Browse

Search for: rheumatoid arthritis    methotrexate    autoimmune disease    biomarker    gene expression    GWAS    HLA genes    non-HLA genes   

ID PMID Title PublicationDate abstract
26935363 Is There an Increased Arterial Stiffness in Patients with Primary Sjögren's Syndrome? 2016 OBJECTIVE: Primary Sjögren's syndrome (pSS) is a common chronic autoimmune disease that primarily affects the salivary and lacrimal glands. Arterial stiffness is one of the earliest detectable manifestations of adverse structural and functional changes within the vessel wall. The aim of this study was to evaluate the relationship between arterial stiffness and pSS. METHODS: In this study, 75 female patients with pSS who fulfilled the American European Consensus Criteria for Sjögren's syndrome, were included. A total of 68 age-, sex- and body mass index-matched subjects were recruited as the control population. Arterial stiffness was assessed by measurement of the carotid-femoral pulse wave velocity (PWV). RESULTS: The mean age of the patients was 54.0±9.3 years and the median duration of the disease was 10 years. Compared with the control subjects, patients with pSS had a higher mean PWV (8.2±1.5 m/s vs. 7.5±1.4 m/s; p=0.01). Correlation analysis showed that the PWV was positively correlated with age, body mass index, serum cholesterol, low-density lipoprotein (LDL) and C-reactive protein levels, blood pressure, mean arterial pressure (MAP), pulse pressure and left ventricular mass index. A multiple linear regression analysis revealed that arterial stiffness was associated with age, MAP and LDL levels in pSS patients. CONCLUSION: Although patients with pSS appear to have increased arterial stiffness, risk factors associated with arterial stiffness in these patients are similar to the general population. However, we cannot exclude the possibility that a higher PWV in pSS patients is caused, not by pSS itself, but by the use of steroids, hypertension and dyslipidemia.
26834930 Epidemiology and outcome of articular complications in adult onset Still's disease. 2015 The adult onset Still's disease is a rare inflammatory pathology of unknown pathogeny. The clinical features are variable. The diagnosis is difficult since exclusion of infectious, systemic and tumoral pathologies should be done. The articular complications are frequent and can be revelatory of this pathology. The articular prognosis depends on the diagnosis delay and the treatment efficiency. Our study aims to analyze different aspects of articular manifestations complicating adult onset Still disease to define epidemiological, clinical and evolving characteristics of these complications. It was a cross-sectional study concerning 18 cases of adult onset Still disease diagnosed from 1990 to 2014 in the internal medicine A department of Charles Nicolle Hospital in Tunis, meeting Yamaguchi criteria. We identified clinical, radiological, evolving and therapeutic profile of the articular manifestations occurred in these patients. There were 11 women and 7 men. The average age was 27 years. The arthralgias were reported in all cases; while, the arthritis interested thirteen patients. A hand deformation was found in four patients. A wrist ankylosis was noted in one case and a flexion elbow in one patient. The Standard articular radiographs were normal in ten cases. The treatment associated essentially non-steroidal anti-inflammatory and/or corticosteroids and/or methotrexate. Concerning the evolving profile, the monocyclic form was present in 25% of the cases, the intermittent form in 40% and the chronic articular form in 35% of our patients. The adult onset Still's disease is rare and heterogeneous. The articular disturbances are frequent and have various outcomes.
