ABSTRACT
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of diverse autoantibodies with various systemic organ involvements. In patients with SLE, autoantibodies, such as antinuclear antibody (ANA) and anti-dsDNA antibody, play an important role not only in diagnosing the disease, but also representing the pathogenesis of the disease. ANA is the main screening tool in diagnosis and serum complement levels and anti-dsDNA antibody level are closely related to the disease activities. Nevertheless, exceptionally, some patients represent with negative ANA and/or anti-dsDNA antibody leading to difficulties in diagnosing the disease. Here, we report a case of 37-year old female SLE patient with negative ANA, negative anti-dsDNA antibody, and positive anti-Ro/SSA antibody, which manifested with nephrotic syndrome.
Subject(s)
Female , Humans , Antibodies, Antinuclear , Autoantibodies , Autoimmune Diseases , Complement System Proteins , Glomerulonephritis, Membranous , Lupus Erythematosus, Systemic , Mass Screening , Nephrotic SyndromeABSTRACT
Objective: To investigate the association between deficiencies of early components in the classical complement pathway and the development of SLE. Methods: Forty inbred C57BL/6J mice and 40 knockout C4 complement gene (C4KO) mice, which included 10 mice in each age group (2, 4, 6, and 8 months) were used. The enumeration of CD4+CD25+ Tregs frequencies in bone marrow, spleen and peripheral blood from both normal and C4KO groups were performed by flow cytometry. The expression levels of Foxp3 and TGF-b in the same tested tissues were measured using real time PCR. The antinuclear antibodies (ANA) were semi-quantitatively measured using ELISA. Results: We report decreased frequencies of CD4+CD25+ Tregs and reduced expression levels of Foxp3 and TGF-β, which efficiently program the development and function of Tregs, in lymphoid tissues and peripheral blood of C4KO mice. In this study, C4KO mice have higher titers of ANA than those of normal mice. Higher frequencies of mice positive for ANA are also found in older mice.Conclusions: The deficiency of the C4 gene induces the decreased numbers of Tregs that further increase the production of ANA resulting in the development of an autoimmune disorder. The outcomes of our study help us to understand the association between the deficiency of C4 in the classical complement pathway and development of autoimmune disorder via the role of Tregs.
ABSTRACT
Objective: To explore the influence of estradiol benzoate in induction of BALB/c mouse systemic lupus erythematosus model. Methods: Gonadectomied BALB/c mice were immunized with homologous spleen cell activated by concanavalin A three times,and at the same time injected with different doses estradial benzoate. Serum antinuclear antibody, antihistone antibody and estradiol in serum and kidney tissue homogenate were detected by enzyme linked immunosorbent assay. Kidney lesions were detected by hematoxylin-eosine(HE) and periodic acid-Schiff(PAS) staining, and by transmission electron microscope for detecting electron-dense agent as well as by direct fluorometric method for detecting immunoglobulin G antigen-antibody complex. Results: BALB/c mice immunized with homologous spleen cells activated by concanavalin A all developed systemic lupus erythematosus, but a statistically significant variation was observed in both autoantibody production and kidney damage were observed with a statistically significant variatron in the groups receiving different dosage of estradiol benzoate group(P