Letters to the Editor – Brief Case Reports
Encapsulating peritoneal sclerosis: A review of 3 cases
Esclerosis peritoneal encapsulante: revisión de 3 casos
Carmen Merino Bueno, Laura del Rio García, José Joaquín Bande Fernández, Raúl García, Carlos Hidalgo Ordoñez, Carmen Rodríguez-Suárez, José Emilio Sánchez-Álvarez
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Servicio de Nefrología, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain
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Laura del Rio García, José Joaquín Bande Fernández, Raúl García, Carlos Hidalgo Ordoñez, Carmen Rodríguez-Suárez, José Emilio Sánchez-Álvarez" "autores" => array:7 [ 0 => array:2 [ "nombre" => "Carmen" "apellidos" => "Merino Bueno" ] 1 => array:2 [ "nombre" => "Laura" "apellidos" => "del Rio García" ] 2 => array:2 [ "nombre" => "José Joaquín" "apellidos" => "Bande Fernández" ] 3 => array:2 [ "nombre" => "Raúl" "apellidos" => "García" ] 4 => array:2 [ "nombre" => "Carlos" "apellidos" => "Hidalgo Ordoñez" ] 5 => array:2 [ "nombre" => "Carmen" "apellidos" => "Rodríguez-Suárez" ] 6 => array:4 [ "nombre" => "José Emilio" "apellidos" => "Sánchez-Álvarez" "email" => array:1 [ 0 => "jesastur@hotmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Nefrología, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Esclerosis peritoneal encapsulante: revisión de 3 casos" ] ] "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" => 1272 "Ancho" => 1958 "Tamanyo" => 333868 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">(A) CT scan: gastric and duodenal dilatation, with abundant intra-abdominal loculated fluid, and shift of small intestine loops. (B) Peritoneal biopsy; haematoxylin and eosin staining, and immunohistochemistry techniques.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Encapsulating peritoneal sclerosis (EPS) is an uncommon but severe complication among patients on peritoneal dialysis (PD). This report describes three patients from our centre who were diagnosed of EPS collected during a 20-year period.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Case 1</span><p id="par0010" class="elsevierStylePara elsevierViewall">A female patient with Laurence-Moon-Biedl syndrome. Haemodialysis (HD) was started at the age of 11. She received her first renal transplant in the following year, which failed due to thrombosis of the vein graft. Two years later, she was started on PD because of difficulties with vascular access. The patient developed several peritonitis and severe hyperparathyroidism. Her peritoneal transport was medium–high at baseline. After 12 years of dialysis, this patient had a new onset of peritonitis, associated with tunnel infection and peritoneal ventral hernia in the catheter site. Ultrasounds revealed sclerosis and peritoneal calcification with compressed abdominal viscera. The patient received antibiotics, the catheter was withdrawn, and she was started on HD. She later developed acute ischaemia in the right lower limb as a result of femoral–popliteal obstruction. Surgery was not considered due to the poor patient's clinical condition, and she died soon after.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Case 2</span><p id="par0015" class="elsevierStylePara elsevierViewall">A male patient diagnosed of rapidly progressive glomerulonephritis with basal glomerular anti-membrane antibodies was treated with steroids, cyclophosphamide, and plasmapheresis with no positive results. In his 5 years of PD, the patient developed 10 episodes of peritonitis, 8 of which were due to <span class="elsevierStyleItalic">Staphylococcus aureus</span>. The last peritonitis was caused by <span class="elsevierStyleItalic">Candida albicans</span>; and for this reason, the catheter was withdrawn and the patient started HD.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Peritoneal transport was originally medium–high, and finally became high. Six months later the patient developed abdominal pain and a mass effect was reflected in the suprapubic region. Peritoneal thickening, pelvic fluid collection with septa comprising loops, and large gastric and intestinal dilatation were observed in a CT scan (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Surgery was not considered and the patient was hospitalised several times because of intestinal occlusion; his general condition became progressively worse and he died soon after.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Case 3</span><p id="par0025" class="elsevierStylePara elsevierViewall">A male patient who had focal segmental hyalinosis which was refractory to steroids, cyclophosphamide, and vincristine. He started PD in 1990. During the following 11 years he received 3 renal transplants, with a total dialysis-free time of 5 years. He underwent parathyroidectomy due to severe hyperparathyroidism.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Peritoneal transport remained medium–high with a tendency to increase. Ultrafiltration decreased over time and the need for osmotic solutions was greater. CA-125 was measured in the peritoneal effluent several times and a gradual decrease was observed over time. The patient had 12 peritonitis, all of them caused by Gram positive germs. HD was indicated, but the patient still tried to avoid it by all means; he finally started HD in 2011.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Six months later, he was admitted with pain and abdominal distension. A CT scan of the abdomen (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>A) showed gastric and duodenal dilatation with free loculated fluid and oedematous bowel. A peritoneal biopsy revealed proliferation of small vessels, inflammatory cellularity, and fibrosis (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>B). After EPS was diagnosed, treatment with prednisone and tamoxifen was started. The size of the collection had decreased after six months, and no loop dilatation was observed. The patient did not developed more complications. He is currently receiving HD and tamoxifen.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">The incidence of EPS in our Nephrology service was 0.47% lower compared to other case studies where it ranges between 0.7 and 7.3%.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">1–3</span></a> It is an uncommon but severe complication characterised by a high mortality rate (two of our patients died). Pathogenesis is unclear, and constant peritoneal inflammation associated with glucose and glucose-degradation products is thought to enhance peritoneal permeability to certain substances, including fibrin, thereby promoting fibrosis; in addition, a second stimulus, such as peritonitis, abdominal surgery, bioincompatibility of solutions, or long time in PD,<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">4–7</span></a> may accelerate progression or ultimately trigger EPS.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">2</span></a> Some of these have been clearly implicated in the development of EPS in our patients.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Finally, both the interruption of the washout and the build-up of profibrotic factors,<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">8</span></a> as well as the use of anti-calcineurin drugs, give rise to increased fibrosis.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">9</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Clinical manifestations of EPS consist of signs and symptoms of bowel obstruction.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">10</span></a> X-rays show an image of “cocoon” (encased loops due to fibrosis), adhesions, air-fluid levels, and peritoneal calcification. Diagnosis is confirmed by histological findings: mesothelial detachment and thickened peritoneum with fibrin deposition, inflammatory infiltrates, capillary angiogenesis, and calcification.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">10</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">No biochemical markers or X-ray examinations are able to screen patients at risk of EPS. Low CA-125 and high IL-6 in the peritoneal effluent have been suggested as prognostic factors.<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">11</span></a> Our third patient developed a progressive decrease during PD.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Prevention is the treatment cornerstone, including stronger and earlier options, such as the use of biocompatible solutions and early screening. Immunosuppresants, particularly corticosteroids and tamoxiphen, are the treatment of choice for the management of inflammation as a result of their anti-fibrotic effects. In advanced stages, adhesions and encased loops can be released by surgery, and yet, even though intestinal obstruction is reverted, peritoneal impairment is not halted.