Recurrent aseptic meningitis: a new CSF complication of Sjogren’s syndrome

Cetuximab induced aseptic meningitis

Lacrimal Gland in Sjögren's Syndrome

OP0279 A UNIQUE IL-21 SIGNATURE CHARACTERIZES LESIONAL AND CIRCULATING T-FOLLICULAR HELPER CELLS IN SJÖGREN’S SYNDROME PATIENTS WITH ECTOPIC GERMINAL CENTRES AND MALT LYMPHOMA

OP0079 Salivary Gland Epithelial Cells (SGECS) Promote the Differentiation of B Cells

Salivary glands of patients with Sjögren's syndrome (SS) have been shown to be a site of anti-SS-B/La antibody production. The present study investigated differences in the localization of the SS-B/La antigen in labial salivary gland (LSG) tissue between SS and non-SS patients, which may explain the local antigen-driven anti-SS-B/La response. Distribution of SS-B/La was studied immunohistologically in the LSG biopsy samples of 9 SS patients, 10 non-SS patients, and in normal tissues obtained at autopsy within 2 hours after death, using a mouse monoclonal antibody directed to SS-B/La. In 3 SS and 3 non-SS patients, LSGs were also studied with affinity-purified biotinylated human antibodies directed against SS-B/La. In the non-SS patients, SS-B/La was primarily observed in the nucleoli of acinic cells of the LSGs. Patients with either primary SS or secondary SS showed an accumulation of SS-B/La in the nucleoplasm of acinic cells. In the SS patients, SS-B/La was also detected in the cytoplasm as a diffuse or perinuclear staining. Sometimes, SS-B/La was found along the membrane of acinic cells as well. This aberrant nuclear and cytoplasmic distribution of SS-B/La in SS patients correlated well with abnormalities in the composition of the plasma cell population in the LSGs, but not with a lymphocytic focus score > 1. The accumulation and redistribution of SS-B/La in the LSGs may play an important role in the local antigen-driven anti-SS-B/La response in SS, and can also be used to improve the diagnostic possibilities of the LSG biopsy.

AB0196 MESENCHYMAL STEM CELLS TUNE THE DIFFERENTIATION OF MYELOID-DERIVED SUPPRESSOR CELLS IN SJöGREN’S SYNDROME THROUGH INHIBITING IL-12

We report a 29-year-old man with subacute necrotizing lymphadenitis (SNL) associated with recurrent aseptic meningitis following an 11-year remission period. In both episodes, headache and fever were followed by lymphadenopathy, with increased serum IgE level. Although pleocytosis in cerebrospinal fluid was confirmed at admission in the first episode, it appeared at one week after admission in the second episode. Administration of glucocorticoid was effective for treating meningitis. The present case suggests a pathomechanism for SNL that involves both an immunological background and an acute viral infection as triggers of exacerbation of aseptic meningitis.

We read with great interest the article by Erten et al1 entitled “Comparison of plasma vitamin D levels in patients with Sjögren's syndrome and healthy subjects”. Erten et al compared the levels of plasma vitamin D in 107 Sjögren's syndrome (SS) cases and 74 healthy controls. The authors reported that plasma vitamin D levels in patients with SS (20.5 ± 10.5 μg/L) were significantly lower than in the control group (28.4 ± 15.2 μg/L). However, the association between the level of vitamin D and SS remains controversial. Vitamin D deficiency may result in an increased risk of developing some immune-mediated diseases, including SS,2 suggesting a possible role of vitamin D in the pathogenesis of the disease. However, some studies were unable to demonstrate the correlation between low levels of vitamin D and SS. This study aimed to perform a meta-analysis on the association between vitamin D level and SS. We searched PubMed, Embase, Google Scholar and Web of Science using key words for available articles (up to November 2018). The following terms were used: Sjogren's syndrome, SS, 25(OH)D, 1,25(OH)D and vitamin D. We included case-control studies in English that compared vitamin D levels in SS patients and healthy controls. Abstracts, review articles, case reports or conference proceedings were excluded. The strength of the difference of vitamin D between SS and controls was assessed by standard mean differences (SMDs) with 95% confidence intervals (CIs), and median (range) was transformed into mean ± SD.3 The extent of heterogeneity was quantified by I2. If I2 < 50%, it was considered that the variation between studies is small, then a fixed effect model was used; otherwise, a random effect model was used. All the statistical analyses were performed by the RevMan software, version 5.0. All tests were two-sided. A difference of P < 0.05 was considered statistically significant. Of the 159 articles identified from our initial search, nine articles satisfying the inclusion criteria were identified and were included in this meta-analysis.1, 4, 5 Two studies4, 12 included the same individuals13 as control group; according to the Cochrane Reviewers' Handbook, we divided out the control group evenly into two groups to perform the comparison in this meta-analysis. The nine studies were published between 1999 and 2018, and involved 549 SS patients and 1082 healthy controls. Four of the nine studies reported significantly lower vitamin D level in SS patients than in healthy controls, while the other five did not. The I2 value was 93%, then a random effect model was used. Figure 1 showed that the average level of 25(OH)D in SS patients was comparable with that in healthy controls (SMD = −0.43, 95% CI = −0.90 to 0.04, P = 0.07). This result indicated there is no evidence to confirm the different vitamin D levels between SS patients and healthy controls. This meta-analysis has several limitations that need more consideration. First, subgroup analysis and meta-regression were not performed because the number of included studies is relatively small. Second, there were some confounders in this meta-analysis, such as age, ethnicity, season, outdoor activity, diet intake, treatment and so on. However, not all the included studies generated adjusted values, so we could not pool the result by adjusting for confounders. Therefore, further longitudinal, prospective and well-designed cohort studies are needed to elucidate the role of vitamin D in the pathogenesis of SS.

