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Sjogren’s syndrome - Top 30 Publications

Small Fiber Neuropathy: Diagnosis, Causes, and Treatment.

Small fiber neuropathy, which affects the sensory A≏ and C fibers, is now a major diagnostic and therapeutic challenge. Nearly 7% of the general population have chronic neuropathic pain responsible for severe quality-of-life impairments. Awareness must therefore be raised among clinicians of the somatosensory and autonomic symptoms that can reveal small fiber neuropathy, appropriate diagnostic investigations, most common causes, and best treatment options for each patient profile. To help achieve this goal, the present review article discusses the clinical presentation of neuropathic pain and paresthesia and/or autonomic dysfunction due to involvement of nerves supplying exocrine glands and smooth muscle; normal findings from standard electrophysiological investigations; most informative diagnostic tests (epidermal nerve fiber density in a skin biopsy, laser-evoked potentials, heat- and cold-detection thresholds, electrochemical skin conductance); main causes, which consist chiefly of metabolic diseases (diabetes mellitus, glucose intolerance), dysimmunity syndromes (Sjögren's syndrome, sarcoidosis, monoclonal gammopathy), and genetic abnormalities (familial amyloidosis due to a transthyretin mutation, Fabry disease, sodium channel diseases); and the available symptomatic and etiological treatments.

"Is it Sjögren's syndrome or burning mouth syndrome? Distinct pathoses with similar oral symptoms"-Response.

Y RNA derived small RNAs in Sjögren's syndrome: Candidate biomarkers?

Anti-Ro and anti-La antibodies are important in pathogenesis and diagnosis of Sjögren's syndrome (SS). Ro60, Ro52 and La are RNA binding proteins of Y RNA, which were discovered more than three decades ago. Significance of Y RNA is not appreciated as much as Ro and La in SS. It can be hypothesised that 5'-YsRNA, short fragment derived from Y RNA may be recognized by TLR7 in pDC, which induces type I interferon signature in SS. New genomics tools, namely RNA seq, enables assay of 5'-YsRNA in blood. 5'-YsRNA has the potential to be a novel biomarker of SS.

A Case of Dermatomyositis Complicated by Digital Ischemia and Lung Adenocarcinoma in a Patient with Positive Anti-signal Recognition Particle Antibodies.

A 58-year-old Japanese woman was diagnosed with anti-signal recognition particle (SRP)-positive dermatomyositis associated with Sjögren's syndrome, rheumatoid arthritis and lung adenocarcinoma. She presented with cutaneous lesions, including ulceration of her right middle finger. Tissue specimens obtained from her right deltoid muscle were positive for CD4(+) T-cell infiltration and the sarcolemma showed the upregulation of major histocompatibility complex (MHC) class I antigens. The present case suggests that overlapping autoimmune diseases or complications of malignancy may result in an atypical clinical presentations and histological findings in patients with anti-SRP antibody-positive dermatomyositis.

Clinical and serological associations of anti-ribosomal P0 protein antibodies in systemic lupus erythematosus.

The purpose of this study is to investigate the clinical and serological associations of anti-ribosomal P0 protein antibodies (anti-Rib-P0) in patients with systemic lupus erythematosus (SLE). The sera of 470 patients with SLE and 124 patients with primary Sjogren's Syndrome (pSS) were collected. Line immunoassay (LIA) was used to detect anti-Rib-P0 and other related antibodies. A complete laboratory evaluation and clinical examination were also performed in each SLE patient. The prevalence of anti-Rib-P0 in SLE patients was significantly higher than that in pSS patients (35.74 vs 6.45%) (P < 0.001). There was a significantly lower prevalence of cardiac involvement in anti-Rib-P0-positive SLE patients compared to anti-Rib-P0-negative SLE patients (P = 0.019); no significant associations of anti-Rib-P0 antibodies with encephalopathy manifestations and other vital organs involvement were observed. Anti-nucleosomes, anti-dsDNA, anti-Histones, anti-SmD1, and anti-U1snRNP were significantly associated with serum anti-Rib-P0 antibodies positivity in SLE patients (all P < 0.05). The sensitivity and specificity of the anti-Rib-P0 antibodies to diagnose SLE were 35.74 and 93.55%, respectively. There is a higher prevalence of anti-Rib-P0 in SLE patients. Anti-Rib-P0 positivity may indicate lower cardiac involvement for SLE patients. It may serve as an important complementary parameter in SLE, in addition to anti-dsDNA, anti-SmD1, and anti-nucleosomes.

