• 文章类型: Case Reports
    背景:胸腺粘膜相关淋巴组织(MALT)淋巴瘤罕见,已知与干燥综合征(SjS)相关。SjS很少伴有浆膜炎。这里,我们描述了首例与SjS相关的胸腺MALT淋巴瘤患者术后心脏压塞和急性胸膜炎。
    方法:一名33岁女性患者在胸部计算机断层扫描中表现为前纵隔肿块,这是为了进一步检查病情。怀疑胸腺MALT淋巴瘤或胸腺上皮肿瘤,进行了胸腺全切除术.纵隔肿块在组织病理学上被诊断为胸腺MALT淋巴瘤。患者术后出院,病程良好,但术后30天因呼吸困难就诊。观察到心脏填塞并进行引流。心包引流4天后,胸部X线检查显示左侧大量胸腔积液,并进行胸腔引流。该患者被诊断为与SjS相关的浆膜炎,并接受甲基强的松龙治疗,缓解了心脏压塞和胸膜炎。
    结论:与SjS相关的胸腺MALT淋巴瘤的手术侵袭可能导致浆膜炎。术后应进行随访,考虑到由于浆膜炎引起的心脏填塞或急性胸膜炎作为术后并发症的可能性。
    BACKGROUND: Thymic mucosa-associated lymphoid tissue (MALT) lymphoma is rare and is known to be associated with Sjögren\'s syndrome (SjS). SjS is rarely accompanied by serositis. Here, we describe the first case of postoperative cardiac tamponade and acute pleuritis in a patient with thymic MALT lymphoma associated with SjS.
    METHODS: A 33-year-old woman with SjS presented with an anterior mediastinal mass on chest computed tomography, which was performed for further examination of the condition. Suspecting a thymic MALT lymphoma or thymic epithelial tumor, total thymectomy was performed. The mediastinal mass was histopathologically diagnosed as a thymic MALT lymphoma. The patient was discharged with a good postoperative course but visited the hospital 30 days after surgery for dyspnea. Cardiac tamponade was observed and drainage was performed. Four days after pericardial drainage, chest radiography revealed massive left pleural effusion, and thoracic drainage was performed. The patient was diagnosed with serositis associated with SjS and treated with methylprednisolone, which relieved cardiac tamponade and pleuritis.
    CONCLUSIONS: Surgical invasion of thymic MALT lymphomas associated with SjS may cause serositis. Postoperative follow-up should be conducted, considering the possibility of cardiac tamponade or acute pleuritis due to serositis as postoperative complications.
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  • 文章类型: Journal Article
    背景:干眼病(DED)是由代谢综合征和炎症增加引起的血脂异常(DLP)的并发症。这项研究旨在评估白细胞和全身性炎症指数比率作为干眼症(DLP-DED)血脂异常患者全身性炎症的潜在生物标志物。
    方法:在32例DLP-DED患者(研究组)和63例仅DLP患者(对照组)中研究了几种血液生物标志物。评估的血液生物标志物包括特定的全身炎症指数比率,例如中性粒细胞与淋巴细胞比率(NLR),血小板与淋巴细胞比率(PLR),单核细胞与淋巴细胞比率(MLR),中性粒细胞与淋巴细胞和血小板比率(NLPR),和脂质分布,如总胆固醇(TC),高密度脂蛋白胆固醇(HDL),低密度脂蛋白胆固醇(LDL),甘油三酯(TG),白蛋白(ALB),和C反应蛋白(CRP)水平。
    结果:DLP-DED组的淋巴细胞水平明显高于仅DLP组(P=0.044)。此外,HDL与NLPR之间的显著负相关(P=0.007;r=-0.428)和血清ALB浓度与PLR之间的显著负相关(P=0.008;r=-0.420)被鉴定为DLP-DED的潜在炎症预测因子.
    结论:这项研究的结果表明,DLP-DED患者可能从血常规监测其升高的血脂和血液炎症生物标志物中受益。如CRP,白细胞,和全身炎症指数比率(NLR,PLR,MLR,和NLPR),减少DLP对眼部健康的并发症。相关数据表明,NLPR,PLR,血清ALB浓度,和血清HDL浓度可能是DLP-DED患者有价值的炎症生物标志物。需要更多的研究来确定NLR的重要性,PLR,MLR,和NLPR以及白细胞发挥的累加作用。
    BACKGROUND: Dry eye disease (DED) is a complication of dyslipidemia (DLP) that is caused by metabolic syndrome and increased inflammation. This research aimed to assess leukocyte and systemic inflammation index ratios as potential biomarkers for systemic inflammation in dyslipidemia patients with dry eye disease (DLP-DED).
