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The aims of this pilot investigation were to calculate the levels of sensitivity and specificity of salivary glands ultrasonography (SGUS) in diagnosing Sjögren syndrome (SS) and to assess the ultrasonographic findings of parotid and submandibular glands.Patients diagnosed with SS or dry mouth and healthy controls were enrolled. Bilateral parotid and submandibular glands were assessed for (1) parenchymal inhomogeneity (PIH), (2) median size of the glands, (3) visibility of glandular posterior borders, and (4) size of sialolith, if present.This study included 34 female patients, of whom 12 had SS (35.3%), 12 had dry mouth (35.3%), and 10 were healthy controls (29.4%). Patients with SS showed higher PIH scores in all glands with the median differences being statistically higher in the right and left parotids and left submandibular glands (P.001, P = .012, and P.001, respectively). SGUS, with a PIH cutoff ≥2, showed a sensitivity of 100% and a specificity of 81.6% for detecting SS. The majority of SS had invisible glandular posterior borders (P.001). Median size of the glands and size of the sialolith did not show any statistically significant differences between groups.SGUS is a noninvasive imaging modality with good sensitivity and specificity that might be valuable as a diagnostic aid for SS.

The Food and Drug Administration has recently approved the off-label use of hydroxychloroquine (HCQ) for the treatment of coronavirus disease 2019 (COVID-19) infections. However, a recent study not only failed to demonstrate HCQ efficacy but also documented a serious side effect of COVID-19 therapy with HCQ: QT prolongation and secondary arrhythmia. HCQ has been used as an off-label drug and deemed safe and effective for the treatment of oral lesions, such as Sjogren syndrome (SS), chronic ulcerative stomatitis (CUS), and oral lichen planus (OLP). Because HCQ may be appropriately used for the off-label treatment of SS, CUS, and OLP, relevant safety concerns regarding HCQ therapy with regard to dosage, drug-to-drug interactions, and QT prolongation and secondary arrhythmia are discussed here. Because of the possibility of decreased pharmacy supplies of HCQ, replacement drugs with respect to patients with SS, CUS, and OLP being successfully treated with HCQ are also discussed.

Purpose To compare the burden related to dry eye with systemic symptoms of Sjogren syndrome; to estimate the burden related to ocular treatments; and to compare the impact of dry eye and extraocular manifestations of Sjogren syndrome on various aspects of patient life. Design Cross-sectional study. Methods We conducted a postal survey of adult patients with a history of physician-diagnosed Sjogren syndrome. Results The survey was completed by 2,961 patients (mean age 65.1 years, standard deviation 11.7 years), most of whom were women (96%) and white (94%). Forty-one patients younger than 18 years of age were excluded. More than half (53%) experienced severe dry eye (ie, dry eye daily/almost daily with major impact on their life). Corresponding proportions for dry mouth and fatigue were 48% and 45%, respectively. Almost all patients (97%) had used nonprescription eye drops/artificial tears/ointments. Compared with patients who did not experience dry eye, those who experienced significant dry eye (ie, daily/almost daily dry eye) more often agreed that living with Sjogren syndrome made every day a challenge (adjusted odds ratio [OR] 3.81, 95% confidence interval [CI] 2.49 to 5.86) and added a significant emotional burden (adjusted OR 2.22, 95% CI 1.49 to 3.31). Adjusted ORs for the impact of dry eye were generally lower than those for fatigue, but were similar to dry mouth and considerably higher than use of systemic treatments for serious manifestations of the disease and diagnosis of lymphoma. Conclusions Sjogren-related dry eye is more burdensome than systemic manifestations of the disease. While fatigue has the greatest impact on patient life, the impact of dry eye is comparable to that of other systemic manifestations.

Pulmonary involvement in Sjogren syndrome (SS) could manifest as cystic lung disease (CLD). CLD in SS includes lymphocytic interstitial pneumonia (LIP) and pulmonary amyloidosis. Differential diagnosis usually requires surgical lung biopsy, whereas CT-guided percutaneous fine needle aspiration biopsy (CT-FNAB) has not yet explored. We describe the case of a 63-year-old never smoker Caucasian female with a SS diagnosis who displayed a newly detected diffuse CLD at high-resolution computed tomography, though totally asymptomatic. Given the favorable location of one big lesion at the superior left lobe, a CT-FNAB was proposed instead of a more invasive SLB. At histology examination a diagnosis of pulmonary nodular AL kappa amyloidosis in the context of SS was established. In conclusion, CT-FNAB might represent an alternative and less invasive diagnostic procedure than SLB in the differential diagnosis of CLD, even if further research is needed. Moreover, this case presents an unusual association between SS and pulmonary nodular AL kappa amyloidosis, with pulmonary nodules and cysts without systemic manifestations.

