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Task Force Report on Non-criteria Manifestations: Nephropathy

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Antiphospholipid Syndrome

Abstract

A small-vessel vaso-occlusive nephropathy defined as antiphospholipid syndrome-associated nephropathy (APS nephropathy) has been recently recognized. APS nephropathy was first described in primary APS patients, characterized by acute thrombotic lesions in glomeruli and/or arterioles (thrombotic microangiopathy) and chronic vascular lesions, such as fibrous intimal hyperplasia of arterioles and interlobular arteries, organized thrombi with or without recanalization, and fibrous arterial and arteriolar occlusions or focal cortical atrophy. Similar lesions were further detected in systemic lupus erythematosus (SLE)-related APS and SLE/non-APS patients with positive antiphospholipid antibodies (aPLs), independently of lupus nephritis. Hypertension is the predominant clinical manifestation of APS nephropathy, followed by hematuria, proteinuria (from mild-to-nephrotic range), and renal insufficiency. Arterial thromboses (especially stroke), pulmonary embolism, livedo reticularis, anticardiolipin antibodies, and a positive lupus anticoagulant test are strongly associated with histological lesions of APS nephropathy. According to the report of a task force group on non-criteria APS manifestations held in the context of the 13th International Congress on aPL, APS nephropathy occurs almost exclusively among patients with positive aPL (with or without APS) in comparison with those without aPL and it is more frequent in APS patients (primary or SLE related) than in SLE/aPL/non-APS patients. aPLs correlate with both acute and chronic lesions of APS nephropathy. Currently, there is no consensus on the management of APS nephropathy that it may occur or improve despite full-dose anticoagulation. Multicenter studies are needed to examine the full spectrum of clinical and histological characteristics, renal outcome, and treatment approach of this nephropathy.

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Acknowledgments

The members of the “Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations” that contributed to the discussions that are presented in this chapter are as follows: Ricard Cervera and Gerard Espinosa (Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Catalonia, Spain), Maria G. Tektonidou (First Department of Internal Medicine, Medical School, National University of Athens, Athens, Greece), Antonio R. Cabral (Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico), Emilio B. González (Division of Rheumatology, Department of Medicine, The University of Texas Medical Branch, Galveston, Texas, USA), Doruk Erkan (The Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Medical College of Cornell University, New York, NY, USA), Smita Vadya (Department of Pathology, University of Texas Medical Branch, Galveston, Texas, USA), Horacio E. Adrogué (The Methodist Hospital Transplant Center, Fannin, Houston, Texas, USA), Michal Solomon (Department of Dermatology, Chaim Sheba Medical Center, Tel Hashomer, Israel), Gisele Zandman-Goddard (Department of Medicine C, Wolfson Medical Center, Tel Hashomer, Israel), and Yehuda Shoenfeld (Zublodovitz Center for Autoimmune Diseases and Department of Medicine B, Chaim Sheba Medical Center, Tel Hashomer, Israel).

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Authors and Affiliations

  1. First Department of Internal Medicine, University of Athens Medical School, 19 Kamariotou Street, Nea Filothei, Athens, 11524, Greece

    Maria G. Tektonidou MD, PhD

  2. Department of Internal Medicine, The Methodist Hospital, Houston, TX, USA

    Horacio E. Adrogué MD

  3. Department of Pathology, University of Texas Medical Branch, Galveston, TX, USA

    Smita Vaidya PhD

Authors
  1. Maria G. Tektonidou MD, PhD
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  2. Horacio E. Adrogué MD
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  3. Smita Vaidya PhD
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Correspondence to Maria G. Tektonidou MD, PhD .

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Editors and Affiliations

  1. The Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, New York, NY, USA

    Doruk Erkan

  2. University of Texas Medical Branch, 301 University Boulevard, Galveston, 77555-0883, Texas, USA

    Silvia S. Pierangeli

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Tektonidou, M.G., Adrogué, H.E., Vaidya, S. (2012). Task Force Report on Non-criteria Manifestations: Nephropathy. In: Erkan, D., Pierangeli, S. (eds) Antiphospholipid Syndrome. Springer, Boston, MA. https://doi.org/10.1007/978-1-4614-3194-7_14

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  • DOI: https://doi.org/10.1007/978-1-4614-3194-7_14

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