Antiphospholipid syndrome in northwest Italy (APS Piedmont Cohort): demographic features, risk factors, clinical and laboratory profile

Abstract

We report the experience from the Antiphospholipid Antibodies (aPL) Regional Consortium in northwest Italy, meant to support clinical research and foster collaboration among health professionals regarding the diagnosis and management of antiphospholipid syndrome (APS) patients. This cohort-study (APS Piedmont Cohort) was designed to register the clinical characteristics at inception and associated immunological manifestations at diagnosis (if any) of patients who strictly fulfilled the current criteria for APS, all recruited at the Piedmont and Valle d'Aosta regions. Clinical and laboratory data from 217 APS patients (171 with vascular events, 33 with pregnancy morbidity and 13 with both), from 16 centres within the geographical area were collected. Venous thrombosis was recorded in 45.6% of patients, arterial thrombosis in 35%, small-vessel thrombosis in 1.12% and mixed arterial and venous thrombosis in the remaining 19.4% of the cases. Pregnancy morbidity included 19 patients with unexplained fetal death beyond the 10th week of pregnancy, 17 with premature birth before the 34th week and 10 with three or more unexplained spontaneous abortions before the 10th week of gestation. This consortium represents an instrument by which to audit clinical practice, to provide counselling to local centres and to sustain future basic and clinical APS research.

Thrombotic recurrences and bleeding events in APS vascular patients: A review from the literature and a comparison with the APS Piedmont Cohort

“…It must be underlined that a great deal of these patients was under OAT, and the higher the INR intensity, the higher the recurrence rate. Thrombotic recurrences are generally higher (when calculated) in the first year of followup [9], [12], [18] and [21].…”

“…Second, the possibility that a clinical subset of APS vascular exists, i.e., those who have recurrences despite correct OAT, must be seriously considered. This fact represents a treatment failure, and some studies have shown that this is not at all a rare event [9], [12] and [37]. In particular, this happens in higher risk patients for aPL profile (triple positivity, Miyakis types 1 and 2a) [38].…”

“…The aims of the Consortium are: 1) to diagnose, treat and follow up APS patients, and to record data at diagnosis in the Regional Registry for Rare Diseases (National Health Service) 2) to improve knowledge of the syndrome, and to share up-to-date diagnostic and therapeutic strategies; and 3) to discuss controversial clinical cases and to support clinical and laboratory research. The APS Piedmont Cohort study is an ambispective study that includes 217 consecutive APS patients, selected from a broader group of about 400 suspected cases [12]. Enrolled patients strictly met the ongoing clinical and laboratory classification criteria, and APS diagnosis was confirmed by a centralized board.…”

“…Generic cardiovascular risk factors and inherited thrombophilia can also further increase thrombotic risk [12] and [20].…”

“…Nowadays the high frequency of thrombotic recurrences still represents a major clinical criticism and a yet unsolved therapeutic challenge. The aim of this paper was to evaluate the incidence of thrombotic recurrences, as well as the patients' risk factors, the aPL profile, and the bleeding events that have been reported in the literature, and to compare these data to the recent experience of the APS Piedmont Cohort [12].…”