Diversity in presenting manifestations of systemic lupus erythematosus in children.

OBJECTIVE: To describe the diversity in presenting manifestations of systemic lupus erythematosus (SLE) in children. STUDY DESIGN: Initial clinical and laboratory manifestations of 39 children, who fulfilled >/=4 American College of Rheumatology criteria for SLE, were retrospectively analyzed. RESULTS: Median age at onset was 12 years. The male to female ratio was 1:18.5, and racial/ethnic backgrounds were white 41%, black 33%, and Hispanic 26%. Initial manifestations included musculoskeletal 74%, cutaneous 72%, constitutional 67%, neurologic 28%, renal 28%, lymphadenopathy 15%, and Raynaud's phenomenon 10%. Laboratory abnormalities at presentation to our clinic included elevated erythrocyte sedimentation rate 87%, anemia 72%, lymphopenia 59%, leukopenia 31%, proteinuria or cellular casts 44%, low C(3) or C(4) level 77%, antinuclear antibodies 97%, and anti-double-stranded DNA 95%. One third (33%) presented with features not initially suggestive of SLE. Six patients presented with unusual manifestations including parotitis, quadriplegia, chorea, severe abdominal pain, persistent cough, and dizziness. However, 85% of patients with atypical manifestations had abnormal complete blood count or urinalysis results at presentation. CONCLUSION: Presenting manifestations of SLE in children are diverse. A detailed history, thorough review of systems, complete physical examination, complete blood count, urinalysis, and a high index of suspicion help to make the correct diagnosis of SLE in patients with atypical presentations.     

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。     

儿童系统性红斑狼疮临床特点与起病年龄相关性研究

目的 探讨儿童期起病系统性红斑狼疮(SLE)临床特征及预后与起病年龄的相关性.方法 回顾性分析2011年1月至2016年10月于重庆医科大学附属儿童医院风湿免疫科住院初诊为SLE患儿159例临床资料及预后,采用Spearman相关性分析评估其与起病年龄相关性.结果 159例SLE患儿平均起病年龄(11.05±2.73)...     

Systemic lupus erythematosus in Indian children

Twenty cases of systemic lupus erythematosus (SLE) in prepubertal children (less than 14 years of age) were seen over a period of 14 years. The male:female ratio was 1:2.3, and the mean age at onset was 9.37 years. Fever with joint involvement was the commonest presenting manifestation (60%), followed by nephrotic syndrome (25%). Notable clinical features included a high incidence of renal involvement (75%), significant hypertension (45%) and reversibility of acute renal failure (2 cases). The other organs and systems involved included: mucocutaneous manifestations (60%), cardiovascular system (30%), respiratory system (25%), neuropsychiatric manifestations (45%), and anemia (75%). Raynaud's phenomenon and thrombocytopenia were rare while leucopenia was not seen in a single patient. Immunological abnormalities noted were 100% positivity for antinuclear antibodies, and 87.5 and 75% positivity for antibodies to double-stranded and single-stranded DNA, respectively. Hypocomplementemia was seen in 75% of patients tested.     

Distinct phenotypes of multisystem inflammatory syndrome in children: a cohort study

To characterize the clinical features and outcomes of childhood-onset primary Sjögren’s syndrome (pSS). Patients less than 18 years old who were diagnosed with pSS by paediatric rheumatologists were included, and all patients were applied the 2002 American-European Consensus Group (ACEG) criteria, the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for pSS, or the 1999 proposed juvenile pSS criteria. The electronic medical records of patients with pSS from 2013 to 2020 were collected and analysed. Thirty-nine patients were included. Of them, 27 (69.2%), 38 (97.4%) and 35 (89.7%) patients fulfilled the AECG criteria, ACR/EULAR criteria and proposed juvenile pSS criteria, respectively. The female:male ratio was 3.9:1. The median ages at first signs or symptoms and at diagnosis were 9.2 (4.7, 14.5) years and 10.9 (6.3, 15.0) years, respectively. The main clinical manifestations were rash or purpura (20, 51.3%), followed by fever (12, 30.8%), glandular enlargement/recurrent parotitis (10, 25.6%), and dry mouth and/or dry eyes (9, 23.1%). Twenty-eight (56.4%) patients had systemic damage, the most common of which was haematological involvement (14, 35.9%), followed by hepatic (13, 33.3%) and renal involvement (8, 20.5%). Thirty-eight (97.4%) patients underwent labial minor salivary gland biopsy, and all exhibited focal lymphocytic sialadenitis. All patients had a global ESSDAI score ≥ 1 at diagnosis, and the median total score at diagnosis was 8 (2, 31). Thirty-six (92.3%) patients were followed up for a median time of 23.6 (7.9, 79.5) months, and three patients developed systemic lupus erythematosus (SLE) at follow-up times of 13.3, 38.8 and 63.8 months. The presentation of childhood-onset pSS is atypical, and extraglandular manifestations and systemic involvement are more common than in adult-onset pSS. Labial salivary gland biopsy is vital for patients with probable pSS. Some patients may develop SLE over time.     

