Role of ranulas in early diagnosis of Sjögren’s syndrome: A case report

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.

Iguratimod in treatment of primary Sjögren’s syndrome concomitant with autoimmune hemolytic anemia:A case report

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.

Celiac disease and Sjögren’s syndrome:A case report and review of literature

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.

Cutaneous mucosa-associated lymphoid tissue lymphoma complicating Sjögren's syndrome:A case report and review of literature

BACKGROUND The association of Sjögren's syndrome(SS)and lymphoma is similar.Mucosaassociated lymphoid tissue(MALT)or extranodal marginal zone B-cell lymphoma was the most common lymphomatous histology in SS patients.MALT in SS patients is frequently located in the parotid gland,while MALT lymphoma of the skin with SS is an exceedingly rare entity that needs to be recognized.CASE SUMMARY A 60-year-old woman presented with a 3-year history of progressive dry mouth associated with a 1-year history of enlarging cutaneous nodules.Physical examination revealed two hard subcutaneous nodules on her right lower leg.The results of Schirmer’s test were positive,despite the absence of dry eyes.Labial salivary gland biopsy revealed lymphocytic infiltration and chronic inflammation with a focus score of 2.The patient was diagnosed with SS.She underwent resection of one cutaneous nodule,and histopathological analysis identified the nodule as MALT lymphoma.Her dry mouth symptoms improved,and the nodules decreased after 6 mo of treatment with hydroxychloroquine sulfate and chemotherapy(thalidomide,cyclophosphamide,and dexamethasone).CONCLUSION Lymphoma is a severe complication of SS,shown by the reported unique case of cutaneous MALT lymphoma with SS.

Enzalutamide Associated with Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) Overlap: A Case Report

Enzalutamide is a hormonal therapy that blocks the action of androgens, such as testosterone in the treatment of metastatic castration-resistant prostate cancer. Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) overlap and are part of an adverse drug reaction continuum of disease, in which there is a 10% - 30% involvement of the skin surface with mucositis, blisters, skin slough, and a macular rash. A 66-year-old male was treated with enzalutamide for metastatic prostate cancer and developed SJS/TEN overlap with 25% total body surface area skin involvement. The patient received a seven-day course of cyclosporine to which he responded by re-epithelialization but succumbed to multi-organ failure. While SJS/TEN has been reported with apalutamide, to our knowledge, this is the first case of SJS/TEN overlap with enzalutamide.

AA Amyloidosis Secondary to Primary Sjögren Syndrome: Can It Be Developed without Chronic Inflammation?

Background: The association of primary Sjögren syndrome (PSS) and AA amyloidosis is a rare occurrence. Objective: To describe the phenotype of patients with this association through our two cases and a literature review. Materials and methods: A report of two cases of AA amyloidosis complicating primary Sjögren syndrome with a literature review. Results: Eight patients of Primary Sjögren’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. Conclusion: 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.

Peripheral Neuropathies Revealing Gougerot-Sjögren’s Syndrome: Description of 3 Cases

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.

Sjögren’s Syndrome Revealed by Obstructive Renal Failure: A Case Report and Review of the Literature

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.

原发性干燥综合征合并血细胞减少中医诊治研究进展

原发性干燥综合征(pSS)是一种慢性炎症性自身免疫病,除累及外分泌腺之外,还可累及多器官、系统,其中以血液系统受累最为常见,多表现为一系或多系血细胞减少。目前对于pSS合并血细胞减少缺乏统一公认的治疗方案,西医治疗多以糖皮质激素、免疫抑制剂为主,而中医药治疗本病具有较好疗效。通过总结pSS合并血细胞减少的中医诊治经验,并对相关研究进行综述后发现,现代医家认为pSS核心病机为气阴亏虚,久之则产生热、毒、瘀等多种病理产物,相互影响,导致全身气血津液失常,引起血细胞减少。本病病位多责之于肝、脾、肾,治以滋阴、益气、养血之法,辅以清热解毒、化瘀通络之法,并根据其合并血细胞减少类型的不同以及是否使用糖皮质激素用药各有侧重。中医药治疗本病较西药治疗能够更好地改善血细胞水平且不良事件发生率更低,具有一定优势。

