Primary Sjogren Research Articles

Hypokalemic Periodic Paralysis as Initial Manifestation of Primary Sjogrens Syndrome: A Case Report

Hypokalemic Periodic Paralysis as Initial Manifestation of Primary Sjogrens Syndrome: A Case Report

A case report on Sjogren syndrome with autoimmune liver disorder

Background: Primary Sjogren’s syndrome (pSS) is a chronic systemic and autoimmune disorder characterized by inflammation and dysfunction of exocrine glands. Liver involvement was the first extraglandular manifestations to be reported in patients with pSS. Tubulointerstitial nephritis (TIN) is the main renal involvement associated with pSS and manifest as distal renal tubular acidosis (RTA), and others, of which RTA is the main clinical presentation. RTA has been reported in 4.3 to 9% of pSS patients. In this case, patient experiences RTA. Case Presentation: A 19 year old female patient was presented with complaints of abdominal pain, fatigue for 2 months and loose stools. She had history of autoimmune hepatitis and decompensated liver cirrhosis with portal hypertension (autoimmune), bilateral peroneal axonal neuropathy, grade II hepatic encephalopathy resolved and recurrent pustular lesion for past 2 years which rupture spontaneously and heals with scary. She also presented with recurrent hypokalemia which was corrected. No history of similar complaints among the family members. Conclusion: This case explains the autoimmune liver disorder with newly diagnosed primary sjogren’s syndrome associated with recurrent hypokalemia.

Choroidal, retinal, and optic nerve changes in rheumatoid arthritis and primary sjogren's syndrome patients: comparıson with each other and healthy subjects.

The present study aims to evaluate the optic nerve, macula, and choroidal changes in both rheumatoid arthritis (RA) and primary Sjögren's syndrome (SjS) patients, and to compare these findings with age-matched healthy volunteers. This study included 46 RA patients, 33 primary SjS patients, and 37 age-matched healthy volunteers. All of the patients underwent a thorough ophthalmological examination, during which measurements of the retinal nerve fiber layer (RNFL), ganglion cell layer(GCL), and subfoveal choroidal thickness (CT) were taken using OCT (optical coherence tomography). The measurements taken from the right eye of each patient were used to compare among the groups. RNFL thickness in superior quadrant was found to be statistically significantly thinner in the eyes with RA when compared to the control group (p = 0.022). In the nasal quadrant, the RNFL thickness was significantly thinner in patients with primary SjS compared to healthy individuals (p = 0.036). Also, the temporal quadrant RNFL was significantly thinner in RA patients than in the primary SjS patients (p = 0.033). GCL thickness was observed to be thinner in all quadrants of both RA and primary SjS groups compared to the control group. However, the difference was not found to be statistically significant. Subfoveal CT was observed to be thicker in RA and SjS groups compared to the control group, but this difference was also not statistically significant. Systemic autoimmune diseases like RA and primary SjS can lead to a decrease in RNLF and GCL thickness, which can impair visual acuity even in the absence of ocular symptoms. Therefore, monitoring changes in the optic nerve, retina, and choroid layer are crucial in these patients.

Unraveling the transcriptome-based network of tfh cells in primary sjogren syndrome: insights from a systems biology approach.

