Damage index for antiphospholipid syndrome during long term follow-up: Correlation between organ damage accrual and quality of life

Abstract

Background

Consequences of organ damage in primary antiphospholipid syndrome (PAPS) are diverse, our aim was to determine organ damage over time and the correlation of organ damage accrual with health-related quality of life (HRQoL) in PAPS.

Methods

First phase: retrospective cohort applying Damage Index for Antiphospholipid Syndrome (DIAPS) at 1, 5, 10, 20 years, or longer since diagnosis. Second phase: cross-sectional study, assessing HRQoL by the Medical Outcomes Study Short Form 36 (SF-36), and organ damage accrual. Descriptive statistics and Spearman correlation coefficient were used.

Results

Sixty-seven patients were included, mean follow-up:15 years. Deep vein thrombosis prevailed (71.6%), pulmonary embolism (35.8%) and stroke (32.8%). Organ damage was found in 98.5%, with a cumulative DIAPS value of 3, with greater involvement in the neuropsychiatric and peripheral vascular domains. Regarding HRQoL, deterioration in the physical component summary (PCS) was found in 89.6%. Organ damage accrual correlated inversely and significantly with all the SF-36 domains, mainly with the total score and PCS. Body pain and PCS correlated the most (rho = −0.503, rho = −0.475).

Conclusions

Organ damage accrual impaired HRQoL in PAPS. Secondary thromboprophylxis through adequate systemic management and control of cardiovascular risk factors are necessary to prevent further impairment.

Keywords

Primary antiphospholipid syndrome Damage Index for Antiphospholipid Syndrome health related quality of life neuropsychiatric damage organ damage accrual

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References

Sammaritano

LR.

Antiphospholipid syndrome. Best Pract Res Clin Rheumatol 2020; 34: 101463.

Cervera

Antiphospholipid syndrome. Thromb Res 2017; 151: S43–S47.

Noureldine

MHA

Nour-Eldine

Khamashta

Uthman

Insights into the diagnosis and pathogenesis of the antiphospholipid syndrome. Semin Arthritis Rheum 2019; 48: 860–866.

Dall’Ara

Reggia

Taraborelli

, et al. Patients with longstanding primary antiphospholipid syndrome: retrospective analysis of organ damage and mortality. Lupus 2014; 23: 1255–1258.

Taraborelli

Reggia

Dall’Ara

, et al. Long-term outcome of patients with primary antiphospholipid syndrome: a retrospective multicenter study. J Rheumatol 2017; 44: 1165–1172.

Kravvariti

Koutsogianni

Samoli

Sfikakis

Tektonidou

MG.

The effect of hydroxychloroquine on thrombosis prevention and antiphospholipid antibody levels in primary antiphospholipid syndrome: a pilot open-label randomized prospective study. Autoimmun Rev 2020; 19: 102491.

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: 927–934.

Georgopoulou

Efraimidou

MacLennan

Ibrahim

Cox

The relationship between social support and health-related quality of life in patients with antiphospholipid (Hughes) syndrome. Mod Rheumatol 2018; 28: 147–155.

Georgopoulou

Efraimidou

MacLennan

Ibrahim

Cox

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

10.

Zuily

Rat

Regnault

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

11.

Alba

Gómez-Puerta

Goycochea-Robles

Amigo

MC.

Organ damage and quality of life in antiphospholipid syndrome. Curr Rheumatol Rep 2016; 18: 7–14.

12.

Torricelli

Ugolini-Lopes

Bonfá

Andrade

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

13.

Miyakis

Lockshin

Atsumi

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

14.

Ware

Kosinski

Keller

SK.

SF-36® physical and mental health summary scales: a user's manual. Boston, MA: The Health Institute, 1994.

15.

Ware

Jr Kosinski

Bayliss

, et al. Comparison of methods for the scoring and statistical analysis of SF-36 health profile and summary measures: summary of results from the medical outcomes study. Med Care 1995; 33: AS264–AS279.

16.

Gandek

Ware

Jr.

Methods for validating and norming translations of health status questionnaires: the IQOLA project approach. International quality of life assessment. J Clin Epidemiol 1998; 51: 953–959.

17.

Durán-Arenas

Gallegos-Carrillo

Salinas-Escudero

Martínez-Salgado

Hacia una base normativa mexicana en la medición de calidad de vida relacionada con la salud, mediante el formato corto 36. Salud Publica Mex 2004; 46: 306–315.

18.

Hernández-Molina

González-Pérez

Pacheco-Molina

Cabral

AR.

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

19.

Elera-Fitzcarrald

Alva

Gamboa-Cardenas

, et al. Factors associated with health-related quality of life in Peruvian patients with systemic lupus erythematosus. Lupus 2018; 27: 913–919.

20.

Bermúdez Bermejo

Sánchez Rodríguez

Turrent Carriles

Amigo Castañeda

MC.

Assessment of chronic damage in patients with antiphospholipid syndrome with the damage index in antiphospholipid syndrome (DIAPS). An Med Asoc Med Hosp ABC 2016; 61: 177–181.

21.

Castillo-Martínez

Rivera

Mouneu-Ornelas

, et al. Levels of anti-Müllerian hormone in premenopausal women with the antiphospholipid syndrome and its association with the risk of clinical complications. Lupus 2019; 28: 427–431.

22.

Moreno-Ramírez

Amezcua-Guerra

Gomez

VAE

, et al. Performance and external validation of the damage index in antiphospholipid syndrome in primary and secondary APS patients. Arthritis Rheum 2016; 68: 1400–1401.

23.

Jatuworapruk

Bhoopat

Hanvivadhanakul

Clinical and immunological characteristics of antiphospholipid syndrome in an Asian population: a retrospective study. Asian Pac J Allergy Immunol 2018; 26: 13–20.

24.

Dufrost

Risse

Reshetnyak

, et al. Increased risk of thrombosis in antiphospholipid syndrome patients treated with direct oral anticoagulants. Results from an international patient-level data Meta-analysis. Autoimmun Rev 2018; 17: 1011–1021.

25.

Signorelli

Balbi

GGM

Domingues

Levy

RA.

New and upcoming treatments in antiphospholipid syndrome: a comprehensive review. Pharmacol Res 2018; 133: 108–120.

26.

Schmidt-Tanguy

Voswinkel

Henrion

, et al. Antithrombotic effects of hydroxychloroquine in primary antiphospholipid syndrome patients. J Thromb Haemost 2013; 11: 1927–1929.

27.

Wang

Lim

What is the role of hydroxychloroquine in reducing thrombotic risk in patients with antiphospholipid antibodies?

Hematology Am Soc Hematol Educ Program 2016; 2016: 714–716.

28.

Nuri

Taraborelli

Andreoli

, et al. Long-term use of hydroxychloroquine reduces antiphospholipid antibodies levels in patients with primary antiphospholipid syndrome. Immunol Res 2017; 65: 17–24.

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