Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients

Abstract

Objective. To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression.Methods. The clinical and serologic features of APS (Sapporo preliminary criteria) in 1,000 patients from 13 European countries were analyzed using a computerized database.Results. The cohort consisted of 820 female patients (82.0%) and 180 male patients (18.0%) with a mean ؎ SD age of 42 ؎ 14 years at study entry. "Primary" APS was present in 53.1% of the patients; APS was associated with systemic lupus erythematosus (SLE) in 36.2%, with lupus-like syndrome in 5.0%, and with other diseases in 5.9%. A variety of thrombotic manifestations affecting the majority of organs were recorded. A catastrophic APS occurred in 0.8% of the patients. Patients with APS associated with SLE had more episodes of arthritis and livedo reticularis, and more frequently exhibited thrombocytopenia and leukopenia. Female patients had a higher frequency of arthritis, livedo reticularis, and migraine. Male patients had a higher frequency of myocardial infarction, epilepsy, and arterial thrombosis in the lower legs and feet. In 28 patients (2.8%), disease onset occurred before age 15; these patients had more episodes of chorea and jugular vein thrombosis than the remaining patients. In 127 patients (12.7%), disease onset occurred after age 50; most of these patients were men. These patients had a

The prevalence, onset, and clinical significance of antiphospholipid antibodies prior to diagnosis of systemic lupus erythematosus

“…The presence of aPL has been shown to variably increase risk for the development of these clinical symptoms from 2-fold to 10-fold, consistent with the notion that they play an important role in disease pathogenesis (7)(8)(9)(10)22). They have further been demonstrated to often precede these clinical events, although by an unspecified amount of time (12)(13)(14)(15).…”

“…Thus, these antibodies are directed not only against negatively charged phospholipids, but also against portions of lipid-protein complexes. The lupus anticoagulant and anticardiolipin antibodies (aCL) have traditionally been shown to be associated with thrombotic symptoms of APS and SLE (7)(8)(9)(10)(11)(12)(13)(14)(15). However, aPL have also been shown to bind antigens such as prothrombin, annexin V, and ␤ 2 -glycoprotein I (␤ 2 GPI).…”

“…Clinical symptoms associated with aPL include, but are not limited to, arterial and venous thrombosis, recurrent fetal loss, thrombocytopenia, hemolytic anemia, and aPL-associated nephropathy (7)(8)(9)(10)22). The presence of aPL has been shown to variably increase risk for the development of these clinical symptoms from 2-fold to 10-fold, consistent with the notion that they play an important role in disease pathogenesis (7)(8)(9)(10)22).…”

“…In fact, studies have demonstrated that anywhere from 5% to 70% of lupus patients produce some type of aPL (9,10). However, very little is known about the actual prevalence and onset of aPL in patients prior to SLE diagnosis.…”

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

“…Unlike the Sapporo criteria, superficial thrombophlebitis and TIAs have been excluded, as having all nonobjectively or histologically documented events. Indeed, other clinical manifestations such as aPL positive thrombocytopenia, livedo reticularis and migraine, which are often associated to the syndrome [6] and [7], are actually considered non-criteria manifestations, and are followed separately. Laboratory diagnosis is now more standardized both for the LA test and for the cut-off values for aCL, and furthermore the antibeta2glycoprotein I assay has been added.…”