Case Report
Tjalma Syndrome (Pseudo-pseudo Meigs’) as Initial Manifestation of Juvenile-onset Systemic Lupus Erythematosus
Síndrome de Tjalma (pseudo-pseudo Meigs) como manifestación inicial de lupus eritematoso sistémico de inicio juvenil
Alfonso Ragnar Torres Jiméneza,
, Eunice Solís-Vallejoa, Adriana Ivonne Céspedes-Cruza, Maritza Zeferino Cruza, Edna Zoraida Rojas-Curielb, Berenice Sánchez-Jarac
Corresponding author
a Reumatología Pediátrica, Hospital General, Centro Médico Nacional La Raza, IMSS, Mexico City, Mexico
b Cirugía Oncológica Pediátrica, Hospital General, Centro Médico Nacional La Raza, IMSS, Mexico City, Mexico
c Hematología Pediátrica, Hospital General, Centro Médico Nacional La Raza, IMSS, Mexico City, Mexico
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true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figura 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1112 "Ancho" => 1633 "Tamanyo" => 203476 ] ] "descripcion" => array:1 [ "es" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Laparotomía exploradora que muestra ovario izquierdo de características normales (*), sin características macroscópicas de tumor, asas intestinales edematosas y peritoneo con fragilidad capilar (flecha).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Alfonso Ragnar Torres Jiménez, Eunice Solís-Vallejo, Adriana Ivonne Céspedes-Cruz, Maritza Zeferino Cruz, Edna Zoraida Rojas-Curiel, Berenice Sánchez-Jara" "autores" => array:6 [ 0 => array:2 [ "nombre" => "Alfonso Ragnar" "apellidos" => "Torres Jiménez" ] 1 => array:2 [ "nombre" => "Eunice" 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"entidad" => "Cirugía Oncológica Pediátrica, Hospital General, Centro Médico Nacional La Raza, IMSS, Mexico City, Mexico" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Hematología Pediátrica, Hospital General, Centro Médico Nacional La Raza, IMSS, Mexico City, Mexico" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome de Tjalma (pseudo-pseudo Meigs) como manifestación inicial de lupus eritematoso sistémico de inicio juvenil" ] ] "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" => 1157 "Ancho" => 1633 "Tamanyo" => 219953 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Massive ascites in our patient.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">The presence of ovarian tumor (primary fibroma, thecoma, Brenner tumor), pleural effusion and ascites is referred to as Meigs’ syndrome. When it is associated with tumors other than those mentioned above, it is known as pseudo-Meigs’ syndrome. Tjalma described pseudo-pseudo-Meigs’ syndrome as a clinical condition that is accompanied by pleural effusion, ascites and elevated cancer antigen (CA) 125, with no associated benign or malignant ovarian tumor, in patients with systemic lupus erythematosus (SLE).<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">1–4</span></a> We present the case of a 14-year-old girl with Tjalma syndrome, to date the only report of this condition in a pediatric patient.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case Report</span><p id="par0010" class="elsevierStylePara elsevierViewall">The patient was a 14-year-old girl who had a history of 2 weeks of edema in her lower limbs, photosensitivity, oral ulcers, cyanosis, weight loss and fever, with a diagnosis of pyelonephritis. The symptoms persisted and she came to our hospital. We found that she had arthritis, ascites, pallor and anemia of chronic disease. Her platelet count was 340,000/μL, the lymphocyte count was 870/μL, creatinine was 1.66<span class="elsevierStyleHsp" style=""></span>mg/dL, urea was 164<span class="elsevierStyleHsp" style=""></span>mg/dL, blood urea nitrogen was 76<span class="elsevierStyleHsp" style=""></span>mg/dL, albumin was 2.14<span class="elsevierStyleHsp" style=""></span>g/dL, C3 was 53<span class="elsevierStyleHsp" style=""></span>mg/dL, C4 was 3.9<span class="elsevierStyleHsp" style=""></span>mg/dL, antinuclear antibodies (homogeneous pattern) 1:160, anti-DNA<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>240, urinalysis showed red blood cells in the urine and casts; SLE was diagnosed with a Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) of 26. Treatment was initiated with methylprednisolone pulses (5 doses of 30<span class="elsevierStyleHsp" style=""></span>mg/kg body weight [bw]/day), and she developed respiratory distress and left pleural effusion. She continued to have fever, anemia, acute kidney injury, thrombocytopenia, hypertension, neuropsychiatric disorders, peripheral blood smear with schistocytes, left diaphragmatic hypomotility according to thoracic ultrasound (US) and pulmonary function tests revealed a severe restrictive pattern, with a diagnosis of shrinking lung syndrome and thrombotic thrombocytopenic purpura (TTP). The treatment was plasma infusion at 30<span class="elsevierStyleHsp" style=""></span>mL/kg bw/day, subcutaneous cyclophosphamide (1<span class="elsevierStyleHsp" style=""></span>g/m<span class="elsevierStyleSup">2</span>/month) and rituximab (total dose of 1.5<span class="elsevierStyleHsp" style=""></span>g, divided into 2 doses at an interval of 14 days). Despite treatment, ascites persisted and the patient underwent paracentesis. She had acute pulmonary edema, with passage to phase III of ventilation, and cardiorespiratory arrest and acute kidney injury managed with hemodialysis. She was extubated a week later; acute kidney injury, ascites and TTP persisted. She had 5 sessions of plasmapheresis, and TTP remitted. Given refractory ascites (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>), persistent pleural effusion and US showing