Diabetes Insipidus, Neurogenic

尿崩症,神经性
  • 文章类型: Journal Article
    背景:术后中枢性尿崩症(CDI)通常见于颅咽管瘤(CP)患者,炎症反应在CPs中起重要作用。我们旨在评估术前外周炎症标志物及其组合对CPsCDI发生的预测价值。
    方法:回顾性分析208例接受手术治疗的CP患者的临床资料,包括术前外周炎症指标。术前外周血白细胞(WBC),中性粒细胞,淋巴细胞,单核细胞,血小板(PLT),中性粒细胞与淋巴细胞比率(NLR),派生NLR(dNLR),在总共208例CP患者以及不同年龄和手术方式的CP患者亚组中评估了单核细胞与淋巴细胞比率(MLR)和PLT与淋巴细胞比率(PLR).通过受试者操作员特征曲线分析评估其预测值。
    结果:术前外周白细胞,中性粒细胞,NLR,dNLR,MLR,与PLR呈正相关,淋巴细胞与CP患者术后CDI的发生呈负相关,特别是当白细胞≥6.66×109/L或淋巴细胞≤1.86×109/L时同时,多因素logistic回归分析显示,年龄>18岁的患者WBC>6.39×109/L,经颅入路患者WBC>6.88×109/L或淋巴细胞≤1.85×109/L与术后CDI发生率升高密切相关。此外,从受试者操作特征曲线分析获得的曲线下面积显示,炎症标志物的最佳预测因子是总CP患者的NLR,≤18岁年龄组和经蝶窦组的MLR,>18岁年龄组的NLR和经颅组的dNLR。值得注意的是,NLR+dNLR组合指数在所有组中显示出最有价值的预测因子。
    结论:术前外周炎症标志物,尤其是WBC,淋巴细胞和NLR+dNLR,是CP术后CDI的有希望的预测因子。
    BACKGROUND: Postoperative central diabetes insipidus (CDI) is commonly observed in craniopharyngioma (CP) patients, and the inflammatory response plays an important role in CPs. We aimed to evaluate the predictive value of preoperative peripheral inflammatory markers and their combinations regarding CDI occurrence in CPs.
    METHODS: The clinical data including preoperative peripheral inflammatory markers of 208 CP patients who underwent surgical treatment were retrospectively collected and analyzed. The preoperative peripheral white blood cells (WBC), neutrophils, lymphocytes, monocytes, platelet (PLT), neutrophil-to-lymphocyte ratio (NLR), derived-NLR (dNLR), monocyte-to-lymphocyte ratio (MLR) and PLT-to-lymphocyte ratio (PLR) were assessed in total 208 CP patients and different age and surgical approach CP patient subgroups. Their predictive values were evaluated by the receiver operator characteristic curve analysis.
    RESULTS: Preoperative peripheral WBC, neutrophils, NLR, dNLR, MLR, and PLR were positively correlated and lymphocyte was negatively associated with postoperative CDI occurrence in CP patients, especially when WBC ≥ 6.66 × 109/L or lymphocyte ≤ 1.86 × 109/L. Meanwhile, multiple logistic regression analysis showed that WBC > 6.39 × 109/L in the > 18 yrs age patients, WBC > 6.88 × 109/L or lymphocytes ≤ 1.85 × 109/L in the transcranial approach patients were closely associated with the elevated incidence of postoperative CDI. Furthermore, the area under the curve obtained from the receiver operator characteristic curve analysis showed that the best predictors of inflammatory markers were the NLR in total CP patients, the MLR in the ≤ 18 yrs age group and the transsphenoidal group, the NLR in the > 18 yrs age group and the dNLR in the transcranial group. Notably, the combination index NLR + dNLR demonstrated the most valuable predictor in all groups.
    CONCLUSIONS: Preoperative peripheral inflammatory markers, especially WBC, lymphocytes and NLR + dNLR, are promising predictors of postoperative CDI in CPs.
