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"documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Med Clin. 2021;157:85-6" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "en" => array:10 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Scientific letter</span>" "titulo" => "Effectiveness of sofosbuvir/velpatasvir/voxilaprevir in patients with chronic hepatitis C virus infection and virologic failure to direct-acting antivirals" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "85" "paginaFinal" => "86" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Efectividad de sofosbuvir/velpatasvir/voxilaprevir en pacientes con infección crónica por el virus de la hepatitis C y fracaso virológico a otros antivirales de acción directa" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Sonia Ruiz-Boy, Montserrat Rodriguez-Reyes, Jose Miguel Sotoca-Momblona" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Sonia" "apellidos" => "Ruiz-Boy" ] 1 => array:2 [ "nombre" => "Montserrat" "apellidos" => "Rodriguez-Reyes" ] 2 => array:2 [ "nombre" => "Jose Miguel" "apellidos" => "Sotoca-Momblona" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0025775320305078" "doi" => "10.1016/j.medcli.2020.05.054" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775320305078?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020621003223?idApp=UINPBA00004N" "url" => "/23870206/0000015700000002/v1_202107200643/S2387020621003223/v1_202107200643/en/main.assets" ] "en" => array:14 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Scientific letter</span>" "titulo" => "Idiopathic retroperitoneal fibrosis (Ormond's disease) as a transient risk factor for venous thromboembolism" "tieneTextoCompleto" => true "saludo" => "Dear Editor:" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "87" "paginaFinal" => "88" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Rubén Alonso-Beato, Francisco Galeano-Valle, Pablo Demelo-Rodriguez" "autores" => array:3 [ 0 => array:3 [ "nombre" => "Rubén" "apellidos" => "Alonso-Beato" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:4 [ "nombre" => "Francisco" "apellidos" => "Galeano-Valle" "email" => array:1 [ 0 => "paco.galeano.valle@gmail.com" ] "referencia" => array:3 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 2 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 2 => array:3 [ "nombre" => "Pablo" "apellidos" => "Demelo-Rodriguez" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Venous Thromboembolism Unit, Internal Medicine, Hospital General Universitario Gregorio Marañón, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Instituto de investigación sanitaria Gregorio Marañón, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Fibrosis retroperitoneal idiopática (enferemdad de Ormond) como factor de riesgo transitorio de tromboembolia venosa" ] ] "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" => 2232 "Ancho" => 1634 "Tamanyo" => 228779 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Bilateral femoral deep vein thrombosis. (B) Retroperitoneal fibrosis. Left renal helium compression.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We present a 59-year-old male recently diagnosed with arterial hypertension and a prostatic nodule. The patient arrived at the Emergency Department with a 10-day history of pain and edema in the left lower extremity, progressive abdominal pain and minimal effort dyspnea. The physical examination revealed tachycardia, a distended bladder and an increased volume of the left lower extremity. The blood test showed acute kidney injury (creatinine 7.6<span class="elsevierStyleHsp" style=""></span>mg/dl), hyperkalemia (7.1<span class="elsevierStyleHsp" style=""></span>mmol/l), metabolic acidosis (pH 7.31), D-Dimer value of 42,000<span class="elsevierStyleHsp" style=""></span>ng/ml and C-reactive protein 24.2<span class="elsevierStyleHsp" style=""></span>mg/dl. ECG revealed peaked T waves. Initially, a urinary catheter was placed and potassium levels were controlled with medical treatment. Afterwards, a compression ultrasound showed bilateral deep vein thrombosis (DVT) in left iliac vein and both saphenous, femoral and popliteal veins, so enoxaparin was started. A body CT disclosed a bilateral pulmonary embolism and an infiltrative retroperitoneal mass causing venous compression and hydronephrosis (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A, B). A prostatic biopsy was negative for malignancy. Serum IgG levels were 1180<span class="elsevierStyleHsp" style=""></span>mg/dl (normal range: 670–1650<span class="elsevierStyleHsp" style=""></span>mg/dl), and a retroperitoneal biopsy was not performed because of the surgical risk. As an outpatient, a PET scan performed one week after hospital discharge, excluded malignancy and inflammatory activity with a maximum SUV of 2.2. Steroid therapy was started with prednisone at a dose of 50<span class="elsevierStyleHsp" style=""></span>mg/day for one month, followed by tapering doses. A CT scan was repeated three months later, without evidence of retroperitoneal fibrosis. All laboratory values were also normalized, including obstructive renal failure. The patient received anticoagulation with acenocoumarol for 6 months.