宁波市第二医院-胆囊结石伴急性胆囊炎-专家在线问诊-京东健康互联网医院

简介:

宁波市第二医院由于结石阻塞胆囊管,造成胆囊内胆汁滞留,继发细菌感染而引起的急性炎症。大多数急性胆囊炎患者为急性结石性胆囊炎。，常见的致病因素是胆囊管梗阻,大部分患者是由胆囊结石引起的,当胆囊管梗阻后,胆汁浓缩,浓度高的胆汁酸盐会损害胆囊黏膜上皮,引起炎症的变化。还有部分患者是致病细菌入侵,大多通过胆道逆行而入侵胆囊。致病细菌主要是大肠杆菌,其他有克雷伯氏、粪肠球菌及绿脓杆菌等。，胆囊，对症状较轻微的急性结石性胆囊炎,可考虑先用非手术疗法控制炎症,待进一步查明病情后进行择期手术。对较重的急性化脓性或坏疽性结石性胆囊炎或胆囊穿孔,应及时进行手术治疗,但必须作好术前准备,包括纠正水电解质和酸碱平衡的失调,以及应用抗生素等。非手术疗法对大多数早期急性结石性胆囊炎的患者有效。此法包括解痉镇痛,抗生素的应用,纠正水电解质和酸碱平衡失调,以及全身的支持疗法。在非手术疗法治疗期间,必须密切观察病情变化,如症状和体征有发展,应及时改为手术治疗。特别是老年人和糖尿病患者,病情变化较快,更应注意。 1.手术治疗有下列情况时,应经短时的对症治疗准备后,施行紧急手术：（1）发病在48～72小时内者。（2）临床症状重,经非手术治疗无效或者病情恶化者。（3）化脓性结石性胆囊炎有寒战、高热、白细胞计数明显增多者,有胆囊穿孔、弥漫性腹膜炎、合并急性化脓性胆囊炎、急性重症胰腺炎等并发症者。（4）老年或者合并糖尿病患者,胆囊容易发生坏疽及穿孔、结石,症状较重者应及早手术。手术方法包括：①胆囊切除术包括开腹或腹腔镜胆囊切除,目前以腹腔镜胆囊切除为首选。在急性期,胆囊周围组织水肿,解剖关系常不清楚,操作必须细心,此免误伤胆管和邻近重要组织,手术力求简单、安全、有效；②部分胆囊切除术对于胆囊炎症重,胆囊床分离困难或可能出血多,患者不能耐受较大创伤或较长手术时间者,可选择胆囊部分切除；③胆囊造口术对于一些老年患者,一般情况较差或伴有严重的心肺疾病,估计不能耐受胆囊切除手术者,有时在急性期胆囊周围解剖不清而致手术操作困难者,也可先作胆囊造口术。胆囊造口手术可在局麻下进行,其目的是采用简单的方法引流胆囊炎症,使患者渡过危险期,待其情况稳定后,一般于胆囊造口术后3个月,再作胆囊切除以根治病灶。 2.非手术治疗非手术疗法包括卧床休息、禁食、输液、纠正水和电解质紊乱,应用抗生素及维生素,必要时进行胃肠减压。腹痛时可给予解痉剂和镇痛剂,如阿托品、度冷丁等,同时应密切观察病情变化。，无，禁忌喝酒、吃辛辣食物，腹部X线 B超静脉胆道造影CT或核磁共振（MRI），。

false 严旺副主任医师

三级甲等宁波市第二医院

好评率：- 接诊量：- 平均响应：-

脑梗死，脑出血，脑血管病，癫痫，痴呆，帕金森，脑炎，重症肌无力，肌张力障碍，周围神经病，神经肌肉病等疾病的诊治

true 周银杰副主任医师

三级其他宁波市第二医院

好评率：- 接诊量：2 平均响应：-

普胸外科相关疾病，如气胸、漏斗胸、肺肿瘤、食管肿瘤、贲门失驰症、纵膈肿瘤、手汗症等

true 董彩军副主任医师

三级其他宁波市第二医院

好评率：- 接诊量：- 平均响应：-

擅长肺癌，食管癌，纵隔肿瘤的疾病的诊断和微创手术治疗，以及全程管理。

false 沈海波主任医师

三级甲等宁波市第二医院

好评率：- 接诊量：- 平均响应：-

胸部微创手术,肺、食管、纵隔、胸壁疾病的外科治疗。

true 何立峰主任医师

三级其他宁波市第二医院

好评率：- 接诊量：- 平均响应：-

在肺部小结节，肺肿瘤，食管肿瘤及纵隔疾病的诊治方面积累了丰富的临床经验，并且擅长全胸腔镜下肺癌根治，胸腺纵隔肿瘤切除，食管肿瘤切除，手汗症，漏斗胸等的微创手术。

true 陈光烈主任医师

三级其他宁波市第二医院

好评率：- 接诊量：- 平均响应：-

专业神经外科领域三十多年，在神经创伤救治，先天性疾病的治疗，神经系统的肿瘤包括脊髓肿瘤的治疗有一定的经验和心得

false 李峰主任医师

三级其他宁波市第二医院

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李峰，男，主任医师，毕业于南昌大学江西医学院医学影像专业，1997年始从事放射临床影像诊断工作。擅长骨关节、肺部弥漫性疾病及神经、消化系统疾病的影像诊断。主攻创伤、退变及免疫性等关节病变、骨肿瘤和尘肺病诊疗。本院线下门诊时间：周三下午、周五上午（关节炎康复联合门诊）

true 余哲主任医师

三级其他宁波市第二医院

好评率：- 接诊量：- 平均响应：-

肺癌、慢性阻塞性肺病、哮喘等疾病诊治

true 应骏主任医师

三级其他宁波市第二医院

好评率：- 接诊量：- 平均响应：-

对慢性咳嗽、支气管哮喘、COPD、慢性支气管炎及肺气肿、肺部肿瘤、胸腔积液的诊治有所专长；能熟练掌握支气管镜、肺穿刺等诊疗操作。

false 蔡赵勇主治医师

三级其他宁波市第二医院

好评率：100% 接诊量：8 平均响应：15分钟

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2024-10-31 09:27:52

科普文章

胆囊结石和胆囊炎，你知道多少？

科普健康知识，远离疾病困扰，大家好，我是肝胆外科帅医生。最近有很多朋友向我咨询关于胆囊结石和胆囊炎的一些问题，我发现他们许多人缺乏对胆囊的基本常识，当然更不清楚胆囊疾病进一步发展会造成的严重后果。因此，普及胆囊及相关疾病的医学常识，能够使更多人认识胆囊疾病，及早的去医疗机构就诊，采取有效的治疗方法，预防疾病的进一步发展。

1、胆囊的解剖特点

胆囊形态呈梨形，位于肝脏胆囊窝内，是胆囊管的终末膨大部分，长8-12cm，宽3-5cm，容量40-60ml。胆囊分为底、体、颈、管四部分。胆囊颈部呈囊性扩大，医学上称为Hartmann袋，胆囊结石常滞留于此。

2、胆囊的生理功能

胆囊是贮存和浓缩胆汁的囊状器官，很多人顾名思义，误以为胆汁是由胆囊产生的；其实胆汁主要是由肝细胞分泌的，占3/4，另外还有胆管细胞分泌的黏液物质，占1/4；胆汁的分泌是每日每刻都在进行的，正常成人每日分泌量约800-1200ml，主要作用是促进油脂类物质（脂肪、胆固醇和脂溶性维生素等）的吸收。胆囊体积虽小，但24小时能接纳约500ml的胆汁，并可将胆汁浓缩5-10倍而存储于胆囊内。当进食时，受不同食物的刺激，胆囊平滑肌收缩将胆汁排出，参与油脂类物质的消化吸收，每次排胆运动完成后，仍会有约15%的胆汁留存于胆囊内。

3、胆囊结石的主要成分是什么，又是如何形成的呢？

胆囊结石主要为胆固醇结石或以胆固醇为主的混合性结石和黑色素结石。但胆囊结石的成因非常复杂，受多种因素影响。一般来说，胆石的成因多系胆汁滞留、代谢障碍和胆道感染等因素综合作用所致，可以说任何影响胆固醇与胆汁酸和磷脂浓度比例和造成胆汁瘀滞的因素均能导致结石的形成。一般认为胆汁滞留是各种胆石形成的首要条件，假如我们将胆汁比喻成黄河之水的话，如果黄河下游阻塞，河水无法入海，则积聚在河道内，那么混合在河水内的沙子则沉落在河底，形成胆泥，在胆囊的不断收缩运动下，这些胆泥便逐渐形成大小、形态不同的胆囊结石。

4、什么是胆囊炎，胆囊炎症又是如何发生的

胆囊炎是胆囊管梗阻和细菌感染引起的胆囊炎症，主要原因为结石梗阻，约占95%，我们称之为胆囊结石性胆囊炎；而约5%的患者胆囊内无结石，我们称为非结石性胆囊炎。胆汁滞留和细菌感染是胆囊炎发生时互为影响的两大因素，当胆囊管受结石或其它因素梗阻时，胆汁流出障碍，细菌则在积聚的胆汁内繁殖，引起胆囊炎症，其主要的致病菌为大肠埃希菌、粪肠球菌、铜绿假单胞菌等。

5、胆囊炎有哪些表现，我们该如何去判断是否患了胆囊炎？
由于胆囊炎的病因、病变程度不同，以及有无胆囊结石的存在，其症状表现也有所不同。胆囊炎主要的临床表现如下：

①上腹部疼痛，以右上腹为著。开始时仅有上腹胀痛不适，逐渐发展至阵发性绞痛。腹痛常于夜间发作，尤其以晚十点至凌晨三时左右发作最为常见，与胆囊节律性运动有关，饱餐或进食油腻食物常是诱发原因。典型的临床表现常有右肩、背部放射痛，是协助判断的重要表现。

