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Introduction: Sjogren's syndrome (SS) is an autoimmune chronic inflammatory disorder affecting women in their fourth to sixth decade, affecting gastrointestinal, and musculoskeletal systems. A 35-year-old female with SS with polymyositis (PM) presented with symptoms of weakness in all four limbs, difficulty in sitting, rising, swallowing solid foods, vomiting, and difficulty in climbing stairs. She was diagnosed with SS in 2018 and was treated with prednisolone, Vitamin D, calcium, pyridoxine, methylcobalamin, artificial tears, pilocarpine, and painkillers. However, a muscle biopsy was never done. Methods: The data were collected from the patient's file along with her consent when she came for follow-up. Results: A muscle biopsy was done at our center to confirm the diagnosis of PM. The patient was prescribed IV prednisolone and other symptomatic treatment until symptoms resolved and was discharged with oral drugs when they were manageable. Conclusion: The diagnosis of primary SS along with PM is a rare occurrence. Although it does not change the treatment plan much, its diagnosis is very important for managing any complications that may arise from it. Patients' noncompliance and loss of follow-up can create issues in the treatment. Such cases help in forming the guidelines for the future and restructuring the classification of autoimmune conditions. [ABSTRACT FROM AUTHOR]

Celiac disease (CeD) is an immune-mediated disorder affecting the small bowel, associated with genetic factors and increasing global prevalence. This study explores the association between CeD, Systemic Lupus Erythematosus (SLE), primary Sjogren syndrome (pSS), and Systemic Sclerosis (SSc). 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. 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. 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. [ABSTRACT FROM AUTHOR]

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. All the papers on cheilitis were comprehensively retrieved from the Scopus database, and divided into rheumatologists and stomatologists groups. 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. 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. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to determine physical activity levels and understand the factors influencing an active lifestyle among patients with primary Sjögren's syndrome (pSS). Methods: Ninety-seven patients participated in this multicentric study. Physical activity levels were assessed using the International Physical Activity Questionnaire-Short Form (IPAQ-SF). The Inflammatory Arthritis Facilitators and Barriers (IFAB) questionnaire was used to evaluate perceived barriers and facilitators to physical activity. Results: Forty-six patients were physically inactive and the rest of them were moderately active. Commonly identified barriers included a lack of motivation, fatigue, and pain. Conversely, knowledge of the health and mood benefits for physical activity emerged as a key motivator. Patients with better scores on facilitators and lower scores on barriers exhibited higher physical activity levels (p<0.05). Notably, a high level of perceived facilitators of physical activity (odds ratio [OR]: 1.02; 95% confidence interval [CI], 1.00 -- 1.05) and reduced pain (OR: 0.81; 95% CI: 0.69 -- 0.95) were linked to an active lifestyle. Conclusions: This study emphasizes the role of motivation and awareness of the benefits of physical activity for health and mood in driving physical activity for patients with primary Sjögren's syndrome. Tailored physical activity programs that address psychological aspects and disease-related pain, and fatigue should be designed to counter sedentary lifestyles in pSS patients. [ABSTRACT FROM AUTHOR]

We herein report a case of diffuse large B-cell lymphoma (DLBCL) of the palate secondary to Sjögren syndrome. A 55-year-old woman was found to have redness of the palate at a nearby dental clinic and was referred to the oral surgery department of another hospital in February 2007. It was difficult to distinguish mucosa-associated lymphoid tissue (MALT) lymphoma from severe inflammation based on a biopsy, so the patient was referred to our department. The mass showed redness, measuring 25 × 20 mm, and was smooth-surfaced and elastic hard. The results of a rebiopsy in our department suggested the possibility of MALT lymphoma, but the diagnosis could not be confirmed. At the same time, a systemic examination revealed complications with Sjögren's syndrome. The mass subsequently increased in size, and the entire mass was resected to confirm the diagnosis. The pathological final diagnosis was DLBCL. Complete remission has been maintained for over 14 years since therapy was performed. [ABSTRACT FROM AUTHOR]

Anti-aquaporin-4 antibody (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) and Sjögren syndrome (SS) are likely comorbidities. However, the exact effects of age and disease duration on the positivity rates of serum anti-Ro/SSA and anti-La/SSB (anti-SSA/SSB) antibodies and the presence of sicca symptoms in patients with AQP4-IgG remain unknown. In the present study, we evaluated the data from patients with suspected NMOSD who had neurological episodes and tested for serum AQP4-IgG. Associations between the presence of serum AQP4-IgG and SS-related findings were evaluated. The presence of anti-SSA/SSB antibodies [odds ratio (OR), 7.34; 95% confidence interval (CI), 5.71-9.43; p < 0.0001] and that of sicca symptoms (OR, 2.08; 95% CI, 1.67-2.58; p < 0.0001) were both higher in patients with AQP4-IgG (n = 1,651) than in those without AQP4-IgG (n = 2,796). Meanwhile, neither age nor the elapsed time from neurological onset was linked to the prevalence of anti-SSA/SSB antibodies or sicca symptoms, and the prevalence rates of the SS-related factors were elevated since the onset of neurological episodes in those with AQP4-IgG. The frequency of sicca symptoms among those with anti-SSA/SSB antibodies was irrespective of AQP4-IgG (OR, 1.11; 95% CI, 0.67-1.85; p = 0.6892). The measured AQP4-IgG titers did not differ significantly according to the presence of anti-SSA/SSB antibodies (p = 0.2386; Mann-Whitney U test). In summary, age and duration of NMOSD were not the factors producing an elevated prevalence of anti-SSA/SSB antibodies and sicca symptoms in patients with AQP4-IgG, implying that the occurrence of comorbid SS is likely to temporarily precede or synchronize with the onset of AQP4-IgG-positive NMOSD. [ABSTRACT FROM AUTHOR]