Primary Sjogren Syndrome (pSS) is an autoimmune disease characterized by immune cell infiltration. While the presence of follicular T helper (Tfh) cells in the glandular microenvironment has been observed, their biological functions and clinical significance remain poorly understood. We enrolled a total of 106 patients with pSS and 46 patients without pSS for this study. Clinical data and labial salivary gland (LSG) biopsies were collected from all participants. Histological staining was performed to assess the distribution of Tfh cells and B cells. Transcriptome analysis using RNA-sequencing (RNA-seq) was conducted on 56 patients with pSS and 26 patients without pSS to uncover the underlying molecular mechanisms of Tfh cells. To categorize patients, we employed the single-sample gene set enrichment analysis (ssGSEA) algorithm, dividing them into low- and high-Tfh groups. We then utilized gene set enrichment analysis (GSEA), weighted gene co-expression network analysis (WGCNA), and deconvolution tools to explore functional and immune infiltration differences between the low- and high-Tfh groups. Patients with pSS had a higher positive rate of the antinuclear antibody (ANA), anti-Ro52, anti-SSA, anti-SSB and hypergammaglobulinaemia and higher levels of serum IgG compared to the non-pSS. Histopathologic analyses revealed the presence of Tfh cells (CD4+CXCR5+ICOS+) in germinal centers (GC) within the labial glands of pSS patients. GSEA, WGCNA, and correlation analysis indicated that the high-Tfh group was associated with an immune response related to virus-mediated IFN response and metabolic processes, primarily characterized by hypoxia, elevated glycolysis, and oxidative phosphorylation levels. In pSS, most immune cell types exhibited significantly higher infiltration levels in the high-Tfh group compared to the low-Tfh group. Additionally, patients in the Tfh-high group demonstrated a higher positive rate of the ANA, rheumatoid factor (RF), and hypergammaglobulinaemia, as well as higher serum IgG levels. Our study suggests that Tfh cells may play a crucial role in the pathogenesis of pSS and could serve as potential therapeutic targets in pSS patients.

The characteristics of chest HRCT and pulmonary function tests in lung-onset primary sjogren's syndrome.

Interstitial lung disease (ILD) can manifest before the diagnosis of primary Sjögren's syndrome (pSS), however, the underlying mechanisms remain unclear. The aim of this study is to investigate the characteristics of lung-onset pSS using chest high-resolution computerized tomography (HRCT) and pulmonary function tests (PFTs). The data of 102 patients with pSS-ILD were retrospectively analyzed. The patients were divided into two groups: lung-onset group and the nonlung-onset group. The chest HRCT, PFTs, and clinical and laboratory data were evaluated and compared. Among the 102 patients with pSS-ILD, 59 (57.8%) were lung-onset and 43 (42.2%) were nonlung-onset. Chest HRCT in the lung-onset group showed higher percentage of usual interstitial pneumonia and nonspecific interstitial pneumonia, the difference did not reach statistical significance. The total HRCT score was higher in the lung-onset group, compared with the nonlung-onset group (2 [2, 3], vs. 2 [1, 2], p = .014). Total lung capacity (TLC) (%pred) [(75.4 ± 16.2) versus (82.8 ± 19.4), p = .049] and forced vital capacity (FVC) (%pred) [(82.2 ± 19.9) versus (91.6 ± 28.3), p = .050] were significantly lower in the lung-onset group, compared with the nonlung-onset group.Residual volume (RV)/TLC (%) significantly increased more than 40% in the lung-onset group (p = .015). Restricted ventilation disorder, small airway obstruction and reduced diffusing capacity of the lung for carbon monoxide/alveolar volume (%Pred) were more common in the lung-onset group (p = .038, p = .050, and p = .050, respectively). Correlation analysis showed that HRCT score was positively correlated with the interval between the onset of pulmonary symptoms and the diagnosis of ILD, serum CA125, and serum CEA. TCL (%pred), VC (%pred), FVC (%pred) were negatively correlated with serum CA125. Lung-onset is common in pSS patients with more severe lung function impairments. Serum biomarkers, such CA125, CEA, and ALB, were associated with the severity of lung damage.

The decreased of peripheral blood natural killer cell is associated with serum IL-2 level in the renal tubular acidosis in patients with primary sjogren’s syndrome

