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  • Review Article
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Renal involvement in antiphospholipid syndrome

Nature Reviews Nephrology volume 10, pages 279–289 (2014)Cite this article

Subjects

Key Points

  • Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent arterial or venous thrombosis and/or pregnancy morbidity and persistently elevated levels of antiphospholipid antibodies

  • Renal involvement can be caused by thrombosis at any level within the vasculature of the kidney

  • Clinical features include renal artery stenosis, renal infarction, renal vein thrombosis, thrombotic microangiopathy and hypertension

  • Treatment of renal involvement in APS is centred on anticoagulation with long-term warfarin

  • Patients who are refractory to standard anticoagulation therapy or who have catastrophic APS might benefit from targeted therapy, including the use of intravenous immunoglobulins, rituximab or eculizumab

Abstract

Antiphospholipid syndrome (APS) is an autoimmune disease defined by the presence of arterial or venous thrombotic events and/or pregnancy morbidity in patients who test positive for antiphospholipid antibodies (aPLs). APS can be isolated (known as primary APS) or associated with other autoimmune diseases, such as systemic lupus erythematosus (SLE; known as secondary APS). The kidney is a major target organ in APS and renal thrombosis can occur at any level within the vasculature of the kidney (renal arteries, intrarenal arteries, glomerular capillaries and renal veins); events reflect the site and size of the involved vessels. Histological findings vary widely, including ischaemic glomeruli and thrombotic lesions without glomerular or arterial immune deposits on immunofluorescence. Renal prognosis is affected by the presence of aPLs in patients with lupus nephritis and can be poor. In patients with SLE and aPLs, biopsy should be performed because inflammatory and thrombotic lesions require different therapeutic approaches. Renal involvement in patients with definite APS is treated by anticoagulation with long-term warfarin. The range of renal manifestations associated with APS is broadening and, therefore, aPLs have increasing relevance in end-stage renal disease, transplantation and pregnancy.

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Figure 1: Venn diagram of the clinical presentation of APS.
Figure 2: Pathophysiological mechanisms of thrombogenesis induced by antiphospholipid antibodies.
Figure 3: Histological characterization of APS-associated nephropathy.

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References

  1. Hughes, G. R. Thrombosis, abortion, cerebral disease, and the lupus anticoagulant. Br. Med. J. (Clin. Res. Ed.) 287, 1088–1089 (1983).

    Article  CAS  Google Scholar 

  2. Khamashta, M., Sciascia, S. & Bertolaccini, M. L. in Autoantibodies Vol 1 3rd edition (eds Shoenfeld, Y., Meroni, P. & Gershwin, M.) 75–98 (Elsevier, 2013).

    Google Scholar 

  3. Wilson, W. A. et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum. 42, 1309–1311 (1999).

    Article  CAS  PubMed  Google Scholar 

  4. Miyakis, S. et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J. Thromb. Haemost. 4, 295–306 (2006).

    Article  CAS  PubMed  Google Scholar 

  5. Petri, M. in Hughes Syndrome Vol. 1 2nd edition (ed. Khamashta, M. A.) 22–28 (Springer, 2006).

    Book  Google Scholar 

  6. Petri, M. Epidemiology of the antiphospholipid antibody syndrome. J. Autoimmun. 15, 145–151 (2000).

    Article  CAS  PubMed  Google Scholar 

  7. Khamashta, M. in Hughes Syndrome Vol. 1 2nd edition (ed. Khamashta, M. A.) 3–8 (Springer, 2006).

    Book  Google Scholar 

  8. Cervera, R. et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 46, 1019–1027 (2002).

    Article  PubMed  Google Scholar 

  9. Cervera, R. et al. Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (I): catastrophic APS, APS nephropathy and heart valve lesions. Lupus 20, 165–173 (2011).

    Article  CAS  PubMed  Google Scholar 

  10. Meroni, P. L. & Riboldi, P. Pathogenic mechanisms mediating antiphospholipid syndrome. Curr. Opin. Rheumatol. 13, 377–382 (2001).

    Article  CAS  PubMed  Google Scholar 

  11. Giannakopoulos, B. & Krilis, S. A. The pathogenesis of the antiphospholipid syndrome. N. Engl. J. Med. 368, 1033–1044 (2013).

