Absence of <Emphasis Type="Italic">Chlamydia pneumoniae</Emphasis> and Signs of Atherosclerotic Cardiovascular Disease in Adolescents with Systemic Lupus Erythematosus

Patients with systemic lupus erythematosus (SLE) have accelerated atherogenesis. A recent study suggested that Chlamydia pneumoniae infection might also be a contributing factor in the development of atherogenesis in patients with SLE. The objective of this study was to investigate the possible association of C. pneumoniae infection with markers of atherosclerosis in adolescents with SLE compared with age-matched healthy controls. History and exam focused on cardiovascular risk factors were obtained from 20 patients with SLE and 20 age- and sex-matched controls. Laboratory studies included serum lipid profile and high-sensitivity C-reactive protein (hsCRP). Detection of C. pneumoniae in peripheral blood mononuclear cells (PBMCs) and in nasopharyngeal swab specimens was performed. Carotid Intima-Media Thickness (CIMT) was determined by sonography in all subjects. C. pneumoniae DNA was not detected in PBMCs of any of the patients or controls. Nasopharyngeal cultures were also negative for C. pneumoniae in all patients. CIMT was slightly higher in the SLE group (0.48 ± 0.049) compared with controls (0.454 ± 0.041, p = 0.29). There was no significant difference between the two groups in body mass index, blood pressure, hsCRP, and serum cholesterol (total, LDL and HDL). Serum triglycerides were higher in the lupus group (p = 0.03). Children and adolescents with SLE might have accelerated atherosclerosis; however, we did not observe an association with C. pneumoniae infection in this population.     

Systemic lupus erythematosus of childhood onset: correlation between T cells expressing early and late activation antigens and disease activity

Correlation between T cell phenotypes, especially activated T cells expressing early (EA1) and late (HLA-DR) activation antigens and clinical features were investigated in 22 patients with systemic lupus erythematosus (SLE) of childhood onset. Percentages of T cells expressing EA1 and HLA-DR in 22 patients with SLE were significantly higher than those in controls. Comparison of T cell phenotypes between patients with active and inactive SLE showed that eight patients with active disease had significantly increased percentages of HLA-DR positive T cells than 14 with inactive disease (P<0.01). Serial examinations showed that the percentages of HLA-DR positive T cells were decreased after therapy in seven with active non-renal or active non-renal and renal diseases but not in one with only active renal disease. A possible significance of T cells expressing EA1 and HLA-DR in the management of patients with SLE is discussed.Abbreviations HLA-DR human leukocyte antigens-DR - SLE systemic lupus erythematosus     

Gonarthritis due to Salmonella enteritidis in a patient with systemic lupus erythematosus

Extraintestinal manifestations of Salmonella infection occur more frequently in immunocompromised patients than in healthy persons. About 25 % present as septic arthritis. Particularly patients with SLE are predisposed. We report a case of a 16-year old girl with systemic lupus erythematosus who developed septic arthritis of the left knee. Delayed diagnosis because of similar symptoms of arthritis due to lupus and purulent arthritis led to a destruction of the joint despite systemic antibiotic treatment. Seven months later an endoprosthesis was implanted with good outcome. Early diagnosis, adequate antibiotic therapy and, if necessary, surgical intervention are essential for successful treatment. In patients with SLE suffering from fever or arthritis it is necessary to think of infections particularly due to salmonella.     

狼疮性呼吸系统危象表现

系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种多发于女性、累及多脏器的自身免疫性结缔组织病,SLE合并呼吸系统病变在儿童起病急、病情重,有潜在的致命性。因此,要认识其复杂性和严重性,特别要注意狼疮性呼吸系统危象表现的发生。狼疮性呼吸系统危象主要指的是:①急性狼疮性肺炎,②狼疮性肺出血。两者发病率虽较低,但病死率高,如能尽早结合临床症状和体征、胸部X线、肺功能与病理改变及时明确诊断,同时给予尽快尽早使用大剂量皮质激素及免疫抑制剂的治疗,可明显提高近期生存率,改善预后。     

Acute Raoultella planticola cystitis in a child with rhabdomyosarcoma of the bladder neck

Raoultella planticola is a Gram‐negative, non‐motile, aerobic bacillus. It is an environmental bacteria found in soil and water, and a very rare cause of local or systemic infection in humans. Although some adult cases of R. planticola infection have been reported, childhood local or systemic infection caused by R. planticola is very rare. Reported herein is a rare case of acute cystitis due to R. planticola in a 16‐month‐old boy with rhabdomyosarcoma of the bladder neck, and a review of the literature.     

