Skip to main content

Advertisement

SpringerLink
Log in

Primary antiphospholipid syndrome presenting as renal vein thrombosis and membranous nephropathy

  • Brief Report
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript

Abstract

Antiphospholipid syndrome is a multisystem auto-immune disorder characterized by thrombotic events and the presence of circulating antiphospholipid antibodies. Large vessel involvement in the form of thrombosis/stenosis and thrombotic microangiopathy is a commonly described renal finding. However, non-thrombotic glomerulopathies are increasingly being recognized in patients with antiphospholipid syndrome. We report a rare occurrence of both renal vein thrombosis and membranous nephropathy in a previously healthy adolescent male. Investigations revealed persistently positive antiphospholipid antibodies in the absence of an underlying systemic autoimmune disorder or malignancy. Our patient responded favourably to anti-proteinuric therapy and anticoagulation with complete resolution of proteinuria and a nearly occlusive thrombus.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

References

  1. Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, Derksen RH, DEG PG, Koike T, Meroni PL, Reber G, Shoenfeld Y, Tincani A, Vlachoyiannopoulos PG, Krilis SA (2006) International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 4:295–306

    Article  CAS  Google Scholar 

  2. Levine JS, Branch DW, Rauch J (2002) The antiphospholipid syndrome. N Engl J Med 346:752–763

    Article  CAS  Google Scholar 

  3. Uthman I, Khamashta M (2006) Antiphospholipid syndrome and the kidneys. Semin Arthritis Rheum 35:360–367

    Article  CAS  Google Scholar 

  4. D'Agati V, Kunis C, Williams G, Appel GB (1990) Anti-cardiolipin antibody and renal disease: a report three cases. J Am Soc Nephrol 1:777–784

    CAS  PubMed  Google Scholar 

  5. Levy Y, George J, Ziporen L, Cledes J, Amital H, Bar-Dayan Y, Afek A, Youinou P, Shoenfeld Y (1998) Massive proteinuria as a main manifestation of primary antiphospholipid syndrome. Pathobiology 66:49–52

    Article  CAS  Google Scholar 

  6. Dorel M, Daniel L, Liprandi A, Lerda D, Pellissier JF (2000) Idiopathic membranous glomerulonephritis associated with primary antiphospholipid syndrome. Nephron 86:366–367

    Article  CAS  Google Scholar 

  7. Fakhouri F, Noel LH, Zuber J, Beaufils H, Martinez F, Lebon P, Papo T, Chauveau D, Bletry O, Grunfeld JP, Piette JC, Lesavre P (2003) The expanding spectrum of renal diseases associated with antiphospholipid syndrome. Am J Kidney Dis 41:1205–1211

    Article  Google Scholar 

  8. Pengo V, Tripodi A, Reber G, Rand JH, Ortel TL, Galli M, De Groot PG (2009) Update of the guidelines for lupus anticoagulant detection. J Thromb Haemost 7:1737–1740

    Article  CAS  Google Scholar 

  9. Hanly JG (2003) Antiphospholipid syndrome: an overview. Can Med Assoc J 168:1675–1682

    Google Scholar 

  10. de Laat B, Mertens K, de Groot PG (2008) Mechanisms of disease: antiphospholipid antibodies-from clinical association to pathologic mechanism. Nat Clin Pract Rheumatol 4:192–199

    Article  Google Scholar 

  11. Espinosa G, Cervera R, Font J, Shoenfeld Y (2003) Antiphospholipid syndrome: pathogenic mechanisms. Autoimmun Rev 2:86–93

    Article  Google Scholar 

  12. Eddy AA, Symons JM (2003) Nephrotic syndrome in childhood. Lancet 362:629–639

    Article  Google Scholar 

  13. Kerlin BA, Blatt NB, Fuh B, Zhao S, Lehman A, Blanchong C, Mahan JD, Smoyer WE (2009) Epidemiology and risk factors for thromboembolic complications of childhood nephrotic syndrome: a Midwest Pediatric Nephrology Consortium (MWPNC) study. J Pediatr 155:105–110

    Article  Google Scholar 

  14. Favaloro EJ, Silvestrini R (2002) Assessing the usefulness of anticardiolipin antibody assays: a cautious approach is suggested by high variation and limited consensus in multilaboratory testing. Am J Clin Pathol 118:548–557

    Article  Google Scholar 

  15. Male C, Foulon D, Hoogendoorn H, Vegh P, Silverman E, David M, Mitchell L (2005) Predictive value of persistent versus transient antiphospholipid antibody subtypes for the risk of thrombotic events in pediatric patients with systemic lupus erythematosus. Blood 106:4152–4158

    Article  CAS  Google Scholar 

  16. Berkun Y, Padeh S, Barash J, Uziel Y, Harel L, Mukamel M, Revel-Vilk S, Kenet G (2006) Antiphospholipid syndrome and recurrent thrombosis in children. Arthritis Rheum 55:850–855

    Article  CAS  Google Scholar 

  17. Gattorno M, Falcini F, Ravelli A, Zulian F, Buoncompagni A, Martini G, Resti M, Picco P, Martini A (2003) Outcome of primary antiphospholipid syndrome in childhood. Lupus 12:449–453

    Article  CAS  Google Scholar 

  18. McClain MT, Arbuckle MR, Heinlen LD, Dennis GJ, Roebuck J, Rubertone MV, Harley JB, James JA (2004) The prevalence, onset, and clinical significance of antiphospholipid antibodies prior to diagnosis of systemic lupus erythematosus. Arthritis Rheum 50:1226–1232

