title: Antiphospholipid syndrome and kidney disease.
abstract: The antiphospholipid syndrome is a common autoimmune disease caused by pathogenic antiphospholipid antibodies, leading to recurrent thrombosis and/or obstetrical complications. Importantly for nephrologists, antiphospholipid antibodies are associated with various renal manifestations including large renal vessel thrombosis, renal artery stenosis, and a constellation of intrarenal lesions that has been termed antiphospholipid nephropathy. This last condition associates various degrees of acute thrombotic microangiopathy, proliferative and fibrotic lesions of the intrarenal vessels, and ischemic modifications of the renal parenchyma. The course of the disease can range from indolent nephropathy to devastating acute renal failure. The pejorative impact of antiphospholipid antibody-related renal complication is well established in the context of systemic lupus erythematous or after renal transplantation. In contrast, the exact significance of isolated antiphospholipid nephropathy remains uncertain. The evidence to guide management of the renal complications of antiphospholipid syndrome is limited. However, the recent recognition of the heterogeneous molecular mechanisms underlying the progression of intrarenal vascular lesions in antiphospholipid syndrome have opened promising tracks for patient monitoring and targeted therapeutic intervention.
authors.affiliation: Université Paris Descartes, Sorbonne Paris Cité, Hôpital Necker-Enfants Malades, Paris, France; Service d'Explorations Fonctionnelles, Hôpital Necker-Enfants Malades, Paris, France; INSERM U1151, Institut Necker Enfants Malades, Hôpital Necker-Enfants Malades, Paris, France.
authors.forename: Frank
authors.initials: F
authors.lastname: Bienaimé
authors.affiliation: Université Paris Descartes, Sorbonne Paris Cité, Hôpital Necker-Enfants Malades, Paris, France; INSERM U1151, Institut Necker Enfants Malades, Hôpital Necker-Enfants Malades, Paris, France; Service de Néphrologie Transplantation Adultes, Hôpital Necker-Enfants Malades, Paris, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Legendre
authors.affiliation: Université Paris Descartes, Sorbonne Paris Cité, Hôpital Necker-Enfants Malades, Paris, France; INSERM U1151, Institut Necker Enfants Malades, Hôpital Necker-Enfants Malades, Paris, France.
authors.forename: Fabiola
authors.initials: F
authors.lastname: Terzi
authors.affiliation: Université Paris Descartes, Sorbonne Paris Cité, Hôpital Necker-Enfants Malades, Paris, France; INSERM U1151, Institut Necker Enfants Malades, Hôpital Necker-Enfants Malades, Paris, France; Service de Néphrologie Transplantation Adultes, Hôpital Necker-Enfants Malades, Paris, France. Electronic address: guillaume.canaud@inserm.fr.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Canaud
mesh_terms: D064591:Allografts / Q000098:blood supply / Q000276:immunology / Q000473:pathology; D057911:Angiotensin Receptor Antagonists / Q000627:therapeutic use; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000818:Animals; D017152:Antibodies, Antiphospholipid / Q000276:immunology*; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000276:immunology / Q000628:therapy; D018450:Disease Progression; D006801:Humans; D006973:Hypertension / Q000209:etiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000098:blood supply / Q000276:immunology / Q000473:pathology; D007674:Kidney Diseases / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy*; D016030:Kidney Transplantation / Q000009:adverse effects; D008279:Magnetic Resonance Imaging; D010951:Plasma Exchange; D012078:Renal Artery Obstruction / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000276:immunology*; D013927:Thrombosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000453:epidemiology / Q000276:immunology*; D053482:beta 2-Glycoprotein I / Q000276:immunology
chemical_list: D057911:Angiotensin Receptor Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors; D017152:Antibodies, Antiphospholipid; D000925:Anticoagulants; D007166:Immunosuppressive Agents; D053482:beta 2-Glycoprotein I
keywords: antiphospholipid antibodies; mTORC pathway; vasculopathy