Lupus Anticoagulant Positivity in Pediatric Patients With Prolonged Activated Partial Thromboplastin Time: A Single-Center Experience and Review of Literature

The presence of lupus anticoagulants (LAs) is an important cause of activated partial thromboplastin time (aPTT) prolongation found in children after an infection or during screening tests before surgical intervention. The authors retrospectively reviewed the charts of 68 patients who have been consulted from surgery departments with prolonged aPTT. These patients were reevaluated with aPTT analysis after 1 week. Thirteen patients had normal aPTTs. Therefore, 55 patients remained with prolonged aPTTs. LA positivity was detected in 39 patients. Sixteen of these had prolonged aPTT prior to surgery (41%). Others with LA positivity had systemic lupus erythematosus (SLE; n = 6), infection (n = 5), leukemia (n = 3), hemolytic uremic syndrome (n = 2), epistaxis (n = 2), antiphospholipid syndrome (APS; n = 1), chronic immune thrombocytopenic purpura (n = 1), acute poststreptococcal glomerulonephritis (n = 1), central nervous system (CNS) thrombosis (n = 1), and congenital heart disease (n = 1). None of the patients had bleeding history. LA positivity rarely leads to bleeding and/or thrombosis. Specific therapy is usually not needed. Further prospective multicenter studies are required to understand clinical outcomes and laboratory correlation in children with positive LA.     

Pulsed methylprednisolone therapy compared to high dose prednisone in systemic lupus erythematosus nephritis

This study was done to determine whether intravenous methylprednisolone therapy given concomitantly with low-dose daily, oral prednisone would be as effective as highdose daily prednisone in the treatment of patients with active systemic lupus erythematosus (SLE) nephritis.Thirteen patients with active SLE nephritis were started on 2 mg/kg prednisone per day, considered the high prednisone phase. Therapy was continued until remission was achieved. Prednisone administration was then tapered to less than 0.5 but more than 0.2 mg/kg per day. On later relapse, these patients received three doses of methylprednisolone (20 mg/kg per dose) on alternate days and continued on the same daily dose of prednisone (<0.5 >0.2 mg/kg per day) prior to pulse therapy; this was the methylprednisolone phase. The 13 patients were studied in both phases, serving as their own controls.After 1 month of therapy, no significant differences were observed between treatment phases as to improvement in clinical and laboratory findings. A significant increase in the serum concentration of C₃ and C₄ was seen both in the highdose prednisone and methylprednisolone phases, while the serum concentration of anti-ds DNA antibody significantly decreased.Methylprednisolone therapy seems as effective as highdose prednisone in patients with relapse of SLE nephritis. Because side effects are minimal, methylprednisolone administration may be tried as the therapy of choice for these patients.Abbreviation SLE systemic lupus erythematosus     

ToRCH “co‐infections” are associated with increased risk of abortion in pregnant women

ToRCH infections (toxoplasmosis, rubella, cytomegalovirus and Herpes simplex virus) have long been known to be associated with bad obstetric outcomes. However, little information is available about the impact of ToRCH co‐infections on the outcome of pregnancy. Hence, we tested the IgG and IgM antibodies to Toxoplasma gondii, Rubella, Cytomegalovirus and Herpes Simplex Virus among 81 pregnant women with abortion (case group) and 98 pregnant women with normal delivery (control group). In the single‐infection model, only CMV‐IgM seropositivity was significantly increased in case than control group (25.9% in case and 12.2 % in control, OR = 2.5, P = 0.019). In the co‐infection model, 14 patterns were recognized, but two patterns were significantly increased in the case than the control group. Co‐infection of T. gondii IgG + CMV IgM was 9.1‐fold increased in the case than the control group (8.6% in the case and 1% in control, OR = 9.1; P = 0.024). Also, co‐infection of T. gondii IgG + HSV IgG + CMV IgM was 7.7‐fold increased in case than the control group (7.4% in case and 1 % in control, OR = 7.7; P = 0.04). Although the OR of other co‐infections was higher in the case than the control group, the difference was not statistically significant. These findings indicate that ToRCH co‐infections are associated with increased risk of abortion than single infection. Hence, the rates of co‐infections should be considered in prenatal screening of ToRCH infections.     