Juvenile polyarteritis: results of a multicenter survey of 110 children

OBJECTIVE: To characterize pediatric patients who had been diagnosed with polyarteritis nodosa (PAN) through necrotizing vasculitis of the small and mid-size arteries or those with characteristic findings on angiograms data were collected. STUDY DESIGN: Pediatricians were asked to classify their patients into one of the four suggested groups for juvenile PAN. Twenty-one pediatric centers worldwide participated with 110 patients. RESULTS: The girl:boy ratio was 56:54, with a mean age of 9.05 +/- 3.57 years. The cases were classified as: 33 (30%) cutaneous PAN; 5 (4.6%) classic PAN associated with hepatitis B surface antigen (HBs Ag); 9 (8.1%) microscopic polyarteritis of adults associated with antineutrophil cytoplasmic antibodies (ANCA); and 63 (57.2%) systemic PAN. Cutaneous PAN was disease confined to the skin and musculoskeletal system. All patients with HBs Ag-associated classic PAN were diagnosed with renal angiograms. Antiviral treatment was administered in most cases. Microscopic PAN patients had pulmonary-renal disease, in combination or separately. ANCA was present in 87%, and 2 patients progressed to end-stage renal failure. Patients classified with systemic PAN had multiple system involvement, almost all had constitutional symptoms, and all had elevated acute phase reactants. Corticosteroids and cyclophosphamide were the first choices of immunosuppressive treatment. The overall mortality was 1.1%. CONCLUSIONS: There were remarkable differences among pediatric patients with PAN, with different clinical manifestations and overall better survival and lower relapse rates when compared with adults.     

Current issues in pediatric lupus nephritis: role of revised histopathological classification

Childhood-onset systemic lupus erythematosus (SLE) has an unpredictable natural history with variable clinical manifestations. The prognosis of SLE is linked closely to renal involvement with lupus nephritis (LN), which is more severe in patients with childhood-onset compared with adult-onset disease. The histopathological classification of LN facilitates treatment decisions, protocols, and clinical research. After the World Health Organization and modified WHO classifications of LN from 1974 to 1995, the International Society of Nephrology and Renal Pathology Society Working Group revised the histopathological classification of LN. The reclassification was published in 2004 after their consensus conference held at Columbia University in New York in May 2002. The aims of the reclassification were to standardize definitions, emphasize clinically relevant lesions, and encourage uniform and reproducible reporting among centers. Although the revised classification is time-consuming, it is important for future international collaboration on multicenter trials of disease-modifying agents. The prognosis of SLE and LN is linked to the histopathology of the renal lesion, but the clinical manifestations of LN, including nephrotic syndrome and hypertension, cannot predict the degree of renal involvement. However, we are many years away from completely understanding the etiopathogenesis of LN and the predictive role of the revised histological classification for direction of patient management.     

Rituximab therapy for childhood-onset systemic lupus erythematosus

OBJECTIVE: To describe the safety and efficacy of rituximab in the treatment of childhood-onset systemic lupus erythematosus (SLE). STUDY DESIGN: We conducted a French multicenter retrospective study of childhood-onset SLE treated with rituximab. RESULTS: Eleven girls with severe SLE, including 8 girls with class IV or V lupus nephritis, 2 girls with severe autoimmune cytopenia, and 1 girl with antiprothrombin antibody with severe hemorrhage, were treated with rituximab. The mean age at onset of rituximab treatment was 13.9 years. Patients received 2 to 12 intravenous infusions of rituximab (350-450 mg/m2/infusion), with corticosteroids. Six patients also received different standard immunosuppressive agents, including Cyclophosphamide (2 patients). Remission was achieved in 6 of 8 patients with lupus nephritis and in the 2 patients with autoimmune cytopenia. Steroid therapy was tapered in 5 patients who responded to treatment, and low-dose prednisone treatment was maintained in 1 patient. The mean follow-up period was 13.2 months (range, 6-26 months), and remission lasted in all who patients who responded to treatment, except 1 patient who was successfully retreated with a second course of rituximab. Anti-double-stranded DNA antibody levels decreased in 6 of 11 patients, and anticardiolipin antibody levels decreased in 3 of 4 patients. Severe adverse events developed in 5 patients. Effective depletion of peripheral blood B cells was observed in 7 of 8 patients who were examined, and this paralleled the remission. CONCLUSION: Rituximab may be an effective co-therapy; however, further investigations are required because severe adverse events occurred in 45% of the patients in this study.     