②恶心、呕吐，约60%以上的患者伴有恶心，1/3以上的患者伴有呕吐，特别是疼痛加剧时更为明显。胆囊炎症时胆汁排出不畅，无法促进食物的消化吸收，以及疼痛时迷走神经兴奋性增强，是引起恶心、呕吐的主要原因。

③发热、寒战，胆囊炎大多仅有轻至中度的发热，一般不超过38.5℃，常无寒战、高热，若出现这类症状提示病情较重，已发展至化脓性胆囊炎、坏疽性胆囊炎、胆囊穿孔或合并有胆管炎。

④腹部体征，患者呈急性病容，表情痛苦，有的烦躁不安，辗转反侧或呈被动体位，以缓解疼痛。典型的体征为右上腹肋缘下胆囊区压痛、腹肌紧张，拒按。部分患者可于右上腹触及肿大胆囊。

6、怀疑胆囊炎，需要做哪些检查？
①血液检查包括血常规、肝功能检查。血常规检查约85%的患者可有白细胞水平升高，中性粒细胞百分百增加，但老年人可不增加；肝功能检查可有谷丙转氨酶、谷草转氨酶、碱性磷酸酶的升高，胆红素则一般升高不明显。

②影像学检查腹部彩超是胆囊结石、胆囊炎时的首选检查，诊断率高，价格便宜；上腹部CT检查，可使医生直观的了解胆囊结石及胆囊炎症情况，预估手术困难情况，但需要指出的是胆囊结石因主要成分为胆固醇，CT影像下不显影，我们称之为“阴性结石”，而当结石成分中含有钙元素时才可在CT图像中看到结石，所以不要以为CT检查没有发现结石就排除胆囊结石的诊断；磁共振检查（MRCP），非必须的影像学检查，但部分患者存在胆管变异，磁共振检查可行胆道成形，了解胆道走形。若患者拟行手术治疗，且经济条件允许建议完善该项检查，能够降低术中胆管损伤风险。

7、胆囊结石、胆囊炎该如何治疗？对于单纯胆囊炎的患者，如症状轻微，可采取内科对症治疗，包括清淡饮食、规律饮食、改变饮食结构、口服消炎利胆药物等，多可缓解症状，甚至痊愈。若为急性胆囊炎，症状较重，需住院治疗，给予禁饮食、抗感染、解痉止痛、抑酸护胃、营养支持及补液等对症治疗；如果反复急性胆囊炎发作，则需行手术治疗，以免酿成严重后果。目前对于胆囊结石的治疗医学界已达成共识，即无论胆囊结石大小，原则上均应行胆囊切除手术，以防止诱发急性胆囊炎或引发其他严重的并发症，如急性胰腺炎、胆囊癌变等。

8、胆囊结石和胆囊炎的手术方法有哪些？
①腹腔镜下胆囊切除术（LC）即人们常说的“微创手术”，该术式创伤小，恢复快，术后3-5日即可出院，甚至有些医院开设了日间病房，术后1日即可出院。

②开腹手术适合那些存在腔镜手术禁忌，而又需手术治疗的患者，如既往有过腹部大手术史，预估腹腔粘连较重，或胆囊炎症较重，腔镜手术难以完成的患者。

③超声引导下经皮经肝胆囊穿刺置管引流术（PTGD）可降低胆囊内压，缓解腹痛症状，急性期过后再择期手术。适用于病情危重又不适合急诊行胆囊切除术的患者。

9、腹腔镜下保胆取石手术可行吗？
随着腹腔镜下胆囊切除术在国内的逐步开展和成熟，国内逐步发展出了腹腔镜下保胆取石的手术方式，并曾一度为大家所推崇。但随后不断有临床科研报道这种手术方式的结石高复发率，就目前的医学技术而言，保胆取石手术是被国内主流医学界所摒弃的一种术式。美、英等国家则不提倡在没有充分理论依据的前提下、也不允许不经充分论证的情况下开展保胆取石手术。那么为什么保胆取石手术具有高复发率呢，主要是因为保胆取石手术破坏了胆囊壁的完整性，术后创面会是结石形成的诱因，而致使胆囊结石高复发率。但看待问题不能片面的去理解一种事物，随着医学技术的不断进步及医疗设备的不断更新换代、普及和成熟，经胆囊管保胆取石，而不去破坏胆囊壁的完整性，那么保胆取石手术仍是具有发展前景的术式，但这种发展路程将会是漫长的。而就目前而言，不应仅为去取悦自身的心理需求、盲目的以自身局限的医学认知、而不听从医生的建议而要求医生实施保胆取石手术，可能会给自身带来更多的伤痛、恶变风险和长期服用预防结石形成药物的经济负担，当应谨而又慎。

10、胆囊结石为什么非要行胆囊切除手术，非手术治疗不行吗？
这也是大多数胆囊结石患者的疑问，答案是不行！胆囊结石的存在，会反复摩擦胆囊壁，破坏胆囊壁的完整性和屏障功能，导致胆囊炎症的反复发作，若结石导致胆囊管的完全梗阻，则腹痛更为剧烈，若梗阻不能尽早解除，会发展成胆囊化脓、胆囊坏疽和胆囊穿孔。若胆囊结石落入胆总管内，可形成胆总管结石，若日后行手术治疗，需行胆总管切开取石术，增加手术的复杂性；若结石将胆总管完全阻塞，则可能造成急性梗阻性化脓性胆管炎，会危及生命。若结石阻塞胰管的出口，可致胆源性胰腺炎，这也是大部分胰腺炎的发病病因。最为严重的是，长期的胆囊结石，尤其是超过十年的胆囊结石，可致使胆囊萎缩、胆囊癌变，最终追悔莫及。因为胆囊癌是全身恶性程度最高的一种恶性肿瘤，无论手术与否，预后极差，因此胆囊癌最佳的治疗方式重在预防，及时行胆囊切除手术。

11、胆囊炎时可服用哪些药物治疗？
①消炎利胆片（具体以药物说明书为准）

适应症：清热、祛湿、利胆。用于肝胆湿热所致的胁痛、口苦；急性胆囊炎、胆管炎见上述证候者。

用法用量：口服，一次6片，一日3次。

②舒胆片（具体以药物说明书为准）

适应症：清热化湿、利胆排石、行气止痛。用于肝胆湿热，黄疸胁痛，发热口苦，尿赤便燥；胆囊炎、胆道感染、胆石症见上述证候者。

用法用量：口服。一次5-6片，一日3次，小儿酌减，或遵医嘱。

③胆宁片（具体以药物说明书为准）

适应症：疏肝利胆，清热通下。用于肝郁气滞，湿热未清所致右上腹隐隐作痛、食入作胀、胃纳不香、嗳气、便秘；慢性胆囊炎见上述症候者。

用法用量：饭后服，一次5片，一日3次。

④熊去氧胆酸胶囊（优思弗）（具体以药物说明书为准）

适应症：1. 固醇性胆囊结石-必须是X射线能穿透的结石，同时胆囊收缩功能须正常;2. 胆汁郁积性肝病(如:原发性胆汁性肝硬化);3. 胆汁反流性胃炎。

用法用量：

按时用少量水送服。按体重每日剂量为10mg/kg，即:溶石治疗:一般需6~24个月，服用12个月后结石未见变小者，停止服用。治疗结果根据每6个月进行超声波或X射线检查判断。

注意：该药需在医生指导下应用。

12、胆囊结石、胆囊炎患者日常的注意事项有哪些？

①一日三餐好，胆囊无烦恼

不吃早餐，或者饮食无规律，会导致空腹时间过长，胆囊缺乏食物的刺激，导致胆汁排出减少，而胆囊的浓缩功能不受影响，使胆汁中的胆固醇处于过饱和状态，易结晶析出，形成胆囊结石。

②暴饮暴食不可取，餐餐进食别太饱

饱餐和暴饮暴食会刺激胆汁的大量分泌，促使胆囊强烈收缩，可诱发胆绞痛和急性胆囊炎症。因此应少量多餐，细嚼慢咽，勿过饱，使胆囊规律的排出胆汁。

③饮食记得要清淡，油腻食物少入口

大量进食高脂饮食，如富含饱和脂肪酸和胆固醇的动物内脏、肥肉、蛋黄、油炸食品等，会刺激胆囊的收缩，增加胆囊疾病发作的危险。大量进食油脂类食物，会影响胆汁各成分的结构，胆汁酸分泌减少，胆固醇及胆色素增加，利于胆囊结石的形成。另外，高脂饮食还会导致胆固醇与脂肪酸呈过饱和状态，促进结晶、析出和结石的形成。

④忌辛辣食物和“发物”

中医讲的“发物”和辛辣食物，如辣椒、花椒、花椒、芥末等能够刺激胆囊的收缩，并使oddi氏括约肌痉挛，使胆结石易于嵌顿，胆汁排出不畅，诱发胆囊炎症的发生。因此，患有胆囊结石、胆囊炎的患者应忌食辛辣食物。

⑤多饮水，降低碳水化合物的摄入

多饮白开水可以降低胆汁的浓度，降低结石形成的风险，建议每日饮水量应在2000ml以上。若常喝碳酸饮料、含糖的果汁以及甜食，会使胰岛素分泌增加，加速胆固醇的积累，造成胆汁内胆固醇、胆汁酸、磷脂的比例失调。另外，进食过多的糖类食物，血液中的葡萄糖浓度升高，会通过三羧酸循环转变为脂肪酸，使血脂升高，有利于结石的形成。