BackgroundPrimary Sjogren’s Syndrome (pSS) is a lymphoproliferative disease with autoimmune characteristics, which is characterized by lymphocyte infiltration of exocrine glands and involvement and dysfunction of extraglandular organs. Renal tubular acidosis (RTA) is a common renal involvement in pSS. This study investigated the phenotypic characteristics of peripheral blood lymphocyte subsets and cytokines in pSS patients complicated with RTA (pSS-RTA).MethodThis retrospective study included 25 pSS patients complicated with RTA and 54 pSS patients without RTA (pSS-no-RTA). To examine the level of peripheral lymphocytes subsets, flow cytometry analysis was used. The level of serum cytokines were detected by flow cytometry bead array(CBA). The influencing factors related to the occurrence of pSS-RTA were identified through logistic regression analyze.ResultsThe absolute number of CD4 + T cells and Th2 cells in peripheral blood were decreased in pSS-RTA patients than pSS-no-RTA patients. Moreover, the absolute number of NK cells and Treg cells were also decreased in pSS-RTA patients than pSS-no-RTA. The level of serum IL-2 was higher in pSS-RTA patients than pSS-no-RTA patients, and is negatively correlated with the number of NK cells, the number and percentage of Th17 cells, and Th17/Treg. Serum IL-2 level is also correlated with various cytokines. Multivariate logistic analysis proved that elevated ESR and ALP were risk factors for pSS complicated with RTA, while Treg was a protective factor.ConclusionThe increase of serum IL-2 level and the decrease of peripheral blood NK cells and Treg cells may be the immune mechanism of the development of pSS-RTA disease.

Efficacy of cyclosporine eyedrop 0.1% in the treatment of dry eye in primary sjogren's syndrome: A prospective Indian study

Objectives: The objective of this study was to evaluate the efficacy and safety of 0.1% cyclosporine A (CsA) eyedrop in patients having primary Sjogren's syndrome (SS) in India. Methods: A prospective consecutive case series of total 20 patients with moderate-to-severe dry eyes were treated with 0.1% CsA eyedrop twice daily along with lubricating eyedrop for 3 months. Efficacy measures included ocular staining score (OSS), van Bijsterveld score (VBS), and Schirmer's score of each eye based on the 2016 ACR-EULAR classification. Subjective improvement was also recorded at the final visit. Any adverse effects were noted. Results: Treatment with 0.1% CsA eyedrop showed a statistically significant improvement at 3 months for both OSS and VBS in each eye of study patients. A similar improvement in Schirmer's score was noted in each eye of the subjects. Subjective improvement of ocular sicca symptoms was also demonstrated. There was excellent compliance to therapy. The 0.1% CsA formulation did not show any topical or systemic side effects in any patient. Conclusion: The 0.1% ophthalmic preparation of CsA is safe and effective in the management of moderate-to-severe dry eyes in patients with primary SS in this pilot study in Indian patients.

LYMPHOMATOUS TURN DURING GOUGEROT SJOGREN SYNDROME MIMICKING BREAST CANCER: A CASE REPORT

Gougerot sjogren syndrome is a mild, slowly progressive autoimmune disease associated with low morbidity and mortality, characterized primarily by organ-specific autoimmune manifestations and systemic manifestations. Indeed, these complications, feared during the evolution, have been known and reported for many years. This risk of non-hodgkin lymphoma is, moreover, notably greater during primary Gougerot sjogren syndrome than during any other autoimmune pathology. The most commonly affected tissue is the exocrine salivary glands, but other tissues may be involved: stomach, nasopharynx, skin, liver, kidney, lung, breast. In both models, primary Sjögrens syndrome non-Hodgkins lymphoma or chronic viral hepatitis infection, autoreactive B lymphocytes with rheumatoid factor activity continuously stimulated by the autoimmune process are particularly exposed to clonal transformation by a second oncogenic event.

Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary sjogren syndrome: results of an epidemiologic systematic review/meta-analysis and a consensus guideline from the Brazilian Society of Rheumatology (articular, pulmonary and renal)

Sjogren's Syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, these systemic manifestations are not routinely evaluated and there is no homogenous approach to their diagnosis or evaluation. To close this gap, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of epidemiologic and clinical features of these manifestations and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. The first part of this guideline summarizes the most important topics, and 11 recommendations are provided for the articular, pulmonary, and renal care of SS patients.