RESUMEN El síndrome de Sjögren primario (SSp) es una enfermedad autoinmune que afecta principalmente al tejido glandular. A pesar de ello, puede involucrar otros sistemas, siendo el compromiso neuropsiquiátrico una manifestación extraglandular común. Su presentación clínica varía ampliamente según el dominio que se encuentre afectado, y por tanto puede dividirse en tres grandes categorías: sistema nervioso central, sistema nervioso periférico y psiquiátrico. Algunas de estas complicaciones comparten mecanismos fisiopatológicos comunes, entre los principales la vasculitis/vasculopatía, la infiltración linfocítica y la presencia de anticuerpos antineuronales. La diversidad en la presentación clínica de esta entidad impide hacer una aproximación diagnóstica común, por lo cual la utilización de estudios específicos depende de un adecuado reconocimiento y de la localización por parte del clínico. El tratamiento debe dirigirse al mecanismo fisiopatológico implicado y, de acuerdo con el tipo de manifestación, puede incluso estar limitado al manejo sintomático. ABSTRACT Primary Sjögren's syndrome is an autoimmune disease that mainly involves glandular tissue. Despite this, it can potentially develop systemic involvement, within which neuropsychiatric manifestations are common. The clinical presentation may vary widely depending on the domain affected, and may thus be classified into three categories: central nervous system, peripheral nervous system, and psychiatric. Some of these complications share a common pathophysiology, amongst which are vasculitis/ vasculopathy, lymphocytic infiltration and positive antineuronal antibodies. The wide clinical presentation makes it difficult to establish a common diagnostic approach, making it essential for the clinician to recognise and localise the type of compromise, so that diagnostic tools can be more advantageously employed. Treatment must be directed towards the underlying pathophysiology, and depending on the type of compromise, it can even be limited solely to the management of symptoms.

PurposeThe goal of this study is to determine a link between benign essential blepharospasm and Sjogren's syndrome by analyzing the presence of extractable nuclear antigens in this population.MethodsSeventy-two patients with benign essential blepharospasm (BEB) were included in this study. We eliminated patients with hemifacial spasm or blepharospasm secondary to corneal pathology. We collected the values of the Schirmer I test and the results of the anti-SSA and anti-SSB antibodies.ResultsOur study included 72 patients (144 eyes) whose 62 women (86.1%). Mean age was 74.3 years ± 10.73. Average Schirmer I test was 3.14 mm ± 4.00 mm. Five women (8% of this female population) had positive anti-SSA and SSB antibodies. Their mean age was 65.66 years ± 13.24 whereas the negative antibody patients had an average age of 75.42 ± 9.27. There was no significant difference between their Schimer I test and the Schirmer I of negative antibody population.ConclusionThis study illustrates the possible association between the presence of Sjögren's syndrome and the occurrence of a BEB justifying the search for anti-SSA and anti SSB in blepharospasm patients., ObjectifÉtudier s’il existe un lien entre le blépharospasme essentiel et le syndrome de Sjögren en analysant la présence d’anticorps nucléaires solubles dans cette population.MéthodesSoixante douze patients atteints de blépharospasme essentiel (BSE) ont été inclus dans cette étude. Nous avons éliminé les patients atteints de spasme hémifacial, ou de blépharospasme secondaire à une pathologie cornéenne avérée. Nous avons recueilli les valeurs des test de Schirmer I et les résultats des anticorps anti-SSA et anti-SSB.RésultatsNotre étude a regroupé 72 patients (144 yeux) dont 62 femmes (86,1 %). L’âge moyen était de 74,3 ans ± 10,73. Le test de Schirmer I était en moyenne de 3,14 mm ± 4,00 mm. Cinq femmes (8 % de cette population féminine) ont révélés des anticorps anti-SSA et SSB positifs. Leur moyenne d’âge était de 65,66 ans ± 13,24 alors que les patients anticorps négatifs avaient une moyenne d’âge de 75,42 ± 9,27. Il n’existait pas de différence statistiquement significative entre leur test de Schimer I et celui des patients anticorps négatifs.ConclusionCette étude illustre la possible association entre la présence d’une maladie de Sjögren et la présence d’un BSE justifiant la recherche d’anticorps anti-SSA et SSB chez ces patients.

Soft tissue calcifications associated with various connective tissue diseases such as dermatomyositis and scleroderma have been well documented Plaque-like sheets of subcutaneous calcifications presenting as an indurated soft tissue mass in a patient with primary Sjogren syndrome have been rarely documented in the literature. We present the magnetic resonance and conventional radiographic findings of calcinosis cutis and calcinosis circumscripta of a 47-year-old woman with biopsy proven Sjogren syndrome. We also delineate various types of soft tissue calcification, histopathology of calcinosis cutis, and current treatment options. Recognizing the magnetic resonance characteristics of this phenomenon may prove useful to radiologists, especially in the absence of clinical history and conventional radiographs.