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  • 文章类型: Journal Article
    目的:尿崩症(ADI)是一种危及生命的疾病。它的特征是精氨酸加压素缺乏和口渴缺失。关于ADI的临床特征的数据很少。本研究调查了住院ADI患者的临床特征。
    方法:对2014年1月至2021年12月华山医院内分泌科收治的ADI患者进行回顾性研究,并与口渴正常的中央性尿崩症(CDI)患者进行比较。
    结果:在研究期间,共有507名CDI住院患者,其中50例为ADI,占9.9%。40%的ADI患者因高钠血症入院,但对照组没有因高钠血症入院.ADI患者的病变更可能位于鞍上区(100%vs.66%,P<0.05)。下丘脑功能障碍的患病率较高(76%vs.8%,P<0.001),中枢甲状腺功能减退症(100%vs.90%,P=0.031),高血糖(66%vs.32%,P<0.001),血脂异常(92%vs.71%,P=0.006)和高尿酸血症(64%vs.37%,ADI组的P=0.003)高于对照组。ADI组在入院和出院时的高钠血症比例均较高(90%vs.8%,68%vs.8%,分别,两者均为P<0.001),导致更高的并发症发生率,如肾功能不全,静脉血栓形成和感染。
    结论:ADI患者高钠血症的患病率较高,垂体功能减退,下丘脑功能障碍,代谢紊乱和并发症,对综合管理提出了巨大挑战。
    OBJECTIVE: Adipsic diabetes insipidus (ADI) is a life-threatening disease. It is characterized by arginine vasopressin deficiency and thirst absence. Data about clinical characteristics of ADI were scarce. This study investigated the clinical features of hospitalized ADI patients.
    METHODS: A retrospective study was conducted of hospitalized ADI patients admitted to the Endocrinology Department of Huashan Hospital between January 2014 and December 2021, and compared with central diabetes insipidus (CDI) patients with normal thirst.
    RESULTS: During the study period, there were a total of 507 hospitalized CDI patients, among which 50 cases were ADI, accounting for 9.9%. Forty percent of ADI patients were admitted due to hypernatremia, but there were no admissions due to hypernatremia in the control group. The lesions of ADI patients were more likely to be located in the suprasellar area (100% vs 66%, P < .05). Higher prevalence of hypothalamic dysfunction (76% vs 8%, P < .001), central hypothyroidism (100% vs 90%, P = .031), hyperglycemia (66% vs 32%, P < .001), dyslipidemia (92% vs 71%, P = .006), and hyperuricemia (64% vs 37%, P = .003) was found in the ADI group than in the control group. The proportions of hypernatremia were higher in the ADI group both at admission and at discharge (90% vs 8%, 68% vs 8%, respectively, both with P < .001), contributing to higher prevalence of complications, such as renal insufficiency, venous thrombosis, and infection.
    CONCLUSIONS: ADI patients were found with higher prevalence of hypernatremia, hypopituitarism, hypothalamic dysfunction, metabolic disorders, and complications, posing a great challenge for comprehensive management.
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  • 文章类型: Journal Article
    生活质量(QoL)一直是肿瘤学中的一个多因素问题。我们旨在检查颅咽管瘤患者的术前QoL,并探讨QoL参数与临床指标之间的潜在相关性。
    我们共纳入109例颅咽管瘤患者。我们使用了简短表格36(SF-36),症状检查表-90,广义焦虑症问卷量表(GAD7),患者健康问卷抑郁(PHQ9)和匹兹堡睡眠质量指数前瞻性评估他们的QoL。在根据Puget分类划分的亚组之间比较了QoL参数和临床指标。进行了相关分析和回归分析,以检测对自我报告的健康有影响的决定因素。
    与普通人群相比,患者的QoL受损(p<0.001),由SF-36评估。相关性分析表明,由中心性尿崩症(CDI)引起的有害影响。多元线性回归揭示了CDI对精神成分汇总的不利影响(系数=-13.869,p=0.007),GAD7总分(系数=2.072,p=0.049)和PHQ9总分(系数=3.721,p=0.001)。多因素logistic回归分析证实CDI是抑郁症状发生的危险因素(OR=6.160,p=0.001)。
    颅咽管瘤患者手术前QoL明显受损。CDI对患者的QoL产生有害影响,它可能作为早期识别有抑郁症风险的患者的标志。
    UNASSIGNED: Quality of Life (QoL) has been a multifactorial concerning issue in oncology. We aimed to inspect the pre-operative QoL among patients with craniopharyngioma and to explore the potential correlations between parameters of QoL and clinical indices.