English

BACKGROUND Dry eye diseases (DED) tend to be more seen in patients with auto immune disease. This study was an attempt to assess the prevalence of DED among the patients who were diagnosed and confirmed cases of Rheumatoid arthritis (RA), SLE and Primary Sjogren’s syndrome (Primary SS). Objective of the study was to assess the prevalence of Dry eye disease among the study participants and determine its severity in connective tissue disorders. METHODS This was a cross sectional study conducted at a tertiary care hospital in the South Kerala. The study period was six months. 30 study participants were enrolled for the study who fulfilled the selection criteria. The study participants were evaluated for DED by Schirmer’s test, Tear break up time and ocular surface staining. Data collected was entered in Microsoft Excel and descriptive data was expressed as frequencies and percentage. RESULTS The overall prevalence of the Dry eye disease was found to be 54%. The prevalence was significantly higher in patients with Primary SS which accounted for 70% followed by patients with RA; which accounted for 52. Dry eye disease was seen only in 40 % of the study participants diagnosed with SLE. Similarly, the severity of Dry eye disease was found higher in patients with Primary SS; where about 35 % had severe to very severe disease. 22 % of the study participants with RA had severe to very severe dry eye disease. There was no severe or very severe dry eye disease in the study participants who had SLE. CONCLUSIONS The overall prevalence of Dry eye disease in patients with autoimmune disease was found to be significantly higher especially in patients who were diagnosed to have Primary SS and RA. There is a need for early diagnosis of dry eye disease in such patients which can improve the cure rate, reduce the complications of the disease and reduce the visual morbidity. KEY WORDS Dry Eye Disease, Primary sjogrens Syndrome, Rheumatoid Arthritis, SLE

Dry eye disease associated with Primary Sjogren syndrome: An update

Primary Sjögren’s syndrome (pSS) is a chronic, multisystem autoimmune disorder, characterized by mononuclear infiltration of exocrine glands and other organs, resulting in dry eye, dry mouth and extra-glandular systemic findings. Primary Sjögren syndrome is of particular interest to ophthalmologists as it constitutes an important differential diagnosis in conditions with dry eye disease. The ocular tests are of great importance for diagnosis and monitoring of primary sjogren’s syndrome. Also a better understanding of immunological mechanisms and molecular pathways have resulted in discovery of new therapeutics for local and systemic treatment. This article illustrates an update regarding pathogenesis, diagnosis, investigative procedures and treatment options for dry eye related to Sjogren’s syndrome.

Prevalence of Dry Eye Disease in Connective Tissue Disorders

Aim: To assess prevalence of dry eye disease (DED) and its severity in patients of Systemic lupus erythematosus (SLE) and Primary Sjogren’s syndrome in a tertiary care hospital in Gujarat.
 Methods: It is a Prospective cross-sectional study of 94 patients diagnosed with SLE and Sjogren’s syndrome. All patients underwent: 1. History taking 1. Visual acuity assessment using standard illuminated Snellen chart 2. Slit lamp examination with dry eye tests 3. Fundus examination The findings were recorded in the proforma and subsequent analysis was done using SSPS software.
 Results: DED prevalence was 51.1% overall, 42% among SLE and 66.66%in Primary Sjogren’s syndrome. Severity of DED was noted to be more in Primary sjogren’swith 33.33% having severe DED.
 Conclusion: DED overall prevalence in connective tissue disorders is significant, highest being in Sjogren’s. The findings highlight the severity of DED in rheumatoid arthritisgreater to when compared with other connective tissue disorders. Awareness and earlydetection of dry eye disease is of paramount to initiate appropriate treatment and reducevisual morbidity.