    Article  CAS  PubMed  Google Scholar 

  12. Cuadrado, M. J. et al. Vascular endothelial growth factor expression in monocytes from patients with primary antiphospholipid syndrome. J. Thromb. Haemost. 4, 2461–2469 (2006).

    Article  CAS  PubMed  Google Scholar 

  13. Sciascia, S. et al. GAPSS: the global anti-phospholipid syndrome score. Rheumatology (Oxford) 52, 1397–1403 (2013).

    Article  Google Scholar 

  14. Lie, J. T. Vasculitis in the antiphospholipid syndrome: culprit or consort? J. Rheumatol. 21, 397–399 (1994).

    CAS  PubMed  Google Scholar 

  15. Daugas, E. et al. Antiphospholipid syndrome nephropathy in systemic lupus erythematosus. J. Am. Soc. Nephrol. 13, 42–52 (2002).

    PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  17. Amigo, M. C., Garcia-Torres, R., Robles, M., Bochicchio, T. & Reyes, P. A. Renal involvement in primary antiphospholipid syndrome. J. Rheumatol. 19, 1181–1185 (1992).

    CAS  PubMed  Google Scholar 

  18. Tektonidou, M. G., Sotsiou, F., Nakopoulou, L., Vlachoyiannopoulos, P. G. & Moutsopoulos, H. M. Antiphospholipid syndrome nephropathy in patients with systemic lupus erythematosus and antiphospholipid antibodies: prevalence, clinical associations, and long-term outcome. Arthritis Rheum. 50, 2569–2579 (2004).

    Article  PubMed  Google Scholar 

  19. Nochy, D. et al. The intrarenal vascular lesions associated with primary antiphospholipid syndrome. J. Am. Soc. Nephrol. 10, 507–518 (1999).

    CAS  PubMed  Google Scholar 

  20. Griffiths, M. H., Papadaki, L. & Neild, G. H. The renal pathology of primary antiphospholipid syndrome: a distinctive form of endothelial injury. Q. J. Med. 93, 457–467 (2000).

    Article  CAS  Google Scholar 

  21. Alchi, B., Griffiths, M. & Jayne, D. What nephrologists need to know about antiphospholipid syndrome. Nephrol. Dial. Transplant. 25, 3147–3154 (2010).

    Article  PubMed  Google Scholar 

  22. Barbour, T., Johnson, S., Cohney, S. & Hughes, P. Thrombotic microangiopathy and associated renal disorders. Nephrol. Dial. Transplant. 27, 2673–2685 (2012).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Sciascia, S., Lopez-Pedrera, C., Roccatello, D. & Cuadrado, M. J. Catastrophic antiphospholipid syndrome (CAPS). Best Pract. Res. Clin. Rheumatol. 26, 535–541 (2012).

    Article  CAS  PubMed  Google Scholar 

  24. Fakhouri, F. et al. The expanding spectrum of renal diseases associated with antiphospholipid syndrome. Am. J. Kidney Dis. 41, 1205–1211 (2003).

    Article  PubMed  Google Scholar 

  25. Sinico, R. A. et al. Renal involvement in primary antiphospholipid syndrome: retrospective analysis of 160 patients. Clin. J. Am. Soc. Nephrol. 5, 1211–1217 (2010).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Zea Mendoza, A. et al. Antiphospholipid antibodies in systemic lupus erythematosus: incidence, significance and relation to lupus nephritis [Spanish]. Med. Clin. (Barc.) 92, 724–728 (1989).

    CAS  Google Scholar 

  27. Moroni, G. et al. Antiphospholipid antibodies are associated with an increased risk for chronic renal insufficiency in patients with lupus nephritis. Am. J. Kidney Dis. 43, 28–36 (2004).

    Article  CAS  PubMed  Google Scholar 

  28. Fofi, C. et al. Lack of association between antiphospholipid antibody and WHO classification in lupus nephritis. Clin. Exp. Rheumatol. 19, 75–77 (2001).

