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ID PMID Title PublicationDate abstract
24332336 Essential mixed cryoglobulinemia manifesting as recurrent perioral edema: report of a case 2014 Jan Cryoglobulins are immunoglobulins that precipitate in vitro at temperatures <37°C but that redissolve if heated; they are broadly divided into 3 classes. Cryoglobulinemia has a plethora of clinical manifestations, but it typically presents a vasculitic syndrome, most notably a triad of purpura, arthralgia, and weakness secondary to cryoglobulin deposition in small or medium-sized blood vessels. The highly variable clinical presentation and inconsistent laboratory isolation of cryoglobulins mean that the diagnosis may be one of exclusion or of retrospective review. We describe a case of a 67-year-old woman whose principal symptom was recurrent perioral edema, which was characteristically induced by cold. Raised cryoglobulins combined with a positive autoantibody screen suggested mixed cryoglobulinemia secondary to Sjögren syndrome. However, in the subsequent 6 years, she has remained generally well, having developed no autoimmune, neoplastic, or other disease, suggesting the diagnosis is one of "essential" mixed cryoglobulinemia. No treatment other than symptomatic analgesia has been required.
24287191 Salivary gland biopsy for Sjögren's syndrome. 2014 Feb Salivary gland biopsy is a technique broadly applied for the diagnosis of Sjögren's syndrome (SS), lymphoma accompanying SS, sarcoidosis, amyloidosis, and other connective tissue disorders. SS has characteristic microscopic findings involving lymphocytic infiltration surrounding the excretory ducts in combination with destruction of acinar tissue. This article focuses on the main techniques used for taking labial and parotid salivary gland biopsies in the diagnostic workup of SS with respect to their advantages, their postoperative complications, and their usefulness for diagnostic procedures, monitoring disease progression, and treatment evaluation.
24170112 Tuberculosis infection in primary Sjögren's syndrome: a nationwide population-based study 2014 Mar Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease and may complicate with interstitial lung disease. The risk of Mycobacterium tuberculosis (TB) infection in patients with pSS has not been determined. This nationwide population-based study aimed to explore the incidence and risk factors of TB infection in patients with pSS. We identified 4,822 pSS patients from the Taiwan National Health Insurance database and compared the incidence rates of TB infection in these patients with 48,220 randomly selected age-, sex-, and comorbidity-matched subjects without pSS. The Cox proportional hazard model was used to identify risk factors for TB in patients with pSS. The risk of TB was higher in the pSS cohort than in the control cohort with an incidence rate ratio (IRR) of 1.58 (95% confidence interval [95% CI] 1.13-2.18, p = 0.006). The risk factors for TB in the pSS cohort were age ≥60 years (hazard ratio [HR] 3.22, 95% CI 1.78-5.84; p < 0.001), and corticosteroid usage, which had a dose-dependent effect in the pSS patients compared to the nonusers (daily prednisolone dose or equivalent less than 5 mg/day: HR 2.34; p = 0.020, 95% CI 1.14-4.78; 5 mg/day to less than 10 mg/day: HR 4.79, 95% CI 2.15-10.68; p < 0.001; 10 mg/day or more: HR 12.19, 95% CI 4.42-33.63; p < 0.001). Patients with pSS had a higher risk of pulmonary TB in Taiwan, which was related to age ≥60 years and corticosteroid usage.
24035411 Ultrasound assessment of the entheses in primary Sjögren syndrome. 2013 Dec Although tendon pain is commonly reported in primary Sjögren syndrome (pSS), the underlying mechanism is unknown. The objective of this study was to determine whether tendon pain in pSS is related to involvement of the tendons and entheses. We performed a physical examination of 288 entheseal insertion sites in 16 patients with active pSS and 162 entheseal insertion sites in 9 healthy controls without symptoms or medications. Then ultrasound was used to assess entheseal and tendon sites of these patients (n = 208) and controls (n = 117). Ultrasound was performed in B mode first to detect structural damages, and in power Doppler mode to detect blood flow abnormalities. By physical examination, 9 (56%) of the 16 pSS patients had pain to palpation of fibromyalgia tender points, two of whom had more than 11 tender points. The number of positive points ranged from 2 to 18, with a mean of 8 ± 6.0. None of the healthy controls had positive tender points. None had structural or blood-flow abnormalities by ultrasound. To conclude, this study provides the first data on ultrasound findings in patients with active pSS. Although 56% of patients had clinical tender points, none had structural or blood-flow abnormalities by ultrasound, suggesting the absence of inflammation of the tendons and entheses in this disease.
