BACKGROUND Although the presentations of Sjögren’s syndrome(SS)are variable,ranging from mild dryness to wider systemic involvement,ranulas as early clinical signs were scarcely reported.Here,we present an adult...BACKGROUND Although the presentations of Sjögren’s syndrome(SS)are variable,ranging from mild dryness to wider systemic involvement,ranulas as early clinical signs were scarcely reported.Here,we present an adult patient with SS,who developed a unilateral simple ranula and was diagnosed primary SS 3 years later.We also provide a review of cases of SS and ranulas from 1980 to 2020.CASE SUMMARY A 22-year-old girl was found to have a left painless floor-of-mouth lesion 3 years ago,without obvious trauma or inducement.The diagnosis of a unilateral(left)simple ranula was made,and the ranula was surgically treated.Within 3 years after the ranula surgery,she developed acute lymphadenectasis in unilateral parotid twice without inducement,and ultrasonic examination revealed diffuse lesions in bilateral parotids and submandibular glands,which strongly suggested SS.Serologic tests and the unstimulated whole saliva flow rate confirmed the SS diagnosis.CONCLUSION Our study underlines that ranulas are early clinical signs of SS.As early diagnosis and early intervention of SS are important to obtain better outcomes,our findings underline the need for histopathological test after sublingual adenectomy and imaging detection of exocrine glands for the patients with ranulas.展开更多
BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.Ho...BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.However,there are few reports about the application of IGU in pSS concomitant with AIHA.CASE SUMMARY Here,we present the case of a patient with pSS concomitant with AIHA but without eye and mouth dryness.The patient was initially diagnosed with hyperplastic anemia and AIHA while pSS was missed,and was finally diagnosed with pSS concomitant with AIHA.The patient was treated with IGU along with prednisone and hydroxychloroquine,and her hemoglobin,reticulocytes and IgG returned to normal levels.CONCLUSION IGU was effective for and well tolerated by our patient with pSS concomitant with AIHA,and may be a promising therapy for the treatment of this disease.展开更多
BACKGROUND Celiac disease(CD)is a systemic,chronic immune-mediated disease triggered by gluten ingestion in genetically-susceptible individuals,with a prevalence of 1%worldwide.Sjogren's syndrome(SS)is also a syst...BACKGROUND Celiac disease(CD)is a systemic,chronic immune-mediated disease triggered by gluten ingestion in genetically-susceptible individuals,with a prevalence of 1%worldwide.Sjogren's syndrome(SS)is also a systemic autoimmune disease,mainly characterized by ocular and oral sicca symptoms and signs.Sharing a common genetic background,CD and SS are known associated autoimmune diseases,but currently available guidelines are not reporting it.CASE SUMMARY We report the case of a 39-year-old woman,who was in the care of her rheumatologist for 2 years with SS.On routine follow-up she was found to have iron deficiency,without anemia.She had no gastrointestinal complaints and denied any obvious source of blood loss.IgA tissue transglutaminase antibodies were positive and endoscopy with duodenal biopsies revealed crypt hyperplasia and villous atrophy.A diagnosis of CD was set and gluten-free diet was recommended.CONCLUSION We present a review of existing data in the literature regarding the association of the two diseases,summarizing prevalence studies of CD in SS patients and the other way around.Screening recommendations and future research perspectives are also discussed,highlighting clinically relevant unanswered questions with respect to the association of CD with SS.展开更多