Serum levels of vascular endothelial growth factor in children and adolescents with systemic lupus erythematosus

The angiogenic cytokine vascular endothelial growth factor (VEGF) may have a role in the pathogenesis of collagen diseases. We aimed to assess its serum levels in children and adolescents with systemic lupus erythematosus (SLE) and to elucidate its correlation with clinical features, laboratory parameters, and the overall disease activity. This study comprised 25 children and adolescents with SLE and 30 healthy controls. Disease activity was evaluated by SLE disease activity index (SLEDAI) score. Laboratory investigations included complete blood count, erythrocyte sedimentation rate (ESR), urine analysis, 24-h total urinary protein, assay of serum creatinine, ANA, anti-DNA, complement component C3, lupus anticoagulant, and VEGF. Serum levels of VEGF were significantly increased in SLE patients (579.5 +/- 184.7 pg/ml) when compared with controls (113.2 +/- 30.8 pg/ml) (p < 0.0001). VEGF serum levels were significantly increased in patients having renal involvement and neurologic symptoms than those who did not have them (p < 0.0001, p < 0.005, respectively). Serum levels of VEGF were higher in patients with antiphospholipid syndrome, vasculitis, and skin symptoms than those without, but the difference did not reach statistical significance. Meanwhile, they were similar in patients with and without arthritis (p > 0.05). VEGF serum levels were not correlated to age; inversely correlated to platelet count, serum C3 level; and positively correlated to ESR. SLEDAI score was positively correlated to VEGF serum level (r = 0.86, p < 0.0001). VEGF may be relevant to SLE pathogenesis. Its concentration seems to be a marker of SLE activity, which could help in disease monitoring and planning of treatment.     

Cutaneous vasculitis associated with Mycoplasma pneumoniae infection: case report and literature review

Mycoplasma pneumoniae is an important bacterial agent that causes pneumonia in pediatric patients; it can also affect other organs or systems. Extrapulmonary manifestations include neurological, cardiac, hematologic, renal, gastrointestinal, osteoarticular, cutaneous, and ocular involvement. This report presents a 7-year-old male affected with cutaneous and retinal vasculitis due to M pneumoniae infection without pulmonary detection. The available literature on cutaneous vasculitis and M pneumoniae infection is also reviewed.     

Differential manifestations of prepubescent, pubescent and postpubescent pediatric patients with systemic lupus erythematosus: A retrospective study of 96 Chinese children and adolescents

Background

Children represent 10-20% of all systemic lupus erythematosus (SLE) patients. Their clinical manifestations and outcomes vary with age. We aim to clarify the relationship between pubescent status and the clinical manifestations of pediatric SLE.

Methods

In this study, pediatric SLE patients were divided into three groups, based on age at disease onset (?Q8, 8?C13 &; 13?C18?years), defined as prepubescent, pubescent and postpubescent, respectively. Initial clinical manifestations and laboratory characteristics at diagnosis were analyzed.

Results

Ninety-six patients were entered into the study: 8 had disease onset before age 8, while 49 were between 8?C13 and 39 of them were 13?C18. Female predominance was noted in all three groups (2.5-7.0:1). Postpubescents showed significantly more renal involvement and lymphopenia, along with lower levels of C3 and C4, when compared with prepubescents. They also showed significantly more lymphopenia when compared with pubescents. Pubescents showed significantly more renal involvement, leukopenia and lupus anticoagulant (LAC) positivity, along with lower C3 and C4 levels, when compared with prepubescents. Pubescents also showed significantly higher anti-Sm antibody positivity when compared with postpubescents. Prepubescents showed significantly more splenomegaly and anti-Jo-1 antibody positivity when compared with those of pubescents. The results showed that the disease activity (SLEDAI-2K score) correlated positively with age at disease onset and negatively with disease duration before diagnosis (p?=?0.011).

