Background:Ziwan and Taoren(ZT)is a classic medicine pair in the formula of Mai Dong Di Shao Decoction,has been used to treat primary Sjogren’s syndrome(pSS)for more than 20 years.But its action mechanism is still un...Background:Ziwan and Taoren(ZT)is a classic medicine pair in the formula of Mai Dong Di Shao Decoction,has been used to treat primary Sjogren’s syndrome(pSS)for more than 20 years.But its action mechanism is still unknown.This study is aimed to reveal the potential mechanism of ZT treated pSS and discover its active compounds of ZT and therapeutic target for pSS.Methods:Firstly,the potential pathways of ZT for pSS treatment were predicted through network pharmacology and GO and KEGG enrichment analysis.Secondly,the inter-structural relationships between active compounds of ZT and target proteins were visualized using molecular docking techniques.Finally,efficacy and mechanism were conducted through in vivo experiments,such as water intake,spleen index,hematoxylin-eosin staining pathological changes,ELISA,Western Blot analysis,and immunofluorescence staining.Results:Nine active compounds were extracted from network pharmacology,including quercitrin,luteolin,kaempferol,β-sitosterol,isorhamnetin,galangin,hederagenin,diosmetin and gibberellin 7.Seven disease targets were identified:RELA,TP53,AKT1,interleukin(IL)6,MAPK1,ESR1,IL10;with RELA being the most core target.KEGG and GO enrichment analysis indicated that ZT may act through the TLR/NF-κB/RELA inflammatory mechanism process.preliminary results of molecular docking showed that ZT’s active compounds bind well to the RELA(p65)receptor.In vivo results demonstrated that a high dose of ZT significantly improved water intake and reduced lymphocytes infiltration in submandibular gland pathology in NOD mice.The expression content of AQP5 and vasoactive intestinal peptide in the submaxillary gland was significantly increased,while levels of inflammatory factors such as tumor necrosis factor-α,IL-6,and IL-1βalong with protein expressions including toll-like receptor4,p-p65 and p-IKKα/βin NF-κB pathway were reduced.Conclusions:The ZT treatment exhibits a promising efficacy in mitigating dryness symptoms of pSS,potentially attributed to its capacity for suppressing the TLR/NF-κB inflammatory signaling pathway.展开更多
This case study aims to contribute to the literature in order to highlight the importance of this collaboration between medical specialties. A female patient R.N.N. F, age 66, from the city of Manaus, with a previous ...This case study aims to contribute to the literature in order to highlight the importance of this collaboration between medical specialties. A female patient R.N.N. F, age 66, from the city of Manaus, with a previous diagnosis of Sjogren’s syndrome in regular follow-up by the Rheumatology team at the Araujo Lima outpatient clinic and referred to the Ophthalmology sector for complementary evaluation related to visual discomfort. The fundoscopy performed in the patient was within normal limits, but the symptoms experienced by her proved to be an important clinical finding, which has ratified the need for regular and multidisciplinary follow-up. This report unequivocally demonstrates that even in the face of tests considered within the expected limits for a given population, the clinical presentation can be specific and particular for each analyzed individual. Early screening exams should contemplate the patient in a holistic and individualized way whenever possible.展开更多
AIM:To focus on different visual resolution tasks under photopic and mesopic conditions in Sjogren’s syndrome patients compared to age-matched healthy controls.METHODS:The visual resolution measurements included high...AIM:To focus on different visual resolution tasks under photopic and mesopic conditions in Sjogren’s syndrome patients compared to age-matched healthy controls.METHODS:The visual resolution measurements included high and low visual acuities and contrast sensitivity functions.These tests were conducted under photopic and then mesopic conditions.Twenty-one Sjögren’s syndrome patients and 21 aged-matched healthy volunteers completed all the measurements in this study.RESULTS:Sjogren’s syndrome patients have greater impairment in contrast sensitivity than standardized visual acuity.This reduction was significant under the mesopic condition.Also,Sjogren’s syndrome patients treated with pilocarpine suffer more than patients without pilocarpine treatment under low light conditions.CONCLUSION:Sjogren’s syndrome patients shows greater impairment in different visual resolution tasks due to dry eye symptoms.展开更多
AIM:To compare the corneal biomechanics of Sj?gren's syndrome(SS) and non-SS dry eyes with Corneal Visualization Scheimpflug Technology(CorV is ST).METHODS:Corneal biomechanics and tear film parameters, namely...AIM:To compare the corneal biomechanics of Sj?gren's syndrome(SS) and non-SS dry eyes with Corneal Visualization Scheimpflug Technology(CorV is ST).METHODS:Corneal biomechanics and tear film parameters, namely the Schirmer I test value, tear film break-up time(TBUT) and corneal staining score(CSS) were detected in 34 eyes of 34 dry eye patients with SS(SSDE group) and 34 dry eye subjects without SS(NSSDE group) using CorV is ST. The differences of the above parameters between the two groups were examined, and the relationship between corneal biomechanics and tear film parameters were observed. RESULTS:The differences in age, sex, intraocular pressure(IOP) and central corneal thickness(CCT) were not significant between the two groups(P〉0.05). The tear film parameters had significant differences between the SSDE group and NSSDE group(all P〈0.05). Patients in the SSDE group had significantly lower A1-time and HC-time, but higher DA(P=0.01, 0.02, and 0.02, respectively) compared with the NSSDE group. In the SSDE group, DA was negatively correlated with TBUT(rho=-0.38, P=0.03); HC-time was negatively correlated with CSS(rho=-0.43, P=0.02). In the NSSDE group, HC-time was again negatively correlated with CSS(rho=-0.39, P=0.02).CONCLUSION:There are differences in corneal biomechanical properties between SSDE and NSSDE. The cornea of SSDE tends to show less "stiffness", as seen by a significantly shorter A1-time and HC-time, but larger DA, compared with the cornea of NSSDE. Biomechanical parameters can be influenced by different tear film parameters in both groups.展开更多
Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with (n = 6) or without primary Sjogr...Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with (n = 6) or without primary Sjogreffs syndrome (n = 10). All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjogren's syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinudear autoantibody, anti-Sjogren's-syndrome-related antigen A an- tibodies, anti-Sjogren's-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjogren's syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% (4/6) of patients with primary Sjogren's syndrome and in 60% (6/10) of patients without primary Sj6gren's syndrome. More brain abnormalities were observed in patients without primary Sj6gren's syndrome than in those with primary Sj6gren's syndrome. Segments lesions (〉 3 centrum) were noted in 50% (5/10) of patients without primary Sj6gren's syndrome and in 67% (4/6) of patients with primary Sjogren's syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjogren's syndrome are similar. However, neu- romyelitis optica spectrum disorder patients without primary Sjogreffs syndrome have a high frequency of brain abnormalities.展开更多