Sisters with α-mannosidosis and systemic lupus erythematosus

Alpha-mannosidosis is an autosomal recessive disorder caused by deficiency of lysosomal -mannosidase (LAMAN). Here, we report two sisters with -mannosidosis who developed systemic lupus erythematosus (SLE). The sisters were both homozygous for a one bp deletion within the LAMAN gene resulting in a truncated gene product. The coincidence of -mannosidosis and SLE are discussed with regard to both clinical and molecular findings. Conclusion:-mannnosidosis may contribute to the onset of systemic lupus erythematosus in predisposed patients.Abbreviations ACR American College of Rheumatology - LAMAN lysosomal -mannosidase - SLE systemic lupus erythematosus     

Lupusnephritis

Renal involvement is common in systemic lupus erythematosus (SLE). It can manifest just as proteinuria and/or haematuria, but various serious courses are possible. Detection of haematuria and/or proteinuria in a patient with SLE is an indication for diagnostic tests targeting kidney function. To confirm a diagnosis of nephritis induced by lupus and assess its severity (WHO classification) it is necessary to examine a kidney biopsy specimen. In lupus nephritis type 1 no special treatment is needed in view of the excellent prognosis as far as renal function is concerned. In WHO types 2 and 3A immediate therapy is also not necessary, but close monitoring is essential. In type 3B, in contrast, intensive immunosuppression and consistent blood pressure stabilisation are of importance. In type 4 a terminal kidney failure can rapidly supervene without adequate immunosuppressive therapy. Type 5 patients benefit from administration of an ACE inhibitor in addition. Type 6 is a residual condition in the form of scarring, and in these cases more intensive immunosuppression is pointless. Up to 20% of paediatric patients with lupus nephritis come to need dialysis, though kidney function can be at least partly restored.     

Myocardial infarct in a child with systemic lupus erythematosus

An unusual case of systemic lupus erythematosus (SLE) in a young child is reported with sudden death from myocardial infarction. The diagnosis of lupus erythematosus in this patient was made by renal biopsy at the age of 3 years. Atherosclerosis of the coronary arteries and aorta was found at autopsy with occlusion of the anterior descending branch of the left coronary artery. It is suggested that the vascular changes in this case were related to hypertriglyceridemia and prolonged prednisone therapy superimposed on a hypersensitivity vasculitis related to SLE.     

系统性红斑狼疮合并妊娠子代早产儿42例临床分析

目的 探讨系统性红斑狼疮（SLE）合并妊娠子代早产儿的临床特点,提高新生儿科医师对这部分早产儿的认识。方法 收集2000年1月至2012年6月间SLE合并妊娠子代早产儿的临床资料进行回顾性分析,并与同期出生的除SLE合并妊娠子代早产儿以外的2 308例早产儿住院期间并发症发生情况进行对比。结果 SLE合并妊娠子代早产儿共42例,女婴比例明显高于男婴,其中确诊新生儿狼疮综合征4例。新生儿感染为SLE合并妊娠子代早产儿最常见并发症（47.62%）,其次为小于胎龄儿（28.57%）,新生儿呼吸窘迫综合征（26.19%）,新生儿窒息和先天性心脏病（均为14.29%）,肺出血（4.76%）;与同期收治的2 308例早产儿合并新生儿感染（16.81%）、小于胎龄儿（13.21%）和先天性心脏病（5.16%）的发生率进行比较,差异均有统计学意义（均P<0.05）;其他合并症的发生率在两组早产儿间比较差异均无统计学意义。结论 SLE合并妊娠子代早产儿易合并感染性疾病、小于胎龄儿及先天性心脏病,而呼吸方面并发症与同期其他早产儿相比无显著差异。     

Neonatal Lupus erythematosus Following Rheumatoid Arthritis: Case Report and Literature Review

Background: Neonatal lupus erythematosus (NLE) is a transient autoimmune disease of developing fetus and neonate in mothers with systemic lupus erythematosus (SLE). In this report we introduce an infant with NLE whose mother had rheumatoid arthritis. Case Presentation: Our case was a 40 day old male infant with discoid-like and annular skin lesions over forehead and neck, irritability and low grade fever. There was a history of prematurity due to preeclampsia. There was no cytopenia or cardiac involvement but liver enzymes were more than 5-fold increased. FANA, Anti Ro and La were negative. The mother had a history of un-controlled rheumatoid arthritis for 12 years with deformity in metacarpal and PIP and ulnar deviation in hands. FANA=1/640 and anti-SSB/La was positive in the mother but there was no other clinical and paraclinical sign of SLE. Without any treatment and during months, the skin and mucosal lesions gradually disappeared without any scar and liver enzymes reached the normal level. After 6 months follow up, he was symptom free with normal growth and development. Conclusion: We recommend to check anti SSA/Ro and anti SSB/La antibodies in all pregnant women with connective tissue diseases to prevent life-threatening involvement of the infant.Key Words: Neonatal Lupus, Rheumatoid Arthritis, Anti SSA/Ro, Anti SSB/La     