⑥多吃新鲜蔬菜和水果，增加膳食纤维。

新鲜蔬菜和水果富含多种维生素和微量元素，能够调节机体能量代谢和新陈代谢，降低体内胆固醇和胆汁酸浓度，降低胆囊结石形成的风险。另外，适当的增加膳食纤维可刺激肠道蠕动，也有利于预防胆囊炎的发作。

⑦改变烹饪方式

炒菜用油应以植物油为主，如花生油、大豆油、橄榄油等，少食或不食动物油。烹饪方式尽可能采用煮、炖和清蒸的方式，而少油炸、油煎的方式，因为在高温的油脂中，含有丙烯醛的裂解产物会增加，可刺激胆道，引起胆道痉挛的发作。

⑧戒烟、戒酒，健康生活方式

香烟、酒可以刺激胃酸的分泌，促进胆囊的收缩和oddi氏括约肌痉挛，胆汁排出受阻，诱发胆囊炎症及胆绞痛。而且吸烟和饮酒会增加肺癌和肝硬化的发病率，因此应戒烟戒酒，提倡健康生活方式。

⑨定期复查很重要，及时治疗效果好

胆囊结石和胆囊炎的患者应坚持定期复查，至少每半年或一年复查一次腹部彩超，病情变化及时复查。这样可以很及时的了解胆囊结石的变化，及早的发现有无胆囊癌变，避免后悔终生的惨剧发生。

胆石症的症状？

观众朋友们大家好，胆石症是临床上比较多见的急腹症之一。很多患者往往误以为是胃部疾病，反复吃保护胃的药物，等到出现高烧，黄疸，甚至腹膜炎等表现时才检查出来是胆石症的原因。这错过了疾病最佳治疗时机，对后续的康复增加了困难。今天我们一起学习一下胆石症的常见临床症状，便于患者自行判断病情。

胆石症临床上最常见的是肝外胆管结石引起来的症状，其中以胆囊结石和胆总管结石最多见。

胆囊结石合并急性胆囊炎时伴有右上腹疼痛不适，典型的表现是右上腹绞痛伴有右侧肩背部放射性疼痛，患者多合并恶心呕吐，呕吐物多含有胆汁样液体，呕吐后患者腹痛，腹胀症状可稍缓解。胆道炎症感染较重者可伴有黄疸，高热等表现，患者自行服用胃药多不能完全缓解症状。这种情况下患者可完善一个肝胆的彩超检查，多可发现胆囊肿大，胆囊壁水肿明显，胆囊内可见结石。少部分患者可以有胆囊周围积液，积脓等表现。

胆总管结石合并急性胆管炎常常会引起严重的并发症，如胆源性胰腺炎，急性梗阻性化脓性胆管炎等等，患者常见的症状有剑突下压痛明显，可伴有腰背部放射痛。结石完全堵塞胆管可引起寒战，高热，腹痛，感染性休克，神智模糊等胆管炎表现，患者完善磁共振+胆管成像可明确诊断。

以上就是今天的内容，欢迎大家留言讨论，我们相互交流。

胆石症的中医治疗方法

如今的中青年人往往以车代步，久坐不动，热衷于玩手机，饮食又喜欢吃辛辣刺激和肥甘，年轻的女性则另一个极端节食减肥，吃什么代餐拒绝主食。再加上心情浮躁喜怒无常，导致腹痛反复到医院来就诊，自以为是胃病，检查结果则提示胆结石，中医属于胁痛的范畴。

当然对于胆结石首选是手术治疗，对一些没有达到手术的指证，又有上腹部不适的、腹痛的等中医疗效甚佳，中医如何调理胆结石，首先是饮食规律营养均衡，多吃含有维生素A和维生素的水果，保持心情愉悦，中药治疗当然要辨证施治。常见的证型是肝胆湿热、肝气郁结、湿热内蘊等。

一、肝胆湿热型：症状胁肋胀痛、口苦纳呆、呕恶,腹胀、大便不调、小便短赤、舌红苔黄腻、脉弦滑数，治则清热利湿，处方大排石汤加减：龙胆草12克、柴胡12克，白芍15克，金钱草30克，郁金草10克，五灵脂10克等

二、湿热内蕴型：症状热势缠绵、午后热高、身重疲乏、胸脘痞满、大便粘腻不爽、小便不利或黄赤等，治则健脾祛湿，舒肝清热，小排石汤加减：陈皮6克，泽泻12克，野菊花15克，大黄10克，枳实10克，虎杖15克，郁金15克，金钱草30克等。

三、肝郁气滞型：症状情志抑郁，胸胁或少腹胀满窜痛，善太息，或见有咽部异物感，或颈部瘿瘤，或有胁下肿块，或月经不调，痛经。舌苔薄白，脉弦。病情轻重与情绪变化有关。方药大柴胡汤加减。

胆囊结石的痛，该重视

临床上胆囊结石通常可以引起 3 类疼痛，而且分别有其诊断的意义。

第一种:慢性胆囊炎引起的感痛

这种疼痛比较轻微，但是非常常见，常常是饭后出现，病人的感觉就是胃部的隐隐作痛，会慢慢消失。而典型的会出现背部右肩胛骨附近的隐痛，这种在医学上被称为“牵涉痛”。

第二种:急性胆囊炎引起的疼痛

胆囊急性发炎时疼痛最典型，很多病人都是这时被诊断出来的。这种疼痛的位置在右上腹，精确的描述在右侧肋弓和腹直肌右缘交汇处，医学上称为“胆囊点”。此时右上腹按压上去会出现疼痛。另外，急性胆囊炎发作时有些病人也会出现右肩胛骨区域的疼痛，这样的话就更典型了。

第三种：胆绞痛

这种疼痛常常比较剧烈，虽说是胆囊的绞痛，但是疼痛的位置常常在心窝的下方，并不是“胆囊点”的地方。发生胆绞痛的原因很简单，就是胆囊结石卡在了胆囊管开口处，胆绞痛发作时往往疼痛剧烈，常常会误以为是急性胆囊炎的发作。这种疼痛一般数小时可以缓解。

胆囊结石的形成

我们胆汁里的胆固醇一般情况下溶解在胆汁酸和磷脂里，就像糖或盐溶解在水里一样，如果浓度太高，其中一部分就会形成结晶，在钙离子的作用下形成结石，逐渐长大变成团块，停留或者堵塞在胆道系统(如胆囊、胆管)，影响胆汁的流动，进而形成恶性循环。

急性胆囊炎的治疗原则

急性胆囊炎的治疗原则分为两大部分，第一个就是非手术的治疗，第二个就是手术的治疗。

非手术治疗包括解痉、镇痛、抗生素的应用，纠正水电解质和酸碱平衡的失调，以及全身的支持治疗。在非手术治疗期间必须要密切观察病情的变化，如症状和体质的发展，应及时改为手术的治疗；特别是老年人和糖尿病患者病情变化快更应该要注意。

第二种方案就是手术的方案，一般认为应该要尽早期的手术，手术不等于急诊手术，而是在病人入院时经过一段时间的非手术治疗和术前准备以后，并同时应用彩超和检查确诊以后，在发病时间不超过 72 小时的前提下下进行的手术，手术主要采用的是两种方案，一种是胆囊切除术，另一种是胆囊造口术。

什么是胆石症？

胆石症

　　胆囊结石属于胆道系统的常见病，随着经济的发展、人们饮食结构的变化,胆囊结石的发病率呈现出显着升高的趋势。

　　胆结石的首选检查方法是超声，对于超声发现胆结石的患者，往往会接受CT检查来进一步确诊，但如果怀疑患者患有胆石症，能否可以只做CT检查？CT检查对于胆结石的诊断比超声更敏感、更准确吗？

　　答案是否定的！

　　1/3～1/2的胆石症可始终没有症状，患者常不及时诊治，胆石症的主要症状是右上腹痛和黄疸，并发胆管炎可出现高热等。胆石症的病理基础是在胆汁淤滞和胆道感染等因素的影响下，胆汁中的胆色素、胆固醇、黏液物质和钙盐析出、凝集而成胆结石。发生在胆囊内的称胆囊结石，发生在胆管内的称胆管结石，统称胆石症。

　　按化学成分可将胆石分为３种类型：胆固醇类结石：胆固醇含量占８０％以上；胆色素类结石：胆固醇含量少于２５％；混合类结石：胆固醇含量占５５％～７０％。

　　阳性结石是Ｘ线不易透过、密度较高的结石；阴性结石是Ｘ线易透过的结石，其密度偏低。

　　胆石的ＣＴ平扫表现与其化学成分密度相关，其ＣＴ值与胆固醇含量呈负相关，与胆红素和含钙呈正相关。因此，CT平扫发现的低密度结石可判断其主要成分是胆固醇。

　　胆石可位于胆囊或胆管内，其症状与胆石的大小、部位及有无并发胆囊炎有关。肝内胆管结石直接征象表现为肝内高密度影，多发的肝内胆管结石呈铸型征象。肝内胆管结石要与肝内钙化相鉴别，后者ＣＴ值较高，前者ＣＴ值常为100HU左右，后者无肝内胆扩张，前者常多发，多层面仍可见。肝内胆管结石的间接征象表现为肝内胆管的扩张，肝外胆管结石则见梗阻段以上的胆管扩张。阴性结石主要以间接征象，表现为胆囊扩张、胆囊底密度增高、液平等。Ｂ超诊断优于ＣＴ平扫。