Conclusions

Age at disease onset is related to initial manifestations in pediatric SLE patients at our center. Certain parameters such as renal involvement, splenomegaly, low C3 level, low C4 level, lymphopenia, leukopenia, and anti-Sm &; anti-Jo-1 antibody were found to be significantly different among the age groups. Renal involvement might be the key symptom that varies with age.     

Post-streptococcal reactive arthritis in children: a distinct entity from acute rheumatic fever

Inflammatory myositis is reported in 4-16% of adult systemic lupus erythematosus (SLE) patients. The aim of this study was to determine the prevalence of myositis in a cohort of pediatric SLE patients in the southeastern United States. A retrospective chart review was performed of 55 SLE patients evaluated by Pediatric Rheumatologists in Alabama since January 1, 2008. Patients were defined as having myositis if they satisfied one of the following categories: 1) Proximal muscle weakness on exam with lower extremity muscle edema on MRI; 2) Proximal muscle weakness with elevation in CK, AST, aldolase, or LDH muscle enzymes; or 3) Patient reported weakness or muscle pain and an elevated CK. Inflammatory myositis was present as a feature of SLE in 31% (n = 17) with a 95% confidence interval of 19-45%, statistically different from the reported rates of 4-16% (p < 0.0001). Myositis was positively associated with the presence of anti-ribonucleoprotein antibodies (p = 0.009). Negative associations with myositis were the presence of anti-double stranded DNA antibodies (p = 0.02) and hematologic disorders (p = 0.02). Thus, in the state of Alabama, pediatric SLE myositis is present at a statistically higher rate than previously published values of adult SLE myositis, possibly reflecting geographic (genetic or environmental) and/or age-of-onset related influence(s).     

Kikuchi-Fujimoto disease with prolonged fever in children

We reviewed 12 patients who had Kikuchi-Fujimoto disease (KFD) and presented with prolonged fever and lymphadenopathy. The clinical and laboratory aspects of the patients confirmed by excisional lymph node biopsy were analyzed. The mean age of the children was 11.0 +/- 3.0 years (range: 6-15 years). The male-to-female ratio was 1.4:1. The median duration of fever before admission and the total duration of fever was 13 days (range: 7-65 days) and 19.5 days (range: 9-75 days), respectively. One patient had supraclavicular lymphadenopathy, 10 had cervical involvement, and 1 had axillary lymphadenopathy. All of the histologic findings of the lymph node biopsies showed the characteristic findings consistent with KFD, such as paracortical necrosis with karyorrhexis and an increase in the number of phagocytic histiocytes and atypical lymphocytes. As for the laboratory findings, leukopenia (3600 +/- 900 per mm3), anemia (hemoglobin 11.4 +/- 1.2 g/dL), an elevated erythrocyte sedimentation rate (44 +/- 18 mm/hour), and a relatively low C-reactive protein level (1.3 +/- 1.1 mg/dL) were noted. Eight patients received conservative therapy with antipyretics, and 3 patients were treated with prednisolone. KFD is a rare disease yet should be considered in the differential diagnosis for older children with prolonged fever and lymphadenopathy.     

Gastrointestinal manifestations of hemolytic uremic syndrome: recognition of pancreatitis.

A retrospective study of 76 children with hemolytic uremic syndrome (HUS) who were admitted to the Alberta Children's Hospital in Calgary. Alberta between January 1982 and December 1988 was undertaken to explore the gastrointestinal manifestations of the syndrome. The children (mean age of 4.0 +/- 3.1 years) presented primarily during the summer months with a microangiopathic hemolytic anemia (Hgb 94 +/- 26 g/L), thrombocytopenia (platelets 87 +/- 83 X 10(9)/L), and acute renal failure (oligoanuria with a BUN of 26 +/- 15 mmol/L, and a creatinine of 294 +/- 90 mumol/L). Forty-three children required dialysis for 10 +/- 17 days. The duration of hospitalization was 17 +/- 17 days. Four children died of complications attributable to HUS. The following symptoms and gastrointestinal manifestations of HUS were noted: fever (33%), vomiting (80%), abdominal discomfort/tenderness (59%), diarrhea (100%), hemorrhagic colitis (79%), rectal prolapse (13%), colonic stricture (3%), colonic perforation (1%), intussusception (1%), indirect hyperbilirubinemia (49%), and elevated hepatocellular enzymes (58%). Of the last 29 children studied, 19 (66%) had elevated levels of amylase and lipase in the presence of acute renal failure, and six (21%) had a marked elevation of lipase (more than four times normal) with additional supportive evidence of pancreatitis. The additional supportive evidence included persistent elevation of lipase after the resolution of acute renal failure in four children, a marked increment in lipase in association with abdominal pain and an abnormal ultrasound of the pancreas after the initiation of oral feeding in a fifth child, and pancreatic exocrine and endocrine necrosis at autopsy in a sixth child.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cutaneous manifestations of neonatal lupus without heart block: characteristics of mothers and children enrolled in a national registry

