ABSTRACT
Plasmacytoid dendritic cells (pDCs) are a unique subset of dendritic cells that can rapidly produce large amounts of type Ⅰ interferon after activation. They are critical in antiviral immunity and involved in the initiation and development of many autoimmune diseases. Systemic sclerosis (SSc) is an autoimmune disease characterized by immune dysregulation, vasculopathy and progressive fibrosis of the skin and internal organs. Recent studies have found that pDCs are involved in the pathogenesis of SSc. Inhibiting the function of pDCs can effectively prevent inflammation and fibrosis in SSc, highlighting the role of pDCs in the pathogenesis of SSc. Therefore, an in-depth understanding of pDCs and their role in the pathogenesis of SSc is important for the development of pDCs and their mediators as therapeutic targets for SSc.
ABSTRACT
Objective To identify the clinical characteristics and therapeutic effect of systemic lupus erythematosus (SLE) patients with diffuse alveolar hemorrhage (DAH).Methods Clinical characteristcs,diagnosis,treatment and outcome in 34 patients hospitalized in The First Affiliated Hospital of Guangxi Medical University from January 2006 to December 2016 were retrospectively analyzed.Results The incidence of DAH involvement of SLE was about 0.39％.Median age was 19 (interquartile range (IQR) 11.75 ～ 32) years.The duration of SLE before clinical DAH was 6 months (IQR 2 ～ 15.75) months.The main clinical manifestations of DAH were cough,dyspnea and fever.The SLE disease activity index (SLEDAI) score was 19.5 (16 ～ 25)points and anti-dsDNA antibody titer elevated markedly (38.2％).The overall mortality rate was 73.5％,however patients who chose department of rheumatism have lower mortality (52.9％).And treatment of CTX was associated with mortality (OR =0.059,95％ CI 0.006 ～ 0.554,P =0.013),as well as steroids and immunosuppressant combination therapy.Conclusions The clinical symptoms of SLE with DAH is often atypical.There is manifestation of cough,fever,dyspnea and etc.Imaging and broncoscopy can assist the diagnosis and its prone to the pulmonary infection and high mortality.Early diagnosis and application of CTX combined with conventional dose of hormone theraphy can in early diagnosis and reduce the mortality.
ABSTRACT
Systemic lupus erythematosus (SLE) is a severe systemic autoimmune disease, which is characterized by excessive production of autoantibodies caused by B cell hyperactivity. Thus,reducing au-toantibody production can control the development of SLE,and understanding the molecular and cellular fac-tors involved in the differentiation of B cells will provide new therapeutic targets. Follicular helper T cells (Tfh) are defined as a new subset of CD4+T cells specialized in providing help to B cells,which is suspec-ted to play a critical role in the pathogenesis of SLE. In the present review,we give an overview of key mole-cules involved in the differentiation,regulation and functions of Tfh,discuss the roles of Tfh in SLE and de-scribe some potential therapeutic targets for SLE.
ABSTRACT
Objective To investigate the secretory capacity and apoptosis of interleukin ( IL)-21 induced normal B cells by co-culture with serum from patients with systemic lupus erythematosus (SLE). Methods Serum from twenty new-onset SLE patients and 20 healthy donors were collected .CD1+9 B cells from the normal controls were co-cultured with serum from SLE patients in the presence or absence of IL-21-R-FC(4 μg/ml).Supernatant IgG and IgM concentration were measured by immunoturbidimetric assay on day 5.Supernatant anti-dsDNA level was determined by ELISA .The percentage of apoptotic cells was detected by flow cytometer.Results IgG,IgM and anti-dsDNA levels in normal B cells with SLE serum were significantly higher than those in the serum of SLE patients alone [ ( 5.84 ±1.79 ) g/L vs ( 4.25 ± 1.48)g/L,P=0.000;(0.46 ±0.21)g/L vs (0.43 ±0.21)g/L,P=0.003;(127.76 ±70.24)IU/ml vs (115.15 ±63.88) IU/ml,P=0.014 respectively].However, no significant differences were found in the group of normal B cells with non-homologous serum from normal controls (P>0.05).Supernatant IgG, IgM and anti-dsDNA levels in normal B cells with SLE serum significantly decreased while IL-21R-fusion protein was added [(5.26 ±1.62)g/L vs (5.84 ±1.79)g/L, P=0.006;(0.42 ±0.20)g/L vs (0.46 ±0.21)g/L, P=0.002;( 118.00 ±69.62 ) IU/ml vs ( 127.76 ±70.24 ) IU/ml, P =0.012 respectively ] .The apoptotic rate of B cells with SLE serum was significantly higher than that with normal serum [ ( 47.88 ± 12.65)%vs (38.86 ±10.32)%,P =0.004].But adding IL-21R-fusion conversed the apoptotic rates [(42.08 ±12.52)%vs (47.88 ±12.65)%,P=0.001].Conclusions SLE serum could induce normal B cells to form immunoglobulin secreting cells and producing autoantibodies , or apoptosis in pathological conditions.IL-21 might be considered as a potential therapeutic target of SLE .