Predictors of damage accrual and its impact on health-related quality of life of thrombotic antiphospholipid syndrome: Independent validation of the damage index for antiphospholipid syndrome (DIAPS)

Abstract

Objectives

We aim to independently assess the validity of the damage index for antiphospholipid syndrome (DIAPS) in thrombotic antiphospholipid syndrome (APS) patients by exploring the prevalence and risk factors of organ damage and evaluating its impact on health-related quality of life (HR-QoL).

Methods

Cross-sectional study including all thrombotic APS patients (Sydney criteria) attending a Portuguese tertiary centre. Damage was assessed using the DIAPS, and HR-QoL using the 3- and 5-level EuroQol HR-QoL (EQ-D5-3L and 5L), and Visual Analogue Scale (VAS) applied via a phone questionnaire. Spearman’s correlation between DIAPS and the HR-QoL scales was performed. Risk factors for damage accrual and HR-QoL impairment were explored using univariate and multivariate logistic regression.

Results

Among the 108 patients (female, 65.7%; white, 90.7%; primary APS, 75.9%; median disease duration, 6 years), damage (DIAPS≥1) developed in 48.2% of patients (mean ± SD DIAPS, 3.08 ± 1.83). DIAPS’s neuropsychiatric domain was the most affected (24.2%), followed by the peripheral vascular domain (20.3%). No clinical, demographic nor laboratory parameters were significantly associated with damage. Regarding HR-QoL, pain/discomfort, anxiety/depression and usual activities domains were the most frequently impaired in both scales. DIAPS’s domains correlated similarly with the EQ-5D-3L and 5L scales’ individual domains. Female sex, medical disorders, secondary APS and type of presenting thrombosis (arterial) increased the risk of HR-QoL impairment. Total DIAPS was associated with higher odds of mobility, self-care and pain/discomfort impairment in both EQ-5D-3L and 5L scales but lost its independent risk in multivariable analysis.

Conclusion

This external validation of DIAPS reinforces the ability of the score to correlate with HR-QoL while also highlighting risk factors for HR-QoL impairment other than damage accrual.

Keywords

Antiphospholipid syndrome damage index damage index for antiphospholipid syndrome organ damage quality of life

Get full access to this article

View all access options for this article.

References

Miyakis

Lockshin

Atsumi

, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemostasis 2006; 4(2): 295–306.

Tektonidou

Andreoli

Limper

, et al. EULAR recommendations for the management of antiphospholipid syndrome in adults. Ann Rheum Dis 2019; 78(10): 1296–1304.

Tektonidou

. Prognostic factors and clustering of serious clinical outcomes in antiphospholipid syndrome. QJM 2000; 93(8): 523–530.

Cervera

Serrano

Pons-Estel

, et al. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis 2015; 74(6): 1011–1018.

Amigo

Goycochea-Robles

Espinosa-Cuervo

, et al. Development and initial validation of a damage index (DIAPS) in patients with thrombotic antiphospholipid syndrome (APS). Lupus 2015; 24(9): 927–934.

Gaspar

Cohen

Isenberg

. The assessment of patients with the antiphospholipid antibody syndrome: where are we now? Rheumatology 2020; 59(7): 1489–1494.

Desnoyers

Dufrost

Wahl

, et al. Health-related quality of life in antiphospholipid syndrome: current knowledge and future perspectives. Curr Rheumatol Rep 2020; 22(6): 20.

Radin

Foddai

Cecchi

, et al. Quality of life in patients with antiphospholipid antibodies differs according to antiphospholipid syndrome damage index (DIAPS). Eur J Intern Med 2021; 92: 134–136.

Martinez-Martinez

Chacon-Perez

MJNN

Amigo

. AB0466 damage index in patients with thrombotic antiphospholipid syndrome: retrospective cohort study (Abstract). Ann Rheum Dis 2016; 75(Suppl 2): 1065.

10.

Medina

Cimé Aké

Vera-Lastra

, et al. Damage index for antiphospholipid syndrome during long term follow-up: correlation between organ damage accrual and quality of life. Lupus 2021; 30(1): 96–102.

11.

Carpenter

. Racial/ethnic differences in quality of life for people living with arthritis who see a primary care physician. Open Rheumatol J 2011; 5(1): 24–29.

12.

Bolen

Schieb

Hootman

, et al. Differences in the prevalence and impact of arthritis among racial/ethnic groups in the United States. National Health Interview Survey. 2010; 7(3A64).

13.

Ferreira

Ribeiro

, et al. Comparing the performance of the EQ-5D-3L and the EQ-5D-5L in young Portuguese adults. Health Qual Life Outcome 2016; 14(1): 89.

14.

Herdman

Gudex

Lloyd

, et al. Development and preliminary testing of the new five-level version of EQ-5D (EQ-5D-5L). Qual Life Res 2011; 20(10): 1727–1736.

15.

Janssen

Birnie

Haagsma

, et al. Comparing the standard EQ-5D three-level system with a five-level version. Value Health 2008; 11(2): 275–284.

16.

Ferreira

Pereira

, et al. The valuation of the EQ-5D in Portugal. Qual Life Res 2014; 23(2): 413–423.

17.

Ferreira

Antunes

Ferreira

, et al. A hybrid modelling approach for eliciting health state preferences: the Portuguese EQ-5D-5L value set. Qual Life Res 2019; 28(12): 3163–3175.

18.