Introduction: Sjögren’s syndrome is an autoimmune epithelitis with various extraglandular signs, among which are neurological, with a variable frequency according to studies. We report three cases of periphe...Introduction: Sjögren’s syndrome is an autoimmune epithelitis with various extraglandular signs, among which are neurological, with a variable frequency according to studies. We report three cases of peripheral neuropathy revealing Gougerot-Sjögren’s syndrome, collected in the Neurology Department of the Fann University Hospital in Dakar (Senegal). Observations: The first patient, aged 48 years, presented with a length-dependent sensitivomotor polyneuropathy associated with retrobulbar optic neuritis, with dry eyes and dry mouth noticed by the patient for several years. The second patient, aged 28 years, was admitted to the hospital with chronic generalized paresthesia in the context of xerostomia and xerophthalmia. The results of the clinical examination and the electroeneuromyogram were in favour of pure sensory neuronopathy. The third patient was 32 years old female, with a history of thyroidectomy and acute inflammatory demyelinating polyneuropathy (AIDP), who was seen for acute ascending flaccid tetraplegia with facial diplegia, preceded by diffuse paresthesia. The diagnosis of recurrence of acute demyelinating polyradiculonueropathy was retained in view of the rapidly increasing character of the deficit, the hyperproteinorachy at the lumbar puncture, and the signs of demyelination at the ENMG. The diagnosis of Gougerot-Sjögren’s syndrome in our three patients was established on the basis of the 2016 ACR/EULAR criteria. Indeed, the anti-SSA antibodies (Ro) were positive in our 3 patients with a biopsy of the salivary glands which showed stage 3 in the first patient and stage 4 in the two others. Corticosteroid therapy and immunosuppressive treatment resulted in a favourable clinical evolution on the neurological and general levels. Conclusion: Gougerot-Sjögren’s syndrome is an autoimmune exocrinopathy that may present with peripheral neuropathy, which may precede the diagnosis of Sjögren’s syndrome, be concomitant or occur during the course of the disease.展开更多
Introduction: Primary Sjögren’s syndrome (SS) is the most common connective tissue disease after rheumatoid arthritis and affects mostly women between 30 and 40 years of age with an estimated prevalence between ...Introduction: Primary Sjögren’s syndrome (SS) is the most common connective tissue disease after rheumatoid arthritis and affects mostly women between 30 and 40 years of age with an estimated prevalence between 0.1% and 0.6%. This observation illustrates an incidental finding of a case of SS in a young female patient in a context of obstructive renal failure (ARF) due to uterine fibroids. Observation: This was a 31-year-old woman hospitalized for anuric AKI (Acute Kidney Injury) with a creatinine level of 1247 μmol/l. Her history included sickle cell disease A/C and an unoperated uterine fibroid diagnosed 3 years ago. Approximately 2 months before her admission, her symptomatology was made of dizziness, physical asthenia, vomiting, poly-arthralgia, morning rash, pollakiuria and oral dryness. Abdominal examination showed a painless transverse mass in the pelvis. Biological examination showed a CRP (C-reactive protein) level of 488 mg/l. The cytobacteriological examination of the urine was normal and the proteinuria was 1.35 g/24 hours. The CT scan showed kidneys measuring 110 mm on the right and 113 mm on the left associated with bilateral pyelo-caliceal dilatation on a large polymyomatous uterus of interstitial and submucosal type. Immunologically, the anti-nuclear factor, the rheumatoid factor and the anti-SSA antibodies were positive. The resumption of the interrogation within the framework of the research of the subjective dry syndrome to find a notion of intermittent xerophthalmia 4 months ago. The Schirmer test was positive in the left eye. The initial management consisted of a polymyomectomy after 3 sessions of hemodialysis. Background treatment combining prednisone 5 mg/day and methotrexate 20 mg/week was started in parallel with the use of artificial tears. The evolution after twelve (12) months of treatment was favorable with a complete disappearance of the signs dry syndrome and full recovery of renal function. Conclusion: SS can have an insidious evolution and remain stable for many years, hence its fortuitous discovery in this case of obstructive ARF on uterine fibroid. In this context we insist on the interest of the immunological assessment in a patient in period of genital activity with a significant proteinuria and non-specific extrarenal signs.展开更多