OBJECTIVE To study the therapeutic effects of TGP on SS both in C57BL/6J mice immunized by immu⁃nological induction(SS mice)and NOD/ShiltJNju(NOD)mice.METHODS TGP(180,360,720 mg·kg^-1)was intragastri⁃cally admini...OBJECTIVE To study the therapeutic effects of TGP on SS both in C57BL/6J mice immunized by immu⁃nological induction(SS mice)and NOD/ShiltJNju(NOD)mice.METHODS TGP(180,360,720 mg·kg^-1)was intragastri⁃cally administered for 6 or 16 weeks for SS mice and NOD mice,respectively.Weekly food and water intake,saliva flow,submandibular gland(SMG)and spleen index,and SMG pathology were measured.ELISA was used to evaluate serum interleukin-6(IL-6),tumor necrosis factor-α(TNF-α),interferon-γ(IFN-γ)and autoantigens(SSA/Ro,SSB/La,andα-fodrin).Real-time PCR and Luminex liquid suspension chip assay were applied to analyze SMG inflammatory cytokines mRNA TNF-α,IL-17A,CXCL9,CXCL13,and B-cell activating factor(BAFF)and protein(IL-1β,IL-6,TNF-α,and IFN-γ)expres⁃sion.RESULTS Compared with SS mice,TGP(720 mg·kg^-1)treatment increased saliva flow,reduced organ indexes,and decreased serum IL-6 and IFN-γ concentration.TGP(360 mg·kg^-1)treatment decreased serum IFN-γ concentra⁃tion.TGP(180,360,720 mg·kg^-1)treatment improved SMG pathological damage.Compared with NOD mice,the saliva flowincreased from 9 to 15 weeks of administration.After 2 weeks of administration,TGP(720 mg·kg^-1)treatment decreased serum SSA/Ro,SSB/La and a-fodrin concentration,increased SMG index,inhibited SMG IFN-γ concentra⁃tion,and down-regulated SMG TNF-α,IL-17A,CXCL9,CXCL13 and BAFF mRNA expression.TGP(360 mg·kg^-1)treat⁃ment decreased serum SSB/La and a-fodrin,and SMG TNF-α and IFN-γ concentration,and down-regulated SMG TNF-α,IL-17A,CXCL9 and BAFF mRNA expression.TGP(180 mg·kg^-1)treatment decreased serum SSB/La,a-fodrin,and SMG IL-1β concentration,and down-regulated SMG TNF-α,IL-17A and BAFF mRNA expression.After 8 weeks of administration,TGP(180,360,720 mg·kg^-1)treatment increased SMG index,and decreased serum a-fodrin concentra⁃tion.TGP(720 mg·kg^-1)treatment down-regulated mRNA expression of SMG TNF-α,IL-17A,CXCL9,CXCL13,and BAFF.TGP(360 mg·kg^-1)treatment reduced mRNA expression of TNF-α,CXCL9,CXCL13 and BAFF,and concentra⁃tion of IL-6 and TNF-α.TGP(180 mg·kg^-1)treatment down-regulated mRNA expression of TNF-α,CXCL9,and CXCL13,and decreased IL-6 and TNF-αconcentration in SMG.After 16 weeks of administration,TGP(180,360,720 mg·kg^-1)treatment reduced serum SSA/Ro and a-fodrin concentration,increased SMG index,and decreased SMG CXCL13 and BAFF mRNA expression.TGP(360,720 mg·kg^-1)treatment decreased serum SSB/Laconcentration and SMG TNF-α,IL-17A and CXCL9 mRNA expression.Besides,TGP(180,360,720 mg·kg^-1)treatment alleviated the pathological damage of SMG after 2 and 16 weeks of administration.CONCLUSION TGP has a certain therapeutic effect onmice through inhibiting inflammatory responses.展开更多
The recognition of the profound impact of the human gastrointestinal microbiome(GM) on human autoimmune diseases has gradually increased thanks to deeper research efforts. As a systemic autoimmune disease, primary Sjo...The recognition of the profound impact of the human gastrointestinal microbiome(GM) on human autoimmune diseases has gradually increased thanks to deeper research efforts. As a systemic autoimmune disease, primary Sjogren’s syndrome(pSS) cannot be completely cured. Human studies have revealed that GM species and diversity are altered in patients with p SS compared with healthy individuals. Animal studies have provided possible mechanisms for the association between pSS and GM. The potential role of GM in pSS is exerted through several mechanisms. GM dysbiosis leads to increased intestinal permeability, which increases the risk of GM antigen exposure and activates specific autoreactive T lymphocytes via “molecular mimicry”. In addition, GM antigen exposure and intestinal immune tolerance loss caused by GM dysbiosis together induce chronic local gut mucosal inflammation, which deteriorates to systemic chronic non-specific inflammation with the circulation of pro-inflammatory lymphocytes and cytokines. These factors eventually activate autoreactive B lymphocytes and lead to pSS. If GM plays a key role in the pathogenesis of pSS, clarifying the underlying mechanisms will be helpful for the development of new therapies targeting GM for dry eye associated with pSS. This review summarizes the latest knowledge about the relationship between GM and p SS,with the aim of contributing to future research and to the development of new clinical applications.展开更多
BACKGROUND Sjogren’s syndrome(SS),which affect salivary gland function,is an autoimmune disease.SS may involve extraglandular organs.Approximately 10 to 20 percent of SS patients have clinically significant lung dise...BACKGROUND Sjogren’s syndrome(SS),which affect salivary gland function,is an autoimmune disease.SS may involve extraglandular organs.Approximately 10 to 20 percent of SS patients have clinically significant lung disease,but presentation of pulmonary amylodosis is extremly rare.The incidence of benign monoclonal gammopathy in SS patients is high,but multiple myeloma is rare.No case involving the simultaneous occurrence of two rare diseases,pulmonary amyloidosis and multiple myeloma,in the same patient with SS has been reported so far.CASE SUMMARY A 41-year-old male patient was referred to our hematology department due to incidentally detected gastric plasmacytoma.He had been diagnosed with SS four years earlier.Multiple miliary nodules,ground glass opacity in both lung fields,and enlargement of both inguinal lymph nodes was observed on chest and abdomen computer tomography.Based on the pathological findings of lung and lymph node biopsied specimens,the patient was diagnosed with pulmonary amyloidosis and multiple myeloma.Pulmonary amyloidosis and multiple myeloma associated with SS has rarely been reported.CONCLUSION This is an extremely rare case of simultaneous pulmonary amyloidosis and multiple myeloma in the same patient with SS.展开更多
This paper reports the diagnosis of 65 patients with Sjogren′s Syndrome. Among these patients, 20 patients(30.77%) were complicated with oral candidiasis. The twenty patients were treated by topical nystatin smearing...This paper reports the diagnosis of 65 patients with Sjogren′s Syndrome. Among these patients, 20 patients(30.77%) were complicated with oral candidiasis. The twenty patients were treated by topical nystatin smearing, 2% sodium bicarbonate solution rinsing, and transfer factor injecting for about 2 months. The soreness, erythema, unpleasant feeling, angular cheilitis in mouths significantly diminished or completely disappeared, following two months treatments. The good results have been kept 6 months after follow up. However, dry mouth and atrophic changes, though showed no furture development, the therapeutic response was not significant.展开更多
BACKGROUND Sj?gren syndrome(SS)is a chronic and systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands.And histoplasmosis is an invasive mycosis caused by the saprophytic dimorphi...BACKGROUND Sj?gren syndrome(SS)is a chronic and systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands.And histoplasmosis is an invasive mycosis caused by the saprophytic dimorphic fungus H.capsulatum.In patients with primary SS(PSS),disseminated histoplasmosis(DH)is extremely rare.CASE SUMMARY We report a 37-year-old female patient admitted to our hospital with exacerbating fatigue,somnolence,and pancytopenia as the main symptoms.She was eventually diagnosed with DH based on pancytopenia,splenomegaly,and findings of bone marrow smears.The atypical clinical symptoms made the diagnosis process more tortuous.Unfortunately,she died of respiratory failure on the day the diagnosis was confirmed.CONCLUSION We present a rare and interesting case of DH in a PSS patient.This case updates the geographic distribution of histoplasmosis in China,and expands the clinical manifestations of DH in PSS,highlighting the significance of constantly improving the understanding of PSS with DH.展开更多