Connective tissue disorders are now understood to include autoimmune and genetic diseases affecting organs, blood vessels, and surrounding fascia. Many of these diseases predominantly affect women in childbearing years and are associated with neurologic complications. Pregnancy can affect disease activity (such as flares of systemic lupus erythematosus), and the diseases can affect pregnancy outcome (such as increased risk of preterm labor). We review the neurologic complications and changes with pregnancy for systemic lupus erythematosus, Sjögren syndrome, idiopathic inflammatory myopathy, and Marfan syndrome.

Sjögren syndrome (SS) is an autoimmune connective tissue disorder (CTD) that principally affects the lacrimal and salivary glands. Although SS is 1 of the 3 most common autoimmune CTDs alongside systemic lupus erythematosus and progressive systemic sclerosis, it is the least researched CTD overall. SS poses a particular diagnostic challenge because it shares multiple clinical and immunologic features with other CTDs. However, there are some characteristic cutaneous clinical features that can precede the well-known sicca symptoms by years. By familiarizing themselves with these clinical features and having a high suspicion for SS, dermatologists can play an important role in the early diagnosis and treatment of this disease.

Juvenile systemic lupus erythematosus (jSLE), mixed connective tissue disease (jMCTD), and Sjögren syndrome (jSS) are systemic autoimmune and inflammatory disorders with distinct patterns of organ involvement. All are characterized by autoantibody formation, with antinuclear (ANA) and anti-double-stranded DNA common in jSLE, ANA with high-titer ribonucleoprotein antibody in jMCTD, and Sjögren syndrome A and Sjögren syndrome B antibodies + ANA in jSS. Recognition, monitoring, and management for primary care providers are discussed, focusing on the role of primary physicians in recognizing and helping maintain optimal health in children with these potentially life-threatening diseases.

The cutaneous manifestations of the common rheumatologic disorders, such as systemic lupus erythematosus, dermatomyositis, and systemic sclerosis, are well known. In contrast, the dermatologic findings of less common rheumatologic disorders, including Sjögren syndrome, mixed connective tissue disease, and relapsing polychondritis, are less widely known. The cutaneous manifestations of these connective tissue disorders are reviewed.

The salivary glands are commonly affected in systemic autoimmune disease and diseases of unknown pathogenesis. Sjögren syndrome (SjS) can be affected by other systemic diseases. Immunoglobulin G4-related disease (IgG4-RD) commonly affects salivary glands. Imaging findings are usually nonspecific; however, radiologists should be familiar with the manifestations to avoid diagnostic delay. Findings of early-stage SjS are difficult to identify on routine computed tomography or MR imaging. Chronic SjS can be diagnosed from MR imaging and sialographic findings. Multiglandular and localized involvement of IgG4-RD is difficult to differentiate from malignant lymphoma for multiglandular disease and salivary gland carcinoma for localized disease.

Objective This study aimed to examine whether Sjogren syndrome (SS) is related to periodontal status. Study Design A systematic review was performed on the basis of PRISMA (PROSPERO: CRD42017055202). A search was performed in the PubMed/MEDLINE, LILACS, Web of Science, and Science Direct databases. Hand searches and review of the gray literature were also performed. Three researchers independently selected studies, extracted data, and assessed methodologic quality. Studies that correlated primary and/or secondary SS with plaque index, gingival index, probing depth, and bleeding on probing were included. The risk of bias was estimated on the basis of the Newcastle-Ottawa scale. Results Seventeen studies were included in the review and 9 included in the meta-analysis, with a total of 518 and 544 patients, with or without SS, respectively. The mean difference of plaque index (0.29; 95% confidence interval [CI] 0.17-0.41), gingival index (0.52; 95% CI 0.14-0.89), and bleeding on probing (9.92; 95% CI 4.37-15.47) were larger in patients with SS than in controls. In primary SS (0.47; 95% CI 0.10-0.83) and secondary SS (0.74; 95% CI 0.10-1.38), only the mean gingival index was larger compared with that in control group. The majority of the included studies were judged as having a high risk of bias. Conclusions The present review did not provide strong evidence that periodontal status is affected by SS.