Hypokalemic periodic paralysis as initial manifestation of primary Sjogren’s syndrome: A case report

Sjogren’s syndrome (SS) is common autoimmune diseases manifesting with various glandular and extra glandular features which are difficult to evaluate and manage. Hypokalemic flaccid paralysis may be the rare extra glandular clinical manifestation of SS. We present a case of acute flaccid paralysis which on evaluation turned out to be primary sjogren’s syndrome (pSS). We present a case of 30 year old young woman with no known comorbidities presented with acute flaccid paralysis. Her evaluation reveals severe hypokalemia with K-1.6 meq/l, metabolic acidosis with pH-7.28 and urinary pH ≥6.5. An initial diagnosis of hypokalemic periodic paralysis (HPP) secondary to distal renal tubular acidosis (dRTA) was made. She was later found to have sjogren’s syndrome on the basis of positive ANA & Anti-Ro (SS-A) Antibody and salivary gland biopsy. She was managed with potassium supplements, sodium bicarbonate and immunomodulators. Primary Sjogren’s syndrome usually associated with distal renal tubular acidosis which may cause hypokalemia leading to HPP, rarely which may be the initial presenting symptom in young women as in our case. Early diagnosis and treatment of these patients would lead to less complication and better quality of life.

IMMUNE-MEDIATED INTRAOCULAR INFLAMMATION. AREVIEW.

Immune mediated inflammatory diseases are categorized into autoimmune and autoinflammatory. Autoimmune etiology is represented by autoreactive lymphocytes or autoantibodies, e.g. primary Sjögrenssyndrome or rheumatoid arthritis. Ocular specific diseases with presumed autoimmune origin are sympathetic ophthalmia or birdshot chorioretinopathy. Autoinflammatory diseases are caused by mutations in regulatory genes for specific immunity. Hereditary periodic fevers represent monogenic autoinflammatory diseases; eye specific is Blau syndrome also named sarcoidosis with early onset. This article reviews the actual knowledge about immune mediated uveitides, their immunological mechanisms and the possible trigger role of infection in autoimmune inflammation. Immune privilege provides aprotection of the eye against any strong immune reaction to foreign antigen, based on physical, immune, humoral and molecular mechanisms. Antigens hidden within the eye are revealed in case of damage of hematoretinal barrier caused by infection or mechanical insult. These ocular antigens have not been set as tolerable during the development and immune reaction is initiated subsequently. Current studies demonstrate that uveogenic trigger might be generated by own microbiome, particularly when dysregulated, so called dysbiosis. There is aknown association between idiopathic inflammatory bowel disease with ankylosing spondylitis and anterior uveitis in humans. Intensive research is focused on microbiome and immune mediated inflammatory disease to influence therapeutically the intestinal microbiome. The animal models are used to study the immunopathological mechanisms of uveitis and the new therapeutic strategies, because of relatively low incidence of immune mediated uveitis in humans.

The relationship between Sjogren's syndrome, systemic sclerosis and lymphoproliferative diseases

