Clinical Rheumatology

, Volume 35, Issue 8, pp 2131–2136 | Cite as

Pure lupus podocytopathy first presenting as thrombotic thrombocytopenic purpura-like syndrome

  • Guillermo Delgado-García
  • Carlos Cámara-Lemarroy
  • Adrián Infante-Valenzuela
  • Perla Colunga-Pedraza
  • Gabriela Alarcón-Galván
  • Arian Armenta-González
  • Héctor Jorge Villarreal-Velázquez
Case Based Review


Lupus podocytopathy (LP) is an uncommon proteinuric disorder in the spectrum of lupus nephropathy. Its histological features are similar to those described in minimal change disease (MCD) with or without mesangial immune deposits. Although infrequent, a close relationship between systemic lupus erythematosus (SLE) and thrombotic thrombocytopenic purpura (TTP) is well accepted. Proteinuria in the setting of SLE has previously been associated with the development of TTP-like syndrome. As far as we know, LP first presenting as a TTP-like syndrome has never been reported. Here, we describe the case of a previously healthy 45-year-old woman who developed simultaneously these two conditions and then we briefly review the literature on the topic, emphasizing the previous cases of concurrent initial diagnosis of both SLE and MCD (n = 7) and SLE and TTP (n = 72). In conclusion, renal biopsy is central to the management of SLE patients with nephrotic syndrome. Furthermore, in a SLE patient with anemia and thrombocytopenia, TTP should be part of the differential diagnosis, even when no schistocytes were detected in peripheral blood smear.


Minimal change disease NSAIDs Podocytopathy Systemic lupus erythematosus Thrombotic microangiopathy Thrombotic thrombopenic purpura 