Resume Introduction La secheresse oculaire est une pathologie plurifactorielle de la surface oculaire dont la frequence elevee, le retentissement significatif sur la qualite de vie et sur la vision, ainsi que le cout financier, en font un veritable probleme de sante publique. Si le traitement des formes minimes a moderees est generalement simple et efficace, celui des formes severes est souvent decevant. L’utilisation de collyres a base de serum autologue (CSA) represente une alternative therapeutique envisageable pour les cas les plus severes. L’objectif de notre etude est d’evaluer l’efficacite du traitement par CSA a long-terme chez des patients presentant des syndromes secs isoles refractaires aux traitements conventionnels ou secondaires a d’autres pathologies generales telles que le syndrome de Gougerot-Sjogren et la maladie du greffon contre l’hote (GVH), ou a des pathologies oculaires telles que les keratites neurotrophiques, les brulures chimiques et les pemphigoides cicatricielles oculaires. Patients et methodes Il s’agit d’une etude observationnelle retrospective monocentrique menee sur 47 patients, soit 83 yeux traites par collyre de serum autologue a l’Assistance Publique-Hopitaux de Marseille entre avril 2014 et avril 2017 pour syndrome sec oculaire severe isole ou secondaire. Les symptomes subjectifs des patients (score ocular surface disease index [OSDI]), leur degre de satisfaction ainsi que les effets secondaires ont ete recueillis a l’aide de questionnaires. Le break up time (BUT) et le score de Schirmer ont ete notes. Une evaluation clinique fondee sur un test de coloration corneenne a la fluoresceine (score Oxford) est realisee avant le traitement par CSA a P0 puis selon 5 periodicites : P1 (entre 1 et 3 mois), P2 (3 et 9 mois), P3 (9 a 15 mois) et P4 (15 mois a 24 mois), P5 (> 24 mois). Resultats Sur les 83 yeux traites, l’âge moyen etait de 54,39 ans ± 21,56 dont 20 hommes (42,55 %) et 27 femmes (57,44 %), les indications de traitement comprenaient principalement 25,53 % de GVH, 21,27 % de syndromes secs oculaires severes primitifs et 19,14 % de syndrome de Goujerot-Sjogren primaire. Le suivi moyen a ete de 9,82 mois ± 15,50. La variation du score OSDI entre P0 et P1 montre une diminution de 19,32 points ± 29,37 (p Discussion Des analyses biologiques complementaires sur la composition des CSA sont en cours afin de mettre en evidence des facteurs predictifs d’efficacite. Ainsi, les patients non repondeurs au traitement par CSA pourraient repondre aux collyres de serum allogenique, fabriques a partir de sang peripherique preleve chez un donneur sain a partir de sang de cordon. Conclusion Sur cette premiere serie de 83 yeux traites par CSA, une efficacite clinique a ete notee chez la majorite des patients. Aucune complication infectieuse n’a ete rapportee et le taux de satisfaction a ete tres eleve.

Resume Introduction Le phenomene de Raynaud (PR) est un trouble vasomoteur paroxystique reversible, declenche par le froid ou l’emotion. Deux entites independantes sont a distinguer : la maladie de Raynaud et le phenomene de Raynaud secondaire (PRS). Le but de cette etude etait de determiner le profil etiologique du phenomene de Raynaud secondaire dans un service de medecine interne. Methodes Il s’agit d’une etude retrospective descriptive incluant les patients ayant presente un phenomene de Raynaud secondaire, suivis dans un service de medecine interne entre 2000 et 2013. Resultats Nous avons inclus 121 patients. L’âge moyen au moment de l’apparition du PR etait de 41,7 ans. L’âge moyen des patients au moment du diagnostic etiologique etait de 47,3 ans. Le sex-ratio H/F etait de 0,16. Le delai moyen entre le debut du PR et la date de consultation etait de 41,33 mois. Le PR touchait les mains dans tous les cas et les pieds dans 16 % des cas. La forme typique du PR etait la plus frequente (41,4 %). Les complications du phenomene de Raynaud (ulcerations, necroses digitales ou de cicatrices deprimees) etaient notees dans 32,23 % des cas. La capillaroscopie peri-ungeale objectivait une microangiopathie specifique avec presence de megacapillaires chez 49,52 % des patients. Les anticorps antinucleaires etaient positifs chez 88,49 % des patients. Une pneumopathie interstitielle etait associee chez 54,04 %. Les connectivites etaient diagnostiquees chez 86,77 % de nos patients. Les autres etiologies du PRS chez les patients hospitalises en medecine interne etaient les vascularites (6,61 %), la maladie atheromateuse (1,65 %), les causes medicamenteuses et professionnelles (1,65 %). La connectivite la plus frequente etait la sclerodermie systemique (62 %) suivie par le lupus erythemateux systemique (11,57 %) et enfin, par le syndrome de Sjogren primitif dans 6,61 % des cas. Conclusion Dans notre serie, le phenomene de Raynaud etait le plus souvent secondaire a une connectivite, il peut s’agir d’un biais de selection puisqu’il s’agit d’un service de troisieme ligne ou les malades sont souvent adresses pour une suspicion d’une maladie systemique.

Parotitis is a frequent disease in general pediatrics. Pneumoparotitis is a rare affection that belongs to differential diagnoses of parotitis, along with infections, lymphadenitis, autoimmune disorders, inflammatory conditions, vascular malformations or neoplasms. It is usually described in musicians using wind instruments or in other situations involving a Vasalva maneuver. We report the case of a 12 years old boy with severe idiopathic pneumoparotitis without any of these well-known causes and whose autoimmune familial background of Sjögren syndrome might be relevant.