26315557 Main causes and risk factors for hospitalisation in patients with primary Sjögren's syndr 2015 Sep OBJECTIVES: To identify the causes and risk factors for hospitalisation in primary Sjögren's syndrome (pSS). METHODS: We included 170 pSS patients who regularly attended our Institution (2000-2013) and retrospectively collected demographic, clinical (glandular and extraglandular features) and serological (anti-Ro/SSA, anti-La/SSB, RF, low C3 or C4 and immunoglobulin levels) data. If they were hospitalised, a rheumatologist determined the primary cause. We registered the length of hospitalisation, need for Intensive Care Unit (ICU) admission, number of hospitalisations and death. The Disease Damage Index (SSDDI) (excluding the oral and ocular items) and the Charlson comorbidity Index were assessed. We used a logistic regression analysis and multiple imputation method for missing data. RESULTS: Fifty-five (32%) patients were hospitalised, representing 111 hospitalisations (28 patients had ≥1 hospital admission). The hospitalisation incidence density rate was 6.49/100 patient / years. The median length of hospital stay was 9 days (IQR 6-15), there were 7 ICU admissions and 6 deaths. The main causes of admissions were disease activity (33.3%) and infection (32.4%). At the multivariate analysis, the variables associated with hospitalisation were hepatic involvement (OR=5.4; 95% CI 1.61-18.15; p=0.006), vasculitis (OR=3.8; 95% CI 1.11-13.09; p=0.03), the SSDDI (OR=1.3; 95% CI 1.01-1.66; p=0.03) and the use of antimalarials (OR=0.08; 95% CI 0.02-0.22; p<0.001). CONCLUSIONS: The major causes for hospitalisation were disease activity and infection. Patients with hepatic involvement, vasculitis and more damage accrual had the highest risk for being hospitalised, while the use of antimalarials was protective.
26271272 Endogenous event-related potentials in patients with primary Sjögren's syndrome without c 2015 OBJECTIVES: Endogenous cognitive event-related potentials (CERPs) reflect higher-level processing of sensory information and can be used to evaluate cognitive functions. The aim of this paper was to determine whether there are any abnormalities in the electrophysiological parameters of CERPs in patients with primary Sjögren's syndrome (pSS) but without symptoms of central nervous system (CNS) involvement or mental disorder. The analysis of CERP parameters was then correlated with the clinical status of the patients and with some of the immunological parameters in the patient group. METHOD: Thirty consecutive patients with pSS (29 females, one male) were included in the study. All the patients underwent CERP examination. RESULTS: There was a significant prolongation of the latency of P300 and N200 potentials in patients with pSS. Abnormalities in electrophysiological parameters of CERPs correlated with the duration of the disease, salivary gland abnormalities, and elevated erythrocyte sedimentation rate (ESR) values. Patients with coexisting chronic fatigue syndrome (CFS) had larger P300 amplitudes. There were no statistically significant changes in the electrophysiological parameters of CERPs in patients with pSS dependent on the presence of peripheral nervous system (PNS) lesions, skin changes, arthritis, abnormalities in white blood cells and the immune system or the levels of blood lipids. CONCLUSIONS: The results of the study suggest the presence of a minor cognitive dysfunction in patients with pSS without symptoms of CNS involvement or mental disorder. Cognitive dysfunction correlated with the disease duration time and the severity of inflammatory changes (salivary gland abnormalities and inflammatory markers in the blood). Further and larger longitudinal studies are necessary for confirmation of this correlation.
25396761 Acquired inhibitors to factor VIII and fibrinogen in the setting of T-cell large granular 2015 Mar Large granular lymphocyte (LGL) leukemia is an indolent lymphoproliferative malignancy which dysregulates humoral immunity and underlies the myriad autoimmune phenomena. We describe a 62-year-old woman with Felty's syndrome who developed a severe bleeding diathesis. Laboratory evaluation demonstrated acquired inhibitors to both factor VIII (FVIII) and fibrinogen, likely secondary to T-cell LGL leukemia. After a complicated course, the patient's inhibitors were extinguished with rituximab and high-dose corticosteroids. Bleeding was controlled with alternating FEIBA (factor eight inhibitor bypassing activity) and recombinant activated FVII. This report reviews the literature comparing the efficacy of various treatment modalities for both disorders. To our knowledge, this is the first reported case of a patient with LGL leukemia acquiring an inhibitor to FVIII or fibrinogen.