    CAS  PubMed  Google Scholar 

  29. Ostuni, P. A. et al. Renal-artery thrombosis and hypertension in a 13-year-old girl with antiphospholipid syndrome. Ann. Rheum. Dis. 49, 184–187 (1990).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Asherson, R. A., Noble, G. E. & Hughes, G. R. Hypertension, renal artery stenosis and the “primary” antiphospholipid syndrome. J. Rheumatol. 18, 1413–1415 (1991).

    CAS  PubMed  Google Scholar 

  31. Ames, P. R. et al. Bilateral renal artery occlusion in a patient with primary antiphospholipid antibody syndrome: thrombosis, vasculitis or both? J. Rheumatol. 19, 1802–1806 (1992).

    CAS  PubMed  Google Scholar 

  32. Sangle, S. R. et al. Renal artery stenosis in the antiphospholipid (Hughes) syndrome and hypertension. Ann. Rheum. Dis. 62, 999–1002 (2003).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Tektonidou, M. G. Renal involvement in the antiphospholipid syndrome (APS)-APS nephropathy. Clin. Rev. Allergy Immunol. 36, 131–140 (2009).

    Article  CAS  PubMed  Google Scholar 

  34. Voiculescu, A. et al. Noninvasive investigation for renal artery stenosis: contrast-enhanced magnetic resonance angiography and color Doppler sonography as compared to digital subtraction angiography. Clin. Exp. Hypertens. 23, 521–531 (2001).

    Article  CAS  PubMed  Google Scholar 

  35. Rountas, C. Imaging modalities for renal artery stenosis in suspected renovasuclar hypertension: prospective intraindividual comparison of color Doppler, US, CT angiography, GD-enhanced MR angiography, and digital substraction angiography. Ren. Fail. 29, 295–302 (2007).

    Article  CAS  PubMed  Google Scholar 

  36. Chaturvedi, S., Brandao, L., Geary, D. & Licht, C. Primary antiphospholipid syndrome presenting as renal vein thrombosis and membranous nephropathy. Pediatr. Nephrol. 26, 979–985 (2011).

    Article  PubMed  Google Scholar 

  37. Glueck, H. I. et al. Thrombosis in systemic lupus erythematosus. Relation to the presence of circulating anticoagulants. Arch. Intern. Med. 145, 1389–1395 (1985).

    Article  CAS  PubMed  Google Scholar 

  38. Asherson, R. A., Khamashta, M. A. & Hughes, G. R. Hypertension and the antiphospholipid antibodies. Clin. Exp. Rheumatol. 11, 465–467 (1993).

    CAS  PubMed  Google Scholar 

  39. Moss, K. E. & Isenberg, D. A. Comparison of renal disease severity and outcome in patients with primary antiphospholipid syndrome, antiphospholipid syndrome secondary to systemic lupus erythematosus (SLE) and SLE alone. Rheumatology (Oxford) 40, 863–867 (2001).

    Article  CAS  Google Scholar 

  40. Oliveira, C. S. et al. Renal transplantation in lupus nephritis: a Brazilian cohort. Lupus 21, 570–574 (2012).

    Article  CAS  PubMed  Google Scholar 

  41. Padilla-Fernandez, B. et al. Bilateral renal infarction in a lupus patient: an unusual pathology. Clin. Med. Insights Case Rep. 6, 87–91 (2013).

    Article  PubMed  PubMed Central  Google Scholar 

  42. Scully, P., Leckstroem, D. C., McGrath, A., Chambers, J. & Goldsmith, D. J. Repeated renal infarction in native and transplanted kidneys due to left ventricular thrombus formation caused by antiphospholipid antibody syndrome. Int. Med. Case Rep. J. 6, 7–12 (2013).

    PubMed  PubMed Central  Google Scholar 

  43. Poux, J. M. et al. Renal infarction and thrombosis of the infrarenal aorta in a 35-year-old man with primary antiphospholipid syndrome. Am. J. Kidney Dis. 27, 721–725 (1996).

    Article  CAS  PubMed  Google Scholar 

  44. Zenone, T. & Knefati, Y. Renal infarction in systemic lupus with antiphospholipid syndrome: role of hydroxychloroquine withdrawal? [French] Rev. Med. Interne 32, 261–262 (2011).