Objective To evaluate the saliva ferning test (SFT) as diagnostic test for xerostomia in patients with Sjogren’s Syndrome (SS). Methods In this study, dried samples of freshly produced saliva from 78 patients with es...Objective To evaluate the saliva ferning test (SFT) as diagnostic test for xerostomia in patients with Sjogren’s Syndrome (SS). Methods In this study, dried samples of freshly produced saliva from 78 patients with established SS according to European Community criteria and 80 healthy controls were examined by light microscopy. The crystallization was classified into 4 types according to the ferning phenomenon: uniformity, branching, spreading and integrity (type I normal and type Ⅱ,Ⅲ,Ⅳ abnormal ). Then, the 78 patients underwent lebial salivary gland biopsy. According to Tarpley’s classifica- tion, minor salivary gland biopsy (≥2 + ) was considered to be positive . Results 1. The sensitivity of SFT was high (70/ 78 = 89. 74% ). And the specificity wes also high (67/80 = 83. 75% ). 2. Abnormal SFT was observed in 70/78 (89. 74% ) samples from patients group and in 13 /80 (16. 25% ) samples from healthy controls. The differences of SFT in patients group versus controls were statistically significant (P< 0. 01 ). 3. The sensitivity of SFT and mini labial gland biopsy had no significant differences (P > 0. 05) as diagnostic tests in SS. Conclusion SFT was simple, sensitive and specific as diagnostic test in SS suspect patients just as mini labial gland biopsy.展开更多
Susceptibility to pneumococcal infections in Sj?gren syndrome (SS)―an autoimmune inflammatory disease―patients is not well known, although these patients frequently develop respiratory diseases. The relative risk of...Susceptibility to pneumococcal infections in Sj?gren syndrome (SS)―an autoimmune inflammatory disease―patients is not well known, although these patients frequently develop respiratory diseases. The relative risk of developing pneumococcal disease in SS patients versus diabetes mellitus type 2 (DM-2) patients, matched by age, gender, and length of enrolment was studied. From January 1998 to September 2013 the records of Donostia University Hospital were analyzed, which among other patient’s data includes: number and type of hospital admissions and number and type of laboratory determinations. Streptococcus pneumoniae isolates of the same serotype causing recurrent infections were characterized by PFGE. The study comprised 127 patients in the SS group (69 primary and 58 secondary) and 127 in the DM-2 group as control. In 12 SS patients, (9.4%) 22 pneumococcal disease episodes were detected. Two patients (1.6%) with a single episode each one were observed among DM-2 patients, p = 0.01, RR for SS patients 6 (95% CI 1.4 to 26.3). No differences could be demonstrated between the two groups of patients in infections caused by Staphylococcus aureus or Streptococcus agalactiae. Most pneumococcal serotypes in SS patients belonged to the 13-valent (50%) and 23-valent (75%) anti-pneumococcal vaccine. SS patients are associated with and increased risk of suffering from pneumococcal disease. Vaccination should be considered in this group of patients.展开更多
Background:Sjögren syndrome(SS)is a long-term autoimmune disease that affects the body’s moisture-producing glands.It can occur independently of other health problems(primary disease)or as a result of another co...Background:Sjögren syndrome(SS)is a long-term autoimmune disease that affects the body’s moisture-producing glands.It can occur independently of other health problems(primary disease)or as a result of another connective tissue disorder(secondary disease).Medication is directed at the person’s symptoms.Recently,it is reported that prescription of Chinese medicine has a great effect on SS and reduce adverse drugs reaction,which can provide a more safer treatment to SS.Case presentation:We here report a 43 year-old Chinese patient with continuous immune thrombocytopenia diagnosed as Sjögren syndrome,who was not response to routine medication such as Methylprednisolone and acyclovir.After that,the patient was treated by prescription of Chinese medicine,and the symptoms were improved apparently.We suppose that prescription of Chinese medicine has an unexpected effect on SS,which can alleviate patient’s symptoms.Conclusion:The clinical efficacy of Sjogren syndrome(SS)is effective in the treatment of prescription of Chinese medicine.It can improve patient’s symptoms and reduce adverse drug reactions.展开更多
Salts of calcium phosphate and inorganic phosphate are normally found in serum and extracellular fluids, balancing through poorly understood factors that prevent abnormal tissue deposition of these minerals. However, ...Salts of calcium phosphate and inorganic phosphate are normally found in serum and extracellular fluids, balancing through poorly understood factors that prevent abnormal tissue deposition of these minerals. However, in those tissues that are injured, especially due to chronic inflammatory processes, a predisposition to the deposition of these minerals is developed, triggering what has been called Dystrophic Calcinosis (DC), common in different Connective Tissue Diseases (CTD), especially dermatomyositis and scleroderma, but there is no a frecuent association with diseases like Rheumatoid Arthritis (RA) and Sj?gren Syndrome (SS). We report a case of a female patient of 63 years old with RA and Secundary SS who presents with DC in the hands and no evidence of other connective tissue.展开更多
We report a 9-year-old Japanese girl who presented with muscle weakness and elevated serum levels of muscle-derived enzymes following mycoplasma infection. Rhabdomyolysis or myositis was suggested by magnetic resonanc...We report a 9-year-old Japanese girl who presented with muscle weakness and elevated serum levels of muscle-derived enzymes following mycoplasma infection. Rhabdomyolysis or myositis was suggested by magnetic resonance imaging and repeated four times within 4 years. Each episode developed following respiratory infection and spontaneously recovered. The diagnosis of Sjogren’s syndrome was made by decreased salivary secretion, MR sialography, lip biopsy, and positive anti-SSA/Ro antibody. Given the rarity of rhabdomyolysis/myositis, recurrent episode could be induced by infectious disease on the basis of underlying Sjogren’s syndrome. Conclusion: Sjogren’s syndrome should be considered as an underlying disease of recurrent infection-induced rhabdomyolysis/ myositis.展开更多
Hypokalemic acidosis can complicate a primary Sjögren’s syndrome. The clinical feature is rarely revealed by manifestations due to hypokalemia. We report the case of a 46-year-old woman, admitted to explore ...Hypokalemic acidosis can complicate a primary Sjögren’s syndrome. The clinical feature is rarely revealed by manifestations due to hypokalemia. We report the case of a 46-year-old woman, admitted to explore a paresthesia and paresis of inferior limbs. The diagnosis of Sjögren’s syndrome was retained since there was the association of xerophthalmia, sialadenitis at the labial biopsy and positive immunological results (anti-SSA and anti-SSB). The absence of another auto-immune or systemic illness allowed us to consider that the Sjögren’s syndrome was primary. The biological explorations revealed a hyperchloremic and hypokalemic acidosis. The treatment was based on corticosteroid and potassium supplementation. The follow-up was marked by a clinical and biological amelioration.展开更多
