Lysine acetyltransferase 6A maintains CD4+ T cell response via epigenetic reprogramming of glucose metabolism in autoimmunity.

Augmented CD4+ T cell response in autoimmunity is characterized by extensive metabolic reprogramming. However, the epigenetic molecule that drives the metabolic adaptation of CD4+ T cells remains largely unknown. Here, we show that lysine acetyltransferase 6A (KAT6A), an epigenetic modulator that is clinically associated with autoimmunity, orchestrates the metabolic reprogramming of glucose in CD4+ T cells. KAT6A is required for the proliferation and differentiation of proinflammatory CD4+ T cell subsets in vitro, and mice with KAT6A-deficient CD4+ T cells are less susceptible to experimental autoimmune encephalomyelitis and colitis. Mechanistically, KAT6A orchestrates the abundance of histone acetylation at the chromatin where several glycolytic genes are located, thus affecting glucose metabolic reprogramming and subsequent CD4+ T cell responses. Treatment with KAT6A small-molecule inhibitors in mouse models shows high therapeutic value for targeting KAT6A in autoimmunity. Our study provides novel insights into the epigenetic programming of immunometabolism and suggests potential therapeutic targets for patients with autoimmunity.

HECT, UBA and WWE domain containing 1 represses cholesterol efflux during CD4+ T cell activation in Sjögren's syndrome.

Introduction: Sjögren's syndrome (SS) is a chronic autoimmune disorder characterized by exocrine gland dysfunction, leading to loss of salivary function. Histological analysis of salivary glands from SS patients reveals a high infiltration of immune cells, particularly activated CD4+ T cells. Thus, interventions targeting abnormal activation of CD4+ T cells may provide promising therapeutic strategies for SS. Here, we demonstrate that Hect, uba, and wwe domain containing 1 (HUWE1), a member of the eukaryotic Hect E3 ubiquitin ligase family, plays a critical role in CD4+ T-cell activation and SS pathophysiology. Methods: In the context of HUWE1 inhibition, we investigated the impact of the HUWE1 inhibitor BI8626 and sh-Huwe1 on CD4+ T cells in mice, focusing on the assessment of activation levels, proliferation capacity, and cholesterol abundance. Furthermore, we examined the therapeutic potential of BI8626 in NOD/ShiLtj mice and evaluated its efficacy as a treatment strategy. Results: Inhibition of HUWE1 reduces ABCA1 ubiquitination and promotes cholesterol efflux, decreasing intracellular cholesterol and reducing the expression of phosphorylated ZAP-70, CD25, and other activation markers, culminating in the suppressed proliferation of CD4+ T cells. Moreover, pharmacological inhibition of HUWE1 significantly reduces CD4+ T-cell infiltration in the submandibular glands and improves salivary flow rate in NOD/ShiLtj mice. Conclusion: These findings suggest that HUWE1 may regulate CD4+ T-cell activation and SS development by modulating ABCA1-mediated cholesterol efflux and presents a promising target for SS treatment.

Metagenomic next-generation sequencing for the diagnosis of oral and maxillofacial space infections.

Background/purpose: Metagenomic next-generation sequencing (mNGS) has been widely used for the detection of pathogens causing infectious diseases. This study aimed to evaluate the potential ability of mNGS to detect pathogens causing oral and maxillofacial space infection (OMSI) and compare the results with those of the traditional diagnostic microbial culture method. Materials and methods: We retrospectively reviewed the data of 218 patients diagnosed with OMSI who underwent microbial culture and mNGS at the Department of Oral Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, from July 2020 to January 2022. Results: The positivity rate of mNGS (216 cases) was significantly higher than that of microbial culture (123 cases). The most frequently detected bacteria were different between these two detection methods. Streptococcus constellatus (16.05%, 35), Streptococcus anginosus (15.69%, 34) and Klebsiella pneumoniae (6.88%, 15) were the most commonly isolated bacteria by culture. However, Peptostreptococcus stomatis (61.47%, 134), Parvimonas micra (68.35%, 149) and Streptococcus constellatus (57.34%, 125) were the most commonly detected bacteria by mNGS. mNGS also has advantages in diagnosing viral infections. The optimal numbers of diagnostic reads were 1162 and 588 for the diagnosis of Streptococcus anginosus and Streptococcus constellatus infections, respectively. Read numbers were significantly correlated with C-reactive protein (CRP), procalcitonin (PCT), and blood glucose levels and neutrophil percentage (NEUT%). Conclusion: For pathogens causing OMSI, mNGS had a higher rate of microbial pathogen detection and remarkable advantages in identifying coinfections involving viruses and fungi. The read numbers for mNGS are important for diagnostic accuracy and disease severity evaluation.

