Relapses are common in severe hematologic systemic lupus erythematosus and may be prevented by early institution of immunosuppressive agents: Α real-life single-center study

Georgia-Savina Moysidou; Panagiotis Garantziotis; Dionysis Nikolopoulos; Pelagia Katsimbri; Antonis Fanouriakis; Dimitrios T Boumpas

doi:10.1177/09612033221144425

Relapses are common in severe hematologic systemic lupus erythematosus and may be prevented by early institution of immunosuppressive agents: Α real-life single-center study

Lupus. 2023 Feb;32(2):225-230. doi: 10.1177/09612033221144425. Epub 2022 Dec 9.

Authors

Georgia-Savina Moysidou^{1

2}, Panagiotis Garantziotis², Dionysis Nikolopoulos², Pelagia Katsimbri¹, Antonis Fanouriakis^{1

3

4}, Dimitrios T Boumpas^{1

2

4}

Affiliations

¹ Rheumatology and Clinical Immunology Unit, 4th Department of Internal Medicine, Attikon University Hospital, Athens, Greece.
² Faculty of Medicine, National and Kapodistrian University of Athens, Inflammation & Autoimmunity Lab, Biomedical Research Foundation of the Academy of Athens (BRFAA), Athens, Greece.
³ 1st Department of Propaedeutic Internal Medicine, "Laiko" General Hospital, Athens, Greece.
⁴ Joint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece.

PMID: 36490217
DOI: 10.1177/09612033221144425

Abstract

Background: Hematologic manifestations are common in systemic lupus erythematosus (SLE), either at initial presentation or during the course of the disease, but data regarding their natural history are scarce.

Objective: To describe the characteristics, treatments, and outcomes of severe hematological manifestations in a large cohort of lupus patients.

Methods: Retrospective cohort study of patients in the "Attikon" lupus cohort who had a history of a severe hematologic manifestation, defined as autoimmune hemolytic anemia (AIHA) with hemoglobin < 8 g/dL, thrombocytopenia with platelet count < 30,000/mm³, Evans syndrome with hemoglobin < 8 g/dL, and/or platelet count < 30,000/mm³, neutropenia with < 500 neutrophils/mm³, thrombotic microangiopathy (TMA)/thrombotic thrombocytopenic purpura (TTP)-like syndrome, or macrophage activation syndrome (MAS). Demographic and clinical characteristics, treatments, and outcomes were recorded.

Results: From over 300 patients with hematologic manifestations, 41 qualified as severe (70.7% women, mean [SD] age at SLE diagnosis 42.6 [18.0] years). Hematologic manifestations preceded SLE diagnosis in 13 patients (31.7%), was concomitant to SLE diagnosis in 16 patients (39%), and occurred during the course of the disease in 12 (29.3%) patients, with a mean (SD) disease duration of 8.7 (5.5) years. Thrombocytopenia was the most common severe hematological manifestation (56.1%), followed by AIHA (17.1%) and TTP-like syndrome (12.2%). For initial treatment, all patients were treated with glucocorticoids (GC), while rituximab and cyclophosphamide were the most frequently used immunosuppressive agents. Following initial treatment, relapse occurred in 22 patients (53.7%). Compared to patients that did not relapse, those that relapsed had less often received concomitant immunosuppressive agents following treatment of initial episode (n = 17/23, 73.9% vs 5/17, 29.4%, p = 0.005).

Conclusion: Severe hematologic disease in SLE has a high risk of relapse, which may be mitigated by the early institution of GC-sparing agents.

Keywords: haematologic changes; systemic lupus erythematosus; treatment.

MeSH terms

Adolescent
Anemia, Hemolytic, Autoimmune* / diagnosis
Female
Humans
Immunosuppressive Agents / therapeutic use
Leukopenia*
Lupus Erythematosus, Systemic* / complications
Lupus Erythematosus, Systemic* / drug therapy
Male
Retrospective Studies
Thrombocytopenia* / drug therapy
Thrombocytopenia* / epidemiology
Thrombocytopenia* / etiology

Substances

Immunosuppressive Agents