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Mucosa-associated lymphoid tissue (MALT) lymphoma commonly arises from chronic inflammation or autoimmune diseases, such as Sjögren syndrome (SjS). Although rare, amyloid deposition in MALT lymphoma has been reported. We present a rare case of parotid gland MALT lymphoma in a 49-year-old woman, in whom preoperative diagnosis was challenging due to atypical imaging findings resulting from amyloid deposits. MRI showed T2-hypointense and T1-iso- to slightly hyperintense masses in the left parotid gland and right sublingual gland, with predominant marginal contrast enhancement and no significant diffusion restriction. Additionally, atrophy and fatty replacement of the parenchyma were noted in bilateral parotid glands, suggesting SjS. Left superficial parotidectomy was performed and pathological findings confirmed MALT lymphoma with extensive amyloid deposition. Histopathological findings of the resected parotid gland parenchyma also suggested SjS. MALT lymphoma should be considered in the differential diagnosis of multiple salivary gland masses in patients with suspected SjS. If MRI reveals atypical imaging findings for malignant lymphoma, particularly T2-hypointensity with no significant diffusion restriction, the possibility of amyloid deposition in MALT lymphoma should be considered.

Background/purpose: The diagnosis and treatment of Sjogren's syndrome (SS) are commonly conducted by rheumatologists and stomatologists. The purpose of this study was to compare the scientometric characteristics of SS publications by rheumatologists and stomatologists. Materials and methods: All the papers on cheilitis were comprehensively retrieved from the Scopus database, and divided into rheumatologists and stomatologists groups. Results: There were 3245 and 1209 papers on SS were published by rheumatologists and stomatologists, respectively. For the most-cited top-200 papers, the total citation count was 29,764 and the h index was 108 for SS publications by rheumatologists; whereas the count is 19,891 and h index is 81 for publications by stomatologists. Interestingly, we observed that accumulated citations of the publications by stomatologists cooperated with rheumatologists were larger than those by stomatologists alone during 2012–2022. The more common keywords such as saliva, salivation, minor salivary glands, parotid gland, submandibular gland, sialography, lip, dental caries, and hyposalivation were reported by stomatologists. The more frequent keywords such as rheumatoid factor, fatigue, lymphoma, interstitial lung disease, arthralgia, Raynaud phenomenon, lymphadenopathy, and vasculitis were reported by rheumatologists. Conclusion: This study firstly reports the scientometric characteristics of SS publications by rheumatologists and stomatologists. The scale and citations of rheumatologists' publications greatly outweigh those of stomatologists, suggesting stomatologists can cooperate more with rheumatologists regarding SS research.

Summary: Background: What baseline predictors would be involved in mortality in people with primary Sjögren syndrome (SjS) remains uncertain. This study aimed to investigate the baseline characteristics collected at the time of diagnosis of SjS associated with mortality and to identify mortality risk factors for all-cause death and deaths related to systemic SjS activity measured by the ESSDAI score. Methods: In this international, real-world, retrospective, cohort study, we retrospectively collected data from 27 countries on mortality and causes of death from the Big Data Sjögren Registry. Inclusion criteria consisted of fulfilling 2002/2016 SjS classification criteria, and exclusion criteria included chronic HCV/HIV infections and associated systemic autoimmune diseases. A statistical approach based on a directed acyclic graph was used, with all-cause and Sjögren-related mortality as primary endpoints. The key determinants that defined the disease phenotype at diagnosis (glandular, systemic, and immunological) were analysed as independent variables. Findings: Between January 1st, 2014 and December 31, 2023, data from 11,372 patients with primary SjS (93.5% women, 78.4% classified as White, mean age at diagnosis of 51.1 years) included in the Registry were analysed. 876 (7.7%) deaths were recorded after a mean follow-up of 8.6 years (SD 7.12). Univariate analysis of prognostic factors for all-cause death identified eight Sjögren-related variables (ocular and oral tests, salivary biopsy, ESSDAI, ANA, anti-Ro, anti-La, and cryoglobulins). The multivariate CPH model adjusted for these variables and the epidemiological features showed that DAS-ESSDAI (high vs no high: HR = 1.68; 95% CI, 1.27–2.22) and cryoglobulins (positive vs negative: HR = 1.72; 95% CI, 1.22–2.42) were independent predictors of all-cause death. Of the 640 deaths with available information detailing the specific cause of death, 14% were due to systemic SjS. Univariate analysis of prognostic factors for Sjögren-cause death identified five Sjögren-related variables (oral tests, clinESSDAI, DAS-ESSDAI, ANA, and cryoglobulins). The multivariate competing risks CPH model adjusted for these variables and the epidemiological features showed that oral tests (abnormal vs normal results: HR = 1.38; 95% CI, 1.01–1.87), DAS-ESSDAI (high vs no high: HR = 1.55; 95% CI, 1.22–1.96) and cryoglobulins (positive vs negative: HR = 1.52; 95% CI, 1.16–2) were independent predictors of SjS-related death. Interpretation: The key mortality risk factors at the time of SjS diagnosis were positive cryoglobulins and a high systemic activity scored using the ESSDAI, conferring a 2-times increased risk of all-cause and SjS-related death. ESSDAI measurement and cryoglobulin testing should be considered mandatory when an individual is diagnosed with SjS. Funding: Novartis.