　　由于通过胆囊切除治疗胆囊结石的治疗方法,不仅术后可能有较高的并发症发生率,还会损伤患者的身体机能,使得右半结肠癌的发生风险升高,甚至还有2％的死亡率。别是对于身体条件较差,无法耐受手术或者有保留胆囊意愿的患者,如能通过保守治疗获得较为理想的效果具有重要意义。

　　当超声发现胆石症时，应进一步接受CT检查，利用螺旋CT平扫,不仅能判断结石的数目、大小,还能判断结石的密度,推测其组成成分,并为低密度的胆固醇结石、低钙盐小结石以及CT为阴性的等密度结石等提供新的治疗方向，如胆固醇结石用超声体外碎石，或化学药物溶石效果较好。随着医学影像设备普及，ＣＴ平扫将为临床提供更大价值的影像资料。

保胆还是切胆好？

随着胆囊结石伴胆囊炎发病率的升高，越来越多的病人需要行手术治疗。很多病人在选择手术治疗的同时，也产生了这样的疑虑，胆囊切掉以后对身体影响是不是很大呀？我很年轻，能不能选择不切除胆囊，就把结石去除呀？

面对这样的疑虑，我只想说，部分病人可以选择腹腔镜保胆取石术，但是选择这种手术方式的病人指征非常严格，大部分病人并不适合保胆，需要行腹腔镜胆囊切除术，具体还是需要医生来综合评估。那么哪些情况适合行保胆手术，我来向大家简单介绍一下。

主要考虑以下几个方面：

胆囊结石合并胆囊炎未引起明显的腹痛或者腹胀症状，或者症状轻微。胆囊急性炎症期不适合保胆。

B 超提示胆囊结石数目尽量在 3 个以内，胆囊壁光滑，没有增厚。

脂肪餐试验提示胆囊收缩功能正常。

手术中发现胆囊形态正常，胆道镜明确胆囊粘膜面正常，结石可以取干净，胆囊管通畅，可见胆汁反流。

病人有保胆意愿，可以接受结石复发，胆囊炎再次发作风险。

病例31-2023：一名79岁男性，身体不自主运动，一过性反应迟钝

以下内容来源于新英格兰医学杂志。

Presentation of Case

Dr. Carrie Chui (Neurology): A 79-year-old man was admitted to this hospital because of involuntary movements on the left side and transient unresponsiveness.

The patient had been in his usual state of health until 9 months before admission, when involuntary movements of the left shoulder and left side of the face developed. The movements were described by the patient as twitching, were not associated with a change in the level of consciousness, and resolved after 1 to 2 minutes. During the next 6 months, the patient had similar episodes approximately once per month, but the episodes increased in duration, lasting 5 to 6 minutes.

Three months before admission, the episodes of involuntary movements increased in frequency, and the patient was evaluated by his primary care physician. The physical examination was normal. Results of kidney-function tests were normal, as were blood levels of glucose and electrolytes, except for the sodium level, which was 129 mmol per liter (reference range, 135 to 145). There was a history of inappropriate antidiuretic hormone secretion, and the sodium level was similar to levels obtained during the past 4 years. Magnetic resonance imaging (MRI) of the head (Figure 1A), performed before and after the administration of intravenous contrast material, revealed a focus of enhancement in the right middle frontal gyrus that was thought to be a small vascular anomaly. Electroencephalography (EEG), performed with the patient in awake and drowsy states, revealed rare, brief, focal slowing in the left temporal lobe during drowsiness; no epileptiform abnormalities were present.

Figure 1

MRI of the Head and CT Angiogram of the Head and Neck.

Two months before admission, the patient was evaluated in the epilepsy clinic affiliated with this hospital. He reported that the episodes of involuntary movements had increased in both frequency and duration, occurring once or twice per day and lasting approximately 10 minutes. Episodes began with tingling and numbness in the left leg that prompted the patient to voluntarily stomp the left foot to relieve the uncomfortable sensation. Then, the patient had involuntary movements that he described as an uncontrollable invisible force moving the left leg and arm, with hyperextension of the arm backward and pronation of the wrist. There was associated numbness in the distal portions of the left third, fourth, and fifth fingers and involuntary movement of the left cheek. No prodromal symptoms occurred. The patient had awareness during the episodes, and after the episodes, he felt fatigued but had a normal level of consciousness, without confusion. The examination in the epilepsy clinic was normal. A diagnosis of seizure disorder was considered, and treatment with levetiracetam was started.

Three weeks before admission, the patient was again evaluated in the epilepsy clinic. He reported that the episodes of involuntary movements still occurred on a daily basis but had decreased in duration and involved only the left leg, without abnormal movements of the arm or face. Dizziness, headache, and weakness had developed and were attributed to the use of levetiracetam. The patient’s family had recorded a video of one of the episodes of involuntary movements. After reviewing the video, the patient’s neurologist thought that the episodes were less likely to be caused by seizures and more consistent with choreoathetoid movements. Cross-tapering of medications — with the simultaneous administration of levetiracetam in decreasing doses and clobazam in increasing doses — was initiated, and the patient was referred to the movement disorders clinic affiliated with this hospital.

On the morning of admission, an episode of involuntary movements of the left leg and left shoulder occurred and persisted for 1 hour. Several hours after the symptoms abated, the patient’s wife found the patient to be unresponsive; he was sitting in a chair. Emergency medical services were called, and when they arrived, the patient was responsive. The fingerstick blood glucose level was 180 mg per deciliter (10.0 mmol per liter) and the blood pressure 110/80 mm Hg. The patient was transported to the emergency department of this hospital for further evaluation.

In the emergency department, the patient reported dysuria and increased urinary frequency. The patient’s daughter noted that he had been more anxious during the past 3 years and occasionally had trouble with memory. Other medical history included Barrett’s esophagus, benign prostatic hypertrophy, chronic hepatitis B virus infection, eczema, gastroesophageal reflux disease, hypertension, nonischemic cardiomyopathy, and osteoporosis. There was no history of head trauma or extended loss of consciousness. Medications included aspirin, atorvastatin, doxazosin, finasteride, omeprazole, metoprolol, sacubitril, and valsartan. There were no known drug allergies. The patient was a lifelong nonsmoker and drank alcohol rarely; he did not use illicit drugs. His mother had had gastric cancer, and his sister had had esophageal cancer; there was no family history of seizures.

On examination, the temporal temperature was 36.8°C, the blood pressure 152/97 mm Hg, the pulse 65 beats per minute, the respiratory rate 16 breaths per minute, and the oxygen saturation 96% while the patient was breathing ambient air. The body-mass index (the weight in kilograms divided by the square of the height in meters) was 21.7. The blood pressure decreased to 130/63 mm Hg with standing. The patient was alert and interactive. The lower jaw was held to the left, but the nasolabial folds and smile were symmetric with activation. There were nonrhythmic, nonstereotyped, writhing movements of the left arm. Tone was normal, and strength was assessed as 5 out of 5 in the arms and legs. Results of liver-function and kidney-function tests were normal, as were blood levels of glucose and electrolytes, except for the sodium level, which was 125 mmol per liter. The lactate level was 2.1 mmol per liter (19 mg per deciliter; reference range, 0.5 to 2.0 mmol per liter [5 to 18 mg per deciliter]). The urinalysis was normal. Intravenous fluids were administered. Imaging studies were obtained.

Dr. Rajiv Gupta: Computed tomographic (CT) angiography of the head and neck (Figure 1B) revealed extensively calcified plaque with severe stenosis of the distal right common carotid artery (CCA), extending into the proximal right internal carotid artery (ICA), as well as stenosis of the right and left paraclinoid ICAs and the left vertebral artery at its origin. There was no vascular abnormality on the CT angiogram that corresponded to the abnormality in the right middle frontal gyrus seen on the previous MRI.

Dr. Chui: The patient was admitted to the hospital. On the second hospital day, the sodium level had increased to 130 mmol per liter, and the lactate level was normal. Additional imaging studies were obtained.

Dr. Gupta: MRI of the head showed no evidence of acute infarction. The focus of enhancement in the right frontal lobe that had been noted previously was not seen on the current MRI.

Dr. Chui: Blood levels of thyrotropin, cobalamin, and glycated hemoglobin and results of coagulation tests were normal. Screening tests for Lyme disease, tuberculosis, and syphilis were negative, as were tests for antibodies to cardiolipin and β₂-glycoprotein. A test for antinuclear antibodies was positive, at a titer of 1:160 in a homogeneous pattern. During a physical therapy session, the patient had abnormal movements of the left leg, left arm, and left side of the face. The abnormal movements diminished when the patient used distraction techniques, such as thigh tapping, finger snapping, and walking while holding a glass of water.

The transient unresponsiveness that led to the patient’s admission was attributed to a combination of sedation from clobazam and hypovolemia. Treatment with clobazam was stopped, and hydration was encouraged. A diagnosis of functional neurologic disorder was considered; outpatient physical therapy with continued use of distraction techniques was recommended. The patient was discharged home on the third hospital day.

Episodes of involuntary movements continued to occur on a daily basis at home. Two weeks after discharge, when the patient was doing exercises while sitting in a chair and having a conversation with his wife, he suddenly stopped talking. She found him slumped in the chair with his eyes closed, no longer exercising. When she asked him questions, he repeatedly said “yes.” Emergency medical services were called, and when they arrived, the patient was alert, diaphoretic, and nonverbal. He had a facial droop on the left side and a right gaze preference. The fingerstick blood glucose level was 130 mg per deciliter (7.2 mmol per liter) and the blood pressure 120/60 mm Hg. The patient was transported to the emergency department of this hospital for further evaluation.