Background: We aimed to explore the underlying mechanism of the total glucoside of peony (TGP) in treating Sjogren syndrome (SS) using the network pharmacology approach. Methods: The protein targets of TGP and SS were identified by database search. Then, the intersection of the two groups was studied. The drug--target network between TGP and the overlapping genes was constructed, visualized, and analyzed by Cytoscape software. Gene Ontology and Kyoto Encyclopedia of Genes and Genomes enrichment were performed to analyze these genes. Finally, the predictions of potential targets were evaluated by docking study. Results: Forty-six overlapping genes were discovered. The results suggested that TGP used in the treatment of SS is associated with cellular tumor antigen p53, neurotrophic tyrosine kinase receptor type 1, and epidermal growth factor receptor, as well as their related 3372 protein networks, which regulate intrinsic apoptotic signaling pathway, cellular response to oxidative stress, rhythmic process, and other processes. Molecular docking analysis proved that hydrogen bonding is the main form of interaction. Conclusion: Our research provided the protein targets affected by TGP in SS treatment. The key targets (caspase 3, vascular endothelial growth factor A, glyceraldehyde-3-phosphate dehydrogenase, etc.), which involve 3372 proteins, are the multitarget mechanism of TGP in SS treatment. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Sjögren syndrome (SS) is an autoimmune disorder, affecting about 16,000 individuals in Italy, yet lacking a standardized therapy protocol and a plain inclusion in the reimbursed healthcare services. This raises many controversial issues about how managing the SS patient, to relief pain and discomfort and improve patients' health and social life. The ozone therapy resulted successful in previous reports, and therefore, it was used in this case report. CASE PRESENTATION: A 69-years old female outpatient, showing positivity to Schirmer's test, was previously diagnosed as a primary Sjögren syndrome, who later developed an autoimmune thyroiditis and showed the presence of rheumatoid factors. The patient suffered from a marked ocular dryness, subsequently to a purported endothelitis, alongside with fatigue and pain. Laboratory tests showed a positive ANA 1:320 in a speckled pattern with negative anti-SSA and anti-SSB tests. From December 2020 to January 2021 she underwent 2 routes of three sessions of oxygen-ozone autohemotherapy (O2-O3 AHT), as described below and improved, with only 2 sessions, her symptomatology and clinical outcome, as ocular dryness, fatigue and pain, rapidly disappeared. CONCLUSIONS: The use of ozone in the therapy of SS is a straightforward, affordable and feasible approach to treat primary Sjögren syndrome without significant side effects. [ABSTRACT FROM AUTHOR]

L'atteinte articulaire est une des manifestations extra-glandulaires les plus fréquentes dans le syndrome de Sjögren primitif (SSp) ou maladie de Sjögren. On éliminera toujours une PR, un lupus systémique et une spondyloarthrite qui peuvent avoir des présentations cliniques proches et peuvent s'associer au SS. L'atteinte articulaire touche près de la moitié des patients SSp avec des arthrites cliniques pour 16 % d'entre eux. Le plus souvent, il s'agit d'arthrites symétriques, des interphalangiennes proximales, métacarpophalangiennes (MCP) et poignets, touchant moins de 5 articulations. L'échographie met en évidence des synovites de grade 1 ou 2, avec un signal Doppler positif dans moins d'un quart des cas et des ténosynovites, majoritairement des fléchisseurs des doigts. Il n'y a pas d'arthrite érosive radiographique dans le SSp. L'utilisation d'imagerie plus sensible que la radiographie telles que l'échographie et l'IRM montre fréquemment des érosions des MCP et des poignets. Il faudra alors rechercher une polyarthrite rhumatoïde associée à un SS secondaire. Cinq à dix pour cents des patients atteints de SSp ont des anticorps anti-CCP. La présence d'anticorps anti-CCP n'exclut pas forcément le diagnostic de SSp mais doit alerter sur le risque d'un développement ultérieur d'une polyarthrite rhumatoïde et nécessite donc un suivi radiographique même si le SSp évolue depuis de nombreuses années. L'atteinte articulaire est de bon pronostic et semble être un facteur prédictif d'évolution clinique favorable. La prise en charge thérapeutique doit être guidée par le niveau d'activité clinique et reposera sur les AINS ou les corticoïdes ± l'hydroxychloroquine. Les traitements de fond synthétiques seront proposés à visée d'épargne cortisonique. Joint involvement is one of the most common extra-glandular manifestations in primary Sjögren's syndrome (pSS), affecting almost half of the patients with clinical arthritis in 16 %. Most commonly, these are symmetrical arthritis, affecting fewer than 5 joints. Ultrasound shows grade 1 or 2 synovitis, with a positive doppler signal in less than a fourth of cases, and tenosynovitis, mostly of the finger flexors. Radiographs should not show erosion. In presence of erosions on ultrasound or MRI, which may be found on MCP and wrists, RA with secondary SS should be excluded. Five to 10 % of patients with SSp have anti-CCP antibodies. The presence of anti-CCP antibodies does not necessarily exclude the diagnosis of SSp, but it should alert to the risk of further development of RA, therefore requiring radiographic follow-up even if the pSS has been evolving for many years. Joint involvement has a good prognosis and appears to be a predictor of a favourable clinical course. Treatment should be guided by the level of clinical activity and should be based on NSAIDs or corticosteroids ± hydroxychloroquine. Synthetic disease-modifying anti-rheumatic drugs should be offered for corticosteroid sparing. [ABSTRACT FROM AUTHOR]