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">4</span></a></p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:5 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Case 1" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Case 2" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Case 3" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Discussion" ] 4 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2014-11-25" "fechaAceptado" => "2015-03-15" "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Merino Bueno C, del Rio García L, Bande Fernández JJ, García R, Hidalgo Ordoñez C, Rodríguez-Suárez C, et al. Esclerosis peritoneal encapsulante: revisión de 3 casos. Nefrologia. 2015;35:588–590.</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" => 556 "Ancho" => 1958 "Tamanyo" => 114122 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">CT scan: thickened peritoneum associated with pelvic fluid collection with septa inside and air-fluid levels.</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" => 1272 "Ancho" => 1958 "Tamanyo" => 333868 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">(A) CT scan: gastric and duodenal dilatation, with abundant intra-abdominal loculated fluid, and shift of small intestine loops. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 7 | 8 | 15 |
| 2024 October | 53 | 55 | 108 |
| 2024 September | 55 | 34 | 89 |
| 2024 August | 56 | 41 | 97 |
| 2024 July | 38 | 30 | 68 |
| 2024 June | 82 | 23 | 105 |
| 2024 May | 55 | 29 | 84 |
| 2024 April | 46 | 28 | 74 |
| 2024 March | 58 | 32 | 90 |
| 2024 February | 50 | 34 | 84 |
| 2024 January | 46 | 29 | 75 |
| 2023 December | 46 | 23 | 69 |
| 2023 November | 42 | 32 | 74 |
| 2023 October | 40 | 34 | 74 |
| 2023 September | 34 | 18 | 52 |
| 2023 August | 41 | 30 | 71 |
| 2023 July | 40 | 25 | 65 |
| 2023 June | 28 | 15 | 43 |
| 2023 May | 41 | 27 | 68 |
| 2023 April | 46 | 10 | 56 |
| 2023 March | 57 | 20 | 77 |
| 2023 February | 50 | 22 | 72 |
| 2023 January | 38 | 22 | 60 |
| 2022 December | 46 | 26 | 72 |
| 2022 November | 58 | 26 | 84 |
| 2022 October | 32 | 32 | 64 |
| 2022 September | 37 | 28 | 65 |
| 2022 August | 52 | 50 | 102 |
| 2022 July | 39 | 39 | 78 |
| 2022 June | 43 | 23 | 66 |
| 2022 May | 44 | 31 | 75 |
| 2022 April | 40 | 41 | 81 |
| 2022 March | 40 | 50 | 90 |
| 2022 February | 49 | 40 | 89 |
| 2022 January | 44 | 37 | 81 |
| 2021 December | 46 | 42 | 88 |
| 2021 November | 23 | 34 | 57 |
| 2021 October | 73 | 52 | 125 |
| 2021 September | 38 | 30 | 68 |
| 2021 August | 57 | 40 | 97 |
| 2021 July | 40 | 40 | 80 |
| 2021 June | 51 | 27 | 78 |
| 2021 May | 45 | 29 | 74 |
| 2021 April | 80 | 40 | 120 |
| 2021 March | 62 | 30 | 92 |
| 2021 February | 57 | 22 | 79 |
| 2021 January | 52 | 21 | 73 |
| 2020 December | 37 | 15 | 52 |
| 2020 November | 37 | 19 | 56 |
| 2020 October | 28 | 22 | 50 |
| 2020 September | 29 | 14 | 43 |
| 2020 August | 46 | 17 | 63 |
| 2020 July | 34 | 16 | 50 |
| 2020 June | 39 | 10 | 49 |
| 2020 May | 62 | 13 | 75 |
| 2020 April | 37 | 18 | 55 |
| 2020 March | 47 | 12 | 59 |
| 2020 February | 37 | 24 | 61 |
| 2020 January | 49 | 15 | 64 |
| 2019 December | 69 | 21 | 90 |
| 2019 November | 55 | 27 | 82 |
| 2019 October | 37 | 16 | 53 |
| 2019 September | 52 | 14 | 66 |
| 2019 August | 42 | 14 | 56 |
| 2019 July | 41 | 26 | 67 |
| 2019 June | 32 | 19 | 51 |
| 2019 May | 44 | 19 | 63 |
| 2019 April | 75 | 38 | 113 |
| 2019 March | 48 | 27 | 75 |
| 2019 February | 36 | 20 | 56 |
| 2019 January | 39 | 17 | 56 |
| 2018 December | 154 | 37 | 191 |
| 2018 November | 260 | 18 | 278 |
| 2018 October | 192 | 16 | 208 |
| 2018 September | 108 | 14 | 122 |
| 2018 August | 66 | 9 | 75 |
| 2018 July | 58 | 12 | 70 |
| 2018 June | 56 | 19 | 75 |
| 2018 May | 82 | 11 | 93 |
| 2018 April | 87 | 11 | 98 |
| 2018 March | 100 | 13 | 113 |
| 2018 February | 76 | 12 | 88 |
| 2018 January | 56 | 14 | 70 |
| 2017 December | 47 | 9 | 56 |
| 2017 November | 54 | 9 | 63 |
| 2017 October | 36 | 7 | 43 |
| 2017 September | 41 | 10 | 51 |
| 2017 August | 34 | 7 | 41 |
| 2017 July | 35 | 8 | 43 |
| 2017 June | 32 | 5 | 37 |
| 2017 May | 32 | 13 | 45 |
| 2017 April | 31 | 11 | 42 |
| 2017 March | 21 | 6 | 27 |
| 2017 February | 37 | 14 | 51 |
| 2017 January | 28 | 9 | 37 |
| 2016 December | 47 | 7 | 54 |
| 2016 November | 52 | 11 | 63 |
| 2016 October | 94 | 8 | 102 |
| 2016 September | 106 | 1 | 107 |
| 2016 August | 98 | 2 | 100 |
| 2016 July | 144 | 4 | 148 |
| 2016 June | 86 | 0 | 86 |
| 2016 May | 141 | 0 | 141 |
| 2016 April | 81 | 0 | 81 |
| 2016 March | 69 | 0 | 69 |
| 2016 February | 72 | 0 | 72 |
| 2016 January | 34 | 0 | 34 |
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