Background: Systemic lupus erythematosus (SLE) is an unknown cause chronic autoimmune disorder which characterized by the spread of the lesion in many organs of body. Childhood-onset systemic lupus erythematosus is often more acute and severe than adult-onset systemic lupus erythematosus. Objective: 1. To survey clinical, laboratory and immunological symptoms of childhood-onset SLE. 2. To survey immune disorders of childhood-onset SLE. Subjects and method: A retrospective study on clinical, laboratory and immunological indexes was carried out in 29 patients with childhood-onset SLE admitted in the Department of Allergy and Clinical Immunity, Bach Mai hospital, from June/2001 to June/2006.Results: The female to male ratio was 13.5:1, and the mean age at disease onset was 12.3\xb12.4 years. The most common initial manifestations were arthritis (62.1%), malar or butterfly rash (41.4%), fatigue, malaise, weight loss (41.4%) and fever (38%). The most common manifestations in the advanced stage of the disease were arthritis (82.8%), alopecia (62.1%), nephritis (89.7), anemia (72.4%), leucopenia (58.6%), thrombocytopenia (51.7%), neurological disorders (58.6%), fever (55.2%) and malar rash (48.3%). 93.1% of the patients had elevated erythrocyte sedimentation rate. The rates of patients with ANA, anti ds-DNA antibody and LE cells were 88%, 64%, and 10.5%, respectively. Conclusion: The author recommended that on the suspicious cases, necessary tests for detecting childhood onset SLE should be conducted soon for differential diagnosis. The SLE with onset in childhood, often more severe than later life must be aggressively treated.
Lupus Erythematosus ; Systemic/ epidemiology ; pathology ; Child

Atherosclerosis ; etiology ; therapy ; Humans ; Lupus Erythematosus, Systemic ; pathology ; therapy

The new revised classification of glomerulonephritis in systemic lupus erythematosus under the auspice of the International Society of Nephrology and the Renal Pathology Society (ISN/ RPS) was proposed in 2003. The revised classification preserves the simplicity of the original WHO classification, incorporates selective refinements concerning activity and chronicity from the 1982 and 1995 revisions, and adds a number of new modifications. Overall, it bears a strong similarity to the 1974 classification, but introduces several important modifications concerning quantitative and qualitative differences between class III and IV lesions. The new classification provides a clear and unequivocal description of the various lesions and classes of lupus nephritis as well as definitions for diagnostic terms. This review is introduced the ISN/RPS 2003 classification which will facilitates accurate communication between pathologists and clinicians.
Biopsy ; Classification* ; Glomerulonephritis ; Kidney ; Lupus Erythematosus, Systemic ; Lupus Nephritis* ; Nephrology ; Pathology*

OBJECTIVETo study feasibility of Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2000) and British Isles Lupus Assessment Group 2004 (BILAG-2004) scoring systems for assessing renal disease activity in children with lupus nephritis (LN).

METHODSThe clinical data of 159 children with systemic lupus erythematosus (SLE) and LN were collected, and disease activity was assessed by SLEDAI-2000 and BILAG-2004 scoring systems. The correlations between SLEDAI-2000 and BILAG-2004 scores and 24-hour urinary protein excretion and renal pathology index were analyzed. The SLEDAI-2000 and BILAG-2004 scoring systems were evaluated using ROC curve.

RESULTSApproximately one third (31.5%) of the 159 children had a moderate level of 24-hour urinary protein excretion. Among the 37 patients undergoing renal biopsy, 46.0% had diffuse LN (type Ⅳ). 24-hour urinary protein excretion was positively correlated with both SLEDAI-2000 (r=0.36, P<0.05) and BILAG-2004 scores (r= 0.39, P<0.05). Children with types Ⅰ, Ⅱ, Ⅲ, and Ⅳ LN had pathology activity index (AI) which positively correlated with SLEDAI-2000 scores (r=0.86, 0.88, 0.84, 0.77 respectively; P<0.05) and BILAG-2004 scores (r= 0.88, 0.98, 0.86, 0.89 respectively; P<0.05). SLEDAI-2000 score showed the best correlation with AI in patients with type Ⅱ LN, followed by those with type Ⅰ LN. BIILAG-2004 score showed the best correlation with AI in patients with type Ⅱ LN, followed by those with type Ⅳ LN. The BILAG-2004 scoring system had an area under the ROC curve (AUC) of 0.93, and the SLEDAI-2000 scoring system had an AUC of 0.88.

CONCLUSIONSBILAG-2004 and SLEDAI-2000 scoring systems can be used to assess renal disease activity of patients with LN. The BILAG-2004 scoring system can provide more reliable and comprehensive assessment.

