Differential diagnosis of critical digital ischemia in systemic sclerosis: Report of five cases and review of the literature

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• 作者：Charlotte A. Sharp ; MBChB ; MRCP^a ; ^{Charlotte.a.sharp@gmail.com" class="auth_mail" title="E-mail the corresponding author} ; Qasim Akram ; MBChB ; MRCP^a ; Michael Hughes ; MSc ; MRCP^b ; Lindsay Muir ; MB ; MCh (Orth) ; FRCS (Orth)^c ; Ariane L. Herrick ; MD ; FRCP^b ; ^d ; ¹
• 关键词：Systemic sclerosis ; Scleroderma ; Digital vasculopathy ; Raynaud&rsquo ; s phenomenon ; Critical digital ischemia ; Digital ulcer ; Embolism ; Vasculitis
• 刊名：Seminars in Arthritis and Rheumatism
• 出版年：2016
• 出版时间：October 2016
• 年：2016
• 卷：46
• 期：2
• 页码：209-216
• 全文大小：902 K

文摘

Critical digital ischemia is a rare, but serious complication of systemic sclerosis (SSc) and is not always due solely to the non-inflammatory angiopathy that characterizes the SSc disease process. Our objective was to illustrate the range of presentations and causes of critical digital ischemia in patients with SSc in order to highlight how optimal management is dependent upon establishing the correct diagnosis.

Methods

Five cases exemplifying differential diagnoses were identified and their case notes reviewed in order to extract clinically relevant data and images. A review of the literature was performed in PubMed in English.

Results

Causes of critical digital ischemia included typical micro-angiopathic changes and proximal (large vessel) disease. One case highlighted the difficulty of ascertaining whether an inflammatory cause is also present in SSc/SLE overlap syndrome. Two cases demonstrated embolic causes (thromboembolism due to atrial fibrillation and septic emboli).

Conclusions

Critical digital ischemia in patients with SSc requires thorough investigation in order to avoid missing additional potentially modifiable causes including large vessel disease, inflammation, embolism, infection, and paraneoplastic syndromes. A firm evidence base for current medical and surgical interventions is lacking, highlighting the need for further research into the optimum management of this rare, but painful, debilitating, and limb-threatening complication of SSc.