23812021 Primary adenoid cystic carcinoma of the skin metastatic to the lymph nodes: immunohistoche 2014 Mar Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare adnexal skin tumor first described in 1975, of which merely 62 cases have so far been studied in detail and reported in the English literature. PCACC is usually regarded as apocrine in origin/differentiation, but its precise histogenesis is still not well known. PCACC has in most cases a rather indolent course but can produce local recurrences and, more rarely, regional (lymph node) and distant (pulmonary) metastases. We report herein a Greek woman with a long-standing PCACC that grew slowly over several years and produced metastasis in the regional lymph nodes, highlighting the potentially aggressive course of this tumor. The primary and metastatic tumors were studied immunohistochemically and proved to express several (sweat gland-related) antigens (such as keratin 7, epithelial membrane antigen, CD10, and CD117) but neither hormonal receptors nor p63 or Gross Cystic disease Fluid Protein 15. The salient clinicopathologic features of this rare cutaneous adnexal tumor are reviewed.
23806216 Cut-off levels of salivary beta2-microglobulin and sodium differentiating patients with Sj 2013 Sep OBJECTIVES: For the diagnosis of Sjögren's syndrome (SS), cut-off levels of β2-microglobulin (β2MG) and sodium (Na+) in unstimulated whole saliva have not yet been shown. We aimed to determine the cut-off levels of salivary β2MG and Na+ which differentiate SS patients from non-SS patients and healthy controls. METHODS: Seventy-one patients of primary SS (pSS, 69 females/2 males, 60.0±16.8 years old), 50 of secondary SS (sSS, 49/1, 55.8±17.4), 54 of connective tissue diseases other than SS (non-SS-CTD, 43/11, 60.0±16.0), and 75 healthy volunteers (HC, 43/32, 50.7±15.6) were included. Unstimulated whole saliva were examined for levels of β2MG, Na+, potassium (K+), and chloride (Cl-). Receiver-operating characteristic curve analysis was carried out. RESULTS: β2MG, Na+, and Cl- levels in the SS group (pSS and sSS) were significantly higher than those in the non-SS group (non-SS-CTD and HC). The salivary β2MG level was 5.3±4.6 mg/L in pSS, 5.1±2.0 in sSS, 2.5±2.1 in non-SS-CTD, and 1.2±0.7 in HC, respectively. The Na+ level was 39.2±25.2 mEq/L, 36.4±26.1, 19.6±16.8, and 16.5±7.3, and the Cl- level was 51.1±25.0, 47.8±24.3, 32.1±16.6, and 27.0±7.9 in the same order. The K+ level in the SS group was significantly higher than that in HC. The optimal cut-off β2 MG and Na+ levels that differentiate the SS group from the non-SS group were 2.3 mg/L and 23 mEq/L. CONCLUSIONS: Salivary β2MG and Na+ levels are useful markers for differentiating SS patients from non-SS-CTD patients and HC.
23411513 [Detection of oral fungal flora in patients with primary Sjogren's syndrome by real-time P 2013 Feb 18 OBJECTIVE: To compare the quantity of the main important Candida species in the oral cavity between the patients with primary Sjogren's syndrome (pSS) and no dry mouth healthy controls,and to explore the discriminative species of fungi between the two groups. METHODS: In this study, 25 pSS patients from Department of Oral Medicine of Peking University School and Hospital of Stomatology were enrolled, with 25 residents from a community in Beijing as the control group. All the participants were examined for the oral mucosa status, and 5 Candida DNA loads were compared between the two groups by use of the FQ-PCR technique. RESULTS: Candida albicans was the dominant Candida in both of the groups. In addition, we found that the species of Candida tropicalis and Parapsilosis were more detected in the pSS group by use of the FQ-PCR technique. Quantitative of Candida tropicalis and Candida parapsilosis in pSS group (10.6 ± 1.07, 9.47 ± 4.86) was significantly higher than that in healthy controls group (8.30 ± 3.82, 5.24 ± 6.05), the difference was statistically significant (P <0.05). Quantitative of fluconazole-sensitive strains in pSS group (12.21 ± 0.82) compared with which in healthy controls group (11.53 ± 0.81) was significantly increased (P <0.01). CONCLUSION: Candida albicans was the dominant Candida in both of the groups. Candida tropicalis and Candida parapsilosis were more detected in the pSS group.