an increase in volume of the structures that support left ovary, we suspected Meigs’ syndrome, with a result of CA-125 elevated by 59<span class="elsevierStyleHsp" style=""></span>IU/mL. Ovarian tumor was ruled out by laparotomy (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>), and we concluded that the diagnosis was Tjalma syndrome. To treat the refractory ascites, we decided to utilize an intraperitoneal steroid (dexamethasone with 2 monthly doses of 12<span class="elsevierStyleHsp" style=""></span>mg). Hemodialysis was discontinued 3 months later. The patient completed the treatment with 12 monthly cycles of cyclophosphamide and gradual tapering of the steroid. She is currently taking 2<span class="elsevierStyleHsp" style=""></span>g/day of mycophenolate mofetil and 5<span class="elsevierStyleHsp" style=""></span>mg/day of prednisone. In the latest determination of CA-125 the level was 23<span class="elsevierStyleHsp" style=""></span>IU/mL (normal value: 0–35<span class="elsevierStyleHsp" style=""></span>IU/mL).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0015" class="elsevierStylePara elsevierViewall">Tumor markers are utilized to detect recurrences, for the evaluation of the response to treatment or for diagnosis. CA-125 is a glycoprotein initially described by Bast in 1981. It reacted with epithelial tumor cells of ovarian cancer. Its use as a diagnostic tool is limited, since it is also expressed on the surface of cells derived from coelomic epithelium (Fallopian tubes, endometrium, endocervix, ovary, pleura, peritoneum and pericardium), lung, breast, prostate and conjunctiva, and the levels of certain cytokines like vascular endothelial growth factor (VEGF) and fibroblast growth factor increase on interacting with mesothelial cells. Their elevation is associated with other conditions, like early pregnancy, ascites, menstruation, nephrotic syndrome, endometriosis, leiomyomas, congestive heart failure, cirrhosis, SLE, rheumatoid arthritis and tuberculosis.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">2,5–7</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Elevated CA-125 levels in SLE are probably secondary to the activation of mesothelial cells. Cytokines like interleukin (IL) 1 and interferon (IFN) γ increase the expression of CA-125 in human peritoneal mesothelial cells.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">2,4</span></a> The VEGF levels are elevated in patients with nephrotic syndrome, a fact that may explain the increase in CA-125.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">1,8</span></a><a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> shows the features of reported cases of Tjalma syndrome. Moncayo et al. measured the levels of CA-125 in 37 SLE patients and found elevated levels in patients with active disease.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">6</span></a> Szekanecz observed CA-125 elevation with disease activity, but there was no renal involvement.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">6</span></a> This contrasts with the findings of Miret et al.,<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">6</span></a> who evaluated serum CA-125 levels in 59 patients with SLE and their relationship with activity. They found an elevation in 3 of them, 2 with disease activity and 1 with inactive disease, but no correlation with activity, although there was a link to nephrotic syndrome. Yang et al.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">6</span></a> conducted a study involving 156 SLE patients, and observed an elevation of CA-125 in 48. Compared to patients with a normal CA-125 level, those in which it was elevated were more likely to have serositis (37.5% vs 1.9%); <span class="elsevierStyleItalic">P</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>.001), pulmonary involvement (37.5% vs 12%; <span class="elsevierStyleItalic">P</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>.001) and a high SLEDAI score <span class="elsevierStyleItalic">P</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>.007). They generated a receiver operating characteristic ROC curve, and determined the area under the curve to assess the strength of CA-125 elevation in the diagnosis of serositis due to SLE. We identified a cutoff value of 38<span class="elsevierStyleHsp" style=""></span>IU/mL (sensitivity of 85%, specificity of 75%). The determination was repeated in 15 patients when the serositis resolved, and the level was normal in 14 and remained high in 1.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">6</span></a> Our patient had an SLEDAI score of 26, as well as uncommon and severe complications, that warranted aggressive therapy with cyclophosphamide, rituximab and plasmapheresis. For refractory peritonitis, we utilized an intraperitoneal steroid (dexamethasone at 12<span class="elsevierStyleHsp" style=""></span>mg monthly in 2 doses), as reported by Yemil Atisha (Mexican Rheumatology Congress, 2015). In our case, we attributed CA-125 elevation to the presence of high disease activity, refractory serositis, congestive heart failure and nephrotic syndrome.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Tjalma syndrome is a rare association that had not been reported until now in the pediatric age group. Although CA-125 elevation requires ruling out cancer, it is also necessary to assess other causes of the elevation of CA-125 like those mentioned above.