DOI: 10.12890/2026_007169

Triple-positive antiphospholipid syndrome with splenic infarction triggered by tocilizumab withdrawal in a high-titer ACPA-positive rheumatoid arthritis patient: an unmasking storm of polyautoimmunity

Authors

  • Francesco Elios Coviello Rheumatology Clinic, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy 0009-0009-7597-9245
  • Cinzia Rotondo Rheumatology Clinic, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy 0000-0003-1356-8423
  • Francesca D’Onofrio Rheumatology Clinic, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy
  • Raffaele Barile Rheumatology Clinic, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy 0009-0000-1081-7928
  • Francesco Paolo Cantatore Rheumatology Clinic, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy 0000-0001-9929-9177
  • Addolorata Corrado Rheumatology Clinic, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy 0000-0001-6823-1970

francescoelioscoviello@gmail.com

Keywords

Rheumatoid arthritis, splenic infarction, antiphospholipid syndrome, tocilizumab, anti-TNF, pathogenesis

Abstract

Introduction: We describe the occurrence of an uncommon thrombotic event, namely splenic infarction, as the onset of antiphospholipid syndrome (APS) after temporary suspension of anti-IL-6 treatment in a patient with rheumatoid arthritis (RA).
Case description: A 53-year-old patient with anti-citrullinated protein antibody (ACPA) and rheumatoid factor-positive RA, previously showing transient positivity for immunoglobulin (Ig)G anticardiolipin antibodies and lupus anticoagulant (LAC), refractory to multiple biological disease-modifying anti-rheumatic drugs (DMARDs), anti-tumour necrosis factor (TNF)-α agents, and rituximab, had been treated with tocilizumab since 2013 with good clinical response. After temporary suspension of tocilizumab for minor orthopaedic surgery complicated by skin abscess, he developed acute abdominal pain and computed tomography evidence of splenic infarction. Laboratory evaluation revealed elevated inflammatory markers and triple positivity for LAC, aCL IgG, and aB2GPI IgG, leading to the diagnosis of APS. Treatment with warfarin, methotrexate, and baricitinib resulted in stable disease control without recurrent thrombotic events.
Conclusion: Clinical stability in RA is a delicate balance between control of inflammation and avoidance of medication toxicity, especially in patients with high thrombotic risk and previous antiphospholipid positivity. Temporary withdrawal of biologic therapy may induce immunological rebound and thrombotic complications. This case highlights the importance of thrombotic risk stratification and multidisciplinary management when biologic therapies are suspended.

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References

  1. Barbhaiya M, Zuily S, Naden R, Hendry A, Manneville F, Amigo MC, et al. The 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria. Arthritis Rheumatol. 2023;75(10):1687-1702. doi: 10.1002/art.42624.
  2. Grygiel-Górniak B, Mazurkiewicz Ł. Positive antiphospholipid antibodies: observation or treatment? J Thromb Thrombolysis. 2023;56(2):301-314. doi: 10.1007/s11239-023-02834-6.
  3. Vaz JL, Fernandes V, Nogueira F, Arnóbio A, Levy RA. Infliximab-induced autoantibodies: a multicenter study. Clin Rheumatol. 2016;35(2):325-32. doi: 10.1007/s10067-015-3140-6.
  4. Hadfield MJ, Thumar JR. Antiphospholipid Syndrome Presenting With Splenic Infarct. J Clin Rheumatol. 2021;27(1):e19. doi: 10.1097/RHU.0000000000001207.
  5. Wand O, Tayer-Shifman OE, Khoury S, Hershko AY. A practical approach to infarction of the spleen as a rare manifestation of multiple common diseases. Ann Med. 2018;50(6):494-500. doi: 10.1080/07853890.2018.1492148.
  6. Jeleniewicz R, Majdan M, Targońska-Stępniak B, Dryglewska M. Prevalence of antiphospholipid antibodies in rheumatoid arthritis patients and relationship with disease activity. Pol Arch Med Wewn. 2012;122(10):480-6. doi: 10.20452/pamw.1415.
  7. Wu S, Peng W, Liang X, Wang W. Anti-citrullinated protein antibodies are associated with neutrophil extracellular trap formation in rheumatoid arthritis. J Clin Lab Anal. 2021;35(3):e23662. doi: 10.1002/jcla.23662.
  8. Duarte JH. Connective tissue diseases: Neutrophil extracellular traps--a mechanism of thrombosis in patients with antiphospholipid syndrome? Nat Rev Rheumatol. 2015;11(8):444. doi: 10.1038/nrrheum.2015.96.
  9. Amital H, Govoni M, Maya R, Meroni PL, Ori B, Shoenfeld Y, et al. Role of infectious agents in systemic rheumatic diseases. Clin Exp Rheumatol. 2008;26(1 Suppl 48):S27-32.
  10. Mancuso F, Angelini J, Zabotti A, Russiani F, Baraldo M, Quartuccio L, et al. Perioperative Management of Biologic and Targeted Synthetic DMARDs in Orthopedic Surgery: Balancing Infection Risk and Disease Control. Microorganisms. 2026;14(2):398. doi: 10.3390/microorganisms14020398.

Published

2026-10-21

How to Cite

1.
Coviello FE, Rotondo C, D’Onofrio F, Barile R, Cantatore FP, Corrado A. Triple-positive antiphospholipid syndrome with splenic infarction triggered by tocilizumab withdrawal in a high-titer ACPA-positive rheumatoid arthritis patient: an unmasking storm of polyautoimmunity. EJCRIM [Internet]. 2026 Oct. 21 [cited 2026 Nov. 5];13. Available from: https://ejcrim.com/index.php/ejcrim/article/view/7169