25316606 Sjögren Syndrome-associated lymphomas: an update on pathogenesis and management. 2015 Feb Primary Sjögren Syndrome (pSS) is an autoimmune disease associated with an increased risk of lymphoma. Lymphomas complicating pSS are mostly low-grade B cell non-Hodgkin lymphomas, predominantly of marginal zone histological type. Mucosal localization is predominant, notably mucosa-associated lymphoid tissue lymphomas. Lymphomas often develop in organs where pSS is active, such as salivary glands. Germinal centre (GC)-like structures, high TNFSF13B (BAFF) and Flt3-ligand (FLT3LG) levels and genetic impairment of TNFAIP3 are new predictors of lymphoma development. These new findings allow a better understanding of the pathogenic mechanisms leading to lymphoma. We propose the following scenario: auto-immune B cells with rheumatoid factor (RF) activity are continuously stimulated by immune complexes containing antibodies against more specific auto-antigens, such as SSA/Ro, SSB/La or others. Germline abnormality of TNFAIP3 leads to a decreased control of the NF-kB pathway and thus promotes survival of B cells and oncogenic mutations especially in GC structure. Moreover, B cells are stimulated by a positive loop of activation induced by BAFF secretion. Thus, lymphomagenesis associated with pSS exemplifies the development of antigen-driven B-cell lymphoma. The control of disease activity by a well-targeted immunosuppressor is the primary objective of the management of the patient in order to repress chronic B cell stimulation.
25682089 The coexistence of Sjögren's syndrome and primary biliary cirrhosis: a comprehensive revi 2015 Jun Organ-specific and systemic autoimmune diseases share numerous features and often coexist in the same patient. Autoimmune cholangitis/primary biliary cirrhosis and Sjogren syndrome represent paradigmatic examples of the common grounds of different autoimmunity phenotypes based on similarities in clinical manifestations and immunopathogenesis. In fact, primary biliary cirrhosis and Sjogren's syndrome have both been coined as an autoimmune epithelitis in which apoptosis may be in both cases the key element to explain the organ-specific immune-mediated injury against the biliary and exocrine gland epithelia, respectively. Further, growing evidence supports in both diseases the view that B cells, T cytotoxic cells, and T helper cells are involved in chronic inflammation, likely via the altered expression of pro-inflammatory cytokines. The presence of estrogen receptors on the biliary and exocrine gland epithelia has been advocated as a key to the female predominance encountered in primary biliary cirrhosis and Sjogren's syndrome. Sadly, despite available data, therapeutic approaches remain largely unsatisfactory and recent studies with mechanistic approaches (as in the case of B cell depletion with rituximab) have been of partial benefit only. Future studies should focus on new molecular tools (single-cell transcriptomics, microRNA, epigenetics) to provide unique insights into common mechanisms.
30641625 [Efficay Observation of Xinfeng Capsule for Treating 58 Cases of Sjogren's Syndrome]. 2016 Nov Objective To observe the clinical effect of Xinfeng Capsule (XFC) in treating Sjögren's syndrome (SS) and its effect on coagulation parameters, peripheral blood cytokines, and NF- kappa B signaling pathway protein. Methods Totally 58 SS patients were assigned to the treatment group and the control group according to random digit table, 29 cases in each group. Patients in the treat- ment group took XFC, 3 pills each time, 3 times per day. Those in the control group took Hydroxychloro- quine ( HCQ) Sulfate, 0. 1 g per tablet, 2 tablets each time, twice per day. Three months consisted of one therapeutic course and one course for all. Another 20 healthy subjects were recruited as a normal control group. Coagulation parameters (APTT, PT, FIB,TT, D-D) were detected using automatic coagulation an- alyzer in the two groups before and after treatment as well as in the healthy control group. The expres- sions of peripheral blood cytokines (IL-1β, TNF-α, IL-10) and NF-κB signaling pathway proteins (P65, P50, IκBα) were detected before and after treatment using ELISA. Erythrocyte sedimentation rate (ESR) was determined using Westergren method before and after treatment. High sensitivity C-reactive protein (hs-CRP) level was determined using automatic biochemical analyzer before and after treatment. Results Totally 44 SS patients had abnormal coagulation parameters, accounting for 75. 9% of the total. Com- pared with the healthy control group, the levels of D-D, FIB, IL-1 , TNF-α,P50, P65, IκBα, hs-CRP, and ESR increased (P <0.05, P <0. 01), and the IL-10 level decreased in SS patient groups (P <0.01). Spearman correlation analysis showed that coagulation parameters were significantly correlated with cy- tokines, NF-κB signal transduction pathway, and inflammation indices (P <0. 01 , P <0. 05). After drug in- tervention blood coagulation parameters and laboratory indices were partially improved in the two groups. The effective rate and the total effective rate were 59% and 86% in the treatment group, obviously higher than those of the control group with statistical difference (38% and 72%; P <0. 05). Besides, the treat- ment group was superior to the control group in reducing the levels of FIB, D-D, P50, P65, ESR, and hs- CRP, down-regulating levels of TNF-α and IL-1 β, as well as up-regulating the expression of IL-10 (P < 0. 05, P <0. 01). Conclusions There is a hypercoagulable state in SS patients, which is related to abnor- mal activation of cytokines/NF-kappa B signaling pathway. XFC could effectively improve the hypercoagulative state of SS patients. Its mechanism might be related to inhibiting cytokines/NF-κB signaling pathway.