The case is presented of a 50-year-old woman with persistent abnormal uterine bleeding, secondary anemia, adynamia, and asthenia, in whom bicytopenia is documented with microcytic normochromic anemia and thrombocytopenia. After conducting a thorough study of the causes of bicytopenia, the diagnosis of Sjogren's syndrome was made, in the absence of dry symptoms.

Radioactive iodine (131I) is used effectively for the treatment of differentiated thyroid cancers. Because it is actively secreted by the salivary glands, radiation damage to these glands can occur. Obstructive swellings after mealtime salivary stimulation are common occurrences. Dry mouth is not usually seen if low doses of 131I are used. A subjective complaint of xerostomia in a patient treated with 131I 75.8 mCi proved to be related to the simultaneous presence of Sjogren syndrome (SS). Serologic, histologic, scintigraphic, and salivary volume findings and the patient's subjective complaints served to establish the pre-existence of SS.

The immunomodulatory effects of vitamin D have been extensively studied in the context of autoimmunity. Multiple studies have demonstrated a high prevalence of vitamin D deficiency in autoimmune diseases. Recently, a possible protective role of vitamin D in autoimmunity has been described; however, this function remains controversial. Few studies have investigated the role of vitamin D in patients with Sjögren syndrome (SS). In this review, we compiled the main features of SS pathogenesis, the vitamin D immunomodulatory effects and the possible interaction between both. Data suggests that vitamin D may play a role in the SS pathogenesis. In addition, vitamin D low levels have been found in SS patients, which are associated with extra-glandular manifestations, such as lymphoma or neuropathy, suggesting a possible benefit effect of vitamin D in SS.

Objective: The aim of this study was to verify, through a literature review, the use of saliva as a diagnostic method for oral and systemic diseases. Study Design: Articles published in English were selected from PubMed database with the descriptors saliva, biomarkers, and diagnosis. Articles in the English language from the last 5 years have been included with 245 articles obtained. The study contained 17 of these. Results: Salivary diagnosis has been useful for a variety of diseases, including (1) bacterial diseases such as dental caries, by the detection of Streptococcus mutans and Lactobacillus spp; (2) autoimmune diseases such as Sjogren syndrome, indicated not only by the reduction of salivary flow, but also by the increase in the concentration of sodium, chlorine, immunoglobulin A (IgA), IgG, and prostaglandin E2 (PGE2); (3) viral diseases as in the auxiliary diagnosis of HIV 1 and 2 by IgG expression; and (4) in the early diagnosis of malignant diseases such as squamous cell carcinoma and breast cancer by the detection of antibodies against p53 protein and the presence of tumor markers such as c-erbB-2. Conclusion: Salivary diagnosis is a simple, noninvasive, and very promising method for the diagnosis and monitoring of innumerable pathologies.

Objectives We sought to analyze the types and quantity of dry mouth products patients use as well as the reported dry mouth symptom severity and frequency in relation to salivary flows. We hypothesized that respondents with higher stimulated flow would report better responses to products that stimulate flow. Methods Patients with complaints of dry mouth who had documented unstimulated and stimulated whole salivary flows (UWS and SWS, respectively) completed a questionnaire to assess dry mouth products used, current symptoms, and response to therapy. Statistical analyses included descriptive analyses and associations between dichotomized (low/normal) salivary flow levels, and symptom severity was assessed using nonparametric Wilcoxon rank-sum tests. Results Eighty-seven patients completed the questionnaire; 38 patients had a diagnosis of Sjogren syndrome. More than half of patients (55%; n = 48) reported using 4 or more dry mouth products. The most common product used was water (n = 78), followed by rinses (n = 54) and lozenges (n = 48). Twenty-five patients (29%) reported use of parasympathomimetics. More than half (56%) of patients using parasympathomimetics reported that their mouth felt “much better” compared with less than one-third of patients using other methods: water (29%), gum (23%), lozenges (27%), candies (4%), rinse (26%), spray (24%), or gel (20%). Among parasympathomimetic users with normal SWS, 71% reported their mouth felt “much better” compared with 36% of those with low SWS. For water, gum, lozenges, candies, and sprays, greater than 50% of respondents reported improvement lasting less than 1 hour. Regarding rinses, gels, and parasympathomimetics, greater than 50% of respondents reported improvement lasting greater than or equal to 1 hour. The dichotomized level of UWS rate was not associated with any measures of symptom severity, whereas the low SWS rate was associated with the following measures: dryness of the mouth (P = .004), difficulty speaking due to dryness (P = .03), and difficulty swallowing due to dryness (P = .004). Conclusions Different treatment categories for dry mouth symptoms provided varying degrees of relief. Patients with normal vs low stimulated flow who used parasympathomimetics reported the greatest treatment response and longest relief of dry mouth symptoms. Assessment of salivary flow levels may be a useful guide for more targeted recommendations of dry mouth products.

