Clinical Rheumatology

, Volume 37, Issue 1, pp 213–218 | Cite as

TMA secondary to SLE: rituximab improves overall but not renal survival

  • Fangfang Sun
  • Xiaodong Wang
  • Wanlong Wu
  • Kaiwen Wang
  • Zhiwei Chen
  • Ting LiEmail author
  • Shuang YeEmail author
Original Article


Thrombotic microangiopathy (TMA) includes a series of life-threatening disorders. Systemic lupus erythematosus (SLE) is one of the most common acquired causes. To identify predictors of prognosis in patients with TMA secondary to SLE, we conducted a single-center historical study. From January 2013 to June 2016, of 2182 SLE hospitalized patients in the Ren Ji Hospital, a total of 21 consecutive patients with TMA secondary to SLE were identified. The 90-day short-term mortality was 33.3%. The kidney involvement (66.7%) was associated with poor prognosis, while the administration of rituximab (n = 13) was an independent protective factor according to logistic regression analysis. Compared to conventional treatment, i.e., plasma exchange, high-dose glucocorticoids, and intravenous immunoglobulin, the overall survival is significantly higher among patients receiving rituximab add-on (92.2 vs 33.3%, p = 0.0173); however, five out of seven patients with renal involvement in the rituximab group were eventually hemodialysis dependent. Our data indicated that add-on rituximab in the background of conventional therapy may improve the overall but not the renal survival in SLE-TMA patients.


Rituximab Systemic lupus erythematosus Thrombotic microangiopathy Thrombotic thrombocytopenic purpura 



We are grateful for the technical support from colleagues of IT department.

Author contributions

All authors took part in revising the article and approved the final version to be published. S.Y. and T.L. contributed to the conception and design. W.W., K.W., and Z.C. were responsible for collecting the data. F.S. and X.W. performed the analysis and drafted the article.

F.S. and X.W. contributed equally to this article.


This study was supported by the Scientific Research Foundation of Ren Ji Hospital South Campus, School of Medicine, Shanghai Jiao Tong University (2016PWGZR03).

Compliance with ethical standards

Ethics approval and consent to participate

The protocol of this study complied with the recommendations of the Declaration of Helsinki and was approved by the ethics committees of the Ren Ji Hospital. An informed consent for off-label use of rituximab had been obtained from all patients.




