The European cohort study has indicated about CD74 IgG-autoantibodies as potential marker for axial spondyloarthritis (axSpA) diagnosis. However, multiple studies have questioned the diagnostic value of various disease-specific autoantibodies in different ethnic groups. Here, we have tried to assess the diagnostic value of anti-CD74 IgG and IgA autoantibodies in axSpA patients from Chinese Han population.
View Article and Find Full Text PDFChin Med J (Engl)
November 2020
Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease that presents with sicca symptoms of the main mucosal surfaces. Patients with pSS have a broad spectrum of laboratory features, such as cytopenias and hypergammaglobulinemia. Although hematological abnormalities are usually seen in pSS patients, agranulocytosis and autoimmune hemolytic anemia (AIHA) are rare.
View Article and Find Full Text PDFObjectives: This study aimed to examine the long-term efficacy, remission and survival of patients with severe systemic lupus erythematosus (SLE) after the combination treatment with high-dose immunosuppressive therapy (HDIT) and autologous peripheral blood stem cell transplantation (APBSCT).
Methods: Chinese patients with severe SLE receiving combination therapy with HDIT and APBSCT in Peking Union Medical College Hospital were enrolled from July 1999 to October 2005. Disease activity, treatment, and adverse effects of these patients were evaluated.
With this study, we provide insight into the clinical characteristics, laboratory characteristics, and organ damage associated with incomplete lupus syndromes (ILE) and search for predictors of organ damage in ILE. A retrospective chart review was performed on 77 hospitalized patients with ILE. The control patient group comprised 2104 systemic lupus erythematosus (SLE) patients who were entered into the Chinese SLE Treatment and Research group (CSTAR).
View Article and Find Full Text PDFObjective: To explore the clinical features of immunoglobin (IgG4)-related Mikulicz's disease (MD).
Methods: Since December 2010, a prospective cohort study of IgG4 related disease (IgG4RD) was performed our hospital. A total of 42 IgG4RD patients were recruited along with 18 MD patients.
We present a case of hypertrophic osteoarthropathy (PHO), with painful synovium hyperplasia involving both knees that was refractory to corticosteroid treatment. His rheumatoid factor and anti-CCP antibody was negative, and his serum ESR and CRP level was within normal range. Histological examination of the synovium obtained from his right knee revealed endothelial hyperplasia and vascular thickening without inflammation that was in association with aberrant expression of CD200/CD200R1, which highlighted the importance of aberrant CD200/CD200R1 in the regulation of the endothelial activation that contributed to the development of synovium hyperplasia in this PHO patient.
View Article and Find Full Text PDFWe describe a case of systemic sclerosis (SSc) complicated with portal hypertensive ascites which did not improve with diuretics and ascitic drainage. When corticosteroid added, her ascites diminished dramatically. Though portal hypertension can be imputed to other causes, such as polycystic liver in this case, it can occur in limited SSc with positive anti-centromere antibody and respond to corticosteroid treatment.
View Article and Find Full Text PDFObjective: To investigate clinical features of cutaneous sarcoidosis.
Methods: A retrospective analysis was carried out based on the clinic data of 35 patients with cutaneous sarcoidosis who were hospitalized in Peking Union Medical College Hospital during 1980 - 2009. They were divided into two groups, the group without systemic involvement (skin group) and the group with systemic involvement (systemic group).
Objective: To compare the T cell receptor recombination excision cycle (TREC) levels in peripheral blood mononuclear cells (PBMC) of systemic lupus erythematosus (SLE) patients with normal age- and gender- matched controls. To investigate the correlations between TREC levels of SLE patients and their clinical features.
Methods: We studied TREC levels in peripheral blood mononuclear cells (PBMC) of 21 SLE patients and 22 normal age- and sex-matched controls.
Zhonghua Nei Ke Za Zhi
August 2009
Objective: To investigate the clinical features and treatment strategy for the thymoma-associated systemic lupus erythematosus (SLE) in a worldwide perspective.
Methods: All articles between 1975 to 2008 were searched with the key words "SLE Thymoma" or "SLE thymectomy" in the database of Pubmed & Medline; the Chinese articles were searched with the same key words in the database of China Hospital Knowledge Database (CHKD). Data were extracted and analyzed.
