Membranous nephropathy in primary antiphospholipid syndrome: Description of a case and induction of renal injury in SCID mice

Article type: Research Article

Authors: Levy, Y.^a | Ziporen, L.^a | Gilburd, B.^a | George, J.^a | Polak-Charcon, S.^b | Amital, H.^a | Cledes, J.^c | Youinou, P.^d | Shoenfeld, Y.^{a; *}

Affiliations: [a] Department of Medicine B and the Research Unit of Autoimmune Disease, Institute of Pathology | [b] Research Unit of Autoimmune Disease, Institute of Pathology | [c] Department of Nephrology Sheba Medical Center, Tel-Hashomer, Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel | [d] Laboratory of Immunology, Brest University Medical School Hospital, F 29609 Brest, Cedex, France

Correspondence: [*] Correspondence and reprint requests to Y. Shoenfeld MD, Dept. Med 'B, Sheba Medical Center, Tel-Hashomer 52621, Israel.

Abstract: Primary antiphospholipid syndrome (PAPS) is a recently recognized clinical entity encompassing the combination of thromboembolic phenomena, thrombocytopenia and recurrent abortions in the presence of antiphopholipid antibodies. We present a patient with PAPS accompanied by renal involvement, manifested as membranous nephropathy, as proven by a renal biopsy. To investigate further the possible association between PAPS and the renal lesion we attempted to induce similar renal manifestations by transferring peripheral blood lymphocytes (PBL) from this patient to severe combined immunodeficiency (SCID) mice. The mice transfused with PBL from the affected patient exemplified antiphospolipid antibodies (aPL) following which a renal lesion consistent with the human membranous nephropathy lesion was precipitated. This study substantiates the role of aPL as possible inducers of renal damage.

Keywords: Antiphospholipid syndrome, nephrotic syndrome, thrombotic microangiopathy, SCID mice, membranous glomerulopathy

DOI: 10.3233/HAB-1996-7301

Journal: Human Antibodies, vol. 7, no. 3, pp. 91-96, 1996