Primary Sjögren syndrome (pSS) is a systemic autoimmune inflammatory disease. Up to now, the role of regulatory T cells (Tregs) and their subgroups in pSS is still in controversial. In this study we tried to elucidate the roles of Tregs and its subgroups in pSS. Total 43 pSS patients and 23 health persons as control were enrolled in this study. We grouped the pSS patients according to the anti-SSa/SSb and the EULAR Sjögren's syndrome disease activity index (ESSDAI). Among the 43 pSS patients, 14 patients were followed after treatment. The percentage of rTregs (resting Treg cells) among Tregs was increased in the pSS group, and decreased after treatment. In the high disease activity subpopulation (ESSDAI ≥ 5), the percentage of rTregs among Tregs decreased after treatment. On the contrary, the percentage of aTregs (activated Treg cells) increased after treatment. It was in an inverse correlation between the percentage of aTreg and rTreg in pSS patients. The Tregs are co-cultured with responder T cells. Tregs from pSS patients showed poorer proliferation inhibitory function. Our results show that the percentages of Tregs and their subgroups altered in pSS patients. The percentage of aTreg and the percentage of rTreg have an inverse correlation in pSS patients. Compared to the control group, the percentage of rTregs among Tregs was increased in the pSS patients and decreased after the treatment. Our study also showed that The Tregs from pSS patients may have poorer inhibitory functions.

Dr. Irwin M. Braverman has advanced our understanding of cutaneous manifestations of autoimmune connective tissue diseases (AI-CTD). Prior to the era of molecular diagnostics, Dr. Braverman's careful clinical observations and detailed descriptions of patients with AI-CTD in his textbook Skin Signs of Systemic Disease provided insights into disease characterization, progression, and evolution. Patients from Dr. Braverman's career who did not fulfill criteria for a single clinical entity, but rather were diagnosed with undifferentiated connective tissue disease or mixed connective tissue disease, are discussed. In addition, key observations of Dr. Braverman into the cutaneous manifestations of Sjögren syndrome and the systemic complications of urticarial vasculitis are highlighted., Competing Interests: Declaration of competing interest Matthew D. Vesely, MD, PhD – none, (Copyright © 2024. Published by Elsevier Inc.)

Sjögren's Syndrome (SS) is an autoimmune inflammatory disease, characterized by lymphocytic infiltration of exocrine glands. Approximately 10% of patients with SS have pulmonary involvement as the first manifestation of their disease, the most common being non-specific interstitial pneumonia.We present the case of a 51-year-old man with organizing pneumonia as the presenting feature of primary SS.Pulmonary involvement as the presenting feature of SS is uncommon, especially when the pattern on CT-scan is that of organized pneumonia. Initial management includes steroids and other immunosuppressants agents, with a better response in organized pneumonia secondary SS cases.