Le syndrome de Sjögren (SS) primaire ou maladie de Sögren est une pathologie auto-immune à forte prédominance féminine (9/1) avec un pic de fréquence vers 50 ans, mais il existe des formes débutant avant 20 ans. Les formes avec la triade classique « sécheresse, fatigue et douleurs » sont un mode habituel d'entrée dans la maladie et constituent environ 30 % des patients avec SS primitif. Les atteintes systémiques avec une ou plusieurs atteintes viscérales se voient dans environ 70 % des cas. Phénomène de Raynaud tardif, atteinte bronchiolaire, atteinte interstitielle pulmonaire, synovites, neuropathies surtout sensitives, néphrite tubulo-interstitielle, poussées de parotidomégalie sont autant de modes de révélation possibles de la maladie, car le syndrome sec peut être au second plan. Enfin, le SS doit aussi être évoqué en cas de vascularite cryoglobulinémique dont l'expression associe souvent glomérulonéphrite membranoproliférative, purpura, livedo et mononeuropathie multiple. Plus rarement, c'est la découverte d'un lymphome de MALT ou la survenue d'un bloc auriculo-ventriculaire congénital qui fera découvrir un SS associé. Primary Sjögren's syndrome (SS) or Sjögren disease is an autoimmune disorder with a high female ratio (9:1) with a peak of frequency around 50-year-old, but there are also cases with early onset before the age of 20. Forms with the classic triad of dryness, fatigue and pain are a common mode of entry into the disease and account for about 30% of patients with primary SS. Systemic involvement with one or more visceral manifestations is seen in about 70% of cases. Late Raynaud's phenomenon, bronchiolar involvement, interstitial lung disease, synovitis, neuropathies (particularly sensory neuropathies), tubulointerstitial nephritis, and parotid enlargement are all possible entry points to the disease since the dryness may be in the background. Finally, SS should also be considered in cases of cryoglobulinemic vasculitis, which often combines membranoproliferative glomerulonephritis, purpura, livedo and multiple mononeuropathy. More rarely, it is the discovery of a MALT lymphoma or the occurrence of a congenital atrioventricular heart block that will lead to the diagnosis of SS. [ABSTRACT FROM AUTHOR]

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Les pathologies parotidiennes peuvent être d'origine inflammatoire (sialite), métabolique ou tumorale. De nombreuses maladies systémiques au premier rang desquelles le syndrome de Sjögren et la sarcoïdose peuvent engendrer une hypertrophie parotidienne. Le diagnostic différentiel, parfois difficile, malgré les examens complémentaires les plus poussés (échographie, IRM, ponction cytologique à l'aiguille fine) sera infectieux, lithiasique ou tumoral. Parotid enlargement can be of inflammatory (sialitis), metabolic or tumoral. Many systemic diseases, mainly Sjögren's syndrome and sarcoidosis, can lead to parotid hypertrophy. The differential diagnosis, which is sometimes difficult, despite radiological investigations) (ultrasound, MRI, cytological fine needle puncture) will be infectious, lithiasis or tumor. [ABSTRACT FROM AUTHOR]

Introduction: Combined central and peripheral demyelination (CCPD) is a rare entity in which central and peripheral nervous system demyelination coexist. Herein, we present a patient with coexistence of Sjögren syndrome (SS) and CCPD. Case Report: A 58-year-old female patient was admitted to our neurology clinic with paraparesis, difficulty walking, imbalance, and paresthesia. Neurological examination showed paraparesis, absence of lower extremity deep tendon reflex, sensory deficit at the T8 level, loss of deep sensory position, and vibration. Spinal magnetic resonance imaging revealed multiple focal T2-hyperintense and contrast-enhancing cord lesions. Fat-suppressed imaging disclosed T2 hyperintensity in lumbar nerve roots, diffuse linear enhancement of the cauda equina, and diffuse increased enhancement in lumbar nerve roots. Electrodiagnostic findings fulfilled the diagnostic criteria for chronic inflammatory demyelinating polyneuropathy. Extensive laboratory workup excluded all possible pathologies. The Schirmer test detected positive in both eyes and minor salivary gland biopsy resulted in grade 3. These results were consistent with SS. The patient received intravenous methylprednisolone, azathioprine hydroxychloroquine. Approximately 2 years later, her complaints had completely disappeared, except for mild sensory complaints. Conclusion: It is unclear whether the association of central nervous system and peripheral nervous system demyelination and SS is a coincidence or a consequence. Our patient shows that patients with SS can have CCPD, and a significant clinical response can be obtained with early treatment. We hope that this unique case sheds light on the pathophysiology of CCPD. [ABSTRACT FROM AUTHOR]

Sjögren's disease (SjD) is a heterogenous disease with a wide range of manifestations, ranging from symptoms of dryness, fatigue, and pain, to systemic involvement. Considerable advances have been made to evaluate systemic activity or patient-reported outcomes, but most of the instruments were not able to assess all domains of this multifaceted disease. The aim of this scoping review was to generate domains that have been assessed in randomized controlled trials, as the first phase of the Outcome Measures in Rheumatology (OMERACT) process of core domain set development. We systematically searched Medline (Pubmed) and EMBASE between 2002 and March 2023 to identify all randomized controlled trials assessing relevant domains, using both a manual approach and an artificial intelligence software (BIBOT) that applies natural language processing to automatically identify relevant abstracts. Domains were mapped to core areas, as suggested by the OMERACT 2.1 Filter. Among the 5,420 references, we included 60 randomized controlled trials, focusing either on overall disease manifestations (53%) or on a single organ/symptom: dry eyes (17%), xerostomia (15%), fatigue (12%), or pulmonary function (3%). The most frequently assessed domains were perceived dryness (52% for overall dryness), fatigue (57%), pain (52%), systemic disease activity (45%), lacrimal gland function (47%) and salivary function (55%), B-cell activation (60%), and health-related quality of life (40%). Our scoping review highlighted the heterogeneity of SjD, in the study designs and domains. This will inform the OMERACT SjD working group to select the most appropriate core domains to be used in SjD clinical trials and to guide the future agenda for outcome measure research in SjD [ABSTRACT FROM AUTHOR]

