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ID PMID Title PublicationDate abstract
8190036 Hyperimmunoglobulinemia D and periodic fever syndrome. The clinical spectrum in a series o 1994 May We studied 50 patients (28 male and 22 female) with the hyper-IgD and periodic fever syndrome. Most patients originated from Europe, namely The Netherlands (28 cases; 56%), France (10 cases, 20%), and Italy (3 cases, 6%), but 1 patient was from Japan. A hereditary component is suggested by 18 patients coming from 8 families. The syndrome is typified by a very early age at onset (median, 0.5 years) and life-long persistence of periodic fever. Characteristically, attacks occur every 4-8 weeks and continue for 3-7 days, but the individual variation is large. Attacks feature high spiking fever, preceded by chills in 76% of patients. Lymphadenopathy is commonly present (94% of patients). During attacks, 72% of patients complained of abdominal pains, 56% of vomiting, 82% of diarrhea, and 52% of headache. Joint involvement is common in the hyper-IgD syndrome with poly-arthralgia in 80% and a non-destructive arthritis, mainly of the large joints (knee and ankle), in 68% of patients. Eighty-two percent of patients reported skin lesions with some attacks; these demonstrated vasculitis histologically. Serositis has been seen in only 3 patients (6%), while amyloidosis has not been recorded in any of the patients with this syndrome. Immunizations precipitated attacks in 54% of patients. All patients had a persistently elevated serum IgD level (> 100 U/mL), and in 82% of cases the serum IgA was likewise elevated. During attacks there is an acute-phase response adjudged by leukocytosis, neutrophilia, and increased ESR. The etiology remains to be elucidated, and treatment is supportive. The hyper-IgD syndrome is distinct from other periodic fever syndromes like systemic-onset juvenile rheumatoid arthritis, adult-onset Still disease, and familial Mediterranean fever.
8934926 The association between immunodeficiency and the development of autoimmune disease. 1996 Apr There is a paradoxical relationship between immunodeficiency diseases and autoimmunity. While not all individuals with immunodeficiency develop autoimmunity, nor are all individuals with autoimmunity immunodeficient, defects within certain components of the immune system carry a high risk for the development of autoimmune disease. Inherited deficiencies of the complement system have a high incidence of systemic lupus erythematosus (SLE), glomerulonephritis, and vasculitis. Carrier mothers of children with chronic granulomatous disease, an X-linked defect of phagocytosis, often develop discoid lupus. Several antibody deficiencies are associated with autoimmune disease. Autoimmune cytopenias are commonly observed in individuals with selective IgA deficiency and common variable immune deficiency. Polyarticular arthritis can be seen in children with X-linked agammaglobulinemia. Combined cellular and antibody deficiencies, such as Wiskott-Aldrich syndrome, carry an increased risk for juvenile rheumatoid arthritis and autoimmune hemolytic anemia. Several hypothetical mechanisms have been proposed to explain the associations between autoimmunity and immunodeficiency. Immunologic defects may result in a failure to exclude microbial antigens, resulting in chronic immunologic activation and autoimmune symptoms. There may be shared genetic factors, such as common HLA alleles, which predispose an individual to both autoimmunity and immunodeficiency. Defects within one component of the immune system may alter the way a pathogen induces an immune response and lead to an inflammatory response directed at self-antigens. An understanding of the immunologic defects that contribute to the development of autoimmunity will provide an insight into the pathogenesis of the autoimmune process.
