Protein strongZstrong Deficiency in Antiphospholipid-Negative Sneddon’s.pdfVIP

Protein Z Deficiency in Antiphospholipid-Negative Sneddon’s Syndrome Nakhle´ Ayoub, MD; Gaetan Esposito, Dr; Ste´phane Barete, MD; Claudine Soria, PhD; Jean-Charles Piette, MD; Camille France`s, MD Background—Sneddon’s syndrome is characterized by the association of ischemic cerebrovascular events and widespread livedo racemosa. The pathophysiology of Sneddon’s syndrome remains elusive, but various prothrombotic abnormal- ities have been previously reported in this setting. Low levels of protein Z, a downregulator of coagulation, have been recently linked to an increased risk of arterial thrombosis. The purpose of this study was to investigate the levels of protein Z in a series of Sneddon’s syndrome patients without circulating antiphospholipid antibodies in comparison with an age- and sex-matched control population. Methods—Twenty-six patients and 78 healthy controls had determination of their protein Z blood levels by an enzyme-linked immunoassay test. Patients’ thrombotic and vascular risk factors, including tobacco smoking, arterial hypertension, oral contraceptive agents, dyslipidemia, factor V Leiden, and factor II mutation were recorded. Results—Protein Z plasma levels were significantly lower in patients (mean 1.47 mg/L) than in controls (mean 1.93 mg/L) (P 0.02). Prevalence of protein Z deficiency (level 1 mg/L) was significantly higher (P 0.001) among patients (31%) than among controls (3.8%). Factor V Leiden and heavy smoking were observed in 4 and 7 patients, respectively. Conclusions—Sneddon’s syndrome could be viewed as the peculiar clinical expression of various and sometimes associated coagulation abnormalities. Low levels of protein Z may account, at least partly, for the thrombot