    METHODS: Several blood biomarkers were studied in 32 patients with DLP-DED (study group) and 63 patients with DLP-only (control group). The evaluated blood biomarkers included specific systemic inflammation index ratios, such as the neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), monocyte-to-lymphocyte ratio (MLR), and neutrophil-to-lymphocyte and platelet ratio (NLPR), and lipid profiles, such as total cholesterol (TC), high-density lipoprotein cholesterol (HDL), low-density lipoprotein cholesterol (LDL), triglyceride (TG), albumin (ALB), and C-reactive protein (CRP) levels.
    RESULTS: Lymphocyte levels were significantly greater in the DLP-DED group than in the DLP-only group (P = 0.044). In addition, a significant negative correlation between HDL and the NLPR (P = 0.007; r= -0.428) and a significant negative correlation between the serum ALB concentration and the PLR (P = 0.008; r= -0.420) were identified as potential inflammatory predictors of DLP-DED.
    CONCLUSIONS: The findings of this study suggest that patients with DLP-DED may benefit from routine blood monitoring of their elevated lipid profile and blood inflammatory biomarkers, such as CRP, leukocytes, and systemic inflammation index ratios (NLR, PLR, MLR, and NLPR), to reduce the complications of DLP on ocular health. The correlation data suggest that the NLPR, PLR, serum ALB concentration, and serum HDL concentration may be valuable inflammatory biomarkers in DLP-DED patients. More research is required to ascertain the significance of the NLR, PLR, MLR, and NLPR and the additive role that leukocytes play.
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  • 文章类型: Journal Article
    结论:体内稳态的丧失和慢性炎症导致干眼的眼表损害,在确定的病例中,这也与角膜变薄有关。然而,角膜厚度与新的干眼病例之间的相关性仍然没有得到足够的证据支持.
    目的:本研究旨在比较新发干眼病例与年龄和性别匹配的对照组的中央角膜厚度。
    方法:将45例干眼症患者与61例年龄和性别匹配的非干眼症患者进行比较。眼表疾病指数(OSDI)问卷用于评估症状,用NidekCEM-530镜面反射显微镜测量中央角膜厚度。根据疾病严重程度(OSDI评分)对患者进行分组,两组间比较裂隙灯检查的临床表现,Schirmer\'sItest,和泪膜破裂时间。
    结果:患者的中位年龄为25.0(四分位距[IQR],20.0至32.0)和27.0(IQR,对照组和干眼症组20.0至32.0)年,分别(p=0.63)。OSDI分数的中位数(IQR)值,泪膜破裂时间分数,对照组的Schirmer测试测量值为10.4(8.3至10.4),12.0(11.0到14.0)秒,和16.0(13.5至19.5)毫米,分别,这与干眼症组不同(p<0.0001)。干眼症组的这些值是29.1(25.0至39.5),4.0(3.0到8.0)秒,和8.0(3.5至11.0)毫米,分别。干眼患者的中央角膜厚度低于对照组(p<0.01)。干眼症患者和对照组的平均±标准差中央角膜厚度分别为520.3±26.8和545.3±18.8μm,分别。
    结论:干眼症的中央角膜厚度与对照组相比明显降低。这些发现可能有助于监测和管理干眼,应在眼内压测量和屈光手术中予以考虑。
    CONCLUSIONS: Loss of homeostasis and chronic inflammation result in ocular surface damage in dry eyes, which is also associated with corneal thinning in established cases. Yet, the correlation between corneal thickness and new cases of dry eyes remains inadequately supported by evidence.
    OBJECTIVE: This study aimed to compare the central corneal thickness of new cases of dry eyes to that of age- and gender-matched controls.