Does Aromatase Inhibitors Cause Sjogren's Syndrome and Polyneuropathy?

Aromatase inhibitors (AIs) are the indispensible part of hormone-responsive breast cancer treatment. A potential relation between autoimmunity and AIs has been described before. Herein, we report a case of Sjogren's syndrome (SjS) and polyneuropathy which developed during treatment with anastrozole. A 70 years old female patient having a history of breast cancer was referred to our clinic with numbness in both legs for 1 year. She was receiving anastrazole since 2006. She was having sicca symptoms for 3 years. After laboratory evaluation, salivary gland biopsy and electroneuromyography, the patient was evaluated as SjS and polyneuropathy. Intravenous immunoglobulin treatment (400 mg/kg/day, 5 days, 6 months) was initiated. A potential pathogenic linkage between AI therapy and autoimmunity is mentioned. Only few cases of rheumatoid arthritis and SjS related with AIs have been reported. But, our case is the first in the literature having definite SjS and neuropathy in this setting.

Psychological comorbidities associated with subclinical atherosclerosis in Greek patients with primary Sjögren's syndrome: a potential contribution of sleep impairment.

Impaired sleep and psychological disorders are increasingly recognised as prevalent comorbidities in patients with primary Sjögren's syndrome (pSS), as well as important contributors of atherosclerosis in the general population. In the current study we sought to explore a potential role of psychological comorbidities in the pronounced atherosclerotic risk of pSS patients.

Minor salivary gland fibrosis in Sjögren's syndrome is elevated, associated with focus score and not solely a consequence of aging.

Evaluate the presence of minor salivary gland (SG) fibrosis in primary Sjögren's syndrome (pSS) as a function of disease pathology or a consequence of ageing.

Monoclonal gammopathy of renal significance presenting as monotypic plasma cell interstitial nephritis in two patients with Sjögren's syndrome.

Autoantibodies binding to stathmin-4: new marker for polyneuropathy in primary Sjögren's syndrome.

Familial aggregation of myasthenia gravis in affected families: a population-based study.

Myasthenia gravis (MG) is clinically heterogeneous and can be life-threatening if bulbar or respiratory muscles are involved. However, relative contributions of genetic, shared, and nonshared environmental factors to MG susceptibility remain unclear. The aim of this study was to examine the familial aggregation and heritability of MG and the relative risks (RRs) of other autoimmune diseases in the relatives of patients with MG.

Sjögren's syndrome initially presented as thrombotic thrombocytopenic purpura in a male patient: a case report and literature review.

Thrombotic thrombocytopenic purpura (TTP) is a potentially lethal multisystem disorder which could be caused by autoimmune diseases. However, the concomitant occurrence of TTP and Sjögren's syndrome (SS) is an extremely uncommon scenario, especially in male patients. A 56-year-old Chinese male was admitted for the appearance of diffuse ecchymosis. Then he gradually developed transient slurred speech, progressive confusion, agitation, extremity weakness, and fever. Laboratory investigations suggested anemia, thrombocytopenia, significantly increased lactic dehydrogenase, schistocytes in peripheral blood smear, and a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 (ADAMTS13) activity deficiency with high inhibitor titers. TTP was thus diagnosed. The patient also had positive anti-nuclear antibody, anti-SSA, and anti-SSB; however, anti-double stranded DNA (dsDNA) was negative. These drove us to perform ocular and dental sicca evaluation and the finial diagnosis was TTP secondary to SS. Plasma exchange and corticosteroid therapy were effective to control TTP. Cyclophosphamide was subsequently added when the platelet count was stable. The total duration of corticosteroid and cyclophosphamide was 8 and 6 months, respectively. The patient recovered without relapse at 1-year follow-up. To our knowledge, this was the first case of SS initially presented as TTP in a male patient. The case also elucidated the importance of autoantibody screen in the workup of TTP and the benefits of adjunctive immunosuppressive therapy in relapse prevention.

SATB1 Conditional Knockout Results in Sjögren's Syndrome in Mice.