Sialendoscopy-assisted combined approach for parotid sialolithotomy: Our long-term experience.

OBJECTIVES: To assess the long-term outcome of sialendoscopy-assisted combined approach for parotid sialolithotomy with gland preservation. PATIENTS AND METHODS: A retrospective study of patients treated with a combined sialendoscopic and open approach was conducted between 2011 and 2020. Demographic data of patients such as operative technique, stone size, stone location, complications, and symptom relief were collected. Patients were followed up via clinical examination and questionnaires. RESULTS: Seventy-four patients were included and underwent endoscopy-assisted combined operations for the removal of 98 parotid stones. Of the 98 stones, 92(94%) stones were completely removed and 6(6%) were partially removed. At a mean follow-up of 47.1 ± 35 months, 65 of 74 patients (88%) achieved long-term success. Patients with stone incomplete removal were significantly more often to develop the recurrence of obstructive symptoms (p = 0.000) There were no cases of facial nerve injury or fistula formation. Gland function was preserved in 73 of 74 patients (99%). CONCLUSIONS: The combined approach for parotid stones is a safe and gland-preserving alternative to parotidectomy. The techniques described here show high success rates and good long-term results, and they avoided the need for gland resection in >95% of cases.

Pharmacological Inhibition of Glutaminase 1 Normalized the Metabolic State and CD4+ T Cell Response in Sjogren's Syndrome.

Previous studies have shown that abnormal metabolic reprogramming in CD4+ T cells could explain the occurrence of several autoimmune disorders, including Sjogren's syndrome (SS). However, therapeutic targets of the abnormal metabolism of CD4+ T cells remain to be explored. Here, we report that glutaminase 1 (Gls1), a pivotal factor in glutaminolysis, might be involved in the pathogenesis of SS. The expression of Gls1 was upregulated in infiltrated labial CD4+ T cells and circulating CD4+ T cells of SS patients. Inhibiting Gls1 with BPTES significantly abolished the proliferation rate, as indicated by EdU, CFSE, and Western blot analyses. Additionally, BPTES downregulated the extracellular acidification rate (ECAR) and oxygen consumption rate (OCR) values of activated CD4+ T cells from SS mice. In vivo, we injected different doses of BPTES into SS-like NOD/Ltj mice and found that 10 mg/kg BPTES significantly restored the salivary flow rate. Histological and qRT-PCR analyses showed that this concentration of BPTES attenuated lymphocytic infiltration and the numbers of PCNA-positive cells and CD4+ T cells. The proportions of IFNγ-producing cells and IL-17A-producing cells and the expression of several proinflammatory cytokines, including IFNγ and IL-17A, were also affected in the salivary glands of SS-like mice. Cytokine production in circulating serum was analyzed and showed that BPTES downregulated the effector functions of Th17 cells and Th1 cells. Collectively, these results indicate a positive relationship between Gls1 and SS development. Pharmacological inhibition of Gls1 with BPTES could normalize the effector functions of CD4+ T cells and effectively attenuate the symptoms of SS.

Characterization of comprehensive dynamic epigenetic changes during human primary Sjögren's syndrome progression.

BACKGROUND: Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterized by reduced exocrine gland (principally the salivary and lacrimal glands) activity caused by chronic lymphocytic infiltration. Although pSS has been closely associated with an increased risk of mucosa-associated lymphoid tissue (MALT) lymphoma, the dynamic epigenetic changes in the gland cells that accompany the pathogenesis are not entirely understood. METHODS: In this study, we harvested tissue samples from the labial gland with (LG_pSS) or without pSS (LG_NC) before MALT development, as well as the parotid gland with tumor tissues (PG_MALT) and paracancerous tissues (PG_NC) of two pSS patients with MALT lymphoma, and conducted RNA-seq and ChIP-seq for tri-methylated histone 3 lysine 4, 9, 27, 36, and 79 (H3K4/9/27/36/79me3). RESULTS: Transcriptome landscapes indicated two outcomes of pSS progression with or without MALT lymphoma represented by distinct populations of differentially expressed genes and their functions. Furthermore, the epigenetic atlas of genome-wide H3K4/9/27/36/79me3 was in different stages for various samples, indicating that the variance of H3K4me3 was the earliest event, followed by selective alterations of H3K9/27/36/79me3. These four epigenetic modifications determine the final outcome of pSS progression. CONCLUSIONS: Our results not only advance the understanding of the dynamics of pSS progression and highlight the importance of epigenetic alterations in regulating transcription during this pathological process, but also identify potential therapeutic targets for pSS treatment and lymphoma intervention.