Background and Aims There are conflicting data on the association between inflammatory bowel diseases [IBD] and autoimmunity disorders. The aim of this study was to explore this association including the effect of medications. Methods We utilized health administrative data collected by three of the four health maintenance organizations [HMOs] in Israel, covering 52% of the country's population. We explored the prevalence of the following autoimmune disorders: insulin-dependent diabetes mellitus [IDDM], psoriasis, Sjögren syndrome, coeliac disease, systemic lupus erythematosus [SLE], primary sclerosis cholangitis [PSC] and autoimmune thyroiditis, among all IBD patients vs non-IBD controls. Case ascertainment was determined according to validated computerized algorithms. Results In total, 12625 IBD patients were compared to 12625 controls. A total of 1395 [11.1%] IBD patients had at least one autoimmune disease compared with 740 [5.9%] of non-IBD controls (odds ratio [OR] = 1.99 [95% confidence interval 1.81–2.19]; p < 0.05); all autoimmune diseases, except for thyroiditis, were more prevalent among IBD patients. Adjusted for confounding variables, anti-tumour necrosis factor [anti-TNF] medications were associated with a higher prevalence of psoriasis (54 [5.7%] in IBD vs 177 [4.1%] in controls; OR = 1.50 [1.07–2.08]; p < 0.05) but lower prevalence of Sjögren (1 [0.1%] vs 39 [0.9%]; OR [95% CI] = 0.13 [0.02–0.94]; p < 0.05) and coeliac disease (11 [1.2%] vs 68 [1.6%]; OR [95% CI] = 0.51 [0.27–0.99]; p < 0.05). Thiopurines and 5-aminosalicylates were not associated with any autoimmune disorder. Conclusion IBD is associated with all autoimmune diseases explored here except for thyroiditis. Anti-TNF users have a higher prevalence of psoriasis, and lower prevalence of Sjögren and coeliac disease. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To describe a patient with Sjögren syndrome (SS) associated with obsessive-compulsive disorder (OCD). PATIENTS AND METHODS: Case report and systematic review of the literature. CASE REPORT: A 40-year-old female patient with a history of xerostomia and xerophthalmia initiated in 2015. She also had a history of changing her behavior, and she practices rituals, recurrent obsessions, and compulsions. She was diagnosed with OCD. She was treated with fluoxetine associated with risperidone and then was changed for aripiprazole 10 mg/ day. Cognitive-behavioral therapy was also applied. She had good control of the obsessions with these drugs and psychotherapy. Laboratory tests showed positive antinuclear antibodies, anti-Ro/SS-A, and-La/SS-B antibodies. Schirmer test, break up time, and positive green lisamin were all positive. Scintigraphy and ultrasound of salivary glands were positive. A diagnosis of Sjögren syndrome was determined. She was treated with HCQ, vitamin D3 50,000 IU/week, omega-3 2 g, and artificial tears with a good response. Currently, 5 years later, the patient is asymptomatic and has OCD under adequate control even without drugs. CONCLUSIONS: This case illustrates a rare case of a patient with SS who evolved with OCD. [ABSTRACT FROM AUTHOR]

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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. [ABSTRACT FROM AUTHOR]

Abstract Objective We examined the symptoms in the oral and maxillofacial regions of patients with IgG4-related disease (IgG4-RD) and considered the meaning of a labial minor salivary gland (LMSG) biopsy in such patients. Methods Sixty-three patients diagnosed with IgG4-RD in our hospital, were examined for symptoms in the oral and maxillofacial regions. Out of the 24 patients who were referred to our department, 20 underwent immunohistochemical analysis of the LMSG for IgG and IgG4. Results Symptoms in the oral and maxillofacial region were observed in 18 of the 63 patients. Out of the 18 patients, 15 had swelling of the submandibular glands, 5 had severe xerostomia, 4 had swelling of the parotid glands, and 2 experienced irritation and pain while eating. IgG4-positive plasma cells in the LMSG biopsy samples were observed in 19 of the 20 patients examined (95%). In 6 patients, more than 40% of the infiltrated plasma cells were positive for IgG4; in 2 patients, more than 30% of the plasma cells were positive; in 3 patients, more than 20% of the plasma cells were positive; in 7 patients, more than 10% of the plasma cells were positive; in 1 patient, more than 5% of the plasma cells were positive. Conclusions Out of the 63 patients, only 18 (29%) had symptoms in the oral and maxillofacial regions. Infiltration of IgG4-positive plasma cells, regardless of their intensity in the non-symptomatic LMSG, might be considered as one of the important symptoms of IgG4-RD. [ABSTRACT FROM AUTHOR]

Organophosphate esters (OPEs) are widely used as flame retardants and plasticizers due to their excellent properties. The interference of OPEs on immune function has been proven, but the epidemiological data on OPEs exposure to related immune function diseases, such as sjögren syndrome (SjS), is limited. In cross-sectional study, 283 serum samples were collected from healthy individuals (n = 145) and patients with SjS (n = 138) in Hangzhou, China. Eight OPEs, triethyl phosphate (TEP), tributyl phosphate (TBP), tris (2-chloroethyl) phosphine (TCEP), triphenyl phosphate (TPHP), tri (1-chloro-2-propyl) phosphate (TCIPP), 2-ethylhexyldi-phenyl phosphate (EHDPP), tris (1,3-dichloro-2-propyl) phosphate (TDCIPP), and tri (2-butoxyethyl) phosphate (TBOEP), were frequently measured in serum samples. In addition, we explored the associations between the serum OPEs concentration and the risk of SjS. Results showed that TEP (mean controls 2.17 and cases 3.63 ng/mL) was the most abundant OPEs in the serum samples of the control and case groups, followed by TCIPP (mean controls 0.54 and cases 0.78 ng/mL). Serum TEP, TPHP, and EHDPP concentrations were positively correlated with SjS [odds ratio (OR): 1.97, 1.96, and 2.42, respectively; 95% confidence interval (CI):1.34–2.89, 1.34–2.87, and 1.34–2.87, respectively] in the adjusted model, and a negative correlation of TBP concentrations with SjS in the adjusted model (OR: 0.35, 95% CI: 0.17–0.70) was observed. Compared with the lowest quartile concentrations, the ORs of SjS at the highest quartile concentrations of TEP (OR: 4.93, 95% CI: 2.24–10.82) and TPHP (OR: 4.75, 95% CI:1.89–11.94) were significantly higher. This study suggests that human exposure to OPEs may increase the risk of SjS. [Display omitted] • Distribution of OPEs in serum of Hangzhou population in China was studied. • TEP was the major OPE in all serum samples. • TEP, TPHP, and EHDPP levels were significantly associated with risk of SjS. • We firstly found that human exposure to OPEs may increase the risk of SjS. [ABSTRACT FROM AUTHOR]