Pediatric autoimmune myelofibrosis: Experience from a large pediatric tertiary care center

Autoimmune myelofibrosis (AIMF) is a rare disorder characterized by cytopenias and autoimmunity, with characteristic bone marrow findings that include lymphocytic infiltration and fibrosis. AIMF is described predominantly in adult populations who have systemic lupus erythematosis (SLE), with scant pediatric cases described mainly in older adolescents with SLE. Here, we described the largest single-center pediatric experience of pediatric autoimmune myelofibrosis (PAIMF) series, demonstrating both similarities and distinctions from the adult experience. Patients overall respond well to steroid therapy, but these patients were significantly younger, infrequently carried a diagnosis of SLE, and causative genetic lesions were identified in many cases.     

Klinefelter's syndrome (47,XXY) among men with systemic lupus erythematosus

Aim: To determine the rate of Klinefelter’s syndrome among men with systemic lupus erythematosus (SLE), and to determine whether the manifestations of SLE in these men are different from that seen in 46,XY men. Methods: A total of 276 men with SLE underwent a real‐time PCR procedure to screen for more than one X chromosome. Those with results consistent with two X chromosomes were further characterized by karyotype and FISH. Clinical manifestations of SLE were determined by interview, questionnaire and medical chart review. Each man with Klinefelter’s and SLE was matched to four 46,XY men with SLE. Rates of SLE manifestations were compared with chi‐square analyses. Results: We found seven of the 286 men with SLE had Klinefelter’s syndrome. Four of these seven were nonmosaic 47,XXY, while two were mosaic 46,XY/47,XXY and one was 46,XX/47,XXY. The men with 47,XXY did not have severe manifestations of SLE including no proliferative renal disease, neurological disease, thrombocytopenia, autoimmune haemolytic anaemia, discoid skin disease or anti‐RNP/Sm. Conclusion: 47,XXY is found in excess among men with SLE. Men commonly have SLE that is more severe than that found among women, but the 47,XXY men had less severe SLE than other men.     

Pediatric hypervirulent Klebsiella pneumoniae septic arthritis

Cases of infection with hypervirulent Klebsiella pneumoniae are gradually increasing in number, and cause life‐threatening community‐acquired infection even in immunocompetent patients. A 14‐year‐old boy developed septic hip arthritis due to hypervirulent K. pneumoniae (sequence type 23, serotype K1, magA positive). The patient initially seemed to have been successfully treated with antibiotics and surgical intervention, but septic arthritis developed into osteomyelitis of the femoral head and myositis, which required long‐term antibiotic therapy and additional surgical intervention. This is the first pediatric case of hypervirulent K. pneumoniae septic hip arthritis. Treatment plans should mainly consist of antibiotic therapy and surgical intervention. Clinicians, even pediatricians, in developed countries should be aware of the increasing incidence of hypervirulent Klebsiella pneumoniae infection.     

Nierenbeteiligung bei Systemerkrankungen

The most important systemic disorders involving the kidney are systemic lupus erythematosus (SLE) and systemic vasculitis. The kidney is a critical organ for the outcome of the diseases. Early diagnosis and adequate therapy may reverse or even heal glomerulonephritis and kidney vasculitis. This contribution is restricted to SLE, the ANCA-associated forms of vasculitis, and the classic Periarteriitis nodosa. Depending on the size of involved vessels, different forms of glomerulonephritis (small vessel disease) or necroses (medium-sized vessel disease) are noted. All diseases mentioned need aggressive immune suppressive therapy (induction with glucocorticoid steroids and cyclophosphamide). In SLE, this is indicated mainly for diffuse proliferative glomerulonephritis (type IV), very severe forms of focal-proliferative glomerulonephritis (type III), and membranous glomerulonephritis (type V). To avoid toxicity, cyclophosphamide should be substituted by azathioprine or mycophenolate mofetil as early as possible. This can be performed for ANCA-associated forms of vasculitis 3–6 months after the start of therapy as was shown in randomized studies, whereas such studies are missing for SLE.     