<div style="text-align:justify;"> <span style="font-family:Verdana;"><strong>Background:</strong> The association of primary Sj<span style="white-space:nowrap;"...<div style="text-align:justify;"> <span style="font-family:Verdana;"><strong>Background:</strong> The association of primary Sj<span style="white-space:nowrap;">ö</span>gren syndrome (PSS) and AA amyloidosis is a rare occurrence. <strong>Objective: </strong>To describe the phenotype of patients with this association through our two cases and a literature review. <strong>Materials and methods:</strong> A report of two cases of AA amyloidosis complicating primary Sj<span style="white-space:nowrap;">ö</span>gren syndrome with a literature review. <strong>Results:</strong> Eight patients of Primary Sj<span style="white-space:nowrap;">ö</span>gren’s Syndrome complicated by AA amyloidosis were studies. Six cases were reported in the literature by consulting several databases. 50% of patients had a positive immunological assessment, three cases with kidney damage, and three cases lung damage. <strong>Conclusion: </strong>The immunological activity in the Primary Sjogren’s Syndrome requires the search not only a lymphoma but also AA amyloidosis apart from any clinical or biological chronic inflammation.</span> </div>展开更多
Primary Sjögren’s syndrome(pSS)is a systemic autoimmune disease with high prevalence and possible poor prognosis.Though the pathogenesis of pSS has not been fully elucidated,B cell hyperactivity is considered as...Primary Sjögren’s syndrome(pSS)is a systemic autoimmune disease with high prevalence and possible poor prognosis.Though the pathogenesis of pSS has not been fully elucidated,B cell hyperactivity is considered as one of the fundamental abnormalities in pSS patients.It has long been identified that Janus kinases-signal transducer and activator of transcription(JAK-STAT)signaling pathway contributes to rheumatoid arthritis and systemic lupus erythematosus.Recently,increasing numbers of studies have provided evidence that JAK-STAT pathway also has an important role in the pathogenesis of pSS via direct or indirect activation of B cells.Signal transducer and activator of transcription 1(STAT1),STAT3,and STAT5 activated by various cytokines and ribonucleic acid contribute to pSS development,respectively or synergically.These results reveal the potential application of Janus kinase inhibitors for treatment of pSS,which may fundamentally improve the quality of life and prognosis of patients with pSS.展开更多
Background:Sjögren’s syndrome(SS)is an autoimmune disorder characterized by sicca syndrome and/or systemic manifestations.The treatment is still challenging.This study aimed to explore the therapeutic role and m...Background:Sjögren’s syndrome(SS)is an autoimmune disorder characterized by sicca syndrome and/or systemic manifestations.The treatment is still challenging.This study aimed to explore the therapeutic role and mechanism of exosomes obtained from the supernatant of stem cells derived from human exfoliated deciduous teeth(SHED-exos)in sialadenitis caused by SS.Methods:SHED-exos were administered to the submandibular glands(SMGs)of 14-week-old non-obese diabetic(NOD)mice,an animal model of the clinical phase of SS,by local injection or intraductal infusion.The saliva flow rate was measured after pilocarpine intraperitoneal injection in 21-week-old NOD mice.Protein expression was examined by western blot analysis.Exosomal microRNA(miRNAs)were identified by microarray analysis.Paracellular permeability was evaluated by transepithelial electrical resistance measurement.Results:SHED-exos were injected into the SMG of NOD mice and increased saliva secretion.The injected SHED-exos were taken up by glandular epithelial cells,and further increased paracellular permeability mediated by zonula occluden-1(ZO-1).A total of 180 exosomal miRNAs were identified from SHED-exos,and Kyoto Encyclopedia of Genes and Genomes analysis suggested that the phosphatidylinositol 3 kinase(PI3K)/protein kinase B(Akt)pathway might play an important role.SHED-exos treatment down-regulated phospho-Akt(p-Akt)/Akt,phospho-glycogen synthase kinase 3b(p-GSK-3b)/GSK-3b,and Slug expressions and up-regulated ZO-1 expression in SMGs and SMG-C6 cells.Both the increased ZO-1 expression