Pituitary abscess is a rare condition. In the setting of multiple surgical interventions, the risk of its development increases. A 49-year-old man presented with episodes of altered mental status. He...

Purpose: To evaluate the ocular surface temperature (OST) using an infrared thermography camera in Sjogren Syndrome (SS) patients compared with healthy subjects and correlate these results with the dry eye symptomatology, tear volume, stability and matrix metalloproteinase 9 (MMP-9) concentrations. Methods: Twelve patients of primary SS (46.64 ± 13.34 years), and twenty volunteers (41.38 ± 9.67 years) without dry eye, participated in this study. OSDI questionnaire, Schirmer test, tear break up time (TFBUT), matrix metalloproteinase 9 (MMP-9) concentrations and OST were evaluated. Results: Central cornea temperature was statistically higher in SS than in control group (p = 0.014), being 34.81 ± 0.37oC and 34.25 ± 0.65oC respectively. In the control group, the temperature increased in the periphery compared with central cornea, limbus and conjunctiva (p 0.05). The SS patients showed a significant lower Schirmer test and TFBUT compared with control group (p<0.005). Also, the OSDI score and MMP-9 concentration were statistically higher in SS patients compared with the control group (p<0.05). No correlation between central cornea temperature and TFBUT, OSDI and Schirmer test was found. However we found a strong positive correlation between central cornea temperature and MMP-9 concentration been 0.628 (p=0.029). Conclusion: Central cornea temperature is higher in SS compared with healthy subjects. The strong positive correlation between MMP-9 concentration and central cornea temperature suggests that the high temperature in SS could be due to ocular surface inflammation

This study aims to investigate ultrasound (US) findings on salivary glands (SG) in patients with Sjögren syndrome (SS) vs. other connective tissue diseases (CTDs) and to assess the relationship of SGUS abnormalities with autoantibody profile in both groups. We enrolled 81 patients, 45 diagnosed with SS (39 with primary SS, 6 with secondary SS) and 36 diagnosed with other CTDs. All patients underwent a prospective evaluation of sicca symptoms, a Schirmer's test, and a B-mode US assessment of the parotid and submandibular glands, all blinded to the diagnosis. Each SG was semi-quantitatively scored 0-3; a grade ≥ 2 was considered pathological. SGUS involvement was classified as normal or pathological at the patient level and for each pair at the gland level. In addition, a total SGUS score of 0-12 and a parotid/submandibular score of 0-6 were calculated for each patient. Autoimmunity laboratory data were also obtained. All SGUS scores were higher in SS patients than in those with CTD (p < 0.001) and significantly more SS patients showed a pathological global (p < 0.001), parotid (p < 0.001), or submandibular (p = 0.001) US score compared with CTD patients. In SS patients, the presence of autoantibodies was significantly associated with pathological SGUS and higher scores, particularly at the parotid level, while in CTD patients, xerostomia and a pathological Schirmer's test were associated with pathological US and higher scores at the submandibular level (p < 0.05). SGUS showed a different grade of abnormality, site involvement, and associated autoantibody profile in SS patients as compared with other CTD. KEY POINTS: • Patients with SS and other CTDs showed different grades of SGUS abnormality. • Patients with SS and other CTDs showed different gland involvement and associated autoantibody profiles. • Anti-Ro60 and anti-Ro52 Ro60 positivity were associated with the severity of parotid involvement in SS patients.