    METHODS: A total of 45 dry eye patients were compared with 61 age- and gender-matched non-dry eye individuals. The Ocular Surface Disease Index (OSDI) questionnaire was used to evaluate symptoms, and the central corneal thickness was measured with a Nidek CEM-530 specular microscope. Patients were grouped based on disease severity (OSDI scores), and the clinical findings were compared between groups for slit-lamp examinations, Schirmer\'s I test, and tear film breakup time.
    RESULTS: The median age of patients was 25.0 (interquartile range [IQR], 20.0 to 32.0) and 27.0 (IQR, 20.0 to 32.0) years in the control and dry eye groups, respectively (p=0.63). The median (IQR) values of the OSDI scores, tear film breakup time scores, and Schirmer\'s test measurements in the control groups were 10.4 (8.3 to 10.4), 12.0 (11.0 to 14.0) seconds, and 16.0 (13.5 to 19.5) mm, respectively, which differed from the dry eye groups (p<0.0001). These values in the dry eye group were 29.1 (25.0 to 39.5), 4.0 (3.0 to 8.0) seconds, and 8.0 (3.5 to 11.0) mm, respectively. Patients with dry eyes had lower central corneal thickness than controls (p<0.01). The mean ± standard deviation central corneal thicknesses in patients with dry eyes and the control group were 520.3 ± 26.8 and 545.3 ± 18.8 μm, respectively.
    CONCLUSIONS: The central corneal thickness in dry eyes was significantly reduced compared with the control group. These findings may be useful in monitoring and managing dry eyes and should be considered in intraocular pressure measurements and refractive surgical procedures.
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  • 文章类型: Case Reports
    暂无摘要。
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  • 文章类型: Journal Article
    背景:血栓性血小板减少性紫癜(TTP)是一种罕见的血栓性微血管病,由vonWillebrand因子裂解蛋白酶(ADAMTS13)的活性降低引起,可能会危及生命.本病例研究中报告的患者还并发干燥综合征和肾功能损害,表现出多种症状,对治疗构成巨大挑战。
    方法:一名25岁妇女在产后8天前剖宫产后,由于意识淡漠超过1天,到医院就诊。
    方法:血小板显著减少,血红蛋白,肌酐,和ADAMTS13水平。经过眼科医生的咨询检查,她被诊断为右眼视网膜出血和双眼干眼综合征。
    方法:被诊断为TTP伴Sjögren综合征和肾功能损害,她接受血浆置换联合利妥昔单抗的反复治疗.
    结果:治疗后和随访期间,患者的血小板计数和出血症状明显改善。
    结论:TTP死亡率高,当合并干燥综合征和肾功能损害时,它对治疗提出了更大的挑战。然而,在给予标准血浆置换联合利妥昔单抗治疗后,治疗效果良好。
    BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy caused by reduced activity of the von Willebrand factor-cleaving protease (ADAMTS13), which can be life-threatening. The patient reported in this case study also had concurrent Sjögren syndrome and renal impairment, presenting multiple symptoms and posing a great challenge in treatment.
    METHODS: A 25-year-old woman in the postpartum period visited the hospital due to indifference in consciousness for more than 1 day following cesarean section 8 days prior.
    METHODS: Notable decreases were observed in platelets, hemoglobin, creatinine, and ADAMTS13 levels. After a consultative examination by an ophthalmologist, she was diagnosed with retinal hemorrhage in the right eye and dry eye syndrome in both eyes.
    METHODS: Having been diagnosed with TTP with Sjögren syndrome and renal impairment, she received repeated treatments with plasmapheresis combined with rituximab.
    RESULTS: Following treatment and during the follow-up period, the patient\'s platelet counts and bleeding symptoms significantly improved.
    CONCLUSIONS: TTP has a high mortality rate, and when combined with Sjögren syndrome and renal impairment, it poses an even greater challenge in treatment. However, after administering standard plasmapheresis combined with rituximab treatment, the treatment outcome is favorable.
