Primary antiphospholipid syndrome and antiphospholipid syndrome associated to systemic lupus: Are they different entities?

Cristina Belizna (1, 2) , Ljudmila Stojanovich (3) , Jan Willem Cohen-Tervaert (4) , Céline Fassot (1, 2) , Daniel Henrion (1, 2) , Laurent Loufrani (1, 2, 5) , Gyorgy Nagy (6) , Christian Muchardt (7) , Milena Hasan (8) , Marie Noelle Ungeheuer (9) , Laurent Arnaud (10) , Jaume Aliotaj-Reig (11) , Enrique Esteve-Valverde , Ferdinando Nicoletti (12) , Patrick Saulnier (13) , Alban Godon (13) , Pascal Reynier (1, 2, 13) , Jean Marie Chrétien (13) , Laura Damian , Loukman Omarjee (14, 15) , Guillaume Mahe (14, 15) , Marc Antoine Pistorius (16) , Pier Luigi Meroni (17) , Katrien Devreese (18)

1 MITOVASC - MitoVasc - Physiopathologie Cardiovasculaire et Mitochondriale
2 BNMI - Biologie Neurovasculaire et Mitochondriale Intégrée
3 University of Belgrade [Belgrade]
4 Maastricht University [Maastricht]
5 BNVI - Biologie Neurovasculaire Intégrée
6 Semmelweis University [Budapest]
7 Régulation épigénétique - Epigenetic regulation
8 UTechS CB - Cytometrie et Biomarqueurs – Cytometry and Biomarkers
9 ICAReB - Investigation Clinique et d’Accès aux Ressources Biologiques (Plate-forme) - Clinical Investigation and Access to BioResources
10 CHU Strasbourg
11 UAB - Universitat Autònoma de Barcelona
12 University of Catania [Italy]
13 CHU Angers - Centre Hospitalier Universitaire d'Angers
14 CIC - Centre d'Investigation Clinique [Rennes]
15 CHU Pontchaillou [Rennes]
16 CHU Nantes - Centre hospitalier universitaire de Nantes
17 IRCCS - Istituti di Ricovero e Cura a Carattere Scientifico
18 Ghent University Hospital

Primary antiphospholipid syndrome (PAPS) and antiphospholipid syndrome associated to lupus (SAPS) have several overlapping characteristics. As systemic manifestations are also reported in patients with PAPS, and as a subgroup of PAPS patients could evaluate to a SAPS, the differentiation between the two types of APS could be performed based on the clinical experience of the medical teams and is related to a variety of clinical, biological, histological and genetic features. Several data are available in the literature with respect to the identification of distinctive features between these two entities. However, there are some limitation in the interpretation of results issued from studies performed prior to updated Sydney criteria. Based on recent data, a certain number of features more frequent in one type of APS as compared to the other could be distinguished. The major differentiation between these two entities is genetical. New genetic data allowing the identification of specific subgroups of APS are ongoing.

Keywords: Secondary antiphospholipid syndrome; Primary antiphospholipid syndrome; Antiphospholipid syndrome

Domains: Life Sciences [q-bio]; Human health and pathology; Rhumatology and musculoskeletal system; Life Sciences [q-bio]; Immunology

Cristina Belizna, Ljudmila Stojanovich, Jan Willem Cohen-Tervaert, Céline Fassot, Daniel Henrion, et al.. Primary antiphospholipid syndrome and antiphospholipid syndrome associated to systemic lupus: Are they different entities?. Autoimmunity Reviews, 2018, 17 (8), pp.739-745. ⟨10.1016/j.autrev.2018.01.027⟩. ⟨hal-01833939⟩