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. 2024 Aug 29;10(3):e004426.
doi: 10.1136/rmdopen-2024-004426.

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

Justine Luciano  1   2 , Laurent Gilardin  3   4   5 , Gaétane Nocturne  1   2 , Raïda Bouzid  4   5 , Agnès Veyradier  4   5   6 , Xavier Mariette  1   2 , Paul Coppo  4   5   7 , Isabelle Bonnet  2   8 , Bérangère S Joly  9   5   6
Affiliations

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

Justine Luciano et al. RMD Open. .

Abstract

Objectives: The association between immune-mediated thrombotic thrombocytopenic purpura (iTTP) and Sjögren disease (SjD) has been poorly investigated. This study presents the first retrospective cohort of iTTP-SjD aiming to identify risk factors for iTTP occurrence in SjD patients and examine their clinical course.

Methods: Patients with iTTP-SjD were identified within the French TTP Registry based on American College of Rheumatology/European League Against Rheumatism 2016 criteria. A comparative analysis was conducted with two control groups comprising primary SjD (pSjD) patients from the French ASSESS cohort and idiopathic iTTP patients from the French TTP Registry. Demographic, clinical and biological data were retrospectively collected.

Results: Thirty iTTP-SjD patients were included and compared with 65 pSjD and 45 idiopathic iTTP patients. The majority of iTTP-SjD patients (n=18) were diagnosed with SjD at the time of iTTP diagnosis. In comparison with the pSjD cohort, iTTP-SjD patients were diagnosed with SjD at a younger age (p=0.039) and showed a higher prevalence of anti-SjS-related antigen A antibody positivity and xerostomia (p=0.015, p=0.035, respectively). EULAR Sjogren's Syndrome Disease Activity Index showed similar activity levels between the two groups. iTTP-SjD patients were treated with plasma exchange (n=28), corticosteroids, rituximab (n=19) and caplacizumab (n=3). In comparison with the idiopathic iTTP cohort, mortality rates (log-rank tests, p=0.228), biological and clinical iTTP relapses (multivariate analysis, p=0.181) were comparable and short-term outcomes (survival at day 30, relapse) were favourable.

Conclusion: iTTP can be a rare complication in patients with SjD. Further studies involving larger cohorts and long-term follow-up are warranted to confirm these findings and to explore the efficacy of immunomodulators and caplacizumab in iTTP-SjD patients.

Keywords: Autoantibodies; B cells; Sjogren's Syndrome; Thrombocytopenia.

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Conflict of interest statement

Competing interests: GN received speaker fees from Novartis, Amgen, Galapagos and BMS. AV is a member of the French advisory boards for Sanofi and Takeda and received speaker fees from Sanofi, Octapharma, LFB-Biomédicaments and Takeda. XM received fees from BMS, Galapagos, GSK, Novartis and Pfizer. PC is a member of advisory boards and received speaker fees from Sanofi, Alexion, Octapharma and Takeda. IB received speaker fees from Galapagos. BSJ received speaker fees from Sanofi, Takeda and LFB-Biomédicaments. JL, LG and RB declare no conflicts of interest.

Figures

Figure 1
Figure 1. Flow chart of the study. ACR-EULAR, American College of Rheumatology-EULAR; CRI, Club Rhumatismes et Inflammation; iTTP, immune-mediated TTP; SjD, Sjögren’s disease; TMA, thrombotic microangiopathy; TTP, thrombotic thrombocytopenic purpura.
Figure 2
Figure 2. Kaplan-Meier curves showing clinical and ADAMTS13-relapse free survival by the time of diagnosis in the two groups. ADAMTS13, A Disintegrin And Metalloproteinase with Thrombospondin Motifs 13; iTTP, immune-mediated TTP; SjD, Sjögren’s disease; TTP, thrombotic thrombocytopenic purpura.
See this image and copyright information in PMC

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