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.

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.

Background: Celiac disease (CeD) is an immune-mediated disorder affecting the small bowel, associated with genetic factors and increasing global prevalence., Aim: This study explores the association between CeD, Systemic Lupus Erythematosus (SLE), primary Sjogren syndrome (pSS), and Systemic Sclerosis (SSc)., Methods: A systematic review and meta-analysis were conducted following PRISMA guidelines. Searches across multiple databases yielded 2728 articles, with 15 studies selected. Data extraction included study characteristics, prevalence of CeD and CeD antibodies in SLE, pSS, and SSc. Quality assessment utilized the Newcastle-Ottawa Scale., Results: The meta-analysis revealed a pooled prevalence of biopsy-proven CeD in SLE, pSS, and SSc of approximately 3%. Seroprevalence of any CeD antibody in SLE, pSS, and SSc ranged from 3% to 10%. Notably, pSS exhibited the highest prevalence at 5.59%. High heterogeneity was observed in seroprevalence across autoimmune conditions. Quality assessment indicated robust methodological quality in the selected studies., Conclusion: This study highlights a significantly higher prevalence of CeD, especially pSS, compared to the general population. The findings underscore the importance of recognizing elevated CeD antibodies in patients with SLE, pSS and SSc emphasizing the need for early detection and comprehensive care for gastrointestinal symptoms in these conditions., Competing Interests: Conflict of interest The authors whose names are listed immediately below certify that they have NO affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript. Author names: Renato Beas MD, Euler Altamirano-Farfan MD, Diego Izquierdo-Veraza MD, Dalton A. Norwood MD, Adrian Riva-Moscoso MD, Ambar Godoy MD, Eleazar E. Montalvan-Sanchez MD, Mirian Ramirez BS, Daniel A. Guifarro MD, Emily Kitchin MD, Monika Fischer MD, Satya Kurada MD., (Copyright © 2024 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

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.

Exposure to endocrine-disrupting chemicals (EDCs) has been linked to various immune deficiency disorders, including autoimmune diseases like Sjögren Syndrome (SjS). However, the detrimental effects of exposure to EDCs, including bisphenols, parabens, and triclosan (TCS), on SjS have been inadequately documented. Thus, we conducted a cross-sectional study that included both healthy individuals (controls) and patients with SjS (cases). We assessed serum concentrations of bisphenol A (BPA), bisphenol S (BPS), methyl parabens (MeP), ethyl parabens (EtP), and TCS. The relationship between the five EDCs levels and the risk of SjS was also explored. Additionally, we conducted an in-depth analysis of the collective influence of these EDCs mixtures on SjS, employing a weighted quantile sum regression model. Out of the five EDCs analyzed, EtP displayed the highest mean concentration (2.80 ng/mL), followed by BPA (2.66 ng/mL) and MeP (1.99 ng/mL), with TCS registering the lowest level (0.36 ng/mL). Notably, BPS exposure was significantly positively associated with the risk of being diagnosed with SjS (with an odds ratio [OR] of 1.17, p = 0.042). No statistically significant associations with SjS were observed for BPA, MeP, EtP, and TCS (p > 0.05). And we did not observe any significant effects of the EDCs mixture on SjS. To the best of our knowledge, this study is the first to suggest that BPS may potentially increase the risk of SjS. Although no significant effects were observed between other EDCs and SjS risk, we cannot disregard the potential harm of EDCs due to their non-monotonic dose response., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Autoimmune diseases have specific pathophysiologic mechanisms leading to an increased risk of arterial and venous thrombosis. The risk of venous thromboembolism (VTE) varies according to the type and stage of the disease, and to concomitant treatments. In this review, we revise the most common autoimmune disease such as antiphospholipid syndrome, inflammatory myositis, polymyositis and dermatomyositis, rheumatoid arthritis, sarcoidosis, Sjogren syndrome, autoimmune haemolytic anaemia, systemic lupus erythematosus, systemic sclerosis, vasculitis and inflammatory bowel disease. We also provide an overview of pathophysiology responsible for the risk of VTE in each autoimmune disorder, and report current indications to anticoagulant treatment for primary and secondary prevention of VTE., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023. Published by Elsevier B.V.)