五运六气与干燥综合征发病特点及中医证候的相关性研究

目的:探讨原发性干燥综合征(pSS)患者出生时的五运六气等相关信息与pSS发病所累及的系统损害及中医证候的相关性。方法:采用回顾性研究方法,从门诊及住院部收集2020年1月—2021年12月就诊的pSS患者,收集其出生时的运气学相关信息资料,统计运、气因素出现的频次,分析其与pSS发病所累及的系统损害及中医证候相关的运气因素,并运用中医理论进行分析、总结、探讨。结果:岁运因素中“土不及”是pSS患者的易患禀赋因素;岁运因素中“土不及”、六气因素中的“太阴司天、太阳在泉”及“厥阴司天、少阳在泉”是pSS气阴两虚证的易患禀赋因素;岁运因素中“土不及”“木太过”“金太过”“水太过”“水不及”,六气因素中“少阴司天、阳明在泉”及“少阳司天、厥阴在泉”为阴虚津亏证pSS的易患禀赋因素;岁运因素中“火太过”,六气因素中“太阴司天、太阳在泉”“少阴司天、阳明在泉”是阴虚热毒证pSS的易患禀赋因素;岁运因素中“木太过”“金不及”“木不及”,六气因素中“厥阴司天、少阳在泉”为阴虚血瘀证pSS的易患禀赋因素;岁运因素中“土不及”“金不及”,六气因素中“太阴司天、太阳在泉”为pSS患者关节受累的易患禀赋因素;岁运因素中“土不及”“金不及”为pSS患者肺受累的易患禀赋因素;岁运因素中“火太过”“土太过”“土不及”,六气因素中“厥阴司天、少阳在泉”为pSS患者血液系统受累的易患禀赋因素。结论:原发性干燥综合征的发病特点及其中医证候与五运六气具有一定的相关性。

原发性干燥综合征并肝损害13例临床特征及中医证型分析

目的:分析原发性干燥综合征并肝损害临床特征及中医证型状况,为早期改善患者预后提供参考。方法:选择2018年9月至2022年1月广州中医药大学东莞医院风湿科诊治的原发性干燥综合征患者72例作为研究对象,调查、记录患者的电子病历系统,随访患者的预后,重点记录临床特征及中医证型。结果:72例患者中肝损害13例,占比18.1%,女性13例,临床上首发主要症状以口干、眼干为主。肝损害以GGT、ALP升高为主,ALT、AST升高不明显,13例患者均出现抗核抗体(ANA)阳性,中医分型判定为肝郁脾虚型6例、脾肾不足型4例、气阴两虚型2例、阴虚津亏型1例。13例患者经过中西医结合治疗后都顺利出院,2~3周后复查肝功能恢复至正常。结论:临床医师应对原发性干燥综合征肝损害、自身免疫性肝病的异同有充分认识,分析各种实验室检查结果,及早明确诊断、分析中医证型并给予相应治疗方案。

汪龙德运用疏肝健脾法从脾胃论治干燥综合征经验

介绍汪龙德主任医师运用疏肝健脾法从脾胃论治干燥综合征的临床经验和学术思想。认为干燥综合征的关键病机是肝郁脾虚,发病部位在肝脾,属虚实夹杂证,临证强调运用疏肝健脾法为基本治法,使肝气畅达,疏泄正常,气行血行,则瘀血渐除,气机布散津液于全身;脾胃健运,气血津液化生有源,痰饮水湿自化,则清窍腠理得以濡润。辅以清热润燥、养血生津、活血祛瘀,标本同治,可获良效。并结合案例,介绍了疏肝健脾法治疗干燥综合征的应用,以期为临床诊疗提供参考。

