Longterm Outcome of Patients with Primary Antiphospholipid Syndrome: A Retrospective Multicenter Study

Mara Taraborelli; Rossella Reggia; Francesca Dall'Ara; Micaela Fredi; Laura Andreoli; Maria Gerosa; Ariela Hoxha; Laura Massaro; Marta Tonello; Nathalie Costedoat-Chalumeau; Patrice Cacoub; Franco Franceschini; Pier Luigi Meroni; Jean Charles Piette; Amelia Ruffatti; Guido Valesini; Eon Nigel Harris; Angela Tincani

doi:10.3899/jrheum.161364

Longterm Outcome of Patients with Primary Antiphospholipid Syndrome: A Retrospective Multicenter Study

J Rheumatol. 2017 Aug;44(8):1165-1172. doi: 10.3899/jrheum.161364. Epub 2017 Jun 1.

Authors

Mara Taraborelli^{1

2}, Rossella Reggia^{1

2}, Francesca Dall'Ara^{1

2}, Micaela Fredi^{1

2}, Laura Andreoli^{1

2}, Maria Gerosa^{1

2}, Ariela Hoxha^{1

2}, Laura Massaro^{1

2}, Marta Tonello^{1

2}, Nathalie Costedoat-Chalumeau^{1

2}, Patrice Cacoub^{1

2}, Franco Franceschini^{1

2}, Pier Luigi Meroni^{1

2}, Jean Charles Piette^{1

2}, Amelia Ruffatti^{1

2}, Guido Valesini^{1

2}, Eon Nigel Harris^{1

2}, Angela Tincani^{3

4}

Affiliations

¹ From the Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia, Brescia; Division of Rheumatology, Department of Clinical Sciences and Community Health, Ospedale Gaetano Pini, University of Milan, Milan; Rheumatology Unit, Department of Medicine - DIMED, University of Padua, Padua; Internal Medicine and Medical Specialties Department, Policlinico Umberto I, La Sapienza University of Rome, Rome, Italy; AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne; AP-HP, Hospital Pitié-Salpêtrière, Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne, Pierre et Marie Curie Paris VI University, Paris, France; University of the West Indies, Kingston, Jamaica.
² M. Taraborelli, MD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia; R. Reggia, MD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia; F. Dall'Ara, MD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia; M. Fredi, MD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia; L. Andreoli, MD, PhD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia; M. Gerosa, MD, PhD, Division of Rheumatology, Department of Clinical Sciences and Community Health, Ospedale Gaetano Pini, University of Milan; A. Hoxha, MD, PhD, Rheumatology Unit, Department of Medicine - DIMED, University of Padua; L. Massaro, MD, Internal Medicine and Medical Specialties Department, Policlinico Umberto I, La Sapienza University of Rome; M. Tonello, BSc, PhD, Rheumatology Unit, Department of Medicine - DIMED, University of Padua; N. Costedoat-Chalumeau, MD, PhD, AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne; P. Cacoub, MD, PhD, AP-HP, Hospital Pitié-Salpêtrière, Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne, Pierre et Marie Curie Paris VI University; F. Franceschini, MD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia; P.L. Meroni, MD, Division of Rheumatology, Department of Clinical Sciences and Community Health, Ospedale Gaetano Pini, University of Milan; J.C. Piette, MD, PhD, AP-HP, Hospital Pitié-Salpêtrière, Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne, Pierre et Marie Curie Paris VI University; A. Ruffatti, MD, PhD, Rheumatology Unit, Department of Medicine - DIMED, University of Padua; G. Valesini, MD, Internal Medicine and Medical Specialties Department, Policlinico Umberto I, La Sapienza University of Rome; E.N. Harris, MPhil, MD, DM, Vice Chancellor Emeritus, University of the West Indies; A. Tincani, MD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia. Drs. Taraborelli, Reggia, and Dall'Ara contributed equally to this paper.
³ From the Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia, Brescia; Division of Rheumatology, Department of Clinical Sciences and Community Health, Ospedale Gaetano Pini, University of Milan, Milan; Rheumatology Unit, Department of Medicine - DIMED, University of Padua, Padua; Internal Medicine and Medical Specialties Department, Policlinico Umberto I, La Sapienza University of Rome, Rome, Italy; AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne; AP-HP, Hospital Pitié-Salpêtrière, Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne, Pierre et Marie Curie Paris VI University, Paris, France; University of the West Indies, Kingston, Jamaica. angela.tincani@unibs.it.
⁴ M. Taraborelli, MD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia; R. Reggia, MD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia; F. Dall'Ara, MD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia; M. Fredi, MD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia; L. Andreoli, MD, PhD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia; M. Gerosa, MD, PhD, Division of Rheumatology, Department of Clinical Sciences and Community Health, Ospedale Gaetano Pini, University of Milan; A. Hoxha, MD, PhD, Rheumatology Unit, Department of Medicine - DIMED, University of Padua; L. Massaro, MD, Internal Medicine and Medical Specialties Department, Policlinico Umberto I, La Sapienza University of Rome; M. Tonello, BSc, PhD, Rheumatology Unit, Department of Medicine - DIMED, University of Padua; N. Costedoat-Chalumeau, MD, PhD, AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne; P. Cacoub, MD, PhD, AP-HP, Hospital Pitié-Salpêtrière, Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne, Pierre et Marie Curie Paris VI University; F. Franceschini, MD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia; P.L. Meroni, MD, Division of Rheumatology, Department of Clinical Sciences and Community Health, Ospedale Gaetano Pini, University of Milan; J.C. Piette, MD, PhD, AP-HP, Hospital Pitié-Salpêtrière, Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne, Pierre et Marie Curie Paris VI University; A. Ruffatti, MD, PhD, Rheumatology Unit, Department of Medicine - DIMED, University of Padua; G. Valesini, MD, Internal Medicine and Medical Specialties Department, Policlinico Umberto I, La Sapienza University of Rome; E.N. Harris, MPhil, MD, DM, Vice Chancellor Emeritus, University of the West Indies; A. Tincani, MD, Rheumatology and Clinical Immunology Department, Spedali Civili and University of Brescia. Drs. Taraborelli, Reggia, and Dall'Ara contributed equally to this paper. angela.tincani@unibs.it.

