Sjögren's disease & overlap diseases

Clinical, biological, prognostic characteristics of patients with immune-mediated thrombotic thrombocytopenic purpura and Sjögren’s disease

RMD OpenPublished Retrospective cohort study

Authors' conclusion

iTTP can be a rare complication in patients with SjD.

Study context

Population
Patients with immune-mediated thrombotic thrombocytopenic purpura and Sjögren disease (SjD), French TTP Registry
Participants
30 / 65 / 45
Exposure
iTTP-SjD vs primary SjD (pSjD) and vs idiopathic iTTP
  • iTTP-SjDn = 30
  • pSjDn = 65reference
  • Idiopathic iTTPn = 45reference
Outcome
Characteristics of iTTP-SjD vs pSjD
Methodological detailsregistry-based, two control groups
  • Registry-based
  • Two control groups

Main finding

As reported in the abstract

iTTP-SjD diagnosed with SjD at the time of iTTP diagnosis
18

Observational data: associations, not effects.

Further findings

OutcomeP value
Mortality and iTTP relapses, iTTP-SjD vs idiopathic iTTPiTTP-SjD vs idiopathic iTTP · comparablelog-rank tests, p=0.228
Mortality and iTTP relapses, iTTP-SjD vs idiopathic iTTPiTTP-SjD vs idiopathic iTTP · comparablemultivariate analysis, p=0.181

Mortality and iTTP relapses, iTTP-SjD vs idiopathic iTTP

Short-term outcomes (survival at day 30, relapse) were favourable

Treatments in iTTP-SjD

Plasma exchange
28
Rituximab
19
Caplacizumab
3
Corticosteroids

Original publication

Clinical, biological, prognostic characteristics of patients with immune-mediated thrombotic thrombocytopenic purpura and Sjögren’s disease. RMD Open. Published 29 August 2024. doi:10.1136/rmdopen-2024-004426

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