Background: The risk of fetal atrioventricular block in anti-Ro/SSA antibody-exposed pregnancies with no previous affected offspring is approximately 2%. A high antibody titer is necessary but not sufficient for atrioventricular block, and specific antibody titers do not predict risk. However, there are no data on the negative predictive value of antibody titer to identify pregnancies at low risk of fetal atrioventricular block, and may not require surveillance., Objective: This study aimed to define anti-Ro52 and anti-Ro60 antibody thresholds for the identification of fetuses unlikely to develop atrioventricular block using clinically validated and research laboratory tests., Study Design: This study performed a multicenter review of pregnant subjects who tested positive in their local commercial laboratories for anti-Ro/SSA antibodies at the University of Colorado Children's Hospital (2014-2021) and Phoenix Children's Hospital (2014-2021) and enrolled in the Research Registry for Neonatal Lupus (RRNL) at New York University Langone Medical Center (2002-2021). The subjects were referred on the basis of rheumatologic symptoms or history of atrioventricular block in a previous pregnancy and were retrospectively grouped on the basis of pregnancy outcome. Group 1 indicated no fetal atrioventricular block in current or past pregnancies; group 2 indicated fetal atrioventricular block in the current pregnancy; and group 3 indicated normal current pregnancy but with fetal atrioventricular block in a previous pregnancy. Maternal sera were analyzed for anti-Ro52 and anti-Ro60 antibodies using a clinically validated multiplex bead assay (Associated Regional and University Pathologists Laboratories, Salt Lake City, UT) and a research enzyme-linked immunosorbent immunoassay (New York University). This study calculated the negative predictive value separately for anti-Ro52 and anti-Ro60 antibodies and for the 2 combined using a logistic regression model and a parallel testing strategy., Results: This study recruited 270 subjects (141 in group 1, 66 in group 2, and 63 in group 3). Of note, 89 subjects in group 1 had data on hydroxychloroquine treatment: anti-Ro/SSA antibody titers were no different between those treated (n=46) and untreated (n=43). Mean anti-Ro52 and anti-Ro60 titers were the lowest in group 1 and not different between groups 2 and 3. No case of fetal atrioventricular block developed among subjects with anti-Ro52 and anti-Ro60 titers of <110 arbitrary units per milliliter using the multiplex bead assay of the Associated Regional and University Pathologists Laboratories (n=141). No case of fetal atrioventricular block developed among subjects with research laboratory anti-Ro52 titers of <650 and anti-Ro60 of <4060 enzyme-linked immunosorbent immunoassay units (n=94). Using these 100% negative predictive value thresholds, more than 50% of the anti-Ro/SSA antibody pregnancies that ultimately had no fetal atrioventricular block could be excluded from surveillance based on clinical and research titers, respectively., Conclusion: Study data suggested that there is a clinical immunoassay level of maternal anti-Ro/SSA antibodies below which the pregnancy is at low risk of fetal atrioventricular block. This study speculated that prospectively applying these data may avert the costly serial echocardiograms currently recommended for all anti-Ro/SSA-antibody positive pregnancies and guide future management., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: To present a case of Sjogren syndrome with pulmonary manifestations in an adult female and discuss its assessment and management., Design: Case Report., Setting: Tertiary care hospital., Patient: One., Results: A 50 yrs female admitted with complaints of dryness of eyes with decreased salivation causing difficulty in swallowing since last 3 years, with persistent dry cough since 10-15 days and progressive dyspnea since 4-5 days. Anti-nuclear antibody (ANA) profile revealed Anti- Ro/SS-A and Anti- La/SS-B Positive. Also, sub-lingual excisional biopsy was done which was consistent with findings of Sjogren's syndrome. Patient showed significant improvement after starting oral glucocorticoids, systemic anti inflammatory agents (Tab. HCQS), artificial tear drops, oral iron supplements and other supportive treatment., Conclusion: Sjögren syndrome (SS) is a chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function and associated with lymphocytic infiltration of exocrine glands, and can affect extraglandular organ systems including the skin, lung, heart, kidney, neural, and hematopoietic systems. We present a case of Sjogren syndrome in an adult female presenting with xerostomia and dyspnea and was diagnosed upon detection of anti-Ro and anti-La antibodies and confirmed by histopathological examination of lip biopsy. Patient was started on oral steroids and other supportive treatment, General condition improved significantly and is doing very well on regular follow-up., (Copyright © 2020. Published by Elsevier B.V.)