In the emergency department, the temporal temperature was 36.6°C, the blood pressure 143/63 mm Hg, the pulse 66 beats per minute, the respiratory rate 18 breaths per minute, and the oxygen saturation 98% while the patient was breathing ambient air. He was alert and interactive. There was a facial droop on the left side. There was no effort against gravity in the left arm. The patient was able to lift the left leg off the bed for 1 to 2 seconds. He had a right gaze deviation that could not be overcome and mild dysarthria. The remainder of the examination was normal. A diagnosis of stroke was considered, and emergency CT angiography was performed.

Dr. Gupta: CT angiography showed no evidence of acute territorial infarction and no changes in cerebrovascular disease.

Dr. Chui: On repeat physical examination performed after CT angiography, the gaze deviation and dysarthria had resolved, and strength was normal. Mild facial paralysis was present.

A diagnosis was made.

Differential Diagnosis

Dr. Albert Y. Hung: This 79-year-old man initially presented with involuntary movements of the left shoulder and face without associated loss of consciousness. Diagnosis of an unusual movement disorder, especially one that is present episodically, can be challenging. Videos brought in by the patient can be very useful.¹ Most movement disorders result from abnormal functioning of extrapyramidal circuits involving the basal ganglia, rather than a specific neuroanatomical lesion, and the first step toward diagnosis is to identify the type of abnormal movements.²

Four salient aspects of this patient’s involuntary movements can help in characterizing the movement disorder before generating a differential diagnosis. First, the movements were paroxysmal, lasting for short periods of time with resolution between episodes. Second, the movements were nonstereotyped, appearing randomly and variably. Third, the movements were restricted to the left side of his body throughout the course, localizing the disease process to the right cerebral hemisphere. Finally, the symptoms were progressive, increasing in both duration and frequency.

Movement Disorders

This patient had abnormal involuntary movements, symptoms indicative of a hyperkinetic movement disorder. Tremor, the most common hyperkinetic disorder, is unlikely because the patient did not have rhythmic movements. Dystonia is also unlikely, because he did not have sustained muscle contractions that were causing twisting or abnormal postures of the legs, arms, head, neck, or face. Although the patient initially described the movements as twitching, his later descriptions are not suggestive of myoclonus or tics, which manifest as sudden, rapid, recurrent movements.

This patient’s neurologist described the involuntary movements as “choreoathetoid” after reviewing a video of an episode. Chorea, athetosis, and ballism make up a spectrum of involuntary movements that often occur in combination. Chorea refers to involuntary movements that are “dancelike” — irregular, random, unintended, and flowing from one body part to another. When these movements are slow and writhing (with a lower amplitude) and involve the distal limbs, the term athetosis is used. The presence of both chorea and athetosis in the same patient is referred to as choreoathetosis. When the movements are fast and flinging (with a higher amplitude) and involve the proximal limbs, the term ballism is used. Although the description of this patient’s movements was not clearly suggestive of ballism, hemichorea and hemiballismus often occur together.

The term dyskinesia can refer to any abnormal movements and is often used to describe hyperkinetic disorders that are induced by specific drugs, such as tardive dyskinesia induced by dopamine antagonists or dyskinesia induced by levodopa in patients with Parkinson’s disease. Often, dyskinesia manifests as chorea or choreoathetoid movements, but chorea and dyskinesia are not synonymous. This patient appears to have involuntary dyskinesia with choreoathetosis as the primary phenomenology. Before constructing a differential diagnosis for dyskinesia in this patient, I will consider two conditions that mimic dyskinesia: seizures and functional movement disorder.

Seizures

Various movement disorders may be mistaken for seizures, although these movement disorders are not associated with EEG abnormalities during the episode. Patients with some forms of epilepsy may present with abnormal movements without other features that are typically associated with seizures, such as aura, change in responsiveness, incontinence, or a postictal state.^3,4 Seizures were initially suspected in this patient, and he was referred to the epilepsy clinic. Recurrent focal seizures were probably suspected because of the transient nature of the episodes. Initial MRI had shown a small abnormality in the right middle frontal gyrus, but this finding was not seen on follow-up imaging, which makes it unlikely to be related to the overall presentation. Baseline EEG had shown only brief left temporal slowing, without epileptiform abnormalities. The EEG was an interictal study, so the findings do not rule out seizures. However, the slowing was ipsilateral to the abnormal movements, so it is unlikely to be related to the episodes. In addition, the patient’s involuntary movements were nonstereotyped and nonrhythmic, which makes his presentation unlikely to be due to a seizure disorder.

Functional Movement Disorder

Because this patient’s movements diminished with the use of distraction techniques, a diagnosis of functional movement disorder was considered. Most cases of functional movement disorder begin abruptly after a trigger, such as a mild physical injury or illness; a psychological stressor can be present but is not required for diagnosis. Symptoms are typically most severe around the time of onset and may wax and wane over time. Although distractibility is a finding associated with functional disorders, abnormal movements that occur with nonfunctional syndromes can sometimes be suppressed by action or incorporated into voluntary movements in a manner that may appear distractible. Several clinical features in this patient make a diagnosis of functional disorder unlikely. Functional movement disorder is more common in women than in men, and the average age at onset is 40 years.⁵ In addition, tremor is the most common clinical phenotype seen in patients with functional movement disorder; chorea or choreoathetosis, which was seen in this patient, is very unusual in patients with functional movement disorder. Overall, functional movement disorder is unlikely to explain this patient’s presentation.

Dyskinesia

Primary paroxysmal dyskinesia refers to a group of heterogeneous syndromes characterized by recurrent involuntary movements that occur episodically and abruptly, without loss of consciousness.⁶ These disorders usually begin in childhood or young adulthood. Both the age of this patient and the described phenomenology make a diagnosis of primary paroxysmal dyskinesia unlikely.

The differential diagnosis in this case is therefore focused on causes of secondary dyskinesia, of which there are many.⁷ MRI ruled out the presence of a mass lesion suggestive of cancer. The patient had no history of acute illness suggestive of a viral or other infectious encephalitis, and there was no history of trauma or exposure to drugs or other toxins. Although his daughter mentioned trouble with memory, there was no compelling history suggestive of a neurodegenerative disease.

A common metabolic cause of secondary dyskinesia is diabetic striatopathy, a syndrome involving the acute-to-subacute onset of chorea and ballism in the context of hyperglycemia.⁸ This syndrome can occur as the initial manifestation of type 2 diabetes mellitus or as a complication of poorly controlled diabetes. Diabetic striatopathy is more likely to develop in women than in men, and the average age at onset is 70 years. Most patients present with hemichorea and hemiballismus, rather than bilateral symptoms. CT shows hyperdensity, and T1-weighted MRI shows hyperintensity, in the contralateral basal ganglia. However, this patient had no history of diabetes and had a normal blood glycated hemoglobin level, features that rule out a diagnosis of diabetic striatopathy.

Choreiform movements can also be a manifestation of autoimmune conditions.⁹ This patient’s initial presentation with unilateral shoulder and face movements would have suggested the possibility of faciobrachial dystonic seizures associated with anti–leucine-rich, glioma-inactivated 1 (anti-LGI1) encephalitis.¹⁰ This condition is often associated with hyponatremia, which was present in this patient. However, as the case evolved, leg involvement and sensory changes developed that would be atypical for anti-LGI1 encephalitis.

One key clue in this case is that the patient did not have an isolated movement disorder. In addition to motor symptoms, he had a variety of sensory symptoms involving both the left arm and the left leg. His first hospital admission was precipitated by an episode of unresponsiveness. The clinical event that led to his second presentation to the emergency department was distinctly different: an acute onset of speech difficulty accompanied by left hemiparesis and right gaze deviation that was worrisome for an acute right middle cerebral artery (MCA) syndrome. The symptoms resolved without intervention, which indicates that he may have had an acute transient ischemic attack (TIA). The most relevant imaging finding was severe cerebrovascular disease, including severe stenosis of the distal right CCA and proximal right ICA. Could this patient’s movement disorder be explained by a vascular lesion?

Limb-Shaking TIAs

Limb-shaking TIAs were first described by C. Miller Fisher in 1962.¹¹ In most case reports, these episodes are associated with high-grade stenosis of the ICA, which was seen in this patient.^12,13 The mechanism is thought to be cerebral hypoperfusion, and changes in posture or head position that decrease cerebral blood flow can precipitate these episodes. In this patient, the first episode of unresponsiveness that led to hospital admission occurred when he was sitting. He then had an acute episode involving right gaze preference that was provoked by exercise and was very suggestive of a TIA in the right MCA territory. These findings are highly suggestive of a diagnosis of limb-shaking TIAs, and I would refer this patient for emergency carotid endarterectomy.

Clinical Impression and Initial Management

Dr. Scott B. Silverman: When I evaluated this patient, his transient right gaze preference and left hemiparesis were consistent with a right MCA syndrome due to a TIA from symptomatic severe stenosis of the right ICA. The mechanism of this event was either artery-to-artery embolism or hypoperfusion. His previous, recurrent episodes of transient choreoathetosis on the left side that had occurred mainly while he was sitting, standing, or exercising were consistent with limb-shaking TIAs from hypoperfusion or low flow.