BST-2/Tetherin is involved in BAFF-enhanced proliferation and survival via canonical NF-κB signaling in neoplastic B-lymphoid cells.

The development of Sjögren's syndrome (SS) is accompanied by B cell hyperproliferation and mutation. Our previous study identified aberrant expression of BST-2 (also known as Tetherin/CD317) in B cells from either the peripheral blood or infiltrated salivary glands. However, the roles of BST-2 in the regulation of B cell activation remain unknown. In this study, we identified that BST-2 can respond to BAFF simulation but not to other B cell simulators in neoplastic B cell lines. A CCK-8 assay, an EdU assay and Annexin V/PI staining indicated that BST-2 inhibition attenuated BAFF-enhanced proliferation and survival in both Raji cells and Daudi cells. Screening of BAFF-related signaling in neoplastic B-lymphoid cells indicated that BST-2 was involved in the regulation of NF-κB signaling upon BAFF simulation. However, inhibition of NF-κB by JSH-23 significantly reduced the proliferation and survival of Raji and Daudi cells under both normal and BAFF-simulated conditions. Collectively, our results indicate that BST-2/Tetherin is a BAFF-responsive membrane factor involved in the regulation of NF-κB signaling, thereby assisting in the proliferation and survival of neoplastic B-lymphoid cells. Our study provides a potential molecular mechanism underlying aberrant overactivation of B cells upon SS development.

Prediction of 3-month treatment outcome of IgG4-DS based on BP artificial neural network.

OBJECTIVE: The study aimed to establish an effective back-Propagation artificial neural network (BP-ANN) model for automatic prediction of 3-month treatment outcome of IgG4-DS. METHODS: A total of 26 IgG4-DS patients at Shanghai Ninth People's Hospital from January 2018 to December 2019 were involved in the study. They were all followed for >3 months. The primary outcome was reduction of serum IgG4 (sIgG4) after 3-month treatment. The association between risk factors and reduction of sIgG4 was analyzed by Spearman's rank correlation test. According to the R values, we built a BP-ANN model by MATLAB R2019b. RESULTS: The average reduction of sIgG4 was 5.55 ± 5.03. After Spearman's rank correlation test, ESR, sIgG4, and sIgG were independently associated with reduction of sIgG4 (p < .05) and were selected as input variables. Take into account these parameters, BP-ANN model was developed and the coefficient of determination (R2 ) model was 0.95512. CONCLUSION: The BP-ANN model based on ESR, sIgG4, and sIgG could predict the 3-month reduction of sIgG4 for IgG4-DS patients. It showed potential clinical application value.

Juvenile recurrent parotitis: Soft foods contribute to the delayed development of salivary glands.

BACKGROUND: Juvenile recurrent parotitis (JRP) is the second-most common childhood disease of the salivary glands after mumps. Since popularisation of mumps vaccination, children suffered from JRP more often, and the aetiology remains unclear. Chinese children had the habit of soft foods due to the special dietary habit of Asia. OBJECTIVES: To clarify whether mastication was related to the pathogenesis of JRP and whether the growth of salivary glands was influenced by soft diet. METHODS: Investigation of dietary habit and masticatory efficiency from 2015 to 2018 of children diagnosed with JRP compared with the normal children by the dentition. Mice had been fed a soft diet beginning in their development phase. The gland weight, amount of saliva, salivary amylase, histological and ultrastructural observation and the expression levels of EGF, FGFr2 and Wnt3a had been tested. RESULTS: The JRP children preferred soft foods and had a significantly lower masticatory efficiency than do normal children. When normalised by body weight, the gland weight, amount of saliva and amount of salivary amylase in the experimental group were significantly lower. The ultrastructural results showed that the acinar cells in the experimental groups were smaller and contained fewer electron-dense secretory granules than those in the control groups. The expression levels of EGF, FGFr2 and Wnt3a in the salivary glands of mice in the experimental groups were significantly lower than those of mice in the control groups. CONCLUSION: The soft diet indeed influenced the salivary gland through insufficient mastication, which could be one of the primary factors inducing JRP.