Adolescent ; Child ; Feasibility Studies ; Female ; Humans ; Kidney ; pathology ; Lupus Erythematosus, Systemic ; pathology ; Lupus Nephritis ; pathology ; Male ; Proteinuria ; classification ; ROC Curve

We report a case of patient with documented SLE who displayed dysuria, gastrointestinal (GI) symptoms and renal insufficiency associated with the unusual occurrence of bilateral hydroureteronephrosis due to urterovesical junction stricture (obstructive uropathy). Pathologic investigations disclosed chronic interstitial cystitis (IC) with evidence of focal immune complex deposition in the blood vessel walls of the bladder. The GI symptoms and dysuria regressed with initial therapy for SLE with steroids. However, the persistent obstructive uropathy (OU) and renal insufficiency required bilateral nephrostomy followed by steroids plus intravenous pulse injection of cyclophosphamide. The obstructive uropathy was relieved even after removing the nephrostomy tube and renal function remained stable. Including this case, nineteen SLE patients associated with clinical and radiographic findings of OU were found in the world literature and reviewed to find any consistent pattern of clinical features. Most of the patients with OU in SLE were female (mean age, 31.7 yr) and orientals (63%), and had interstitial cystitis (89%) as a common underlying cause with concomitant involvement of the GI tract (89%) and WHO class IV or V advanced glomerulonephritis (67%). Despite the remarkable response (68%) to steroids in majority of OU patients associated with SLE, certain patients still required surgical correction (32%) and some even died (32%). OU, potentially reversible, was not an exception in patients with SLE, which might be overshadowed by other major organ involvement of SLE.
Adolescent ; Adult ; Female ; Human ; Lupus Erythematosus, Systemic/*complications/*pathology ; Urethral Obstruction/*complications/*pathology

Systemic lupus erythematosus (SLE) is an autoimmune disease caused by abnormal immune regulation and excessive production of autoantibodies, which characterized by T and B cell dysfunction and excessive production of pathological cytokines and autoantibodies. Vascular endothelia and subendothelial collagen were injured by harmful antibodies, so that the body was in a thrombophilic state, increasing the multi-system and multi-organ damage of body. Mesenchymal stem cells (MSC) are as multipotent cells, capable of multilineage differentiation, self-renewal, homing, inflammatory chemotaxis, immune regulation and reconstruction. To date, MSC are known to affect not only T cells, but also other cells of the immune system. MSC can inhibit or promote B cell proliferation, suppress NK cell activation and modulate the cytokine secretion profile of dendritic cells and macrophages. Thus decreasing the secretion of harmful cytokines and autoantibodies, can ease the thrombosis-prone state of the body, reducing the incidence of thrombosis. In addition, MSC are able to differentiate into various types of tissue cells, such as hematopoietic cells, endothelial cells, liver cells, nerve cells, bone cells, cartilage cells etc, therefore, MSC can repair the damaged tissues and organs. In this article, the advance of studies on immune regulation and repair mechanisms of MSC on incident thrombosis in SLE is reviewed.
Humans ; Lupus Erythematosus, Systemic ; immunology ; pathology ; Mesenchymal Stromal Cells ; immunology ; Thrombosis ; immunology ; pathology

Adolescent ; Heart Valve Diseases ; etiology ; Heart Valves ; pathology ; Humans ; Lupus Erythematosus, Systemic ; complications ; pathology ; Male

Adolescent ; Child ; Female ; Humans ; Kidney ; pathology ; Lupus Erythematosus, Systemic ; complications ; pathology ; Male ; Sex Characteristics

Lupus is a systemic autoimmune disease of an unknown origin, and systemic lupus erythematosus (SLE) can be triggered by numerous stimuli. Bee venom therapy is an alternative therapy that is believed to be effective for various kinds of arthritis. We present here a case of a 49-year-old female who experienced a new onset lupus after undergoing bee venom therapy, and this looked like a case of angioedema. The patient was successfully treated with high dose steroids and antimalarial drugs. We discuss the possibility of bee venom contributing to the development of SLE, and we suggest that such treatment should be avoided in patients with lupus.
Bee Venoms/*adverse effects ; Female ; Humans ; Lupus Erythematosus, Systemic/*etiology/pathology ; Middle Aged

A 16-year-old female patient with systemic lupus erythematosus (SLE) presented with abdominal pain and oliguria, after one month of hospital treatment with methylprednisolone and cyclophosphamide. Computed tomography of the abdomen revealed the calcification of bilateral superior segmental ureteral wall. Computed tomography urography revealed stenosis of bilateral ureters. This calcification may be associated with lupus-induced inflammatory reaction of ureteral endomembrane. By receiving the ureteral catheters, her abdominal pain and oliguria had been completely resolved. After discharge, she underwent the surgery of cutaneous ureterostomy in the local hospital. During two years follow-up, her condition is stable without any symptom of the urinary system.
Adolescent ; Calcinosis ; diagnosis ; etiology ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; Ureter ; pathology