The pathogenesis of a low-flow state related to severe carotid stenosis resulting in limb-shaking TIAs is described in a small case series.¹⁴ In six out of eight patients, the transient, stereotyped, involuntary movements were eliminated with carotid artery revascularization. Positional cerebral ischemia in patients without orthostatic hypotension has been described.¹⁵

Treatment with atorvastatin was continued, the dose of aspirin was increased to 325 mg per day, and an intravenous heparin infusion was started. The strategy of permissive hypertension was used, with high blood pressure allowed to a maximum systolic blood pressure of 180 mm Hg. The patient was admitted to the stroke service, and carotid artery duplex ultrasonography was performed.

Dr. Gupta: Doppler ultrasonography of the carotid arteries (Figure 2) revealed markedly elevated Doppler flow velocities within the proximal right ICA. There was a parvus et tardus waveform in the distal right ICA, a finding indicative of low flow related to the more proximal high-grade stenosis. The Doppler waveform contours had poststenotic turbulence.

Figure 2

Doppler Ultrasound Image.

Dr. Silverman: The vascular surgery service was consulted, and the patient underwent right carotid endarterectomy.

Clinical Diagnosis

Limb-shaking transient ischemic attacks.

Dr. Albert Y. Hung’s Diagnosis

Limb-shaking transient ischemic attacks due to severe carotid stenosis, with secondary paroxysmal dyskinesia.

Pathological Discussion

Dr. Caroline F. Hilburn: The endarterectomy specimen included the carotid bifurcation and was notable for firm arterial walls, a finding consistent with calcification. On gross examination (Figure 3A), a large plaque was centered at the carotid bifurcation and protruded into the lumen, resulting in a maximal luminal stenosis of 80%. The plaque had an irregular and focally friable surface. On microscopic examination (Figure 3B), the plaque was characterized by extensive calcification. Some regions of the plaque had a smooth, healed fibrous cap, whereas other regions had an irregular surface suggestive of ulceration, which indicated potential sites of plaque rupture. Multiple smaller calcified plaques were present, affecting both branches of the artery.

Figure 3

Endarterectomy Specimen.

Pathological Diagnosis

Complex atherosclerotic plaque with portions of attached media.

Additional Management

Dr. Silverman: After the procedure, the patient had an uneventful recovery and was discharged home on the fifth hospital day. He was seen 1 month after discharge in the stroke prevention clinic. There had been no further episodes of involuntary movements or choreoathetosis and no stroke or TIA. The patient continues to take aspirin, atorvastatin, and antihypertensive medications.

Final Diagnosis

Limb-shaking transient ischemic attacks.

病例28-2024：一名75岁女性，患有水肿、关节炎和蛋白尿

以下内容来源于新英格兰医学杂志。

Presentation of Case

Dr. Christine M. Parsons (Medicine): A 75-year-old woman was evaluated at this hospital because of arthritis, abdominal pain, edema, malaise, and fever.

Three weeks before the current admission, the patient noticed waxing and waning “throbbing” pain in the right upper abdomen, which she rated at 9 (on a scale of 0 to 10, with 10 indicating the most severe pain) at its maximal intensity. The pain was associated with nausea and fever with a temperature of up to 39.0°C. Pain worsened after food consumption and was relieved with acetaminophen. During the 3 weeks before the current admission, edema developed in both legs; it had started at the ankles and gradually progressed upward to the hips. When the edema began to affect her ambulation, she presented to the emergency department of this hospital.

A review of systems that was obtained from the patient and her family was notable for intermittent fever, abdominal bloating, anorexia, and fatigue that had progressed during the previous 3 weeks. The patient reported new orthopnea and nonproductive cough. Approximately 4 weeks earlier, she had had diarrhea for several days. During the 6 weeks before the current admission, the patient had lost 9 kg unintentionally; she also had had pain in the wrists and hands, 3 days of burning and dryness of the eyes, and diffuse myalgias. She had not had night sweats, dry mouth, jaw claudication, vision changes, urinary symptoms, or oral, nasal, or genital ulcers.

The patient’s medical history was notable for multiple myeloma (for which treatment with thalidomide and melphalan had been initiated 2 years earlier and was stopped approximately 1 year before the current admission); hypothyroidism; chikungunya virus infection (diagnosed 7 years earlier); seropositive erosive rheumatoid arthritis affecting the hands, wrists, elbows, and shoulders (diagnosed 3 years earlier); vitiligo; and osteoarthritis of the right hip, for which she had undergone arthroplasty. Evidence of gastritis was reportedly seen on endoscopy that had been performed 6 months earlier. Medications included daily treatment with levothyroxine and acetaminophen and pipazethate hydrochloride as needed for cough. The patient consumed chamomile and horsetail herbal teas. She had no known allergies to medications, but she had been advised not to take nonsteroidal antiinflammatory drugs after her diagnosis of multiple myeloma.

Approximately 5 months before the current admission, the patient had emigrated from Central America. She lived with her daughter and grandchildren in an urban area of New England. She had previously worked in health care. She had no history of alcohol, tobacco, or other substance use. There was no family history of cancer or autoimmune, renal, gastrointestinal, pulmonary, or cardiac disease.

On examination, the temporal temperature was 37.1°C, the heart rate 106 beats per minute, the blood pressure 152/67 mm Hg, and the oxygen saturation 100% while the patient was breathing ambient air. She had a frail appearance and bitemporal cachexia. The weight was 41 kg and the body-mass index (the weight in kilograms divided by the square of the height in meters) 15.2. Her dentition was poor; most of the teeth were missing, caries were present in the remaining teeth, and the mucous membranes were dry. She had abdominal tenderness on the right side and mild abdominal distention, without organomegaly or guarding. Bilateral axillary lymphadenopathy was palpable. Infrequent inspiratory wheezing was noted.

The patient had swan-neck deformity, boutonnière deformity, ulnar deviation, and distal hyperextensibility of the thumbs (Fig. 1). Subcutaneous nodules were observed on the proximal interphalangeal joints of the second and third fingers of the right hand and on the proximal interphalangeal joint of the fourth finger of the left hand. Synovial thickening of the metacarpophalangeal joints of the second fingers was noted. There was mild swelling and tenderness of the wrists. She had pain with flexion of the shoulders and right hip, and there was subtle swelling of the shoulders and right knee. Pitting edema (3+) and vitiligo were noted on the legs. No sclerodactyly, digital pitting, telangiectasias, appreciable calcinosis, nodules, nail changes (including pitting), or tophi were present. The remainder of the examination was normal.

Figure 1

Photograph of the Hands.

The blood levels of glucose, alanine aminotransferase, aspartate aminotransferase, bilirubin, globulin, lactate, lipase, magnesium, and phosphorus were normal, as were the prothrombin time and international normalized ratio; other laboratory test results are shown in Table 1. Urinalysis showed 3+ protein and 3+ blood, and microscopic examination of the sediment revealed 5 to 10 red cells per high-power field and granular casts. Urine and blood were obtained for culture. An electrocardiogram met (at a borderline level) the voltage criteria for left ventricular hypertrophy.

Table 1

Laboratory Data.

Dr. Rene Balza Romero: Computed tomography (CT) of the chest, abdomen, and pelvis, performed after the intravenous administration of contrast material, revealed scattered subcentimeter pulmonary nodules (including clusters in the right middle lobe and patchy and ground-glass opacities in the left upper lobe), trace pleural effusion in the left lung, coronary and valvular calcifications, and trace pericardial effusion, ascites, and anasarca. The scans also showed slight enlargement of the axillary lymph nodes (up to 11 mm in the short axis) bilaterally and a chronic-appearing compression fracture involving the T12 vertebral body.

Dr. Parsons: Morphine and lactated Ringer’s solution were administered intravenously. On the second day in the emergency department (also referred to as hospital day 2), the blood levels of haptoglobin, folate, and vitamin B₁₂ were normal; other laboratory test results are shown in Table 1. A rapid antigen test for malaria was positive. Wright–Giemsa staining of thick and thin peripheral-blood smears was negative for parasites; the smears also showed Döhle bodies and basophilic stippling. Antigliadin antibodies and anti–tissue transglutaminase antibodies were not detected. Tests for hepatitis A IgG and hepatitis C antibodies were positive. Tests for hepatitis B core and surface antibodies were negative. A test for human immunodeficiency virus type 1 (HIV-1) and type 2 (HIV-2) was negative.

Findings on abdominal ultrasound imaging performed on the second day (Fig. 2A and 2B) were notable for a small volume of ascites and kidneys with echogenic parenchyma. Ultrasonography of the legs showed no deep venous thrombosis. An echocardiogram showed normal ventricular size and function, aortic sclerosis with mild aortic insufficiency, moderate tricuspid regurgitation, a right ventricular systolic pressure of 39 mm Hg, and a small circumferential pericardial effusion. Intravenous hydromorphone was administered, and the patient was admitted to the hospital.

Figure 2

Imaging Studies of the Abdomen and Hands.

On the third day (also referred to as hospital day 3), nucleic acid testing for cytomegalovirus, Epstein–Barr virus, and hepatitis C virus was negative, and a stool antigen test for Helicobacter pylori was negative. An interferon-γ release assay for Mycobacterium tuberculosis was also negative. Oral acetaminophen and ivermectin and intravenous hydromorphone and furosemide were administered.

Dr. Balza Romero: Radiographs of the hands (Fig. 2C through 2F) showed joint-space narrowing of both radiocarpal joints and proximal interphalangeal erosions involving both hands. Radiographs of the shoulders showed arthritis of the glenohumeral joint and alignment suggestive of a tear of the right rotator cuff. A radiograph of the pelvis showed diffuse joint-space narrowing of the left hip, without osteophytosis, and an intact right hip prosthesis.

Dr. Parsons: Diagnostic tests were performed, and management decisions were made.

