碍及失语各1例,抽搐、记忆力减退和共济失调各3例,出现病理反射及脑膜刺激征各4例。血清学均呈狼疮活动表现。3例脑脊液常规无异常,寡克隆抗体明显升高。其中10例行头颅CT检查,异常9例;10例患儿行脑电图检查,正常1例,且与CT无交叉。经个体化综合治疗,11例患儿神经精神症状均有不同程度的改善。结论系统性红斑狼疮累及神经系统症状最常表现为头痛。目前NPSLE主要根据临床表现作出诊断,血清学、影像学及脑电图检查有助于狼疮性脑病的诊断。对NPSLE的治疗强调个体化的综合治疗。     

Clinical presentations and outcomes of Filipino juvenile systemic lupus erythematosus

Objective  

Juvenile Systemic Lupus Erythematosus (SLE) varies by location and ethnicity. This study describes the clinical, laboratory profile and outcome of juvenile SLE seen at Philippine General Hospital (PGH) from 2004-2008.     

Gold-induced systemic lupus erythematosus

After 10 months of treatment with sodiumaureo-thiomalate a 12 year old girl with severe exsudative polyarthritis developed pericarditis and high titers of antinuclear antibodies as well as antibodies to native double-stranded DNA. At that time the cumulative gold dose was 550 mg. After withdrawal of gold medication the clinical symptoms rapidly disappeared and did not recur after a follow-up period of now 5 years. The titers of autoantibodies decreased to normal levels within a year.     

儿童系统性红斑狼疮35例

目的　了解系统性红斑狼疮 (SLE)临床的特点。方法　对 35例儿童SLE的临床资料进行分析。结果　 35例中病程短于 1年者 2 7例 (77.1% ) ,均有 3个以上系统同时受累。肾脏损害 2 9例 (81.4 % ) ,病初肾脏受累率高达 6 0 .0 % (2 1例 ) ;其他常见表现为皮损、血液系统受累、发热、抗核抗体 (ANA)阳性、低补体血症等。病初误诊率高达 6 0 .0 % ,最易误诊为肾炎和肾病综合征等疾病。结论　儿童SLE具有起病急、肾脏最易受累、临床表现多样、早期易误诊的特点     

Prolidase deficiency and systemic lupus erythematosus

The case is reported of an infant with hyperammonaemia secondary to severe distal renal tubular acidosis. A clinical association between increased concentrations of ammonia in serum and renal tubular acidosis has not previously been described. In response to acidosis the infant's kidneys presumably increased ammonia synthesis but did not excrete ammonia, resulting in hyperammonaemia. The patient showed poor feeding, frequent vomiting, and failure to thrive, but did not have an inborn error of metabolism. This case report should alert doctors to consider renal tubular acidosis in the differential diagnosis of severely ill infants with metabolic acidosis and hyperammonaemia.     

维生素D与系统性红斑狼疮的研究进展

维生素D(vitamin D,VitD)是一种类固醇激素,通过与VitD受体(vitamin D receptor,VDR)结合调节机体钙磷代谢,并可调节多种免疫细胞的增殖、分化及功能,调节免疫系统.系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种自身免疫性疾病,以自身抗体形成及多器官、多脏器受累为特点.VitD及VDR可通过抑制自身反应性淋巴细胞活化,抑制自身抗体产生等在SLE发病及发病机制中发挥重要作用,补充VitD可改善SLE患者预后.     

Anemia in systemic lupus erythematosus

Anemia is a common finding in children with systemic lupus erythematosus (SLE). Anemia in SLE may result from several mechanisms and more than one may be operative at any time. Anemia of chronic disease is the most common, but a low hemoglobin can be caused by auto-antibodies to red cells as part of the auto-immunity, or it may be the result of impaired erythropoietin production by kidneys involved in the SLE, gastrointestinal blood loss from anti-inflammatory therapy, increased red cell destruction from hypersplenism or a drug-induced immune phenomenon. We briefly review the important processes that can lead to a low hemoglobin in children with SLE, their clinical features and their treatment.     

Childhood-onset systemic lupus erythematosus]

Systemic Lupus Erythematosus (SLE) remains a challenging autoimmune disease in term of etiology, pathogenesis and treatment. It is estimated that 10-17% of lupus patients present before the age of 16. SLE in children appears to have more severe organ involvement than in adults. The outcome of childhood SLE has improved during the last decade, but the morbidity remains high.     

Cerebral systemic lupus erythematosus.

Cerebral systemic lupus erythematosus is uncommon in childhood. Three further cases are presented to draw attention to the unusual and varied clinical manifestations of this condition.     

Choledochal cyst and systemic lupus erythematosus

A 16-year-old female with systemic lupus erythematosus was diagnosed with choledochal cyst. As high-dose steroids had been prescribed for CNS lupus, surgery was performed under steroid cover. Despite postoperative pneumonia and wound infection, she is well 2 years later. There is no similar report in the literature.     

系统性红斑狼疮的胃肠道损害

系统性红斑狼疮(SLE)是一种系统性自身免疫性疾病,可以累及多个系统.相对于其他受累的系统,胃肠道受累并未受到重视.SLE的胃肠道损害因临床表现多种多样,且缺乏特异性,易与药物的胃肠道不良反应相混淆,而不能得到准确而及时的诊断.但有些SLE相关的胃肠道受累如未得到恰当的治疗可能危及生命.现对SLE常见的胃肠道损害,包括食管及胃受累、狼疮性肠系膜血管炎、狼疮性胰腺炎、狼疮性腹膜炎、假性肠梗阻、蛋白丢失性肠病、肝脾受累的临床表现,诊断和治疗进行阐述.以帮助临床医师更好地认识SLE的胃肠道损害,提高对该类疾病的重视程度,以期得到准确、及时的诊断和治疗.     

Lysinuric protein intolerance and systemic lupus erythematosus

We report lysinuric protein intolerance (LPI) and systemic lupus erythematosus (SLE) in a 14-year-old girl. The co-existence of LPI and SLE in the same patient has been reported before and suggests that SLE is directly connected with LPI. Conclusion Possibly in LPI the metabolic derangement can cause immunological abnormalities. Therefore, when multisystem involvement is observed in LPI patients, SLE should be considered as a rare complication. Received: 12 May 1997 / Accepted in revised form 31 July 1997