Polyautoimmunity is defined as the presence of more than one well-defined autoimmune disease (AD) in a single patient. Polyautoimmunity is a frequent condition in Sjögren syndrome (SS) and follows a grouping pattern. The most frequent ADs observed in SS are autoimmune thyroid disease, rheumatoid arthritis, and systemic lupus erythematosus. Main factors associated with polyautoimmunity in SS are tobacco smoking and some genetic variants. The study of polyautoimmunity provides important clues for elucidating the common mechanisms of autoimmne diseases (ie, the autoimmune tautology). © 2016 Elsevier Inc.

Xerostomia is the subjective feeling of dry mouth, and it is often related to salivary hypofunction. Besides medication-related salivary hypofunction, Sjögren syndrome and head-and-neck radiation are two common etiologies that have garnered considerable attention. Approaches to treating and/or preventing salivary hypofunction in patients with these conditions will likely incorporate gene therapy, stem cell therapy, and tissue engineering. Advances in these disciplines are central to current research in the cure for xerostomia and will be key to eventual treatment.

Saliva is one of the most versatile, multifunctional substances produced by the body and has a critical role in the preservation of the oropharyngeal health. It comprises a serous and mucinous component and is secreted by the major salivary glands. The mucins in the saliva serve to protect and lubricate the hard and soft tissues of the mouth, protecting them from chemical and mechanical damage. Hyposalivation can be managed by various salivary substitutes, peripheral sialagogues, and central sialagogues.

SUMMARY Follicular helper T cells (TFH) are critical for vaccine and infection elicitation of long-lived humoral immunity, but exaggerated TFH responses can promote autoimmunity and other pathologies. It is unfortunate that no clinical interventions exist for the selective depletion of follicular T cells to alleviate these diseases. We engineered a chimeric antigen receptor (CAR) facilitating the specific targeting of cells with high expression levels of human programmed cell death protein 1 (PD-1), a cardinal feature of follicular T cells. CAR-expressing human natural killer (NK) cells robustly and discriminately eliminated PD-1high follicular human T cells in vitro and in a humanized mouse model of lupus-like disease while sparing B cells and other PD-1low T cell subsets, including regulatory T cells. These results establish a strategy for specific targeting of PD-1high T cells that can be advanced as a clinical tool for the selective depletion of pathogenic follicular T cells or other PD-1high target cells in certain disease states., Graphical Abstract, In Brief Exaggerated follicular helper T cell (TFH) responses promote autoimmunity and other pathologies, yet clinical tools to specifically target TFH are lacking. Reighard et al. describe programmed death-ligand 1 (PD-L1)-based chimeric antigen receptor-expressing human natural killer cells that selectively eliminate human TFH in vitro and in humanized mice based on the high expression of PD-1 by the targeted TFH cells.

Systemic lupus erythematosus (SLE) is a life-threatening multisystem inflammatory condition that may affect almost any part of the eye. We provide an update for the practicing ophthalmologist comprising a systematic review of the recent literature presented in the context of current knowledge of the pathogenesis, diagnosis, and treatment of this condition. We review recent advances in the understanding of the influence of genetic and environmental factors on the development of SLE. Recent changes in the diagnostic criteria for SLE are considered. We assess the potential for novel molecular biomarkers to find a clinical application in disease diagnosis and stratification and in the development of therapeutic agents. We discuss limited forms of SLE and their differentiation from other collagen vascular disorders and review recent evidence underlying the use of established and novel therapeutics in this condition, including specific implications regarding monitoring for ocular toxicity associated with antimalarials.

ObjectiveTo carry out the cross-cultural adaptation of EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) for Portuguese language and evaluate its psychometric properties.MethodCross-sectional study of patients with primary Sjögren's syndrome (SS). The psychometric properties (intraobserver reproducibility and construct validity) were studied. In construct validity, ESSPRI was compared with the Patient's Global Assessment (PGA), Profile of Fatigue and Discomfort (Profad), Sicca Symptoms Inventory (SSI) and Functional Assessment of Chronic Illness Therapy (Facit-F). Statistical tests used were: Cronbach's alpha, intraclass correlation coefficient (ICC), Bland–Altman method and Spearman coefficient. A value of p≤0.05 was considered significant.ResultsThere was no difference between versions in both languages; thus, a Brazilian consensual version was obtained. All subjects were women aged 49.4±11.6 years, with onset of symptoms of 7.2±5.4 years, and time of diagnosis of 3.0±3.3 years. The mean ESSPRI was 6.87±1.97. The intraobserver reproducibility was high and significant (0.911) and, with Bland–Altman method, there was no systematic bias in the agreement of measures among evaluations. A moderate correlation of ESSPRI with all tested instruments was observed.ConclusionThe Brazilian Portuguese version of ESSPRI is a valid and reproducible version.