and paracellular permeability induced by SHED-exos were abolished by insulin-like growth factor 1,a PI3K agonist.Slug bound to the ZO-1 promoter and suppressed its expression.For safer and more effective clinical application,SHED-exos were intraductally infused into the SMGs of NOD mice,and saliva secretion was increased and accompanied by decreased levels of p-Akt/Akt,p-GSK-3b/GSK-3b,and Slug and increased ZO-1 expression.Conclusion:Local application of SHED-exos in SMGs can ameliorate Sjögren syndrome-induced hyposalivation by increasing the paracellular permeability of glandular epithelial cells through Akt/GSK-3b/Slug pathway-mediated ZO-1 expression.展开更多
基金Supported by the National Natural Science Foundation of China,No.81801600。
文摘BACKGROUND Although the presentations of Sjögren’s syndrome(SS)are variable,ranging from mild dryness to wider systemic involvement,ranulas as early clinical signs were scarcely reported.Here,we present an adult patient with SS,who developed a unilateral simple ranula and was diagnosed primary SS 3 years later.We also provide a review of cases of SS and ranulas from 1980 to 2020.CASE SUMMARY A 22-year-old girl was found to have a left painless floor-of-mouth lesion 3 years ago,without obvious trauma or inducement.The diagnosis of a unilateral(left)simple ranula was made,and the ranula was surgically treated.Within 3 years after the ranula surgery,she developed acute lymphadenectasis in unilateral parotid twice without inducement,and ultrasonic examination revealed diffuse lesions in bilateral parotids and submandibular glands,which strongly suggested SS.Serologic tests and the unstimulated whole saliva flow rate confirmed the SS diagnosis.CONCLUSION Our study underlines that ranulas are early clinical signs of SS.As early diagnosis and early intervention of SS are important to obtain better outcomes,our findings underline the need for histopathological test after sublingual adenectomy and imaging detection of exocrine glands for the patients with ranulas.
基金Supported by the Lanzhou Science and Technology Plan Project,No.2018-3-48Lanzhou Talent Innovation and Entrepreneurship Project,No.2019-RC-35and 2019 Special Fund for Doctoral Training of Lanzhou University Second Hospital,No.YJS-BD-15.
文摘BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.However,there are few reports about the application of IGU in pSS concomitant with AIHA.CASE SUMMARY Here,we present the case of a patient with pSS concomitant with AIHA but without eye and mouth dryness.The patient was initially diagnosed with hyperplastic anemia and AIHA while pSS was missed,and was finally diagnosed with pSS concomitant with AIHA.The patient was treated with IGU along with prednisone and hydroxychloroquine,and her hemoglobin,reticulocytes and IgG returned to normal levels.CONCLUSION IGU was effective for and well tolerated by our patient with pSS concomitant with AIHA,and may be a promising therapy for the treatment of this disease.
文摘BACKGROUND Celiac disease(CD)is a systemic,chronic immune-mediated disease triggered by gluten ingestion in genetically-susceptible individuals,with a prevalence of 1%worldwide.Sjogren's syndrome(SS)is also a systemic autoimmune disease,mainly characterized by ocular and oral sicca symptoms and signs.Sharing a common genetic background,CD and SS are known associated autoimmune diseases,but currently available guidelines are not reporting it.CASE SUMMARY We report the case of a 39-year-old woman,who was in the care of her rheumatologist for 2 years with SS.On routine follow-up she was found to have iron deficiency,without anemia.She had no gastrointestinal complaints and denied any obvious source of blood loss.IgA tissue transglutaminase antibodies were positive and endoscopy with duodenal biopsies revealed crypt hyperplasia and villous atrophy.A diagnosis of CD was set and gluten-free diet was recommended.CONCLUSION We present a review of existing data in the literature regarding the association of the two diseases,summarizing prevalence studies of CD in SS patients and the other way around.Screening recommendations and future research perspectives are also discussed,highlighting clinically relevant unanswered questions with respect to the association of CD with SS.