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Ethical Disclosures</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Protection of human and animal subjects</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors declare that the procedures followed were in accordance with the regulations of the relevant clinical research ethics committee and with those of the Code of Ethics of the World Medical Association (Declaration of Helsinki).</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Confidentiality of data</span><p id="par0035" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work center on the publication of patient data.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Right to privacy and informed consent</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors have obtained the written informed consent of the patients or subjects mentioned in the article. The corresponding author is in possession of this document.</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conflicts of Interest</span><p id="par0045" class="elsevierStylePara elsevierViewall">The authors declare they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres1258201" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1165536" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1258202" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1165535" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Case Report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:3 [ "identificador" => "sec0020" "titulo" => "Ethical Disclosures" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0025" "titulo" => "Protection of human and animal subjects" ] 1 => array:2 [ "identificador" => "sec0030" "titulo" => "Confidentiality of data" ] 2 => array:2 [ "identificador" => "sec0035" "titulo" => "Right to privacy and informed consent" ] ] ] 8 => array:2 [ "identificador" => "sec0040" "titulo" => "Conflicts of Interest" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2016-11-03" "fechaAceptado" => "2017-04-05" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1165536" "palabras" => array:4 [ 0 => "Tjalma syndrome" 1 => "Pseudo-pseudo Meigs’" 2 => "Systemic lupus erythematosus" 3 => "CA-125" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1165535" "palabras" => array:4 [ 0 => "Síndrome de Tjalma" 1 => "Pseudo-pseudo Meigs" 2 => "Lupus eritematoso sistémico" 3 => "CA-125" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Tjalma syndrome or pseudo-pseudo Meigs’ syndrome is a clinical condition characterized by pleural effusion, ascites and elevated CA-125 with no associated benign or malignant ovarian tumor in a patient with systemic lupus erythematosus (SLE). Tjalma described the first case of a patient with SLE, pleural effusion, ascites and elevated CA-125. We report the first case in a 14-year old patient who presented with ascites and pleural effusion refractory to treatment and elevated CA-125, in the absence of an ovarian tumor, that warranted aggressive management.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El síndrome de Tjalma o pseudo-pseudo Meigs es una entidad clínica que se presenta con derrame pleural, ascitis y elevación de CA-125 sin asociación a tumor ovárico benigno o maligno en un paciente con lupus eritematoso sistémico (LES). Tjalma describió el primer caso de un paciente con LES, ascitis, derrame pleural y elevación de CA-125. Presentamos el primer caso en una paciente pediátrica de 14 años, que se presentó con ascitis y derrame pleural refractarios a tratamiento con elevación de CA-125, sin encontrar tumor ovárico, que ameritó manejo agresivo.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Torres Jiménez AR, Solís-Vallejo E, Céspedes-Cruz AI, Zeferino Cruz M, Rojas-Curiel EZ, Sánchez-Jara B. Síndrome de Tjalma (pseudo-pseudo Meigs) como manifestación inicial de lupus eritematoso sistémico de inicio juvenil. Reumatol Clin. 2019;15:e41–e43.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1157 "Ancho" => 1633 "Tamanyo" => 219953 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Massive ascites in our patient.</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" => 1112 "Ancho" => 1633 "Tamanyo" => 203476 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Exploratory laparotomy showing left ovary with normal characteristics (*), and no macroscopic features indicative of tumor, edematous intestinal loops and peritoneum with capillary fragility (arrow).</p>" ] ] 2 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">ANA, antinuclear antibodies; bw, body weight; CA, cancer antigen; CVD, cerebrovascular disease; DNA, deoxyribonucleic acid.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Reference \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Age (years) \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Ascites/pleural effusion \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Manifestations of lupus \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">CA-125 \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Ovarian tumor \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Treatment \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Bes et al.