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ID PMID Title PublicationDate abstract
16737433 [Pneumonia by Corynebacterium pseudodiphteriticum: an infection to consider]. 2006 Mar Corynebacterium pseudodiphteriticum has been considered a very infrequent respiratory pathogen. We report three cases of pneumonia due to C. pseudodiphteriticum, describing their clinical and microbiological features. There were two patients with pre-existing chronic respiratory disease, one of their with steroidal therapy, and other associated with endotracheal intubation. The diagnostic was made by Gram stain and quantitative cultures from respiratory tract specimens. All patients were cured after treatment with amoxicillin-clavulanate, ceftriaxone and vancomycin respectively. C. pseudodiphteriticum must be consider as a possible causal agent of pneumonia in patients with underlying respiratory disease or endotracheal intubation. Antimicrobial susceptibility testing of C. pseudodiphteriticum may be useful for correct treatment of infected patients, but beta-lactam antibiotics are an appropriate therapeutic option against this bacteria.
16300639 c-Jun NH2-terminal kinase mediates interleukin-1beta-induced inhibition of lacrimal gland 2006 Jan Sjögren's syndrome, an inflammatory disease affecting the lacrimal and salivary glands, is the leading cause of aqueous tear-deficient type of dry eye. We previously showed that interleukin-1beta (IL-1beta) protein is up regulated in the lacrimal gland of a murine model of Sjögren's syndrome and that exogenous addition of this cytokine inhibits neurotransmitter release and lacrimal gland protein secretion. In the present study we investigated the role of c-Jun NH2-terminal kinase (JNK) in IL-1beta-mediated inhibition of lacrimal gland secretion and tear production. In vitro, IL-1beta induced a time-dependent activation of JNK with a maximum 7.5-fold at 30 min. SP600125, a JNK inhibitor, inhibited, in a concentration-dependent manner, IL-1beta-induced activation of JNK with a maximum of 87% at 10(-4) m. In vivo, IL-1beta stimulated JNK and the expression of the inducible isoform of nitric oxide synthase (iNOS). IL-1beta inhibited high KCl and adrenergic agonist induced protein secretion by 85% and 66%, respectively. SP600125 alleviated the inhibitory effect of IL-1beta on KCl- and agonist-induced protein secretion by 79% and 47%, respectively, and completely blocked the expression of iNOS. Treatment for 7 days with SP600125 increased tear production in a murine model of Sjögren's syndrome dry eye. We conclude that JNK plays a pivotal role in IL-1beta-mediated inhibition of lacrimal gland secretion and subsequent dry eye.
15700116 Anti-CENP-H antibodies in patients with Sjogren's syndrome. 2006 Feb Anti-centromere antibody (ACA) has been reported to be associated with Sjogren's syndrome (SS) and the clinical significance of anti-CENP-H antibody remains unknown. To determine the clinical significance of anti-CENP-H and anti-centromere antibodies in primary SS, sera from 62 patients with primary SS and 40 normal controls were examined for anti-SS-A/SS-B antibodies, ACA and anti-CENP-H antibodies, by enzyme-linked immunosorbent assay and indirect immunofluorescence (IIF), respectively. Of the 62 serum samples with primary SS, 17 were positive with ACA and anti-CENP-H antibodies. Sera from SS patients with anti-CENP-H and ACA antibodies do not contain anti-SS-A/Ro and/or anti-SS-B/La antibodies. No anti-CENP-H antibody was found in sera of normal controls. An increased frequency of ACA and anti-CENP-H antibodies was found for the first time in patients with SS. Anti-CENP-H antibodies and anti-SS-A/Ro or anti-SS-B/La antibodies are present mutually exclusive. Patients with anti-CENP-H antibodies had a lower frequency of rheumatoid factor (RF). SS can be subdivided serologically into two groups; group one with anti-SS-A/Ro and/or anti-SS-B/La antibody, group two with ACA and/or anti-CENP-H antibodies. We recommend that ACA or anti-CENP-H antibodies should be considered as one of the serological markers for SS.
