Thromb Haemost 2022; 122(09): 1612-1620
DOI: 10.1055/a-1798-2400
Stroke, Systemic or Venous Thromboembolism

Clinical Delphi on aPL Negativization: Report from the APS Study Group of the Italian Society for Rheumatology (SIR-APS)

1   SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Center of Research of Immunopathology and Rare Diseases—Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
,
Silvia Grazietta Foddai*
1   SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Center of Research of Immunopathology and Rare Diseases—Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
,
Cristiano Alessandri
2   Reumatologia, Lupus Clinic, Dipartimento di Medicina Interna e Specialità Mediche, Sapienza Università di Roma, Italy
,
Alessia Alunno
3   Internal Medicine and Nephrology Unit, Department of Life, Health and Environmental Sciences, University of L'Aquila, San Salvatore Hospital, L'Aquila, Italy
,
Laura Andreoli
4   Unit of Rheumatology and Clinical Immunology, ASST Spedali Civili, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy
,
Alice Barinotti
1   SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Center of Research of Immunopathology and Rare Diseases—Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
,
Antonia Calligaro
5   Rheumatology Unit, Department of Medicine, University of Padua, Padua, Italy
,
Valentina Canti
6   Internal Medicine, IRCCS San Raffaele Scientific Institute, Milan, Italy
,
Francesco Carubbi
3   Internal Medicine and Nephrology Unit, Department of Life, Health and Environmental Sciences, University of L'Aquila, San Salvatore Hospital, L'Aquila, Italy
,
Irene Cecchi
1   SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Center of Research of Immunopathology and Rare Diseases—Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
,
Cecillia B. Chighizola
7   Division of Rheumatology, Department of Clinical Sciences and Community Health, Ospedale Gaetano Pini, University of Milan, Milan, Italy
,
Fabrizio Conti
2   Reumatologia, Lupus Clinic, Dipartimento di Medicina Interna e Specialità Mediche, Sapienza Università di Roma, Italy
,
Giacomo Emmi
8   Internal Interdisciplinary Medicine Unit, Lupus Clinic, Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy
,
Antonella Fioravanti
9   Rheumatology Unit, Department of Medicine, Surgery and Neuroscience, Azienda Ospedaliera Universitaria Senese, Siena, Italy
,
Fabio Fischetti
10   Rheumatology Unit, SUGI and Clinical University Department of Medical, Surgery and Health Sciences, University of Trieste, Trieste, Italy
,
Franco Franceschini
4   Unit of Rheumatology and Clinical Immunology, ASST Spedali Civili, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy
,
Maria Gerosa
7   Division of Rheumatology, Department of Clinical Sciences and Community Health, Ospedale Gaetano Pini, University of Milan, Milan, Italy
,
Ariela Hoxha
11   Internal Medicine, University of Padua, Padua, Italy
,
Maddalena Larosa
12   Rheumatology, Department of Locomotor System, ASL3, Genova, Italy
,
Maria-Grazia Lazzaroni
4   Unit of Rheumatology and Clinical Immunology, ASST Spedali Civili, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy
,
Cecilia Nalli
4   Unit of Rheumatology and Clinical Immunology, ASST Spedali Civili, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy
,
Giulia Pazzola
13   Rheumatology Unit, Department of Internal Medicine, Azienda USL-IRCCS di Reggio Emilia, Università di Modenae Reggio Emilia, Reggio Emilia, Italy
,
Massimo Radin
1   SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Center of Research of Immunopathology and Rare Diseases—Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
,
Bernd Raffeiner
14   Rheumatology Unit, Department of Medicine, Azienda Sanitaria dell'Alto Adige, Bolzano, Italy
,
Veronique L. Ramoni
15   Internal Medicine, Ospedale di Lodi, Italy
,
Elena Rubini
1   SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Center of Research of Immunopathology and Rare Diseases—Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
,
Gian Domenico Sebastiani
16   U.O.C. Reumatologia, Ospedale San Camillo-Forlanini, Roma, Italy
,
Simona Truglia
2   Reumatologia, Lupus Clinic, Dipartimento di Medicina Interna e Specialità Mediche, Sapienza Università di Roma, Italy
,
Maria Letizia Urban
8   Internal Interdisciplinary Medicine Unit, Lupus Clinic, Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy
,
Dario Roccatello
1   SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Center of Research of Immunopathology and Rare Diseases—Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
,
Angela Tincani
4   Unit of Rheumatology and Clinical Immunology, ASST Spedali Civili, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy
› Author Affiliations
Funding None.

Abstract

Background The rate of antiphospholipid antibody (aPL) negativization in antiphospholipid syndrome (APS) patients is uncertain, but it is estimated to be as high as 8%. Currently, a consensus definition of aPL negativization is lacking, as well as international recommendations on how to approach treatment in patients with a persistent aPL-negative seroconversion.

Aim The aim of the Delphi survey was to evaluate the clinical approach and level of consensus among experts from the APS Study Group of the Italian Society for Rheumatology (SIR-APS) in different clinical scenarios.

Methods Experts of SIR-APS were contacted using a survey methodology.

Results A structured survey was circulated among 30 experts. Up to 90% of the interviewed experts agreed on defining aPL negativization as the presence of two negative determinations, 1 year apart (90%). Almost full consensus exists among experts in some clinical settings, including: (1) the role of aPL negativization in the management of a thrombotic event determined by concomitant presence of cardiovascular risk factors, both modifiable and not modifiable (90%); (2) approach to young patients with triple aPL positivity who experienced pulmonary arterial thrombotic events and tested negative for aPL detection after 5 years of vitamin K antagonist (VKA) treatment (90%); (3) the use of “extra criteria” aPL antibody testing before pondering VKA suspension (93%).

Conclusion A substantial agreement exists among experts on how to define aPL negativization. VKA suspension should be embraced with extreme caution, particularly in case of previous thrombotic events and/or triple aPL positivity. Nevertheless, VKA cessation might be considered when risk factors are carefully monitored/treated and the presence of “extra criteria” aPL is ruled out.

Author Contributions

C.A., A.A., L.A., A.B., A.C., V.C., F.C., I.C., C.C., F.C., G.E., A.F., F. Fischetti, F. Franceschini, M.G., A.H., M.L., M-.G.L., C.N., G.P., M.R., B.R., V.L.R., E.R., G. D. S., S.T., M.L.U., D.R. participated in data collection and critically reviewed the data and the manuscript. S.S., A.T., and S.G.F. conceived and planned the study and drafted the manuscript. All the authors read and approved the final manuscript. A list of collaborators is provided [Supplementary Appendix A] (available in the online version).


* These authors have equally contributed to the study.


A list of collaborators is provided Appendix A.


Supplementary Material



Publication History

Received: 22 January 2022

Accepted: 13 March 2022

Accepted Manuscript online:
15 March 2022

Article published online:
28 June 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany