Lupus & antiphospholipid syndrome

Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome

Lupus Sci MedPublished Registry-based matched cohort study

Authors' conclusion

Patients with iTTP are prone to develop autoimmune features, and patients with SLE-iTTP have distinct clinical features and outcome.

Study context

Population
Patients with iTTP recruited in a registryiTTP registry
Participants
1,409 patients
Exposure
iTTP with coexistent SLE vs age-sex matched idiopathic iTTP without SLE
  • SLE-iTTPn = 79
Outcome
Clinical presentation and outcome
Methodological detailsage-sex matched
  • Age-sex matched

Main finding

As reported in the abstract

  • SLE-iTTP had more severe renal involvement and more prevalent cardiac involvement; CNS involvement less common than in idiopathic iTTP
  • SLE-iTTP received more immunosuppressive agents
  • No difference in response categories during the acute phase
  • SLE-iTTP had superior ADAMTS13 relapse-free survival than idiopathic iTTP during follow-up
  • Matched cohort

Observational data: associations, not effects.

Further findings

Development of clinical SLE in idiopathic iTTP during follow-up
0 · events

Detectable ANAs without other clinical features of SLE

Patients with detectable ANAs without other SLE features
437 (31%)
Of these, diagnosed with SLE following iTTP diagnosis
33 (8%)
Time to SLE diagnosis, months (IQR 7-65)
27
Of these, developed another systemic autoimmune disease
32 (7%)

Original publication

Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome. Lupus Sci Med. Published 28 July 2025. doi:10.1136/lupus-2025-001691

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