To study demographic, clinical, laboratory and immunological characteristics of patients with a combination of primary Sjogrens syndrome (pSS) and SSc and diagnosed lymphoproliferative diseases (LPDs); to characterize morphological/immunomorphological variants and course of non-Hodgkins lymphomas (NHL), developing in patients with these rheumatic diseases (RDs). In 19982018 at the Nasonova Research Institute of Rheumatology, 13 patients with clinical and laboratory manifestations of pSS (12) and SSc (13) were diagnosed with various lymphoproliferative diseases (LPDs). In 3 cases, an induced RD was observed: 1 case of a diffuse, rapidly progressive form of SSc, 2 cases of pSS in combination with a limited form of SSc after chemotherapy and radiation therapy of Hodgkins lymphoma (1), B-cell NHL (1) and CR of the breast (1) respectively. The first 2 cases were excluded from the analysis, since the development of lymphomas is not pathogenetically associated with RD. Of 11 patients with LPDs, 10 after a long course of RDs were diagnosed with NHL [MALT lymphoma of the parotid salivary glands 7, disseminated MALT lymphoma 2, disseminated MALT lymphoma with transformation into diffuse large B-cell lymphoma (DLBCL) 1]. RDs debuted with Raynauds phenomenon (RP) in 64.5% and pSS manifestations in 45.5% of patients. Stomatological manifestations of pSS were characterized by recurrent parotitis in 36%, significant parotid gland enlargement with massive infiltration of labial salivary glands (focus score 4) in 100%, severe xerostomia in 70%, extraglandular manifestations and lymphadenopathy in 50% of patients. The course of the SSc was characterized by mild RP with various types of capillaroscopic changes and mild lung changes and non-significant progression during long-term follow-up (median 22 years). The entire spectrum of SSс specific antibodies (anticentromere antibodies 60%, antibodies to ribonucleoprotease III 30%, Pm/Scl 10%), excepting antibodies to topoisomerase I, as well as pSS specific autoantibodies (antiRo/La 70%, RF (rheumatoid factor) 90%), were detected in patients with a combination of these RDs. pSS is often combined with a limited form of SSc regardless of the type of autoantibodies detected. The presence of pSS, rather than SSc, is a high-risk factor for the development of NHL in this group of patients. The patients with pSS and SSc are characterized by a steady progression of pSS with a slow and mild course of SSc throughout the observation period. The development of severe stomatological manifestations and high immunological activity of pSS contribute to the development of localized MALT lymphomas (70%) and disseminated MALT lymphomas (30%) with primary lesions of the salivary glands and transformation into DLBCL in case of their late diagnosis. The optimal method for preventing the development of NHL in this group of patients is the early diagnosis of pSS, the appointment of alkylating cytotoxic agents and/or anti-B-cell therapy in the early stages of pSS. Given the possibility of transformation of localized NHL into DLBCL, for early diagnosis, minimally invasive surgical biopsies of significantly enlarged parotid salivary glands should be performed before glucocorticoids are prescribed. Detection of positive B-cell clonality and lymphoepithelial lesions in the parotid salivary gland is considered a predictor of MALT lymphoma development during follow-up. Localized and disseminated MALT lymphomas in patients with pSS and SSc respond well to therapy, in contrast to MALT lymphomas transformed into DLBCL.

SIALOGRAPHY FOR SJOGRENS SYNDROME: AN IMPORTANT DIAGNOSTIC TOOL

Sjogrens syndrome (SS) is a chronic autoimmune disorder that affects the exocrine glands. Primary Sjogrens syndrome is associated with dry mouth dry eyes, and is frequently found in middle middle-aged females in their fourth or fifth decade. This article presents an atypical case of Sjogrens syndrome in a 21-year-old woman who complained of bilateral parotid swelling and diagnosis was made with the help of parotid sialography. Though the confirmatory diagnosis was achieved with the help of serological and histopathological examination, an oral medicine specialist can play an important role in the detection of this disease.