PURPOSE: Autologous serum eye drops are considered safe and efficient for the treatment of various ocular surface disorders, including dry eye diseases (DED) caused by the primary and secondary Sjogren syndrome (SS). However, the serum components in patients of SS may be different from those of normal patients and can thus lead to unpredictable therapeutic effects. This study divided the SS patients into active and inactive types based on the erythrocyte sedimentation rate and the presence or absence of active rheumatoid arthritis. METHODS: We compared the serum components of these two groups with standard and multiplex enzyme linked immunosorbent assay arrays and predicted the therapeutic effects of topical autologous serum for the treatment of DED with ocular surface disease index (OSDI) and Oxford Schema scale (OSS). RESULTS: Hyaluronic acid and transforming growth factor b1 levels were significantly higher in the active SS group compared to the inactive SS group (P < 0.01), whereas epidermal growth factors, insulin growth factor 1, and fibroblast growth factor b had no significant differences between these two groups. Active SS group had significantly higher expressions of interleukin (IL) 1 beta, IL 6, and tumor necrosis factor alpha compared to inactive SS patients (P < 0.05). There were no statistical differences in therapeutic effects between these two groups, as measured with the OSDI or OSS. CONCLUSION: Dividing the Sjogren dry eye patients into active and inactive groups may appear as a reasonable method to predict the quality of autologous serum eye drops, but there seems to be no significant predictability to the therapeutic effects. [ABSTRACT FROM AUTHOR]

Copyright of Implant Dentistry is the property of Lippincott Williams & Wilkins and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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. [ABSTRACT FROM AUTHOR]

Multicentric reticulohistiocytosis is a rare, non-Langerhans cell histiocytosis characterized by specific skin lesions and destructive arthritis. Clinically, it generally presents with multiple reddish-brown papulonodules over the hands, face, and trunk. The associated destructive arthropathy tends to be severe, with 50% of patients developing arthritis mutilans. Approximately 25–30% of patients present with neoplasia. Coexisting autoimmune diseases are also commonly reported. Here we report the case of a 59-year-old Taiwanese woman with underlying Sjögren syndrome who presented with the typical skin manifestations of multicentric reticulohistiocytosis and early interphalangeal joint involvement. The patient later showed partial response to methotrexate treatment. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

Germline mutations of signal transducer and activator of transcription 3 (STAT3) are responsible for 2 distinct human diseases: autosomal-dominant hyper-IgE syndrome (AD-HIES) caused by STAT3 loss-of-function mutations and STAT3 gain-of-function disease. So far, these entities have been regarded as antithetic, with AD-HIES mainly associated with characteristic infections and a connective tissue phenotype and STAT3 gain-of-function characterized by lymphoproliferation and poly-autoimmunity. The R335W substitution in the DNA-binding domain of STAT3 was initially described in 2 patients with typical AD-HIES, but paradoxically, recent functional analysis demonstrated a gain-of-function effect of this variant. A patient with Sjögren syndrome and features of AD-HIES with this mutation is described and the molecular consequences are further characterized. This study provides a clinical and immunological description of the patient. STAT phosphorylation in primary patient cells was studied and A4 cells transfected with the patient allele were used to study phosphorylation kinetics, transcriptional activity, and target-gene induction. The hybrid clinical features of the patient were associated with normal T H 17 cells. Enhanced and prolonged STAT3 phosphorylation, an increased STAT3 driven luciferase reporter activity upon IL-6 stimulation, but reduced IL-6–induced SOCS3 production were all observed. The germline R335W-STAT3 variant displays a mixed behavior in vitro that mainly shows gain-of-function, but also loss-of-function features. This is matched by an ambiguous clinical and immunological phenotype that dismantles the classical antithetic dualism of gain- versus loss-of-function. Germline STAT3 mutation–related disease represents a pathological spectrum with the p.R335W associated phenotype locating between the 2 recognized clinical disease patterns. [ABSTRACT FROM AUTHOR]

Epidemiological studies have associated air particulate matter (PM) inhalation with a decline in lung function and increased morbo-mortality due to cardiorespiratory diseases, particularly in susceptible populations. Sjögren's Syndrome (SS) is a chronic autoimmune disease characterized by cellular infiltration in exocrine glands and extraglandular tissue, being the respiratory tract an important target. We evaluated the effect of PM on the airways of NOD mice, which develop SS and BALB/c mice. BALB/c or NOD mice (2–3 months) were randomized in two groups and exposed to intranasal instillation either with saline (control) or ROFA solution (1 mg/kg body weight). After 24 h, mice were euthanized in order to perform lung histology, or measure total cell number (TCN), differential cell count (DCC) and superoxide anion generation in the bronchoalveolar lavage (BAL) fluid. BALB/c mice showed normal histoarchitecture, while NOD mice showed lymphocytic peribronchial infiltrates. ROFA exposure affected the respiratory tract from both BALB/c and NOD mice, with a significant increase in the TCN ( p < 0.05) and generation of O 2 − ( p < 0.05), as well as an imbalance in the DCC ( p < 0.05). All histological observations correlated with the cellular parameters evaluated. Lesions in NOD mice were more severe than those of BALB/c, showing cellular infiltration in the alveoli and leading to a greater decrease in the alveolar space. We have proved that in this experimental Sjögren's Syndrome animal model (NOD mice); airborne pollution exacerbates pre-existing pulmonary lesions. These findings show experimental evidence on the harmful effects of airborne pollution on the airways of patients with Sjögren's Syndrome. [ABSTRACT FROM AUTHOR]

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Copyright of Cukurova Medical Journal / Çukurova Üniversitesi Tip Fakültesi Dergisi is the property of Cukurova University, Faculty of Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract: Mikulicz's disease is a systemic IgG4-related condition that has recently been referred to as IgG4-related dacryoadenitis and sialoadenitis. We describe relapsed IgG4-related dacryoadenitis and sialoadenitis, so called Mikulicz's disease in a 69-year-old man who was administered with prednisolone (40mg/day) during March 2007. The dosage was tapered to 0.5mg/day over a period of 2 years with observation of the patient's clinical course. However, the bilateral submandibular and parotid glands became swollen in July 2009. A whole-body examination revealed no other illnesses or complications. A biopsy of the submandibular gland revealed IgG4-positive plasma cells. Relapsed Mikulicz's disease was diagnosed and the patient was started on prednisolone (40mg/day) again. At the time of writing, the patient is being maintained on 7mg/day of prednisolone. [Copyright &y& Elsevier]

