Pediatric Nephrology

, 26:979

Primary antiphospholipid syndrome presenting as renal vein thrombosis and membranous nephropathy

  • Swasti Chaturvedi
  • Leonardo Brandao
  • Denis Geary
  • Christoph Licht
Brief Report

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.

Keywords

Antiphospholipid syndrome Nephrotic syndrome Membranous nephropathy Deep venous thrombosis 

References

  1. 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 4:295–306PubMedCrossRefGoogle Scholar
  2. 2.
    Levine JS, Branch DW, Rauch J (2002) The antiphospholipid syndrome. N Engl J Med 346:752–763PubMedCrossRefGoogle Scholar
  3. 3.
    Uthman I, Khamashta M (2006) Antiphospholipid syndrome and the kidneys. Semin Arthritis Rheum 35:360–367PubMedCrossRefGoogle Scholar
  4. 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 1:777–784PubMedGoogle Scholar
  5. 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 66:49–52PubMedCrossRefGoogle Scholar
  6. 6.
    Dorel M, Daniel L, Liprandi A, Lerda D, Pellissier JF (2000) Idiopathic membranous glomerulonephritis associated with primary antiphospholipid syndrome. Nephron 86:366–367PubMedCrossRefGoogle Scholar
  7. 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 41:1205–1211PubMedCrossRefGoogle Scholar
  8. 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 7:1737–1740PubMedCrossRefGoogle Scholar
  9. 9.
    Hanly JG (2003) Antiphospholipid syndrome: an overview. Can Med Assoc J 168:1675–1682Google Scholar
  10. 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 4:192–199PubMedCrossRefGoogle Scholar
  11. 11.
    Espinosa G, Cervera R, Font J, Shoenfeld Y (2003) Antiphospholipid syndrome: pathogenic mechanisms. Autoimmun Rev 2:86–93PubMedCrossRefGoogle Scholar
  12. 12.
    Eddy AA, Symons JM (2003) Nephrotic syndrome in childhood. Lancet 362:629–639PubMedCrossRefGoogle Scholar
  13. 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 155:105–110PubMedCrossRefGoogle Scholar
  14. 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 118:548–557PubMedCrossRefGoogle Scholar
  15. 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 106:4152–4158PubMedCrossRefGoogle Scholar
  16. 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 55:850–855PubMedCrossRefGoogle Scholar
  17. 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 12:449–453PubMedCrossRefGoogle Scholar
  18. 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 50:1226–1232PubMedCrossRefGoogle Scholar
  19. 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 349:1526–1533PubMedCrossRefGoogle Scholar
  20. 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 5:1211-1217Google Scholar
  21. 21.
    Ohtomo Y, Nagaoka R, Watanabe H, Kaneko K, Ohyama S, Yamashiro Y (2000) Non-lupus nephropathy associated with antiphospholipid antibodies. Pediatr Nephrol 15:136–138PubMedCrossRefGoogle Scholar
  22. 22.
    Butani L (2004) End-stage renal disease from glomerulonephritis associated with anti-phospholipid syndrome. Pediatr Nephrol 19:812–814PubMedCrossRefGoogle Scholar
  23. 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 13:330–333PubMedCrossRefGoogle Scholar
  24. 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 361:11–21PubMedCrossRefGoogle Scholar
  25. 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 7:91–96PubMedGoogle Scholar
  26. 26.
    Fervenza FC, Sethi S, Specks U (2008) Idiopathic membranous nephropathy: diagnosis and treatment. Clin J Am Soc Nephrol 3:905–919PubMedCrossRefGoogle Scholar
  27. 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 74:133–156PubMedGoogle Scholar
  28. 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 320:210–215PubMedCrossRefGoogle Scholar
  29. 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 310:946–950PubMedCrossRefGoogle Scholar
  30. 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 339:233-238Google Scholar
  31. 31.
    Praga M, Barrio V, Juarez G, Luno J (2007) Tacrolimus monotherapy in membranous nephropathy: a randomized controlled trial. Kidney Int 71:924–930PubMedCrossRefGoogle Scholar
  32. 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 4:734–744PubMedCrossRefGoogle Scholar
  33. 33.
    Erkan D, Lockshin MD (2009) New approaches for managing antiphospholipid syndrome. Nat Clin Pract Rheumatol 5:160–170PubMedCrossRefGoogle Scholar
  34. 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–968SPubMedCrossRefGoogle Scholar

Copyright information

© IPNA 2011

Authors and Affiliations

  • Swasti Chaturvedi
    • 1
  • Leonardo Brandao
    • 2
    • 3
  • Denis Geary
    • 1
    • 3
  • Christoph Licht
    • 1
    • 3
  1. 1.Division of NephrologyThe Hospital for Sick ChildrenTorontoCanada
  2. 2.Division of HaematologyThe Hospital for Sick ChildrenTorontoCanada
  3. 3.University of TorontoTorontoCanada

Personalised recommendations