Torricelli

Ugolini-Lopes

Bonfá

, et al. Antiphospholipid syndrome damage index (DIAPS): distinct long-term kinetic in primary antiphospholipid syndrome and antiphospholipid syndrome related to systemic lupus erythematosus. Lupus 2020; 29(3): 256–262.

19.

Gaspar

Farinha

Sayar

, et al. A one-point increase in the damage index for antiphospholipid syndrome (DIAPS) predicts mortality in thrombotic antiphospholipid syndrome. Clin Exp Rheumatol. 2023; 41(3): 605–612.

20.

Balbi

GGM

Ahmadzadeh

Tektonidou

, et al. Damage measured by Damage Index for Antiphospholipid Syndrome (DIAPS) in antiphospholipid antibody-positive patients included in the APS ACTION registry. Rheumatology. 2023; 63(3): 772–779.

21.

Hernández-Molina

González-Pérez

Pacheco-Molina

, et al. Quality of life in patients with antiphospholipid syndrome is related to disease burden and anticoagulant therapy. Int J Rheum Dis 2017; 20(6): 755–759.

22.

Uludağ

Çene

Gurel

, et al. Description of damage in different clusters of patients with antiphospholipid syndrome. Lupus 2022; 31(4): 433–442.

23.

Chayoua

Kelchtermans

Moore

, et al. Identification of high thrombotic risk triple‐positive antiphospholipid syndrome patients is dependent on anticardiolipin and anti β2glycoprotein I antibody detection assays. J Thromb Haemostasis 2018; 16(10): 2016–2023.

24.

Erkan

Yazici

Sobel

, et al. Primary antiphospholipid syndrome: functional outcome after 10 years. J Rheumatol; 27(12): 2817–2821.

25.

Zuily

Rat

Regnault

, et al. Impairment of quality of life in patients with antiphospholipid syndrome. Lupus 2015; 24(11): 1161–1168.

26.

Cohen

Sayar

Efthymiou

, et al. Management of anticoagulant-refractory thrombotic antiphospholipid syndrome. Lancet Haematol 2020; 7(8): e613–e623.

27.

Balitsky

Peeva

, et al. Thrombovascular events affect quality of life in patients with systemic lupus erythematosus. J Rheumatol 2011; 38(6): 1017–1019.

28.

Georgopoulou

Efraimidou

MacLennan

, et al. Antiphospholipid (Hughes) syndrome: description of population and health-related quality of life (HRQoL) using the SF-36. Lupus 2015; 24(2): 174–179.

29.

Fanouriakis

Kostopoulou

Andersen

, et al. EULAR recommendations for the management of systemic lupus erythematosus: 2023 update. Ann Rheum Dis 2024; 83(1): 15–29.

30.

Cohen

Hunt

Efthymiou

, et al. Rivaroxaban versus warfarin to treat patients with thrombotic antiphospholipid syndrome, with or without systemic lupus erythematosus (RAPS): a randomised, controlled, open-label, phase 2/3, non-inferiority trial. Lancet Haematol 2016; 3(9): e426–e436.

31.

De Oliveira Vaz

De Moraes Mazetto Fonseca

Nascimento Silva Vasconcelos

, et al. Self-perceived quality of life of primary antiphospholipid syndrome patients using vitamin K antagonist. Lupus 2021; 30(5): 707–714.

32.

Gaspar

Mittal

Cohen

, et al. Bleeding events in thrombotic antiphospholipid syndrome: prevalence, severity, and associated damage accrual. Res Pract Thromb Haemost 2024; 8(1): 102327.

33.

Khairani

Bejjani

Piazza

, et al. Direct oral anticoagulants vs vitamin K antagonists in patients with antiphospholipid syndromes. J Am Coll Cardiol 2023; 81(1): 16–30.

34.

Garratt

Macdonald

Ruta

, et al. Towards measurement of outcome for patients with varicose veins. Qual Saf Health Care 1993; 2(1): 5–10.

35.

Launois

Reboul-Marty

Henry

. Construction and validation of a quality of life questionnaire in chronic lower limb venous insufficiency (CIVIQ). Qual Life Res 1996; 5(6): 539–544.

36.

Kuet

Lane

Anwar

, et al. Comparison of disease-specific quality of life tools in patients with chronic venous disease. Phlebol J Venous Dis 2014; 29(10): 648–653.

37.

Farrell

Sutter

Tavri

, et al. Incidence and interventions for post-thrombotic syndrome. Cardiovasc Diagn Ther 2016; 6(6): 623–631.

38.

Hassan

Naffaa

Saab

, et al. Cognitive impairment in anti-phospholipid syndrome and anti-phospholipid antibody carriers. Brain Sci 2022; 12(2): 222.

39.

Riaz

Shen

Lateef

, et al. Differential impact of disease activity and damage on health-related quality of life in patients with systemic lupus erythematosus. Lupus 2022; 31(9): 1121–1126.

40.

Chaigne

Chizzolini

Perneger

, et al. Impact of disease activity on health-related quality of life in systemic lupus erythematosus – a cross-sectional analysis of the Swiss Systemic Lupus Erythematosus Cohort Study (SSCS). BMC Immunol 2017; 18(1): 17.

Supplementary Material

Please find the following supplemental material available below.

For Open Access articles published under a Creative Commons License, all supplemental material carries the same license as the article it is associated with.

For non-Open Access articles published, all supplemental material carries a non-exclusive license, and permission requests for re-use of supplemental material or any part of supplemental material shall be sent directly to the copyright owner as specified in the copyright notice associated with the article.

0.00 MB

1.16 MB