Annular erythema (AE) in Sjögren's syndrome (SS) usually develops on areas of sun-exposed skin and is exacerbated during summer. To evaluate photosensitivity in SS and to investigate the involvement of ultraviolet (UV) radiation in the development of AE in SS. Phototesting with UVA and UVB was performed on 14 SS patients, including 10 with primary SS. Clinical and histological features as well as expression of inducible nitric oxide synthase (iNOS) in the evoked skin lesions were compared with those of lupus erythematosus (LE). Eleven SS patients had a history of photosensitive AE (n = 4), papules (n = 3) or other types (n = 4) of lesions on their sun-exposed skin that were induced or aggravated by sunlight exposure. Phototesting induced a prolonged erythematous response (n = 8), infiltrated erythema (IE) (n = 4) and/or papules (n = 3) in 11 of 14 SS patients, including one with primary SS without a history of photosensitivity. Histologically, the induced IE and papules showed coat-sleeve-like or sparse perivascular infiltration of lymphocytes similar to that in primary skin lesions of AE in SS. No epidermal changes characteristic for LE were found except for partial and mild liquefaction degeneration in three cases. In contrast, two cases were indistinguishable from the papular type of polymorphic light eruption in several aspects, including their primary skin lesions and early response to a photoprovocation test. Immunohistochemistry revealed diffuse expression of iNOS throughout the epidermis, which is characteristic for LE, in the three SS patients with minimal liquefaction degeneration, while the remaining seven SS patients examined exhibited no iNOS staining or a normal expression pattern. Our results indicate that photosensitivity exists in certain primary SS patients, and that UV is critical to the development of AE in SS, probably through a pathological mechanism distinct from that in LE.

SAT0546 COMPARISON OF SHEAR WAVE ELASTOGRAPHY AND CONVENTIONAL ULTRASONOGRAPHY OF SALIVARY GLANDS IN PATIENTS WITH PRIMARY SJOGREN'S SYNDROME: CAN SHEAR WAVE ELASTOGRAPHY CAPTURE LESIONS THAT ARE DIFFICULT TO DIAGNOSE WITH CONVENTIONAL ULTRASONOGRAPHY?

Objective To investigate the expression of miRNA-31 in peripheral blood mononuclear cells (PBMCs) of rheumatoid arthritis (RA) patients, and the relationship between miRNA-31 and disease activity of RA. Methods After obtaining the informed consent, peripheral blood samples of 56 RA patients, 12 systemic lupus erythematosus (SLE) patients, 6 Sjogren's syndrome (SS) patients and 30 healthy controls were collected from the Department of Rheumatology, Peking University Third Hospital. RNA was extracted from the PBMCs which were separated by Ficoll-Paque PLUS. The expression of miRNA-31 in the PBMCs of RA patients, SLE patients, SS patients and healthy controls was detected by real-time Polymerase Chain Reaction (PCR). Furthermore, according to the RA disease activity score (DAS28), RA patients were divided into high, moderate and low disease activity groups and remission group, and miRNA-31 expression was compared between different groups. Data were analyzed using t test or Mann-Whitney U test. Results The expression of miRNA-31 in PBMCs of RA patients was 7.25 times (P=0.003 8) higher when compared with that of the control group. To be specific, the expression of miRNA-31 was 10.63 times in PBMCs of high activity RA group (P=0.01) and 8.95 times in moderate activity RA group (P=0.000 3) when compared with that of the control group, and there was no significant difference between low activity, remission groups and control groups in terms of miRNA-31 expression. Furthermore, the expression of miRNA-31 in PBMCs of SLE patients was not significantly different from the control and miRNA-31 expression in PBMCs of SS patients was 1.64 times (P=0.02) higher than that of the RA patients, but the average level of miRNA-31 was much less than that of RA patients. The increased miRNA-31 may serve as a diagnostic marker for disease activity of RA. Key words: Arthritis, rheumatoid; Monocytes; MicoroRNAs

Background:Systemic sclerosis (SSc) is characterized by progressive fibrosis and microvascular dysfunction that involves multiple organ systems, including kidneys. Kidney involvement, beyond the scleroderma renal crisis (SRC), is often asymptomatic and...