18282173 Biologic treatments for systemic rheumatic diseases. 2008 Apr Many rheumatologic disorders, most notably Sjögren's syndrome, are associated with dental complications and in some cases oral diseases may trigger or drive connective tissue disease. During the past three decades the treatment in rheumatology was revolutionized by the introduction of disease-modifying anti-rheumatic drugs. Advances in our understanding of the pathogenesis of rheumatic diseases have led to the discovery of critical mechanisms of inflammation and autoimmunity and the invention of new target-specific biologic agents. In this review, we will summarize the current state of biologic therapies in rheumatology and discuss the implications of these on oral health and disease.
18089681 Decreased CD4+CD25+bright T cells in peripheral blood of patients with primary Sjogren's s 2008 Jan CD4+CD25+bright T cells played a crucial role in the suppression of immune response. Recently, decreased levels of CD4+CD25+bright T cells in the peripheral blood of patients with systemic lupus erythematosus were reported, suggesting the potential role of CD4+CD25+bright T cells in human autoimmune diseases. Primary Sjögren's syndrome (pSS) is another common human systemic autoimmune disease. The present study aimed to investigate the levels of CD4+CD25+bright T cells in pSS and to correlate their levels with some biomarkers of inflammation and immune activation. Thirty-three patients with pSS and 35 age- and sex-matched normal individuals were enrolled in the study. The flowcytometric method was applied in the measurement of CD4+CD25+bright T cells. The results showed that patients with pSS had statistically lower levels of CD4+CD25+bright T cells than normal controls, expressed either as absolute cell numbers (mean+/-SD: 47.07+/-25.53 cells/mm3 versus 79.55+/-34.56 cells/mm3, P<0.001) or as percentages of peripheral blood mononuclear cells (mean+/-SD: 2.79+/-1.06% versus 3.84+/-1.42%, P<0.001) or as percentages of CD4+ T cells (mean+/-SD: 7.85+/-2.62% versus 11.68+/-3.78%, P<0.005). Moreover, there were statistically significant inverse correlations between the levels of CD4+CD25+bright T cells and some parameters of inflammation or immune activation including erythrocyte sedimentation rate, C-reactive protein, IgG and rheumatoid factors. The result suggested that CD4+CD25+bright T cells were likely to play anti-inflammatory and immunosuppressive roles in the pathogenesis of pSS. However, the exact functions of decreased circulating CD4+CD25+bright T cells in pSS need further elucidated.
17407216 Toll-like receptor in salivary glands from patients with Sjögren's syndrome: functional a 2007 May OBJECTIVE: We investigated expression of toll-like receptor (TLR) in labial salivary glands of patients with Sjögren's syndrome (SS) and functional TLR expression in the cultured salivary gland cell line. METHODS: Expression of TLR2, TLR3, TLR4, and myeloid differentiation factor 88 (MyD88) in labial salivary glands was examined by immunohistochemistry. Human salivary gland (HSG) cell-line cells were cultured with TLR ligands [peptidoglycan, poly (I:C) and lipopolysaccharide], and CD54 expression and interleukin 6 (IL-6) production was studied. Phosphorylation of extracellular signal-regulated kinase (ERK), c-Jun N-terminal kinase (JNK), p38, and Akt was examined by Western blotting. Activation of nuclear factor-kappaB (NF-kappaB) p65 in HSG cells was studied by NF-kappaB p65 nuclear translocation by microscopic immunofluorescence or chemiluminescent electrophoretic mobility shift assay and detection of NF-kappaB p65 phosphorylation. RESULTS: TLR2, TLR3, TLR4, and MyD88 were more strongly expressed in the labial salivary glands of SS patients (n =12) than in control subjects (n = 4), and were found in salivary-infiltrating mononuclear cells as well as acinar cells and ductal epithelial cells. In cultured HSG cells, a similar expression pattern was observed, and TLR ligands stimulated CD54 expression and IL-6 production. TLR ligands induced phosphorylation of ERK, JNK, and p38 in HSG cells, but not Akt phosphorylation or activation of NF-kappaB p65. CONCLUSION: Although the putative ligands remain to be determined, our study indicated the activation of the TLR-mediated immune response in SS, and suggested that the TLR effect is mediated through the mitogen-activated protein kinase pathway.