Our understanding of the mechanisms leading to rheumatic diseases is growing at unprecedented pace thanks to the worldwide network of clinical and translational researchers who gather at major scientific meetings to share their progresses. Further, these meetings allow the contamination of unrelated research areas and thus the spreading of ideas, hypotheses, and research tools. The annual meeting of the American College of Rheumatology (ACR) serves this purpose by allowing thousands of rheumatologists, immunologists, health care professionals, and basic scientists to attend the same sessions and present their work. The 2014 ACR meeting was held in Boston, MA, and was attended by over 16,000 participants who had the opportunity to directly witness the presentation of over 3000 abstracts. As such is the case, a full attendance of all update opportunities was not feasible. To fill this gap we arbitrarily selected the abstracts the appeared most interesting in a few fields of interest and we herein discuss the presented data and their further implications. In particular, we were intrigued by research advances in biomarkers for rheumatic diseases, and by advances on Sjögren syndrome, neuropsychiatric systemic lupus erythematosus, fibromyalgia, and B cell mechanisms. While we are well aware of the numerous blind spots that are expected in this type of article, we submit that this is far from a comprehensive overview and refer to the abstract book for a more complete analysis of the presented abstracts.

Interstitial lung disease is a common and often life-threatening manifestation of different connective tissue disorders, often affecting its overall prognosis. Systemic lupus erythematosus, Sjögren syndrome, and mixed connective tissue disease, although all unique diseases, can have lung manifestations as an important part of these conditions. This article reviews the different pulmonary manifestations seen in these 3 systemic rheumatologic conditions.

Resumen La amiloidosis secundaria es una complicacion de enfermedades inflamatorias cronicas, sin embargo, no es frecuente su asociacion con lupus eritematoso sistemico, siendo pocos casos los reportados en la literatura. Se describe un caso de una paciente de 50 anos con lupus eritematoso sistemico, quien presenta linfadenopatias cervicales con compresion extrinseca traqueal, en las que se documenta amiloidosis secundaria.

Resumen Objetivo Realizar una revision narrativa no sistematica de la literatura sobre las diferentes manifestaciones hepaticas, correlacion clinico-serologica y los efectos adversos derivados de la terapia, de las principales enfermedades autoinmunes sistemicas. Materiales y metodos Se realizo una revision narrativa no sistematica de la literatura desarrollada en idiomas ingles y espanol, acorde con el objetivo de estudio, para articulos referenciados entre los anos 1964 y 2014, en bases de datos primarios como: Pubmed, Embase y Lilacs. Los terminos MESH (medical subject headings) utilizados fueron: liver [Majr]; autoimmune diseases [Majr]; rheumatic diseases [Majr]; hepatotoxicity [Majr]; se combinaron utilizando operadores booleanos (AND, OR). Ademas, se uso la estrategia de agrupamiento retrogrado. Resultados Se identificaron 622 articulos para tamizaje: 604 resultados encontrados en bases de datos; 18 articulos por agrupacion retrograda. Despues de revisar titulos y resumenes, se excluyeron 533 articulos irrelevantes, entre ellos cartas del editor y articulos que no evaluaban los datos de interes, articulos en idiomas diferentes al espanol e ingles: 8, articulos sin resumen disponible: 16. En ultima instancia se identificaron 65 articulos en texto completo que brindaban la informacion necesaria para realizar la revision. Conclusiones Es importante para el clinico tener en cuenta y monitorizar la funcion hepatica, no solo como parte de la busqueda de eventos adversos derivados de la farmacoterapia, sino tambien como busqueda de compromiso hepatico primario que, en muchas ocasiones, puede ser el resultado de diferentes enfermedades autoinmunes sistemicas.

Sjogren syndrome (SS) is a systemic autoimmune disease characterized by hypofunction of the salivary and lacrimal glands. The objective of this study is to report 2 clinical cases emphasizing the importance of labial salivary gland (LSG) biopsy for the diagnosis of SS. Both cases are of women with a mean age of 57 years and a history of oral and ocular dryness. LSG biopsy of each patient was performed and the histopathologic examination revealed focal lymphocytic sialadenitis. Anti-SSA/SSB positivity followed by Schirmer testing and oral symptoms favored the SS diagnosis. Currently, the patients have been followed by a multidisciplinary team to control the oral and systemic manifestations of the disease.