文摘Introduction: Sjögren’s syndrome is an autoimmune epithelitis with various extraglandular signs, among which are neurological, with a variable frequency according to studies. We report three cases of peripheral neuropathy revealing Gougerot-Sjögren’s syndrome, collected in the Neurology Department of the Fann University Hospital in Dakar (Senegal). Observations: The first patient, aged 48 years, presented with a length-dependent sensitivomotor polyneuropathy associated with retrobulbar optic neuritis, with dry eyes and dry mouth noticed by the patient for several years. The second patient, aged 28 years, was admitted to the hospital with chronic generalized paresthesia in the context of xerostomia and xerophthalmia. The results of the clinical examination and the electroeneuromyogram were in favour of pure sensory neuronopathy. The third patient was 32 years old female, with a history of thyroidectomy and acute inflammatory demyelinating polyneuropathy (AIDP), who was seen for acute ascending flaccid tetraplegia with facial diplegia, preceded by diffuse paresthesia. The diagnosis of recurrence of acute demyelinating polyradiculonueropathy was retained in view of the rapidly increasing character of the deficit, the hyperproteinorachy at the lumbar puncture, and the signs of demyelination at the ENMG. The diagnosis of Gougerot-Sjögren’s syndrome in our three patients was established on the basis of the 2016 ACR/EULAR criteria. Indeed, the anti-SSA antibodies (Ro) were positive in our 3 patients with a biopsy of the salivary glands which showed stage 3 in the first patient and stage 4 in the two others. Corticosteroid therapy and immunosuppressive treatment resulted in a favourable clinical evolution on the neurological and general levels. Conclusion: Gougerot-Sjögren’s syndrome is an autoimmune exocrinopathy that may present with peripheral neuropathy, which may precede the diagnosis of Sjögren’s syndrome, be concomitant or occur during the course of the disease.
文摘Introduction: Primary Sjögren’s syndrome (SS) is the most common connective tissue disease after rheumatoid arthritis and affects mostly women between 30 and 40 years of age with an estimated prevalence between 0.1% and 0.6%. This observation illustrates an incidental finding of a case of SS in a young female patient in a context of obstructive renal failure (ARF) due to uterine fibroids. Observation: This was a 31-year-old woman hospitalized for anuric AKI (Acute Kidney Injury) with a creatinine level of 1247 μmol/l. Her history included sickle cell disease A/C and an unoperated uterine fibroid diagnosed 3 years ago. Approximately 2 months before her admission, her symptomatology was made of dizziness, physical asthenia, vomiting, poly-arthralgia, morning rash, pollakiuria and oral dryness. Abdominal examination showed a painless transverse mass in the pelvis. Biological examination showed a CRP (C-reactive protein) level of 488 mg/l. The cytobacteriological examination of the urine was normal and the proteinuria was 1.35 g/24 hours. The CT scan showed kidneys measuring 110 mm on the right and 113 mm on the left associated with bilateral pyelo-caliceal dilatation on a large polymyomatous uterus of interstitial and submucosal type. Immunologically, the anti-nuclear factor, the rheumatoid factor and the anti-SSA antibodies were positive. The resumption of the interrogation within the framework of the research of the subjective dry syndrome to find a notion of intermittent xerophthalmia 4 months ago. The Schirmer test was positive in the left eye. The initial management consisted of a polymyomectomy after 3 sessions of hemodialysis. Background treatment combining prednisone 5 mg/day and methotrexate 20 mg/week was started in parallel with the use of artificial tears. The evolution after twelve (12) months of treatment was favorable with a complete disappearance of the signs dry syndrome and full recovery of renal function. Conclusion: SS can have an insidious evolution and remain stable for many years, hence its fortuitous discovery in this case of obstructive ARF on uterine fibroid. In this context we insist on the interest of the immunological assessment in a patient in period of genital activity with a significant proteinuria and non-specific extrarenal signs.