Purpose: To report the magnetic resonance (MR) imaging features of the spinal cord and brain in patients of neurbmyelitis optica (NMO). Materials and Methods: Between January 2001 and March .2010, the MR images (spinal cord, brain, and orbit) and the clinical and serologic findings of 11 NMO pa- tients were retrospectively reviewed. The contrast-enhancement of the spinal cord was performed (20/23). The presence and pattern of the contrast-enhancement in the spinal cord were classified into 5 types. Results: Acute myelitis was monophasic in 8 patients (8/11, 72.7°/o); and optic neu- ritis preceded acute myelitis in most patients. Longitudinally extensive cord lesion (average, 7.3 vertebral segments) was invoved. The most common type was the dif- fuse and subtle enhancement of the spinal cord with a multifocal nodular, linear or segmental intense enhancement (45°/o). Most of the brain lesions (5/11, 10 lesions) were located in the brain stem, thalamus and callososeptal interphase. Anti-Ro au- toantibody was positive in 2 patients, and they showed a high relapse rate of acute myelitis. Anti-NMO lgG was positive in 4 patients (4/7, 66.7%). Conclusion: The imaging findings of acute myelitis in NMO may helpful in making an early diagnosis of NMO which can result in a severe damage to the spinal cord, and to make a differential diagnosis of multiple sclerosis and inflammatory diseases of the spinal cord such as toxocariasis. [ABSTRACT FROM AUTHOR]

Abstract: Pulmonary complications associated with Sjögren syndrome (SS) have attracted attention in recent years. Sjögren syndrome has been associated with small cyst formation in salivary glands, thymus, and lungs and has been recently brought to the forefront by radiologists due to high-resolution techniques. However, pathologists are less aware of this finding unless clinico-radiologic-pathologic correlation is sought. Formation of large bullae in SS is a rare complication with potential for confusion with other diseases. Here, we present the clinical, radiologic, and pathologic findings in 3 patients with SS associated with multiple pulmonary cystic lesions. All 3 patients had a variable mixed restrictive and obstructive component of the disease. There was good correlation with the pulmonary function tests (PFTs), high-resolution computed tomographic scan, and morphology with regard to the restrictive component. The small cysts appear to correlate with the extent of obstructive changes on the PFTs. However, the large bullae do not, implying noncommunication with the conducting airways. This noncorrelation between the PFTs and extent of bullous disease with predominant involvement of lower lobes in SS enables distinction from bullous emphysema. The mechanism of bulla formation in SS appears to be different from bullous emphysema. A check valve mechanism has been proposed previously in SS, which does not explain cyst formation in the thymus. Alternately, inflammation may play a role with the key suspects being CD4 T-helper cells and perhaps NK cells. This is the first report of a clinico-radiologic-pathologic correlation with analysis of lymphocyte subsets. [Copyright &y& Elsevier]

Background: Previous studies provide evidence that there is a greater frequency of autoimmune diseases among patients with psoriasis than in the general population. Objective: This study examined the association between psoriasis and 21 common autoimmune diseases, specified a priori. Methods: A retrospective cohort study was conducted among persons who were members of Kaiser Permanente Southern California from 2004 to 2011. A total of 25,341 patients with 2 or more diagnosis codes for any psoriatic disease were evaluated. Five persons not meeting this case definition were matched to each psoriatic patient based on age, sex, and length of enrollment. Results: Patients with psoriasis were more likely to have at least 1 other autoimmune disease (odds ratio [OR] 1.6; 95% confidence interval [CI] 1.5-1.7) and to have at least 2 other autoimmune diseases (1.9; 95% CI 1.6-2.4). Of the 17 conditions evaluated, associations with 14 were found to be statistically significant. The strongest association was with rheumatoid arthritis (3.6; 95% CI 3.4-3.9). Limitations: Patients with autoimmune conditions are likely to have a greater number of health care encounters, which may result in overascertainment and misascertainment of immune-mediated conditions, although the patients included in the study averaged 5.2 years of observation and the comparison subjects were matched on length of enrollment. Conclusions: The study suggests a genetic or environmental cause common across autoimmune diseases. Further investigation of individuals with multiple autoimmune diseases may yield important clues about the origin and pathogenesis of the disease. [ABSTRACT FROM AUTHOR]

Background: Chronic urticaria (CU) is a common disease in which most cases were considered to be idiopathic. Recent evidence indicates that at least a subset of cases of chronic idiopathic urticaria are autoimmune in origin. Objective: We aimed to characterize the association between CU, autoimmune diseases, and autoimmune/inflammatory serologic markers in a large unselected population. Methods: Data on 12,778 patients given a diagnosis of CU by either allergy or dermatology specialists during 17 years in a large health maintenance organization in Israel were collected. For each patient, we collected information on diagnosis of major, well-defined autoimmune diseases and autoimmunity- and inflammatory-related serologic markers. Similar data were collected for a control group comprised of 10,714 patients who visited dermatologists, family physicians, or allergy specialists and had no indication of CU. Results: Having CU was associated with an increased odds ratio for hypothyroidism, hyperthyroidism, and antithyroid antibodies. Female patients with CU had a significantly higher incidence of rheumatoid arthritis, Sjögren syndrome, celiac disease, type I diabetes mellitus, and systemic lupus erythematosus, mostly diagnosed during the 10 years after the diagnosis of CU. High mean platelet volume, positive rheumatoid factor, and antinuclear antibodies were all significantly more prevalent in patients with CU. Conclusions: A strong association was found between CU and major autoimmune diseases. A common pathogenic mechanism is implied by the high prevalence of autoantibodies and the existence of a chronic inflammatory process expressed by the high mean platelet volume. These findings have implications for the diagnosis, management, and prognosis of patients with CU. [ABSTRACT FROM AUTHOR]