Mesenteric Panniculitis is a benign fibro-inflammatory process involving adipose tissue of the mesentery. It is characterised by fat necrosis, chronic inflammation and fibrosis, causing thickening and shortening of th...Mesenteric Panniculitis is a benign fibro-inflammatory process involving adipose tissue of the mesentery. It is characterised by fat necrosis, chronic inflammation and fibrosis, causing thickening and shortening of the mesentery. Patients may present with localised abdominal pain, abdominal mass, intestinal obstruction and ischaemic colitis. We report a case of mesenteric panniculitis causing abdominal pain in a patient with active Primary Sjogren’s Syndrome. The rarity of this case makes it of interest. We review the current literature on mesenteric panniculitis and its association with connective tissue disease and inflammatory conditions. A 64-year-old Caucasian male presented in 1994 with dry mouth. A diagnosis of Primary Sjogren’s Syndrome (PSS) was made on salivary gland biopsy. In 2010 he presented with an exacerbation of his eye symptoms, muscle pain and fatigue. He complained of abdominal pain and night sweating, but denied any weight loss or change in bowel habit. There was no significant past medical history other than PSS. On examination he had a small right submandibular node and mild synovitis at the right proximal interphalangeal joint and carpometacarpal joint. Examination of the abdomen showed marked umbilical tenderness but no organomegally. Blood tests at this time showed an active inflammation: CRP of 61 (NR 5 mg/L), ESR 39 (NR 20 mm/s), strongly positive ENA Ro and La. IgG was elevated at 18.6 (NR 5.8-15.4), Complement was low at 0.17 (NR 0.18-0.6). An abdominal ultrasound scan demonstrated a 6 × 3 ×3 cmarea of diffuse homogenous fat encasing some mesenteric vessels in the area of focal tenderness. CT abdomen and pelvis showed oedematous mesenteric fat and lymph nodes in the jejunal small bowel mesentery, consistent with mesenteric panniculitis. Laparoscopic biopsy was discussed with the surgical team, but was felt not indicated as risk outweighed potential benefit. The patient was treated with a 9-week reducing course of oral steroids. His abdominal symptoms resolved although CT abdomen showed little improvement in mesenteric panniculitis. A review of the literature suggests that currently there is no standard treatment and management should be guided by patient symptoms. Mesenteric Panniculitis is rare;as a result evidence for treatment is limited to individual case reports. There is no clear link between symptom improvement and radiological resolution of mesenteric panniculitis. It has, therefore been suggested that follow-up imaging should be limited to those with persistent symptoms. Overall the prognosis for mesenteric panniculitis is good, up to half of patients do not require treatment, and recurrence of symptoms is uncommon.展开更多
Tubulointerstitial nephritis complicated by primary Sj?gren’s syndrome in a patient under treatment for type 2 diabetes mellitus was diagnosed in the early stage of the disease by renal biopsy. The symptoms of primar...Tubulointerstitial nephritis complicated by primary Sj?gren’s syndrome in a patient under treatment for type 2 diabetes mellitus was diagnosed in the early stage of the disease by renal biopsy. The symptoms of primary Sj?gren’s syndrome, such as thirst and polydipsia, were masked by the characteristic symptoms of type 2 diabetes mellitus. An association between sicca symptoms and diabetes mellitus (types 1 and 2) has been previously reported. Hence, it is possible that there are common underlying immunological mechanisms between primary Sj?gren’s syndrome and diabetes mellitus of both types. Intervention with steroids in a timely manner appears to have prevented or slowed the progression of re- nal impairment.展开更多
Sjogren’s syndrome is a chronic autoimmune exocrinopathy associated with dry eyes and dry mouth as major clinical manifestations. It is characterized by lymphocytic infiltration of lacrimal and salivary glands and au...Sjogren’s syndrome is a chronic autoimmune exocrinopathy associated with dry eyes and dry mouth as major clinical manifestations. It is characterized by lymphocytic infiltration of lacrimal and salivary glands and autoantibody production, especially anti-Ro (or SSA) and anti-La (or SSB). Lymphoproliferative disorders are a feature of Sjogren’s syndrome, and can be considered an extraglandular manifestation of the disease. Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare form of lymphadenitis. It is reported more often in young adult women with localized lymphadenopathy (usually cervical), fever, rashes, and leukopenia. It is a self-limiting disease with resolution within 1 - 4 months in almost all patients. Sjogren’s syndrome has been reported in patients with other systemic diseases including SLE and lymphomas. Here we present a patient with Kikuchi-Fujimoto disease who developed Sjogren’s Syndrome 8 years after her diagnosis of Kikuchi-Fujimoto disease.展开更多
Interleukin-2(IL-2)is an important cytokine that plays a key role in the immune response.The IL-2 receptor(IL-2R)is composed of three subunits,alpha,beta,and gamma,with the alpha subunit having the highest affinity fo...Interleukin-2(IL-2)is an important cytokine that plays a key role in the immune response.The IL-2 receptor(IL-2R)is composed of three subunits,alpha,beta,and gamma,with the alpha subunit having the highest affinity for IL-2.Several studies reported that immune dysregulation of IL-2 may cause tissue injury as well as damage leading to the pathogenesis of various autoimmune diseases such as acute necrotizing vasculitis in systemic lupus erythematosus(SLE),inflammatory synovitis in rheumatoid arthritis(RA),salivary and lacrimal gland dysfunction in Sjogren syndrome(SS),obliterative vasculopathy fibrosis in systemic sclerosis(SSc),and inflammatory demyelination in multiple sclerosis(MS).The aim of this review paper was to examine the role of IL-2/IL-2R in various autoimmune disorders,taking into account recent advancements and discoveries,gaps in the current literature,ongoing debates,and potential avenues for future research.The focus of this review is on systemic lupus erythematosus,rheumatoid arthritis,systemic sclerosis,sjogren syndrome,and multiple sclerosis,which are all linked to the malfunctioning of IL-2/IL-2R.In genetic studies,gene polymorphisms of IL-2 such as IL-2330/T,IL-2330/G,and rs2069763 are involved in increasing the risk of SLE.Furthermore,genetic associations of IL-2/IL-2R such as rs791588,rs2281089,rs2104286,rs11594656,and rs35285258 are significantly associated with RA susceptibility.The IL-2 polymorphism including rs2069762A,rs6822844T,rs6835457G,and rs907715T are significant connections with systemic sclerosis.In addition,rs2104286(IL-2),rs11594656(IL-2RA),rs35285258(IL-2RB)gene polymorphism significant increases the risk of multiple sclerosis.In therapeutic approaches,low-dose IL-2 therapy could regulate Tfr and Tfh cells,resulting in a reduction in disease activity in the SLE patients.In addition,elevated sIL-2R levels in the peripheral blood of SLE patients could be linked to an immunoregulatory imbalance,which may contribute to the onset and progression of SLE.Consequently,sIL-2R could potentially be a target for future SLE therapy.Moreover,Low dose-IL2 was well-tolerated,and low levels of Treg and high levels of IL-21 wereassociated with positive responses to Ld-IL2 suggested to be a safe and effective treatment for RA.Additionally,low-dose IL-2 treatment improves the exocrine glands'ability to secrete saliva in SS-affected mice.Whereas,Basiliximab targets the alpha chain of the IL-2 receptor suggested as a potential treatment for SSc.Also,pre-andpost-treatment with Tregs,MDSCs,and IL-2 may have the potential to prevent EAE induction in patients with MS.It is suggested that further studies should be conducted on IL-2 polymorphism in Sjogren syndrome.展开更多
基金grants from the National Natural Science Foundation of China(82274454).
