Letter to the Editor
Podocytopathy as the onset of systemic lupus erythematosus
Podocitopatía como inicio de un lupus eritematoso sistémico
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Cabezuelo Romero" "autores" => array:5 [ 0 => array:4 [ "nombre" => "Salomé" "apellidos" => "Muray Cases" "email" => array:1 [ 0 => "salomuray@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "María Teresa" "apellidos" => "Herranz Marín" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "Concepción" "apellidos" => "Alcázar Fajardo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "Alberto Javier" "apellidos" => "Andreu Muñoz" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "Juan B." "apellidos" => "Cabezuelo Romero" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Nefrología, Hospital General Universitario Reina Sofía, Murcia, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Medicina Interna, Hospital General Universitario Morales Meseguer, Murcia, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Podocitopatía como inicio de un lupus eritematoso sistémico" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1567 "Ancho" => 2119 "Tamanyo" => 142512 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Evolution of proteinuria and serum albumin with the treatment.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">MMF: mycophenolate mofetil; Pd: prednisone.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Lupus podocytopathy (LP) is not included as a subtype in the classification of lupus nephropathy (LN) of the ISN/RPS 2003.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> Presently the frequency, prognosis and treatment of LP are not well established.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We describe the case of a 34-year-old woman, with no prior medical history of interest, who presented severe thrombocytopenia and nephrotic syndrome, with no other associated symptoms. In the lab work, the following stood out: platelets 36,000/μl, hemoglobin 12.1<span class="elsevierStyleHsp" style=""></span>g/dl, creatinine 0.61<span class="elsevierStyleHsp" style=""></span>mg/dl, cholesterol 258<span class="elsevierStyleHsp" style=""></span>mg/dl, serum albumin 2.7<span class="elsevierStyleHsp" style=""></span>g/dl; haptoglobin and LDH were normal; proteinogram without significant alterations. In the urine, the sediment was normal with proteinuria of 7<span class="elsevierStyleHsp" style=""></span>g/day, Bence Jones proteinuria was negative. Positive ANA at 1/320, anti-DNA IgG: 98<span class="elsevierStyleHsp" style=""></span>IU/ml (normal value <15); anti-La was + and anti-Ro +++. Coombs, C3, C4, antiphospholipid antibodies, ANCA, anti-GBM, cryoglobulins, immunoglobulins, serologies for hepatitis B and C, HIV were normal or negative. The renal biopsy contained 42 glomeruli, 2 of them sclerotic, and in the majority there were no abnormalities at optical microscopy, only and very focal, in some glomeruli, there was minor mesangial proliferation. There were no tubulointerstitial or vascular abnormalities. In the immunofluorescence there were mesangial granular deposits of moderate intensity of IgG and negative for IgA, IgM, C3, C1q, kappa and lambda and fibrinogen. No electronic microscopy was available.</p><p id="par0015" class="elsevierStylePara elsevierViewall">It was suspected the presence of LP and treatment with hydroxychloroquine and prednisone was started at a dose of 1<span class="elsevierStyleHsp" style=""></span>mg/kg per day, which was maintained until 2 weeks after the remission of the nephrotic syndrome (achieved after 2 months of treatment) (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Then, the dose of prednisone was reduced slowly and progressively and mycophenolate mofetil was associated to the treatment. At month 18, the patient was still in complete remission, prednisone was discontinued and mycophenolate mofetil was maintained. Throughout the follow-up, the patient was normotensive, the glomerular filtration rate and complement were normal and the ANA and anti-DNA persisted positive, but at a lower level. Platelets recovered partially, after administration of romiplostim before the renal biopsy and, later, with the treatment, platelets remained in normal range throughout the follow-up. Besides these hematologic abnormalities there were no other extrarenal clinical manifestations.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The LP is characterized by nephrotic syndrome with normal glomeruli or with slight mesangial proliferation, or rarely, a segmental and focal glomerulosclerosis, in the context of a systemic lupus erythematosus (SLE). The essential criteria for the diagnosis are: (1) absence of endocapillary proliferation; (2) absence of deposits in the capillary wall (although they may exist in the mesangium) and (3) diffuse fusion of podocyte pedicels. In the presence of mesangial deposits, alone or accompanied by mesangial proliferation, some authors<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> prefer to make the diagnose of LN type I or II, respectively, associated with podocytopathy. The LP usually presents at the beginning of SLE, and, generally, ANAs are positive, but anti-DNA may be negative and with a normal complement.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,6</span></a> Most frequently associated extrarenal manifestations are cutaneous and hematological.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,6</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Our patient presented a nephrotic syndrome together with 3 criteria of SLE (thrombocytopenia, ANA and anti-DNA positive).