Post‐transplant immune complex nephritis in a patient with systemic lupus erythematosus associated with ANCA vasculitis

Nearly 20% of SLE corresponds to the pediatric population, and 75% of them have kidney involvement representing an important etiology of chronic kidney disease. A correlation between SLE and ANCA‐associated vasculitis has been identified as an overlapping syndrome. Kidney allograft recurrence is rare in SLE when disease control is achieved and with nowadays immunosuppression treatment. Histologic transformation is unusual, especially when there are negative serologic markers and no immune complex deposition reported in native kidneys. A 17‐year‐old female with crescentic glomerulonephritis, p‐ANCA‐positive antibodies with pauci‐immune pattern in kidney biopsy develops end‐stage renal disease requiring hemodialysis. Deceased donor kidney transplant was performed receiving triple immunosuppression thereafter. Thirteen months later serum creatinine rises without evidence of infection, urinary obstruction, or clinical and serologic disease relapse. Allograft biopsy reports mesangial proliferation and “full‐house” immunofluorescence. The role of ANCA in SLE physiopathology is controversial, and its relation with lupus nephritis is also discordant. ANCA could represent an important factor in the heterogeneity of systemic lupus erythematosus and lupus nephritis.     

早期诊断和监测系统性红斑狼疮患儿活动性人类巨细胞病毒感染的研究

目的：了解系统性红斑狼疮（systemic lupus erythematosus,SLE)患儿活动性人类巨细胞病毒（human cytomegalovirus,HCMV)感染的状况,并比较不同实验室检测方法的诊断价值,方法：实验组观察了２１例初诊为ＳＬＥ并接受免疫抑制治疗的患儿,对照组观察了２１例免疫力正常的骨科患儿,治疗前后应用间接免疫荧光法检测外周自多形核白细胞（polymorphonuclear leukocytes,PMNLs）中的ＨＣＭＶpp65抗原和p７２抗原,ＰＣＲ方法检测血清中的ＨＣＭＶＤＮＡ,ＥＬＩＳＡ方法检测血清中的ＨＣＭＶＩgM和ＩgG抗体,结果,实验组活动性ＨＣＭＶ感染发生率为２９％（６／２１）,４例发生于免疫抑制治疗后,另外２例发生免疫抑制治疗前,对照组无１例发生活动性感染,两组相比差异有显著性（Ｐ＝０．０２７）,实验组各项实验室检测的阳性率分别为:pp6524%(5/21),p7214%(3/21),DNA43%(9/21),IgM10%(2/21),IgG91%(19/21).实验组各项实验室检测方法诊断活动性ＨＣＭＶ感染的敏感性分别为：pp6583%(5/6),p7250%(3/6),NDA100%(6/6),IgM33%(2/6),IgG(双份血清抗体滴度呈≥４倍增高）５０％（３／６）,特异性ＰＣＲ方法为８０％（１２／１５）外,其余均为１００％（１５／１５）,结论：ＳＬＥ患儿活动性ＨＣＭＶ感染发生率较免疫力正常的儿童显著增高,免疫抑制治疗前后可发生,ＨＣＭＶpp65抗原血症检测是早期诊断和监测ＳＬＥ患儿活动性ＨＣＭＶ感染的较好指标。     

Ultrasound findings in children with toxocariasis: report on 18 cases

Purpose. To evaluate abdominal ultrasound (US) findings in children infected with Toxocara canis. Materials and methods. Eighteen children, 18 months to 7 years of age, with serological diagnosis of T. canis infection underwent abdominal US. Eosinophil counts, hemoglobin levels and immunoglobulin E titers were measured for all patients. Results. Abdominal ultrasound revealed multiple hypoechoic areas in the livers of 15 patients (83.3 %). Hepatohilar lymph-node enlargement was present in 14 patients (77.7 %), 2 of whom also showed peripancreatic lymph-node enlargement. Hepatomegaly was present in 13 patients (72.7 %) and splenomegaly in 9 (50 %). Conclusion. The most prevalent findings of abdominal ultrasound examination of children with T. canis infection are hepatic granulomas and abdominal lymph-node enlargement. This infection should be considered in the differential diagnosis of any children who exhibit these findings on abdominal US examination, especially for those with eosinophilia. Received: 10 November 1998 Accepted: 21 December 1998