We report a patient with systemic lupus erythematosus (SLE), who had developed metabolic alkalosis during plasmapheresis. The metabolic alkalosis could be promptly corrected by reducing the amount of citrate load. The development of metabolic alkalosis can be explained by the citrate load during plasmapheresis. Careful monitoring of acid base status is mandatory in patients with limited renal function and the reduction of citrate load may be advisable in plasmapheresis.
Adolescent ; Alkalosis/*etiology ; Citrates ; Citric Acid ; Female ; Humans ; Lupus Erythematosus, Systemic/*metabolism/therapy ; Plasmapheresis/*adverse effects/methods

Candidiasis ; drug therapy ; immunology ; metabolism ; Diabetes Mellitus, Type 1 ; complications ; drug therapy ; immunology ; therapy ; Humans ; Lupus Erythematosus, Systemic ; drug therapy ; immunology ; metabolism ; Mycoses ; complications ; immunology ; therapy ; Paraneoplastic Syndromes ; drug therapy ; immunology ; metabolism ; Pemphigus ; drug therapy ; immunology ; metabolism

OBJECTIVETo investigate the effect of Lang-chuang-ding Decoction (, LCD) on the expression of DNA methylation of CD70 gene promoter in peripheral blood mononuclear cells (PBMCs) of females with systemic lupus erythematosus (SLE).

METHODSPBMCs isolated from female patients with SLE or healthy donors were cultured and treated with LCD medicated serum or normal serum for 24 or 48 h. The mRNA expressions of CD70 gene in PBMCs were detected by reverse transcription polymerase chain reaction (PCR); the DNA methylation of the CD70 gene promoter region was detected by methylation-specific PCR.

RESULTSAfter treated with medicated serum for 48 h, the mRNA expression levels of CD70 in PBMCs of SLE patients were signifificantly higher than those of healthy donors (P<0.05); the DNA methylation levels of CD70 promoter region in PBMCs of SLE patients treated with medicated serum for 48 h were signifificantly higher than those treated with fetal bovine serum (P<0.01).

CONCLUSIONLCD could inhibit CD70 gene expression in PBMCs of SLE patients by promoting the DNA methylation of CD70 gene promoter.

Adult ; CD27 Ligand ; genetics ; metabolism ; DNA Methylation ; drug effects ; genetics ; Drugs, Chinese Herbal ; pharmacology ; Female ; Gene Expression Regulation ; drug effects ; Humans ; Leukocytes, Mononuclear ; drug effects ; metabolism ; Lupus Erythematosus, Systemic ; drug therapy ; genetics ; Promoter Regions, Genetic ; RNA, Messenger ; genetics ; metabolism

OBJECTIVETo observe the therapeutic effect of Yiqi Yangyin principle (YQYY, the treating principle in TCM to supplement Qi and nourish Yin) on corticosteroid withdrawal in patients with systemic lupus erythematosus (SLE) in remission stage and its influence on some immune parameters.

METHODSThe SLE patients were divided into two groups, 30 in the treated group and 10 in the control group, who were treated by conventional method with corticosteroids and/or immunosuppressant in acute progressive stage, and YQYY was added to the treated group in remission stage.

RESULTSThe total effective rate was 93.3% in the treated group and 90.0% in the control group, comparison between the two groups showed significant difference by Ridit test (P < 0.05). The immune parameters, IgG and C3 were significantly improved after treatment in the treated group (P < 0.01), but changed insignificantly in the control group. The maintaining dose of prednisone used in the two groups was 7.08 +/- 5.26 mg/d and 11.72 +/- 6.48 mg/d respectively, the amount used in the treated group was significantly lower than that in the control.

CONCLUSIONUsing mainly YQYY Principle to treat SLE in remission stage could withdraw the corticosteroid smoothly, relieve symptoms and improve immune function.

Adolescent ; Adult ; Child ; Complement C3 ; metabolism ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Humans ; Immunoglobulin G ; blood ; Lupus Erythematosus, Systemic ; drug therapy ; Male ; Middle Aged ; Phytotherapy ; Prednisone ; adverse effects ; therapeutic use ; Substance Withdrawal Syndrome ; drug therapy

OBJECTIVETo observe the therapeutic efficacy and safety of Haoqin Qingdan Decoction (HQD) in treating active stage of systemic lupus erythematosus (SLE).

METHODSOne hundred and twenty patients with SLE were randomly assigned into two groups, the treated group receiving HQD and prednisone, and the control group receiving prednisone and cyclophosphamide (CTX), both were treated for 3 months. SLE disease activity index (SLEDAI), various immunological indexes, blood and urine routine, hepatic and renal function, as well as the other adverse reaction of the treatment were observed.

