Shanmugam Victoria K, Price Patricia, Attinger Christopher E, Steen Virginia D
Division of Rheumatology, Immunology and Allergy, Georgetown University Hospital, 3800 Reservoir Road, N.W., Hington, DC 20007, USA.
Int J Rheumatol. 2010;2010. doi: 10.1155/2010/747946. Epub 2010 Aug 18.
Nondigital lower extremity ulcers are a difficult to treat complication of scleroderma, and a significant cause of morbidity. The purpose of this study was to evaluate the prevalence of nondigital lower extremity ulcers in scleroderma and describe the associations with autoantibodies and genetic prothrombotic states. A cohort of 249 consecutive scleroderma patients seen in the Georgetown University Hosptial Division of Rheumatology was evaluated, 10 of whom had active ulcers, giving a prevalence of 4.0%. Patients with diffuse scleroderma had shorter disease duration at the time of ulcer development (mean 4.05 years ± 0.05) compared to those with limited disease (mean 22.83 years ± 5.612, P value .0078). Ulcers were bilateral in 70%. In the 10 patients with ulcers, antiphospholipid antibodies were positive in 50%, and genetic prothrombotic screen was positive in 70% which is higher than expected based on prevalence reports from the general scleroderma population. Of patients with biopsy specimens available (n = 5), fibrin occlusive vasculopathy was seen in 100%, and all of these patients had either positive antiphospholipid antibody screen, or positive genetic prothrombotic profile. We recommend screening scleroderma patients with lower extremity ulcers for the presence of anti-phospholipid antibodies and genetic prothrombotic states.
非指（趾）部下肢溃疡是硬皮病一种难以治疗的并发症，也是发病的重要原因。本研究的目的是评估硬皮病中非指（趾）部下肢溃疡的患病率，并描述其与自身抗体和遗传性血栓前状态的关联。对在乔治敦大学医院风湿病科连续就诊的249例硬皮病患者进行了评估，其中10例有活动性溃疡，患病率为4.0%。与局限性硬皮病患者相比，弥漫性硬皮病患者在溃疡发生时的病程较短（平均4.05年±0.05），而局限性硬皮病患者病程平均为22.83年±5.612，P值为0.0078。70%的溃疡为双侧性。在10例有溃疡的患者中，50%的抗磷脂抗体呈阳性，70%的遗传性血栓前筛查呈阳性，这高于基于硬皮病总体人群患病率报告的预期。在有活检标本的患者中（n = 5），100%可见纤维蛋白闭塞性血管病，所有这些患者的抗磷脂抗体筛查或遗传性血栓前谱均呈阳性。我们建议对患有下肢溃疡的硬皮病患者筛查抗磷脂抗体和遗传性血栓前状态。
