Lupus & antiphospholipid syndrome

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

LupusPublished Retrospective cohort study

Authors' conclusion

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

Study context

Population
Patients in the "Attikon" lupus cohort with a history of severe haematologic manifestation (autoimmune hemolytic anemia (AIHA) with Hb < 8 g/dL, thrombocytopenia < 30,000/mm³, Evans syndrome, neutropenia < 500/mm³, thrombotic microangiopathy (TMA)/thrombotic thrombocytopenic purpura (TTP)-like syndrome or macrophage activation syndrome (MAS)); 70.7% women, mean (SD) age at systemic lupus erythematosus (SLE) diagnosis 42.6 (18.0) yearsfrom over 300 with haematologic manifestations
Participants
41 patients
Exposure
SLE patients who relapsed vs those who did not relapse after initial treatment of a severe haematologic manifestation
  • Relapsen = 22
Outcome
Relapse after initial treatment
Methodological detailssingle-centre, real-life
  • Single-centre
  • Real-life

Main finding

As reported in the abstract

Relapse after initial treatment

22

53.7%

Observational data: associations, not effects.

Further findings

Concomitant immunosuppressive agents after treatment of initial episode

P = 0.005

  • Concomitant immunosuppressive agents after treatment of initial episode: relapsed less often received concomitant immunosuppressive agents; printed as n = 17/23 vs 5/17

Type of severe haematologic manifestation

Thrombocytopenia (most common)
56.1%
AIHA
17.1%
TTP-like syndrome
12.2%

Timing of haematologic manifestation relative to SLE diagnosis

Preceded SLE diagnosis
13 (31.7%)
Concomitant to SLE diagnosis
16 (39%)
During disease course (mean [SD] disease duration 8.7 [5.5] years)
12 (29.3%)

Initial treatment

All patients treated with glucocorticoids (GC); RTX and CYC the most frequently used immunosuppressive agents

Original publication

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. Published 9 December 2022. doi:10.1177/09612033221144425

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