Long-Term Effect and Safety of Tocilizumab on Severe Adult-Onset Still’s Disease Accompanied by Thrombotic Thrombocytopenic Purpura - Letter to the Editor

Sep 29, 2026

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Letter to the Editor discusses corticosteroid-resistant adult-onset Still’s disease (AOSD) and reports a unique long-term clinical course. A severe AOSD case with thrombotic thrombocytopenic purpura (TTP) was initially treated with etanercept after induction failures, achieving control for several months before AOSD relapse. Switching to tocilizumab (IL-6 inhibitor) plus ongoing immunosuppression maintained complete AOSD remission for 14 years, with manageable infections. Fatal severe pneumonia and septic shock occurred at age 83.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages2

Reading Time5 min

AccessFree to download

Background

Corticosteroid resistance in AOSD

Case Course

Initial therapy and TTP management with etanercept

Relapse and switch to tocilizumab

Long-term outcome and infection events

Discussion and Conclusion

EuropeanJournalof Rheumatology DOI: 10. 5152/eurjrheum.2025.25029 Letter to the Editor Long-Term Effect and Safety of Tocilizumab on Severe Adult-Onset Still’s Disease Accompanied by Thrombotic Thrombocytopenic Purpura Tatsuya Suwabe1,2, Masayuki Yamanouchi1,2, Yoshifumi Ubara1,2, Naoki Sawa1,2 ORCID iDs of the authors: T.S. 0000-0003-0825-2512; M.Y. XXX; Y.U. XXX; N. S. XXX Cite this article as: SuwabeT, Yamanouchi M, UbaraY, Sawa N. Long-term effect and safety of tocilizumab on severe adult-onset Still’s disease accompanied by thrombotic thrombocytopenic purpura. Eur J Rheumatol. 2025, 12(4), 0029, doi: 10. 5152/eurjrheum.2025.25029. 1 Department of Nephrology and Rheumatology, Toranomon Hospital Kajigaya, Kawasaki, Japan 2 Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Tokyo, Japan Corresponding author: Tatsuya Suwabe E-mail: [suwabetat@gmail.com](suwabetat@gmail.com) [Received: March 18](Received: March 18), 2025 Revision Requested: May 27, 2025 Last Revision Received: July 26, 2025 Accepted: July 28, 2025 Publication Date: December 5, 2025 Copyright@Author(s) -Available online at [www.eurjrheumatol.org](www.eurjrheumatol.org). Content of this journal is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. To the Editor, Adult-onset Still’s disease (AOSD) is a multi-systemic inflammatory disease usually treated with corticosteroids; however, patients with AOSD are often resistant to corticosteroid therapy. Although biological products have been recommended for corticosteroid-resistant AOSD,1 their long-term effects and safety for patients with AOSD are unknown. We report a patient with severe AOSD who was successfully treated with tocilizumab for 14 years. We previously reported a 69-year-old Japanese woman suffering from severe AOSD2 who was refractory to intensive induction therapy including high-dose corticosteroid, cyclosporine A, and frequent plasma exchange. Ten days later, she developed thrombotic thrombocytopenic purpura (TTP) including thrombocytopenia, hemolytic anemia, acute renal failure, fever, and neuropsychiatric symptoms. She was in a fatal condition, and we started etanercept (aTNFα inhibitor [TNFαis]) as a salvage therapy. We selected etanercept because it might have a lower risk for serious infection than other drugs. Interleukin-1 (IL-1) inhibitor was not approved in Japan at the time. After the administration of etanercept (25 mg subcutaneous injection, twice/week), her condition improved dramatically and TTP and AOSD were completely controlled. However, after 4 months, symptoms of spike fever; skin rash; and elevated levels of serum ferritin, ALT, and LDH had gradually resumed, despite increasing the PSL dosage to 20 mg/day and the constant administration of etanercept. She was diagnosed with a relapse of AOSD. Etanercept was switched to tocilizumab (IL-6 inhibitor), and she achieved complete remission of AOSD. Subsequently, she continued to receive oral PSL 5 mg/day and cyclosporine A 75 mg/day plus tocilizumab 8 mg/kg (drip intravenous injection) every 4 weeks for 14 years. This controlled her AOSD completely. However, her consciousness disorder caused by multiple cerebral infarctions did not recover, and she remained in a vegetative state. She developed a urinary tract infection at 7 years and pneumonia at 8 years after starting tocilizumab; however, these were not severe, and she completely recovered. The administration of tocilizumab was suspended when she had serious infections. Symptoms related to AOSD relapses did not appear; therefore, it was considered that she had sustained remission. At 14 years after the onset of AOSD, she developed severe pneumonia and septic shock, which caused death at the age of 83 years. Severe AOSD accompanied by TTP is often refractory; however, AOSD in this patient was well controlled by tocilizumab for 14 years. This patient eventually became refractory t

FAQs

Why is tocilizumab considered in corticosteroid-resistant adult-onset Still’s disease?

Biological products are recommended for corticosteroid-resistant AOSD, but long-term effects and safety were unclear. The letter presents long-term experience supporting its use.

What happened after etanercept treatment in the reported case?

Etanercept improved TTP and AOSD initially, but after about 4 months AOSD symptoms gradually returned and the patient was diagnosed with relapse.

How long did tocilizumab control the patient’s severe AOSD, and what was the final outcome?

Tocilizumab maintained complete AOSD remission for 14 years. The patient later developed severe pneumonia and septic shock and died at age 83.

Sep 29, 2026

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