23701206 Involvement of autoimmunity to REG, a regeneration factor, in patients with primary Sjögr 2013 Oct The regenerating gene (Reg) was isolated originally as a gene specifically over-expressed in regenerating pancreatic islets and constitute a growth factor family. Reg gene product (Reg) is important in the pathophysiology of various human inflammatory diseases. Recently, the possible involvement of human REG in the regeneration of salivary ductal epithelial cells of patients with primary Sjögren's syndrome (SS) was reported. However, the expression of the REG family genes in minor salivary glands (MSG) and the occurrence of anti-REG Iα autoantibodies in SS patients were obscured. In this study, we examined the expression of REG family genes in the MSG of SS and screened anti-REG Iα autoantibodies in SS. The mRNA levels of REG family genes in MSG were quantified using real-time reverse transcription-polymerase chain reaction (RT-PCR) and REG Iα expression in the MSG was analysed by immunohistochemistry. The mRNA level of REG Iα in the MSG of SS patients was significantly higher than that of control. REG Iα protein was expressed highly in SS ductal epithelial cells. Anti-REG Iα autoantibodies in the sera were found in 11% of SS. All the MSG in the anti-REG Iα autoantibody-positive group showed REG Iα expression, whereas only 40% showed REG Iα expression in the anti-REG Iα autoantibody-negative group. The anti-REG Iα autoantibody-positive group showed significantly lower saliva secretion and a higher ratio of grade 4 (by Rubin-Holt) in sialography. These data suggest strongly that autoimmunity to REG Iα might play a role in the degeneration of MSG ductal epithelial cells in primary SS.
24304965 Targeting the splicing of mRNA in autoimmune diseases: BAFF inhibition in Sjögren's syndr 2014 Apr BAFF (B-cell-activating factor of the tumor necrosis factor family), a pivotal cytokine for B-cell activation, is overexpressed by salivary gland (SG) epithelial cells in primary Sjogren's syndrome (pSS). ΔBAFF, a physiological inhibitor of BAFF, is a minor alternative splice variant of BAFF. A U7 RNA was reengineered to deliver antisense sequences targeting BAFF splice regions. A major decrease of BAFF messenger RNA (mRNA) and protein secretion, concomitantly with the increase of ΔBAFF mRNA, was observed in vitro. In vivo, SG retrograd instillation of nonobese diabetic mice by the modified U7 cloned into an adeno-associated virus vector significantly decreased BAFF protein expression and lymphocytic infiltrates and improved salivary flow. This study offers a rationale for localized therapeutic BAFF inhibition in pSS and represents a proof of concept of the interest of exon skipping in autoimmune diseases.
23606706 Genetic associations to germinal centre formation in primary Sjogren's syndrome. 2014 Jun BACKGROUND: Primary Sjögren's syndrome (pSS) is an autoimmune rheumatic disease mainly characterised by focal mononuclear cell infiltration in the salivary and lacrimal glands, and by the symptoms xerostomia and keratoconjunctivitis sicca. Germinal centre-like structures (GC) are found in the minor salivary glands of approximately 25% of patients. In this study, we aimed to assess genetic variations in pSS patients with GC-like formations (GC+) compared with patients without such formations (GC-). METHODS: Minor salivary gland biopsies from Swedish and Norwegian pSS patients (n=320) were evaluated for GC-like formations, identifying 76 GC+ and 244 GC- patients. A panel of 1536 single-nucleotide polymorphisms (SNPs) in 107 genes was genotyped. Minor allele frequencies in GC+ and GC- patients were compared using Fisher's exact test, and associations were considered significant when p<4.7×10(-4) and suggestive when p<0.01. RESULTS: In this case-only analysis, we identified two SNPs in CCL11 (eotaxin) associated with GC-like structures (p<4.7×10(-4), OR 0.45 and 0.41, respectively). A haplotype of the two minor alleles was associated with GC status with p=2.6×10(-4), OR 0.40. Suggestive associations (p<0.01) were found in SNPs in the B cell activation and/or GC-formation related genes AICDA, BANK1 and BCL2. Furthermore, SNPs in IL17A, ICA1, PKN1 and SNPs in the NF-κB pathway genes CARD8, IKBKE and TANK were found suggestively associated with GC-like structures. CONCLUSIONS: Our findings suggest that genetic variations may explain why ectopic GC-like structures are present in some pSS patients, and support the hypothesis that GC+ and GC- patients represent distinct disease phenotypes.