RESULTSThe total effective rate in the treated group was 81.67% (49/60), while 76.67% (46/60) in the control group, showing insignificant difference between the two groups. SLEDAI, erythrocyte sedimentation rate (ESR), complement 3 (C3), 24-hr urinary protein excretion and anti-double strain deoxyribonucleic acid (dsDNA) antibody were improved in the treated group, and no obvious adverse reaction was shown.

CONCLUSIONHQD is obviously more effective in treating active stage of SLE with high safety.

Adolescent ; Adult ; Aged ; Complement C3 ; metabolism ; Drug Administration Schedule ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; administration & dosage ; Female ; Humans ; Lupus Erythematosus, Systemic ; drug therapy ; Male ; Middle Aged ; Phytotherapy ; Prednisone ; administration & dosage

BACKGROUND/AIMS: Protein-losing enteropathy (PLE), characterized by severe hypoalbuminemia and peripheral edema, is a rare manifestation of systemic lupus erythematosus. This present study aimed to identify the distinctive features of lupus-related PLE and evaluate the factors related to the treatment response. METHODS: From March 1998 to March 2014, the clinical data of 14 patients with lupus PLE and seven patients with idiopathic PLE from a tertiary center were reviewed. PLE was defined as a demonstration of protein leakage from the gastrointestinal tract by either technetium 99m-labelled human albumin scanning or fecal alpha1-antitrypsin clearance. A positive steroid response was defined as a return of serum albumin to > or = 3.0 g/dL within 4 weeks after initial steroid monotherapy, and remission as maintenance of serum albumin > or = 3.0 g/dL for at least 3 months. A high serum total cholesterol level was defined as a level of > or = 240 mg/dL. RESULTS: The mean age of the lupus-related PLE patients was 37.0 years, and the mean follow-up duration was 55.8 months. Significantly higher erythrocyte sedimentation rate and serum total cholesterol levels were found for lupus PLE than for idiopathic PLE. Among the 14 patients with lupus PLE, eight experienced a positive steroid response, and the serum total cholesterol level was significantly higher in the positive steroid response group. A positive steroid response was associated with an initial high serum total cholesterol level and achievement of remission within 6 months. CONCLUSIONS: In lupus-related PLE, a high serum total cholesterol level could be a predictive factor for the initial steroid response, indicating a good response to steroid therapy alone.
Adult ; Aged ; Biomarkers/blood ; Cholesterol/blood ; Drug Therapy, Combination ; Edema/diagnosis/drug therapy/*etiology ; Female ; Glucocorticoids/therapeutic use ; Humans ; Hypoalbuminemia/diagnosis/drug therapy/*etiology ; Immunosuppressive Agents/therapeutic use ; Lupus Erythematosus, Systemic/*complications/diagnosis/drug therapy ; Male ; Middle Aged ; Protein-Losing Enteropathies/diagnosis/drug therapy/*etiology ; Remission Induction ; Risk Factors ; Serum Albumin/metabolism ; Tertiary Care Centers ; Time Factors ; Treatment Outcome

BACKGROUND: We assessed the efficacy of serial interferon-gamma release assays (IGRAs) for the diagnosis of latent tuberculosis infection (LTBI) in patients receiving immunosuppressive agents for treatment of rheumatic diseases in Korea. METHODS: Of 276 patients who underwent consecutive screening with one of two IGRAs [QuantiFERON-TB Gold or QuantiFERON-TB Gold In-Tube], 66 patients were evaluated by the serial IGRA for detection of LTBI during therapy with immunosuppressive agents. Information on clinical diagnosis, medication, previous TB, blood cell count, tuberculin skin test, and interferon-gamma (IFN-gamma) level measured by IGRA was collected. RESULTS: Of the 66 patients, the initial IGRA was positive in 24.2%, negative in 65.2%, and indeterminate in 10.6%. Forty-six patients (69.7%) showed consistent IGRA results during follow-up, and 13 patients (19.7%) had consistently positive results. IGRA conversion rate was 12.1% (8/66) and reversion rate was 4.5% (3/66). Conversion of IGRA results was only observed in ankylosing spondylitis patients, and the median interval between the two tests in patients with conversion was 8.5 months. The mean IFN-gamma level in the group of patients with consistently positive IGRA results was higher than that in the group with inconsistently positive results, although this trend was not statistically significant (P=0.293). Indeterminate results were observed most frequently in patients with systemic lupus erythematosus. CONCLUSIONS: In patients receiving immunosuppressive agents, both IGRA conversions and reversions were observed. Serial IGRA testing may not be needed in patients with a positive initial IGRA result showing high IFN-gamma levels, because of high consistency in the test results.
Adult ; Blood Cell Count ; Female ; Follow-Up Studies ; Humans ; Immunosuppressive Agents/*therapeutic use ; Interferon-gamma/*analysis ; *Interferon-gamma Release Tests ; Latent Tuberculosis/complications/*diagnosis/metabolism ; Lupus Erythematosus, Systemic/complications/diagnosis/metabolism ; Male ; Middle Aged ; Rheumatic Diseases/complications/diagnosis/drug therapy ; Spondylitis, Ankylosing/complications/diagnosis/metabolism ; Tuberculin Test