Differential Diagnosis

Dr. Beth L. Jonas: This patient is a 75-year-old woman who recently emigrated from Central America. She presented to this hospital with a multisystem disease involving the respiratory, gastrointestinal, renal, and musculoskeletal systems. Her medical history is notable for seropositive erosive rheumatoid arthritis and multiple myeloma, which had been treated with melphalan and thalidomide. Relevant clinical features on presentation include unintended weight loss and cachexia, axillary lymphadenopathy, serositis, cytopenia in two cell lines, hypocomplementemia, and elevated serum free kappa and lambda light-chain levels (with a normal free light-chain ratio) with no monoclonal spike. The white-cell count was elevated, but she had no eosinophilia. CT images of the chest showed scattered subcentimeter pulmonary nodules. With respect to the patient’s anemia, no schistocytes were present, the haptoglobin level was normal, and the iron studies were unremarkable. These findings, in combination with the elevated ferritin level, indicate anemia of chronic inflammation. The renal findings are most salient in the context of the patient’s hypertension, anasarca, elevated cystatin C level, active urinary sediment with proteinuria in the nephrotic range, and small, echogenic kidneys on ultrasonography.

In constructing a differential diagnosis, I will consider medication use, cancer, infectious disease, and autoimmune disease. Medications can be eliminated as the cause of this patient’s illness, since she was taking only levothyroxine, acetaminophen, and the antitussive agent pipazethate.

Cancer

The patient has a history of multiple myeloma, which may manifest with a multisystem disease involving the kidneys, but serum protein electrophoresis showed no monoclonal protein. Given the presence of nephrotic syndrome in the context of multiple myeloma, systemic immunoglobulin light-chain amyloidosis would be highest on the differential diagnosis with respect to cancer; however, the patient’s normal light-chain ratio makes this diagnosis unlikely. The development of myeloid neoplasms, such as acute myeloid leukemia, myelodysplastic syndromes, and myeloproliferative neoplasms, is important to consider in the context of previous treatment with alkylating agents,¹ which this patient had received. However, the peripheral-blood smear showed no findings that would indicate a hematologic cancer, and such a diagnosis would not explain the patient’s acute kidney injury with nephrotic-range proteinuria.

Infectious Disease

Several features of this patient’s case warrant special consideration, including her history of immunosuppression due to rheumatoid arthritis and to previously treated myeloma, along with the fact that she had emigrated from Central America, where certain infections may be prevalent. Infection with hepatitis A virus, hepatitis B virus, hepatitis C virus, HIV-1 and HIV-2, cytomegalovirus, Epstein–Barr virus, H. pylori, and M. tuberculosis can be ruled out on the basis of laboratory studies. A rapid antigen test for plasmodium species was reported to be positive, but this assay has a known cross-reactivity with rheumatoid factor.² Moreover, the thick and thin peripheral-blood smears were negative. Thus, malaria would be an unlikely diagnosis.

The patient has a history of infection with chikungunya virus, an arbovirus transmitted by a mosquito vector that has been responsible for large epidemics in the Americas since 2013.³ Acute symptoms include fever, rash, arthralgia, and myalgia. The development of a chronic arthritis that may meet the classification criteria for rheumatoid arthritis, as defined by the American College of Rheumatology and the European Alliance of Associations for Rheumatology, has been reported in up to 60% of patients infected with chikungunya virus.^4,5 In the context of this discussion, I considered whether chikungunya virus infection could be the cause of this patient’s symptoms, since this infection occurred before the diagnosis of rheumatoid arthritis. However, the degree of erosion and loss of joint space that was visible on radiographs would be most unusual for arthritis associated with chikungunya virus infection and would not explain the renal manifestations.

Strongyloidiasis is a helminth infection (caused by Strongyloides stercoralis) that is widespread in developing countries. Infection usually occurs through contact with soil, and most affected persons are asymptomatic. However, in immunosuppressed persons, strongyloides hyperinfection syndrome or a disseminated infection can develop as a consequence of accelerated autoinfection.⁶ The clinical presentation of strongyloides hyperinfection syndrome can include gastrointestinal symptoms (diarrhea, constipation, nausea, or vomiting), respiratory symptoms (cough, dyspnea, or wheezing), and rash due to migration of larvae through the subcutaneous tissues. Of note, only a minority of patients present with eosinophilia. Several case reports describe the development of nephrotic-range proteinuria, thrombotic microangiopathy, and IgA vasculitis in patients with strongyloides hyperinfection syndrome.^7-9 However, strongyloidiasis would not explain this patient’s cytopenias and hypocomplementemia.

Autoimmune Disease

The patient has a 3-year history of rheumatoid arthritis, although her clinical features of swan-neck deformity, boutonnière deformity, and joint instability suggest a longer duration of disease. We do not know whether she had received previous treatment with disease-modifying antirheumatic drugs or biologic agents, but the possible use of such treatments may be a consideration with respect to her progression of disease and overall degree of immunosuppression. The blood levels of rheumatoid factor and anti–cyclic citrullinated peptide antibodies were elevated, and radiographs of the hands showed erosive disease, although there was a relative paucity of metacarpophalangeal findings. A review of systems was negative for dry mouth, but her physical examination showed poor dentition and dry mouth — findings that make secondary Sjögren’s syndrome a consideration.

Renal disease can occur in patients with Sjögren’s syndrome. The two most typical presentations are tubulointerstitial nephritis and, less commonly, nephritic syndrome (membranoproliferative glomerulonephritis related to cryoglobulinemia). Tubulointerstitial nephritis may manifest with renal disease of varying severity, usually with a bland urinary sediment and often with abnormalities of tubular function such as distal renal tubular acidosis. Membranoproliferative glomerulonephritis caused by cryoglobulinemia is the most common glomerular disease associated with Sjögren’s syndrome. Although nephrotic-range proteinuria can occur with Sjögren’s syndrome, it is relatively uncommon.¹⁰ Renal disease is uncommon in patients with rheumatoid arthritis and is usually related to coexisting cardiovascular conditions. Medications used in the treatment of autoimmune disease — mainly nonsteroidal antiinflammatory drugs — may be associated with renal disease, but I would not expect the presence of an active urinary sediment, as was seen in this patient.

Amyloid A (AA) amyloidosis, a condition that is rare in the era of aggressive management of rheumatoid arthritis, has been described in patients with severe, long-standing seropositive erosive rheumatoid arthritis. Serum amyloid A (SAA) is a protein that is produced in the liver in response to chronic inflammation associated with interleukin-1, interleukin-6, and tumor necrosis factor α (TNF-α) in the context of chronic infections, autoimmune disease (classically rheumatoid arthritis), autoinflammatory disease, and cancers including renal cell carcinoma and non-Hodgkin’s lymphoma.¹¹ Signs and symptoms of AA amyloidosis are related to the deposition of the protein in organs, and patients often present with multisystem signs and symptoms. The kidney is the organ that is most often affected, but deposition can occur in the heart, gastrointestinal tract, nervous system, musculoskeletal system, and lungs. Proteinuria is the first clinical manifestation in almost 95% of patients with AA amyloidosis, and 50% of affected patients present with nephrotic syndrome.¹² The urinary sediment is generally bland, and complement levels in the blood are normal. AA amyloidosis remains on the differential diagnosis in this patient, but it would not completely explain her renal disease.

Hypocomplementemia

The key to this case is understanding the cause of this patient’s hypocomplementemia. Hypocomplementemia can be due to decreased complement production in the context of liver disease, congenital complement deficiency, or increased complement consumption resulting from activation of the innate immune system. This patient has no history of chronic liver disease and her laboratory test results indicated good hepatic synthetic function. Classical complement deficiency (including C4 deficiency) that begins early in life is associated with autoimmune disease, and early C3 deficiency is characterized by severe pyogenic infections. It would be unusual for a patient of this age to be deficient in both C3 and C4 without earlier clinical consequences. I therefore concluded that the hypocomplementemia in this case was related to complement consumption.

Rheumatic diseases that may be associated with prominent renal manifestations include antineutrophil cytoplasmic antibody–associated vasculitis, systemic sclerosis with renal crisis, cryoglobulinemic vasculitis, antiglomerular basement membrane disease, and systemic lupus erythematosus (SLE). Of those conditions, SLE would be the most likely to be manifested by an active urinary sediment and nephrotic-range proteinuria with consumption of both C3 and C4 in the context of fever, thrombocytopenia, and serositis. This patient’s fever, thrombocytopenia, and serositis also fit with this diagnosis.¹³

Because the patient has long-standing seropositive erosive rheumatoid arthritis, a diagnosis of AA amyloidosis is strongly suspected. Moreover, given the presence of thrombocytopenia, hypocomplementemia, and an active urinary sediment, I would recommend a kidney biopsy to evaluate for lupus nephritis and AA amyloidosis.

Dr. Beth L. Jonas’s Diagnosis

Overlap syndrome of rheumatoid arthritis and systemic lupus erythematosus with amyloid A amyloidosis.

Pathological Discussion

Dr. Claire Trivin-Avillach: Testing for autoimmune antibodies was performed. A test for antinuclear antibodies was positive at a titer of 1:5120 with a homogeneous pattern, and a test for anti–double-stranded DNA antibodies was positive at a titer of 1:2560.