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  • 文章类型: Journal Article
    The study presents an analysis of the diagnostic and treatment protocol for a patient with a first episode of nasopharyngeal carcinoma who also has Sjogren\'s syndrome and Epstein-Barr Virus (EBV) positive cerebrospinal fluid, as detected through metagenomic next-generation sequencing (mNGS). It reviews existing literature to examine the connections between EBV and various conditions including Sjogren\'s syndrome, encephalitis or meningitis, and nasopharyngeal carcinoma, emphasizing the importance of EBV positive cerebrospinal fluid. The study focuses on a case from the Eighth Medical Center of the General Hospital of the People\'s Liberation Army, where a patient was admitted with headaches as the primary symptom on March 3, 2021. This patient had a history of Sjogren\'s syndrome and was later diagnosed with nasopharyngeal carcinoma. The research involved reviewing both domestic and international databases for cases related to cerebrospinal fluid EBV positive encephalitis or meningitis, and nasopharyngeal carcinoma. It aimed to aggregate data on demographics, initial symptoms, treatment methods, and patient outcomes. Findings suggest that positive cerebrospinal fluid EBV is linked to autoimmune diseases, viral encephalitis or meningitis, and nasopharyngeal carcinoma, albeit infrequently in the context of Sjogren\'s syndrome. Notably, EBV positive cerebrospinal fluid is commonly associated with recurrent nasopharyngeal carcinoma rather than initial episodes. The study concludes that for patients with an immune condition, exhibiting symptoms like headaches or cranial nerve issues, or in cases where nasopharyngeal carcinoma is suspected, early testing through cerebrospinal fluid mNGS or EBV DNA is recommended. This approach facilitates risk assessment, prognosis determination, and the creation of individualized treatment plans.
    通过回顾2021年3月3日解放军总医院第八医学中心宏基因组学第二代测序技术(mNGS)检测到脑脊液EBV阳性合并干燥综合征的初发鼻咽癌患者1例的诊治过程,检索国内外数据库有关脑脊液EBV阳性的脑炎或脑膜炎、鼻咽癌相关报道,对所有患者的一般资料、首发症状、治疗及预后进行分析。脑脊液EBV阳性与自身免疫病、病毒性脑炎或脑膜炎、鼻咽癌相关,合并干燥综合征少见;脑脊液EBV阳性多在复发鼻咽癌患者中出现,初发鼻咽癌患者脑脊液EBV阳性鲜见报道。对于具有免疫背景、临床以头痛或颅神经受累为主要表现,或者疑诊鼻咽癌的患者,宜尽早行脑脊液mNGS或EBV DNA检测以评估危险分层,判断预后,制定个性化的治疗方案。.
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  • 文章类型: Case Reports
    背景:怀孕会引起重大的生理变化,包括电解质平衡和肾功能的改变。这一点尤其重要,因为某些疾病可能会恶化,并使人们更容易受到电解质异常的影响。其中一种是干燥综合征(SS),可引起远端肾小管酸中毒(dRTA)的自身免疫性疾病。本病例报告为怀孕期间复杂的生理相互作用提供了独特的视角,强调识别和管理电解质异常的关键重要性,特别是在自身免疫性疾病的背景下,如干燥综合征。
    方法:我们报告一例31岁的印度孕妇,妊娠24周时出现发热,胃肠道症状,进行性四肢轻瘫,然后是感觉神经改变。严重的低钾血症和呼吸性酸中毒需要立即插管和通气支持。调查显示低钾血症,正常阴离子间隙代谢性酸中毒,和SS的自身免疫标志物阳性。同时,她的IgM钩端螺旋体检测呈阳性.管理涉及积极纠正电解质失衡并解决潜在的SS和钩端螺旋体病。
    结论:该案例强调,及时识别和管理对于预防自身免疫性疾病孕妇的危及生命的并发症至关重要。本报告揭示了在妊娠期干燥综合征的背景下管理低钾性四肢轻瘫的独特挑战。
    BACKGROUND: Pregnancy imposes significant physiological changes, including alterations in electrolyte balance and renal function. This is especially important because certain disorders might worsen and make people more susceptible to electrolyte abnormalities. One such condition is Sjogren\'s syndrome (SS), an autoimmune disease that can cause distal renal tubular acidosis (dRTA). This case report offers a unique perspective on the intricate physiological interplay during pregnancy, emphasizing the critical importance of recognizing and managing electrolyte abnormalities, particularly in the context of autoimmune disorders such as Sjogren\'s syndrome.