Sjögren disease increasingly is recognized in pediatric patients. Clinical features, primarily parotitis and sicca symptoms, and results of diagnostic tests may be different from those in adult disease. Adult criteria fail to capture most pediatric patients. Pediatric-specific criteria are urgently needed to define the natural history of the disease, identify risk and prognostic factors, and evaluate the impact of therapeutics and other interventions on disease course in young patients., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Patients with rheumatological diseases may present with neurological manifestations of peripheral and/or central nervous system (CNS). Symptoms may be related to underlying rheumatological disease or CNS effects of immune-modulating drugs. Early diagnosis and therapy may help prevent serious complications. Magnetic resonance imaging (MRI), given its excellent soft tissue details, is the preferred imaging modality when evaluating patients with rheumatological disease and suspected CNS involvement. We present a review of the neuroimaging manifestations of various rheumatic diseases with emphasis on the imaging findings on MRI., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Introduction/objectives: To define the performance of Minor Salivary Gland Biopsy (MSGB) and Dry Eye Tests (DET) to detect occult Sjögren Syndrome (SS) among Interstitial Pneumonia with Autoimmune Features (IPAF) patients., Methods: Prospective study. Interstitial Lung Disease (ILD) patients without defined Connective Tissue Disease and one or more IPAF classification domains or xerophthalmia were included. MSGB, Schirmer's test (ST) and Ocular Staining Score (OSS) were performed in a blinded manner by experienced specialists. MSGB with ≥1 focus of lymphocytes and Dry Eye Test (DET) with OSS ≥ 5 and/or ST < 5 s were considered positive. SS was diagnosed according to the ACR 2016 criteria., Results: 534 patients on the first consult were screened. 67 patients had at least one IPAF criteria, 53 (79.1%) female, mean age (SD) 64.2 years old (10.8). Positive ST in 36 (53.7%), positive OSS in 29 (43.3%) and positive MSGB in 36 (53.7%) were found. Finally, 27 (40.3%) met SS diagnostic criteria. 25 (37.3%) and 18 (26.8%) of them did not report dry eyes or dry mouth, respectively. 53 (79.1%) had negative anti SSA/Ro, 57 (85.1%) had negative anti LA/SSB, 30 (44.7%) had negative ANA, and 52 (77.6%) had negative RF, respectively. A significantly higher proportion of ANA (+), anti-SSA/Ro (+), anti-SSB/La (+), positive DET and positive MSGB were found in the SS population., Conclusions: A significant proportion of patients with occult SS were found in our study. MSGB and DET may be considered in the evaluation of IPAF patients., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Immune checkpoint inhibitors (ICIs) can cause numerous and complex immune-related adverse events whose management need a multidisciplinary approach. Herein, we investigated 114 requests, mostly concerning patients suffering from lung cancer, that were submitted to the « ToxImmun » multidisciplinary meeting in Eastern Occitania between December the 17th 2018 and January the 20th 2020. The leading reasons for the request concerned the putative causal link between immunotherapy and immune-toxicity and its management, followed by possible retreatment after temporary withdrawn because of adverse event, and finally the possibility to initiate ICIs in patients with pre-existing autoimmunity. Colitis, hepatitis and myocarditis were the most frequent immune-related adverse events (IRAEs), both all grade and grade 3-4. Sicca syndrome (with or without Sjogren criteria) was also frequent (26% of cases) and seems to be associated with severe toxicity and multi-toxicity. The mean time to first IRAE was 3.8 months, a time shortened with the use of anti-PD-L1 agents or ICI combination. A majority of requests came from initial evaluation by the internist confirming the early and main role of this specialty in the management of immunotoxicity. Expansion of this regional multidisciplinary meeting, coordinated by internists and medical oncologists, could improve management of immune-related adverse events for the patients' benefits., (Copyright © 2021 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Tuberculosis is a common disease but it keeps on surprising with its unique presentations. It has been reported as lung mass but here we are reporting a case of Tuberculosis presented as vasculitic lung mass. A 61 years old female, known case of hypertension and secondary Sjogren syndrome, on treatment, presented with history of mild dyspnea and generalised weakness with incidental finding of Right hilar mass on chest X-ray undergoes a CECT thorax and CT guided biopsy. Histopathology contrary to expectations revealed granulomatous vasculitis with no microbiological evidence of TB, responded to antitubercular treatment and there was almost complete regression of the lesion after 6 weeks of ATT., Competing Interests: Declaration of Competing Interest All authors have none to declare., (Copyright © 2020 Tuberculosis Association of India. Published by Elsevier B.V. All rights reserved.)