    Article  CAS  PubMed  Google Scholar 

  45. Frostegard, J., Wu, R., Gillis-Haegerstrand, C., Lemne, C. & de Faire, U. Antibodies to endothelial cells in borderline hypertension. Circulation 98, 1092–1098 (1998).

    Article  CAS  PubMed  Google Scholar 

  46. Rollino, C. et al. Antiphospholipid antibodies and hypertension. Lupus 13, 769–772 (2004).

    Article  CAS  PubMed  Google Scholar 

  47. Ribera, L., Vallve, M. & Almirall, J. Primary antiphospholipid syndrome associated with malignant hypertension [Spanish]. Nefrologia 24 (Suppl. 3), 93–96 (2004).

    PubMed  Google Scholar 

  48. Sirvent, A. E. et al. Malignant hypertension and antiphospholipid syndrome. Nephron 73, 368–369 (1996).

    Article  CAS  PubMed  Google Scholar 

  49. Cacoub, P. et al. Malignant hypertension in antiphospholipid syndrome without overt lupus nephritis. Clin. Exp. Rheumatol. 11, 479–485 (1993).

    CAS  PubMed  Google Scholar 

  50. Ruffatti, A. et al. Risk factors for a first thrombotic event in antiphospholipid antibody carriers: a prospective multicentre follow-up study. Ann. Rheum. Dis. 70, 1083–1086 (2011).

    Article  PubMed  Google Scholar 

  51. Pérez Velásquez, C., Isenberg, D. & Croca, S. Secondary antiphospholipid syndrome nephropathy and lupus nephritis: a case-control study [abstract THU0308]. Ann. Rheum. Dis. 72 (Suppl. 3), 270 (2013).

    Google Scholar 

  52. Tsuruta, Y., Uchida, K., Itabashi, M., Yumura, W. & Nitta, K. Antiphospholipid antibodies and renal outcomes in patients with lupus nephritis. Intern. Med. 48, 1875–1880 (2009).

    Article  PubMed  Google Scholar 

  53. Mehrani, T. & Petri, M. IgM anti-β2 glycoprotein I is protective against lupus nephritis and renal damage in systemic lupus erythematosus. J. Rheumatol. 38, 450–453 (2011).

    Article  PubMed  Google Scholar 

  54. Kincaid-Smith, P., Fairley, K. F. & Kloss, M. Lupus anticoagulant associated with renal thrombotic microangiopathy and pregnancy-related renal failure. Q. J. Med. 68, 795–815 (1988).

    CAS  PubMed  Google Scholar 

  55. Menon, R. N. & Bichile. L. S. Study of renal histopathological correlation with anticardiolipin antibody status in patients with systemic lupus erythematosus. Indian J. Nephrol. 17, 53–60 (2007).

    Article  Google Scholar 

  56. Bhandari, S., Harnden, P., Brownjohn, A. M. & Turney, J. H. Association of anticardiolipin antibodies with intraglomerular thrombi and renal dysfunction in lupus nephritis. Q. J. Med. 91, 401–409 (1998).

    Article  CAS  Google Scholar 

  57. Khamashta, M. A. et al. The management of thrombosis in the antiphospholipid-antibody syndrome. N. Engl. J. Med. 332, 993–997 (1995).

    Article  CAS  PubMed  Google Scholar 

  58. Jordan, N. P. et al. Association of thrombotic microangiopathy and intimal hyperplasia with bleeding post renal biopsy in antiphospholipid antibody positive patients. Arthritis Care Res. (Hoboken) http://dx.doi.org/10.1002/acr.22200 (2013).

  59. Dayal, N. A. & Isenberg, D. A. Endstage renal failure in primary antiphospholipid syndrome—case report and review of literature. Rheumatology (Oxford) 42, 1128–1129 (2003).

    Article  CAS  Google Scholar 

  60. Vaidya, S. Ten-yr renal allograft survival of patients with antiphospholipid antibody syndrome. Clin. Transplant. 26, 853–856 (2012).