7563995 [Clinical features of subacute interstitial pneumonia--clinico-pathological study based on 1995 Jul A clinico-pathological study was done to elucidate characteristic features of subacute interstitial pneumonia. The patients were four men and mine women, with a mean age of 60 years. In ten patients, the disease was idiopathic, three had collagen vascular disease, (and one was undergoing gold therapy for rheumatoid arthritis). The time interval between onset of symptoms and open lung biopsy was 80 +/- 40 days. Eleven patients had progressive dyspnea, seven had coughing, and only one complained of fever. Fine crakles were heard in ten patients. Mild increases in CRP were observed in all cases. Mild increases in total serum IgG concentration were observed in five of eight cases. Multiple patchy infiltration or diffuse interstitial shadows, located predominantly in the lower fields of both lungs were the characteristic chest roentgenographic findings. The average %VC was 62.7 +/- 17% and the average PaO2 was 68.3 +/- 10 Torr. Bronchoalveolar lavage was done in nine patients, and the mean total cell count was 16.5 +/- 10.2 x 10(4)/ml. A moderate increase in lymphocytes (30.8 +/- 18.6%) with a low CD4/8 ratio (0.48 +/- 0.57), a mild increase in neutrophils (6.2 +/- 9.1%), and a mild increase in eosinophils (2.3 +/- 3.7%) were observed. Pathologically, interstitial cellulo-fibrous changes associated with alveolar space closure due to organization of exudate were the main features. Patients were given steroid pulse therapy or oral steroids. The results were mild to marked improvements in chest roentgenographic findings and lung function.(ABSTRACT TRUNCATED AT 250 WORDS)
8323745 Factors that stimulate the proliferation and survival of eosinophils in eosinophilic pleur 1993 Jun To investigate the mechanism of eosinophilia in patients with eosinophilic pleural effusions, we measured the activities of eosinophil colony-stimulating factor (Eo-CSF) and stimulating factor for eosinophil survival in the eosinophilic pleural fluids of six patients (two with tuberculous pleuritis, two with drug allergy, and one each with chronic eosinophilic pneumonia and pleuritis associated with rheumatoid arthritis). The number of eosinophil colonies formed by the pleural fluid of patients with eosinophilic pleural effusions significantly exceeded that of control patients with noneosinophilic pleural effusions (7.5 +/- 1.9 colonies/10(5) bone marrow cells, n = 6, versus 0.3 +/- 0.1 colonies/10(5) bone marrow cells, n = 6, P < 0.01). Similarly, eosinophil survival evaluated on day 4 of culture with pleural fluid of patients with eosinophilic pleural effusions significantly exceeded that of patients with noneosinophilic pleural effusions (83.9 +/- 9.8% versus 46.1 +/- 11.2%, P < 0.001). Both activities were inhibited mainly by anti-IL-5 antibody and partially by anti-GM-CSF antibody and anti-IL-3 antibody. Mononuclear cells obtained from eosinophilic pleural fluid released the activities of Eo-CSF and stimulating factor for eosinophil survival in vitro. These findings suggest that GM-CSF, IL-5, and IL-3 are important to eosinophil accumulation in pleural cavity as stimulators of proliferation and survival of eosinophils.
8912508 Novel autoantibodies against muscle-cell membrane proteins in patients with myositis. 1996 Nov OBJECTIVE: To search for autoantibodies against muscle cell-specific surface membrane antigens in patients with inflammatory myopathies. METHODS: A cell enzyme-linked immunosorbent assay (cell ELISA) using a human rhabdomyosarcoma cell line (TE-671) was developed and performed in serial dilutions with either nonfixed or fixed cells. A total of 141 different patient sera were tested: 90 from patients with various rheumatic diseases, 12 from patients with cardiomyopathies, 25 from patients with other muscular diseases, and 14 from patients who had undergone major surgery or who had other noninflammatory diseases. As controls, 20 sera were obtained from healthy donors. Results were correlated using immunofluorescence staining and flow cytometry. RESULTS: Using the nonfixed cell ELISA, the proportions of positive sera from the patient groups with rheumatic diseases were 71% with polymyositis (PM), 15% with dermatomyositis (DM), 18% with systemic sclerosis (SSc), 15% with systemic lupus erythematosus (SLE), and 7% with rheumatoid arthritis. Sera from healthy donors, as well as sera from patients with nonrheumatic diseases, did not show significant reactivities. When other cell lines, including a chondrosarcoma, a bladder carcinoma, a pancreas carcinoma, and human foreskin fibroblasts, were used as substrates, positive sera did not react in the cell ELISA. Results obtained with the cell ELISA system using nonfixed cells were confirmed by flow cytometry and immunofluorescence staining. A strong protein band of 50 kd was detected on plasma membrane preparations from TE-671 muscle cells in 33% of PM sera (n = 12). CONCLUSION: In most sera from patients with PM, DM, and some other rheumatic diseases (i.e., SSc and SLE), autoantibodies directed against muscle-cell surface antigens can be detected. Since these molecules are localized in the muscle-cell surface membrane, autoantibodies directed against these antigens could play a major role in the pathogenesis of PM.