  1. 1.
    Lewis EJ (2010) Lupus podocytopathy. In: Lupus Nephritis. Oxford University Press, Oxford, pp 199–210CrossRefGoogle Scholar
  2. 2.
    Mok CC, Cheung TT, Lo WH (2010) Minimal mesangial lupus nephritis: a systematic review. Scand J Rheumatol 39(3):181–189CrossRefPubMedGoogle Scholar
  3. 3.
    Matsuyama T, Kuwana M, Matsumoto M, Isonishi A, Inokuma S, Fujimura Y (2009) Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases. Thromb Haemost 102(2):371–378PubMedGoogle Scholar
  4. 4.
    Jiang H, An X, Li Y, Sun Y, Shen G, Tu Y, Tao J (2014) Clinical features and prognostic factors of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a literature review of 105 cases from 1999 to 2011. Clin Rheumatol 33(3):419–427CrossRefPubMedGoogle Scholar
  5. 5.
    Hunt BJ, Tueger S, Pattison J, Cavenagh J, D’Cruz DP (2007) Microangiopathic haemolytic anaemia secondary to lupus nephritis: an important differential diagnosis of thrombotic thrombocytopenic purpura. Lupus 16(5):358–362CrossRefPubMedGoogle Scholar
  6. 6.
    Wang Y, Yu F, Song D, Wang SX, Zhao MH (2014) Podocyte involvement in lupus nephritis based on the 2003 ISN/RPS system: a large cohort study from a single centre. Rheumatology (Oxford) 53(7):1235–1244CrossRefGoogle Scholar
  7. 7.
    Matsumura N, Dohi K, Shiiki H, Morita H, Yamada H, Fujimoto J et al (1989) Three cases presenting with systemic lupus erythematosus and minimal change nephrotic syndrome. Nihon Jinzo Gakkai Shi 31(9):991–999PubMedGoogle Scholar
  8. 8.
    Guery B, Martinez F, Beaufils H, Deray G (1997) Minimal-change nephropathy associated with systemic lupus erythematosus. Nephrol Dial Transplant 12(9):2030–2031CrossRefPubMedGoogle Scholar
  9. 9.
    Seo DB, Lee SW, Song JH, Lee KJ, Han JY, Kim MJ (2002) Acute renal failure associated with a minimal change nephrotic syndrome in a systemic lupus erythematosus patient. Yonsei Med J 43(1):114–118CrossRefPubMedGoogle Scholar
  10. 10.
    Hertig A, Droz D, Lesavre P, Grünfeld JP, Rieu P (2002) SLE and idiopathic nephrotic syndrome: coincidence or not? Am J Kidney Dis 40(6):1179–1184CrossRefPubMedGoogle Scholar
  11. 11.
    Hsieh RY, Wu AB, Chang YT, Cheng MF, Wein-Chung Chen WC, Huang JJ (2007) Minimal change nephrotic syndrome in two patients with suspected systemic lupus erythematosus: association or co-incidence. Acta Nephrologica 21(1):52–57Google Scholar
  12. 12.
    Moysés-Neto M, Costa RS, Rodrigues FF, Vieira Neto OM, Reis MA, Louzada Júnior P et al (2011) Minimal change disease: a variant of lupus nephritis. NDT Plus 4(1):20–22PubMedGoogle Scholar
  13. 13.
    Shahane A, Khasnis A, Gota C (2012) Rituximab-responsive nephrotic syndrome due to minimal change disease in systemic lupus erythematosus. J Clin Rheumatol 18(4):199–202CrossRefPubMedGoogle Scholar
  14. 14.
    Hu WX, Chen YH, Bao H, Liu ZZ, Wang SF, Zhang HT, Liu ZH (2015) Glucocorticoid with or without additional immunosuppressant therapy for patients with lupus podocytopathy: a retrospective single-center study. Lupus 24:1067–75. doi: 10.1177/0961203315578766 CrossRefPubMedGoogle Scholar
  15. 15.
    Dube GK, Markowitz GS, Radhakrishnan J, Appel GB, D’Agati VD (2002) Minimal change disease in systemic lupus erythematosus. Clin Nephrol 57(2):120–126CrossRefPubMedGoogle Scholar
  16. 16.
    Harirforoosh S, Jamali F (2009) Renal adverse effects of nonsteroidal anti-inflammatory drugs. Expert Opin Drug Saf 8(6):669–681CrossRefPubMedGoogle Scholar
  17. 17.
    Whelton A (1999) Nephrotoxicity of nonsteroidal anti-inflammatory drugs: physiologic foundations and clinical implications. Am J Med 106(5B):13S–24SCrossRefPubMedGoogle Scholar
  18. 18.
    Wang YT, Chou HH, Chen FF, Chen MJ, Chiou YY (2006) A case of minimal-change nephrotic syndrome in pediatric lupus erythematosus: just a coincidence? Lupus 15(4):244–247CrossRefPubMedGoogle Scholar
  19. 19.
    Klonizakis P, Tselios K, Sarantopoulos A, Gougourellas I, Rouka E, Onufriadou Z et al (2013) ADAMTS-13 metalloprotease abnormalities in systemic lupus erythematosus: is there a correlation with disease status? Lupus 22(5):443–452CrossRefPubMedGoogle Scholar
  20. 20.
    Dold S, Singh R, Sarwar H, Menon Y, Candia L, Espinoza LR (2005) Frequency of microangiopathic hemolytic anemia in patients with systemic lupus erythematosus exacerbation: distinction from thrombotic thrombocytopenic purpura, prognosis, and outcome. Arthritis Rheum 53(6):982–985CrossRefPubMedGoogle Scholar
  21. 21.
    Lansigan F, Isufi I, Tagoe CE (2011) Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus: the role of ADAMTS13. Rheumatology (Oxford) 50(5):824–829CrossRefGoogle Scholar
  22. 22.
    Merayo-Chalico J, Demichelis-Gómez R, Rajme-López S, Aparicio-Vera L, Barrera-Vargas A, Alcocer-Varela J et al (2014) Risk factors and clinical profile of thrombotic thrombocytopenic purpura in systemic lupus erythematosus patients. Is this a distinctive clinical entity in the thrombotic microangiopathy spectrum?: a case control study. Thromb Res 134(5):1020–1027CrossRefPubMedGoogle Scholar
  23. 23.
    Musio F, Bohen EM, Yuan CM, Welch PG (1998) Review of thrombotic thrombocytopenic purpura in the setting of systemic lupus erythematosus. Semin Arthritis Rheum 28(1):1–19CrossRefPubMedGoogle Scholar
  24. 24.