    METHODS: We report a case of a 31-year-old pregnant Indian woman at 24 weeks gestation presenting with fever, gastrointestinal symptoms, and progressive quadriparesis followed by altered sensorium. Severe hypokalaemia and respiratory acidosis necessitated immediate intubation and ventilatory support. Investigations revealed hypokalaemia, normal anion gap metabolic acidosis, and positive autoimmune markers for SS. Concurrently, she tested positive for IgM Leptospira. Management involved aggressive correction of electrolyte imbalances and addressing the underlying SS and leptospirosis.
    CONCLUSIONS: This case underscores that prompt recognition and management are paramount to prevent life-threatening complications in pregnant patients with autoimmune disease. This report sheds light on the unique challenge of managing hypokalaemic quadriparesis in the context of Sjogren\'s syndrome during pregnancy.
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  • 文章类型: Systematic Review
    在各种自身免疫性疾病中,针对Ku的抗体的频率各不相同。在2019年,Spielmann等人。基于层次聚类分析确定了两种类型的抗Ku综合征。干燥综合征既发生在第一类型的抗Ku综合征中,也发生在第二类型的抗Ku综合征中。尽管在原发性干燥综合征患者的小唾液腺局灶性唾液腺炎的淋巴细胞中观察到Ku蛋白的组织表达增加,文献中仅描述了49例抗Ku抗体和干燥综合征的表现。一些研究人员检查患者是否存在干燥综合征,只有当他们有抗Ro或抗La抗体时,虽然在文学中,在仅存在分离的抗Ku抗体的情况下,有干燥综合征的描述,就像我们的情况一样。关于抗Ku阳性患者干燥综合征的腺体和腺体外表现的文献数据有限。下面,我们介绍了第一例Sjögren综合征与第一类抗Ku综合征并发粘膜相关淋巴组织(MALT)淋巴瘤的病例。本文还提供了有关干燥综合征与抗Ku抗体关联的文献的系统综述。
    The frequency of antibodies to Ku varies in various autoimmune diseases. In 2019, Spielmann et al. identified two types of anti-Ku syndrome based on a hierarchical clustering analysis. Sjögren\'s syndrome occurs both in the first type of anti-Ku syndrome and in the second type. Despite the fact that increased tissue expression of Ku proteins was noted in lymphocytic cells with focal sialoadenitis of the minor salivary glands in patients with primary Sjogren\'s syndrome, only 49 cases of a combination of anti-Ku antibodies and manifestations of Sjogren\'s syndrome have been described in the literature. Some researchers examined patients for the presence of Sjogren\'s syndrome only if they had anti-Ro or anti-La antibodies, although in the literature, there are descriptions of Sjogren\'s syndrome in the presence of only isolated anti-Ku antibodies, as in our case. Literature data on glandular and extraglandular manifestations of Sjögren\'s syndrome in anti-Ku-positive patients are limited. Below, we present the first case of Sjögren\'s syndrome in combination with the first type of anti-Ku syndrome complicated by the development of mucosa-associated lymphoid tissue (MALT) lymphoma. The article also provides a systematic review of the literature on the association of Sjögren\'s syndrome with anti-Ku antibodies.
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  • 文章类型: Review
    Rosai-Dorfman病(RDD)是一种罕见的非朗格汉斯细胞组织细胞增生症,其特征是巨大的淋巴结病和系统性结外病变。我们介绍了一名28岁女性的病例,她的右眼复发性视力模糊3个月。在演讲前十年,她的左眼出现了失明和萎缩。她随后出现头痛,发烧,和精神状态受损。头颅磁共振成像提示肥厚性硬脑膜炎(HP),和18F-氟-2-脱氧-2-d-葡萄糖(FDG)正电子发射断层扫描/计算机断层扫描显示,左侧硬脑膜吸收了大量FDG。自身免疫测试显示抗核反应升高,反SS-A,和抗SS-B抗体水平。萎缩性眼球的切开活检显示RDD具有明显的多克隆浆细胞增多症。患者被诊断为RDD并伴有多系统受累,包括干燥综合征(SS),全葡萄膜炎,和HP。用甲基强的松龙治疗数周导致显著改善。这是首例报告的RDD,伴有SS并伴有全葡萄膜炎和HP。尽管RDD很少在年轻患者中被诊断出,跨学科合作对于防止延迟诊断至关重要。
    Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by massive lymphadenopathy and systemic extranodal lesions. We present the case of a 28-year-old woman who presented with recurrent blurred vision in her right eye for 3 months. She developed blindness and atrophy in her left eye a decade prior to presentation. She subsequently developed headache, fever, and impaired mental status. Cranial magnetic resonance imaging indicated hypertrophic pachymeningitis (HP), and 18F-fluoro-2-deoxy-2-d-glucose (FDG) positron emission tomography/computed tomography revealed significant FDG uptake in the left dura mater. Autoimmune testing revealed elevated anti-nuclear, anti-SS-A, and anti-SS-B antibody levels. Incisional biopsy of the atrophic eyeball revealed RDD with marked polyclonal plasmacytosis. The patient was diagnosed with RDD accompanied by multisystem involvement, including Sjögren\'s syndrome (SS), panuveitis, and HP. Treatment with methylprednisolone for several weeks resulted in significant improvement. This is the first reported case of RDD presenting with SS in combination with panuveitis and HP. Although RDD is rarely diagnosed in young patients, interdisciplinary collaboration is essential to prevent a delayed diagnosis.