7945466 High levels of bcl-2 protein in circulating T lymphocytes, but not B lymphocytes, of patie 1994 Oct OBJECTIVE: Defective regulation of programmed cell death (apoptosis) may play a role in the development of autoimmune diseases, and the proto-oncogene bcl-2 is involved in the control of apoptosis in immunocompetent cells. We therefore wished to investigate the expression of bcl-2 in the peripheral lymphocytes of patients with systemic lupus erythematosus (SLE), a prototypical autoimmune disease. METHODS: Levels of bcl-2 expression in the lymphocytes of patients with SLE and, for comparison, in the lymphocytes of healthy controls and patients with rheumatoid arthritis (RA), systemic bacterial infections, and chronic B cell lymphocytic leukemia were studied by 2-color cytofluorography and RNA analysis. RESULTS: In SLE patients, a significant proportion of T cells expressed increased amounts of bcl-2 protein. By fluorescence-activated cell sorter analysis, bcl-2--enriched lymphocytes were found in the CD45RO+ as well as in the CD45RO-, and also in the CD4+ and CD8+, lymphocyte subpopulations. Mononuclear cells of patients with SLE showed increased amounts of bcl-2 messenger RNA. An increased percentage of strongly bcl-2 positive peripheral T lymphocytes was found in patients with bacterial infections, but not in those with RA. CONCLUSION: Although the occurrence of circulating T lymphocytes with abnormally high bcl-2 expression is not specific for SLE, it is evidence for a dysregulation of lymphocytic programmed cell death in this autoimmune disease.
8379462 Interleukin-1 receptor antagonist. 1993 IL-1ra is the first described naturally occurring receptor antagonist of any cytokine or hormone-like molecule. IL-1ra is a member of the IL-1 family by three criteria: amino acid sequence homology of 26 to 30% to IL-1 beta and 19% to IL-1 alpha; similarities in gene structure; and common gene localization to human chromosome 2q14. Two structural variants of IL-1ra exist: sIL-1ra, a secretory molecule produced by monocytes, macrophages, neutrophils, fibroblasts, and other cells; and icIL-1ra, an intracellular molecule produced by keratinocytes and other epithelial cells, macrophages, and fibroblasts. IL-1ra production by monocytes, macrophages, and neutrophils may be regulated in a differential fashion with IL-1 beta. Human IL-1ra binds to both human IL-1RIs and IL-1RIIs on cell surfaces, although with 100-fold greater avidity to IL-1RIs. IL-1ra may bind preferentially to soluble IL-1RIs and not at all to soluble IL-1RIIs. IL-1ra competitively inhibits binding of both IL-1 alpha and IL-1 beta to cell surface receptors without inducing any discernible intracellular responses. All three forms of IL-1 may bind to IL-1 receptors in a similar fashion but IL-1ra may lack the secondary interactions necessary to trigger cell responses. A 100-fold or greater excess of IL-1ra over IL-1 may be necessary to inhibit biological responses to IL-1 both in vitro and in vivo. The roles of sIL-1ra and icIL-1ra in normal physiology or in host defense mechanisms remain unclear. The administration of IL-1ra blocks the effects of IL-1 in some animal models of septic shock, inflammatory arthritis, graft-versus-host disease, and inflammatory bowel disease. The preliminary results of clinical trials in humans indicate possible efficacy of IL-1ra in sepsis syndrome, rheumatoid arthritis, and GVHD.