27835913 Circulation autoantibody against Lamin A/C in patients with Sjögren's syndrome. 2016 Dec 6 Lamin A/C proteins are major components of nuclear laminae and were encoded by the LMNA gene. Recent studies have found that in addition to provides nuclear-membrane strength; it also regulates the gene expression. Lamin A/C has been confirmed as an autoantigen in RA, SLE and vasculitis. Anti-Lamin A/C antibodies also have been found by indirect immunofluorescence method. In this study, we used various research methods to confirm Lamin A/C is an autoantigen in Han Chinese patients with confirmed Sjögren's syndrome (SS). To further investigate the relationship between the autoimmune disease antigens, we compared the amino acid sequence of Lamin A/C epitope and several common antigens' antigenic determinant. As a result, we found that Lamin A/C has similar epitopes with U1RNP. It means that the potential relationship exist between Lamin A/C and U1RNP. Clinical data we collected also showed that anti-Lamin A/C and anti-U1RNP antibodies always appear in same serum sample. Therefore, we speculated that cross-reaction may take place between antigen and potential antigen, which have similar epitope. Then, by epitope spreading, the potential antigen can be a new autoantigen. Our study provided a new thinking for further research about the relationship between autoantigens and their development mechanism in autoimmune diseases.
27817092 Comparisons of presentations and outcomes of neuromyelitis optica patients with and withou 2017 Feb Patients with neuromyelitis optica (NMO) often have an accompanying autoimmune disease, most commonly, but not limited to Sjögren's syndrome (SS). The aim of this study was to compare clinical and laboratory features between NMO patients with and without SS and to investigate the prognosis of NMO in patients with and without SS. Twenty-three NMO patients with SS and 42 NMO patients without SS were included. Clinical and laboratory profiles were compared, including annual relapse rate and time from onset of NMO to Expanded Disability Status Scale (EDSS) scores of 4.0 and 6.0. More NMO patients with SS than those without SS had anti-nuclear antibody, anti-SS-A/Ro and anti-SS-B/La antibodies (91.3 vs. 35.7%, p < 0.001, 87.0 vs. 2.3%, p < 0.001, and 34.8 vs. 0.0%, p < 0.001, respectively). Serum immunoglobulins (IgA, IgM and IgG) were markedly increased in NMO patients with SS in comparison with those without SS. Annual relapse rate and the time from disease onset to an EDSS score of 4.0 and 6.0 were not significantly different between the two groups. No differences between the two groups were found for the other parameters, including AQP-4 antibody status, length of spinal cord lesion and brain lesions. These results imply that NMO in SS more likely represents coexistence with SS rather than representing the result of direct central nervous system involvement in SS. Autoimmune response appears to be more intense in the NMO group with SS, but did not cause a more severe prognosis in comparison with the group without SS, indicating that we should pay attention to the potential benefit of the antinuclear antibodies in NMO.
25936787 Ultrasonographic evaluation of major salivary glands in primary Sjögren's syndrome: compa 2015 Sep OBJECTIVE: To assess the diagnostic value of salivary gland ultrasonography (SGUS) for primary SS (pSS) and to compare the usefulness of two existing SGUS scoring systems. METHODS: Ultrasonography examination of major salivary glands was conducted for 105 pSS patients and 41 disease control subjects without SS and 16 healthy control subjects. The imaging features were graded using two different scoring systems (0-16 and 0-48, respectively) obtained from the grades of bilateral parotid and submandibular glands. Receiver operating characteristic curves were used to describe and compare the diagnostic accuracy of the two ultrasonography echostructure scoring systems for pSS. The agreement of diagnosis for pSS between the two scoring systems was determined by κ-statistics. RESULTS: SGUS scores for the pSS group were significantly higher than those for the non-pSS group (P < 0.001). The best score cut-off was 7 in the 0-16 system (80% sensitivity and 93% specificity, respectively), and it was 15 in the 0-48 system (88.6% sensitivity and 84.2% specificity, respectively). Compared with the 0-16 system, combined evaluation of all four glands when using the 0-48 system improved the diagnostic accuracy. Association analysis of both scoring systems showed a positive correlation of SGUS scores with RF and γ-globulin% (P < 0.05, overall). CONCLUSION: SGUS is a feasible method for pSS diagnosis with higher sensitivity using the 0-48 system and better specificity using the 0-16 system. SGUS scores are related to RF and γ-globulin%.