17164992 A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease. 2006 Mikulicz's disease (MD) has been included within the diagnosis of primary Sjögren's syndrome (SS), but it represents a unique condition involving persistent enlargement of the lacrimal and salivary glands characterized by few autoimmune reactions and good responsiveness to glucocorticoids, leading to the recovery of gland function. Mikulicz's disease was recently reported to be associated with elevated immunoglobulin G4 (IgG4) concentrations in the serum and prominent infiltration of plasmacytes expressing IgG4 into the lacrimal and salivary glands. The following features were used for diagnosis: (1) visual confirmation of symmetrical and persistent swelling in more than two lacrimal and major salivary glands; (2) prominent mononuclear cell infiltration of lacrimal and salivary glands; and (3) exclusion of other diseases that present with glandular swelling, such as sarcoidosis and lymphoproliferative disease. These features are not observed in most SS cases. The complications of MD include autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel's thyroiditis, all of which show IgG4 involvement in their pathogenesis. Mikulicz's disease thus differs from SS and may be a systemic IgG4-related plasmacytic disease.
16729722 Interferon alpha and its contribution to autoimmunity. 2006 May It is now well accepted that interferon (IFN)alpha plays a critical role in the pathogenesis and perpetuation of specific autoimmune diseases, including systemic lupus erythematosus (SLE), autoimmune thyroid disease and type 1 diabetes. IFNalpha-based treatments are widely used for the treatment of chronic viral infections, particularly chronic hepatitis C virus infection; however, several case reports have emerged describing autoimmune conditions that have developed during IFNalpha therapy. The data support the pathogenic potential of IFNalpha in autoimmunity, although it is clear that genetic and environmental factors are also key to the development of autoimmune conditions. Several points of interaction between IFNalpha and immune effector cells have been experimentally defined, the functional consequences of many of which remain poorly understood. This review describes the most recent data in support of an important role for IFNalpha in autoimmunity, particularly SLE, and the potential mechanisms by which IFNalpha contributes to immune dysfunction. Future approaches to IFNalpha modulation as a therapeutic strategy for use in the treatment of autoimmune diseases are also discussed.
18050196 Augmented interferon-alpha pathway activation in patients with Sjögren's syndrome treated 2007 Dec OBJECTIVE: Recent clinical trials suggest that etanercept is ineffective in controlling Sjögren's syndrome (SS). To address the hypothesis that tumor necrosis factor blockade can result in increased levels of interferon-alpha (IFNalpha) and BAFF, we quantified those mediators in plasma from etanercept- and placebo-treated SS patients. METHODS: We studied plasma samples from 20 patients with SS treated with etanercept (25 mg twice weekly) or placebo in a 12-week, randomized, double-blind clinical trial. In addition, we studied plasma samples from 29 healthy controls. IFNalpha activity was determined by reporter cell assay, and BAFF levels were determined by enzyme-linked immunosorbent assay. RESULTS: Baseline IFNalpha plasma activity and BAFF levels were increased in SS patients compared with healthy controls (mean +/- SD IFNalpha plasma activity score 4.43 +/- 2.60 versus 2.08 +/- 0.91; P < 0.0001) (mean +/- SD BAFF level 0.83 +/- 0.27 ng/ml versus 0.60 +/- 0.15 ng/ml; P = 0.008). A significant increase in IFNalpha activity was detected after 12 weeks of treatment in the etanercept group, but not in the placebo group (P = 0.04 and P = 0.58, respectively). Furthermore, a statistically significant increase in BAFF levels was noted in patients receiving etanercept, but not in those receiving placebo (P = 0.01 and P = 0.56, respectively). In vitro culture of control peripheral blood mononuclear cells with etanercept resulted in a dose-dependent increase in the expression of IFNalpha and the IFNalpha-inducible genes IFN-induced protein with tetratricopeptide repeats 1 and BAFF. CONCLUSION: IFNalpha activity and BAFF levels are elevated in the plasma of patients with SS compared with healthy controls. Etanercept treatment exacerbates IFNalpha and BAFF overexpression, providing a possible explanation for the lack of efficacy of this agent in SS.