25277651 Upholding the T cell immune-regulatory function of CD31 inhibits the formation of T/B immu 2015 Jan CD31, a trans-homophilic inhibitory receptor expressed on both T- and B-lymphocytes, drives the mutual detachment of interacting leukocytes. Intriguingly, T cell CD31 molecules relocate to the immunological synapse (IS), where the T and B cells establish a stable interaction. Here, we show that intact CD31 molecules, which are able to drive an inhibitory signal, are concentrated at the periphery of the IS but are excluded from the center of the IS. At this site, were the cells establish the closest contact, the CD31 molecules are cleaved, and most of the extracellular portion of the protein, including the trans-homophilic binding sites, is shed from the cell surface. T cells lacking CD31 trans-homophilic binding sites easily establish stable interactions with B cells; at the opposite, CD31 signaling agonists inhibit T/B IS formation as well as the ensuing helper T cell activation and function. Confocal microscopy and flow cytometry analysis of experimental T/B IS shows that the T cell inhibitory effects of CD31 agonists depend on SHP-2 signaling, which reduces the phosphorylation of ZAP70. The analysis of synovial tissue biopsies from patients affected by rheumatoid arthritis showed that T cell CD31 molecules are excluded from the center of the T/B cell synapses in vivo. Interestingly, the administration of CD31 agonists in vivo significantly attenuated the development of the clinical signs of collagen-induced arthritis in DBA1/J mice. Altogether, our data indicate that the T cell co-inhibitory receptor CD31 prevents the formation of functional T/B immunological synapses and that therapeutic strategies aimed at sustaining CD31 signaling will attenuate the development of autoimmune responses in vivo.
24286296 Efficacy and safety of rituximab treatment in early primary Sjögren's syndrome: a prospec 2013 Oct 30 INTRODUCTION: Primary Sjögren's syndrome (pSS) is an autoimmune disorder affecting exocrine glands; however, a subgroup of pSS patients experience systemic extra-glandular involvement leading to a worsening of disease prognosis. Current therapeutic options are mainly empiric and often translated by other autoimmune diseases. In the last few years growing evidence suggests that B-cell depletion by rituximab (RTX) is effective also in pSS. Patients with early active disease appear to be those who could benefit the most from RTX. The aim of this study was to investigate the efficacy and safety of RTX in comparison to disease modifying anti-rheumatic drugs (DMARDs) in early active pSS patients. METHODS: Forty-one patients with early pSS and active disease (EULAR Sjogren's syndrome disease activity index, ESSDAI ≥ 6) were enrolled in the study. Patients were treated with either RTX or DMARDs in two different Rheumatology centers and followed up for 120 weeks. Clinical assessment was performed by ESSDAI every 12 weeks up to week 120 and by self-reported global disease activity pain, sicca symptoms and fatigue on visual analogic scales, unstimulated saliva flow and Schirmer's I test at week 12, 24, 48, 72, 96, and 120. Laboratory assessment was performed every 12 weeks to week 120. Two labial minor salivary gland (MSG) biopsies were obtained from all patients at the time of inclusion in the study and at week 120. RESULTS: Our study demonstrated that RTX treatment results in a faster and more pronounced decrease of ESSDAI and other clinical parameters compared to DMARDs treatment. No adverse events were reported in the two groups. We also observed that RTX is able to reduce glandular infiltrate, interfere with B/T compartmentalization and consequently with the formation of ectopic lymphoid structures and germinal center-like structures in pSS-MSGs. CONCLUSIONS: To our knowledge, this is the first study performed in a large cohort of early active pSS patients for a period of 120 weeks. We showed that RTX is a safe and effective agent to be employed in pSS patients with systemic, extra-glandular involvement. Furthermore, our data on pSS-MSGs provide additional biological basis to employ RTX in this disease.
