Anticentromere antibody as a predictor of digital ischemic loss in patients with systemic sclerosis

Abstract

Objective. To determine the clinical and serologic risk factors for digital ischemic events in patients with systemic sclerosis (SSc).Methods. Systemic sclerosis (SSc; scleroderma) is a heterogenous disorder with a variable course and out-

Current concepts in normal and defective angiogenesis: Implications for systemic sclerosis

“…Anticentromere autoantibodies are most often found in patients with l-SSc who will most likely develop advanced Raynaud's phenomenon, PAH, and digital ulcers [14], whereas antiScl-70 autoantibodies are associated with d-SSc patients who are likely to develop diffuse cutaneous disease and pulmonary fibrosis [6].…”

“…SSc patients with limited cutaneous disease frequently develop extreme tissue ischemia that results in ischemic digital loss (IDL) [14]. To examine the possibility that the two autoantigens cleaved exclusively by granzyme B-CENP-B and fibrillin-may be associated with IDL, HeLa cell lysates and chromosome preparations were incubated with or without granzyme B and then immunoblotted with sera from patients with limited SSc and IDL or limited SSc and no IDL.…”

Systemic sclerosis presenting with severe digital ischemia. A rare case report

“…[2] Out of two prospective cohort studies in UK , one study found that 28 (16%) of 171 SSc patients had at least one digital amputation and 73 (43%) had experienced at least one episode of severe digital ischemia, [3, and in another study 17.4% were found to have complications related to severe digital vasculopathy including digital ulcers, critical digital ischemia, gangrene, or the need for digital sympathectomy. Furthermore, 1.6% of the total cohort developed critical digital ischemia, 1.4% developed digital gangrene, and 0.9% required amputation.…”

Vascular Disease in Scleroderma

“…A survey of patients at one center reported that younger patients with high Rodnan skin score were at higher risk for developing ulcers, and they usually occurred within 5 years of non-Raynaud's symptoms [10]. Digital amputation secondary to occlusion of digital arteries occur in a subset of (11%) patients, usually with limited skin disease with the presence of anticentromere antibody [11][12][13].…”

“…In scleroderma patients, anticentromere antibodies have been significantly associated with thrombotic manifestations such as digital ischemia [226], and antiendothelial cell antibodies have been found that bind to centromeric protein B [227]. This suggests that antiendothelial antibodies target nonendothelial cell specific antigens.…”