Review Article

Vasculitis in Systemic Sclerosis

Table 1

Seven cases of large-vessel vasculitis associated with SSc.

CaseVasculitisAge/SexAge at
SSc Dx
SSc
type
Age at Vasculitis DxPresentationDiagnosisOutcome

Perez-Jimenez
et al. [13]
GCA68/F68L70Headache, jaw claudication;
ESR 53
Temporal artery biopsy: inflammatory infiltrate, giant cellsPrednisone 60 mg daily x 5 weeks then tapered. Symptom-free at 5 years

Hupp [14]GCA70/F?L70Headache, jaw claudication;
ESR 53
Temporal artery biopsy: mononuclear infiltrate, giant cells, destruction of internal elastic laminaPrednisone 80 mg daily, tapered to 30 mg daily over 5 months. Symptom-free and ESR 3 at
5 months.

Sari-Kouzel
et al. [15]
GCA64/F49L64Gangrene of right 2nd and 3rd toes; ESR 14Right knee amputation biopsy: mononuclear infiltrate, giant cells, vascular lumen occlusionPrednisolone 30 mg daily, tapered to 10 mg daily over 6 months. Symptom-free at 6 months.

Passiu et al. [16]TA29/F29D21???

Yago et al. [17]TA68/F66D66Vertigo, bruits in neck, abdomen, and back; asymmetric blood pressure
in arms
Stenosis of right brachiocephalic trunk, celiac and left renal arteries?

Kocabay et al. [18]TA48/F48D47Pulseless in both arms with intermittent claudicationObliteration of bilateral subclavian arteries distal to vertebral artery bifurcation?

Kim et al. [19]TA37/F33L37Weak left radial pulseStenosis of right common carotid artery, right external carotid artery, thoracic and abdominal aorta,
left brachial artery compression by scalenus muscle on abduction
?

GCA = giant cell arteritis; TA = Takayasu’s arteritis; SSc = systemic sclerosis; L = limited; D = diffuse; ESR = erythrocyte sedimentation rate.