Fas、FasL在Sjgren综合征涎腺组织中的表达及意义

目的 观察Sj gren综合征 (Sj gren’ssyndrome,SS)涎腺组织和正常涎腺组织中凋亡相关蛋白Fas、FasL的表达情况 ,探讨SS涎腺组织中细胞凋亡的途径。方法 采用SP免疫组织化学法 ,检测 2 3例SS涎腺组织和 16例正常涎腺组织中Fas、FasL的表达情况。结果 在SS腺泡上皮细胞中 ,Fas、FasL的表达均高于正常组 ,有显著性差异 (P <0 .0 0 5 ) ;两组导管上皮细胞中 ,Fas、FasL的表达均无显著性差异 (P >0 .0 5 )。结论 在SS涎腺组织中Fas、FasL的表达升高。SS涎腺上皮细胞在Fas/FasL介导下过度凋亡 。

Sjgren综合征涎腺组织中雌、雄激素受体的表达

目的 :探讨Sj gren综合征 (Sj gren’ssyndrome ,SS)与性激素的关系 ,以期为临床开展内分泌治疗提供理论依据。方法 :采用免疫组织化学法 ,检测 2 8例SS涎腺组织和 19例正常涎腺组织中雌激素受体 (ER)、雄激素受体 (AR)的表达情况。结果 :正常组与SS组ER的阳性率分别为 5 7.89%和 75 .0 0 % ,无显著性差异(P >0 .0 5 ) ;AR的阳性率分别为 84.2 1%和 5 0 .0 0 % ,有显著性差异 (P <0 .0 5 ) ;ER和AR主要位于腺泡和导管上皮细胞胞核内 ,而淋巴细胞仅偶见染色 ;SS唇腺、腮腺、颌下腺、舌下腺组织中 ,二者都为弱阳性到阳性染色 ,表达情况未见明显差异 (P >0 .0 5 )。结论 :SS涎腺病变与局部雄激素作用降低或丧失有关 ,雌、雄激素通过相应受体直接作用于涎腺上皮细胞 。

实例探讨药物引起SJS或TEN的评分方法——Naranjo评分与ALDEN评分比较

药物引起的史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)是一种罕见严重的不良反应,其特征是广泛性的皮肤表皮剥离和黏膜侵犯。当评估可疑药物和SJS或TEN之间的因果关系时,除了Naranjo评分外,最近根据两个大型的病例对照研究结果所衍生的ALDEN评分也提供了新思路。本文结合1例同时服用对乙酰氨基酚和头孢拉定导致中毒性表皮坏死综合征的案例,应用ALDEN评分及Naranjo评分分析两种药物与ADR间的关联性,并讨论此两种评分方法的差异性及适宜性。

腮腺造影唇腺活检对Sjgren综合征的诊断价值

对100例综合征患者进行腮腺造影及唇腺活检、组织病理学检查，87例患者显示腮腺末梢导管扩张性改变；90例显示局灶性淋巴细胞浸润，符合郑麟蕃氏Ⅱ级，59例符合Chisholm4级。结果提示了疾病中两种腺体损害的一致性，也证实了损害的不均衡性。本研究提示了一种联合观察腮腺造影和唇腺活检结果诊断涎腺损害的新方法。