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Idiopathic retroperitoneal fibrosis (IRF), also called Ormond's disease, is a rare fibro-inflammatory process in the retroperitoneum<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> with an incidence of 1.3:100,000 a year and a 2–3:1 male/female ratio.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2,3</span></a> Patophysiology remains unclear, but IRF and IgG4 related disease (IgG4-RD) share a common autoimmune etiology that may suggest a certain disease spectrum.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Scheel et al. defined three diagnostic criteria for IRF: (1) the tissue proven on CT or MRI; (2) the absence of malignancy and (3) the absence of a systemic multifocal fibrotic process, consistent with IgG4-RD.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">4,5</span></a> IRF poses a clinical challenge due to its highly variable and unspecific clinical presentation, leading to significant diagnostic delay. The fibro-inflammatory changes cause local compression, but no invasion, of the structures in the retroperitoneal space causing renal or vascular complications, i.e. renal colics, hydronephrosis and the complications that our patient presented: newly developed hypertension (frequently of renovascular etiology) and deep venous thrombosis. Laboratory results are usually unspecific, and, among them, inflammatory anemia and elevated serum ferritin are frequent and erythrocyte sedimentation rate and C-reactive protein are elevated in 50–100% of cases, but they do not correlate with the prognosis of the disease. Creatinine and urea levels are elevated in half of the patients due to the ureteral obstruction. Unspecific antibodies are often found. If serum IgG4 is elevated, a retroperitoneal biopsy should be considered to rule out IgG4-RD. Imaging by CT scan and MRI scan are commonly used for diagnosis and evaluation of the fibrosis degree. FDG-PET-CT is useful to quantify the metabolic activity. It could also be used to detect other inflammatory locations in IgG4-RD. The performance of an open or CT-guided retroperitoneal biopsy remains controversial, but it is still the gold standard to diagnose retroperitoneal masses. Alternatively, a classical clinical-radiological presentations treated empirically may be sufficient for the diagnosis. The first-line therapy is a 0.5–1<span class="elsevierStyleHsp" style=""></span>mg/kg/day dose of prednisolone followed by tapering doses. There is no consensus on the duration of the treatment, with variable regimens described from 1 to 3 years.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2,4</span></a> Biopsy is strongly advised when the first-line therapy fails.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> Clinical improvement is usually described after 2–6 weeks. Our patient also reached a quick radiological improvement within three months.</p><p id="par0015" class="elsevierStylePara elsevierViewall">In conclusion, IRF is a rare disease whose pathophysiology is still unclear, but an autoimmune etiology has been recently proposed. Diagnosis is based in imaging and retroperitoneal biopsy when required. Corticosteroids are the main treatment. A long-term follow up is required due to the high relapse rates. In our case, the retroperitoneal mass and the inflammatory state were considered the provoking factors for the development of bilateral DVT and therefore, anticoagulation treatment was withdrawn 6 months after the episode.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "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" => 2232 "Ancho" => 1634 "Tamanyo" => 228779 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Bilateral femoral deep vein thrombosis. (B) Retroperitoneal fibrosis. 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"fecha" => "2013" "volumen" => "92" "paginaInicial" => "82" "paginaFinal" => "91" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23429355" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000015700000002/v1_202107200643/S2387020621003119/v1_202107200643/en/main.assets" "Apartado" => array:4 [ "identificador" => "43311" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Scientific letters" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000015700000002/v1_202107200643/S2387020621003119/v1_202107200643/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020621003119?idApp=UINPBA00004N" ]
Journal Information
Vol. 157. Issue 2.
Pages 87-88 (July 2021)
Vol. 157. Issue 2.
Pages 87-88 (July 2021)
Scientific letter
Idiopathic retroperitoneal fibrosis (Ormond's disease) as a transient risk factor for venous thromboembolism
Fibrosis retroperitoneal idiopática (enferemdad de Ormond) como factor de riesgo transitorio de tromboembolia venosa
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