    El Khayat SS, Medkouri G, Etomba AM, Zamd M, Gharbi MB, Ramadani B (2012) Thrombotic thrombocytopenic purpura and systemic lupus erythematosus: a rare and life-threatening association. Arab J Nephrol Transplant 5(2):103–105PubMedGoogle Scholar
  25. 25.
    George JN, Chen Q, Deford CC, Al-Nouri Z (2012) Ten patient stories illustrating the extraordinarily diverse clinical features of patients with thrombotic thrombocytopenic purpura and severe ADAMTS13 deficiency. J Clin Apher 27(6):302–311CrossRefPubMedGoogle Scholar
  26. 26.
    Campos LM, Spadoni MS, Michelin CM, Jesus AA, Carneiro JD, da Silva CA (2013) Thrombotic thrombocytopenic purpura at presentation of juvenile systemic lupus erythematosus patients. Rev Bras Reumatol 53(1):120–126CrossRefPubMedGoogle Scholar
  27. 27.
    Cui J, Zhu TN, Zhao YQ, Zhang FC (2013) The clinical analysis of 14 cases of systemic lupus erythematosus complicated by thrombotic thrombocytopenic purpura. Zhonghua Nei Ke Za Zhi 52(5):383–386PubMedGoogle Scholar
  28. 28.
    Palaniappan P, Lionel AP, Agarwal I, Mathai S, Mathew L, Agarwal S et al (2014) Cryosupernatant and immunosuppression as effective alternative therapies for TTP in three pediatric SLE patients. Indian J Hematol Blood Transfus 30(Suppl 1):232–236CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Hassan ATA, Elbadry MIY, Tawfik MA (2014) A case report of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: overlapping features. Am J Med Case Rep 2(10):206–213Google Scholar
  30. 30.
    Changcharoen B, Bolger DT Jr (2015) Thrombotic thrombocytopenic purpura as an initial presentation of systemic lupus erythematosus with acquired ADAMTS 13 antibody. BMJ Case Rep 20:2015Google Scholar
  31. 31.
    Prasun Giri P, Sinha R, Pal P, Sarkar B (2015) Therapeutic plasma exchange in paediatric SLE: a case series from India. Lupus 24(8):889–891CrossRefPubMedGoogle Scholar
  32. 32.
    Song D, Wu LH, Wang FM, Yang XW, Zhu D, Chen M et al (2013) The spectrum of renal thrombotic microangiopathy in lupus nephritis. Arthritis Res Ther 15(1):R12CrossRefPubMedPubMedCentralGoogle Scholar
  33. 33.
    Zini G, d’Onofrio G, Briggs C, Erber W, Jou JM, Lee SH et al (2012) ICSH recommendations for identification, diagnostic value, and quantitation of schistocytes. Int J Lab Hematol 34(2):107–116CrossRefPubMedGoogle Scholar
  34. 34.
    Oregel KZ, Ramdial J, Glück S (2013) Nonsteroidal anti-inflammatory drug induced thrombotic thrombocytopenic purpura. Clin Med Insights Blood Disord 6:19–22PubMedPubMedCentralGoogle Scholar
  35. 35.
    Hay SN, Egan JA, Millward PA, Bandarenko N, Brecher ME (2006) Patterns of platelet response in idiopathic TTP/HUS: frequency of declining platelet counts with plasma exchange and the recognition and significance of a pseudo refractory state. Ther Apher Dial 10(3):237–241CrossRefPubMedGoogle Scholar
  36. 36.
    Tovoli F, De Giorgio R, Fabbri A et al (2011) Thrombotic thrombocytopenic purpura mimicking an acute meningoencephalitis. Acta Clin Belg 66(1):55–56CrossRefPubMedGoogle Scholar
  37. 37.
    Nafil H, Tazi I, Mahmal L (2012) Purpura thrombotique thrombocytopénique (PTT) simulant une méningo-encéphalite. Rev Neurol (Paris) 168(11):873–874CrossRefGoogle Scholar
  38. 38.
    Bortoluzzi A, Scirè CA, Bombardieri S, Caniatti L, Conti F, De Vita S et al (2015) Development and validation of a new algorithm for attribution of neuropsychiatric events in systemic lupus erythematosus. Rheumatology (Oxford) 54(5):891–898CrossRefGoogle Scholar
  39. 39.
    Morís G, García-Moncó JC (2014) Drug-induced aseptic meningitis and other mimics. In: CNS infections. Springer, London, pp 249–271CrossRefGoogle Scholar
  40. 40.
    Weksler BB, Lehany AM (1991) Naproxen-induced recurrent aseptic meningitis. DICP 25(11):1183–1184PubMedGoogle Scholar
  41. 41.
    Gao H, Li C, Mu R, Guo YQ, Liu T, Chen S et al (2011) Subclinical hypothyroidism and its association with lupus nephritis: a case control study in a large cohort of Chinese systemic lupus erythematosus patients. Lupus 20(10):1035–1041CrossRefPubMedGoogle Scholar
  42. 42.
    Shen L, Lu G, Dong N, Ma Z, Ruan C (2013) Simvastatin increases ADAMTS13 expression in podocytes. Thromb Res 132(1):94–99CrossRefPubMedGoogle Scholar
  43. 43.
    Thomas MR, McDonald V, Machin SJ, Scully MA (2011) Thrombotic thrombocytopenic purpura associated with statin therapy. Blood Coagul Fibrinolysis 22(8):762–763CrossRefPubMedGoogle Scholar

Copyright information

© International League of Associations for Rheumatology (ILAR) 2015

Authors and Affiliations

  • Guillermo Delgado-García
    • 1
  • Carlos Cámara-Lemarroy
    • 2
  • Adrián Infante-Valenzuela
    • 2
  • Perla Colunga-Pedraza
    • 3
  • Gabriela Alarcón-Galván
    • 4
  • Arian Armenta-González
    • 5
  • Héctor Jorge Villarreal-Velázquez
    • 2
  1. 1.Department of Internal Medicine, University HospitalAutonomous University of Nuevo LeónMonterreyMexico
  2. 2.Division of Neurology, University HospitalAutonomous University of Nuevo LeónMonterreyMexico
  3. 3.Division of Hematology, University HospitalAutonomous University of Nuevo LeónMonterreyMexico
  4. 4.Service of Anatomic Pathology, University HospitalAutonomous University of Nuevo LeónMonterreyMexico
  5. 5.Division of Rheumatology, University HospitalAutonomous University of Nuevo LeónMonterreyMexico

Personalised recommendations