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Diagnosis: Antiphospholipid Syndrome Chronic Nephropathy

Antiphospholipid syndrome (APS) is defined by the presence of antiphospholipid antibodies, recognized as anticardiolipin antibodies and/or anti-Beta2 glycoprotein I and/or lupus anticoagulant, associated with thrombotic events (venous or arterial) and/or fetal loss. Although APS was first described in patients with systemic lupus erythematosus (SLE), more than 50% of patients with APS do not have clinical or laboratory evidence of another autoimmune disease and are classified as having primary antiphospholipid syndrome (PAPS) (Sinico RA, et al. Renal involvement in primary antiphospholipid syndrome: retrospective analysis of 160 patients. Clin J Am Soc Nephrol. 2010;5(7):1211-7. [PubMed link] [Free full text]).

A large spectrum of renal thrombotic manifestations have been described in association with antiphospholipid antibodies, such as renal artery stenosis, renal infarction, renal vein thrombosis, acute or chronic thrombotic microangiopathy, and, more recently, the so-called “antiphospholipid antibodies nephropathy”.

There are two forms of vascular nephropathy: (1) An acute form clinically resembling other thrombotic microangiopathy, such as hemolytic uremic syndrome or thrombotic thrombocytopenic purpura, and (2) a chronic form of renal involvement, often clinically silent, consisting of the development of a vaso-occlusive process at all levels of the renal vasculature, with glomerular and tubuloiterstitial ischemic changes, as in the presented case.

See the Chapter Vascular diseases of our Tutorial (this chapter has only Sapnish version).

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References

• Sinico RA, Cavazzana I, Nuzzo M, Vianelli M, Napodano P, Scaini P, Tincani A. Renal involvement in primary antiphospholipid syndrome: retrospective analysis of 160 patients. Clin J Am Soc Nephrol. 2010;5(7):1211-7. [PubMed link] [Free full text]
• Gigante A, Gasperini ML, Cianci R, Barbano B, Giannakakis K, Di Donato D, Fuiano G, Amoroso A. Antiphospholipid antibodies and renal involvement. Am J Nephrol. 2009;30(5):405-12. [PubMed link] [Free full text]
• D'Cruz D. Renal manifestations of the antiphospholipid syndrome. Curr Rheumatol Rep. 2009;11(1):52-60. [PubMed link]
• Tektonidou MG. Renal involvement in the antiphospholipid syndrome (APS)-APS nephropathy. Clin Rev Allergy Immunol. 2009;36(2-3):131-40. [PubMed link]
• Amigo MC. Kidney disease in antiphospholipid syndrome. Rheum Dis Clin North Am. 2006;32(3):509-22. [PubMed link]
• Joseph RE, Radhakrishnan J, Appel GB. Antiphospholipid antibody syndrome and renal disease. Curr Opin Nephrol Hypertens. 2001;10(2):175-81. [PubMed link]
• Nzerue CM, Hewan-Lowe K, Pierangeli S, Harris EN. "Black swan in the kidney": renal involvement in the antiphospholipid antibody syndrome. Kidney Int. 2002;62(3):733-44. [PubMed link] [Free full text]
• Amigo MC, García-Torres R. Morphology of vascular, renal, and heart lesions in the antiphospholipid syndrome: relationship to pathogenesis. Curr Rheumatol Rep. 2000;2(3):262-70. [PubMed link]
• Griffiths MH, Papadaki L, Neild GH. The renal pathology of primary antiphospholipid syndrome: a distinctive form of endothelial injury. QJM. 2000;93(7):457-67. [PubMed link] [Free full text]

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