23580002 Value of ultrasound in rheumatologic diseases. 2013 Apr The use of musculoskeletal ultrasound in rheumatology clinical practice has rapidly increased over the past decade. Ultrasound has enabled rheumatologists to diagnose, prognosticate and monitor disease outcome. Although international standardization remains a concern still, the use of ultrasound in rheumatology is expected to grow further as costs fall and the opportunity to train in the technique improves. We present a review of value of ultrasound, focusing on major applications of ultrasound in rheumatologic diseases.
25189952 Application of immunosuppressant facilitates the therapy of optic neuritis combined with S 2014 BACKGROUND: Optic neuritis (ON) is often the first symptom of multiple sclerosis (MS) and neuromyelitis optica (NMO) while there has been very little research reported on ON combined with Sjögren's syndrome (SS). The aim of this study is to provide different treatments and services for and NMO patients combined with SS. METHODS: Twenty-seven patients with ON combined SS were divided into two groups: corticosteroid group (C group, methylprednisolone sodium succinate, 14 patients) and corticosteroid+ immunosuppressant group (C+I group, leflunomide, 13 patients). ON relapse times in 1 year after treatment, number of patients who relapsed to NMO/MS in 1 years, visual acuity and retina nerve fiber layer (RNFL) thickness were measured. Mann Whitney-Wilcoxon test was used to compare continuous variables and Chi-square test or Fisher's exact test was to compare proportions. RESULTS: ON combined with SS patients had higher incidence rates in middle-aged women who have binocular damage and heavier visual function damage or when there is an easy relapse, and the patients are often hormone dependent. The patients are more likely anti-aquaporin-4 IgG seropositive (70.4%). They are liable to form a centrocecal scotoma and tubular vision. The times of relapse decreased in patients who used immunosuppressant, and a significant difference was found between immunosuppressant and non-immunosuppressant groups in visual acuity recovery during 6-month follow-up period (P < 0.05); however, the RNFL thickness at the four quadrants was not significantly different. CONCLUSIONS: The effect of immunosuppressant plus corticosteroid on the early onset of ON combined with SS was to provide ON remedy and to prevent recurrence in clinics. This study provides a significant reference for the prevention and treatment of ON on the basis of immunosuppressant and corticosteroid.
23736535 Limited utility of current MRI criteria for distinguishing multiple sclerosis from common 2014 Jan BACKGROUND: Magnetic resonance imaging (MRI) criteria play an important role in making an earlier diagnosis of multiple sclerosis (MS) in patients presenting with clinically isolated syndrome. OBJECTIVE: The objective of this paper is to determine whether MRI criteria may be used to distinguish MS from primary and secondary central nervous system (CNS) vasculitis, lupus, and Sjogren's syndrome. METHODS: MRI criteria were applied retrospectively to images for patients with clinically definite MS (CDMS), primary CNS vasculitis, secondary CNS vasculitis, and autoimmune disorders including systemic lupus erythematosus (SLE) and Sjogren's syndrome. Classical statistics and Bayesian analyses were performed. RESULTS: Overall modified Barkhof's MRI criteria were statistically significant in distinguishing CDMS (60%) from SLE/Sjogren's syndrome (17%, p = 0.0173) but not in distinguishing CDMS from primary CNS vasculitis (50%, p = 0.7376) or secondary CNS vasculitis (58%, p = 1.0000). Four of the five other MRI criteria tested were demonstrated to be superior to modified Barkhof's criteria in predicting MS: nine or more T2 lesions (a component of Barkhof's criteria), one or more ovoid periventricular T2 lesions, one or more perpendicular periventricular T2 lesions, and one or more T2 lesions larger than 6 mm. CONCLUSIONS: MRI criteria, including the modified Barkhof's criteria, were unsuccessful in distinguishing MS from primary CNS vasculitis or secondary CNS vasculitis and mildly successful in distinguishing MS from SLE/Sjogren's syndrome.