8909241 Association of Epstein-Barr virus (EBV) with Sjögren's syndrome: differential EBV express 1996 Nov The association of Epstein Barr virus (EBV) with Sjögren's syndrome (SS) is still in dispute. This study is aimed to investigate the existence of EBV genomes and their products in salivary glands of SS. Salivary gland samples were surgically obtained from Chinese patients. EBV DNA was detected in three of seven cases by dot blot hybridization and in four of seven cases by in situ hybridization. The EBV-encoded small RNA-1 (EBER1) was detected in two of seven cases by in situ hybridization. The immunohistochemical staining of EBV proteins showed that the EBV latent membrane protein-1 was detected in four of seven cases and that BZLF1, BALF2, and gp350/220 proteins associating with virus production were not expressed. In eight controls, no positive signal was observed by these methods. DNA in situ hybridization identified ERV on both epithelial cells and lymphocytes. On the other hand, EBER1-positive signals were exclusively localized on lymphocytes. These results indicate that two forms of EBV infection may exist in salivary glands of SS. One is EBER1-positive latency in lymphocytes, the other is EBER1-negative latency in epithelial cells. Frequent EBV detection in salivary glands of SS suggests that EBV plays a role in the genesis of SS.
7586691 Prevention of adoptive transfer of murine Sjögren's syndrome into severe combined immunod 1995 Nov We have analysed the role of ICAM-1 and LFA-1 during development of autoimmune sialadenitis in MRL/lpr mice by direct analysis of RNA obtained from the salivary gland tissues, and the therapeutic effects with antibody administration on adoptive transfer system into SCID mice. The expression of cell adhesion molecules was assessed by using reverse transcriptase polymerase chain reaction (RT-PCR) and Southern blot analysis, and immunohistochemical analysis. Up-regulated expression of ICAM-1 mRNA was observed before the onset of inflammatory lesions in the salivary glands at 1 month and 2 months old, and thereafter LFA-1 mRNA was expressed within the typical inflammatory lesions, resembling human Sjögren's syndrome in MRL/lpr mice. Immunohistochemically, ICAM-1 was localized exclusively in the endothelial cells of varying sized blood vessels before the onset of disease, and LFA-1 expressing inflammatory cells were found within these lesions. When the therapeutic effects in vivo were examined, antibodies to ICAM-1 in combination with anti-LFA-1 prevented adoptive transfer of Sjögren's syndrome in MRL/lpr mice into SCID mice, while no significant effect was found when treated with either antibody. These findings indicate that in Sjögren's syndrome-like autoimmune lesions in MRL/lpr mice the ICAM-1/LFA-1 pathway may play a crucial role in the initiation and subsequent progression of T cell-mediated autoimmunity in the salivary and lacrimal glands of MRL/lpr mice.
7575850 Renal tubular acidosis complicated with hypokalemic periodic paralysis. 1995 Jul Three Chinese girls with hypokalemic periodic paralysis secondary to different types of renal tubular acidosis are presented. One girl has primary distal renal tubular acidosis complicated with nephrocalcinosis. Another has primary Sjögren syndrome with distal renal tubular acidosis, which occurs rarely with hypokalemic periodic paralysis in children. The third has an isolated proximal renal tubular acidosis complicated with multiple organ abnormalities, unilateral carotid artery stenosis, respiratory failure, and consciousness disturbance. The diagnostic evaluation and emergent and prophylactic treatment for these three types of renal tubular acidosis are discussed.
7746969 [Thyroid disease and multiple autoimmune syndromes. Clinical and immunogenetic aspects apr 1995 The authors report eleven cases of multiple auto-immune syndrome, concerning a total of 15 different auto-immune diseases. This study suggests that auto-immune thyroid disorders (Graves' disease or hypothyroidism resulting from Hashimoto's thyroiditis) are a common feature of multiple auto-immune syndromes, while antithyroid antibodies are constant among our patients. Sjögren's syndrome is also very prevalent, and seems to be non-randomly associated with auto-immune thyroid disorders, a fact which suggests common aetiological factors. Among other auto-immune disorders, the antiphospholipid syndrome has been diagnosed twice. A genetic predisposition to multiple auto-immune syndrome is obvious, as shown by a familial history of auto-immune disorders (found in more than half of the cases), and a predominant HLA phenotype, B8 and/or DR3. The authors plead for a systematic enquiry for multiple auto-immune syndrome in patients with auto-immune thyroid disorders and a family history of auto-immune disorders.