文摘Background:Ziwan and Taoren(ZT)is a classic medicine pair in the formula of Mai Dong Di Shao Decoction,has been used to treat primary Sjogren’s syndrome(pSS)for more than 20 years.But its action mechanism is still unknown.This study is aimed to reveal the potential mechanism of ZT treated pSS and discover its active compounds of ZT and therapeutic target for pSS.Methods:Firstly,the potential pathways of ZT for pSS treatment were predicted through network pharmacology and GO and KEGG enrichment analysis.Secondly,the inter-structural relationships between active compounds of ZT and target proteins were visualized using molecular docking techniques.Finally,efficacy and mechanism were conducted through in vivo experiments,such as water intake,spleen index,hematoxylin-eosin staining pathological changes,ELISA,Western Blot analysis,and immunofluorescence staining.Results:Nine active compounds were extracted from network pharmacology,including quercitrin,luteolin,kaempferol,β-sitosterol,isorhamnetin,galangin,hederagenin,diosmetin and gibberellin 7.Seven disease targets were identified:RELA,TP53,AKT1,interleukin(IL)6,MAPK1,ESR1,IL10;with RELA being the most core target.KEGG and GO enrichment analysis indicated that ZT may act through the TLR/NF-κB/RELA inflammatory mechanism process.preliminary results of molecular docking showed that ZT’s active compounds bind well to the RELA(p65)receptor.In vivo results demonstrated that a high dose of ZT significantly improved water intake and reduced lymphocytes infiltration in submandibular gland pathology in NOD mice.The expression content of AQP5 and vasoactive intestinal peptide in the submaxillary gland was significantly increased,while levels of inflammatory factors such as tumor necrosis factor-α,IL-6,and IL-1βalong with protein expressions including toll-like receptor4,p-p65 and p-IKKα/βin NF-κB pathway were reduced.Conclusions:The ZT treatment exhibits a promising efficacy in mitigating dryness symptoms of pSS,potentially attributed to its capacity for suppressing the TLR/NF-κB inflammatory signaling pathway.
文摘This case study aims to contribute to the literature in order to highlight the importance of this collaboration between medical specialties. A female patient R.N.N. F, age 66, from the city of Manaus, with a previous diagnosis of Sjogren’s syndrome in regular follow-up by the Rheumatology team at the Araujo Lima outpatient clinic and referred to the Ophthalmology sector for complementary evaluation related to visual discomfort. The fundoscopy performed in the patient was within normal limits, but the symptoms experienced by her proved to be an important clinical finding, which has ratified the need for regular and multidisciplinary follow-up. This report unequivocally demonstrates that even in the face of tests considered within the expected limits for a given population, the clinical presentation can be specific and particular for each analyzed individual. Early screening exams should contemplate the patient in a holistic and individualized way whenever possible.
文摘AIM:To focus on different visual resolution tasks under photopic and mesopic conditions in Sjogren’s syndrome patients compared to age-matched healthy controls.METHODS:The visual resolution measurements included high and low visual acuities and contrast sensitivity functions.These tests were conducted under photopic and then mesopic conditions.Twenty-one Sjögren’s syndrome patients and 21 aged-matched healthy volunteers completed all the measurements in this study.RESULTS:Sjogren’s syndrome patients have greater impairment in contrast sensitivity than standardized visual acuity.This reduction was significant under the mesopic condition.Also,Sjogren’s syndrome patients treated with pilocarpine suffer more than patients without pilocarpine treatment under low light conditions.CONCLUSION:Sjogren’s syndrome patients shows greater impairment in different visual resolution tasks due to dry eye symptoms.
基金Supported by the National Natural Science Foundation of China(No.81070755)
文摘AIM:To compare the corneal biomechanics of Sj?gren's syndrome(SS) and non-SS dry eyes with Corneal Visualization Scheimpflug Technology(CorV is ST).METHODS:Corneal biomechanics and tear film parameters, namely the Schirmer I test value, tear film break-up time(TBUT) and corneal staining score(CSS) were detected in 34 eyes of 34 dry eye patients with SS(SSDE group) and 34 dry eye subjects without SS(NSSDE group) using CorV is ST. The differences of the above parameters between the two groups were examined, and the relationship between corneal biomechanics and tear film parameters were observed. RESULTS:The differences in age, sex, intraocular pressure(IOP) and central corneal thickness(CCT) were not significant between the two groups(P〉0.05). The tear film parameters had significant differences between the SSDE group and NSSDE group(all P〈0.05). Patients in the SSDE group had significantly lower A1-time and HC-time, but higher DA(P=0.01, 0.02, and 0.02, respectively) compared with the NSSDE group. In the SSDE group, DA was negatively correlated with TBUT(rho=-0.38, P=0.03); HC-time was negatively correlated with CSS(rho=-0.43, P=0.02). In the NSSDE group, HC-time was again negatively correlated with CSS(rho=-0.39, P=0.02).CONCLUSION:There are differences in corneal biomechanical properties between SSDE and NSSDE. The cornea of SSDE tends to show less "stiffness", as seen by a significantly shorter A1-time and HC-time, but larger DA, compared with the cornea of NSSDE. Biomechanical parameters can be influenced by different tear film parameters in both groups.