The association between malignancy and rheumatic diseases has been demonstrated in a multitude of studies. Little is understood regarding the pathogenesis of rheumatic and musculoskeletal diseases in association with malignancy. There is strong evidence regarding the association between Sjögren syndrome and lymphoma as well as risk factors for development of lymphoma in these patients. This article discusses the accumulating data on various malignancies described in primary Sjögren syndrome, highlighting non-Hodgkin lymphoma and thyroid, multiple myeloma, and skin cancers. These reported associations may have clinical implications in daily practice and contribute to understanding of both autoimmunity and cancer., Competing Interests: Disclosure The authors have no commercial or financial conflicts of interest. This work was supported in part by NIH grants AR053483, AI0822714, and GM104938., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: To explore the association between xerostomia and dry eye symptoms in the older adult population., Methods: Secondary analysis of data from 627 participants (346 male, 291 female), with a mean ± SD age of 75 ± 7 years, recruited through the population-based South Australian Dental Longitudinal Study. Participants completed the validated Summated Xerostomia Inventory-Dutch Version questionnaire (SXI-D), and rated their overall frequency of dry eye symptoms at baseline., Results: Symptomatic dry eye was reported by 31% of participants, and xerostomia by 21%. A positive correlation was observed between dry eye symptoms and SXI-D scores (Spearman's rho = +0.379, p < 0.001). Overall, participants with symptomatic dry eye had higher SXI-D scores than those without (10.6 ± 3.6 vs. 8.1 ± 2.8, p < 0.001), and were more likely to report xerostomia, with an odds ratio (95% CI) of 2.25 (1.52-3.35; p < 0.001)., Conclusions: Symptoms of xerostomia and dry eye were relatively common in the cohort of older adult participants. The potentially debilitating implications of - and positive association between - the two types of sicca symptoms support the routine evaluation of xerostomia symptoms as part of the assessment of dry eye patients, and vice versa., Competing Interests: Declaration of Competing Interest None., (Copyright © 2019 British Contact Lens Association. Published by Elsevier Ltd. All rights reserved.)

Idiopathic CD4 lymphocytopenia (ICL) is a rare disease characterized by marked loss of CD4 T-cells without human immunodeficiency virus infection. CD4 T-cells play an important role in granuloma formation in cryptococcal infection. Thus far, among ICL patients, it has not been concluded definitely whether granuloma is formed or not. We report the case of a 39-year-old woman with ICL and disseminated cryptococcal infection with granuloma formation. She was referred to our department because of a lung mass, osteolytic lesion, and a subcutaneous mass identified on a computed tomography scan, and an elevated C-reactive protein level. Cryptococcus neoformans was isolated from the tissues. She also had marked CD4 lymphocytopenia (33 cells/μL), without human immunodeficiency virus infection. In a biopsy specimen of the lung mass, granulomas containing CD4 T-cells were observed. The cryptococcosis was treated with liposomal amphotericin B followed by fluconazole and she was found to be cured. The CD4 T-cell count was persistently low. This case showed that granulomas containing CD4 T-cells can be formed in ICL patients with cryptococcal infection despite very low CD4 T-cell counts., (Copyright © 2019 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.)

