風濕性疾病相關(guān)綜合征

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)三聯(lián)征：三聯(lián)征：Rheumatoid arthritis (RA)Neutropenia (中性粒細胞減少中性粒細胞減少)Splenomegaly (脾大脾大)并發(fā)癥：并發(fā)癥：反復感染反復感染脾亢所致貧血及血小板減少脾亢所致貧血及血小板減少淋巴結(jié)腫大淋巴結(jié)腫大皮膚色素沉著皮膚色素沉著&皮膚潰爛皮膚潰爛肝臟活檢示結(jié)節(jié)性再生肝臟活檢示結(jié)節(jié)性再生Image donated by: Professor Ian Lauder Donor organization: Leicester University,

4、Department of Pathology 結(jié)節(jié)性竇狀淋巴細胞結(jié)節(jié)性竇狀淋巴細胞*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條中的條中的3條即可診斷條即可診斷局限性硬皮病局限性硬皮病 雷諾現(xiàn)象雷諾現(xiàn)象 指端潰瘍指端潰瘍 毛細血管擴張毛細血管擴張 ACE血管緊張素

5、轉(zhuǎn)換酶血管緊張素轉(zhuǎn)換酶; HSCT造血干細胞移植造血干細胞移植ARBs血管緊張素受體阻滯劑血管緊張素受體阻滯劑; IVIG靜脈注射免疫球蛋白靜脈注射免疫球蛋白ATG抗胸腺細胞球蛋白抗胸腺細胞球蛋白; PDE5磷酸二酯酶磷酸二酯酶5抑制劑抑制劑CCB鈣通道阻滯劑鈣通道阻滯劑; PT/OT物理物理/職業(yè)治療職業(yè)治療EGD食道胃十二指腸鏡食道胃十二指腸鏡; TNF腫瘤壞死因子腫瘤壞死因子GAVE胃竇血管擴張癥胃竇血管擴張癥; GI胃腸道胃腸道Churg-Strauss syndromeSinico, 2005, Sable-Fourtassaou, 2005, Sinicio 2009 (revie

6、w)Kallenberg, Nature Clinical Practice. Rheumatology, 2006Churg-Strauss Syndrome: 診斷診斷Guillevin, Medicine, 1996Churg-Strauss Syndrome: 診斷診斷Hemophagocytic syndrome (嗜血綜合征嗜血綜合征)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：骨髓活檢顯示巨噬細胞的核組成及背景中的細胞碎片上左：骨髓活檢顯示巨噬細胞的核組成及背景中的細胞碎片上右：骨髓活檢的上右：骨髓活檢的CD68染色突顯無數(shù)被巨噬細胞吞噬的細胞的核染色突顯無數(shù)被巨噬細胞吞噬的細胞的核下：骨髓穿刺涂片顯示的嗜血細胞下：骨髓穿刺涂片顯示的嗜血細胞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綜合征患者手背的凹陷性水腫綜合征患者手背的凹陷性水腫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根據(jù)基因缺陷以及受累的膠原類型不同，臨床上根據(jù)基因缺陷以及受累的膠原類型不同，臨床上將將Ehlers-Danlos syndrome分為

10、分為11型型：Sweets syndromeSweets syndrome: 定義定義Images from wikipediaSweets syndrome: 定義定義高達高達50% 的患者有潛在的疾?。旱幕颊哂袧撛诘募膊。?.惡性腫瘤惡性腫瘤：21% Sweets syndrome患者合并惡性腫瘤；其中患者合并惡性腫瘤；其中15%為血液系統(tǒng)的惡性腫瘤，為血液系統(tǒng)的惡性腫瘤，AML最常見；最常見；6%為實體腫瘤，為實體腫瘤，泌尿道、消化道及乳腺腫瘤最常見泌尿道、消化道及乳腺腫瘤最常見2.感染感染：最常見的為上呼吸道及消化道感染，如鏈球菌，結(jié)：最常見的為上呼吸道及消化道感染，如鏈球菌，結(jié)核分枝

11、桿菌，耶爾森氏菌，沙門氏菌，志賀氏菌感染核分枝桿菌，耶爾森氏菌，沙門氏菌，志賀氏菌感染3.炎癥性腸病炎癥性腸病4.懷孕懷孕5.其他其他疾病：如結(jié)節(jié)病、類風濕關(guān)節(jié)炎、甲狀腺疾病疾?。喝缃Y(jié)節(jié)病、類風濕關(guān)節(jié)炎、甲狀腺疾?。℅raves病和橋本甲狀腺炎）等病和橋本甲狀腺炎）等Paraneoplastic Rheumatologic Syndromes(風濕性副癌綜合征風濕性副癌綜合征)風濕性副癌綜合征風濕性副癌綜合征 皮肌炎皮肌炎 (Dermatomyositis)風濕性副癌綜合征風濕性副癌綜合征 蘭伯特蘭伯特 - 伊頓肌無力綜合征伊頓肌無力綜合征(Lambert-Eaton myasthenic syndrome)Abu-Shakra M. Paraneoplastic Rheumatologic Syndr