文摘Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with (n = 6) or without primary Sjogreffs syndrome (n = 10). All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjogren's syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinudear autoantibody, anti-Sjogren's-syndrome-related antigen A an- tibodies, anti-Sjogren's-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjogren's syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% (4/6) of patients with primary Sjogren's syndrome and in 60% (6/10) of patients without primary Sj6gren's syndrome. More brain abnormalities were observed in patients without primary Sj6gren's syndrome than in those with primary Sj6gren's syndrome. Segments lesions (〉 3 centrum) were noted in 50% (5/10) of patients without primary Sj6gren's syndrome and in 67% (4/6) of patients with primary Sjogren's syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjogren's syndrome are similar. However, neu- romyelitis optica spectrum disorder patients without primary Sjogreffs syndrome have a high frequency of brain abnormalities.
文摘OBJECTIVE To study the therapeutic effects of TGP on SS both in C57BL/6J mice immunized by immu⁃nological induction(SS mice)and NOD/ShiltJNju(NOD)mice.METHODS TGP(180,360,720 mg·kg^-1)was intragastri⁃cally administered for 6 or 16 weeks for SS mice and NOD mice,respectively.Weekly food and water intake,saliva flow,submandibular gland(SMG)and spleen index,and SMG pathology were measured.ELISA was used to evaluate serum interleukin-6(IL-6),tumor necrosis factor-α(TNF-α),interferon-γ(IFN-γ)and autoantigens(SSA/Ro,SSB/La,andα-fodrin).Real-time PCR and Luminex liquid suspension chip assay were applied to analyze SMG inflammatory cytokines mRNA TNF-α,IL-17A,CXCL9,CXCL13,and B-cell activating factor(BAFF)and protein(IL-1β,IL-6,TNF-α,and IFN-γ)expres⁃sion.RESULTS Compared with SS mice,TGP(720 mg·kg^-1)treatment increased saliva flow,reduced organ indexes,and decreased serum IL-6 and IFN-γ concentration.TGP(360 mg·kg^-1)treatment decreased serum IFN-γ concentra⁃tion.TGP(180,360,720 mg·kg^-1)treatment improved SMG pathological damage.Compared with NOD mice,the saliva flowincreased from 9 to 15 weeks of administration.After 2 weeks of administration,TGP(720 mg·kg^-1)treatment decreased serum SSA/Ro,SSB/La and a-fodrin concentration,increased SMG index,inhibited SMG IFN-γ concentra⁃tion,and down-regulated SMG TNF-α,IL-17A,CXCL9,CXCL13 and BAFF mRNA expression.TGP(360 mg·kg^-1)treat⁃ment decreased serum SSB/La and a-fodrin,and SMG TNF-α and IFN-γ concentration,and down-regulated SMG TNF-α,IL-17A,CXCL9 and BAFF mRNA expression.TGP(180 mg·kg^-1)treatment decreased serum SSB/La,a-fodrin,and SMG IL-1β concentration,and down-regulated SMG TNF-α,IL-17A and BAFF mRNA expression.After 8 weeks of administration,TGP(180,360,720 mg·kg^-1)treatment increased SMG index,and decreased serum a-fodrin concentra⁃tion.TGP(720 mg·kg^-1)treatment down-regulated mRNA expression of SMG TNF-α,IL-17A,CXCL9,CXCL13,and BAFF.TGP(360 mg·kg^-1)treatment reduced mRNA expression of TNF-α,CXCL9,CXCL13 and BAFF,and concentra⁃tion of IL-6 and TNF-α.TGP(180 mg·kg^-1)treatment down-regulated mRNA expression of TNF-α,CXCL9,and CXCL13,and decreased IL-6 and TNF-αconcentration in SMG.After 16 weeks of administration,TGP(180,360,720 mg·kg^-1)treatment reduced serum SSA/Ro and a-fodrin concentration,increased SMG index,and decreased SMG CXCL13 and BAFF mRNA expression.TGP(360,720 mg·kg^-1)treatment decreased serum SSB/Laconcentration and SMG TNF-α,IL-17A and CXCL9 mRNA expression.Besides,TGP(180,360,720 mg·kg^-1)treatment alleviated the pathological damage of SMG after 2 and 16 weeks of administration.CONCLUSION TGP has a certain therapeutic effect onmice through inhibiting inflammatory responses.
基金Supported by the Science and Technology Program for Basic Research in Shenzhen (No.JCYJ20200109140412476)Clinical Research Project of Shenzhen Second People’s Hospital in 2019 (No.20193357009)。
文摘The recognition of the profound impact of the human gastrointestinal microbiome(GM) on human autoimmune diseases has gradually increased thanks to deeper research efforts. As a systemic autoimmune disease, primary Sjogren’s syndrome(pSS) cannot be completely cured. Human studies have revealed that GM species and diversity are altered in patients with p SS compared with healthy individuals. Animal studies have provided possible mechanisms for the association between pSS and GM. The potential role of GM in pSS is exerted through several mechanisms. GM dysbiosis leads to increased intestinal permeability, which increases the risk of GM antigen exposure and activates specific autoreactive T lymphocytes via “molecular mimicry”. In addition, GM antigen exposure and intestinal immune tolerance loss caused by GM dysbiosis together induce chronic local gut mucosal inflammation, which deteriorates to systemic chronic non-specific inflammation with the circulation of pro-inflammatory lymphocytes and cytokines. These factors eventually activate autoreactive B lymphocytes and lead to pSS. If GM plays a key role in the pathogenesis of pSS, clarifying the underlying mechanisms will be helpful for the development of new therapies targeting GM for dry eye associated with pSS. This review summarizes the latest knowledge about the relationship between GM and p SS,with the aim of contributing to future research and to the development of new clinical applications.
基金Supported by a Research Fund from Chosun University Hospital,2018.
文摘BACKGROUND Sjogren’s syndrome(SS),which affect salivary gland function,is an autoimmune disease.SS may involve extraglandular organs.Approximately 10 to 20 percent of SS patients have clinically significant lung disease,but presentation of pulmonary amylodosis is extremly rare.The incidence of benign monoclonal gammopathy in SS patients is high,but multiple myeloma is rare.No case involving the simultaneous occurrence of two rare diseases,pulmonary amyloidosis and multiple myeloma,in the same patient with SS has been reported so far.CASE SUMMARY A 41-year-old male patient was referred to our hematology department due to incidentally detected gastric plasmacytoma.He had been diagnosed with SS four years earlier.Multiple miliary nodules,ground glass opacity in both lung fields,and enlargement of both inguinal lymph nodes was observed on chest and abdomen computer tomography.Based on the pathological findings of lung and lymph node biopsied specimens,the patient was diagnosed with pulmonary amyloidosis and multiple myeloma.Pulmonary amyloidosis and multiple myeloma associated with SS has rarely been reported.CONCLUSION This is an extremely rare case of simultaneous pulmonary amyloidosis and multiple myeloma in the same patient with SS.
