Corresponding author at: Servicio de Nefrología, Hospital 12 de Octubre, Avda. Andalucía, s/n, 28041 Madrid, Spain. Tel.: +34 609826301.
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array:3 [ "nombre" => "Manuel" "apellidos" => "Praga" "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 12 de Octubre, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital 12 de Octubre, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author at</span>: Servicio de Nefrología, Hospital 12 de Octubre, Avda. Andalucía, s/n, 28041 Madrid, Spain. Tel.: +34 609826301." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Nefropatía mesangial y síndrome antisintetasa: una forma curiosa de asociación" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 640 "Ancho" => 1300 "Tamanyo" => 192392 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">(a) Immunofluorescence study of renal tissue showing mesangial deposits of IgA. (b) Electron microscopy showing electron-dense deposits in the mesangium (white arrow).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Anti-synthetase syndrome (ASS) is a poorly-defined, uncommon syndrome, and until recently was included within the idiopathic inflammatory myopathies, which are characterised by the presence of anti-synthetase antibodies (ASAb) in the serum. ASAbs are IgG antibodies directed against the enzyme synthetase, which mediates the binding of RNA to the specific amino acid, to form transfer RNA. Of the main ASAbs described in the bibliography, the first and most commonly found (15%–30%) is anti-Jo1, which is directed against the synthetase that mediates binding between RNA and histidine.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">During the 1990s, authors began to describe clinical cases of patients with myositis, the presence of ASAbs in serum, and some generally common clinical characteristics that came to be defined as ASS. AAS is characterized by the association of inflammatory myopathy, which is clinically as muscle weakness; interstitial lung disease, which can lead to acute respiratory failure of different histological patterns (bronchiolitis obliterans, interstitial infiltrates of predominantly plasma cells and lymphocytes, and diffuse pulmonary fibrosis); non-erosive inflammatory arthritis; the so-called mechanic's hands, which have hyperkeratotic lesions accompanied by fissures, hyperpigmentation, and scaling on the palm and on the palmar and lateral surfaces of the fingers; and other symptoms such as fever, Raynaud syndrome, and Sjögren syndrome.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">1,2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">We present a case of a 59-year-old man, with no past medical history of note except for an 80 pack-year smoking history until 6 months before his admission. Over the previous 2 months he had developed symptoms of arthralgia, myalgia, and oedema in the upper and lower limbs, with associated loss of muscle strength. He also had moderate exertional dyspnoea. On physical examination he had a blood pressure of 100/76<span class="elsevierStyleHsp" style=""></span>mmHg, oxygen saturation of 91% and mild oedema of both lower limbs. Investigations: arterial blood gas revealed a pH of 7.47, pCO<span class="elsevierStyleInf">2</span> 35<span class="elsevierStyleHsp" style=""></span>mmHg, pO<span class="elsevierStyleInf">2</span> 58<span class="elsevierStyleHsp" style=""></span>mmHg, HCO<span class="elsevierStyleInf">3</span> 25.5<span class="elsevierStyleHsp" style=""></span>mmol/L, oxygen saturation 91.5%. Haemoglobin was 11.4<span class="elsevierStyleHsp" style=""></span>g/dL, and leukocytes and platelets were within normal limits. Biochemistry showed a total protein of 4.7<span class="elsevierStyleHsp" style=""></span>g/dL, albumin 2.74<span class="elsevierStyleHsp" style=""></span>g/dL, ALT 414<span class="elsevierStyleHsp" style=""></span>UI/L, AST 436<span class="elsevierStyleHsp" style=""></span>UI/L, GGT 11<span class="elsevierStyleHsp" style=""></span>UI/L, LDH<span class="elsevierStyleHsp" style=""></span>1.443<span class="elsevierStyleHsp" style=""></span>UI/L, CK<span