- 期刊
Raynaud's Phenomenon As A Rare Presentation of Occult Acquired Immune Deficiency Syndrome ── Possible Pathogenesis and Surgical Management
在隱性的後天免疫缺乏症候群個案裡罕見的以雷諾式徵候群表現－可能的疾病機轉與手術治療
摘要
Background: Raynaud’s phenomenon (RP) is described as episodic, symmetric, acral vasospasm characterized by pallor, cyanosis and blushing of fingers or toes due to secondary causes such as scleroderma or mixed connective tissue disease. Here we present a rare case that developed digital ischemia caused by vasospastic vessels related to Raynaud’s phenomenon due to acquired immune deficiency syndrome (AIDS)-induced cryoglobulinemia and anti-phospholipid syndrome. Aim and Objective: We described the possible pathogenesis and surgical management of this clinical presentation. Material and Methods: A 40-year-old Asian man developed Raynaud’s phenomenon of the bilateral long, ring and little fingers. AIDS was diagnosed unexpectedly with cryoglobulinemia and anti-phospholipid syndrome after negative results of thorough autoimmune and other viral infection surveys. Digital ischemia developed quickly over six months and proceeded to distal digital gangrene of bilateral little fingers. Surgical exploration revealed bilateral 2nd - 4th common palmar digital artery vasospasm with fibrotic bands mainly compressing the left 4th common palmar digital artery. Besides, a firm mass was found occupying a branch of the left common palmar digital nerves of ulnar nerve, compressing the left 3rd common digital artery. The digital neuroma was excised and periarterial sympathectomy was performed on bilateral 2nd, 3rd and 4th common digital arteries combined with digital arterial reconstruction. Results: The progression of digital ischemia was stopped and digital spasm did not recur after periarterial sympathectomy combined with digital arterial reconstruction atone-year follow up. Conclusion: AIDS should be highly suspected in a clinical presentation of acquired RP with no evidence of viral hepatitis or auto-immune disease such as scleroderma, systemic lupus erythematosus or mixed connective tissue disease but only a high level of cryoglobulin and antiphospholipid antibodies. We propose a possible pathogenesis and surgical management for this kind of clinical presentation.
並列摘要
背 景：雷諾氏徵候群的臨床特徵為偶發和對稱的肢端血管痙攣，在手指或腳趾表現出於蒼白，發紺和潮紅的表現。常由於硬皮病或混合結締組織病所造成。我們在這篇文章會提出一個後天免疫缺乏症候群引起冷凝球蛋白症和抗磷脂症候群進而導致雷諾氏徵候群而表現出手指缺血的個案。目 的：我們描述了這個案可能的發病機轉和手術治療方式。方 法：一名40 歲的亞裔男子患有冷球蛋白血症，抗磷脂和後天免疫缺乏症候群，表現出雙側第三至五手指的雷諾氏徵候群。手指缺血已迅速發展超過六個月，並惡化至雙邊第五遠端手指壞疽。手術探查發現雙側第二至四總掌指動脈血管痙攣與有纖維化組織主要壓迫在左側第三總掌指動脈上。此外，有一腫塊在左側第三總掌指神經中間也壓迫在第三總掌指動脈上。於是進行神經瘤切除，雙側第二，第三和第四總掌指動脈周圍的交感神經切除術，及指動脈重建術。結 果：術後指端缺血的情形停止，指動脈痙攣在手術後一年的追蹤亦未復發。結 論：在臨床上有雷諾氏徵候群的表現，在沒有病毒性肝炎和自體免疫疾病的證據，如硬皮病，系統性紅斑狼瘡或混合性結締組織病，只有高的冷凝球蛋白和冷凝纖維蛋白原指數時，應懷疑有感染愛滋病的可能。我們提出了一個對於這種臨床表現的可能的發病機轉和手術治療。