文摘<div style="text-align:justify;"> <span style="font-family:Verdana;"><strong>Background:</strong> The association of primary Sj<span style="white-space:nowrap;">ö</span>gren syndrome (PSS) and AA amyloidosis is a rare occurrence. <strong>Objective: </strong>To describe the phenotype of patients with this association through our two cases and a literature review. <strong>Materials and methods:</strong> A report of two cases of AA amyloidosis complicating primary Sj<span style="white-space:nowrap;">ö</span>gren syndrome with a literature review. <strong>Results:</strong> Eight patients of Primary Sj<span style="white-space:nowrap;">ö</span>gren’s Syndrome complicated by AA amyloidosis were studies. Six cases were reported in the literature by consulting several databases. 50% of patients had a positive immunological assessment, three cases with kidney damage, and three cases lung damage. <strong>Conclusion: </strong>The immunological activity in the Primary Sjogren’s Syndrome requires the search not only a lymphoma but also AA amyloidosis apart from any clinical or biological chronic inflammation.</span> </div>
基金supported by grants from the Chinese National Key Technology R&D Program,Ministry of Science and Technology(Nos.2017YFC0907601,2017YFC0907605)CAMS Innovation Fund for Medical Sciences(CIFMS)(No.2021-I2M-1-005)
文摘Primary Sjögren’s syndrome(pSS)is a systemic autoimmune disease with high prevalence and possible poor prognosis.Though the pathogenesis of pSS has not been fully elucidated,B cell hyperactivity is considered as one of the fundamental abnormalities in pSS patients.It has long been identified that Janus kinases-signal transducer and activator of transcription(JAK-STAT)signaling pathway contributes to rheumatoid arthritis and systemic lupus erythematosus.Recently,increasing numbers of studies have provided evidence that JAK-STAT pathway also has an important role in the pathogenesis of pSS via direct or indirect activation of B cells.Signal transducer and activator of transcription 1(STAT1),STAT3,and STAT5 activated by various cytokines and ribonucleic acid contribute to pSS development,respectively or synergically.These results reveal the potential application of Janus kinase inhibitors for treatment of pSS,which may fundamentally improve the quality of life and prognosis of patients with pSS.
基金supported by grants from the National Natural Science Foundation of China(Nos.81974151 and 81771088)Peking University-Tason Stomatology Development Fund.
文摘Background:Sjögren’s syndrome(SS)is an autoimmune disorder characterized by sicca syndrome and/or systemic manifestations.The treatment is still challenging.This study aimed to explore the therapeutic role and mechanism of exosomes obtained from the supernatant of stem cells derived from human exfoliated deciduous teeth(SHED-exos)in sialadenitis caused by SS.Methods:SHED-exos were administered to the submandibular glands(SMGs)of 14-week-old non-obese diabetic(NOD)mice,an animal model of the clinical phase of SS,by local injection or intraductal infusion.The saliva flow rate was measured after pilocarpine intraperitoneal injection in 21-week-old NOD mice.Protein expression was examined by western blot analysis.Exosomal microRNA(miRNAs)were identified by microarray analysis.Paracellular permeability was evaluated by transepithelial electrical resistance measurement.Results:SHED-exos were injected into the SMG of NOD mice and increased saliva secretion.The injected SHED-exos were taken up by glandular epithelial cells,and further increased paracellular permeability mediated by zonula occluden-1(ZO-1).A total of 180 exosomal miRNAs were identified from SHED-exos,and Kyoto Encyclopedia of Genes and Genomes analysis suggested that the phosphatidylinositol 3 kinase(PI3K)/protein kinase B(Akt)pathway might play an important role.SHED-exos treatment down-regulated phospho-Akt(p-Akt)/Akt,phospho-glycogen synthase kinase 3b(p-GSK-3b)/GSK-3b,and Slug expressions and up-regulated ZO-1 expression in SMGs and SMG-C6 cells.Both the increased ZO-1 expression and paracellular permeability induced by SHED-exos were abolished by insulin-like growth factor 1,a PI3K agonist.Slug bound to the ZO-1 promoter and suppressed its expression.For safer and more effective clinical application,SHED-exos were intraductally infused into the SMGs of NOD mice,and saliva secretion was increased and accompanied by decreased levels of p-Akt/Akt,p-GSK-3b/GSK-3b,and Slug and increased ZO-1 expression.Conclusion:Local application of SHED-exos in SMGs can ameliorate Sjögren syndrome-induced hyposalivation by increasing the paracellular permeability of glandular epithelial cells through Akt/GSK-3b/Slug pathway-mediated ZO-1 expression.