Objectives Salivary glands ultrasonography (SGUS) is an emerging diagnostic aid for Sjogren syndrome (SS) with positive potentials because of its availability, noninvasiveness, and safety. The aim of this investigation was to assess and compare the ultrasonographic features of the parotid and submandibular salivary glands in patients with SS or dry mouth (DM) and in healthy controls and to evaluate the sensitivity and specificity of SGUS in the diagnosis of SS. Study Design This was a prospective observational 3-group comparison study. Patients diagnosed with SS (according to the 2016 American College of Rheumatology [ACR] and European League Against Rheumatism [EULAR] criteria, patients with DM (diagnosed on the basis of the subjective reporting of oral dryness and unstimulated salivary flow of Results Thirty-four female patients were enrolled in this investigation, of whom 76.5% were white. Twelve patients (35.3%) had definitive diagnosis of SS; 12 (35.3%) had DM; and 10 (29.4%) were healthy controls. The median age (interquartile range [IQR]) of patients in all 3 groups was 56.6 (± 14.2) years. Ninety-two percent of patients in both the SS and DM groups were on sialogogues, with the majority using cevimeline (83.3% and 41.6%, respectively). Overall, patients with SS showed significant SGUS features and had higher scores compared with patients with DM and controls. The median inhomogeneity score was significantly higher in the SS group compared with the DM and control groups in the right parotid gland (RPG), left submandibular gland (LSMG), and left parotid gland (LPG) (P = .000, .000, and .012, respectively), with no statistically significant differences detected between the SS and DM groups in the right submandibular gland (RSMG) only (P = .604). The median size of the hypoechogenic bands was statistically higher in all salivary glands (i.e., RSMG, RPG, LSMG, and LPG) in the SS group compared with the DM and healthy control groups (P =.000). When comparing the sizes of the glands, there were no statistically significant differences among the 3 groups in the median transvers dimension and height of the RPG (P = .846 and .137, respectively) and LPG (P = .382 and .538, respectively). Moreover, no statistically significant differences were observed in the median anteroposterior dimension and depth of the RSMG (P = .470 and .147, respectively) and LSMG (P = .495 and .695, respectively) among the 3 groups. Lack of visibility of the posterior borders of all glands was detected in the majority of patients in the SS group (RSMG = 66.6%; RPG = 83.3%; LSMG = 75%; LPG = 83.3%), whereas all patients in the DM and control groups had visible posterior borders (P = .000). The size of the sialolith, if present, did not show any statistically significant differences among the 3 groups in RSMG, RPG, LSMG, and LPG (P = .160, .136, .065, and .377, respectively). The SGUS, with a cutoff value of ≥ 2, showed sensitivity of 100% and specificity of 81.6% for detecting SS ultrasonographic features. Conclusions SGUS is a useful, noninvasive diagnostic modality with high sensitivity and specificity. Parenchymal inhomogeneity scores, sizes of the hypoechogenic bands, and visibility of the glands’ posterior borders may all have a good potential for detection of SS. We propose that the ACR and the EULAR take SGUS into consideration when reviewing/updating the diagnostic criteria for SS in the future.

Background As North Americans live longer, have more chronic conditions and take more medications, adverse oral events are likely to increase and aggravate the symptoms of Sjogren syndrome (SS). Methods A total of 151 adults who self-reported having SS and who had a mean (standard deviation [SD]) age of 65.8 (11.5) years completed a survey that included questions about basic demographic information, current medical conditions, medications used (prescription and over the counter [OTC]) and the use of oral products to manage SS symptoms. Owing to the self-reporting process in our survey, the term "SS" in our study population represented a mixture of people with SS and people with dry mouth symptoms. Results The mean (SD) number of daily medications recorded as prescription, OTC and oral care products were 4.9 (3.5), 4.5 (2.8) and 4.6 (1.4), respectively. Participants with four or more comorbid medical conditions (n = 74; 49.0 percent) had significant differences ( P P Conclusions The survey results indicated that medication use and comorbid medical conditions demonstrated significant differences and may have had a substantial impact on the oral symptoms in adults who self-reported having SS. Clinical Implications Given the prevalence of SS, obtaining an accurate and complete medical and pharmacological history has implications for dental practitioners because medication use and comorbid medical conditions have a significant impact on oral symptoms in patients with SS.

Objective This study examined the application of a simple screening test for salivary gland function by measuring resting and stimulated submandibular and sublingual secretions. Study Design An assay system was designed to use filter paper incorporating the chromophore of melanoidin or stimuli such as capsaicin and citric acid. We investigated the relationship between resting and stimulated secretions and melanoidin migration at 2 minutes for healthy and dry mouth groups. Results The healthy group showed a significant increase in the migration of melanoidin in the paper after citric acid or capsaicin stimulation. In contrast, patients with Sjogren syndrome showed no significant migration in spite of the stimulation. However, some participants with Sjogren syndrome or dry mouth showed a significant increase in the migration of melanoidin after stimulation. Conclusions These results show that the newly developed method should be useful for evaluation of residual salivary gland function and screening for hyposalivation with dry mouth.