title: Pure lupus podocytopathy first presenting as thrombotic thrombocytopenic purpura-like syndrome.
abstract: Lupus podocytopathy (LP) is an uncommon proteinuric disorder in the spectrum of lupus nephropathy. Its histological features are similar to those described in minimal change disease (MCD) with or without mesangial immune deposits. Although infrequent, a close relationship between systemic lupus erythematosus (SLE) and thrombotic thrombocytopenic purpura (TTP) is well accepted. Proteinuria in the setting of SLE has previously been associated with the development of TTP-like syndrome. As far as we know, LP first presenting as a TTP-like syndrome has never been reported. Here, we describe the case of a previously healthy 45-year-old woman who developed simultaneously these two conditions and then we briefly review the literature on the topic, emphasizing the previous cases of concurrent initial diagnosis of both SLE and MCD (n = 7) and SLE and TTP (n = 72). In conclusion, renal biopsy is central to the management of SLE patients with nephrotic syndrome. Furthermore, in a SLE patient with anemia and thrombocytopenia, TTP should be part of the differential diagnosis, even when no schistocytes were detected in peripheral blood smear.
authors.affiliation: Department of Internal Medicine, University Hospital, Autonomous University of Nuevo León, Madero y Gonzalitos s/n, Col. Mitras Centro, C.P. 64460, Monterrey, Nuevo León, Mexico. grdelgadog@gmail.com.
authors.forename: Guillermo
authors.initials: G
authors.lastname: Delgado-García
authors.affiliation: Division of Neurology, University Hospital, Autonomous University of Nuevo León, Monterrey, Nuevo León, Mexico.
authors.forename: Carlos
authors.initials: C
authors.lastname: Cámara-Lemarroy
authors.affiliation: Division of Neurology, University Hospital, Autonomous University of Nuevo León, Monterrey, Nuevo León, Mexico.
authors.forename: Adrián
authors.initials: A
authors.lastname: Infante-Valenzuela
authors.affiliation: Division of Hematology, University Hospital, Autonomous University of Nuevo León, Monterrey, Nuevo León, Mexico.
authors.forename: Perla
authors.initials: P
authors.lastname: Colunga-Pedraza
authors.affiliation: Service of Anatomic Pathology, University Hospital, Autonomous University of Nuevo León, Monterrey, Nuevo León, Mexico.
authors.forename: Gabriela
authors.initials: G
authors.lastname: Alarcón-Galván
authors.affiliation: Division of Rheumatology, University Hospital, Autonomous University of Nuevo León, Monterrey, Nuevo León, Mexico.
authors.forename: Arian
authors.initials: A
authors.lastname: Armenta-González
authors.affiliation: Division of Neurology, University Hospital, Autonomous University of Nuevo León, Monterrey, Nuevo León, Mexico.
authors.forename: Héctor Jorge
authors.initials: HJ
authors.lastname: Villarreal-Velázquez
mesh_terms: D003520:Cyclophosphamide / Q000008:administration & dosage / Q000009:adverse effects; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology*; D007970:Leukopenia / Q000139:chemically induced; D008180:Lupus Erythematosus, Systemic / Q000188:drug therapy / Q000473:pathology*; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000188:drug therapy*; D011697:Purpura, Thrombotic Thrombocytopenic
chemical_list: D003520:Cyclophosphamide; D008775:Methylprednisolone
keywords: Minimal change disease; NSAIDs; Podocytopathy; Systemic lupus erythematosus; Thrombotic microangiopathy; Thrombotic thrombopenic purpura