    Article  PubMed  Google Scholar 

  61. Amigo, M. C. Kidney disease in antiphospholipid syndrome. Rheum. Dis. Clin. North Am. 32, 509–522 (2006).

    Article  PubMed  Google Scholar 

  62. Prieto, L. N. & Suki, W. N. Frequent hemodialysis graft thrombosis: association with antiphospholipid antibodies. Am. J. Kidney Dis. 23, 587–590 (1994).

    Article  CAS  PubMed  Google Scholar 

  63. Garciamartin, F. et al. Anticardiolipin antibodies and lupus anticoagulant in end-stage renal-disease. Nephrol. Dial. Transplant. 6, 543–547 (1991).

    Article  CAS  Google Scholar 

  64. Fastenau, D. R., Wagenknecht, D. R. & McIntyre, J. A. Increased incidence of antiphospholipid antibodies in left ventricular assist system recipients. Ann. Thorac. Surg. 68, 137–142 (1999).

    Article  CAS  PubMed  Google Scholar 

  65. Sitter, T. & Schiffl, H. Anticardiolipin antibodies in patients on regular hemodialysis: an epiphenomenon? Nephron 64, 655–656 (1993).

    Article  CAS  PubMed  Google Scholar 

  66. Vaidya, S. Management of end-stage renal disease patients with antiphospholipid antibody syndrome. Transplant. Proc. 37, 650–651 (2005).

    Article  CAS  PubMed  Google Scholar 

  67. Matsuda, J. et al. β-2-glycoprotein I-dependent and I-independent anticardiolipin antibody in patients with end-stage renal-disease. Thromb. Res. 72, 109–117 (1993).

    Article  CAS  PubMed  Google Scholar 

  68. Ferlazzo, B. et al. Anticardiolipin antibodies in hemodialysis patients and in renal transplant recipients: prevalence and significance [Italian]. Recenti Prog. Med. 89, 434–437 (1998).

    CAS  PubMed  Google Scholar 

  69. Sitter, T., Spannagl, M. & Schiffl, H. Anticardiolipin antibodies and lupus anticoagulant in patients treated with different methods of renal replacement therapy in comparison to patients with systemic lupus erythematosus. Ann. Hematol. 65, 79–82 (1992).

    Article  CAS  PubMed  Google Scholar 

  70. Phillips, A. O., Jones, H. W., Hambley, H., Hillis, A. N. & Hendry, B. M. Prevalence of lupus anticoagulant and anticardiolipin antibodies in haemodialysis patients. Nephron 65, 350–353 (1993).

    Article  CAS  PubMed  Google Scholar 

  71. Ozmen, S., Danis, R., Akin, D. & Batun, S. Anticardiolipin antibodies in hemodialysis patients with hepatitis C and their role in fistula failure. Clin. Nephrol. 72, 193–198 (2009).

    CAS  PubMed  Google Scholar 

  72. Gultekin, F. et al. The relationship between anticardiolipin antibodies and vascular access occlusion in patients on hemodialysis. ASAIO J. 51, 162–164 (2005).

    Article  CAS  PubMed  Google Scholar 

  73. Jamshid, R., Reza, S. A., Abbas, G. & Raha, A. Incidence of arteriovenous thrombosis and the role of anticardiolipin antibodies in hemodialysis patients. Int. Urol. Nephrol. 35, 275–282 (2003).

    Article  CAS  PubMed  Google Scholar 

  74. Adler, S., Szczech, L., Qureshi, A., Bollu, R. & Thomas-John, R. IgM anticardiolipin antibodies are associated with stenosis of vascular access in hemodialysis patients but do not predict thrombosis. Clin. Nephrol. 56, 428–434 (2001).

    CAS  PubMed  Google Scholar 

  75. Haviv, Y. S. Association of anticardiolipin antibodies with vascular access occlusion in hemodialysis patients: cause or effect? Nephron 86, 447–454 (2000).

    Article  CAS  PubMed  Google Scholar 

  76. Prakash, R., Miller, C. C. 3rd & Suki, W. N. Anticardiolipin antibody in patients on maintenance hemodialysis and its association with recurrent arteriovenous graft thrombosis. Am. J. Kidney Dis. 26, 347–352 (1995).

