Prognostic factors and clustering of serious clinical outcomes in antiphospholipid syndrome

Abstract

We assessed whether initial clinical presentations suggestive of antiphospholipid syndrome (APS) predicted the subsequent rate and type of serious clinical outcomes. Eighty-two consecutive patients with anticardiolipin antibodies or lupus anticoagulant were followed for 814 person-years after a first event suggestive of APS (livedo reticularis, thrombocytopenia, autoimmune haemolysis, thrombosis, central nervous system manifestations, recurrent abortions). The hazard of developing a second event was largest in patients with antibodies recognizing beta2 glycoprotein I who had autoimmune haemolysis as the first event (hazard ratio HR 2.70, p=0.018) and smallest in patients without such antibodies who had recurrent abortions as their first event (HR 0.37, p=0.028). Subsequent serious events in patients with venous and arterial thromboses, recurrent abortions, central nervous system manifestations and autoimmune haemolytic anaemia were likely to be of the same type as the presenting event (odds ratio (OR) 3.76, 5.90, 77.7, 6.92, and 7.13, respectively. Adjusting for therapy, the rate of subsequent serious events was 6.86-fold higher (p=0.0001) in patients presenting with two events, 1.56-fold higher (p=0.038) in autoimmune haemolysis presentations, 1.69-fold higher (p=0.004) in patients with anti-beta2-glycoprotein-I antibodies, and 46% (p=0.063) lower in thrombocytopenia presentations. Initial clinical features determine the long-term evolution of APS, and specific types of clinical manifestations cluster during the course of the disease.

Clinical and immunoserological characteristics of the transition from primary to overlap antiphospholipid syndrome

“…Yet, no reliable prognostic autoantibodies could be identified that could predict transition. These results in association with results published by other investigators [15][16][17]21,22,24,25 suggest that PAPS may be considered as a potential pre-phase of transition towards other CTDs. …”

“…Altogether 65.4% of the patients remained in the undifferentiated CTD stage after 5 years, while complete regression occurred in 12.3% of patients. 14 In the present study we assessed the clinical and immunoserological characteristics of patients with 16 observed that the recurring event is similar to the previous thromboembolic events as arterial events were later followed by another arterial thromboembolic manifestation, while venous events were followed by recurrent venous thromboembolism. Mujic et al 17 followed 80 PAPS patients for 12 years and three cases developed SLE within 10 years.…”

Factors and comorbidities associated with central nervous system involvement in systemic lupus erythematosus: a retrospective cross-sectional case–control study from a single center

“…A pivotal role of aPL in NPSLE and their predictive value for NP damage progression are well known [22][23][24][25][26][27][28][29][30][31][32] and substantially confirmed also by the present study. Other factors deemed as related to NP involvement have been previously proposed including high disease activity, caucasian ethnicity, presence of anti-Ro/SSA antibodies [20,21], vasculitis, nephritis, anti-dsDNA, LA and anti-Sm [23][24][25][26][27][28].…”

Role of the Fcγ receptor IIA polymorphism in the antiphospholipid syndrome: An international meta‐analysis

“…We should caution that the classification criteria for APS and SLE are functional criteria and may not fully correspond to the subgrouping of APS and SLE based on genetic predisposition. APS is a remarkably heterogeneous syndrome with different prognostic profiles (35).…”