  1. 1.
    George JN, Nester CM (2014) Syndromes of thrombotic microangiopathy. N Engl J Med 371(7):654–666. CrossRefPubMedGoogle Scholar
  2. 2.
    Blombery P, Kivivali L, Pepperell D, McQuilten Z, Engelbrecht S, Polizzotto MN, Phillips LE, Wood E, Cohney S, committee TTPrs (2016) Diagnosis and management of thrombotic thrombocytopenic purpura (TTP) in Australia: findings from the first 5 years of the Australian TTP/thrombotic microangiopathy registry. Intern Med J 46(1):71–79. CrossRefPubMedGoogle Scholar
  3. 3.
    Mehmood T, Taylor M, Winters JL (2016) Management of thrombotic microangiopathic hemolytic anemias with therapeutic plasma exchange: when it works and when it does not. Hematol Oncol Clin North Am 30(3):679–694. CrossRefPubMedGoogle Scholar
  4. 4.
    Schneider M (2007) Thrombotic microangiopathy (TTP and HUS): advances in differentiation and diagnosis. Clin Lab Sci 20(4):216–220PubMedGoogle Scholar
  5. 5.
    Forzley BR, Clark WF (2009) TTP/HUS and prognosis: the syndrome and the disease(s). Kidney Int Suppl 112:S59–S61. CrossRefGoogle Scholar
  6. 6.
    von Baeyer H (2002) Plasmapheresis in thrombotic microangiopathy-associated syndromes: review of outcome data derived from clinical trials and open studies. Ther Apher 6(4):320–328CrossRefGoogle Scholar
  7. 7.
    Peyvandi F, Scully M, Kremer Hovinga JA, Cataland S, Knobl P, Wu H, Artoni A, Westwood JP, Mansouri Taleghani M, Jilma B, Callewaert F, Ulrichts H, Duby C, Tersago D, Investigators T (2016) Caplacizumab for acquired thrombotic thrombocytopenic purpura. N Engl J Med 374(6):511–522. CrossRefPubMedGoogle Scholar
  8. 8.
    Coppo P (2012) Thrombotic microangiopathies: from empiricism to targeted therapies. Presse Med 41(3 Pt 2):e101–e104. CrossRefPubMedGoogle Scholar
  9. 9.
    Wieland I, Kentouche K, Jentzsch M, Lothschutz D, Graf N, Sykora KW (2015) Long-term remission of recurrent thrombotic thrombocytopenic purpura (TTP) after rituximab in children and young adults. Pediatr Blood Cancer 62(5):823–829. CrossRefPubMedGoogle Scholar
  10. 10.
    Miyakawa Y (2014) Recent progress of diagnosis and treatment for immune-mediated hematological diseases. Topics: IV. Recent topics; 2. Rituximab for ITP and TTP. Nihon Naika Gakkai Zasshi 103(7):1654–1659CrossRefPubMedGoogle Scholar
  11. 11.
    Scully M (2012) Rituximab in the treatment of TTP. Hematology 17(Suppl 1):S22–S24. PubMedGoogle Scholar
  12. 12.
    Kafle P, Malakoff GL (2012) Coexistence of systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case report. Tenn Med 105(5):37–38PubMedGoogle Scholar
  13. 13.
    Niaz FA, Aleem A (2010) Response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus. Saudi J Kidney Dis Transpl 21(1):109–112PubMedGoogle Scholar
  14. 14.
    Kamiya K, Kurasawa K, Arai S, Maezawa R, Hanaoka R, Kumano K, Fukuda T (2010) Rituximab was effective on refractory thrombotic thrombocytopenic purpura but induced a flare of hemophagocytic syndrome in a patient with systemic lupus erythematosus. Mod Rheumatol 20(1):81–85. CrossRefPubMedGoogle Scholar
  15. 15.
    Miyamura T, Watanabe H, Takahama S, Sonomoto K, Nakamura M, Ando H, Minami R, Yamamoto M, Suematsu E (2008) Thrombotic thrombocytopenic purpura in patients with systemic lupus erythematosus. Nihon Rinsho Men'eki Gakkai Kaishi 31(3):159–165CrossRefPubMedGoogle Scholar
  16. 16.
    Hundae A, Peskoe S, Grimsley E, Patel S (2008) Rituximab therapy for refractory thrombotic thrombocytopenic purpura and autoimmune-mediated thrombocytopenia in systemic lupus erythematosus. South Med J 101(9):943–944. CrossRefPubMedGoogle Scholar
  17. 17.
    Shah AA, Higgins JP, Chakravarty EF (2007) Thrombotic microangiopathic hemolytic anemia in a patient with SLE: diagnostic difficulties. Nat Clin Pract Rheumatol 3(6):357–362. CrossRefPubMedGoogle Scholar
  18. 18.
    Chen MH, Chen MH, Chen WS, Mu-Hsin Chang P, Lee HT, Lin HY, Huang DF (2011) Thrombotic microangiopathy in systemic lupus erythematosus: a cohort study in North Taiwan. Rheumatology 50(4):768–775. CrossRefPubMedGoogle Scholar
  19. 19.
    Lansigan F, Isufi I, Tagoe CE (2011) Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus: the role of ADAMTS13. Rheumatology 50(5):824–829. CrossRefPubMedGoogle Scholar
  20. 20.
    Letchumanan P, Ng HJ, Lee LH, Thumboo J (2009) A comparison of thrombotic thrombocytopenic purpura in an inception cohort of patients with and without systemic lupus erythematosus. Rheumatology 48(4):399–403. CrossRefPubMedGoogle Scholar
  21. 21.
    Kwok SK, Ju JH, Cho CS, Kim HY, Park SH (2009) Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: risk factors and clinical outcome: a single centre study. Lupus 18(1):16–21. CrossRefPubMedGoogle Scholar
  22. 22.
    Maruoka H, Koga T, Inoue Y, Honda S, Takeo M, Fukuda T, Aizawa H (2007) Recurrent thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus. Mod Rheumatol 17(5):426–428. CrossRefPubMedGoogle Scholar
  23. 23.
    Benhamou Y, Paintaud G, Azoulay E, Poullin P, Galicier L, Desvignes C, Baudel JL, Peltier J, Mira JP, Pene F, Presne C, Saheb S, Deligny C, Rousseau A, Feger F, Veyradier A, Coppo P, French Reference Center for Thrombotic M (2016) Efficacy of a rituximab regimen based on B cell depletion in thrombotic thrombocytopenic purpura with suboptimal response to standard treatment: results of a phase II, multicenter non-comparative study. Am J Hematol.
  24. 24.
    Cuker A (2016) Adjuvant rituximab to prevent TTP relapse. Blood 127(24):2952–2953. CrossRefPubMedGoogle Scholar
  25. 25.
    Jayabose S, Dunbar J, Nowicki TS, Tugal O, Ozkaynak MF, Sandoval C (2011) Rituximab therapy to prevent relapse in chronic relapsing thrombotic thrombocytopenic purpura (TTP) in a child. Pediatr Hematol Oncol 28(2):167–172. CrossRefPubMedGoogle Scholar
  26. 26.
    Abou-Nassar K, Karsh J, Giulivi A, Allan D (2010) Successful prevention of thrombotic thrombocytopenic purpura (TTP) relapse using monthly prophylactic plasma exchanges throughout pregnancy in a patient with systemic lupus erythematosus and a prior history of refractory TTP and recurrent fetal loss. Transfus Apher Sci 43(1):29–31. CrossRefPubMedGoogle Scholar
  27. 27.
    Jin M, Casper TC, Cataland SR, Kennedy MS, Lin S, Li YJ, Wu HM (2008) Relationship between ADAMTS13 activity in clinical remission and the risk of TTP relapse. Br J Haematol 141(5):651–658. CrossRefPubMedGoogle Scholar

Copyright information

© International League of Associations for Rheumatology (ILAR) 2017

Authors and Affiliations

  1. 1.Department of Rheumatology, South Campus, Ren Ji Hospital, School of MedicineShanghai Jiao Tong UniversityShanghaiChina

Personalised recommendations