Thrombocytopenia and autoimmune hemolytic anemia in antiphospholipid antibody-positive patients: Descriptive analysis of the AntiPhospholipid syndrome alliance for clinical trials and InternatiOnal networking (APS ACTION) clinical database and repository (“Registry”)

Erton, ZB; Leaf, RK; de Andrade, D; Clarke, A; Tektonidou, MG; Pengo, V; Sciascia, S; Pardos-Gea, J; Kello, N; Paredes-Ruiz, D; Lopez-Pedrera, C; Belmont, HM; Fortin, PR; Ramires de Jesús, G; Atsumi, T; Zhang, Z; Efthymiou, M; Branch, DW; Pazzola, G; Andreoli, L; Duarte-García, A; Rodriguez-Almaraz, E; Petri, M; Cervera, R; Artim-Esen, B; Quintana, R; Shi, H; Zuo, Y; Willis, R; Barber, MRW; Skeith, L; Radin, M; Meroni, P; Bertolaccini, ML; Cohen, H; Roubey, R; Erkan, D

doi:10.1177/09612033251332258

Lupus & antiphospholipid syndrome

Thrombocytopenia and autoimmune hemolytic anemia in antiphospholipid antibody-positive patients: Descriptive analysis of the AntiPhospholipid syndrome alliance for clinical trials and InternatiOnal networking (antiphospholipid syndrome (APS) ACTION) clinical database and repository (“Registry”)

LupusPublished 3 Apr 2025Retrospective cohort study

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Authors' conclusion

In our large multi-center international cohort of persistently aPL-positive patients, approximately one-fifth had active or historical TP and/or AIHA at registry entry; half of these patients had additional SLE.

Study context

Population: Persistently aPL-positive patients with or without systemic autoimmune rheumatic diseases, APS ACTION Registryas of April 2022
Participants: 1,039 patients

Exposure: aPL-positive patients with vs without thrombocytopenia (TP) and/or autoimmune hemolytic anemia (AIHA)
TP/AIHA evern = 228
Outcome: Demographic, clinical and serologic characteristics of aPL-positive patients with vs without TP and/or AIHA

Methodological detailsregistry-based, multicentre, international

Registry-based
Multicentre
International

Main finding

As reported in the abstract

Baseline TP and/or AIHA

TP/AIHA ever

22%

228

TP only: 176 [17%]
AIHA only: 35 [3%]
Both TP and AIHA: 17 [2%]

Observational data: associations, not effects.

Further findings

Immunosuppressive use in patients with TP and/or AIHA, primary aPL/APS vs SLE

Primary aPL/APS16%

SLE34%

Immunosuppressive use in patients with TP and/or AIHA, primary aPL/APS vs systemic lupus erythematosus (SLE)

AZA and MMF more commonly reported in lupus patients
Corticosteroid, intravenous immunoglobulin and RTX use similar between groups

Reference

Authors: Erton ZB, Leaf RK, de Andrade D, Clarke A, Tektonidou MG, Pengo V, et al.
All 37 authors
Erton ZB, Leaf RK, de Andrade D, Clarke A, Tektonidou MG, Pengo V, Sciascia S, Pardos-Gea J, Kello N, Paredes-Ruiz D, Lopez-Pedrera C, Belmont HM, Fortin PR, Ramires de Jesús G, Atsumi T, Zhang Z, Efthymiou M, Branch DW, Pazzola G, Andreoli L, Duarte-García A, Rodriguez-Almaraz E, Petri M, Cervera R, Artim-Esen B, Quintana R, Shi H, Zuo Y, Willis R, Barber MRW, Skeith L, Radin M, Meroni P, Bertolaccini ML, Cohen H, Roubey R, Erkan D
Journal: Lupus
Published: 3 Apr 2025
DOI: 10.1177/09612033251332258
PubMed: 40180601

Topic: Lupus & antiphospholipid syndrome

Exposure definitions

TP/AIHA ever: baseline (historical or current) TP and/or AIHA

Key notes

TP and/or AIHA significantly associated with Asian race, SLE classification, cardiac valve disease, catastrophic/microvascular APS, triple aPL positivity, and SLE-related serologic and inflammatory markers

Original publication

Thrombocytopenia and autoimmune hemolytic anemia in antiphospholipid antibody-positive patients: Descriptive analysis of the AntiPhospholipid syndrome alliance for clinical trials and InternatiOnal networking (APS ACTION) clinical database and repository (“Registry”). Lupus. Published 3 April 2025. doi:10.1177/09612033251332258

Read the original article ↗

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