风湿性疾病相关综合征

1、Caplans syndromePalindromic syndrome Shrinking lung syndromeThrombotic thrombocytopenic purpurahemolytic uremic syndrome (TTP-HUS) Neuropsychiatric lupus (NPSLE) syndromeLambert-Eaton myasthenic syndrome (LEMS)Sweets syndrome Chronic Infantile Neurologic Cutaneous and Articular (CINCA) SyndromeParry

2、-Romberg syndromeStiff skin syndromeWerners syndrome Shulmans syndrome or fasciitis-panniculitis syndromeCarcinoid syndromeEosinophilia-myalgia syndromeChronic fatigue syndrome Toxic oil syndromeParry-Romberg syndrome Hypocomplementemic urticarial vasculitis syndromeHypereosinophilic syndrome Papula

3、r-purpuric gloves and socks syndrome (PPGSS)三联征：三联征：Rheumatoid arthritis (RA)Neutropenia (中性粒细胞减少中性粒细胞减少)Splenomegaly (脾大脾大)并发症：并发症：反复感染反复感染脾亢所致贫血及血小板减少脾亢所致贫血及血小板减少淋巴结肿大淋巴结肿大皮肤色素沉着皮肤色素沉着&皮肤溃烂皮肤溃烂肝脏活检示结节性再生肝脏活检示结节性再生Image donated by: Professor Ian Lauder Donor organization: Leicester University,

4、Department of Pathology 结节性窦状淋巴细胞结节性窦状淋巴细胞*Narvez J et al. Biological agents in the management of Feltys syndrome: a systematic review. Semin Arthritis Rheum. 2012 ;41(5):658-68. Images from Kelleys RheumatologyPONCET syndrome具有具有5条中的条中的3条即可诊断条即可诊断局限性硬皮病局限性硬皮病 雷诺现象雷诺现象 指端溃疡指端溃疡 毛细血管扩张毛细血管扩张 ACE血管紧张素

5、转换酶血管紧张素转换酶; HSCT造血干细胞移植造血干细胞移植ARBs血管紧张素受体阻滞剂血管紧张素受体阻滞剂; IVIG静脉注射免疫球蛋白静脉注射免疫球蛋白ATG抗胸腺细胞球蛋白抗胸腺细胞球蛋白; PDE5磷酸二酯酶磷酸二酯酶5抑制剂抑制剂CCB钙通道阻滞剂钙通道阻滞剂; PT/OT物理物理/职业治疗职业治疗EGD食道胃十二指肠镜食道胃十二指肠镜; TNF肿瘤坏死因子肿瘤坏死因子GAVE胃窦血管扩张症胃窦血管扩张症; GI胃肠道胃肠道Churg-Strauss syndromeSinico, 2005, Sable-Fourtassaou, 2005, Sinicio 2009 (revie

6、w)Kallenberg, Nature Clinical Practice. Rheumatology, 2006Churg-Strauss Syndrome: 诊断诊断Guillevin, Medicine, 1996Churg-Strauss Syndrome: 诊断诊断Hemophagocytic syndrome (嗜血综合征嗜血综合征)Flavia G. N. Rosado, Annette S. Kim. Hemophagocytic Lymphohistiocytosis: An Update on Diagnosis and Pathogenesis. Am J Clin P

7、athol 2013;139:713-727.上左：骨髓活检显示巨噬细胞的核组成及背景中的细胞碎片上左：骨髓活检显示巨噬细胞的核组成及背景中的细胞碎片上右：骨髓活检的上右：骨髓活检的CD68染色突显无数被巨噬细胞吞噬的细胞的核染色突显无数被巨噬细胞吞噬的细胞的核下：骨髓穿刺涂片显示的嗜血细胞下：骨髓穿刺涂片显示的嗜血细胞Henter JI, Horne A, Arico M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood C

8、ancer. 2007;48:124-131.SAPHO syndromeImage provided by Dr. Isabel Bielsa RS3PE综合征患者手背的凹陷性水肿综合征患者手背的凹陷性水肿Betsab Serrano Ostoa et al. Remitting Symmetric Seronegative Synovitis With Pitting Edema (RS3PE). Reumatol Clin. 2012;8(3):156157Olive A, del Blanco J, Pons M, et al. The clinical spectrum of rem

9、itting seronegative symmetrical synovitis with pitting edema. The Catalan Group for the Study of RS3PE. J Rheumatology 1997; 24: 333-36Zenone T. Cogan syndrome. Presse Med.2013;42:951-60. Images provided by King Abdulaziz University根据基因缺陷以及受累的胶原类型不同，临床上根据基因缺陷以及受累的胶原类型不同，临床上将将Ehlers-Danlos syndrome分为

10、分为11型型：Sweets syndromeSweets syndrome: 定义定义Images from wikipediaSweets syndrome: 定义定义高达高达50% 的患者有潜在的疾病：的患者有潜在的疾病：1.恶性肿瘤恶性肿瘤：21% Sweets syndrome患者合并恶性肿瘤；其中患者合并恶性肿瘤；其中15%为血液系统的恶性肿瘤，为血液系统的恶性肿瘤，AML最常见；最常见；6%为实体肿瘤，为实体肿瘤，泌尿道、消化道及乳腺肿瘤最常见泌尿道、消化道及乳腺肿瘤最常见2.感染感染：最常见的为上呼吸道及消化道感染，如链球菌，结：最常见的为上呼吸道及消化道感染，如链球菌，结核分枝

11、杆菌，耶尔森氏菌，沙门氏菌，志贺氏菌感染核分枝杆菌，耶尔森氏菌，沙门氏菌，志贺氏菌感染3.炎症性肠病炎症性肠病4.怀孕怀孕5.其他其他疾病：如结节病、类风湿关节炎、甲状腺疾病疾病：如结节病、类风湿关节炎、甲状腺疾病（Graves病和桥本甲状腺炎）等病和桥本甲状腺炎）等Paraneoplastic Rheumatologic Syndromes(风湿性副癌综合征风湿性副癌综合征)风湿性副癌综合征风湿性副癌综合征 皮肌炎皮肌炎 (Dermatomyositis)风湿性副癌综合征风湿性副癌综合征 兰伯特兰伯特 - 伊顿肌无力综合征伊顿肌无力综合征(Lambert-Eaton myasthenic syndrome)Abu-Shakra M. Paraneoplastic Rheumatologic Synd