27015600 Updated Projected Prevalence of Self-Reported Doctor-Diagnosed Arthritis and Arthritis-Att 2016 Jul OBJECTIVE: To update the projected prevalence of arthritis and arthritis-attributable activity limitations among US adults, using a newer baseline for estimates. METHODS: Baseline prevalence data were obtained from the 2010-2012 National Health Interview Survey. Arthritis was defined as an answer of "yes" to the question "Have you ever been told by a doctor or other health professional that you have some form of arthritis, rheumatoid arthritis, gout, lupus or fibromyalgia?" Arthritis-attributable activity limitation was defined as an answer of "yes" to the question "Are you limited in any way in any of your usual activities because of arthritis or joint symptoms?" The baseline prevalence of arthritis and arthritis-attributable activity limitation was stratified according to age and sex and was statistically weighted to account for the complex survey design. The projected prevalence of doctor-diagnosed arthritis and arthritis-attributable activity limitation was calculated by multiplying the age- and sex-stratified population estimates projected for 2015-2040 (in 5-year intervals; provided by the US Census Bureau) by the baseline estimates. Age- and sex-specific prevalences were summed to provide the total prevalence estimates for each year. RESULTS: In 2010-2012, 52.5 million adults in the US (22.7% of all adults) had doctor-diagnosed arthritis, and 22.7 million (9.8%) had arthritis-attributable activity limitation. By 2040, the number of US adults with doctor-diagnosed arthritis is projected to increase 49% to 78.4 million (25.9% of all adults), and the number of adults with arthritis-attributable activity limitation will increase 52% to 34.6 million (11.4% of all adults). CONCLUSION: Updated projections suggest that arthritis and arthritis-attributable activity limitation will remain large and growing problems for clinical and public health systems, which must plan and create policies and resources to address these future needs.
27852561 Pixel or Paper? Validation of a Mobile Technology for Collecting Patient-Reported Outcomes 2016 Nov 16 BACKGROUND: In the management of chronic disease, new models for telemonitoring of patients combined with the choice of electronic patient-reported outcomes (ePRO) are being encouraged, with a clear improvement of both patients' and parents' quality of life. An Italian study demonstrated that ePRO were welcome in patients with rheumatoid arthritis (RA), with excellent matching data. OBJECTIVE: The aim of this study is to evaluate the level of agreement between electronic and paper-and-pencil questionnaire responses. METHODS: This is an observational prospective study. Patients were randomly assigned to first complete the questionnaire by paper and pencil and then by tablet or in the opposite order. The questionnaire consisted of 3 independent self-assessment visual rating scales (Visual Analog Scale, Global Health score, Patient Global Assessment of Disease Activity) commonly used in different adult patients, including those with rheumatic diseases. RESULTS: A total of 185 consecutive RA patients were admitted to hospital and were enrolled and completed the questionnaire both on paper and on electronic versions. For all the evaluated items, the intrarater degree of agreement between 2 approaches was found to be excellent (intraclass correlation coefficient>0.75, P<.001). CONCLUSIONS: An electronic questionnaire is uploaded in a dedicated Web-based tool that could implement a telemonitoring system aimed at improving the follow-up of RA patients. High intrarater reliability between paper and electronic methods of data collection encourage the use of a new digital app with consequent benefit for the overall health care system.
