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



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.



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).



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.
Department of Rheumatology, Salford Royal NHS Foundation Trust, Stott Lane, Manchester, M6 8HD, UK. Electronic address: Charlotte.a.sharp@gmail.com.
Charlotte A
CA
Sharp
Department of Rheumatology, Salford Royal NHS Foundation Trust, Stott Lane, Manchester, M6 8HD, UK.
Qasim
Q
Akram
Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Stott Lane, Manchester, M6 8HD, UK.
Michael
M
Hughes
Department of Orthopedic Surgery, Salford Royal NHS Foundation Trust, Stott Lane, Manchester, M6 8HD, UK.
Lindsay
L
Muir
Centre for Musculoskeletal Research, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Stott Lane, Manchester, M6 8HD, UK; NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, 29 Grafton Street, Manchester, M13 9WU, UK.
Ariane L
AL
Herrick
D000328:Adult; D000368:Aged; D003937:Diagnosis, Differential; D005260:Female; D005385:Fingers / Q000098:blood supply*; D006801:Humans; D007511:Ischemia / Q000175:diagnosis* / Q000209:etiology; D008875:Middle Aged; D011928:Raynaud Disease / Q000175:diagnosis* / Q000209:etiology; D012595:Scleroderma, Systemic / Q000150:complications*; D014034:Toes / Q000098:blood supply*
Critical digital ischemia; Digital ulcer; Digital vasculopathy; Embolism; Raynaud’s phenomenon; Scleroderma; Systemic sclerosis; Vasculitis