17907208 Mapping hand functioning in hand osteoarthritis: comparing self-report instruments with a 2007 Oct 15 OBJECTIVE: To determine which self-report instruments best explain hand functioning measured by a generic comprehensive hand function test. METHODS: Six questionnaires currently used in hand osteoarthritis (OA), namely, the Arthritis Impact Measurement Scales 2 Short Form (AIMS2-SF), the Australian/Canadian Osteoarthritis Hand Index (AUSCAN), the Cochin scale, the Functional Index of Hand OA (FIHOA), the Health Assessment Questionnaire (HAQ), and the Score for Assessment and Quantification of Chronic Rheumatoid Affections of the Hands (SACRAH), were administered once in 100 patients with hand OA together with the Jebsen-Taylor Hand Function Test (JTHFT). In addition, 3 other hand function tests with short administration time were used: the Moberg Picking-Up Test (MPUT), the Button Test (BT), and grip strength. The Short Form 36 was used to describe health status. The relationship between the instruments and the JTHFT was determined by correlation analyses. RESULTS: AIMS2-SF total scores had the highest raw correlation coefficient to the JTHFT, followed by AIMS2-SF upper body limitation subscale, SACRAH stiffness subscale, and SACRAH total score. If controlled for age, the HAQ had the highest correlation coefficient. Of the 3 short hand function tests, the MPUT showed the highest raw correlation coefficient to the JTHFT; if controlled for age, the BT had the highest correlation coefficient. CONCLUSION: To comprehensively assess hand functioning in patients with hand OA, we recommend using both a self-report instrument used more generally in various arthritides and a self-report instrument specifically developed for hand OA. If a short test is preferred, we recommend using the MPUT or BT.
18086728 A novel TNFRSF1A splice mutation associated with increased nuclear factor kappaB (NF-kappa 2008 Nov OBJECTIVE: To characterise and investigate the functional consequences of a novel TNFRSF1A splice site mutation causing tumour necrosis factor receptor associated periodic syndrome (TRAPS) in a 16-year-old male patient and his mother. METHODS: Mutational DNA screening was performed in the patient and his mother. Western blotting was used to analyse protein expression levels of TNFR1. A multiplex bead immunoassay was used to quantify serum levels of range of cytokines, and an ELISA-based transcription factor assay to measure nuclear factor (NF)-kappaB transactivation. Serum levels of soluble TNFR1 (sTNFR1) were measured by ELISA and fluorescence-activated cell sorting (FACS) analysis used to measure monocyte TNFR1 cell surface expression. RESULTS: A novel mutation, c.472+1G>A (C158delinsYERSSPEAKPSPHPRG), involving a splice site in intron 4 of TNFRSF1A, was found in the proband and affected mother leading to a 45 nucleotide insertion of intronic DNA into the mRNA, resulting in an in-frame insertion of 15 amino acids in the mature TNFR1 protein and a deletion of a cysteine residue C129 (158) in cysteine rich domain (CRD)3. The patients had reduced serum sTNFR1 and surface expression levels of TNFR1, with marked increases in pro- and anti-inflammatory cytokine. Their peripheral blood mononuclear cells (PBMC) had increased basal NF-kappaB activation compared with healthy controls and also had increased p50 nuclear expression following tumour necrosis factor (TNF) stimulation compared with PBMC from healthy controls, as well as T50M (T79M) and C88R (C117R) patients with TRAPS and patients with rheumatoid arthritis (RA). CONCLUSION: A novel, TRAPS causing, TNFRSF1A splice site mutation is associated with decreased sTNFR1 levels, cell surface and whole cell extract expression and increased NF-kappaB transcription factor activation.