23264337 Efficacy of rituximab in systemic manifestations of primary Sjogren's syndrome: results in 2013 Jun OBJECTIVES: To evaluate the efficacy and safety of rituximab in patients with primary Sjögren's syndrome (pSS). METHODS: The AutoImmune and Rituximab registry has included 86 patients with pSS treated with rituximab, prospectivey followed up every 6 months for 5 years. RESULTS: Seventy-eight patients with pSS (11 men, 67 women), who already had at least one follow-up visit, were analysed. Median age was 59.8 years (29-83), median duration of disease was 11.9 years (3-32). Indications for treatment were systemic involvement for 74 patients and only severe glandular involvement in four patients. The median European Sjögren's Syndrome disease activity index (ESSDAI) was 11 (2-31). 17 patients were concomitantly treated with another immunosuppressant agent. Median follow-up was 34.9 months (6-81.4) (226 patient-years). Overall efficacy according to the treating physician was observed in 47 patients (60%) after the first cycle of rituximab. Median ESSDAI decreased from 11 (2-31) to 7.5 (0-26) (p<0.0001). Median dosage of corticosteroid decreased from 17.6 mg/day (3-60) to 10.8 mg/day (p=0.1). Forty-one patients were retreated with rituximab. Four infusion reactions and one delayed serum sickness-like disease resulted in rituximab discontinuation. Three serious infections (1.3/100 patient-years) and two cancer-related deaths occurred. CONCLUSIONS: In common practice, the use of rituximab in pSS is mostly restricted to patients with systemic involvement. This prospective study shows good efficacy and tolerance of rituximab in patients with pSS and systemic involvement.
24905866 The traditional Chinese medicine prescription patterns of Sjögren׳s patients in Taiwan: 2014 Aug 8 ETHNOPHARMACOLOGICAL RELEVANCE: Traditional Chinese medicines (TCM), when given for symptom relief, have gained widespread popularity among Sjögren׳s patients. The aim of this study was to analyze the utilization of TCM among Sjögren׳s patients in Taiwan. MATERIALS AND METHODS: The usage, frequency of service, and the Chinese herbal products prescribed among Sjögren׳s patients were evaluated in a cohort of 1,000,000 beneficiaries recruited from the National Health Insurance Research Database. The logistic regression method was employed to estimate the odds ratios (ORs) for utilization of a TCM. RESULTS: More than 90% of Sjögren׳s patients received TCM out-patient services at least once during the study period. Patients with secondary Sjögren׳s syndrome were more likely to seek TCM treatment than those with primary Sjögren׳s syndrome. The aOR for those suffering from at least one rheumatologic disease was 1.56 (95% CI: 1.26-1.93), those with two rheumatologic diseases was 1.98 (95% CI: 1.29-3.04), while those with three or more rheumatologic diseases was 7.86 (95% CI: 1.09-56.58). Compared to Sjögren׳s patients who used no medical treatment, the aOR for those who took one type of conventional medication was 1.55 (95% CI: 1.25-1.92), those who took two types was 1.98 (95% CI: 1.60-2.47) while those who took three or more types was 2.91 (95% CI: 2.20-3.84). Qi-Ju-Di-Huang-Wan (Lycium Berry, Chrysanthemum, and Rehmannia Pill) was the most frequently prescribed formula among Sjögren׳s patients. CONCLUSION: Qi-Ju-Di-Huang-Wan is the most commonly prescribed Chinese herbal formula for Sjögren׳s syndrome and its effects should be taken into account by healthcare providers.
24864436 [Evaluation of dectin-1 and BAFF expression in conjunctival epithelial cells]. 2014 Apr PURPOSE: To investigate the expression of dectin-1 protein in conjunctival epithelial cells and the expression of dectin-1 and B-cell activating factor belonging to the tumor necrosis factor family (BAFF) mRNA in in vivo conjunctival epithelial cells (CECs) and in vitro cultured CECs, and its difference in topographical change and etiology of disorders. SUBJECTS AND METHODS: 1. Investigation of dectin-1 and BAFF expression by cytodiagnosis of CECs. The subjects were 12 eyes of 12 healthy volunteers (control group), 6 eyes of 6 patients with Sjögren syndrome (Sjögren group) and 10 eyes of 10 patients with vernal keratoconjuctivitis (VKC group). CECs were sampled by impression cytology using nitrocellulose membrane. The expression of dectin-1 in CECs was detected by immunofluorescence and the quantitative determination of dectin-1 mRNA and BAFF mRNA expression was performed by real-time polymerase chain reaction(real-time PCR). 