Background and Overview: The authors report the case of a patient with mixed connective tissue disease (MCTD) and Sjögren syndrome, showing signs and symptoms of bilateral trigeminal neuropathy and aseptic meningitis. The patient was assessed by means of quantitative sensory testing (QST) according to the German Research Network on Neuropathic Pain standards, in both the gingiva and forearm, and the results were compared with those of healthy control participants., Case Description: A 27-year-old woman, who had received a diagnosis of MCTD and Sjögren syndrome from a rheumatologist, sought treatment at an orofacial pain clinic for bilateral electriclike pain in the maxillary anterior gingiva, eyelids, and cheeks. QST indicated allodynia and hyperalgesia in response to mechanical and thermal stimuli in both her gingiva and forearm, and cold hyperalgesia in her forearm only. She had been prescribed an oral corticosteroid (prednisone, 7 milligrams per day) by the rheumatologist, and was given lidocaine gel and systemic pregabalin (400 mg/d) at the clinic., Conclusions and Practical Implications: The cause of trigeminal neuropathy in MCTD and Sjögren syndrome (SS) is unknown. The QST data in this case showed that the somatosensory disturbance severity was higher in the gingiva than in the forearm, suggesting that the trigeminal nerve may be more susceptible than other parts of the nervous system in patients with MCTD. If reproducible in future studies, the finding of greater hypersensitivity in the gingiva than in the forearm may provide an opportunity for dentists to play a role in the detection, diagnosis, or both of MCTD and SS. Dentists must be sufficiently familiar with MCTD and SS to include them in their differential diagnoses and should consider performing simple neurosensory testing such as via intraoral cotton swab or pinprick test., (Copyright © 2020 American Dental Association. Published by Elsevier Inc. All rights reserved.)

Sjögren syndrome (SS) is a systemic inflammatory and autoimmune disease characterized by systemic disorders of the exocrine glands, predominantly the salivary and lacrimal glands. Here, we report a 4-year-old boy who presented with the repetition of generalized tonic-clonic seizures for 1-2 min. Initially, he was diagnosed with idiopathic autoimmune encephalitis and was treated with steroids. He was eventually diagnosed with SS based on the examination results, such as inflammatory cell infiltration into the minor salivary glands and positive serum anti-SSA/Ro antibody. Although central nervous system complications are rare in pediatric SS, this condition should be considered in the differential diagnosis when a patient presents with idiopathic autoimmune encephalitis of unknown cause. Furthermore, SS can occur in relatively young children and can present without imaging abnormalities., (Copyright © 2018 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Autoimmune rheumatic diseases are characterised by an abnormal immune system response, complement activation, cytokines dysregulation and inflammation. In last years, despite many progresses in managing these patients, it has been shown that clinical remission is reached in less than 50% of patients and a personalised and tailored therapeutic approach is still lacking resulting in a significant gap between guidelines and real-world practice. In this context, the need for biomarkers facilitating early diagnosis and profiling those individuals at the highest risk for a poor outcome has become of crucial interest. A biomarker generally refers to a measured characteristic which may be used as an indicator of some biological state or condition. Three different types of medical biomarkers has been suggested: i. mechanistic markers; ii. clinical disease markers; iii. therapeutic markers. A combination of biomarkers from these different groups could be used for an ideal more accurate diagnosis and treatment. However, although a growing body of evidence is focused on improving biomarkers, a significant amount of this information is not integrated on standard clinical care. The overarching aim of this work was to clarify the meaning of specific biomarkers during autoimmune diseases; their possible role in confirming diagnosis, predicting outcome and suggesting specific treatments., (Copyright © 2018. Published by Elsevier B.V.)