    Article  CAS  PubMed  Google Scholar 

  77. Chew, S. L. et al. Are antiphospholipid antibodies clinically relevant in dialysis patients? Nephrol. Dial. Transplant. 7, 1194–1198 (1992).

    Article  CAS  PubMed  Google Scholar 

  78. Erkan, D., Yazici, Y., Sobel, R. & Lockshin, M. D. Primary antiphospholipid syndrome: functional outcome after 10 years. J. Rheumatol. 27, 2817–2821 (2000).

    CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  80. Serrano, A. et al. IgA antibodies against β2 glycoprotein I in hemodialysis patients are an independent risk factor for mortality. Kidney Int. 81, 1239–1244 (2012).

    Article  CAS  PubMed  Google Scholar 

  81. Meijide, H., Sciascia, S., Sanna, G., Khamashta, M. A. & Bertolaccini, M. L. The clinical relevance of IgA anticardiolipin and IgA anti-β2 glycoprotein I antiphospholipid antibodies: a systematic review. Autoimmun. Rev. 12, 421–425 (2013).

    Article  CAS  PubMed  Google Scholar 

  82. Stone, J. H., Amend, W. J. C. & Criswell, L. A. Antiphospholipid antibody syndrome in renal transplantation: occurrence of clinical events in96 consecutive patients with systemic lupus erythematosus. Am. J. Kidney Dis. 34, 1040–1047 (1999).

    Article  CAS  PubMed  Google Scholar 

  83. Wagenknecht, D. R. et al. Antiphospholipid antibodies are a risk factor for early renal allograft failure: isolation of antiphospholipid antibodies from a thrombosed renal allograft. Transplant. Proc. 31, 285–288 (1999).

    Article  CAS  PubMed  Google Scholar 

  84. Wagenknecht, D. R., Becker, D. G., LeFor, W. M. & McIntyre, J. A. Antiphospholipid antibodies are a risk factor for early renal allograft failure. Transplantation 68, 241–246 (1999).

    Article  CAS  PubMed  Google Scholar 

  85. McIntyre, J. A. & Wagenknecht, D. R. Antiphospholipid antibodies and renal transplantation: a risk assessment. Lupus 12, 555–559 (2003).

    Article  CAS  PubMed  Google Scholar 

  86. Ducloux, D. et al. Prevalence and clinical significance of antiphospholipid antibodies in renal transplant recipients. Transplantation 67, 90–93 (1999).

    Article  CAS  PubMed  Google Scholar 

  87. Amigo, M. C. & Garcia-Torres, R. Morphology of vascular, renal, and heart lesions in the antiphospholipid syndrome: relationship to pathogenesis. Curr. Rheumatol. Rep. 2, 262–270 (2000).

    Article  CAS  PubMed  Google Scholar 

  88. Canaud, G. et al. Severe vascular lesions and poor functional outcome in kidney transplant recipients with lupus anticoagulant antibodies. Am. J. Transplant. 10, 2051–2060 (2010).

    Article  CAS  PubMed  Google Scholar 

  89. Fernández-Fresnedo, G. et al. Clinical significance of antiphospholipid antibodies on allograft and patient outcome after kidney transplantation. Transplant. Proc. 37, 3710–3711 (2005).

    Article  CAS  PubMed  Google Scholar 

  90. Smyth, A. et al. A systematic review and meta-analysis of pregnancy outcomes in patients with systemic lupus erythematosus and lupus nephritis. Clin. J. Am. Soc. Nephrol. 5, 2060–2068 (2010).

    Article  PubMed  PubMed Central  Google Scholar 

  91. Isom, R., Nickolas, T. L. & Radhakrishnan, J. Nephrological and obstetric complications of the antiphospholipid syndrome. Expert Opin. Investig. Drugs 11, 819–829 (2002).

    Article  CAS  PubMed  Google Scholar 

  92. Vellanki, V. S. et al. Post-partum bilateral renal cortical necrosis in antiphospholipid syndrome and systemic lupus erythematosus. Saudi J. Kidney Dis. Transpl. 24, 549–552 (2013).