Sjögren's syndrome (SS) is an autoimmune disease in which exocrine tissues are affected by cellular and humoral immunity. As a result, the salivary and lacrimal glands of patients with SS are damaged, leading to xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes). Because experimental approaches to investigate SS pathogenesis in human patients are limited, development of a mouse model is indispensable for understanding the disease. In this study, we show that special AT-rich sequence binding protein-1 conditional knockout (SATB1cKO) mice, in which the SATB1 gene is specifically deleted from hematopoietic cells, develop SS by 4 wk of age, soon after weaning. Female mice presented an earlier onset of the disease than males, suggesting that female SATB1cKO mice are more susceptible to SS. T cell-dominant immune cell infiltration was observed in the salivary glands of 4 wk old SATB1cKO mice, and the frequency of B cells gradually increased as the mice aged. Consistently, levels of anti-SSA and anti-SSB Abs were increased around 8 wk of age, after salivary production reached its lowest level in SATB1cKO mice. These results suggest that SATB1cKO mice can be a novel SS model, in which the progression and characteristics of the disease resemble those of human SS.

The 2017 Doyne Lecture: the orbit as a window to systemic disease.

A very large number of disorders affect the orbit, and many of these occur in the setting of systemic disease. This lecture covers selected aspects of orbital diseases with systemic associations in which the author has a particular clinical or research interest. Spontaneous orbital haemorrhage often occurs in the presence of bleeding diatheses. Thrombosis of orbital veins and ischaemic necrosis of orbital and ocular adnexal tissues occur with thrombophilic disorders, vasculitis, and certain bacterial and fungal infections. Non-infectious orbital inflammation commonly occurs with specific inflammatory diseases, including Graves' disease, IgG4-related disease, sarcoidosis, Sjögren's syndrome and granulomatosis with polyangiitis, all of which have systemic manifestations. IgG4-related ophthalmic disease is commoner than all these except Graves' orbitopathy. Some of these orbital inflammatory diseases are associated with an increased risk of B-cell lymphoma, usually marginal zone lymphoma of MALT type. Ocular adnexal lymphoma also has an association with infectious agents including Helicobacter pylori and Chlamydia psittaci. Orbital metastasis may be the first presentation of systemic malignancy. A number of orbital neoplasms occur in the setting of familial cancer syndromes, including Neurofibromatosis types 1 and 2. Study of the genetics and molecular biology of orbital diseases such as Graves' orbitopathy and idiopathic orbital inflammatory disease will yield useful information on their diagnosis and management.Eye advance online publication, 10 November 2017; doi:10.1038/eye.2017.224.

Oral dryness and Sjögren's: an update.

Oral dryness is a very common condition presenting to a general dental practitioner or hospital specialist. The most common cause of oral dryness is drug related, however, patients with Sjögren's syndrome, a multisystem autoimmune condition, may present to their dentist rather than their GP complaining of dry mouth and dry eyes. This update article explores the causes of oral dryness and how to manage it. The update on Sjögren's syndrome explains the latest relevant diagnostic criteria, presenting signs, symptoms, investigations and management principles.

A case of neuromyelitis optica spectrum disorder presenting with undiagnosed Sjogren's syndrome and a single, atypical tumefactive lesion: A diagnostic challenge.

A case of neuromyelitis optica spectrum disorder presenting with undiagnosed Sjogren's syndrome and a single, atypical tumefactive lesion: A clinical conundrum.

Low-level laser therapy for xerostomia in primary Sjögren's syndrome: a randomized trial.

To evaluate the effectiveness of low-level laser therapy (LLLT) in the treatment of xerostomia in primary Sjögren's syndrome (SS), a randomized clinical trial of patients with dry mouth symptoms associated with primary SS receiving care at a university hospital was conducted. Sixty-six patients were randomly assigned with a 1:1 allocation ratio to receive LLLT (laser group, n = 33) or placebo treatment (placebo group, n = 33). Patients in the laser group received LLLT twice a week for 6 weeks, for a total of 12 treatment sessions. Laser irradiation was performed with an aluminum-gallium-arsenide laser diode at a wavelength of 808 nm, 100-mW output power, and energy density of 4.0 J/cm(2) per irradiation point per session. Placebo treatment was performed following the same protocol used for irradiated patients and using the same laser device to mimic a real irradiation, but with no active laser emission and the tip of the laser probe covered with aluminum foil. The outcomes of interest were xerostomia inventory scores, salivary flow rate, salivary beta-2 microglobulin levels, and salivary sodium and chlorine concentrations. Patients in both groups showed no improvement in xerostomia. Likewise, there was no significant improvement in xerostomia inventory scores (p = 0.301) or salivary flow rate (p = 0.643) in either group. There was no difference in salivary beta-2 microglobulin levels, sodium concentration, and chlorine concentration before and after intervention or between the two groups. The LLLT protocol used in this study effected no improvement in xerostomia or salivary flow rate in patients with primary SS. ClinicalTrials.gov Identifier: NCT02066896.