OBJECTIVETo investigate the effect of arsenic trioxide (ATO) on the autoimmunity and survival time in BXSB lupus mice.

METHODSThe model BXSB lupus mice were randomly divided into two groups equally, the ATO treated group and the control group, 17 in each group. Mice in the ATO group were given intraperitoneal injection of ATO at the daily dose of 0.4 mg/kg, once every other day for 105 days or 90 days, respectively, and the observation lasted for 210 days. The survival time between the two groups was compared; the serum levels of anti-dsDNA and IgG were detected by enzyme-linked immunosorbent assay (ELISA), and the interferon-gamma (IFN-gamma) mRNA expression in renal and spleen tissue were measured by reverse transcriptase polymerase chain reaction (RT-PCR) technique.

RESULTSTill the 210th day, the total number of death was 8 in the ATO treated group and 13 in the control group, comparison between the two groups showed significantly different (chi2 = 4.20, P < 0.05). The mean OD value of serum anti-dsDNA antibody was lower in the ATO group (0.392 +/- 0.087) than that in the control group (0.566 +/- 0.080, P < 0.001). The serum level of IgG on day 105 in the ATO group was significantly lower than that in the control group and before treatment (P < 0.05). The expression of IFN-gamma mRNA in spleen tissue and renal tissue in the ATO group and the control group was 0.540 +/- 0.300 and 0.338 +/- 0.163, 2.320 +/- 0.522 and 0.588 +/- 0.104 (P < 0.05 and P < 0.01 respectively).

CONCLUSIONATO can prolong the survival time of BXSB lupus mice, decrease the peripheral level of anti-dsDNA antibody and IgG expression, inhibit the over-expression of IFN-gamma mRNA in spleen and kidney tissues.

Animals ; Antibodies, Antinuclear ; blood ; Arsenicals ; administration & dosage ; therapeutic use ; Autoimmunity ; drug effects ; Enzyme-Linked Immunosorbent Assay ; Immunoglobulin G ; blood ; Injections, Intraperitoneal ; Interferon-gamma ; genetics ; Kidney ; drug effects ; metabolism ; Lupus Erythematosus, Systemic ; drug therapy ; genetics ; immunology ; Mice ; Oxides ; administration & dosage ; therapeutic use ; RNA, Messenger ; genetics ; metabolism ; Random Allocation ; Reverse Transcriptase Polymerase Chain Reaction ; Spleen ; drug effects ; metabolism ; Survival Analysis

OBJECTIVETo study the effect of methylprednisolone (MP) and cyclophosphamide (CPA) intermittent intravenous pulse therapy and the clinical prognosis in children with severe juvenile onset systemic lupus erythematosus (JOSLE).

METHODSThirty patients with JOSLE, diagnosed by clinical, laboratory or renal histological examinations, were enrolled in this study. Of the 30 patients, 27 were females and 3 were males, the mean age was (12 +/- 3) years, and 20 of the 22 patients who had undergone initial therapy had LN, and the clinical courses before being involved in the study were 3 to 12 months in nine patients. Twenty-three of the 30 patients had clinical manifestations of renal damages, of whom 4 patients were proven by initial renal biopsy to have WHO type IV, 2 had type II,1 had type V and 1 had type III, and 7 patients had one or more manifestations of central nervous system, including chorea, seizures, cerebrovascular accident (CVA) and organic brain syndrome (OBS), simultaneously, 9 patients had nervous system symptoms without the clinical manifestations of renal damages, 3 patients had lupus crisis, 7 patients did not have any manifestations of renal or neurological damages. According to the protocol of the therapy, the patients were divided into 3 groups: group A (n = 18) patients were treated with MP plus CPA intermittent intravenous pulse for children with lupus nephritis, and with or without neuropsychiatric lupus erythematosus (NPLE), group B (n = 7) with pulsed doses of MP, followed by prednisone and tripterygium wilfordii hook f(T(whf)) for patients without renal or central nerves system damage, and group C (n = 5) with prednisone alone for patients with LN determined by clinical and laboratory features. The effects of those regimes and the clinical prognosis were observed.