    UNASSIGNED: We enrolled a total of 109 patients with craniopharyngioma. We utilized Short Form 36 (SF-36), Symptom Check List-90, Generalized Anxiety Disorder Questionnaire scale (GAD7), Patient Health Questionnaire Depression (PHQ9) and Pittsburgh Sleep Quality Index to prospectively evaluated their QoL. Parameters of QoL along with clinical indices were compared among sub-groups divided according to Puget classification. Correlation analyses and regression analyses were performed to detect influential determinants to self-reported wellness.
    UNASSIGNED: Patients presented impaired QoL compared with general population (p < 0.001), as assessed by SF-36. Correlation analyses indicated the detrimental influence resulting from central diabetes insipidus (CDI). Multivariate linear regression unveiled the adverse effect of CDI on Mental Component Summary (coefficient = -13.869, p= 0.007), GAD7 total score (coefficient = 2.072, p = 0.049) as well as PHQ9 total score (coefficient = 3.721, p = 0.001). Multivariate logistic regression verified CDI as a risk factor of developing depressive symptoms (OR = 6.160, p = 0.001).
    UNASSIGNED: QoL of patients with craniopharyngioma was remarkably compromised before operation. CDI exerted detrimental influences on patients\' QoL and it might serve as a marker for early identification of patients at risk of depression.
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  • 文章类型: Review
    背景:在IgG4相关疾病(IgG4-RD)的初始治疗方面有相对丰富的经验,但是对复发合并多器官和组织受累的病例的治疗措施知之甚少。
    方法:一名43岁的男性,因复发性右泪腺肿大伴眼睑红斑伴尿崩症而先前诊断为IgG4-RD。
    方法:我们进行了垂体磁共振成像,显示垂体后叶边缘变化,垂体柄不均匀增强和结节样增厚,并进行了断水加压素试验,证实了中央性尿崩症,并结合患者的升高的IgG4水平和过去的医疗条件,我们诊断出了中心性尿崩症,IgG4相关的垂体炎,和IgG4-RD。
    方法:患者入院后,我们每天一次静脉注射甲基强的松龙500mg,连续4天,间隔10天后连续4天。在此期间,与霉酚酸酯250毫克每天两次和醋酸去氨加压素0.1毫克,每天3次。
    结果:对患者进行持续6个月的随访,未发现糖皮质激素治疗的副作用,没有复发的迹象,每日尿量稳定在正常范围内。
    结论:我们认识到IgG4水平不能反映复发或长期控制,糖皮质激素休克疗法是复发患者的可选和可靠的治疗策略。
    BACKGROUND: There is a relative wealth of experience in the initial treatment of IgG4-related disease (IgG4-RD), but little is known about therapeutic measures for recurrent cases combined with multiple organ and tissue involvement.
    METHODS: A 43-year-old man with a previous diagnosis of IgG4-RD due to recurrent right lacrimal gland enlargement with eyelid erythema presented with diabetes insipidus.
    METHODS: We performed a pituitary Magnetic Resonance Imaging which revealed posterior pituitary rim changes with inhomogeneous enhancement and nodular-like thickening of the pituitary stalk, and performed a water-deprivation-vasopressin test confirmed central diabetes insipidus, and in combination with the patient\'s elevated IgG4 levels and past medical conditions, we diagnosed central diabetes insipidus, IgG4-related hypophysitis, and IgG4-RD.
    METHODS: After the patient was admitted to the hospital we gave methylprednisolone 500 mg intravenously once daily for 4 days and again for 4 consecutive days after a 10-day interval. During this period combined with mycophenolate mofetil 250 mg twice daily and desmopressin acetate 0.1 mg 3 times daily.
    RESULTS: The patient was followed up for a sustained period of 6 months and no side effects of glucocorticoid therapy were noted, there were no signs of recurrence, and the daily urine output stabilized in the normal range.
    CONCLUSIONS: We recognized that IgG4 levels do not reflect relapse or long-term control, and that glucocorticoid shock therapy is an optional and reliable treatment strategy for relapsed patients.