唾液钠、钾离子浓度和单位时间的总量及pH值变化与Sjgren综合征的关系

【目的】测定舍格伦综合征 (Sj grensyndrome,SS)患者唾液钠 (Na+ )、钾离子 (K+ )浓度和单位时间的总量及pH变化并探讨其与SS之间的关系。【方法】分组 :①正常对照组 (A组 ,n =30 ) ,风湿免疫组病人分为SS组 (B组 ,n =30 )和非SS组 (C组 ,n =2 4 ) ,单纯性口干组 (D组 ,n =15 ) ,共 4组。②采集各组唾液标本 ,应用离子选择电极电位测定分析法测定其Na+ 、K+ ,并对单位时间分泌总量进行观察 ,测pH值。③分别用t检验和单因素方差分析进行组间比较。【结果】B组Na+ 明显高于其他三组 (P <0 .0 0 1) ;B组K+ 虽高于A组 (P <0 .0 1) ,但与其他两组无显著差异。Na+ 总量B组与其他三组之间差异无显著性 ,但是C组和D组则低于A组 (P <0 .0 1) ;B组、C组、D组的Na+ 总量明显低于A组 (P <0 .0 1) ,B组与C组比较显著降低 (P <0 .0 0 1)。【结论】SS主要损害外分泌腺 ,因涎腺受累而导致唾液电解质浓度变化 ,这一现象可作为疾病的判定指标之一 ,对其诊断及与其他免疫疾病的鉴别诊断有一定价值。

中药人工泪液治疗Sjgren综合征患者干眼的疗效观察

目的 观察中药人工泪对Sj gren综合征患者的干眼治疗效果。 方法 80例有干眼症状的Sj gren综合征患者随机分成 2组 ,第一组用环胞霉素A点眼治疗 ,第二组用环胞霉素A加中药人工泪Ⅰ号滴眼剂治疗。记录治疗前后双眼SchirmerI试验纸湿长度和角膜虎红染色点数的变化。结果 治疗 3周和 5周后的第二组SchirmerI试验纸湿长度和虎红角膜染色点数均比第一组明显改善。结论 中药人工泪液能改善Sj gren综合征患者的干眼体征。

IgG4相关性疾病和原发性干燥综合征相关细胞因子谱的对比

目的:从分子水平探讨IgG4相关性疾病(IgG4-RD)和原发性干燥综合征(pSS)的相关细胞因子特征。方法:获得了17个小唾液腺(9个来自pSS患者,8个来自非pSS患者作为对照)、7个颌下腺(4个来自IgG4-RD患者,3个来自切除的颌下腺肿瘤周围的肉眼正常组织作为对照)的样本RNA-Seq数据,分析IgG4-RD和pSS来源样品中的差异表达基因和富集途径,以揭示这两种疾病的免疫炎症特征。结果:尽管三级淋巴结构(TLS)相关细胞因子(例如CXCL13、CCL19等)的表达显著增加,但对于受累组织的先天免疫反应特征,在IgG4-RD中观察到更显著成纤维细胞相关细胞因子及其受体的表达,巨噬细胞和中性粒细胞相关的细胞因子及其受体在pSS中有更为显著的表达。此外,IgG4-RD受累唾液腺中经典信号通路的富集模式也与pSS不同:在IgG4-RD中,JAK-STAT信号通路、NF-κB信号通路显著富集,而在pSS中,与细胞趋化性相关通路显著富集。结论:在IgG4-RD和pSS之间的转录水平上,相关细胞因子间有不同的分子和生物过程模式,这可能指导其后续的机制研究和药物靶点选择。

Sjgren综合征中T细胞亚群值的测定

目的：研究Ｓｊｏｇｒｅｎ综合征中Ｔ细胞亚群和免疫球蛋白的变化。方法：应用单克隆抗体技术检测Ｔ细胞亚群，用单向免疫扩散法检测ＩｇＡ、ＩｇＧ、ＩｇＭ结果：Ｓｊｏｇｒｅｎ综合征患者外周血中 ＣＤ４降低（Ｐ＜０．０５）、ＣＤ８升高（Ｐ＜０．０５），其程度与病情发展相一致，严重者ＣＤ４／ＣＤ８倒置。ＩｇＡ、ＩｇＧ、ＩｇＭ均升高（Ｐ＜０．０５），尤以ＩｇＧ显著（Ｐ＜０．０１）。结论：Ｓｊｏｇｒｅ综合征中存在免疫调节异常。