Abstract: Introduction: Salivary and lacrimal gland dysfunction is relatively frequent after radioiodine therapy. An association of Sjögren’s syndrome (SS) and other autoimmune rheumatic diseases as fibromyalgia syndrome (FMS) has been reported. Thyroid autoimmunity in FMS patients is higher than normal subjects. Aim of the work: To detect the occurrence of Sjögren syndrome (SS) and any rheumatologic association in cancer thyroid patients after radioactive iodine therapy (I-131) and evaluate the salivary and lacrimal glands function. Patients and Methods: Thirty-one patients with post-surgical differentiated thyroid carcinomas with a mean age 40.13±9.82years, were referred for I-131 therapy (mean dose 212.9±101.63mCi) and continued the follow-up study. All patients had no symptoms or signs of SS. Thorough rheumatological examination was performed for any musculoskeletal manifestation or associated fibromyalgia syndrome (FMS). Before and 8–12months after I-131 therapy, salivary glands function was estimated by sequential scintigraphy, while lacrimal gland function was assessed by Schirmer’s test. Antinuclear antibody (ANA), anti-Ro (SS-A), anti-La (SS-B) and rheumatoid factor (RF) were performed. Results: All patients had a normal salivary glands scintigraphy and Schirmer’s test before I-131 therapy. On follow up, primary SS occurred in 8 patients (25.81%) while a significant decrease in salivary function occurred in 18 (58.1%) patients and significantly correlated with the I-131 dose. Schirmer’s test was significantly abnormal in those with SS. Serum Anti Ro and Anti La levels became significantly higher in SS patients (18.25±11.61 and 25±13.06U/ml) compared to the others (6.57±1.8 and 7.35±1.8U/ml), respectively, (p 0.025 and 0.006). Fibromyalgia syndrome was present in 12 patients (38.71%) and 6 of them developed SS. Conclusion: Assessment and follow up of salivary and lacrimal glands function is essential in patients receiving radioiodine therapy. Abnormal level of anti-Ro and Anti-La increase the risk for SS that should be closely monitored and fibromyalgia is a common association. [Copyright &y& Elsevier]

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Summary: Sjögren''s syndrome (SS) is a chronic autoimmune rheumatic disease characterized by a progressive lymphocytic infiltration of exocrine glands, especially salivary and lachrymal ones, leading to xerostomia, parotid gland enlargement, and xerophtalmia. SS may occur alone (primary) or in association with almost any of the autoimmune rheumatic diseases (secondary), the most frequent being rheumatoid arthritis. The aim of this study is to describe the capillaroscopic pattern of the labial mucosa in patients with SS. Methods: A total of 20 patients affected by SS and 20 healthy controls were examined. The patients with conditions that compromise microcirculation, such as diabetes, hypertension, hyperlipidemia or some pharmacological treatments, were not included in the study. All the patients were non-smokers. Labial capillaroscopy has been used to investigate the features of microcirculation. Visibility, course, tortuosity, as well as the possible presence of microhemorrhage, the average calibre of the capillary loops and the number of visible capillary loops per square millimetre were evaluated for each patient. Results: The results show evident alterations in the capillaries and a typical conformation of the labial microcirculation in SS patients. Conclusion: Labial microcirculation alterations occur in SS patients. The study of the microcirculation in autoimmune pathology such as SS could provide new understanding on the dynamics of the pathology and could help to complete the diagnosis. [Copyright &y& Elsevier]

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Autoimmune diseases are a group of heterogeneous disorders equally characterized by the same pathogenetic mechanism: an immunological reaction against self antigens promoted by antibodies, immuno-complex formation, and self-reactive T lymphocytes. Autoimmune diseases may be separated into organ-restricted diseases and systemic ones. The damage of single organs produced by antibodies focused against specific cellular antigens characterizes the first group of diseases, whereas the latter are produced by a systemic inflammatory process initiated by inappropriate and excess immune activation that leads to immuno-complex formation and deposition onto sensitive tissues. Since connective and vascular tissue are principally damaged in these disorders, systemic autoimmune diseases are more commonly known as “connective tissue diseases” (CTD) and include: systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, Sjogren syndrome, and others. Although they are considered as different from a pathogenetic point of view, they overlap in many aspects, such as general symptoms as fever and fatigue, chronical ongoing, steroid therapy. As patients suffering from CTD are predominantly young women between the ages of 20 and 40 years, which is the period of the highest childbearing potential, particular interest must be regarded to the impact that these diseases and their therapies have on pregnancy and, conversely, the effect of pregnancy on these disorders, which may have long-lasting implications for mothers and neonates. Adverse fetal outcomes, maternal disease flares, and drug potential teratogenic risk are the main reasons why women suffering from CTD and who are pregnant or intend to become pregnant are considered a high-risk population. These patients require integrated, interdisciplinary care, addressing every aspect of rheumatology, obstetrics, and neonatology to reduce maternal, fetal, and neonatal complications. [Copyright &y& Elsevier]

Summary: Connective tissue disorders are a known cause of interstitial pneumonias. But we are reporting here a case of primary Sjogren syndrome, which presented as interstitial pneumonia. There was complete clinical and radiological improvement with treatment with Mycophenolate and Prednisolone. [Copyright &y& Elsevier]

Background: Accurate inflammatory mechanisms in chronic ocular surface diseases (OSDs) cannot routinely be assessed. New techniques for investigating ocular surface inflammatory pathways are of major importance. Objective: To investigate the expressions of CCR4 and CCR5, known to be related to the TH2 and TH1 systems, respectively, and HLA-DR in conjunctival impression cytology specimens from patients with chronic OSDs. Methods: In this case-controlled study, impression cytology specimens were taken in a series of patients with vernal keratoconjunctivitis (n=21), giant papillary conjunctivitis (n=6), or keratoconjunctivitis sicca (KCS; n=17), or receiving topical antiglaucoma treatments (n=31), and from 20 normal subjects. Conjunctival cells were incubated with mAbs to CCR4, CCR5, CD45, and HLA-DR to quantify conjunctival inflammation in a masked manner using flow cytometry. Results: HLA-DR was higher in the glaucoma and KCS groups than in allergic and normal eyes. CCR4 was overexpressed in allergy and glaucoma, whereas CCR5 was higher in the KCS and glaucomatous groups. CD45 was expressed by only few cells in all groups, with almost no significant differences. CCR4 expression was negatively correlated with CCR5 and HLA-DR, whereas CCR5 was positively correlated with HLA-DR. Conclusion: This study confirms the overexpression of chemokine receptors by the conjunctival epithelium in OSDs. CCR4 and CCR5 expression may vary according to the immune pathway involved. Accurate mechanisms in ocular surface inflammatory reactions—that is, those related to the TH1 or TH2 systems—could be differentiated by CCR4/CCR5 profiles. Our results also suggest that long-term use of topical treatments may stimulate both systems. [Copyright &y& Elsevier]