B Cells Are Indispensable for a Novel Mouse Model of Primary Sjögren's Syndrome.

Primary Sjögren's syndrome (pSS) is characterized by a panel of autoantibodies, while it is not clear whether B cells and autoantibodies play an essential role in pathogenesis of the disease. Here, we report a novel mouse model for pSS which is induced by immunization with the Ro60_316-335 peptide containing a predominant T cell epitope. After immunization, mice developed several symptoms mimicking pSS, including a decreased secretion of tears, lymphocytic infiltration into the lacrimal glands, autoantibodies, and increased levels of inflammatory cytokines. Disease susceptibility to this novel mouse model varies among strains, where C3H/HeJ (H2-k) and C3H/HeN (H2-k) are susceptible while DBA/1 (H2-q) and C57BL/6 (H2-b) are resistant. Depletion of B cells using anti-CD20 monoclonal antibodies prevented C3H/HeN mice from development of the pSS-like disease. In addition, HLA-DRB1*0803, a pSS risk allele, was predicted to bind to the hRo60_308-328 which contains a predominant T cell epitope of human Ro60. Therefore, this study provides a novel mouse model for pSS and reveals an indispensable role of B cells in this model. Moreover, it suggests that T cell epitope within Ro60 antigen is potentially pathogenic for pSS.

Salivary scintigraphy for Sjögren's syndrome in patients with xerostomia: a retrospective study.

The value of salivary gland scintigraphy in the diagnosis of Sjögren's syndrome remains controversial. The primary aim of this study was to estimate the diagnostic accuracy of salivary gland scintigraphy in the diagnosis of Sjögren's syndrome among 237 patients with xerostomia.

Neonatal Systemic Lupus Erythematosus Syndrome: a Comprehensive Review.

Neonatal lupus erythematosus is an uncommon syndrome, which is caused by transplacental passage of maternal autoantibodies to Sjögren's syndrome A or B autoantigens. The clinical presentation includes distinctive cutaneous lesions resembling those seen in systemic lupus erythematosus, hepatobiliary disease, and cytopenias, which disappear with the clearance of maternal autoantibodies. The most severe presentation is a total atrioventricular heart block, which begins during the second trimester of gestation and is irreversible. The risk of having a child with neonatal lupus erythematosus in mothers who test positive for autoantibodies to Sjögren's syndrome autoantigens is approximately 2% for first pregnancies or if previous babies were healthy. The risk increases by approximately tenfold if a previous child had neonatal lupus erythematosus syndrome. The diagnosis of neonatal lupus erythematosus is made when the mother has autoantibodies to Sjögren's syndrome autoantigens, and the fetus or newborn develops atrioventricular heart block, or the newborn develops the typical rash or hepatic or hematologic manifestations in the absence of other explanation. Fetal echocardiography from the 16th to the 26th week of gestation is advised in mothers with autoantibodies to Sjögren autoantigens. The detection of a slow fetal heart rate or the postnatal diagnosis of atrioventricular heart block warrants immediate maternal testing for these autoantibodies if not previously tested.

The RNA binding protein La/SS-B promotes RIG-I-mediated type I and type III IFN responses following Sendai viral infection.

La/SS-B (or La) is a 48 kDa RNA-binding protein and an autoantigen in autoimmune disorders such as systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS). La involvement in regulating the type I interferon (IFN) response is controversial - acting through both positive and negative regulatory mechanisms; inhibiting the IFN response and enhancing viral growth, or directly inhibiting viral replication. We therefore sought to clarify how La regulates IFN production in response to viral infection. ShRNA knockdown of La in HEK 293 T cells increased Sendai virus infection efficiency, decreased IFN-β, IFN-λ1, and interferon-stimulated chemokine gene expression. In addition, knockdown attenuated CCL-5 and IFN-λ1 secretion. Thus, La has a positive role in enhancing type I and type III IFN production. Mechanistically, we show that La directly binds RIG-I and have mapped this interaction to the CARD domains of RIG-I and the N terminal domain of La. In addition, we showed that this interaction is induced following RIG-I activation and that overexpression of La enhances RIG-I-ligand binding. Together, our results demonstrate a novel role for La in mediating RIG-I-driven responses downstream of viral RNA detection, ultimately leading to enhanced type I and III IFN production and positive regulation of the anti-viral response.