9115577 A frame shift mutation in a hot spot region of the nuclear autoantigen La (SS-B). 1996 Dec A hot spot region was identified in the exon 7 of the nuclear autoantigen La (SS-B). Two La cDNAs were identified which contained a frame shift mutation in the hot spot region. One La cDNA was isolated from a cDNA library made from peripheral blood lymphocytes of an autoimmune patient with primary Sjögren's Syndrome, the other La cDNA was isolated from a human liver cDNA library. The patient's La cDNA had a deletion and the liver La cDNA had an insert of an (A)-residue at the same position. Inserts of 4, 16 and 24 more or less homogeneous (A)-residues were found at the same site in the three La retropseudogenes. The hot spot region located in one of the major autoepitope regions of the La antigen. Both frame shift mutations resulted in premature stop codons. In case of the human liver La cDNA, the premature stop codon located a single amino acid downstream of the frame shift mutation, while it located eleven amino acids downstream of the frame shift mutation in the patient's La cDNA. In consequence, only the sequence of the La peptide encoded by the patient's La cDNA markedly differed from the corresponding La peptide sequence. Translation of the patient's mutant La mRNA in transfected mouse cells resulted in a C-terminally truncated La peptide. Due to the lack of the nuclear location signal it remained in the cytoplasm. The modified La peptide shared homology with (i) La protein itself and (ii) a series of DNA binding proteins including other autoantigens and viral proteins such as topoisomerase I, RNA dependent RNA polymerase of influenza virus and reverse transcriptase. The self-homology region includes the amino acids which the La protein shares with B1 Laminin. It represents a putative neo-epitope that could be involved in triggering of the autoimmune response.
8565310 T cell clones from a Sjögren's syndrome salivary gland biopsy produce high levels of IL-1 1996 Feb Sjögren's syndrome (SS) is characterized by a focal periductal salivary gland infiltrate consisting mainly of T and B lymphocytes. Most of the T cells bear the memory CD4+ Th1-like phenotype and express high levels of class II, though CD8+ cells are also present. We have studied 17 labial salivary gland and 15 peripheral blood T cell clones from a patient with primary SS. The tissue clones were 71% CD8+ and 29% CD4+, and the peripheral blood-derived clones were 60% CD8+ and 40% CD4+. The CD4+ T cell clones from both the salivary gland and autologous peripheral blood were of the Th1 phenotype, in that they produced interferon-gamma (IFN-gamma) and IL-2 but very little IL-4 after 24 h stimulation with phorbol myristate acetate and anti-CD3 antibody. The salivary gland-derived CD4+ clones produced 15 times more IL-10 (7.92 ng/ml) than peripheral blood-derived CD4+ clones (0.52 ng/ml, P < or = 0.02). The tissue CD8+ clones produced 1.2 times (P < 0.04) more IFN-gamma and CD4+ clones produced 3.5 times less IL-2 (P < 0.02) than the respective PBM-derived clones. The accumulation of Th1-type cells producing high levels of IL-10 in the salivary gland suggests a specific immunoregulatory function at the site of inflammation in SS.
8173843 HLA-DR alleles in patients with Sjögren's syndrome over-representing V beta 2 and V beta 1994 May To identify genetic factors that play a role in the pathogenesis of patients with SS over-representing V beta 2 and V beta 13 genes in the lips, HLA-DR and -DQ alleles of 10 primary SS patients with predominant expression of V beta 2 and V beta 13 genes in the lips were analysed, using the polymerase chain reaction (PCR) and sequence specific oligonucleotide probes. The CDR3 amino acid sequences of cDNA clones encoding V beta 2 and V beta 13 genes were also determined by PCR. The results showed that the DRB4*0101 allele was significantly increased (80%) and that the frequency of DRB3 allele was decreased (0%) when compared to findings in healthy subjects (35.6 and 26%, respectively). Sequencing analyses demonstrated that 75% of V beta 2 cDNA clones and 87% of V beta 13 cDNA clones had a glutamine residue at position 106, in the CDR13 region. Moreover, the conserved sequences (Y*TLRNEQ) in the CDR3 of V beta 13-positive T cell were detected in two different clones (27%) from the two individual SS patients. These findings suggest that the decreased DRB3 and increased DRB4*0101 alleles may be associated with the antigens recognized by V beta 2- and V beta 13-positive T cells.