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  • 文章类型: Review
    抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)的特征是全身性小血管血管炎,很少表现为中枢性尿崩症(CDI)。在这项研究中,我们旨在确定AAV相关CDI患者的临床特征和预后.
    这是一项巢式病例对照研究,从2012年1月至2022年4月,在北京协和医院随访了患有CDI的AAV患者。与无CDI的AAV患者进行病例对照匹配(1:5),参与者按年龄匹配,性别,和AAV分类。我们每3-6个月收集一次临床数据,并使用PubMed进行文献综述,以确定1983-2022年发表的相关文章。
    在1203例AAV住院患者中,纳入16例CDI患者(1.3%)。平均年龄49岁,男性占56.3%。肉芽肿性多血管炎(GPA)占患者的87.5%。患有CDI的AAV患者有更多的耳朵,鼻子,和咽喉(ENT)(81.3%)受累,肾功能损害少于对照组(P<0.05)。经过四年的平均随访,50%的患者从AAV缓解,37.5%复发,12.5%死亡。我们的文献综述表明,与西方国家相比,亚洲国家的患者往往是老年男性,并且具有更高的髓过氧化物酶(MPO-ANCA)阳性。此外,蛋白酶3(PR3-ANCA)阳性可以预测疾病复发。
    患有CDI的AAV患者有更多的耳鼻喉受累和更高的eGFR。MPO-ANCA阳性在亚洲国家比西方国家更常见,PR3-ANCA阳性可以预测复发。
    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by systemic small-vessel vasculitis and may rarely present as central diabetes insipidus (CDI). In this study, we aimed to determine the clinical characteristics and prognosis of patients with AAV-associated CDI.
    This was a nested case-control study where AAV patients with CDI at the Peking Union Medical College Hospital were followed from January 2012 to April 2022. Case-control matching with AAV patients without CDI was performed (1:5), and participants were matched by age, sex, and AAV classification. We collected clinical data every 3-6 months and conducted a literature review using PubMed to identify relevant articles published from 1983-2022.
    Among 1203 hospitalized AAV patients, 16 patients with CDI were included (1.3%). The average age was 49 years, and men accounted for 56.3%. Granulomatosis with polyangiitis (GPA) accounted for 87.5% of patients. AAV patients with CDI had more ear, nose, and throat (ENT) (81.3%) involvement and less renal impairment than those in the control group (P<0.05). After a mean follow-up of four years, 50% of patients were in remission from AAV, 37.5% relapsed, and 12.5% died. Our literature review suggested that patients in Asian countries tend to be older men and have higher myeloperoxidase (MPO-ANCA) positivity than those in Western countries. Furthermore, proteinase 3 (PR3-ANCA) positivity may predict disease recurrence.
    AAV patients with CDI had more ENT involvement and a higher eGFR. MPO-ANCA positivity is more commonly observed in Asian countries than Western countries, and PR3-ANCA positivity may predict recurrence.
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  • 文章类型: Meta-Analysis
    目的:尿崩症(DI)的准确诊断对于正确处理非常重要。我们旨在评估和肽素测量在DI和原发性烦渴(PP)之间的鉴别诊断中的诊断准确性。
    方法:1月以来的电子数据库文献检索1st,2005年7月13日,2022年执行。评估和肽素在DI和PP患者中的诊断准确性的主要研究合格。两名评审员独立筛选相关文章并提取数据。使用诊断准确性研究质量评估(QUADAS)-2工具评估纳入研究的质量。使用了分层汇总接收机工作特性模型和双变量方法。
    结果:七项研究,包括422例多饮多尿综合征(PPS)患者,189人(44.79%)患有精氨酸加压素缺乏症(AVP-D,头颅DI)和212(50.24%)与PP,包括在内。刺激的和肽素区分PP和AVP-D的诊断性能的汇总估计为0.93(95%置信区间[CI]0.89-0.97)的敏感性,特异性为0.96(95%CI0.88-1.00),分别。基线和肽素水平具有较高的鉴定AVP-抗性(AVP-R,肾性DI),合并敏感性为1.00(95%CI0.82-1.00),特异性为1.00(95%CI0.98-1.00);然而,它在PP和AVP-D之间的差异中几乎没有价值。
    结论:Copeptin测量是DI和PP患者鉴别诊断的有用工具。在AVP-D的诊断中需要在和肽素测量之前进行刺激。
    OBJECTIVE: Accurate diagnosis of diabetes insipidus (DI) is of significant importance for correct management. We aimed to evaluate the diagnostic accuracy of copeptin level measurements in the differential diagnosis between DI and primary polydipsia (PP).