文摘This paper reports the diagnosis of 65 patients with Sjogren′s Syndrome. Among these patients, 20 patients(30.77%) were complicated with oral candidiasis. The twenty patients were treated by topical nystatin smearing, 2% sodium bicarbonate solution rinsing, and transfer factor injecting for about 2 months. The soreness, erythema, unpleasant feeling, angular cheilitis in mouths significantly diminished or completely disappeared, following two months treatments. The good results have been kept 6 months after follow up. However, dry mouth and atrophic changes, though showed no furture development, the therapeutic response was not significant.
基金National Key R&D Program of China,No.2017YFC0110304.
文摘BACKGROUND Sj?gren syndrome(SS)is a chronic and systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands.And histoplasmosis is an invasive mycosis caused by the saprophytic dimorphic fungus H.capsulatum.In patients with primary SS(PSS),disseminated histoplasmosis(DH)is extremely rare.CASE SUMMARY We report a 37-year-old female patient admitted to our hospital with exacerbating fatigue,somnolence,and pancytopenia as the main symptoms.She was eventually diagnosed with DH based on pancytopenia,splenomegaly,and findings of bone marrow smears.The atypical clinical symptoms made the diagnosis process more tortuous.Unfortunately,she died of respiratory failure on the day the diagnosis was confirmed.CONCLUSION We present a rare and interesting case of DH in a PSS patient.This case updates the geographic distribution of histoplasmosis in China,and expands the clinical manifestations of DH in PSS,highlighting the significance of constantly improving the understanding of PSS with DH.
文摘Objective To evaluate the saliva ferning test (SFT) as diagnostic test for xerostomia in patients with Sjogren’s Syndrome (SS). Methods In this study, dried samples of freshly produced saliva from 78 patients with established SS according to European Community criteria and 80 healthy controls were examined by light microscopy. The crystallization was classified into 4 types according to the ferning phenomenon: uniformity, branching, spreading and integrity (type I normal and type Ⅱ,Ⅲ,Ⅳ abnormal ). Then, the 78 patients underwent lebial salivary gland biopsy. According to Tarpley’s classifica- tion, minor salivary gland biopsy (≥2 + ) was considered to be positive . Results 1. The sensitivity of SFT was high (70/ 78 = 89. 74% ). And the specificity wes also high (67/80 = 83. 75% ). 2. Abnormal SFT was observed in 70/78 (89. 74% ) samples from patients group and in 13 /80 (16. 25% ) samples from healthy controls. The differences of SFT in patients group versus controls were statistically significant (P< 0. 01 ). 3. The sensitivity of SFT and mini labial gland biopsy had no significant differences (P > 0. 05) as diagnostic tests in SS. Conclusion SFT was simple, sensitive and specific as diagnostic test in SS suspect patients just as mini labial gland biopsy.
基金supported by a grant from the Education Department of the Basque Country Government to the UPV/EHU(IT656-13).
文摘Susceptibility to pneumococcal infections in Sj?gren syndrome (SS)―an autoimmune inflammatory disease―patients is not well known, although these patients frequently develop respiratory diseases. The relative risk of developing pneumococcal disease in SS patients versus diabetes mellitus type 2 (DM-2) patients, matched by age, gender, and length of enrolment was studied. From January 1998 to September 2013 the records of Donostia University Hospital were analyzed, which among other patient’s data includes: number and type of hospital admissions and number and type of laboratory determinations. Streptococcus pneumoniae isolates of the same serotype causing recurrent infections were characterized by PFGE. The study comprised 127 patients in the SS group (69 primary and 58 secondary) and 127 in the DM-2 group as control. In 12 SS patients, (9.4%) 22 pneumococcal disease episodes were detected. Two patients (1.6%) with a single episode each one were observed among DM-2 patients, p = 0.01, RR for SS patients 6 (95% CI 1.4 to 26.3). No differences could be demonstrated between the two groups of patients in infections caused by Staphylococcus aureus or Streptococcus agalactiae. Most pneumococcal serotypes in SS patients belonged to the 13-valent (50%) and 23-valent (75%) anti-pneumococcal vaccine. SS patients are associated with and increased risk of suffering from pneumococcal disease. Vaccination should be considered in this group of patients.
基金Traditional Chinese Medicine—Guangxi first-class discipline(No.Gui Jiao Ke Yan[2018]12)Development Program of High-level Talent Team under Qihuang Project of Guangxi University of Chinese Medicine(No.2018005).
文摘Background:Sjögren syndrome(SS)is a long-term autoimmune disease that affects the body’s moisture-producing glands.It can occur independently of other health problems(primary disease)or as a result of another connective tissue disorder(secondary disease).Medication is directed at the person’s symptoms.Recently,it is reported that prescription of Chinese medicine has a great effect on SS and reduce adverse drugs reaction,which can provide a more safer treatment to SS.Case presentation:We here report a 43 year-old Chinese patient with continuous immune thrombocytopenia diagnosed as Sjögren syndrome,who was not response to routine medication such as Methylprednisolone and acyclovir.After that,the patient was treated by prescription of Chinese medicine,and the symptoms were improved apparently.We suppose that prescription of Chinese medicine has an unexpected effect on SS,which can alleviate patient’s symptoms.Conclusion:The clinical efficacy of Sjogren syndrome(SS)is effective in the treatment of prescription of Chinese medicine.It can improve patient’s symptoms and reduce adverse drug reactions.
文摘Salts of calcium phosphate and inorganic phosphate are normally found in serum and extracellular fluids, balancing through poorly understood factors that prevent abnormal tissue deposition of these minerals. However, in those tissues that are injured, especially due to chronic inflammatory processes, a predisposition to the deposition of these minerals is developed, triggering what has been called Dystrophic Calcinosis (DC), common in different Connective Tissue Diseases (CTD), especially dermatomyositis and scleroderma, but there is no a frecuent association with diseases like Rheumatoid Arthritis (RA) and Sj?gren Syndrome (SS). We report a case of a female patient of 63 years old with RA and Secundary SS who presents with DC in the hands and no evidence of other connective tissue.
文摘We report a 9-year-old Japanese girl who presented with muscle weakness and elevated serum levels of muscle-derived enzymes following mycoplasma infection. Rhabdomyolysis or myositis was suggested by magnetic resonance imaging and repeated four times within 4 years. Each episode developed following respiratory infection and spontaneously recovered. The diagnosis of Sjogren’s syndrome was made by decreased salivary secretion, MR sialography, lip biopsy, and positive anti-SSA/Ro antibody. Given the rarity of rhabdomyolysis/myositis, recurrent episode could be induced by infectious disease on the basis of underlying Sjogren’s syndrome. Conclusion: Sjogren’s syndrome should be considered as an underlying disease of recurrent infection-induced rhabdomyolysis/ myositis.
文摘Hypokalemic acidosis can complicate a primary Sjögren’s syndrome. The clinical feature is rarely revealed by manifestations due to hypokalemia. We report the case of a 46-year-old woman, admitted to explore a paresthesia and paresis of inferior limbs. The diagnosis of Sjögren’s syndrome was retained since there was the association of xerophthalmia, sialadenitis at the labial biopsy and positive immunological results (anti-SSA and anti-SSB). The absence of another auto-immune or systemic illness allowed us to consider that the Sjögren’s syndrome was primary. The biological explorations revealed a hyperchloremic and hypokalemic acidosis. The treatment was based on corticosteroid and potassium supplementation. The follow-up was marked by a clinical and biological amelioration.