The diagnostic procedure in this case was a core-needle biopsy of the kidney. Examination of the specimen with light microscopy revealed 20 glomeruli, 45% of which were globally sclerosed, along with fibrosis involving approximately 60% of the interstitium and tubular atrophy. Diffusely enlarged glomeruli with thickened capillary walls and an expanded mesangium were weakly positive on periodic acid–Schiff staining; the glomeruli stained pale blue on Masson’s trichrome staining. Congo red staining revealed metachromatic salmon-colored deposition involving the glomeruli, the blood-vessel walls, and the interstitium, which was associated with apple-green birefringence when viewed under polarized light (Fig. 3A). In addition, mesangial and endocapillary hypercellularity was identified in approximately 30% of the nonsclerosed glomeruli and was associated with karyorrhexis (Fig. 3B). One cellular crescent was also detected. These features are characteristic of active proliferative glomerulonephritis.

Figure 3

Biopsy Specimen of the Kidney.

Immunofluorescence microscopy revealed prominent granular staining for IgG (4+), IgM (4+), C3 (3+), C1q (3+), IgA (1+), kappa (3+), and lambda (3+) along the glomerular basement membranes and within the mesangium, as well as focal granular deposits of IgG and C3 along the tubular basement membrane (Fig. 3C and 3D). Additional immunofluorescence studies showed strong positivity (4+) for SAA within the glomeruli, the blood-vessel walls, and the interstitium (Fig. 3E), whereas staining for beta₂-microglobulin, transthyretin, and apolipoprotein A1 was faint.

Electron microscopy revealed the presence of subendothelial and mesangial electron-dense deposits (with no substructure identified) adjacent to randomly arranged fibrils (measuring 8.2 to 10.6 nm in diameter) within the glomerular basement membranes and the mesangium (Fig. 3F). Glomerular endothelial cells appeared reactive and contained tubuloreticular inclusions, features that were suggestive of interferon-mediated activation.

The findings on Congo red staining were characteristic of amyloidosis with typical birefringent material. The strong positivity of SAA within the deposits as compared with the faint staining of other reactants identified the type of amyloid as SAA, which is consistent with the patient’s history of rheumatoid arthritis. The biopsy also showed an immune complex–mediated proliferative glomerulonephritis with a “full house” pattern (defined as positivity for the three immunoglobulin classes IgG, IgM, and IgA and the two complement components C3 and C1q, in reference to the “full house” hand in a poker game). Immune complex–mediated proliferative glomerulonephritis has been reported in patients with rheumatoid arthritis who were receiving anti–TNF-α therapy,¹⁴ which was not the case in this patient. The positive test for hepatitis C antibodies prompted consideration of hepatitis C–related membranoproliferative glomerulonephritis. However, taken together, the negative nucleic acid test for hepatitis C virus, the full house pattern on immunofluorescence, the tubular basement membrane deposits, and the positive test for anti–double-stranded DNA antibodies favor a diagnosis of lupus nephritis of at least class III (defined as focal proliferative glomerulonephritis), according to the criteria of the International Society of Nephrology and the Renal Pathology Society, superimposed on AA amyloidosis.

Pathological Diagnosis

Proliferative lupus nephritis of International Society of Nephrology and Renal Pathology Society class III, superimposed on amyloid A amyloidosis.

Discussion of Management

Dr. Pui W. Cheung: On the basis of the finding of echogenic kidneys on ultrasonography and the findings of extensive interstitial fibrosis and tubular atrophy on kidney biopsy, we know that this patient has advanced chronic kidney disease that is unlikely to be reversible. The patient is also noted to have a markedly lower glomerular filtration rate (GFR) than that predicted by the blood creatinine level owing to the presence of cachexia, and this is substantiated by the cystatin C–based GFR and a 24-hour creatinine clearance of 22 ml per minute per 1.73 m² of body-surface area. The typical induction therapy for stage III or IV lupus nephritis consists of high-dose glucocorticoids and either mycophenolate mofetil or cyclophosphamide. Other reasonable alternatives for initial therapy include mycophenolate mofetil in combination with either a calcineurin inhibitor or belimumab, or cyclophosphamide in combination with belimumab.¹⁵ Hydroxychloroquine is also recommended as part of the therapy, since it has shown benefits in improving the response to treatment and reducing disease flare.¹⁶ Mycophenolate mofetil and cyclophosphamide have similar efficacy with respect to clinical response, which includes a reduction in proteinuria and either an improvement in renal function or stabilization of renal function; the risks of infections and adverse events associated with these medications are also similar.^17,18

Given the severity of the lupus nephritis with overlying AA amyloidosis from active rheumatoid arthritis, the treatment options proposed were high-dose glucocorticoids and rituximab with either mycophenolate mofetil or cyclophosphamide.¹⁹ After discussions with multidisciplinary consultants from rheumatology, infectious diseases, and nephrology, lingering concerns were raised about infection and patient frailty; ultimately, the decision was made to initiate high-dose glucocorticoid therapy in combination with mycophenolate mofetil, rituximab, and hydroxychloroquine.

The patient’s mycophenolate mofetil dose regimen was inconsistent owing to gastrointestinal side effects, and the treatment was eventually withheld because of pancytopenia and fever. Unfortunately, her kidney function worsened, and renal replacement therapy was initiated within 3 weeks after the start of the induction therapy. The cause of her renal failure was thought to be disease progression, compounded by hemodynamically mediated tubular injury in the context of infection. While the administration of mycophenolate mofetil was stopped, treatment with rituximab was continued, with slow tapering of the glucocorticoid dose at the direction of the rheumatologist. She remained dependent on dialysis and was deemed to have end-stage kidney disease after 3 months of dialysis.

Dr. Lisa G. Criscione-Schreiber: The patient has SLE with nephritis, seropositive erosive rheumatoid arthritis, and systemic AA amyloidosis. AA amyloidosis is rare owing to the availability of effective therapies for rheumatoid arthritis and is managed through aggressive treatment of inflammation due to rheumatoid arthritis. Reports addressing the management of rheumatoid arthritis–induced AA amyloidosis generally cite stability of end-organ damage caused by AA amyloid as evidence of effective management of the condition (through treatment of the inflammation of rheumatoid arthritis). Methotrexate, the cornerstone of treatment for rheumatoid arthritis, is contraindicated in this case owing to the presence of kidney disease. The alkylating agent cyclophosphamide has been reported to be effective for the treatment of AA amyloidosis from rheumatoid arthritis²⁰ and has known efficacy in patients with lupus nephritis, both of which make it a viable treatment option. Rituximab has also been reported to be effective for managing rheumatoid arthritis–induced AA amyloidosis,²¹ is approved for the treatment of rheumatoid arthritis, and is used for manifestations of SLE, including thrombocytopenia and nephritis. Although anti–TNF-α agents, abatacept, and Janus kinase inhibitors are reported to be effective for the treatment of AA amyloidosis in patients with rheumatoid arthritis,²² recent publications have coalesced on the ability of anti–interleukin-6 therapy to block interleukin-6–induced hepatic production of SAA.^23-25

The overlap of seropositive erosive rheumatoid arthritis and SLE (sometimes termed “rhupus”) usually resembles rheumatoid arthritis more than SLE; manifestations include thrombocytosis, leukocytosis, an elevated erythrocyte sedimentation rate, an elevated blood level of C-reactive protein, and the presence of marginal erosions on radiographs.²⁶ In contrast, SLE without seropositive erosive rheumatoid arthritis characteristically manifests with thrombocytopenia, leukopenia, and an elevated erythrocyte sedimentation rate but usually not an elevated C-reactive protein level; in addition, nonerosive inflammatory arthritis with reversible deformities is commonly observed. This patient had a mixed laboratory profile, on the basis of the results of antinuclear antibody and anti–double-stranded DNA antibody tests. The challenge of treating an overlap syndrome of rheumatoid arthritis and SLE is choosing disease-modifying antirheumatic drugs that are effective and safe in both conditions. This patient’s most severe disease manifestation is lupus nephritis; therefore, the treatment regimen must target nephritis along with the AA amyloidosis and inflammatory arthritis.

As noted earlier, current induction therapy for lupus nephritis includes either mycophenolate mofetil or cyclophosphamide. Mycophenolate mofetil may provide inadequate treatment of the rheumatoid arthritis and amyloidosis, whereas cyclophosphamide would treat the lupus nephritis, has possible efficacy for treatment of the AA amyloidosis, and would treat the rheumatoid arthritis. Rituximab could be added to cyclophosphamide or mycophenolate mofetil to treat the rheumatoid arthritis and resultant AA amyloidosis and could also possibly help treat the lupus nephritis. The addition of anti–interleukin-6 therapy to mycophenolate mofetil or cyclophosphamide is an intriguing option that may effectively treat the rheumatoid arthritis and subsequent AA amyloidosis. The addition of belimumab to mycophenolate mofetil or cyclophosphamide has been reported to improve renal response in patients with lupus nephritis,²⁷ as has the addition of voclosporin to mycophenolate mofetil.²⁸ However, belimumab is ineffective for the treatment of rheumatoid arthritis, and voclosporin has not been studied in patients with rheumatoid arthritis or in those with a GFR of 45 milliliters per minute or less. The high-dose glucocorticoids that are used in induction therapy for lupus nephritis will effectively manage this patient’s inflammatory arthritis and probably also the subsequent AA amyloidosis. Finally, it is important that every patient with lupus nephritis receive hydroxychloroquine, which improves the treatment response to induction therapy.²⁹

Follow-up

Dr. Parsons: The patient’s hospital course was further complicated by suspected immune-mediated thrombocytopenia, for which she received intravenous immune globulin. Her pancytopenia and arthritis ultimately abated. Unfortunately, she did not have renal recovery and continues to receive hemodialysis. After a prolonged hospital course, she was discharged home.

Final Diagnosis

Overlap syndrome of rheumatoid arthritis and systemic lupus erythematosus complicated by proliferative lupus nephritis, superimposed on amyloid A amyloidosis.