(PBC) is not a rare disease worldwide. Most patients are diagnosed at the advanced stage, primarily because there are not yet any valid biomarkers available for early diagnosis. Useful biomarkers are absolutely necessary for early detection of PBC.
View Article and Find Full Text PDFObjectives: To assess the safety and efficacy of high-dose immunosuppression and autologous peripheral blood cell transplantation (APBSCT) in severe and refractory primary Sjögren's syndrome (pSS) and to analyze immune reconstitution in pSS.
Methods: Two patients with severe and refractory primary pSS were included in this study. They suffered still with active pSS despite the use of prednisone and immunosuppression agents.
Objective: To investigate the differences in immune reconstitution, hematopoietic reconstitution, efficacy, and complication between the two conditioning regimens with or without total body irradiation (TBI) in patients with refractory and severe autoimmune diseases (AID) who receiving autologous peripheral blood stem cell transplantation (APBSCT).
Methods: Thirty-two AID patients, 5 males and 27 females, aged 29 (15 - 49), underwent APBSCT. The CD34(+) cells were mobilized with cytoxan (CTX) + granulocyte-colony stimulating factor (G-CSF) and selected by clinical magnetic activated cell sorting (CliniMACS).
Objective: To evaluate the feasibility and safety of peripheral CD34+ cell mobilization in patients with severe autoimmune disease.
Methods: Forty-two patients underwent a total of 46 mobilizations by the regimen of cyclophosphamide 2-3 g/m2+ recombinant human granulocyte colony stimulating factor (rhG-CSF) 5 microg x kg(-1) x d(-1). The positive selection of CD34+ cell was performed through the CliniMACS.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao
June 2007
Objective: To investigate the dynamic changes of lymphocyte subsets before and after autologous hemopoietic stem cell transplantation (HSCT) in severe/refractory autoimmune disease (AID) and study the post-transplantation immunological reconstitution in AID.
Methods: Thirteen patients with severe/refractory AID who registered for HSCT from April 2003 to April 2005 in Peking Union Medical College Hospital, including 8 patients with systemic lupus erythematosus, 4 patients with rheumatoid arthritis, and 1 patient with primary Sjögren's syndrome (pSS) were enrolled in this study. Blood samples were collected before/after mobilization, before conditioning, and 2 weeks, 1 month, 3 months, 6 months, 12 months, and 18 months post-transplantation.
Objective: To study the neuropsychiatric (NP) manifestations of systemic lupus erythematosus (SLE) and evaluate treatment with intrathecal (IT) methotrexate (MTX) and dexamethasone (DXM).
Methods: 240 patients with NP syndromes of SLE (NPSLE) from 1990 to 2004 were retrospectively reviewed and IT injection group and non-IT injection group were compared. The side effects of IT injection were also discussed.
Objective: To evaluate the feasibility, efficacy, and safety of high dose immunosuppressive therapy (HDIT) and autologous hemopoietic stem cell transplantation (HSCT) with CD34+ cell selection in patients with severe, refractory autoimmune diseases.
Methods: Twenty-six patients with persistent systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), primary Sjögren's syndrome (pSS), or systemic sclerosis (SSc) who had been treated unsuccessfully with conventional treatment were enrolled in the trial in Peking Union Medical College Hospital from September 1999 to June 2004. The patients received HDIT with 200 mg/kg cyclophosphamide followed by an infusion of autologous stem cells that were CD34 selected.
Objective: To investigate the feasibility, efficacy and safety of high dose immunosuppressive therapy (HDIT) and autologous peripheral blood stem cell transplantation (PBSCT) with CD(34)(+) cell selection in patients with refractory and severe autoimmune diseases.
Methods: Twenty-one patients with SLE, RA, pSS, SSc or MCTD were enrolled in the study from 1999. Autologous haemopoietic stem cells were mobilized with CTX 3 approximately 4 g/m(2) and granulocyte colony stimulating factor (G-CSF).
Zhonghua Yi Xue Za Zhi
August 2004
Objective: To investigate the clinical manifestations, diagnosis and treatment of anti-phospholipid syndrome (APS).
Methods: 61 patients with defined APS admitted from Jan 1986 to Dec 2002 were analyzed retrospectively.
Results: 10 patients with primary APS and 51 patients with secondary APS were analyzed.