Activation of plasmacytoid dendritic cells by apoptotic particles - mechanism for the loss of immunologic tolerance in Sjögren's syndrome.

Sjögren's syndrome (SS) is a common autoimmune disease targeting salivary and lacrimal glands. It is strongly female-dominant characterized by low estrogen levels combined with a local intracrine dihydrotestosterone defect. We hypothesized that these hormonal deficits lead to increased apoptosis of the epithelial cells and plasmacytoid dendritic cell (pDC) mediated pro-inflammatory host responses. Expression of Toll-like receptors (TLRs) 7 and 9 and cytokine profile was studied in pDCs treated with apoptotic particles collected in consecutive centrifugation steps of media from apoptotic cells. Expression and localization of SS autoantigens in these particles was also analysed. Furthermore, the effects of sex steroids were studied in pDCs cultured with several concentrations of dihydrotestosterone and 17-β-estradiol, and in saliva of patient treated with dehydroepiandrosterone. Apoptosis of the epithelial cells led to cleavage and translocation of SS-autoantigens, α-fodrin and SS-A, into apoptotic particles. The apoptosis-induced apoptotic particles contained also other SS-autoantigen hy1-RNA. These particles were internalized by pDCs in size-dependent manner and affected TLRs 7 and 9 expression and the production of proinflammatory cytokines. The analysed androgens protected cells from apoptosis, influenced redistribution of autoantigens and diminished the apoptotic particle -stimulated increase of the TLRs in pDCs. Our findings suggest that the formation of apoptotic particles may play a role in loss of immune tolerance, manifested by production of autoantibodies and the onset of autoinflammation in SS. This article is protected by copyright. All rights reserved.

Acquiring new N-glycosylation sites in variable regions of immunoglobulin genes by somatic hypermutation is a common feature of autoimmune diseases.

Manipulation of Panx1 Activity Increases the Engraftment of Transplanted Lacrimal Gland Epithelial Progenitor Cells.

Sjögren's syndrome is a systemic chronic autoimmune inflammatory disease that primarily targets the salivary and lacrimal glands (LGs). Currently there is no cure; therefore, cell-based regenerative therapy may be a viable option. LG inflammation is facilitated by extracellular ATP and mediated by the Pannexin-1 (Panx1) membrane channel glycoprotein. We propose that suppression of inflammation through manipulation of Panx1 activity can stimulate epithelial cell progenitor (EPCP) engraftment.

Reduced Expression of VAMP8 in Lacrimal Gland Affected by Chronic Graft-versus-Host Disease.

To investigate whether the SNARE protein vesicle-associated membrane protein 8 (VAMP8) was implicated in the development of chronic ocular graft-versus-host disease (GVHD).

miRNA Expression Profile of Mucoepidermoid Carcinoma.

MicroRNAs (miRNAs) are single-stranded RNAs that have been implicated in cancer initiation and progression and act as tumour suppressors or oncogenes. In this study, miRNA profiling was conducted on the most frequent malignancy of salivary glands, mucoepidermoid carcinoma (MEC), with comparison to normal tissues.

Hypertrophic Pachymeningitis in Sjögren's Syndrome.

Although central nervous system manifestations seem common in primary Sjögren's syndrome, hypertrophic pachymeningitis is rare. We herein describe a case of Sjögren's syndrome that was associated with hypertrophic pachymeningitis. Sjögren's syndrome should be considered as a cause of hypertrophic pachymeningitis.

A qualitative exploration of physical, mental and ocular fatigue in patients with primary Sjögren's Syndrome.

Primary Sjögren's Syndrome (pSS) affects exocrine glands such as those producing the tear film, leading to dry and painful eyes, but is also associated with fatigue. The experience of fatigue in pSS, and its relationship with sicca symptoms, is poorly understood.

Productivity Losses and Costs in the Less-Common Systemic Autoimmune Rheumatic Diseases.

We synthesised the literature on productivity losses and costs in the less-common systemic autoimmune rheumatic diseases: Sjogren's syndrome (SjS), systemic sclerosis (SSc), poly/dermatomyositis (PM/DM), and systemic vasculitides (SV).