class="elsevierStyleHsp" style=""></span>9.906<span class="elsevierStyleHsp" style=""></span>UI/L, serum creatinine 0.6<span class="elsevierStyleHsp" style=""></span>mg/dL, and urea 39<span class="elsevierStyleHsp" style=""></span>mg/dL. Acute phase reactants included an erythrocyte sedimentation rate of 37<span class="elsevierStyleHsp" style=""></span>mmHg and CRP of 4.13<span class="elsevierStyleHsp" style=""></span>mg/dL. On the auto-immune study we found negative ANA-IFA, positive ANA-<span class="elsevierStyleItalic">screening multiplex</span>, a positive anti-Jo1/HRS Ab, and the rest of the study was negative, with complement values within normal limits. Urine contained dysmorphic erythrocytes in the urinary sediment and proteinuria of 0.4<span class="elsevierStyleHsp" style=""></span>g/24<span class="elsevierStyleHsp" style=""></span>h. Imaging included chest X-ray which showed a bilateral interstitial pattern confirmed on chest scan (findings of bilateral interstitial disease). Electromyography showed signs of inflammatory myopathy. The muscle biopsy findings were compatible with inflammatory myopathy, an immune myopathy with perimysial (around miocyte) pathology (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>a). On immunohistochemical study there was diffuse overexpression of MHC-1 in the membrane of the muscle fibres (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>b). Renal biopsy showed renal parenchyma with mild mesangial expansion. IgA was present on direct immunofluorescence (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>a). Electron microscopy showed, as the main abnormality, mild mesangial widening from both cells and matrix with frequent homogeneous electron-dense deposits (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>b). The patient received treatment with steroids at a dose of 1<span class="elsevierStyleHsp" style=""></span>mg/kg/day and azathioprine 100<span class="elsevierStyleHsp" style=""></span>mg/day, which was tolerated well, with clinical improvement, a reduction in markers of muscle damage, and resolution of the interstitial pattern.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Although the presence of ASAbs was previously considered specific to polymyositis and dermatomyositis, during the last few years there has been describing a syndrome comprising the presence of these autoantibodies along with interstitial lung disease, arthritis, fever, hyperkeratotic lesions on the hands (mechanic's hands) and Raynaud's phenomenon, which has been called ASS. The clinical presentation varies between pulmonary, muscle, and/or joint disease, but data on renal involvement in this condition are very scarce.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">3</span></a> One excellent French study described 14 cases of nephropathy and inflammatory myopathy (documented with biopsy), of which 3 cases were ASS.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">4</span></a> Two patients had mesangial IgA nephropathy and one patient had a membranous nephropathy. The 2 patients with mesangial nephropathy received immunosuppressive treatment with steroids and methotrexate, achieving complete remission of renal disease. Mesangial IgA nephropathy is the most documented glomerular disease associated with ASS.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">5–7</span></a> The prognosis of such patients is usually determined by pulmonary involvement. The optimal treatment for ASS is based on the administration of steroids and immunosuppressors, although there are other cases described that have been treated successfully with rituximab.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">8</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">In conclusion, although inflammatory myopathies are uncommon connective tissue diseases, we should look for their co-existence in patients with renal disease. In ASS, the most frequently-associated glomerular nephropathy is mesangial IgA nephropathy, which has a favourable outcome in most cases.</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: Morales E, Rabasco C, Panizo N, Gutierrez E, Martinez MA, Toldos O, et al. Nefropatía mesangial y síndrome antisintetasa: una forma curiosa de asociación. Nefrologia. 