OBJECTIVE: To extend the information base on cutaneous manifestations of neonatal lupus erythematosus (NLE) with regard to maternal disease, sex of child, onset, localization, influence of UV light, prognosis, and recurrence rates in subsequent pregnancies. METHODS: Review of records from the Research Registry for Neonatal Lupus. RESULTS: The cohort includes 47 mothers (83% white) whose sera contain anti-SSA/Ro, anti-SSB/La, and/or anti-U1-ribonucleoprotein antibodies and their 57 infants (20 boys and 37 girls) diagnosed with cutaneous NLE (absent heart disease) between 1981 and 1997. At detection of the child's rash, 13 mothers were asymptomatic, 11 had an undifferentiated autoimmune syndrome (UAS), 9 had systemic lupus erythematosus (SLE), 7 Sj?gren's syndrome (SS), 6 SLE/SS, and 1 rheumatoid arthritis/SS; 20 reported photosensitivity. Within 5 years, 7 asymptomatic mothers experienced disease progression: 1 developed photosensitivity, 2 SLE, 3 SS, 1 SLE/SS; in 2 mothers UAS progressed to SLE; and 2 mothers with SS developed SLE. The infant's rash often followed UV light exposure; mean age at detection was 6 weeks, and mean duration was 17 weeks. All had facial involvement (periorbital region most common) followed by the scalp, trunk, extremities, neck, and intertriginous areas. In 37, the rash resolved without sequelae, 43% of which were untreated. A quarter had residual sequelae that included telangiectasia and dyspigmentation. One child developed Hashimoto's thyroiditis, and 2 developed systemic-onset juvenile rheumatoid arthritis. Of 20 subsequent births, 7 children were healthy, 2 had congenital heart block (CHB) only, 4 CHB and skin rash, and 7 skin rash only. CONCLUSIONS: Future pregnancies should be monitored by serial echocardiograms, given the substantial risk for heart block. Affected children should be observed for later development of a rheumatic disease.     

Assessment of myocardial involvement using cardiac troponin-I and echocardiography in rheumatic carditis in Izmir, Turkey.

BACKGROUND: Acute rheumatic carditis is still a major problem in developing countries. Cardiac troponin-I (cTnI) has been identified as a sensitive and specific marker in the diagnosis of myocarditis in children and adults. METHODS: A prospective study was performed using Doppler echocardiography and cTnI in order to detect myocardial involvement in 26 consecutive patients with acute rheumatic valvular disease. Patients were divided into two groups: group 1, rheumatic fever with carditis (n > 16); group 2, rheumatic fever without carditis (n > 10). RESULTS: Clinically age, gender, body temperature, heart rate and white blood count did not differ significantly between the groups and the age-matched control group. C-reactive protein, erythrocyte sedimentation rate, anti-streptolysin-O were significantly different. Left ventricular fractional shortening was normal in all patients (group 1, 37 +/- 10%; group 2, 34 +/- 5%; NS). Left ventricular dimensions were larger in group 1, in which all patients except two had moderate to severe mitral and/or aortic valvular regurgitation (5.05 +/- 0.75 cm/m(2)) compared to group 2, in which none had valvular regurgitation (3.27 +/- 0.26 cm/m(2), P < 0.05). None of the patients in either group presented with or developed pericarditis. Mean cTnI was 0.12 +/- 0.034 ng/mL in group 2 and 0.077 +/- 0.02 in group 1, the difference of which was not statistically significant. Neither significant cTnI elevations nor echocardiographic systolic function abnormalities were found in the present patients with rheumatic carditis. CONCLUSIONS: The present results indicate the absence of myocardial involvement in acute rheumatic carditis without congestive heart failure.     