文摘Mesenteric Panniculitis is a benign fibro-inflammatory process involving adipose tissue of the mesentery. It is characterised by fat necrosis, chronic inflammation and fibrosis, causing thickening and shortening of the mesentery. Patients may present with localised abdominal pain, abdominal mass, intestinal obstruction and ischaemic colitis. We report a case of mesenteric panniculitis causing abdominal pain in a patient with active Primary Sjogren’s Syndrome. The rarity of this case makes it of interest. We review the current literature on mesenteric panniculitis and its association with connective tissue disease and inflammatory conditions. A 64-year-old Caucasian male presented in 1994 with dry mouth. A diagnosis of Primary Sjogren’s Syndrome (PSS) was made on salivary gland biopsy. In 2010 he presented with an exacerbation of his eye symptoms, muscle pain and fatigue. He complained of abdominal pain and night sweating, but denied any weight loss or change in bowel habit. There was no significant past medical history other than PSS. On examination he had a small right submandibular node and mild synovitis at the right proximal interphalangeal joint and carpometacarpal joint. Examination of the abdomen showed marked umbilical tenderness but no organomegally. Blood tests at this time showed an active inflammation: CRP of 61 (NR 5 mg/L), ESR 39 (NR 20 mm/s), strongly positive ENA Ro and La. IgG was elevated at 18.6 (NR 5.8-15.4), Complement was low at 0.17 (NR 0.18-0.6). An abdominal ultrasound scan demonstrated a 6 × 3 ×3 cmarea of diffuse homogenous fat encasing some mesenteric vessels in the area of focal tenderness. CT abdomen and pelvis showed oedematous mesenteric fat and lymph nodes in the jejunal small bowel mesentery, consistent with mesenteric panniculitis. Laparoscopic biopsy was discussed with the surgical team, but was felt not indicated as risk outweighed potential benefit. The patient was treated with a 9-week reducing course of oral steroids. His abdominal symptoms resolved although CT abdomen showed little improvement in mesenteric panniculitis. A review of the literature suggests that currently there is no standard treatment and management should be guided by patient symptoms. Mesenteric Panniculitis is rare;as a result evidence for treatment is limited to individual case reports. There is no clear link between symptom improvement and radiological resolution of mesenteric panniculitis. It has, therefore been suggested that follow-up imaging should be limited to those with persistent symptoms. Overall the prognosis for mesenteric panniculitis is good, up to half of patients do not require treatment, and recurrence of symptoms is uncommon.
文摘Tubulointerstitial nephritis complicated by primary Sj?gren’s syndrome in a patient under treatment for type 2 diabetes mellitus was diagnosed in the early stage of the disease by renal biopsy. The symptoms of primary Sj?gren’s syndrome, such as thirst and polydipsia, were masked by the characteristic symptoms of type 2 diabetes mellitus. An association between sicca symptoms and diabetes mellitus (types 1 and 2) has been previously reported. Hence, it is possible that there are common underlying immunological mechanisms between primary Sj?gren’s syndrome and diabetes mellitus of both types. Intervention with steroids in a timely manner appears to have prevented or slowed the progression of re- nal impairment.
文摘Sjogren’s syndrome is a chronic autoimmune exocrinopathy associated with dry eyes and dry mouth as major clinical manifestations. It is characterized by lymphocytic infiltration of lacrimal and salivary glands and autoantibody production, especially anti-Ro (or SSA) and anti-La (or SSB). Lymphoproliferative disorders are a feature of Sjogren’s syndrome, and can be considered an extraglandular manifestation of the disease. Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare form of lymphadenitis. It is reported more often in young adult women with localized lymphadenopathy (usually cervical), fever, rashes, and leukopenia. It is a self-limiting disease with resolution within 1 - 4 months in almost all patients. Sjogren’s syndrome has been reported in patients with other systemic diseases including SLE and lymphomas. Here we present a patient with Kikuchi-Fujimoto disease who developed Sjogren’s Syndrome 8 years after her diagnosis of Kikuchi-Fujimoto disease.
文摘Interleukin-2(IL-2)is an important cytokine that plays a key role in the immune response.The IL-2 receptor(IL-2R)is composed of three subunits,alpha,beta,and gamma,with the alpha subunit having the highest affinity for IL-2.Several studies reported that immune dysregulation of IL-2 may cause tissue injury as well as damage leading to the pathogenesis of various autoimmune diseases such as acute necrotizing vasculitis in systemic lupus erythematosus(SLE),inflammatory synovitis in rheumatoid arthritis(RA),salivary and lacrimal gland dysfunction in Sjogren syndrome(SS),obliterative vasculopathy fibrosis in systemic sclerosis(SSc),and inflammatory demyelination in multiple sclerosis(MS).The aim of this review paper was to examine the role of IL-2/IL-2R in various autoimmune disorders,taking into account recent advancements and discoveries,gaps in the current literature,ongoing debates,and potential avenues for future research.The focus of this review is on systemic lupus erythematosus,rheumatoid arthritis,systemic sclerosis,sjogren syndrome,and multiple sclerosis,which are all linked to the malfunctioning of IL-2/IL-2R.In genetic studies,gene polymorphisms of IL-2 such as IL-2330/T,IL-2330/G,and rs2069763 are involved in increasing the risk of SLE.Furthermore,genetic associations of IL-2/IL-2R such as rs791588,rs2281089,rs2104286,rs11594656,and rs35285258 are significantly associated with RA susceptibility.The IL-2 polymorphism including rs2069762A,rs6822844T,rs6835457G,and rs907715T are significant connections with systemic sclerosis.In addition,rs2104286(IL-2),rs11594656(IL-2RA),rs35285258(IL-2RB)gene polymorphism significant increases the risk of multiple sclerosis.In therapeutic approaches,low-dose IL-2 therapy could regulate Tfr and Tfh cells,resulting in a reduction in disease activity in the SLE patients.In addition,elevated sIL-2R levels in the peripheral blood of SLE patients could be linked to an immunoregulatory imbalance,which may contribute to the onset and progression of SLE.Consequently,sIL-2R could potentially be a target for future SLE therapy.Moreover,Low dose-IL2 was well-tolerated,and low levels of Treg and high levels of IL-21 wereassociated with positive responses to Ld-IL2 suggested to be a safe and effective treatment for RA.Additionally,low-dose IL-2 treatment improves the exocrine glands'ability to secrete saliva in SS-affected mice.Whereas,Basiliximab targets the alpha chain of the IL-2 receptor suggested as a potential treatment for SSc.Also,pre-andpost-treatment with Tregs,MDSCs,and IL-2 may have the potential to prevent EAE induction in patients with MS.It is suggested that further studies should be conducted on IL-2 polymorphism in Sjogren syndrome.