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Renal involvement is unusual and, when it does present, it is associated with poorer survival.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">1</span></a> Below we describe a case of a patient with RP and associated glomerular disease.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 39-year-old male, who was admitted to hospital in June 1986 due to fever and symmetric migratory polyarthritis of large and small joints, conductive hearing loss secondary to seromucinous otitis media and conjunctivitis. During the admission, he presented with bilateral auricular chondritis with erythema and pain on palpitation. Biopsy of the auricular cartilage was performed, which confirmed the diagnosis of RP.</p><p id="par0015" class="elsevierStylePara elsevierViewall">In the laboratory tests, the patient presented mild normocytic anaemia, leucocytosis, thrombocytosis and elevation of acute-phase reactants (C-reactive protein 22<span class="elsevierStyleHsp" style=""></span>mg/dl), with sterile blood cultures and urine culture. Kidney function was normal, with proteinuria of 0.5<span class="elsevierStyleHsp" style=""></span>g/24<span class="elsevierStyleHsp" style=""></span>h and microscopic haematuria. Antinuclear antibodies and anti-DNA and the rheumatoid factor were negative, complement (C3 and C4) values were in the normal range on several occasions. Kidney function was maintained within normal limits, but the patient presented proteinuria of 2.9<span class="elsevierStyleHsp" style=""></span>g/24<span class="elsevierStyleHsp" style=""></span>h and abnormalities in the urinary sediment which included microscopic haematuria and leucocyturia. This is why a kidney biopsy was performed. Optical microscopy showed findings compatible with focal segmental hyalinosis with periglomerular sclerosis (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Segmental IgM deposits were revealed using the immunofluorescence technique, and an irregular podocyte effacement was observed using electron microscopy (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Treatment was started with prednisone at 1<span class="elsevierStyleHsp" style=""></span>mg/kg weight and captopril at maximum doses, with a favourable clinical course of the patient's polychondritis and renal response with reduction of proteinuria to 0.4<span class="elsevierStyleHsp" style=""></span>g/24<span class="elsevierStyleHsp" style=""></span>h and disappearance of microscopic haematuria. After discharge from Nephrology, steroids were maintained for six months due to his RP and steroid treatment was subsequently received on request for short periods, according to the joint inflammation symptoms. During his clinical course, at points the patient tested positive for cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) against serine proteinase-3 (PR3) at low titres, but without presenting clinical data of vasculitis or other extrarenal manifestations. Anti-myeloperoxidase (MPO) antibodies and cryoglobulins were negative. After 32 years of evolution of his condition, the patient has presented acceptable progress of his kidney function (serum creatinine of 1.4<span class="elsevierStyleHsp" style=""></span>mg/dl and proteinuria of 0.6<span class="elsevierStyleHsp" style=""></span>g/24<span class="elsevierStyleHsp" style=""></span>h) on treatment with renin–angiotensin axis blocking drugs.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">RP is a rare autoimmune disease which, in addition to compromising cartilage structures, also affects non-cartilage structures with a high content of glycosaminoglycans, such as the lungs, heart, eyes and blood vessels. Renal involvement in the clinical course of RP is very rare and it was Neil et al., in 1978, who reported the first cases of glomerular disease associated with RP.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">2</span></a> In the literature review, the prevalence of renal involvement in RP is highly variable. It is possible to find historical series such as that of the Mayo Clinic, with a prevalence of 22% renal involvement according to clinical criteria (presence of haematuria or proteinuria) or histological criteria (with the most prevalent lesions being mesangial nephropathy and segmental necrotising and crescentic glomerulonephritis).<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">1</span></a> However, more recent studies report a significantly lower percentage than that reported previously. In a German series, which included 62 patients with RP, only 6.5% had associated renal damage,<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">3</span></a> and in another large Asian study which evaluated the clinical and prognostic characteristics of 158 patients with RP, the prevalence of renal involvement was only 3%.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">4</span></a> In a recent review, Dion et al. analysed 142 patients with RP and no patient presented renal involvement.