Toll-like receptor 9 signaling promotes autophagy and apoptosis via divergent functions of the p38/JNK pathway in human salivary gland cells.

Abnormal signaling transduction in salivary gland cells is associated with the pathogenesis of Sjögren's syndrome (SS). Previously, we identified aberrant expression of toll-like receptor 9 (TLR9) in gland cells of SS patients and mouse models. In this study, we investigated the role of TLR9 and its downstream p38/mitogen-activated protein kinase (MAPK) and c-Jun N-terminal kinase (JNK) signaling in mediating apoptosis and autophagy in human salivary gland (HSG) cells. We selected either CpG-Odn, a classical TLR9 activator, or lentivirus-packaged TLR9 full-length cDNA to activate TLR9 signaling transduction. Activation of TLR9 signaling induced phosphorylation of its downstream protein kinases, p38/MAPK and JNK, in a time-dependent manner, and decreased HSG cell viability. Western blotting of LC3B-II and p62 in both normal and autophagic flux-administered conditions revealed elevated autophagy upon TLR9 activation. Observing the cell cytoplasm through transmission electron microscopy and mRFP-GFP-LC3B-tagged fluorescence confirmed an increased number of autophagosomes and autolysosomes in TLR9-activated cells. Bax/Bcl-2 ratio calculations, caspase-3 activity assays and Hoechst nuclear staining were utilized to confirm the involvement of apoptosis in TLR9 signaling activation. Furthermore, we selected SB239063, a p38/MAPK signaling inhibitor, and SP600125, a JNK inhibitor, to identify the functions of p38/MAPK and JNK in TLR9-mediated signaling transduction. Multiple approaches, including Western blotting assays, fluorescence assessments and caspase-3 activity measurements, confirmed that inhibition of p38/MAPK signaling ameliorated both autophagy and apoptosis in TLR9-activated HSG cells, whereas inhibition of JNK signaling attenuated apoptosis but failed to modulate autophagy in the models mentioned above. Our results indicate a divergent function of p38/MAPK and JNK in TLR9-mediated autophagy and apoptosis in salivary gland cells.

Long non-coding RNA expression profile in minor salivary gland of primary Sjögren's syndrome.

BACKGROUND: To examine the roles of long noncoding RNAs (lncRNAs) in the regulation of primary Sjögren's syndrome (pSS) and reveal the expression profile of lncRNAs in labial salivary glands (LSGs) in pSS patients. METHOD: The expression of 63,431 lncRNAs and 39,887 mRNAs were determined in the LSG of four pSS patients and four healthy controls using microarray experiments. Validation was performed in 30 pSS patients and 16 controls using real-time PCR. LncRNA-mRNA co-expression and gene-pathway networks were constructed using bioinformatics software. RESULT: A total of 1243 lncRNAs (upregulated: 890, downregulated: 353) and 1457 mRNAs (upregulated: 1141, downregulated: 316) were differentially expressed in the LSGs of pSS patients (fold change >2, P <0.05). Eight of these lncRNAs were validated using real-time PCR. ENST00000420219.1 (3.13-fold), ENST00000455309.1 (2.51-fold), n336161 (2.45-fold), NR_002712 (2.41-fold), ENST00000546086.1 (1.94-fold), Lnc-UTS2D-1:1 (1.79-fold), n340599 (1.69-fold), and TCONS_l2_00014794 (1.28-fold) were significantly upregulated in pSS. There were strong correlations between these lncRNAs and ß2 microglobulin, disease course, erythrocyte sedimentation rate (ESR), rheumatoid factor (RF), IgA, IgM, visual analogue scale (VAS) of parotid swelling and VAS of dry eyes. Computational analyses revealed that 28 of the differentially expressed (DE) mRNAs were associated with eight DE lncRNAs involved in chemokine signaling pathways, the nuclear factor-kappa B (NF-κB) signaling pathway, and tumor necrosis factor (TNF) signaling pathway. CONCLUSIONS: Our study revealed the expression profile of lncRNAs in LSGs of pSS patients. Many novel lncRNA transcripts that play important roles in the pathogenesis of pSS were dysregulated in pSS. Therefore, this study will aid in the development of new diagnostic biomarkers and drug therapies.

Function of the parotid gland in juvenile recurrent parotitis: a case series.