17785864 Interaction between transmembrane TNF and TNFR1/2 mediates the activation of monocytes by 2007 Sep 15 Monocytes and monocytic cells produce proinflammatory cytokines upon direct cell contact with activated T cells. In the autoimmune disease rheumatoid arthritis, the pivotal role of TNF-alpha implies that the interaction between transmembrane TNF-alpha (mTNF) and the TNF receptors (TNFR1 and TNFR2) might participate in the T cell contact-dependent activation of monocytes. Accordingly, treatment of rheumatoid arthritis by administration of a TNF-alpha-blocking Ab was found to significantly decrease TNF-alpha production by monocytes. Several lines of evidence indicated that signaling through TNFR1/2 and through mTNF (reverse signaling) is involved in TNF-alpha production by monocytes after T cell contact: 1) blocking mTNF on activated T cells leads to a significant reduction in TNF-alpha production; 2) down-regulation of TNFR1/2 on monocytes by transfection with small interfering RNA results in diminished TNF-alpha production; 3) blocking or down-regulating TNFR2 on activated T cells inhibits TNF-alpha production, indicating that mTNF on the monocyte surface mediates signaling; 4) ligation of mTNF on monocytes by surface TNFR2 transfected into resting T cells induces TNF-alpha production due to reverse signaling by mTNF; and 5) ligation of mTNF on monocytes by a soluble TNFR2:Ig receptor construct induces TNF-alpha production due to reverse signaling. In conclusion, we identified mTNF and TNFR1/2 as interaction partners contributing to TNF-alpha production in monocytes. Both pathways initiated by mTNF-TNFR interaction are likely to be inhibited by treatment with anti-TNF-alpha Abs.
17407230 Anti-dsDNA antibody assay: high specificity and sensitivity with a filtration radioassay i 2007 Apr OBJECTIVE: To evaluate whether a new fluid-phase filtration radioassay possesses both high sensitivity and specificity compared with the currently used ELISA and Farr assays. METHODS: Sequential sera (25 samples) from 9 patients with systemic lupus erythematosus (SLE), sera from 20 patients with SLE possessing anti-dsDNA antibodies by the Crithidia assay, 75 patients with rheumatoid arthritis possessing rheumatoid factors, 50 healthy control subjects, 767 from patients with type 1 diabetes, and a commercial standard serum sample were tested for anti-dsDNA antibodies with the 3 different assays. RESULTS: Of serial dilutions of a standard anti-dsDNA antibody sample, only the highest positive sample (50 IU/ml) in the ELISA and the highest 2 positive samples (50 and 25 IU/ml) in the Farr assay were above the normal range. In contrast, all dilutions (to 2.5 IU/ml) of the standard anti-dsDNA antibody sample were above the normal range in the filtration radioassay. Using the values of 50 healthy control subjects in each assay to define the normal range, all 25 sequential sera from 9 patients with SLE were positive. In addition, 20/20 of the SLE individual sera, 2/75 (2.7%) of the RA sera, and 12/767 (1.6%) of the diabetes sera were positive (signal above normal range) in the filtration radioassay. The SLE sera were further examined in 2 additional assays, ELISA and Farr assay, and both assays were less sensitive and specific compared with the filtration radioassay. CONCLUSION: The fluid-phase filtration radioassay demonstrated high sensitivity and specificity for the detection of anti-dsDNA antibodies in SLE, with the standard ELISA exhibiting lower specificity. We suggest that testing for anti-dsDNA antibodies can be improved using a fluid-phase filtration radioassay in comparison to commercial assays.