2. Investigation of dectin-1 and BAFF expression using cultured CECs. Cultured CECs which were divided into an OK-432 addition group (addition concentrations: 0.02, 0.1, 0.5KU/mL), a lipopolysaccharide (LPS) addition group (addition concentrations : 80, 160, 320 microg/mL) and an additive-free group were cultured. Quantitative determination of dectin-1 mRNA and BAFF mRNA expression in cultured CECs was performed by real-time PCR. RESULTS: 1. Investigation of dectin-1 and BAFF expression by cytodiagnosis of CECs. In the control group, there was no significant topographical difference in the expression of dectin-1 and the amount of dectin-1 mRNA among superior, inferior tarsal conjunctiva and temporal bulbar conjunctiva. The levels of dectin-1 mRNA expression were 1.5 (0.1-4.0) [median value (range)] for the control group, 2.6 (1.1-4.8) for the Sjögren group and 3.6 (1.7-16.6) for the VKC group. The VKC group showed a significantly higher level of dectin-1 mRNA than the control group (p < 0.01, Kruskal-Walles H-test). The levels of BAFF mRNA expression were 2.8 (0.2-13.8) [median value (range)] for the control group, 6.3 (2.1-15.1) for the Sjögren group and 11.2 (3.5-70.8) for the VKC group. The VKC group showed a significantly higher level of dectin-1 mRNA than the control group (p < 0.01, Kruskal-Walles H-test). Moreover, regarding the relationship between expression level of dectin-1 mRNA and that of BAFF mRNA in all the subjects, there was a significant correlation between them (r = 0.75, p < 0.001, Spearman's rank coefficient). The levels of dectin-1 mRNA expression in the moderate and severe VKC group 9.2 (2.6-16.6) [median value (range)] were significantly higher than those in mild VKC group 2.8 (1.7-3.8) (p < 0.05, Mann-Whitney U-test). The levels of BAFF mRNA expression in the severe and moderate VKC groups 17.4 (9.1-70.8) [median value (range)] were significantly higher than those in the mild VKC group 4.3 (3.5-11.2) (p < 0.05, Mann-Whitney U-test). 2. Investigation of dectin-1 and BAFF expression by cultured CECs. In the OK-432 addition group, the expression levels of dectin-1 mRNA were increased dose-dependently due to the OK-432 stimulation (p < 0.05, Kruskal-Wallis H-test). Moreover, regarding the relationship between the expression level of dectin-1 mRNA and that of BAFF mRNA in all the cultured conjunctival epithelial cells stimulated by OK-432, there was a significant correlation between them (r = 0.85, p < 0.005, Spearman's rank coefficient). CONCLUSIONS: We concluded that dectin-1 expression in CECs was demonstrated, and expression of both dectin-1 and BAFF in CECs is thought to be involved in pathologic aggravation of allergic inflammatory in patients with VKC.
24381188 Severe excessive dynamic airway collapse in a patient with primary Sjögren's syndrome. 2014 Oct Airway and cystic lung diseases can be observed in patients with Sjögren's syndrome. We report a case of such a patient suffering from respiratory failure due to recurrent episodes of right pneumothorax, requiring invasive mechanical ventilation. Despite thoracic drainage and adequate pneumothorax management, the patient could not be weaned from the ventilator. Fiberoptic bronchoscopy revealed severe central excessive dynamic airway collapse of the lower part of the trachea and proximal bronchi. The severity of airway collapse was maximal at the intermediate bronchus level, with a near-complete obstruction during expiration. Inspiratory and expiratory computed tomography studies confirmed the fiberoptic findings and suggested a possible expiratory posterior compression of the intermediate bronchus by parenchymal lung cysts. Stenting was considered, but the patient died from ventilator-associated pneumonia before the procedure could be performed. This case is the first description of severe central excessive dynamic airway collapse in a patient with primary Sjögren's syndrome complicated by diffuse airway and cystic lung disease.
24346882 Congenital multiple clustered dermatofibroma and multiple eruptive dermatofibromas--unusua 2013 Nov Dermatofibroma is one of the most common entities seen in dermatology clinical practice. Several clinical subtypes have nevertheless been described, all of them of uncommon occurrence. The authors present two rare clinical variants of dermatofibromas: congenital multiple clustered dermatofibroma (the presented case is the 4th congenital case to be reported so far) and multiple eruptive dermatofibromas developing in the setting of a Sjögren's syndrome. Since the uncommon subtypes may not be clinically evident, dermatologists should familiarize themselves with their main features and we advise a high level of clinical suspicion in order to reach the correct diagnosis.