2015;35:415–417.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 813 "Ancho" => 2167 "Tamanyo" => 330736 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(a) Haematoxylin–eosin study of skeletal muscle tissue with mononuclear inflammatory infiltrate with preferential perimysial localisation. (b) Immunohistochemical study of muscle tissue for MHC 1 (HLA) showing its overexpression in the membrane of muscle fibres.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 640 "Ancho" => 1300 "Tamanyo" => 192392 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">(a) Immunofluorescence study of renal tissue showing mesangial deposits of IgA. 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Year/Month | Html | Total | |
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2024 November | 14 | 10 | 24 |
2024 October | 73 | 42 | 115 |
2024 September | 79 | 30 | 109 |
2024 August | 90 | 91 | 181 |
2024 July | 76 | 35 | 111 |
2024 June | 107 | 34 | 141 |
2024 May | 117 | 34 | 151 |
2024 April | 102 | 40 | 142 |
2024 March | 75 | 26 | 101 |
2024 February | 67 | 49 | 116 |
2024 January | 70 | 29 | 99 |
2023 December | 53 | 21 | 74 |
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2023 October | 82 | 35 | 117 |
2023 September | 120 | 34 | 154 |
2023 August | 85 | 22 | 107 |
2023 July | 94 | 27 | 121 |
2023 June | 64 | 23 | 87 |
2023 May | 79 | 29 | 108 |
2023 April | 52 | 17 | 69 |
2023 March | 64 | 30 | 94 |
2023 February | 51 | 24 | 75 |
2023 January | 78 | 28 | 106 |
2022 December | 57 | 37 | 94 |
2022 November | 62 | 22 | 84 |
2022 October | 99 | 37 | 136 |
2022 September | 72 | 42 | 114 |
2022 August | 70 | 43 | 113 |
2022 July | 57 | 46 | 103 |
2022 June | 73 | 30 | 103 |
2022 May | 62 | 31 | 93 |
2022 April | 50 | 43 | 93 |
2022 March | 61 | 52 | 113 |
2022 February | 63 | 38 | 101 |
2022 January | 86 | 28 | 114 |
2021 December | 62 | 46 | 108 |
2021 November | 89 | 35 | 124 |
2021 October | 54 | 37 | 91 |
2021 September | 65 | 43 | 108 |
2021 August | 86 | 43 | 129 |
2021 July | 40 | 29 | 69 |
2021 June | 117 | 31 | 148 |
2021 May | 60 | 25 | 85 |
2021 April | 129 | 36 | 165 |
2021 March | 109 | 41 | 150 |
2021 February | 95 | 32 | 127 |
2021 January | 68 | 14 | 82 |
2020 December | 40 | 18 | 58 |
2020 November | 35 | 23 | 58 |
2020 October | 29 | 17 | 46 |
2020 September | 24 | 12 | 36 |
2020 August | 36 | 15 | 51 |
2020 July | 40 | 14 | 54 |
2020 June | 32 | 15 | 47 |
2020 May | 46 | 12 | 58 |
2020 April | 42 | 21 | 63 |
2020 March | 33 | 12 | 45 |
2020 February | 45 | 25 | 70 |
2020 January | 39 | 20 | 59 |
2019 December | 53 | 25 | 78 |
2019 November | 45 | 29 | 74 |
2019 October | 38 | 11 | 49 |
2019 September | 32 | 18 | 50 |
2019 August | 24 | 20 | 44 |
2019 July | 29 | 21 | 50 |
2019 June | 37 | 20 | 57 |
2019 May | 33 | 26 | 59 |
2019 April | 70 | 44 | 114 |
2019 March | 28 | 19 | 47 |
2019 February | 17 | 11 | 28 |
2019 January | 29 | 28 | 57 |
2018 December | 126 | 38 | 164 |
2018 November | 227 | 24 | 251 |
2018 October | 212 | 28 | 240 |
2018 September | 106 | 16 | 122 |
2018 August | 38 | 14 | 52 |
2018 July | 50 | 14 | 64 |
2018 June | 58 | 11 | 69 |
2018 May | 74 | 13 | 87 |
2018 April | 64 | 16 | 80 |
2018 March | 102 | 6 | 108 |
2018 February | 34 | 7 | 41 |
2018 January | 56 | 9 | 65 |
2017 December | 44 | 7 | 51 |
2017 November | 55 | 12 | 67 |
2017 October | 42 | 7 | 49 |
2017 September | 37 | 11 | 48 |
2017 August | 40 | 17 | 57 |
2017 July | 38 | 10 | 48 |
2017 June | 48 | 9 | 57 |
2017 May | 56 | 8 | 64 |
2017 April | 46 | 6 | 52 |
2017 March | 33 | 7 | 40 |
2017 February | 35 | 5 | 40 |
2017 January | 30 | 7 | 37 |
2016 December | 65 | 10 | 75 |
2016 November | 61 | 16 | 77 |
2016 October | 100 | 16 | 116 |
2016 September | 94 | 2 | 96 |
2016 August | 138 | 1 | 139 |
2016 July | 165 | 8 | 173 |
2016 June | 121 | 0 | 121 |
2016 May | 103 | 0 | 103 |
2016 April | 98 | 0 | 98 |
2016 March | 91 | 0 | 91 |
2016 February | 93 | 0 | 93 |
2016 January | 103 | 0 | 103 |
2015 December | 96 | 0 | 96 |
2015 November | 74 | 0 | 74 |
2015 October | 69 | 0 | 69 |