PRIMARY SJOGREN SYNDROME CAUSING PULMONARY EXTRANODAL MARGINAL CELL LYMPHOMA

SESSION TITLE: Medical Student/Resident Lung Pathology SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Extranodal marginal zone lymphoma (MZL) of the lung is a rare pulmonary lymphoid malignancy and World Health Organization (WHO) classifies Mucosa associated lymphoid tissue (MALT) lymphoma as a subtype of B-cell MZL. CASE PRESENTATION: 80 year old Caucasian male with past medical history of obstructive sleep apnea, chronic kidney disease stage III, type II diabetes mellitus, hypertension and tobacco abuse, was first seen in pulmonary clinic for asymptomatic innumerate bilateral pulmonary nodules noted on Computed Tomography (CT) scan of the chest. He complained of occasional chronic dry mouth on further questioning. Antibody testing was positive for Antinuclear antibodies (245 AU/ml) and SS Ro antibody (188 AU/ml). His dry mouth and lower lobe predominant cystic and nodular changes were concerning for lymphoid interstitial pneumonia associated with Sjogren's syndrome. He was referred to rheumatology then subsequently lost to follow up. He presented two years later with increased shortness of breath and progressive weight loss. Pulmonary function tests (PFTs) showed an isolated low diffusing capacity for carbon monoxide at 33% predicted (down from 75% prior). CT scan revealed progression of his bilateral lung nodules and cystic changes in the lower lungs bilaterally. He then underwent bronchoscopy and surgical lung biopsy. This showed a low-grade B- cell lymphoma with plasmacytic differentiation, most consistent with MALT stage 1E Extranodal marginal zone lymphoma. Patient was started on rituximab weekly for 4 weeks and reported some improvement in his sicca symptoms. DISCUSSION: Roughly 10 to 20 percent of SS patients have clinically significant lung disease defined by symptoms most commonly cough and dyspnea. Abnormal imaging and PFTs are common. Thin-walled lung cysts are detected with CT imaging in 12 to 46 percent of SS patients. Interstitial lung disease can occur in SS in several forms (1). In a series of thirteen patients with pulmonary MALT lymphoma performed by Yachoui et. al, 54% were positive for primary sjogren syndrome (PSS) (2). No difference in outcomes was noted on two large retrospectives studies involving different treatment modalities including surgery, chemotherapy or combined therapy. Asymptomatic patients should be observed with no treatment indicated initially. It is indicated for patients with a high burden of lymphadenopathy with splenomegaly, organ dysfunction, bone marrow suppression, and/or rapid disease progression (3). Series performed by Yachoui et. al demonstrated a favorable prognosis in all cases of pulmonary MALT lymphoma with all treatment modalities resulting in complete and sustained response (2). CONCLUSIONS: SS can have wide ranging organ involvement. Prompt referral to rheumatology and active monitoring is paramount. Further investigation is warranted to better understand pulmonary MALT pathogenesis, treatment, and outcomes in PSS. Reference #1: Stojan, G., et al. (2013). “Pulmonary manifestations of Sjögren's syndrome.” Current allergy and asthma reports 13(4): 354-360. Reference #2: Yachoui R, Leon C, Sitwala K, Kreidy M. Pulmonary MALT lymphoma in patients with Sjögren's syndrome. Clinical medicine & research. 2017;15(1-2):6-12. Reference #3: Pinotti G, Zucca E, Roggero E, Pascarella A, Bertoni F, Savio A, et al. Clinical features, treatment and outcome in a series of 93 patients with low-grade gastric MALT lymphoma. Leukemia & lymphoma. 1997;26(5-6):527-37

MiR-744-5p contributes to ocular inflammation in patients with primary Sjogrens Syndrome

In primary Sjögren’s syndrome (pSS) the exocrine glands become infiltrated with lymphocytes instigating severe damage to the salivary and lacrimal glands causing dry eyes and dry mouth. Previous investigations have suggested that dysregulated localized and systemic inflammation contributes to the development and pathogenesis of pSS. A miR microarray performed in primary human conjunctival epithelial cells (PECs) demonstrated significant differences in miR expression at the ocular surface between pSS patients and healthy controls. MicroRNA-744-5p (miR-744-5p) was identified as being of particular interest, as its top predicted target is Pellino3 (PELI3), a known negative regulator of inflammation. Validation studies confirmed that miR-744-5p expression is significantly increased in PECs from pSS patients, whilst PELI3 was significantly reduced. We validated the miR-744 binding site in the 3’ untranslated region (UTR) of PELI3 and demonstrated that increasing PELI3 levels with a miR-744-5p antagomir in an inflammatory environment resulted in reduced levels of IFN dependent chemokines Rantes (CCL5) and CXCL10. These results reveal a novel role for miR-744-5p in mediating ocular inflammation via Pellino3 expression in pSS patients and suggest that miR-744-5p may be a potential therapeutic target for the management of severe dry eye disease and ocular inflammation in pSS patients.