18801600 [Kappa light chain deposition disease, presenting as Sjögren's syndrome, successfully tre 2009 Jan INTRODUCTION: Light chain deposition disease is a systemic disorder characterised by tissue deposition of monoclonal immunoglobulin light chains without tinctorial properties. It has been exceptionally reported with salivary involvement mimicking Sjögren's syndrome and peripheral neuropathy. CASE REPORT: We report a case of light chain deposition disease associated with plasma cell dyscrasia presenting as sicca syndrome with salivary glands hypertrophy and polyneuropathy successfully treated by high dose melphalan and autologous blood stem transplantation. CONCLUSION: Light chain deposition disease should be recognized as an aetiology of sicca syndrome and peripheral neuropathy. Further studies should assess the prevalence of sicca syndrome in light chain deposition disease and better characterise the neurological manifestations.
17584275 Optimal pacing in congenital complete atrioventricular block of immunological origin: inte 2007 Jul An infant with a congenital auriculoventricular block (CAVB) of immunological origin was diagnosed prenatally. The mother had Gougerot-Sjögren disease with positive anti-Sjogren's Syndrome A (SSA) and Sjogren's Syndrome B (SSB) serologies. Cardiac pacing was necessary and the epicardial route was chosen. Considering the left ventricular (LV) dilatation, bi-ventricular (BiV) stimulation was preferred to the usual DDD mode, presumed to have a deleterious long-term effect. Echographic parameters were better with BiV stimulation: the asynchronism induced by mono-RV stimulation was corrected and the QRS complexes were narrower. BiV pacing of a CAVB with LV dilation looks clinically and echographically attractive but needs to be validated in the long term.
18288023 [Soft tissue tumors and pseudotumors of the foot and ankle]. 2008 Jan Soft tissue masses around the foot and ankle are frequent. While benign lesions are two times more frequent than malignant lesions, the latter still represent one third of all lesions. The main purpose of this article is to propose a systematic approach to the differential diagnosis of soft tissue tumors of the foot and ankle based on a combination of 5 elements: clinical history and physical examination, top 10 most frequent diagnoses, patient age, lesion location, and MRI features of the mass. Selected soft tissue tumors will be described and illustrated with emphasis on these 5 elements.
17071741 Sjögren's syndrome-like disease in mice with T cells lacking class 1A phosphoinositide-3- 2006 Nov 7 Sjögren's syndrome (SS) is an autoimmune disease that is characterized by infiltration of exocrine tissues, resulting in xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes). Here, we show that mice with T cell-specific loss of class IA phosphoinositide 3-kinase function develop organ-specific autoimmunity that resembles the human disease SS. Most mutant mice aged 3-8 months develop corneal opacity and eye lesions due to irritation and constant scratching. These mice display cardinal signs of primary SS such as marked lymphocytic infiltration of the lacrimal glands, antinuclear antibodies in the serum, and elevated titer of anti-SS-A antibody, in the absence of kidney pathology. Immunofluorescence studies show the presence of numerous CD4+ T cells with a smaller number of CD8+ T cells and B cells in the lacrimal glands. CD4+ T cells from these mice exhibit aberrant differentiation in vitro. These results indicate that aberrant T cells with impaired class IA phosphoinositide 3-kinase signaling can lead to organ-specific autoimmunity. In addition, the mouse model described here represents a tool to study the pathogenesis and treatment of SS.
16802367 Identification of transitional type II B cells in the salivary glands of patients with Sjà 2006 Jul OBJECTIVE: To identify B cell subpopulations participating in the lymphocyte infiltrate of salivary glands from patients with primary Sjögren's syndrome. A special emphasis was placed on those B lymphocytes included in the ectopic germinal centers (GCs). METHODS: The presence of B cells in salivary glands and their polyclonality were ascertained by phenotyping and reverse transcription-polymerase chain reaction in salivary gland samples from 18 patients. Their phenotype was thoroughly analyzed using a number of double-staining combinations. The results obtained in tissue sections were confirmed by fluorescence-activated cell sorting analysis of B cells eluted from salivary glands, and these findings were compared with those in tonsils. RESULTS: Memory-type B cells were defined as CD20+, CD27+ and were seen in all specimens, whereas GCs were found in only 7 specimens. Furthermore, B cells found in these GCs lacked certain characteristics of centroblasts and centrocytes. Instead, they fulfilled the criteria for transitional type II (TII) B cells and resembled marginal-zone B cells. BAFF (the assistance of which is required for proper transformation of transitional TI B cells into transitional TII B cells) accumulated adjacent to transitional and marginal-zone-like B lymphocytes. Further evidence for the involvement of BAFF came from the expression of its receptors on infiltrating B cells. CONCLUSION: These transitional TII and marginal-zone-like B cells are probably instrumental in the local production of autoantibodies and possibly influential in the ensuing destruction of epithelial cells.