儿童系统性红斑狼疮合并肺高压15例临床分析

目的探讨系统性红斑狼疮(SLE)合并肺高压(PH)患儿的临床和实验室检查特点、诊断治疗及预后。方法选取住院诊断SLE合并PH(SLE-PH)的儿童患者15例,对其临床症状、实验室检查、超声心动图特点、SLE病情活动指标和治疗转归等进行回顾性分析。结果 15例SLE-PH患儿中,从SLE确诊到PH诊断的间隔时间中位数为0.1年(范围:0~6.5年)。除PH相关症状外,40%的患儿合并雷诺现象。反映SLE疾病活动度的指标(如补体C3、C4、ESR水平及抗ds DNA阳性率)在PH轻-中度组与重度组间比较差异无统计学意义。13例患儿接受糖皮质激素及免疫抑制剂治疗,2例同时接受PH靶向药物等治疗。诊断PH后,中位随访时间8.0年(范围:0.5~18.1年),期间2例患儿死亡,其心功能为Ⅲ~Ⅳ级;余13例病情平稳。结论雷诺现象是SLE-PH患儿常见的临床表现。PH轻重程度与SLE疾病活动度无明显关联,应重视SLE患儿肺动脉压筛查。早期诊断、早期治疗有利于改善患儿预后。     

炎症小体病35例临床特征分析

目的:总结炎症小体病临床特点,提高儿科临床医生对该类疾病的认识,帮助早期诊断。方法:回顾性总结2008年1月1日至2020年12月31日由北京协和医院儿科诊断的35例炎症小体病患儿的发热、皮疹、受累系统情况以及实验室检查结果等临床特征。结果:35例炎症小体病患儿中男20例、女15例,起病年龄为1（0,7）岁,诊断年龄为...     

Clinical features of Japanese children and adolescents with systemic lupus erythematosus: Results of 1980–1994 survey

Marked advances have been made in the past decade in the management of adults with systemic lupus erythematosus (SLE). Therefore, a nationwide retrospective survey was conducted between 1980 and 1994 to investigate the clinical manifestations of SLE in Japanese children and adolescents. Questionnaires were sent to 340 hospitals. Of 405 patients reported by 176 hospitals, 373 patients, diagnosed by the criteria established by the Pediatric Study Group of the Japanese Ministry of Health and Welfare in 1985, were enrolled in the study. Forty-nine of the 354 patients (13.8%) had relatives with a connective tissue disease within the third degree of consanguinity. The frequent manifestations in 373 patients were the presence of antinuclear antibody (98.9%), immunologic disorders (93.0%), hypocomplementemia (87.1%), malar rash (79.6%) and fever (74.0%). Lupus nephritis was present in 148 of the 309 patients (47.9%) at their first visit to a clinic, and 261 of the 373 patients (70.0%) developed renal involvement during the observation period. Of 370 patients, 92 patients (24.9%) exhibited central nervous system lupus. Of 368 patients, 192 patients (52.2%) were treated by methylprednisolone pulse therapy and 148 patients (40.2%) received immunosuppressants in combination with steroid therapy at some stage during the observation period. Survival rate at 5 years from onset was 95.9%. Management of infection, coagulopathies, and central nervous system involvement is essential to improve the prognosis of SLE in Japanese children and adolescents.     

Neuropsychiatric involvement in juvenile systemic lupus erythematosus

Neuropsychiatric involvement is an important cause of morbidity and mortality in systemic lupus erythematosus (SLE) and it has been reported to occur in 22-95% of the childhood SLE patients. The aim of this study was to evaluate the neuropsychiatric involvement in our juvenile SLE patients. This was a cross-sectional assessment of patients to investigate the relationship between the involvement of the nervous system and the clinical factors, including autoantibodies, renal involvement and disease activity. We used Symptom Checklist-90-R (SCL-90-R), designed to measure the psychopathological symptoms. As controls, we used 20 healthy adolescents and 20 patients with chronic diseases without any neuropsychiatric manifestations. Overall, 55% (n= 11) of the patients displayed neurological symptoms and/or signs. However, central nervous system (CNS) imaging showed pathological findings only in four of these patients. Patients with headache only had normal CNS imaging. Nine patients had moderate to severe depression. When SLE patients were compared to healthy controls and to adolescents with chronic diseases, they were found to be significantly more depressed. In conclusion, pediatric rheumatologists should be aware of the frequency of neuropsychiatric disturbances in SLE. The neuropsychiatric disorders do not always correlate with disease activity and these children need professional psychological evaluation.