Renal Vein Thrombosis Suggestive of Extramembranous Glomerulonephritis Associated with Sjögren’s Syndrome (Case Report)

Introduction: Glomerular damage during Gougerot-Sjgren syndrome is much rarer than interstitial damage, and is essentially extra-membranous and membrano-proliferative glomerulonephritis. Observation: We report the case of a 44-year-old woman with primary Sjgrens syndrome, confirmed by clinical dryness syndrome, positive anti-SSA and anti-SSB antibodies, and a salivary gland biopsy revealing grade 4 lymphocytic sialadenitis according to CHISHOLMs classification. Later, the patient developed nephrotic syndrome, along with hypertension. Renal function remained normal with a creatinine level of 9.3 mg/l, and hematuria was absent. Only antinuclear antibodies tested positive, while anti-PLA2R antibodies were negative. A renal biopsy was performed, which was complicated on the same day by hemodynamic instability with hematuria. Renal CT scan with contrast injection revealed a posterior perirenal hematoma without contrast extravasation. Additionally, bilateral renal vein thrombosis was incidentally discovered, suggesting extramembranous glomerulonephritis. The patients hemodynamic status stabilized after fluid resuscitation with isotonic saline solution (0.9%), without the need for blood transfusion. Renal biopsy confirmed extramembranous glomerulonephritis with interstitial fibrosis and minimal tubular atrophy. The initial etiological assessment was negative. The patient was started on oral corticosteroids, angiotensin-converting enzyme inhibitors, and therapeutic anticoagulation for renal vein thrombosis. The patients condition improved, with the disappearance of the syndrome and spontaneous regression of the hematoma. Discussion: The association of nephrotic syndrome and renal vein thrombosis primarily suggests glomerulopathy, in particular extra-membranous glomerulonephritis. Sjgrens syndrome can be associated with extra-membranous glomerulonephritis without being its direct cause. Like, it is possible that it is a cause of glomerulonephritis, essentially extra membranous and membrano-proliferative. Conclusion: Sjgrens syndrome is generally underestimated cause of glomerulonephritis, which should be considered in cases of extra-membranous glomerulonephritis.

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.

New-onset primary Sjögren's syndrome following exposure to severe acute respiratory syndrome coronavirus 2: A retrospective cohort study

Background:Understanding the clinical implications of autoimmune manifestations associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is essential to reduce its consequences. This study was aimed at determining the activities of new-onset primary Sjögren syndrome (pSS) since the emergence of SARS-CoV-2.Methods:This retrospective cohort study included data from 471 participants with dry mouths and eyes who had been attending Nanjing Drum Tower Hospital since December 2019. By April 2023, patients diagnosed with pSS were sequentially assigned to vaccinated group ( n = 24) or vaccinated and infected group based on exposure ( n = 20). Patients diagnosed with pSS within 3 months of vaccination against SARS-CoV-2 were assigned to a vaccinated group, and those who had been vaccinated and then developed pSS within 3 months of follow up after direct exposure to SARS-CoV-2 were assigned to a vaccinated and infected group. The controls comprised age- and sex-matched patients who had not been exposed to SARS-CoV-2 before December 2019 ( n = 21). We then compared data among the three groups. Results:The vaccinated and infected patients had more fever, malaise, splenomegaly, and weight loss before diagnosis and a higher European Alliance of Associations for Rheumatology Sjögren's syndrome disease activity index at the time of onset than the other two groups. Vaccinated patients had a higher frequency of anti-nuclear antibody (ANA) titers > 1:320 ( n = 12;50%) and anti-phospholipid antibodies (aPL) ( n = 7;29%) than the controls. The frequency of anti-Ro/SSA antibodies (13, 65%), ANA titers > 1:320 ( n = 16;80%), and aPLs ( n = 7;29%) ( n = 5;25%) were all significantly higher in vaccinated patients with infection than those in the controls. Higher doses of glucocorticoids, cyclosporin A, and tacrolimus were administered to the vaccinated and infected than the vaccinated and control groups ( p < 0.05, for all). Conclusions:Patients with new-onset pSS and a history of vaccination and SARS-CoV-2 infection might have more active disease. Further strengthening the assessment of people with a clear history of SARS-CoV infection and the monitoring of potential populations for autoimmune screening should not be overlooked.