Our aim was to find out how the parotid gland functions in 44 patients with juvenile recurrent parotitis, and to assess the value of measuring the serum amylase activity. Clinical and personal details were recorded, and all patients had their serum amylase activity measured together with sialography during the chronic phase. The function of the gland was classified by sialographic images. The chi square test and Spearman's rank correlation coefficient were used in the statistical analyses. There was a significant association between the degree of glandular function and serum amylase activity (p=0.014). The patients with unilateral and bilateral disease differed significantly in their degree of glandular function (p=0.020), those with bilateral disease having poorer function. There were no significant correlations between other clinical variables and glandular function. Serum amylase activity is an important diagnostic variable in juvenile recurrent parotitis, and poor parotid function reflects the severity of the disease.

Endoscopic-assisted gland preserving therapy for the management of parotid gland sialolithiasis: Our preliminary experience.

OBJECTIVE: To analyze the efficacy and safety of sialendoscopy and a combined transoral or transcutaneous and sialendoscopic approach in the removal of parotid gland sialoliths. METHODS: This retrospective study included 29 patients diagnosed with parotid gland sialolithiasis who required endoscopic-assisted gland preserving therapy. Ultrasonography and computed tomography were used to diagnose parotid sialolithiasis. The use of interventional sialendoscopy, sialendoscopic-transoral, or sialendoscopic-transcutaneous procedures was determined by the characteristics of the parotid gland stones. RESULTS: The stones were extracted by interventional sialendoscopy in nine patients. The transoral procedure was performed in 15 patients with large stones which were impacted in the ductal wall. The remaining five patients were managed through an external approach via a local incision under sialendoscopy. No postoperative complications occurred. The parotid glands were functioning normally after the procedures. CONCLUSION: The combined sialendoscopic-transoral and sialendoscopic-transcutaneous operation appears to be a good alternative for parotid gland sialolithiasis in the absence of lithotripsy devices. This type of therapy can, therefore, decrease the rate of parotidectomy.

Clinical features and treatment outcomes of immunoglobulin g4-related sclerosing sialadenitis.

Idiopathic enlargement of salivary glands used to be confusing in diagnosis until immunoglobulin G4 (IgG4)-related sclerosing sialadenitis was proposed as a possible answer. In this case series, we reported the clinical features and management outcomes in 16 patients with IgG4-related sclerosing sialadenitis. We retrospectively studied 16 patients in clinical examination, serology, pathology, and sonography features. All patients were treated by corticosteroids and followed up for at least 3 months. The results of clinical features showed that all of the patients presented persistent, symmetric bilateral swelling of the salivary glands, elevated levels of serum IgG4, and/or IgG4-positive plasmacytes infiltration and tissue fibrosis. The results of all autoantibody tests were negative. The typical sonographic manifestation revealed multiple hypoechoic foci with an irregular netlike diffuse lesion in salivary glands. Most patients showed excellent response to steroids treatment. We conclude that, for patients who present (1) symmetric swelling of bilateral salivary glands for more than 3 months, (2) elevated serum IgG4 level (>135 mg/dL), and (3) enlargement in bilateral salivary glands with multiple hypoechoic areas (irregular netlike appearance) in the sonography, the diagnosis of IgG4-related sclerosing sialadenitis should be considered. A comprehensive understanding of the medical condition and appropriate pathology examination are the key to diagnose. Steroids treatment is effective, and a treatment plan should be set up and followed in the long-term.

Activation of TLR9-dependent p38MAPK pathway in the pathogenesis of primary Sjögren's syndrome in NOD/Ltj mouse.