27987339 Evaluation of ovarian reserve using anti-müllerian hormone and antral follicle count in S 2017 Feb AIM: The aim of this study was to determine ovarian reserve status using anti-müllerian hormone (AMH) level and antral follicle count (AFC) in patients with Sjögren's syndrome (SS). METHODS: Twenty-four women with SS diagnosed according to the classification criteria proposed by the American-European Consensus Group and 25 healthy women as controls were enrolled in this study. Ovarian reserve was assessed on clinical findings, AFC, and serum AMH and reproductive hormone levels. RESULTS: Compared with the healthy controls, in the SS patients, the duration of menstrual cycle was significantly shorter (P = 0.043); serum AMH (P = 0.001) and AFC (P = 0.001) were significantly lower, and serum luteinizing hormone (LH) was significantly higher (P = 0.019). The right (P = 0.555) and left ovarian (P = 0.386) volumes were also lower but this did not reach statistical significance. Serum follicle-stimulating hormone (P = 0.327), estradiol (P = 0.241), and prolactin (P = 0.55) were similar between the two groups. CONCLUSIONS: Ovarian reserve may be reduced in SS patients. For the assessment of ovarian reserve, serum AMH and ovarian AFC with serum LH may be useful. Further studies with long-term follow-up are required to determine the course of ovarian reserve abnormalities and best possible biomarkers of reduced ovarian reserve in SS patients.
27682894 Interstitial lung disease in primary Sjögren's syndrome. 2017 Jan Interstitial lung disease (ILD) has been reported in 3 to 11% of patients with primary Sjögren's syndrome (pSS). The aims of this retrospective multicenter study were to: 1) analyze characteristics and outcome of ILD in pSS; and 2) evaluate predictive factors associated with ILD onset and deterioration. Twenty-one of 263 patients with pSS (8%) developed ILD. ILD onset preceded pSS diagnosis (n=5), was concurrently identified in association with pSS (n=6) and developed after pSS onset (n=9). Presenting ILD manifestations were: acute/subacute (n=11) onset of ILD, symptomatic progressive onset of ILD (n=5), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT-scan (n=5). ILD therapy included: steroids (n=21), cyclophosphamide (n=1), azathioprine (n=4) and rituximab (n=1). The course of ILD was as follows: improvement (15.8%), stabilization (47.4%) or deterioration (36.8%). Predictive parameters of ILD onset were: older age (p=0.044), Raynaud's phenomenon (p=0.001) and esophageal involvement (p=0.001). Factors associated with ILD deterioration were: older age (p=0.038) and esophageal involvement (p=0.038). Thus, this study underscores the poor outcome of ILD during pSS; thus, systematic screening of pulmonary involvement is required in pSS patients, resulting in both diagnosis and management at early stage of ILD. We also suggest that patients presenting predictive factors of ILD deterioration may need a closer follow-up and a more aggressive therapy.
26535602 A Case of Adult-Onset Still's Disease Treated with Monitoring of Serum Tacrolimus Levels. 2015 Jul Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. The mainstays of treatment are glucocorticoids (GCs) and non-steroidal anti-inflammatory drugs, although most cases are refractory to these conventional therapies. Immunosuppressants,such as methotrexate (MTX), cyclosporine A, tumor necrosis factor-α blockers, an interleukin (IL)-1 blocker, and an IL-6, receptor blocker, have been suggested in previous reports for the treatment of steroid-resistant AOSD. We report herein the case of an AOSD patient who was successfully treated with tacrolimus, another immunosuppressant, in combination with GC and MTX. Blood concentrations of tacrolimus were monitored because of the narrow therapeutic window.
28288719 Marginal zone lymphoma: Associated autoimmunity and auto-immune disorders. 2017 Mar Large epidemiological studies have shown a consistent increased risk for developing lymphoma in the setting of autoimmune disorders (AID). It is known that this link appears to be stronger for some AID and certain non-Hodgkin lymphoma subtypes e.g. Sjögren's syndrome and extra-nodal marginal zone lymphoma of the salivary gland, and thyroid MALT lymphoma in a background of Hashimoto's thyroiditis. B and T-cell hyperactivity due to chronic antigenic stimulation and the consequent presence of acquired lymphoid tissue seems to play a key role in the pathogenesis of AI-related lymphomas. Advanced age at diagnosis, prolonged disease course and disease severity are thought to increase the risk of lymphoma development in AID patients. There is increasing evidence that AI-related lymphomas constitute a different spectrum of entities indicating a different pathobiology with specific clinical features and treatment implications. This chapter will provide a general overview on the epidemiological aspects of the NHL-AID association focussing on marginal zone lymphomas - one of the NHL subtypes mostly implicated in the synchronous/metachronous association with AID. We will review the possible biological mechanisms involved and the risk factors in each autoimmune condition related to this lymphoma.