The clinical significance of lnc-DC in plasma for the diagnosis of primary Sjögren′s syndrome

Objective To explore the long non-coding RNA that ineract within dentridic cell (lnc-DC) in plasma as a novel bio-marker for primary Sjogrens syndrome (pSS). Methods Case-control study. One hundred and thirty-nine patients of pSS,50 patients of systemic lupus erythematosus (SLE) and 50 patients of rheumatoid arthritis (RA) were collected from Xuzhou Central Hospital from January 2017 to January 2018, and 78 healthy individuals were designed as healthy control. The plasma lnc-DC of pSS, SLE and RA patients and healthy control (HC) was determined by real time polymerase chain reaction (RT-PCR). The plasma levels of anti-SSA(anti-Sjogren′s syndrome type A) and anti-SSB(anti-Sjogren′s syndrome type B) were tested by ELISA. The correlation between lnc-DC and dry mouth, dry eyes, ESR,β2-microglobulin, anti-SSA and anti-SSB of pSS patients were analyzed by Spearman′s rank correlation test. Receiver operating characteristic (ROC) curves were used to identify the diagnostic performance of plasma lnc-DC for pSS. Results Compared to healthy control (3.38±0.44), the lnc-DC relative expression (6.82±0.51) was significantly increased in pSS patients (t=4.78, P<0.01) .The lnc-DC had a strong positive correlation with dry mouth (r=0.81, P<0.01) , dry eyes (r=0.75, P<0.01) , ESR (r=0.73, P<0.01) , β2-microglobulin (r=0.63, P<0.01) , anti-SSA (r=0.45, P<0.01) and anti-SSB (r=0.53, P<0.01) .The area under curve (AUC) of plasma of lnc-DC was 0.81, (95% CI: 0.71-0.90). When combination lnc-DC with anti-SSA and anti-SSB, the AUC is 0.86 (95%CI:0.78-0.94). At the optimal cut-off of 5.1, the diagnostic sensitivity and specificity were 0.73(95%CI: 0.60-0.84) and 0.82 (0.66-0.92). And in this point may provided better diagnostic accuracy. Meanwhile, compared with SLE and RA patients, the plasma level of lnc-DC were higher, ROC curves of lnc-DC in pSS patients can distinguish pSS from other autoimmune disease such as SLE and RA. Conclusions Plasma lnc-DC can provide additional diagnostic information beyond other clinican characteristics such as dry mouth,dry eyes,ESR,β2-microglobulin,anti-SSA and anti-SSB. It can be used as a novel bio-marker for pSS patients, and also have significant values in diagnosis of pSS. Key words: Sjogren′s syndrome; RNA, long noncoding; Biomarkers; Sensitivity and specificity

A study of clinicopathological profile and treatment outcomes in patients with autoimmune hemolytic anaemia

Background: Autoimmune haemolytic anaemia (AIHA) is relatively uncommon condition with grave consequences, if not diagnosed and treated early. The literature on the clinical outcome and response to treatment is relatively scarce. Aim was to study the clinic-pathological profile and the treatment outcomes in patients with AIHA.Methods: Around 25 patients with AIHA attending a tertiary care hospital over a period of one year were included in the study. The patients were divided based on severity of anaemia and etiology. All the patients data were analysed for the demographic data, clinico -pathological findings and the response to treatment. All the patients data were analysed using SPSS software (version 22).Results: Out of 25 patients, 76% were females and 24% were males. Based on severity of anaemia, 60%, 28% and 8% had severe, moderate and mild anaemia. Around 48% of the patients had thrombocytopenia along with anaemia. 8 (32%) and 17 (68%) patients have primary and secondary AIHAs respectively. In our study the commonest cause for the secondary AIHA was Systemic Lupus Erythematosus (SLE) followed by haematological malignancy, primary Sjogrens, Anti-phospholipid antibody (APLA) syndrome, carcinoma colon and Wilsons disease. Hepatosplenomegaly and lymphadenopathy were present in 36% and 4% respectively. Out of 22 (88%) patients on corticosteroid therapy, 15 (60%) patients responded to corticosteroids alone and 6(24%) patients required corticosteroid plus immunosuppressive therapy.Conclusions: AIHA has to be ruled out in all anaemia patients with indirect hyperbilirubinemia and abnormal peripheral smear. Secondary AIHA is more common than primary. Corticosteroids and immunosuppressive agents are the mainstay of treatment of AIHA.