The diagnostic evaluation of a patient with suspected postural tachycardia syndrome (POTS) requires a thoughtful diagnostic approach utilizing a careful clinical history and examination, laboratory, and autonomic testing. This article outlines the importance of a thorough history in identifying mechanism of symptom onset, clinical features, associated clinical conditions or disorders, and factors that may result in symptom exacerbation. The clinical examination involves an assessment of pupillary responses, an evaluation for sudomotor and vasomotor signs, and an assessment for joint hypermobility. Laboratory testing helps to exclude mimics of autonomic dysfunction, recognize conditions that may exacerbate symptoms, and to identify conditions that may cause or be associated with autonomic nervous system disease. The purpose of autonomic testing is to confirm a POTS diagnosis, exclude other causes of orthostatic intolerance, and may provide for characterization of POTS into neuropathic and hyperadrenergic subtypes. Other diagnostic studies, such as epidermal skin punch biopsy, exercise testing, radiographic studies, sleep studies, gastrointestinal motility studies, and urodynamic studies should be considered when clinically appropriate., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Tubulointerstitial nephritis (TIN) is the second most common cause of acute intrinsic kidney injury after acute tubular necrosis. Although drug-induced forms of TIN represent the vast majority, rheumatic disease is another common cause and often underdiagnosed. Early diagnosis of acute interstitial nephritis and prompt withdrawal of the culprit medication or a correct treatment can avoid chronic damage and progressive chronic kidney disease. This review highlights the recent updates, clinical features, and treatment in TIN in autoimmune rheumatic disease., (Copyright © 2018 Elsevier Inc. All rights reserved.)

We have reported characteristic cutaneous manifestations of Sjögren syndrome (SS) with special references to autoimmune anhidrosis or hypoidrosis and related mucocutaenous manifestations in addition to annular erythema or cutaneous vasculitis. Although significance of cutaneous manifestations of SS has been gradually recognized in rheumatologists, sudomotor function has not been fully evaluated and recognized in the diagnosis of SS except for dermatologists. SS is a relatively underestimated collagen disease in contrast to SLE, systemic sclerosis, or dermatomyositis, special care should be needed not to make misdiagnosis of SS when we see the patients with common skin disease such as, drug eruption, infections skin disease or xerosis in the daily practice. In contrast to pathomechanisms of dry skin observed in SS, we recently reported that reduced sweating function and dry skin seen in atopic dermatitis (AD) are mediated by histamine or substance P, those are usually restored to normal levels after improvement of the dermatitis by topical corticosteroid ointment with or without oral anti-histamine. Therefore, xerotic skin lesions seen in SS and AD might be attributable to different pathomechanisms with similar dry skin manifestations. We recently reported that SS promotes dry skin when complicated with AD possibly due to acceleration of hypoidrosis. In this review, we would like to summarize our recent understanding of regulatory mechanism of impaired sweating function in allergic inflammatory skin diseases by introducing clinical presentations of AD/SS overlap cases as the model of hypoidrotic inflammatory skin diseases., (Copyright © 2018 Japanese Society of Allergology. Production and hosting by Elsevier B.V. All rights reserved.)

Background and Overview: The authors describe dental treatment for a patient with a complex medical history of secondary Sjögren syndrome with systemic lupus erythematosus and rheumatoid arthritis., Case Description: An 18-year-old woman's rheumatology group referred her for oral evaluation; she had secondary Sjögren syndrome, systemic lupus erythematosus, and rheumatoid arthritis. The patient had multiple advanced carious lesions, extreme sensitivity, and hyposalivation. The patient selected a minimally invasive treatment plan that focused on silver diamine fluoride (SDF), partial caries removal, and glass ionomer cement (GIC) restorations. The SDF treatment and GIC restorations were successful in arresting carious lesions and restoring form and function but may not completely prevent new carious lesions from forming in the future., Conclusions and Practical Implications: The case shows that using less invasive treatments, such as SDF and GIC restorations can be used to manage complex cases involving extreme caries risk and be preferable to endodontic treatment and extractions., (Copyright © 2018 American Dental Association. Published by Elsevier Inc. All rights reserved.)