1540872 Malignant lymphoma of mucosa-associated lymphoid tissue arising in the thymus of a patient 1992 Mar 15 A 59-year-old woman with Sjögren's syndrome had an anterior mediastinal tumor. The tumor had epithelium-lined thymic cysts. Histologically, centrocyte-like (CCL) cells were present as clusters intermingling with small lymphocytes and plasma cells, invaded the epithelium, and formed characteristic lymphoepithelial lesions; the tumor was identified as malignant lymphoma arising in mucosa-associated lymphoid tissue (MALT). Within the tumor, trapped Hassall's corpuscles were recognized. Immunohistochemical staining demonstrated monotypic cytoplasmic kappa light chains in a small portion of the CCL cells. Furthermore, Southern blot hybridization studies showed rearrangements of immunoglobulin heavy chain, immunoglobulin kappa light chain, and T-cell receptor beta genes. The findings are consistent with thymic low-grade B-cell MALT lymphoma.
8964425 Widespread clonal B-cell disorder in Sjögren's syndrome predisposing to Helicobacter pylo 1996 Jun Helicobacter pylori has been identified as a critical antigenic stimulus to the development of gastric lymphoma, but additional factors should be required for such evolution. This topic is now of major importance to clarify the pathobiology of gastric lymphomagenesis. Peculiar autoimmune diseases, such as Sjögren's syndrome, are well known to predispose to B-cell lymphomas. We report on a patient with Sjögren's syndrome and a widespread B-cell lymphoproliferative disorder. A pathological picture of low-grade lymphoma was observed in the stomach, concomitantly with H. pylori infection. However, the B-cell disorder was definitely nonmalignant in the other tissues involved, i.e., the parotid gland and lymph nodes, which are the characteristic targets of Sjögren's syndrome-associated lymphoproliferation. After H. pylori eradication, a dramatic regression of gastric lymphoma into chronic gastritis was observed, but no amelioration occurred in the parotid and nodal involvement. Multiple molecular analyses showed the expansion of the same B-cell clone in synchronous and metachronous lymph node, parotid, and gastric lesions before and after H. pylori eradication. Thus, H. pylori played a crucial role in the local boosting of B-cell lymphoproliferation, but the underlying B-cell disorder was that associated with the autoimmune disease and was nonmalignant. The comprehensive clinical, pathological, and molecular approach allowed us to then distinguish the role of peculiar individual predisposing factors and of local infection in the pathobiology of mucosa-associated lymphoid tissue-associated lymphoproliferation.
8915033 Cytokine gene expression and autoantibody production in Sjögren's syndrome of MRL/lpr mic 1996 In an attempt to elucidate the mechanism of development of organ-specific autoimmune lesions resembling human Sjögren's syndrome of MRL/lpr mice, we have analyzed local cytokine gene expressions and organ-specific autoantibody production in vivo. We have demonstrated that a major proportion of T cells bearing CD4 and V(beta)8 molecules are essentially responsible for triggering the autoimmunity in the salivary glands of MRL/lpr mice. The local cytokine gene expressions including interferon(IFN)-gamma, IL-12(p40) mRNAs were observed during the course of murine Sjogren's syndrome in MRL/lpr autoimmune strain. In particular, a high level of local expressions of IL-12 mRNA was detected earlier in the proinflammatory stage of autoimmune lesions. A significant level of local expression of MHC class-II(I-Ak) mRNA was detected before the onset of inflammatory lesions in the salivary glands, and I-Ak-positive epithelial duct cells were frequently observed in the salivary glands of MRL/lpr mice. In addition, we found the salivary gland-specific autoantibody in sera from MRL/lpr mice with early phase of autoimmune lesions by immunoblot analysis. These results suggest that cytokine gene stimulation and autoantibody production are essentially involved in the development of organ-specific autoimmune lesions in Sjögren's syndrome of MRL/lpr mice.