    Article  PubMed  Google Scholar 

  93. Ateka-Barrutia, O. & Khamashta, M. A. The challenge of pregnancy for patients with SLE. Lupus 22, 1295–1308 (2013).

    Article  CAS  PubMed  Google Scholar 

  94. National Institute for Health and Clinical Excellence. CG107 Hypertension in pregnancy: the management of hypertensive disorders during pregnancy [online], (2014).

  95. Rodríguez-Pintó, I., Espinosa, G. & Cervera, R. Catastrophic antiphospholipid syndrome—20 years later. Curr. Rheumatol. Rev. 9, 73–80 (2013).

    Article  PubMed  Google Scholar 

  96. Cervera, R. CAPS Registry. Lupus 21, 755–757 (2012).

    Article  CAS  PubMed  Google Scholar 

  97. Cervera, R. et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J. Autoimmun. 32, 240–245 (2009).

    Article  CAS  PubMed  Google Scholar 

  98. Cervera, R., Espinosa, G., Bucciarelli, S., Gomez-Puerta, J. A. & Font, J. Lessons from the catastrophic antiphospholipid syndrome (CAPS) registry. Autoimmun. Rev. 6, 81–84 (2006).

    Article  PubMed  Google Scholar 

  99. Tektonidou, M. G., Sotsiou, F. & Moutsopoulos, H. M. Antiphospholipid syndrome (APS) nephropathy in catastrophic, primary, and systemic lupus erythematosus-related APS. J. Rheumatol. 35, 1983–1988 (2008).

    CAS  PubMed  Google Scholar 

  100. Ruiz-Irastorza, G. et al. Evidence-based recommendations for the prevention and long-term management of thrombosis in antiphospholipid antibody-positive patients: report of a task force at the 13th International Congress on antiphospholipid antibodies. Lupus 20, 206–218 (2011).

    Article  CAS  PubMed  Google Scholar 

  101. Erkan, D. et al. 14th International Congress on Antiphospholipid Antibodies Task Force report on antiphospholipid syndrome treatment trends. Autoimmun. Rev. http://dx.doi.org/10.1016/j.autrev.2014.01.053 (2014).

  102. Pengo, V. et al. Incidence of a first thromboembolic event in asymptomatic carriers of high-risk antiphospholipid antibody profile: a multicenter prospective study. Blood 118, 4714–4718 (2011).

    Article  CAS  PubMed  Google Scholar 

  103. Sciascia, S. et al. Clinical accuracy for diagnosis of antiphospholipid syndrome in systemic lupus erythematosus: evaluation of 23 possible combinations of antiphospholipid antibody specificities. J. Thromb. Haemost. 10, 2512–2518 (2012).

    Article  CAS  PubMed  Google Scholar 

  104. Bertero, M. T. Primary prevention in antiphospholipid antibody carriers. Lupus 21, 751–754 (2012).

    Article  CAS  PubMed  Google Scholar 

  105. Ruiz-Irastorza, G., Ramos-Casals, M., Brito-Zeron, P. & Khamashta, M. A. Clinical efficacy and side effects of antimalarials in systemic lupus erythematosus: a systematic review. Ann. Rheum. Dis. 69, 20–28 (2010).

    Article  CAS  PubMed  Google Scholar 

  106. Scoble, T., Wijetilleka, S. & Khamashta, M. A. Management of refractory anti-phospholipid syndrome. Autoimmun. Rev. 10, 669–673 (2011).

    Article  PubMed  Google Scholar 

  107. Ruiz-Irastorza, G., Crowther, M., Branch, W. & Khamashta, M. A. Antiphospholipid syndrome. Lancet 376, 1498–1509 (2010).

    Article  CAS  PubMed  Google Scholar 

  108. Remondino, G. I. et al. A reversible bilateral renal artery stenosis in association with antiphospholipid syndrome. Lupus 9, 65–67 (2000).

    Article  CAS  PubMed  Google Scholar 

  109. Sangle, S. R., D'Cruz, D. P., Abbs, I. C., Khamashta, M. A. & Hughes, G. R. Renal artery stenosis in hypertensive patients with antiphospholipid (Hughes) syndrome: outcome following anticoagulation. Rheumatology (Oxford) 44, 372–377 (2005).