16385503 BAFF-induced changes in B cell antigen receptor-containing lipid rafts in Sjögren's syndr 2006 Jan OBJECTIVE: To determine the effect of excessive production of BAFF on the distribution and function of B cell subsets in patients with primary Sjögren's syndrome (SS). METHODS: The phenotype of B lymphocytes was analyzed by flow cytometry. Differences in the expression level of membrane IgD and CD38 were used to identify B lymphocyte subsets evolving from naive Bm1 through memory Bm5 cells. Based on our finding of a low expression of CD45RA, we sorted Bm2/Bm2' cells to determine the time course of translocation of the CD19 molecule and the B cell receptor into lipid rafts, by confocal microscopy. Serum levels of BAFF were measured by an enzyme-linked immunosorbent assay developed in-house. RESULTS: "Circulating" Bm2/Bm2' cells were expanded in patients with primary SS compared with rheumatic disease controls and with normal controls. In addition, these B cell subsets were functionally abnormal. Prolonged residency of the B cell receptor in lipid rafts in these cells was associated with elevated CD19 expression in B cells, most notably, Bm2 and Bm2' cells, obtained from the patients with primary SS. BAFF levels were higher in the patients than in the normal controls and correlated with the percentage of Bm2/Bm2' cells and their expression of CD19 in primary SS patients. These correlations were confirmed by placing sorted Bm1 or Bm2 cells from normal controls in culture in the presence or absence of BAFF. CONCLUSION: Bm2/Bm2' cells express more CD19 molecules in primary SS patients than in normal controls. BAFF might participate in this elevated expression of CD19. These patients might be suitable candidates for treatment with BAFF antagonists.
17905783 Prevalence of Sjogren syndrome among Nagasaki atomic bomb survivors. 2008 May OBJECTIVES: Through a comprehensive epidemiological study, we determined Sjögren syndrome (SS) prevalence and examined the association between SS and ionising radiation dose. METHODS: A total of 1008 atomic bomb survivors in Nagasaki agreed to undergo the tests comprising a questionnaire for xerophthalmia and xerostomia, Schirmer-I test, Saxon test, and tests of anti-SS-A/Ro and anti-SS-B/La antibodies, and, if necessary, Rose Bengal stain test, salivary ultrasonographic and MRI examination from November 2002 through October 2004. Diagnosis of SS was based on the American-European Consensus Group criteria, or a modified version thereof. RESULTS: Among the 1008 participants (male 398, female 610, average age 71.6 years), 154 participants (15.3%) complained of xerophthalmia, and 264 (26.2%) of xerostomia. Reduced tear flow as assessed by the Schirmer-I test was detected in 371 of 992 participants (37.4%) and reduced saliva flow as assessed by the Saxon test in 203 of 993 participants (20.4%). Among all participants, 38 (3.8%) and 10 (1.0%) participants tested positive for anti-SS-A/Ro and anti-SS-B/La antibodies, respectively. Taking into consideration all the results, 23 participants were diagnosed with SS (primary 20, secondary 3), yielding a prevalence of 2.3%. Although the association between SS and radiation dose was not significant, radiation dose was significantly associated with hyposalivation. CONCLUSIONS: The present comprehensive epidemiological study reveals that the prevalence of SS was 2.3% among Nagasaki atomic bomb survivors and was not associated with radiation dose. The association between radiation dose and hyposalivation supported the possibility that radiation exposure damaged salivary gland function.