Summary: We report here 4 cases of multilocular thymic cysts (MTCs) with reactive lymphoid follicular hyperplasia. They were admitted to our hospital to examine anterior mediastinal masses demonstrated on chest computed tomographic scans. Three patients presented high-grade intermittent fever, and 2 patients were associated with Sjogren syndrome with elevated serum antinuclear antibody levels. All patients were subjected to extended thymectomy. Interestingly, their fever disappeared immediately after surgery. Histologically, the lesions were characterized by several cystic spaces separated by various thick walls with dense lymphoid tissue containing large reactive germinal centers. The inner cyst walls were lined by flattened cuboidal epithelia in some portions. Columnar epithelia with focal cilia were partially observed in 2 cases. These pathological findings led to a diagnosis of MTCs that were thought to result from cystic transformation of medullary duct derivatives by acquired inflammatory processes. The pathological findings, together with clinical courses of our cases, suggest that inflammation accompanied by autoimmune diseases may play, in part, an important role in the development of MTCs. [Copyright &y& Elsevier]

Copyright of West Indian Medical Journal is the property of West Indian Medical Journal (WIMJ) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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Sjögren syndrome (SS) is a systemic autoimmune disease characterized mainly by the presence of keratoconjunctivitis sicca and xerostomia. Patients also may experience inflammation of the joints, muscles, nerves, lungs, gastrointestinal tract, thyroid, lymph nodes, or any other organ of the body, as well as fatigue, disturbed sleep, and memory problems. The etiology and pathophysiology of SS are not well understood, and diagnosis presents a challenge. Although there is no cure, management includes pharmacologic and nonpharmacologic interventions with the goal of improving quality of life by treating the sicca and fatigue symptoms. [ABSTRACT FROM AUTHOR]

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Introduction: Neuromyelitis optica (NMO) combined with large vessel abnormalities has been reported only once in the literature in a patient with Sjögren syndrome. Materials and Methods: Case report. Results: A 30-year-old woman was diagnosed with optic neuritis in her left eye. She was treated with intravenous methylprednisolone and made a full recovery. Seven months later she was readmitted with a 20-day-history of incoercible vomiting, associated with hiccups and diarrhea, followed by dysesthesia in the lower limbs and left arm, and deterioration of vision in her left eye. Brain MRI disclosed lesions in the left optic nerve, periependymal surface of the fourth ventricle, dorsolateral aspect of the medulla oblongata and pons, and hypothalamic region, with some enhanced by gadolinium. Brain MRA revealed segmental stenosis and irregularity of the vertebral and basilar artery walls, and stenosis of the left posterior cerebral artery. Hypoplasia of a segment of the right carotid artery was also observed. An analysis of cerebrospinal fluid revealed an increased protein concentration and IgG index. The remaining laboratory work-up, including indirect immunofluorescence assay for serum NMO-IgG was negative. A presumptive diagnosis of primary vasculitis of the central nervous system was made. Examination disclosed normal visual function in the right eye. In the left eye the visual acuity was counting fingers; there was an inferior altitudinal field defect and a pale optic disc. Pyramidal signs were also present. Serum aquaporin-4-IgG (AQP4-IgG) was detected by cell-based assay. A further brain MRI showed similar lesions as previously seen. Brain MRA showed minimal irregularities of the walls of the vertebral arteries with no abnormalities in the basilar and posterior cerebral arteries. Discussion and Conclusion: Transient vascular abnormalities of large cerebral arteries are rarely found in NMOSD and their mechanisms remain to be determined. Anti-AQP4-IgG seropositive status is helpful to differentiate NMOSD from other causes of stenosis and irregularities of the vessel walls. [ABSTRACT FROM AUTHOR]

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is an immune-mediated disorder in which aquaporin-4-IgG (AQP4-IgG) plays a major role. The high prevalence of associated autoimmune disorders and other autoantibodies in NMOSD suggests a genetic predisposition to autoimmunity. Herein are presented two case reports demonstrating that NMOSD and Sjögren syndrome (SS) co-exist in a common milieu of autoimmunity, irrespective of the AQP4-IgG serostatus of the patient. Materials and Methods: We report on two patients who met the current diagnostic criteria for SS and NMOSD. A review of the PubMed database up to December 2013 was also conducted for similar case reports in the English language. Results: Case 1: 45-year-old white female presented four attacks of optic neuritis since the age of 22 years with full recovery. She was diagnosed with SS at 30 years. Work-up disclosed positive serum anti-SSA, anti-SSB, ANA, anti-thyroglobulin, anti-AchR, and anti-AQP4 antibodies. Brain MRI showed lesions in the left optic nerve and the right frontal cortex. The Schirmer's test, Rose Bengal test, and salivary gland scintigraphy were positive. One year later she developed myelitis. Spinal MRI showed lesions in C2 and from T4 to T10. Case 2: 44-year-old white female with SS since childhood developed myelitis. Spinal MRI showed T2 to T7 lesion. Brain MRI showed lesions in the midbrain, and supratentorial small patches. Tire patient made a full recovery. One month later, she developed bilateral optic neuritis and myelitis. Serum anti-SSB-Ab was positive but anti-AQP4-Ab was negative (cell-based assay). Biopsy of the salivary glands was positive for SS. Adding our patients, a total of 49 cases meet the diagnostic criteria for both SS and NMOSD (42 female; mean age at onset of NMOSD: 36.2 years (10-74 years); full NMO: 27 patients, limited NMO: 13 patients). Discussion and Conclusion: The AQP4-IgG serostatus was positive for the first patient and negative for the second. The present cases, together with those previously reported, suggest that SS and NMOSD coexist in an autoimmune milieu, and are not necessarily dependent on the presence of AQP4 autoimmunity. [ABSTRACT FROM AUTHOR]