7582692 Maternal HLA antigens and antibodies to SS-A/Ro and SS-B/La. Comparison with systemic lupu 1995 Oct To study the maternal immunogenetics in congenital heart block (CHB), 31 mothers of affected children were HLA typed for class I and II antigens, and the results were compared with the corresponding HLA types in 900 healthy controls, in 45 mothers with systemic lupus erythematosus (SLE) and in 21 mothers with primary SS who had healthy children. An enzyme-linked immunosorbent assay was used to study the autoantibody responses to the recombinant 52 and 60 kDa SS-A/Ro, and 48 kDa SS-B/La proteins, and to the affinity-purified SS-A/Ro and SS-B/La antigens. Mothers of children with CHB had HLA B8 and DR3 significantly more often than healthy controls [71 vs 10%; relative risk (RR) 9.8, P < 0.00001 and 74 vs 23%; RR9.8, P < 0.001, respectively]. HLA B35 was protective (RR 0.1, P = 0.0029). Compared to controls with SLE, mothers of children with CHB were more often HLA DR3 and DQ2 positive (RR 4.1, P = 0.0057 and RR 3.1, P = 0.031, respectively), and compared to controls with primary SS less often HLA B15 positive (RR 0.1, P = 0.010). In general, the HLA antigen profile in mothers of children with CHB was more closely related to primary SS than to SLE. Levels of antibodies to all three SS-A/Ro antigens were significantly higher in mothers of children with CHB than in controls with SLE and primary SS (P = 0.0001-0.0014). With regard to SS-B/La, the autoantibody responses were similar (P = 0.32-0.66).(ABSTRACT TRUNCATED AT 250 WORDS)
7493450 Ro/SS-A and La/SS-B: autoantigens in Sjögren's syndrome? 1995 Jul Sjögren's syndrome is characterized serologically by the presence of autoantibodies against Ro/SS-A and La/SS-B. The mechanisms by which these autoantibodies arise is not clear at this moment. B cells capable of producing antibodies to Ro/SS-A and La/SS-B seem to be present in every individual; whether or not an individual indeed makes these antibodies is governed by T cells. Recent experiments from us and from others indicate that T cell tolerance towards Ro/SS-A and La/SS-B can be broken by immunization of normal mice with recombinant human Ro/SS-A or La/SS-B. The specificity of the T cells directing the anti-Ro/SS-A and anti-La/SS-B autoantibody response in these animals has not yet been elucidated. T cells may either be directed against foreign epitopes present on the human immunogen or they may be truly autoreactive. In patients with Sjögren's syndrome, a comparable immunization route might encompass product of viral origin directing the T cell response via RNA/protein complexes. Putative candidates comprise viruses that make use of RNAse polymerase-III, such as Epstein Barr virus, adenovirus, vesicular stomatitis virus en rabies virus.
8788549 Comparison of different methods for the detection of autoantibodies in autoimmune diseases 1995 Different immunological techniques were compared for their sensitivity in detecting some important autoantibodies in the sera of patients with rheumatic diseases. Sera of patients with systemic lupus erythematosus were screened for anti-dsDNA, Sm, and RNP autoantibodies by Crithidia luciliae assay, Farr assay, enzyme immunoassay, and immunoblotting. Sera of patients with Sjögren's syndrome were screened for anti-SSA and anti-SSB antibodies by enzyme immunoassay, counter immunoelectrophoresis, and immunoblotting and sera of patients with scleroderma for SCL-70 autoantibodies by enzyme immunoassay counter immunoelectrophoresis, and immunofluorescence on Hep-2 cells. Enzyme immunoassay and counter immunoelectrophoresis gave the most positive results and the best agreement compared with the other techniques. Immunofluorescence gave few positive results for each antibody evaluated. Immunoblotting gave intermediate results for all autoantibodies except anti-SSA, where the prevalence was low. There was no relationship between levels of dsDNA, Sm, and RNP antibodies and disease activity score in systemic lupus erythematosus patients.