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  • 文章类型: Case Reports
    背景:本报告介绍了一名诊断为原发性干燥综合征的患者的独特病例和相对罕见的中医模式,被称为寒热结合模式和寒湿综合症。使用中草药成功治疗了患者的病情,特别是加味的大柴胡汤和灵桂术甘汤。
    方法:一名56岁的女性患有慢性干眼和口腔超过10年。她最初是用中草药(TCHM)处方管理的,包括增液汤,但治疗效果并不令人满意。随着疾病的发展,由于烦恼和失眠的症状,她被诊断出患有焦虑症。阿普唑仑和文拉法辛治疗未能缓解这些症状。最近,她的一般情况逐渐恶化,症状包括嘴里有苦味,头晕,潮热,发冷,食欲不振,胸部不适,还有便秘.
    方法:经过一系列检查,包括Schirmer试验和唇腺活检,她被诊断出患有干燥综合征。
    方法:尽管常规使用毛果芸香碱治疗,透明质酸钠滴眼液,文拉法辛,和阿普唑仑,口干症状加剧。因此,她通过TCHM寻求进一步干预。
    结果:加味大柴胡汤加减灵桂术甘汤治疗8周后,她报告干燥相关症状和睡眠质量显著改善.
    结论:本病例报告显示TCHM可有效治疗原发性干燥综合征,并应考虑更广泛的应用。此外,这强调了通过在临床环境中确定患者的特定辨证来为患者量身定制治疗方案的重要性。
    BACKGROUND: This report presents a unique case of a patient diagnosed with Primary Sjögren\'s syndrome and a relatively rare traditional Chinese medicine pattern, known as the combined cold and heat pattern and cold-dampness syndrome. The patient\'s condition was successfully managed using Chinese herbal medicine, specifically the modified Da-Chai-Hu decoction and Linggui Zhugan decoction.
    METHODS: A 56-year-old woman had chronic dry eye and mouth for over 10 years. She was initially managed with traditional Chinese herbal medicine (TCHM) prescriptions, including the Zengye decoction, but the therapeutic effects were unsatisfactory. As the disease progressed, she was diagnosed with an anxiety disorder due to symptoms of vexation and insomnia. Treatment with alprazolam and venlafaxine failed to alleviate these symptoms. Recently, her general condition gradually worsened, with symptoms including a bitter taste in her mouth, dizziness, hot flashes, chills, poor appetite, chest discomfort, and constipation.
    METHODS: After a series of examinations, including a Schirmer test and labial gland biopsy, she was diagnosed with Sjögren\'s syndrome.
    METHODS: Despite regular treatment with pilocarpine, sodium hyaluronate eye drops, venlafaxine, and alprazolam, the dry mouth symptoms intensified. Consequently, she sought further intervention through the TCHM.
    RESULTS: After 8 weeks of treatment with the modified Da-Chai-Hu decoction and Linggui Zhugan decoction, she reported a significant improvement in her dryness-related symptoms and sleep quality.
    CONCLUSIONS: This case report demonstrates that TCHM can effectively treat Primary Sjögren\'s syndrome, and should be considered for broader applications. Furthermore, this underscores the importance of tailoring treatment formulas to patients by identifying their specific syndrome differentiation in a clinical setting.
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