27629974 Acute Motor-dominant Polyneuropathy as Guillain-Barré Syndrome and Multiple Mononeuropath 2016 A patient with xerostomia and xerophthalmia due to Sjögren's syndrome presented with acute motor-dominant polyneuropathy and multiple mononeuropathy with antiganglioside antibodies. Nerve conduction studies and a sural nerve biopsy revealed the neuropathy as a mixture of segmental demyelination and axonal degeneration. Positive results were obtained for several antiganglioside antibodies. Corticosteroid treatment proved effective. The neuropathy was considered to represent a mixture of polyneuropathy as Guillain-Barré syndrome and multiple mononeuropathy via Sjögren's syndrome. We speculate that Guillain-Barré syndrome occurred in the patient and Guillain-Barré syndrome itself activated multiple mononeuropathy via Sjögren's syndrome.
27206986 Adipose tissue is prominent in salivary glands of Sjögren's syndrome patients and appears 2016 Aug A minor salivary gland (SG) biopsy with focal lymphocytic sialadenitis and a focus score of ≥1 is today's widely accepted pathological finding confirming the SG component of Sjögren's syndrome (SS). Adipocytes can occupy a large percentage of the SG area although little is known about their significance in SS lesions. This study aimed to characterise adipose tissue infiltration in labial SG biopsies from 27 SS patients and 28 non-SS sicca controls. Biopsies were evaluated by one oral pathologist and assessed for focus score, acinar atrophy, fatty replacement and non-specific chronic inflammation. Moreover, to explore the SG microenvironment, immunohistochemical staining of paraffin-embedded SG tissue was performed using interleukin-6 (IL-6). The fatty replacement was evident in all SS patients possessing autoantibodies (Ro/SSA and/or La/SSB) as well as a positive SG biopsy (focus score ≥1). Additionally, 62% of SS patients having autoantibodies but a negative biopsy showed fatty infiltration (FI) while non-SS controls demonstrated fatty replacement in only 32% of the cases. Overall, the SS group (mean age 53.0 years) had a significantly higher incidence (p value 0.005) of FI than the non-SS controls (mean age 54.8 years). Interestingly, adipocytes were located in IL-6 rich areas, and IL-6 positive adipocytes were detected. As fat deposition seems to be more recurrent in SGs affected by SS, we propose the assessment of adipose tissue replacement as a helpful tool for diagnostic evaluation in SS. Detection of IL-6 positive adipocytes suggests their involvement in immune reactions. Still, functional studies are needed to investigate the SG microenvironment further.
26839471 The Significance of Ectopic Germinal Centers in the Minor Salivary Gland of Patients with 2016 Feb We investigated the clinical and biological significance of germinal centers (GC) present in the minor salivary glands of patients with Sjögren's syndrome (SS). Minor salivary gland tissue biopsies from 93 patients with SS were used to identify GC-like structures, which were confirmed by CD21-positive follicular dendritic cell networks. Patients were compared based upon sociodemographics, glandular and extraglandular manifestations, and laboratory findings including autoantibody profiles, complement, and immunoglobulin levels; EULAR SS disease activity index (ESSDAI) and SS disease damage index (SSDDI) were also measured. GC-like structures were observed in 28 of 93 SS patients (30.1%). Mean focus scores and CRP levels were significantly higher in GC-positive patients than in GC-negative patients; GC-positive patients also exhibit a higher prevalence of rheumatoid factor and anti-SS-A/Ro antibodies compared to GC-negative patients. No differences in glandular or extra-glandular manifestations were evident between groups. In conclusion, SS patients with GC-like structures in the minor salivary glands exhibited laboratory profiles significantly different from those of their GC-negative counterparts. Long-term follow-up of these patients will be necessary to determine whether these laboratory abnormalities are predictive of clinical outcomes.