    Article  Google Scholar 

  19. Arbuckle MR, McClain MT, Rubertone MV, Scofield RH, Dennis GJ, James JA, Harley JB (2003) Development of autoantibodies before the clinical onset of systemic lupus erythematosus. N Engl J Med 349:1526–1533

    Article  CAS  Google Scholar 

  20. Sinico RA, Cavazzana I, Nuzzo M, Vianelli M, Napodano P, Scaini P, Tincani A (2010) Renal involvement in primary antiphospholipid syndrome: retrospective analysis of 160 patients. Clin J Am Soc Nephrol 5:1211-1217

    Article  CAS  Google Scholar 

  21. Ohtomo Y, Nagaoka R, Watanabe H, Kaneko K, Ohyama S, Yamashiro Y (2000) Non-lupus nephropathy associated with antiphospholipid antibodies. Pediatr Nephrol 15:136–138

    Article  CAS  Google Scholar 

  22. Butani L (2004) End-stage renal disease from glomerulonephritis associated with anti-phospholipid syndrome. Pediatr Nephrol 19:812–814

    Article  Google Scholar 

  23. Garbrecht F, Gardner S, Johnson V, Grabowski E (1991) Deep venous thrombosis in a child with nephrotic syndrome associated with a circulating anticoagulant and acquired protein S deficiency. Am J Pediatr Hematol Oncol 13:330–333

    Article  CAS  Google Scholar 

  24. Beck LH Jr, Bonegio RG, Lambeau G, Beck DM, Powell DW, Cummins TD, Klein JB, Salant DJ (2009) M-type phospholipase A2 receptor as target antigen in idiopathic membranous nephropathy. N Engl J Med 361:11–21

    Article  CAS  Google Scholar 

  25. Levy Y, Ziporen L, Gilburd B, George J, Polak-Charcon S, Amital H, Cledes J, Youinou P, Shoenfeld Y (1996) Membranous nephropathy in primary antiphospholipid syndrome: description of a case and induction of renal injury in SCID mice. Hum Antibodies Hybridomas 7:91–96

    Article  CAS  Google Scholar 

  26. Fervenza FC, Sethi S, Specks U (2008) Idiopathic membranous nephropathy: diagnosis and treatment. Clin J Am Soc Nephrol 3:905–919

    Article  Google Scholar 

  27. Cameron JS, Healy MJ, Adu D (1990) The Medical Research Council trial of short-term high-dose alternate day prednisolone in idiopathic membranous nephropathy with nephrotic syndrome in adults. The MRC Glomerulonephritis Working Party. Q J Med 74:133–156

    Article  CAS  Google Scholar 

  28. Cattran DC, Delmore T, Roscoe J, Cole E, Cardella C, Charron R, Ritchie S (1989) A randomized controlled trial of prednisone in patients with idiopathic membranous nephropathy. N Engl J Med 320:210–215

    Article  CAS  Google Scholar 

  29. Ponticelli C, Zucchelli P, Imbasciati E, Cagnoli L, Pozzi C, Passerini P, Grassi C, Limido D, Pasquali S, Volpini T, Sasdelli M, Locatelli F (1984) Controlled trial of methylprednisolone and chlorambucil in idiopathic membranous nephropathy. N Engl J Med 310:946–950

    Article  CAS  Google Scholar 

  30. Chen M, Li H, Li XY, Lu FM, Ni ZH, Xu FF, Li XW, Chen JH, Wang HY (2010) Group CNMS Tacrolimus combined with corticosteroids in treatment of nephrotic idiopathic membranous nephropathy: a multicenter randomized controlled trial. Am J Med Sci 339:233-238

    Article  Google Scholar 

  31. Praga M, Barrio V, Juarez G, Luno J (2007) Tacrolimus monotherapy in membranous nephropathy: a randomized controlled trial. Kidney Int 71:924–930

    Article  CAS  Google Scholar 

  32. Bomback AS, Derebail VK, McGregor JG, Kshirsagar AV, Falk RJ, Nachman PH (2009) Rituximab therapy for membranous nephropathy: a systematic review. Clin J Am Soc Nephrol 4:734–744

    Article  CAS  Google Scholar 

  33. Erkan D, Lockshin MD (2009) New approaches for managing antiphospholipid syndrome. Nat Clin Pract Rheumatol 5:160–170

    PubMed  Google Scholar 

  34. Monagle P, Chalmers E, Chan A, DeVeber G, Kirkham F, Massicotte P, Michelson AD, American College of Chest P, Monagle P, Chalmers E, Chan A, DeVeber G, Kirkham F, Massicotte P, Michelson AD (2008) Antithrombotic therapy in neonates and children: American College of Chest Physicians evidence-based clinical practice guidelines (8th edn). Chest 133:887S–968S

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada

    Swasti Chaturvedi, Denis Geary & Christoph Licht

  2. Division of Haematology, The Hospital for Sick Children, Toronto, ON, Canada

    Leonardo Brandao

  3. University of Toronto, Toronto, ON, Canada

    Leonardo Brandao, Denis Geary & Christoph Licht

Authors
  1. Swasti Chaturvedi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Leonardo Brandao
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Denis Geary
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Christoph Licht
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Christoph Licht.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Chaturvedi, S., Brandao, L., Geary, D. et al. Primary antiphospholipid syndrome presenting as renal vein thrombosis and membranous nephropathy. Pediatr Nephrol 26, 979–985 (2011). https://doi.org/10.1007/s00467-011-1787-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00467-011-1787-z

Keywords

Search

Navigation