    METHODS: A literature search of electronic databases from January 1, 2005, to July 13, 2022, was performed. Primary studies that evaluated the diagnostic accuracy of copeptin concentration in patients with DI and PP were considered eligible. Two reviewers independently screened relevant articles and extracted data. The Quality Assessment of Diagnostic Accuracy Studies 2 tool was used to assess the quality of the included studies. The hierarchical summary receiver operating characteristic model and bivariate method were used.
    RESULTS: Seven studies including 422 patients with polydipsia-polyuria syndrome were included; of the 422 patients, 189 (44.79%) presented with arginine vasopressin deficiency (AVP-D, cranial DI) and 212 (50.24%) with PP. The summary estimates of the diagnostic performance of stimulated copeptin to differentiate between PP and AVP-D were 0.93 (95% CI, 0.89-0.97) for sensitivity and 0.96 (95% CI, 0.88-1.00) for specificity. Baseline copeptin level showed high performance in identifying AVP resistance (nephrogenic DI), with a pooled sensitivity of 1.00 (95% CI, 0.82-1.00) and specificity of 1.00 (95% CI, 0.98-1.00); however, it showed little value in the differentiation between PP and AVP-D.
    CONCLUSIONS: Copeptin level measurement is a useful tool for the differential diagnosis of patients with DI and PP. Stimulation before copeptin measurement is necessary in the diagnosis of AVP-D.
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  • 文章类型: Journal Article
    目的:中心性尿崩症(CDI)是鼻内镜手术(EES)治疗颅咽管瘤最常见的并发症。然而,部分CDI病例在随访期间可自发消退.因此,本研究旨在确定CDI自发消退的预测因素.
    方法:回顾性分析了2009年2月至2021年6月因颅咽管瘤而接受EES治疗的CDI患者的数据。根据随访期间CDI的分辨率将所有患者分为两组:恢复组和无恢复组。基线特征,外科,并对患者的随访结果进行比较。
    结果:我们确定了84例CDI患者(恢复组35例,无恢复组49例)。直接比较表明,保留垂体柄(57.1%vs.14.3%,P=0.000)和无下丘脑损伤(HI)(68.6%vs.20.4%,P=0.000)在恢复组中更常见,而诊断时的脑积水(8.6%vs.46.9%,P=0.000)在无恢复组中明显更常见。随后,我们通过单变量和多变量分析发现,CDI的自发消退与诊断时的脑积水有关(是与否:赔率比[OR],0.198;P=0.045),垂体柄损伤(保留与切片:或,7.055;P=0.004),和Hong等人。HI模式(轻度HIvs.无HI:OR0.183,P=0.038;单侧HIvs.无HI:或0.147,P=0.017;双侧HIvs.NO-HI:OR0.154,P=0.044)。
    结论:诊断时的脑积水,垂体柄损伤,和Hong等人。HI模式可能是颅咽管瘤EES后CDI自发消退的预测因子。
    Central diabetes insipidus (CDI) is the most common complication of endoscopic endonasal surgery (EES) for craniopharyngioma. However, some cases of CDI could spontaneously resolve during the follow-up period. Hence, this study aimed to determine the predictive factors for the spontaneous resolution of CDI.
    Data of patients with CDI who underwent EES for craniopharyngioma between February 2009 and June 2021 were retrospectively reviewed. All patients were divided into 2 groups based on the resolution of CDI during follow-up: the recovery and no recovery groups. The baseline characteristic, surgical, and follow-up results of patients were compared.