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">5</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Although most of the wide range of histological lesions associated with RP which have been reported up to now correspond with segmental necrotising and crescentic glomerulonephritis,<a class="elsevierStyleCrossRefs" href="#bib0085"><span class="elsevierStyleSup">6–8</span></a> other types of lesions have also been reported, such as mesangial IgA nephropathy, minimal change disease and membranous glomerulonephritis.<a class="elsevierStyleCrossRefs" href="#bib0085"><span class="elsevierStyleSup">6,9</span></a> There is considerable lack of knowledge with regard to the aetiopathogenesis of renal damage associated with RP, with a possible autoimmune mechanism gaining increasing importance. For this reason, treatment is empirical and is based on the use of corticosteroids or other immunosuppressants, according to the severity of the disease. In cases of greater renal aggressiveness (necrotising and crescentic glomerulonephritis), the use of cyclophosphamide and plasmapheresis, in addition to corticosteroids, has been reported.<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">7,10</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The presence of ANCA has been reported in a percentage of cases of RP; however, there is diverging information with regard to the aetiopathogenic role of these in the association with vasculitis. Papo et al., in a series of 23 patients with RP, reported ANCA positivity by immunofluorescence in 30% of the cases (three cases were c-ANCA and five were p-ANCA); however, when analysing these same patients with another more specific technique (ELISA), none of those who had c-ANCA were PR3-positive and four of the five p-ANCA patients were MPO-positive.<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">11</span></a> Therefore, although ANCA can be detected in RP, its relevance requires an additional analysis in the clinical context of each patient.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Unlike other reported cases, the clinical course of the kidney disease in our patient has been very favourable, with the administration of corticosteroids and renin–angiotensin axis blocking drugs also coinciding with long periods of remission of his RP.</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: Canllavi E, Alonso M, Fernández M, Gutiérreza E, Morales E. Policondritis recidivante y glomeruloesclerosis segmentaria y focal: coincidencia o causalidad. Nefrologia. 2020;40:360–362.</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" => 1130 "Ancho" => 1505 "Tamanyo" => 567828 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">The image shows a glomerulus with a segmental sclerosing lesion (from left to right and from top to bottom, HE, Masson, PAS, silver; 40×).</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" => 895 "Ancho" => 955 "Tamanyo" => 276085 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">The electron microscopy study shows irregular pedicle fusion which affects approximately 30% of the capillary surface (arrow).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:11 [ 0 => array:3 [ "identificador" => "bib0060" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Renal involvement in relapsing polychondritis" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. 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Year/Month | Html | Total | |
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2024 November | 5 | 8 | 13 |
2024 October | 52 | 42 | 94 |
2024 September | 59 | 40 | 99 |
2024 August | 69 | 65 | 134 |
2024 July | 86 | 35 | 121 |
2024 June | 60 | 45 | 105 |
2024 May | 58 | 61 | 119 |
2024 April | 58 | 34 | 92 |
2024 March | 70 | 34 | 104 |
2024 February | 57 | 43 | 100 |
2024 January | 46 | 39 | 85 |
2023 December | 42 | 28 | 70 |
2023 November | 74 | 47 | 121 |
2023 October | 132 | 32 | 164 |
2023 September | 280 | 35 | 315 |
2023 August | 252 | 34 | 286 |
2023 July | 71 | 27 | 98 |
2023 June | 90 | 17 | 107 |
2023 May | 82 | 37 | 119 |
2023 April | 48 | 19 | 67 |
2023 March | 73 | 21 | 94 |
2023 February | 63 | 21 | 84 |
2023 January | 61 | 26 | 87 |
2022 December | 82 | 39 | 121 |
2022 November | 83 | 39 | 122 |
2022 October | 114 | 40 | 154 |
2022 September | 59 | 33 | 92 |
2022 August | 130 | 72 | 202 |
2022 July | 65 | 48 | 113 |
2022 June | 53 | 51 | 104 |
2022 May | 51 | 21 | 72 |
2022 April | 61 | 60 | 121 |
2022 March | 92 | 36 | 128 |
2022 February | 85 | 41 | 126 |
2022 January | 50 | 31 | 81 |
2021 December | 40 | 47 | 87 |
2021 November | 70 | 41 | 111 |
2021 October | 71 | 59 | 130 |
2021 September | 41 | 39 | 80 |
2021 August | 62 | 43 | 105 |
2021 July | 61 | 47 | 108 |
2021 June | 36 | 16 | 52 |
2021 May | 39 | 43 | 82 |
2021 April | 56 | 61 | 117 |
2021 March | 34 | 29 | 63 |
2021 February | 46 | 30 | 76 |
2021 January | 32 | 14 | 46 |
2020 December | 23 | 19 | 42 |
2020 November | 28 | 10 | 38 |
2020 October | 32 | 20 | 52 |
2020 September | 40 | 25 | 65 |
2020 August | 64 | 24 | 88 |
2020 July | 50 | 32 | 82 |
2020 June | 21 | 20 | 41 |