<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> In the biopsy, there was no endocapillary proliferation or thickening of the capillary wall and the IgG deposits were exclusively mesangial, which exclude proliferative (III, IV) and membranous LN (V). The presence of mesangial IgG indicated that it was not an idiopathic minimal change disease. Therefore, in spite of the lack of electronic microscopy, the diagnosis of LP was very likely, as opposed to an LN I/II with podocytopathy. With this diagnosis it is assumed that our patient presented an SLE<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> with corresponding therapeutic implications. The treatment of LP is not definitively established.<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">8,9</span></a> Although its prognosis is uncertain, relapse rate is high, which appear to be lower and later if one immunosuppressant is added to the treatment.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,6</span></a> So, in addition to the induction treatment, a maintenance treatment is probably required. Corticosteroids are used for induction, although the protocol is not definitely established.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,6,8,9</span></a> In our patient, remission was achieved at 2 months, after maintaining 1<span class="elsevierStyleHsp" style=""></span>mg/kg/day of prednisone. Regarding maintenance treatment, there is not enough evidence to recommend any specific medication (anticalcineurinics, azathioprine, mycophenolate mofetil or ritxumab, among others).<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,6,10</span></a> In the series by Hu et al.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,6</span></a> the few patients treated with mycophenolate mofetil did not relapse. In our case, after remission, mycophenolate mofetil was added, along with a progressive decrease in prednisone, until its suspension at 18 months, without a relapse.</p><p id="par0030" class="elsevierStylePara elsevierViewall">The pathophysiologic relationship between podocytopathy and SLE is unknown, but identifying a LP has diagnostic and therapeutic implications, as our case shows. Finally, taking into account all the above considerations and the fact that cases of relapse have been described in which LP has been transformed into another type of LN, it would possibly be justified to include LP as a subtype in the LN classification. All this would allow, in the future, to define the frequency, prognosis and treatment for this condition.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Muray Cases S, Herranz Marín MT, Alcázar Fajardo C, Andreu Muñoz AJ, Cabezuelo Romero JB. Podocitopatía como inicio de un lupus eritematoso sistémico. Nefrologia. 2018;38:674–675.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1567 "Ancho" => 2119 "Tamanyo" => 142512 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Evolution of proteinuria and serum albumin with the treatment.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">MMF: mycophenolate mofetil; Pd: prednisone.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The classification of glomerulonephritis in systemic lupus erythematosus revisited" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J.J. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 13 | 11 | 24 |
| 2024 October | 64 | 57 | 121 |
| 2024 September | 49 | 31 | 80 |
| 2024 August | 75 | 65 | 140 |
| 2024 July | 38 | 27 | 65 |
| 2024 June | 67 | 46 | 113 |
| 2024 May | 55 | 26 | 81 |
| 2024 April | 46 | 35 | 81 |
| 2024 March | 55 | 29 | 84 |
| 2024 February | 25 | 34 | 59 |
| 2024 January | 64 | 34 | 98 |
| 2023 December | 31 | 29 | 60 |
| 2023 November | 33 | 37 | 70 |
| 2023 October | 79 | 46 | 125 |
| 2023 September | 83 | 32 | 115 |
| 2023 August | 53 | 29 | 82 |
| 2023 July | 48 | 34 | 82 |
| 2023 June | 67 | 34 | 101 |
| 2023 May | 79 | 34 | 113 |
| 2023 April | 51 | 20 | 71 |
| 2023 March | 55 | 22 | 77 |
| 2023 February | 52 | 18 | 70 |
| 2023 January | 40 | 29 | 69 |
| 2022 December | 49 | 29 | 78 |
| 2022 November | 55 | 34 | 89 |
| 2022 October | 53 | 46 | 99 |
| 2022 September | 77 | 37 | 114 |
| 2022 August | 59 | 48 | 107 |
| 2022 July | 48 | 49 | 97 |
| 2022 June | 47 | 51 | 98 |
| 2022 May | 59 | 29 | 88 |
| 2022 April | 79 | 56 | 135 |
| 2022 March | 109 | 51 | 160 |
| 2022 February | 57 | 52 | 109 |
| 2022 January | 67 | 30 | 97 |
| 2021 December | 86 | 47 | 133 |
| 2021 November | 78 | 46 | 124 |
| 2021 October | 114 | 41 | 155 |
| 2021 September | 103 | 46 | 149 |
| 2021 August | 73 | 40 | 113 |
| 2021 July | 59 | 37 | 96 |
| 2021 June | 39 | 29 | 68 |
| 2021 May | 52 | 49 | 101 |
| 2021 April | 115 | 79 | 194 |
| 2021 March | 82 | 54 | 136 |
| 2021 February | 61 | 43 | 104 |
| 2021 January | 69 | 23 | 92 |
| 2020 December | 45 | 21 | 66 |
| 2020 November | 56 | 17 | 73 |
| 2020 October | 33 | 39 | 72 |
| 2020 September | 25 | 18 | 43 |
| 2020 August | 53 | 19 | 72 |
| 2020 July | 38 | 22 | 60 |
| 2020 June | 47 | 22 | 69 |
| 2020 May | 55 | 17 | 72 |
| 2020 April | 41 | 22 | 63 |
| 2020 March | 56 | 17 | 73 |
| 2020 February | 61 | 20 | 81 |
| 2020 January | 62 | 33 | 95 |
| 2019 December | 71 | 34 | 105 |
| 2019 November | 60 | 23 | 83 |
| 2019 October | 66 | 32 | 98 |
| 2019 September | 53 | 23 | 76 |
| 2019 August | 57 | 25 | 82 |
| 2019 July | 47 | 29 | 76 |
| 2019 June | 34 | 35 | 69 |
| 2019 May | 43 | 31 | 74 |
| 2019 April | 60 | 68 | 128 |
| 2019 March | 46 | 25 | 71 |
| 2019 February | 23 | 26 | 49 |
| 2019 January | 30 | 33 | 63 |
| 2018 December | 64 | 52 | 116 |
| 2018 November | 5 | 1 | 6 |
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