RESULTSOn short-term follow-up, the SLEDAI-2K (by weight of the renal damage) showed significant difference between group A and group B, but there was no significant difference at the 9th months of the therapy. The long-term follow-up lasted in average for (37.2 +/- 24.8) months. Nineteen patients were followed up for more than 18 months. At the end of follow-up, the mean age was 14 to 19 years. There was no difference on the effect of both group A and group B, and no frequent infections were seen, ANAs were negative and SLEDAI-2K = 0-point in two patients of each group 12 months after discontinuation of the therapy. Four patients in group C died within 18 months.

CONCLUSIONThe immunosuppressive regimen MP + CPA in patients with severe JOSLE and MP + prednisone + T(whf) in patients without major organs damage were superior to the regimen of prednisone alone.

Adolescent ; Child ; Cyclophosphamide ; administration & dosage ; therapeutic use ; Drug Therapy, Combination ; Female ; Follow-Up Studies ; Glucocorticoids ; administration & dosage ; therapeutic use ; Humans ; Immunosuppressive Agents ; administration & dosage ; therapeutic use ; Lupus Erythematosus, Systemic ; drug therapy ; metabolism ; Male ; Methylprednisolone ; administration & dosage ; therapeutic use ; Pulse Therapy, Drug ; Treatment Outcome

OBJECTIVETo observe the effects of Xuebijing injection on dendritic cells (DCs) and T lymphocytes, and the potential mechanisms of its therapeutic effect on systemic lupus erythematosus (SLE).

METHODSA widely used mouse model, SLE-prone BLLF1 mice aged 8-10 weeks, was employed. Mice were randomly divided into 4 groups: a normal group, a model group and two treatment groups treated with Xuebijing Injection with a dose of 6.4 mL/kg via intraperitoneal administration for SLE-prone BLLF1 mice aged 8 weeks (treatment A group) and 10 weeks (treatment B group). Renal tissue sections were stained with Masson's trichrome and periodic acid-silver methenamine. Histopathological changes in the kidney were evaluated by a light microscopy. The capacity of the DCs isolated from the spleen to stimulate the T cell proliferation in response to concanavalin A (Con A) was determined.

RESULTSCompared with the model group, levels of anti-dsDNA antibodies in the two treatment groups decreased remarkablly (P<0.01, P<0.05), and levels of serum creatinine and blood urea nitrogen increased (P<0.01, P<0.05). Pathological changes were found in the kidney in the model group. Histopathological abnormalities were alleviated in the two treatment groups. Treatment with Xuebijing injection also significantly upregulated the expression of CD80, CD86 and major histocompatibility class II by DCs compared with the model group (P<0.05). When splenic T lymphocytes from BLLF1 mice were co-cultured with DCs at ratios of 1:100, 1:150 and 1:200 for 3 and 5 days, the proliferation of T lymphocytes was suppressed compared with the normal group (P<0.05), but this was restored by Xuebijing Injection under the same conditions. In the model group, levels of tumor necrosis factor (TNF)-α in supernatants were significantly elevated compared with the normal group (P<0.01), interleukin-2 levels decreased (P<0.05), while these changes were significantly alleviated in the Xuebijing treatment groups.

CONCLUSIONSXuebijing Injection alleviated renal injury in SLE-prone BLLF-1 mice. The mechanism might be through influencing T cell polarization mediated by DCs, and Xuebijing Injection might be a potential drug that suppresses immune dysfunction in patients with SLE.

Animals ; Antibodies, Antinuclear ; blood ; Cell Differentiation ; drug effects ; Cell Proliferation ; drug effects ; Concanavalin A ; pharmacology ; Dendritic Cells ; drug effects ; immunology ; pathology ; Drugs, Chinese Herbal ; administration & dosage ; pharmacology ; therapeutic use ; Injections ; Interleukin-2 ; metabolism ; Kidney ; drug effects ; pathology ; physiopathology ; ultrastructure ; Kidney Function Tests ; Lupus Erythematosus, Systemic ; blood ; drug therapy ; immunology ; physiopathology ; Mice ; Phenotype ; T-Lymphocytes ; drug effects ; immunology ; pathology ; Tumor Necrosis Factor-alpha ; metabolism