    We identified 84 patients with CDI (35 in the recovery group and 49 in the no recovery group). A direct comparison showed that retaining the pituitary stalk (57.1% vs. 14.3%, P = 0.000) and no-hypothalamic injury (HI) (68.6% vs. 20.4%, P = 0.000) were more common in the recovery group, whereas hydrocephalus at diagnosis (8.6% vs. 46.9%, P = 0.000) was significantly more common in the no recovery group. Subsequently, we found through univariate and multivariate analysis that the spontaneous resolution of CDI was associated with hydrocephalus at diagnosis (yes vs. no: odds ratio [OR], 0.198; P = 0.045), pituitary stalk injury (retaining vs. sectioning: OR, 7.055; P = 0.004), and the Hong et al HI pattern (mild-HI vs. no-HI: OR, 0.183; P = 0.038; unilateral-HI vs. no-HI: OR, 0.147; P = 0.017; bilateral-HI vs. no-HI: OR, 0.154; P = 0.044).
    Hydrocephalus at diagnosis, pituitary stalk injury, and the Hong et al HI pattern might be predictors of the spontaneous resolution of CDI following EES for craniopharyngioma.
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  • 文章类型: Letter
    暂无摘要。
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  • 文章类型: Case Reports
    背景:免疫球蛋白G4(IgG4)相关疾病(IgG4-RD)是一种全身性疾病,涉及IgG4阳性浆细胞在多个器官中的浸润。木村病(KD)表现为头颈部皮下肿块,经常伴有嗜酸性粒细胞增多和高免疫球蛋白E(IgE)水平。这里,我们报告了一例罕见的并发IgG4-RD和KD的哮喘表现,肺栓塞,伴有肺癌的中心性尿崩症。
    方法:我院收治一名有8年KD病史的65岁中国男性,主诉呼吸困难和咳痰1个月。实验室检查显示,血清嗜酸性粒细胞计数以及总IgE和IgG4水平显着升高。胸部增强计算机断层扫描显示右肺动脉充盈缺损,左下叶结节。胰腺增强磁共振成像(MRI)和磁共振胰胆管造影显示胰尾肿胀,胆总管胰管部分局部狭窄。垂体的增强MRI显示垂体柄增厚。此外,8年前收集的标本的免疫组织化学显示IgG4阳性细胞。在用KD诊断IgG4-RD后,开始使用糖皮质激素和免疫抑制剂;患者的病情迅速改善。出院后一年,由于肺结节增大,患者接受了楔形肺切除术,病理提示肺鳞癌。
    结论:该病例表现为一种罕见的临床病症,其中IgG4-RD和KD的同时存在会导致各种罕见的表现,包括哮喘,肺栓塞,中心性尿崩症,和复杂的肺癌。这突出了在随访期间监测IgG4-RD患者恶性肿瘤的重要性。
    BACKGROUND: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a systemic disease that involves the infiltration of IgG4-positive plasma cells in multiple organs. Kimura disease (KD) presents as subcutaneous masses on the head and neck, frequently accompanied by eosinophilia and high immunoglobulin E (IgE) levels. Here, we report a rare case of concurrence of IgG4-RD and KD with manifestations of asthma, pulmonary embolism, and central diabetes insipidus accompanied by lung carcinoma.
    METHODS: A 65-year-old Chinese male with an eight-year history of KD was admitted to our hospital with complaints of dyspnea and expectoration for one month. Laboratory examination showed a considerable elevation in the serum eosinophil count and total IgE and IgG4 levels. Chest enhanced computed tomography showed filling defects in the right pulmonary artery and a nodule in the left inferior lobe. Pancreatic enhanced magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography showed a swollen pancreatic tail and local stricture of the pancreatic duct section of the common bile duct. Enhanced MRI of the pituitary gland showed thickening of the pituitary stalk. Additionally, immunohistochemistry of the specimens collected eight years prior revealed IgG4-positive cells. Following the diagnosis of IgG4-RD with KD, glucocorticoids with immunosuppressants were initiated; there was a prompt improvement in the patient\'s condition. One-year post-discharge, the patient underwent wedge-shaped resection of the lung due to enlargement of the pulmonary nodule, and the pathology revealed lung squamous carcinoma.
    CONCLUSIONS: This case presents a rare clinical condition in which the concurrence of IgG4-RD and KD causes various rare manifestations including asthma, pulmonary embolism, central diabetes insipidus, and complicated lung carcinoma. This highlights the importance of monitoring for malignancies in IgG4-RD patients during follow-up.
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