OBJECTIVE: The objective of this study was to investigate the potential role of Toll-like receptor 9-dependent p38 MAPK signaling pathway in the pathogenesis of primary Sjögren's syndrome (pSS) in NOD/Ltj mouse, aiming to identify an ideal target therapy model for human pSS. METHODS: NOD/Ltj mice were chosen as a model of pSS. The Toll-like receptor 9 and p-p38 MAPK double-positive peripheral blood mononuclear cells (PBMCs) of 4-, 5-, 8-, 10-, and 15-week-old NOD/Ltj mouse were analyzed by flow cytometry. The expressions of Toll-like receptor 9 and p-p38 MAPK in the submandibular gland (SMG) were also examined by immunohistochemistry. The change of stimulated salivary flow rate was dynamically measured, and the histopathology of SMG was evaluated by hematoxylin and eosin stain. RESULTS: The stimulated salivary flow rate in NOD/Ltj was reduced to 50-60% of the flow rate of control mice since the fifth week onwards. The Toll-like receptor 9 and p-p38 MAPK double-positive PBMCs in both groups increased gradually from 5 weeks, peaked at 8 weeks and then gradually decreased at 10 weeks, yet the percentage of Toll-like receptor 9 and p-p38MAPK double-positive PBMCs in 5-, 8-, and 10-week-old NOD/Ltj mouse was significantly increased compared with those in control subjects. After the 10th week onwards, there were no significant differences in the Toll-like receptor 9 and p-p38 MAPK double-positive PBMCs between NOD/Ltj mice and controls. Immunohistochemical staining showed that Toll-like receptor 9 was positive in the acinar epithelium cells and infiltrating lymphocytes in NOD/Ltj mice. p-p38 MAPK was detected in infiltrating lymphocytes and few ductal or acinar epithelium cells adjacent to infiltrating lymphocytes in NOD/Ltj mice. CONCLUSIONS: From the fifth week till the tenth week, Toll-like receptor 9 and p-p38 MAPK double-positive PBMCs were significantly increased in NOD/Ltj mice, accompanied with reduced stimulated salivary flow rate and Toll-like receptor 9 or p-p38 MAPK positive infiltrating lymphocytes observed in the SMG of NOD/Ltj mouse. Our results indicated that activation of Toll-like receptor 9-depended p38 MAPK signal pathway in PBMCs was an early event in pSS which made NOD/Ltj as an ideal therapy model to test the treatment effects of p38 MAPK or Toll-like receptor 9 inhibitors on pSS.

Foreign body induced sialolithiasis treated by sialoendoscopic intervention.

OBJECTIVE: The study aims to identify the impact of sialolith formation by reviewing the foreign body induced sialolithiasis treated by sialoendoscopic intervention. METHODS: The study group included 13 patients whose sialolithiasis was induced by foreign body. After the routine radiographic examination, sialoendoscopic procedures were performed. Then, the treatment protocol was designed. RESULTS: The occupations of the 13 patients included 5 fishermen, 3 office workers, 2 workers, 1 teacher, 1 farmer, and 1 retired police officer. All patients had a unique diet habit-seafood. Eleven patients had a remembered incident of implanted fish bone and the following symptoms, with either obstructions or infections. Only 2 of the 13 had no memory of such an injury. All the stones were in the ducts of submandibular glands. In 10 procedures, there was 1 solitary stone, whereas 2 stones were encountered in 3 procedures. After being removed, 16 stones were crushed to expose the fish bone nidus of the stone. There was relief of symptoms after the procedures. CONCLUSIONS: This study supported the possibility that some sialoliths resulted from a retrograde migration within the salivary ducts. In our study, the occupations (fisherman), the diet habit (seafood), and the injury history (a remembered incident of implanted fish bone and the following symptoms) were obviously related to the stone formation that was induced by the fish bone.

The serological profiles of subgroup of primary Sjögren's syndrome correlation with the clinical features of parotid glands.

OBJECTIVE: To investigate the difference of serological profile in pSS and their correlation with the clinical characteristics of parotid glands. METHODS: This retrospective study includes 289 patients who fulfilled the 2002 American-European Consensus Group Criteria for pSS. The patients were categorized by the clinical features of parotid glands: Group 1 (massive group), Group 2 (infection group), Group 3 (swelling group) and Group 4 (others). The demographic data and serological profiles among these groups were compared. Statistical analyses of the results between groups were performed using the Student t test, Fisher's exact test, chi-square and analysis of variance. RESULTS: There was a difference of serological profile in the different clinical characteristics of parotid glands of pSS. Serum Ig G value of Group 1 was the greatest, and complement C4 was lowest in the four groups. Serum Ig E value of Group 2 was the greatest and ESR of Group 3 was the greatest in the four groups. CONCLUSION: This study has determined the differences of serological profile in the different clinical features of parotid glands of pSS patients, which may help advance our understanding of the disease and improve patient management.

Bilateral aplasia of the major salivary glands and unilateral atresia of lacrimal duct.

Aplasia of the major salivary glands, especially the parotid gland, is a rare disorder. Up to now, few cases have been reported. Clinically, patients may present with xerostomia, irritable eyes, severe dental caries, or asymptomatic manifestations.According to clinical and radiologic findings, we reported a case of a 20-year-old girl with bilateral aplasia of the major salivary glands and unilateral atresia of the lacrimal duct and made telephone follow-up 1 year later.