SIL1基因变异所致常染色体隐性Marinesco-Sj?gren综合征2例

Marinesco-Sj gren综合征(MSS)是一种罕见的常染色体隐性遗传病,以小脑共济失调、早发型白内障、慢性肌病、不同程度的智力残疾和运动发育迟缓为特征。患者还可表现出身材矮小、促性腺激素过低和肌肉无力导致的骨骼畸形等症状。本文报道2例表现为全面发育迟缓的MSS患儿临床与分子诊断过程,分别检出SIL1基因复合杂合致病变异c.109delG(p.Glu37Serfs*4)、意义未明变异c.353G>C(p.Arg118Thr)和c.443delA(p.Lys148Argfs*10)、可能致病变异c.707A>G(p.Asn236Ser)。明确分子诊断后,予以遗传咨询,评估患儿父母再生育风险。本文通过典型病例报道及既往文献回顾,以期提高临床医生对该疾病的认识,并为该病的诊断提供借鉴。

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.

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.

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: Sj&#246;gren’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-Sj&#246;gren’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-Sj&#246;gren’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-Sj&#246;gren’s syndrome is an autoimmune exocrinopathy that may present with peripheral neuropathy, which may precede the diagnosis of Sj&#246;gren’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。

原发性干燥综合征患者唾液胱抑素D水平与唾液腺损伤的相关性分析

目的探讨原发性干燥综合征(primary Sj gren syndrome,pSS)患者唾液胱抑素D水平与唾液腺损伤的相关性。方法纳入2022年9月1日至2023年6月30日就诊于西安交通大学第一附属医院风湿免疫科pSS组患者51例及年龄、性别匹配的来院健康体检对照组人员51例。检测唾液中胱抑素D水平,采用独立样本t检验评估两组胱抑素D水平的差异,采用Pearson相关性检验评估pSS组胱抑素D与临床指标的关系。结果pSS组唾液中胱抑素D水平明显低于对照组(206.55±108.11 vs 374.32±172.24 pg/mL,P<0.01)。pSS患者胱抑素D水平与其静态唾液流率(r=0.433,P=0.002)及动态唾液流率(r=0.363,P=0.009)呈正相关。高唾液腺超声检查评分的pSS患者唾液胱抑素D水平明显低于低评分患者(腮腺:160.75±85.56 vs 290.53±95.17 pg/mL,P<0.01;颌下腺:157.76±87.59 vs 276.25±97.06 pg/mL,P<0.01)。pSS患者胱抑素D水平与外周血IL-6水平(r=-0.453,P=0.001)及CD4+T细胞计数(r=-0.396,P=0.005)呈负相关。结论pSS患者唾液胱抑素D水平可作为反映唾液腺损伤的指标。

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。

基于“阴火理论”探析干燥综合征口腔溃疡辨治思路

口腔溃疡是干燥综合征患者常见的口腔黏膜病变,易反复发作,令人痛苦不堪。干燥综合征患者口腔溃疡多是由于脾胃虚弱、阴津不足、火热上炎、瘀毒蕴结而导致与阴火的发病机制以及临床表现有相通之处,可从阴火理论治口腔溃疡,故而提出“调补脾胃、兼顾先天”“滋阴润燥、养血生津”“降泻阴火、升提清阳”“行气活、化瘀解毒”的治疗方法,以便临床应用,促进疾病向愈。

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。

从五脏论治干燥综合征

干燥综合征是全身性疾病,治疗要从整体出发,重视脏腑,尤其是五脏的辨治。早期应重在从肺胃治标为主,以润肺燥、清胃热为大法;中期多从肺脾论治,或养肺胃,或补脾土;后期多从肝肾论治,或滋肝肾,或温肾元。治肺以养阴润燥、宣肺布散为主,治脾宜补气健脾、气复津还,治肾补肾滋阴、资水生津,治肝当抑木清肝、疏肝活血,治心多以养心清火、宁神定志为法。该文旨在介绍从五脏论治干燥综合征的常用治法。