    Article  CAS  Google Scholar 

  110. Broder, A. & Putterman, C. Hydroxychloroquine use is associated with lower odds of persistently positive antiphospholipid antibodies and/or lupus anticoagulant in systemic lupus erythematosus. J. Rheumatol. 40, 30–33 (2013).

    Article  CAS  PubMed  Google Scholar 

  111. Sciascia, S., Naretto, C., Rossi, D., Bazzan, M. & Roccatello, D. Treatment-induced downregulation of antiphospholipid antibodies: effect of rituximab alone on clinical and laboratory features of antiphospholipid syndrome. Lupus 20, 1106–1108 (2011).

    Article  CAS  PubMed  Google Scholar 

  112. Korkmaz, C., Kabukcuoglu, S., Isiksoy, S. & Yalcin, A. U. Renal involvement in primary antiphospholipid syndrome and its response to immunosuppressive therapy. Lupus 12, 760–765 (2003).

    Article  CAS  PubMed  Google Scholar 

  113. Sokunbi, D. O. B., Miller, F., Wadhwa, N. K. & Nord, E. P. Reversible renal-failure in the primary antiphospholipid syndrome—a report of 2 cases. J. Am. Soc. Nephrol. 4, 28–35 (1993).

    CAS  PubMed  Google Scholar 

  114. Sciascia, S., Giachino, O. & Roccatello, D. Prevention of thrombosis relapse in antiphospholipid syndrome patients refractory to conventional therapy using intravenous immunoglobulin. Clin. Exp. Rheumatol. 30, 409–413 (2012).

    CAS  PubMed  Google Scholar 

  115. Arachchillage, D. J. & Cohen, H. Use of new oral anticoagulants in antiphospholipid syndrome. Curr. Rheumatol. Rep. 15, 331 (2013).

    Article  CAS  PubMed  Google Scholar 

  116. Giles, I., Khamashta, M., D'Cruz, D. & Cohen, H. A new dawn of anticoagulation for patients with antiphospholipid syndrome? Lupus 21, 1263–1265 (2012).

    Article  PubMed  Google Scholar 

  117. Erkan, D., Vega, J., Ramon, G., Kozora, E. & Lockshin, M. D. A pilot open-label phase II trial of rituximab for non-criteria manifestations of antiphospholipid syndrome. Arthritis Rheum. 65, 464–471 (2013).

    Article  CAS  PubMed  Google Scholar 

  118. Erkan, D. & Lockshin, M. D. New approaches for managing antiphospholipid syndrome. Nat. Clin. Pract. Rheumatol. 5, 160–170 (2009).

    PubMed  Google Scholar 

  119. Velik-Salchner, C., Lederer, W. & Wiedermann, F. Eculizumab and renal transplantation in a patient with catastrophic antiphospholipid syndrome: effect of heparin on complement activation. Lupus 20, 772 (2011).

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Centro di Ricerche di Immunopatologia e Documentazione su Malattie Rare and Università di Torino, Piazza del Donatore di Sangue 3, Turin, 10154, Italy

    Savino Sciascia & Dario Roccatello

  2. Louise Coote Lupus Unit, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, King's College London, Westminster Bridge Road, London, SE1 7EH, UK

    Maria José Cuadrado

  3. Division of Women's Health, Graham Hughes Lupus Research Laboratory, The Rayne Institute, King's College London, Westminster Bridge Road, London, SE1 7EH, UK

    Munther Khamashta

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  1. Savino Sciascia
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  2. Maria José Cuadrado
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  3. Munther Khamashta
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  4. Dario Roccatello
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Contributions

S.S. and D.R. researched data for the article. S.S. and M.J.C. wrote the article and made substantial contribution to discussion of the content. M.K. made substantial contribution to discussion of the content. S.S., M.K. and D.R. reviewed and edited the manuscript before submission.

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Correspondence to Savino Sciascia.

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The authors declare no competing financial interests.

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Sciascia, S., Cuadrado, M., Khamashta, M. et al. Renal involvement in antiphospholipid syndrome. Nat Rev Nephrol 10, 279–289 (2014). https://doi.org/10.1038/nrneph.2014.38

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