PMID: 28572466
DOI: 10.3899/jrheum.161364

Abstract

Objective: To assess the longterm frequency of thrombotic recurrences, obstetrical complications, organ damage, severe comorbidities, and evolution toward connective tissue disease (CTD) in primary antiphospholipid syndrome (PAPS).

Methods: Medical records of patients with PAPS followed in 6 centers for ≥ 15 years were retrospectively reviewed.

Results: One hundred fifteen patients were studied: 88% women, followed between 1983 and 2014 with a mean (± SD) age at diagnosis of 33 (± 10) years. During a median followup of 18 years (range 15-30), 50 patients (44%) had at least a thrombotic event for a total of 75 events and an annual incidence of 3.5%. Thromboses were more frequent in patients with previous thrombotic history (p = 0.002). A catastrophic antiphospholipid syndrome occurred in 6 patients (5%). The use of oral anticoagulants in patients with thrombotic onset did not appear to be protective against recurrences (p = 0.26). Fifty-two women had 87 pregnancies, successful in 78%. Twenty-nine percent of patients accrued functional damage. Damage was significantly associated with a thrombotic history (p = 0.004) and with arterial events (p < 0.001), especially stroke, but not with demographics, serology, or treatment. Twenty-four major bleeding episodes were recorded in 18 patients, all receiving anticoagulants. Severe infections affected 6 patients (5%), with 1 fatality. A solid cancer was diagnosed in 8 patients (7%). Altogether, 16 patients (14%) developed an autoimmune disease and 13 (11%) a full-blown picture of CTD.

Conclusion: Despite therapy, a high proportion of patients experienced new thrombotic events and organ damage, while evolution toward CTD was infrequent.

Keywords: EVOLUTION; ORGAN DAMAGE; PREGNANCY; PRIMARY ANTIPHOSPHOLIPID SYNDROME; RECURRENCES; THROMBOSIS.

Publication types

Multicenter Study

MeSH terms

Adult
Anticoagulants / therapeutic use*
Antiphospholipid Syndrome / complications
Antiphospholipid Syndrome / drug therapy*
Disease Progression
Female
Follow-Up Studies
Humans
Male
Middle Aged
Retrospective Studies
Thrombosis / etiology*
Treatment Outcome
Young Adult

Substances

Anticoagulants