Nephropathy of antiphospholipid antibody syndrome (NAPS) is an increasingly well-recognized aspect of antiphospholipid syndrome. The most characteristic histopathology is that of thrombotic microangiopathy, and thrombosis occurring in the renal vasculature is thought to be the initiating event. Other less common pathologies have been reported, and the mechanisms of these are unclear. Therapy has been largely empiric. We report a case of NAPS in a patient with atypical pathology, who has declined therapy with immunosuppressive agents and anticoagulants and who has maintained normal renal function in 20 years of follow-up.
1 Miyakis S Lockshin MD Atsumi T . International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome. J Thromb Haemost2006; 4: 295–306.
2.
2 Hughson MD McCarty G Brumback RA . Spectrum of vascular pathology affecting patients with the antiphospholipid syndrome. Hum Pathol1995; 26: 716–724.
3.
3 Girardi G Berman J Redecha P . Complement C5a receptors and neutrophils mediate fetal injury in the antiphospholipid syndrome. J Clin Investig2003; 112: 1644–1654.
4.
4 Kincaid-Smith P Fairley KF Kloss M . Lupus anticoagulant associated with renal thrombotic microangiopathy and pregnancy-related renal failure. Q J Med1988; 69: 795–815.
5.
5 Amigo M . Kidney disease in antiphospholipid syndrome. Rheum Dis Clin N Am2006; 32: 509–522.
6.
6 Jennette JC Olson JL Schwartz MM Sliva FG . Renal diseases in pregnancy. In: Heptinstall’s Pathology of the Kidney.Philadelphia: Lippincott Williams & Wilkins; 2007, p. 770–787
7.
7 Sorice M Longo A Capozzi A . Anti-β2-glycoprotein antibodies induce monocyte release of tumor necrosis factor alpha and tissue factor by signal transduction pathways involving lipid rafts. Arthritis Rheum2007; 56: 2687–2697.
8.
8 Hamidou MA Moreau A Jego P . Captopril and aspirin in the treatment of renal microangiopathy in primary antiphospholipid syndrome. Am J Kidney Dis1995; 25: 486–488.
9.
9 Olguin L Calleja C Hernandez C . Primary antiphospholipid syndrome nephropathy despite anticoagulant therapy. Arthritis Rheum2003; 48(Suppl.): S359.
10.
10 Korkmaz C Kabukcuoglu S Isiksoy S Yalcin AU . Renal involvement in primary antiphospholipid syndrome and its response to immunosuppressive therapy. Lupus2003; 12: 760–765.