白芍总苷胶囊联合艾拉莫德片对原发性干燥综合征患者的临床疗效

目的考察白芍总苷胶囊联合艾拉莫德片对原发性干燥综合征患者的临床疗效。方法80例患者随机分为对照组和观察组,每组40例,对照组给予艾拉莫德片,观察组在对照组基础上加用白芍总苷胶囊,疗程3个月。检测临床疗效、中医证候评分、ESSDAI、ESSPRI、外分泌腺功能指标(泪腺分泌量、自然唾液流率)、IgG、CD4^(+)、CD8^(+)、Th17/Treg、IL-17、TNF-α、不良反应发生率变化。结果观察组总有效率高于对照组(P<0.05)。治疗后,2组中医证候评分、ESSDAI、ESSPRI、IgG、CD8^(+)、Th17/Treg、IL-17、TNF-α降低(P<0.05),外分泌腺功能指标、CD4^(+)升高(P<0.05)。2组不良反应发生率比较,差异无统计学意义(P>0.05)。结论白芍总苷胶囊联合艾拉莫德片可安全有效地调节原发性干燥综合征患者T淋巴细胞水平和Th17/Treg平衡,降低IgG水平,改善免疫功能和临床症状,抑制机体炎症,提高外分泌腺功能。

原发性干燥综合征恶性肿瘤死亡的风险分析——多中心、前瞻性队列研究

目的探讨原发性干燥综合征(primary Sj9gren’s Syndrome,pSS)患者发生恶性肿瘤死亡的风险。方法纳入2016年5月至2022年1月在国家风湿病数据中心(Chinese Rheumatism Data Center,CRDC)注册登记、符合pSS 2002年欧美共识小组分类标准或2016年美国风湿病学会/欧洲风湿病联盟分类标准的pSS患者8932例的pSS患者,对临床资料(包括基本人口统计学特征、病程、临床表现、自身免疫抗体、血清补体水平和欧洲风湿病学会干燥综合征疾病活动指数(ESSDAI)评分等指标)进行随访分析,评估因实体肿瘤或血液系统肿瘤导致死亡的风险及风险影响因素。结果共纳入8932例pSS患者,中位随访时间为48.0(32.1,72.0)月。随访期间56例患者发生恶性肿瘤并死亡,粗肿瘤死亡率为0.6%,标准化死亡比(SMR)为0.841(95%CI:0.636~1.093),标准化肿瘤死亡率为0.5%。46例(82.1%)患者因实体肿瘤死亡,10例(17.9%)因血液系统肿瘤死亡。发生恶性肿瘤死亡的危险因素包括年龄增加(SHR=1.056,95%CI:1.028~1.085,P<0.001)、血小板计数减少(SHR=3.982,95%CI:2.123~7.471,P<0.001)、ESSDAI评分中肺高活动度(SHR=5.291,95%CI:1.827~15.328,P=0.002)。免疫抑制治疗治疗为保护因素(SHR=0.348,95%CI:0.164~0.741,P=0.006)。首位实体肿瘤死因为肺癌,共16例,占恶性肿瘤比例为28.6%,粗肺癌死亡率为0.2%,SMR 1.284(95%CI:0.734~2.085),标准化肿瘤死亡率为0.2%。发生肺癌死亡的危险因素包括男性(HR=8.356,95%CI:2.676~26.100,P<0.001)、年龄增加(HR=1.102,95%CI:1.053~1.153,P<0.001)、ESSDAI评分高度肺活动性(2 vs.0分:HR=7.041,95%CI:2.233~22.208,P=0.001,3 vs.0分:HR=17.349,95%CI:3.593~83.764,P<0.001)。羟氯喹治疗为其保护因素(HR=0.213,95%CI:0.069~0.663,P=0.008)。结论pSS患者因恶性肿瘤死亡的风险与普通人群无明显差异。发生恶性肿瘤死亡事件的危险因素包括高龄、血小板计数减少、ESSDAI评分中肺高度活动性;免疫抑制治疗可以降低总体肿瘤死亡风险。肺癌死亡的危险因素为男性、年龄增加、ESSDAI评分中肺高活动度,羟氯喹降低肺癌死亡风险。