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">1</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">42 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">+/+ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">ThrombocytopeniaANA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>anti-DNA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>decreased C3 and C4 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">91<span class="elsevierStyleHsp" style=""></span>IU/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">3 pulses of methylprednisolone1/mg/kg bw/dayMonthly cyclophosphamide pulses for 6 months \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Bes y Soy<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">8</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">47 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">+/+ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">ArthritisPericardial effusionLeukopenia, lymphopeniaANA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>anti-DNA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>complement consumption \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">233<span class="elsevierStyleHsp" style=""></span>IU/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Oral steroidsHydroxychloroquine \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Lee et al.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">2</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">29 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">+/+ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">PancytopeniaCutaneousANA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>anti-DNA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>complement consumptionPericardial effusion \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">345<span class="elsevierStyleHsp" style=""></span>IU/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Steroid pulsesHydroxychloroquine \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Lee et al.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">2</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">54 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">+/+ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">ANA+, anti-DNA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>nephritisArthritisThrombocytopeniaComplement consumption \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">344<span class="elsevierStyleHsp" style=""></span>IU/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">MethylprednisoloneCyclophosphamide \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Ural et al.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">5</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">38 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">+/+ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">CutaneousAnti-DNA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>anti-Ro<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>C3 consumption \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1229<span class="elsevierStyleHsp" style=""></span>IU/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">SteroidsHydroxychloroquine \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Tjalma<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">3</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">38 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">+/+ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">ThrombocytopeniaSjögren's syndromeAntiphospholipid antibodies++CVD \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">887<span class="elsevierStyleHsp" style=""></span>IU/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">SteroidsAzathioprine \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Dalvi et al.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">4</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">56 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">+/+ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">ANA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>anti-DNA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>leukopenia arthritisComplement consumptionPericardial effusion \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">70<span class="elsevierStyleHsp" style=""></span>IU/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Prednisone 1<span class="elsevierStyleHsp" style=""></span>mg/kg bw/dayMycophenolate mofetil \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Our patient \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">14 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">+/+ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">ANA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>anti-DNA<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>thrombotic thrombocytopenic purpuraShrinking lung syndromeNephritisArthritisComplement consumptionPericardial effusion \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">59<span class="elsevierStyleHsp" style=""></span>IU/mL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Steroid pulsesOral prednisone 1<span class="elsevierStyleHsp" style=""></span>mg/kg bw/dayCyclophosphamideRituximabPlasmapheresisIntraperitoneal steroid \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2151620.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Characteristics of Tjalma Syndrome Patients.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:8 [ 0 => array:3 [ "identificador" => "bib0045" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "A rare form of SLE: pseudo-pseudo Meigs síndrome and hydrocephalus" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "C. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 11 | 6 | 17 |
| 2024 October | 269 | 47 | 316 |
| 2024 September | 298 | 49 | 347 |
| 2024 August | 305 | 96 | 401 |
| 2024 July | 295 | 65 | 360 |
| 2024 June | 254 | 50 | 304 |
| 2024 May | 216 | 40 | 256 |
| 2024 April | 212 | 53 | 265 |
| 2024 March | 273 | 50 | 323 |
| 2024 February | 227 | 61 | 288 |
| 2024 January | 259 | 34 | 293 |
| 2023 December | 156 | 36 | 192 |
| 2023 November | 190 | 60 | 250 |
| 2023 October | 280 | 47 | 327 |
| 2023 September | 212 | 42 | 254 |
| 2023 August | 160 | 28 | 188 |
| 2023 July | 154 | 47 | 201 |
| 2023 June | 127 | 42 | 169 |
| 2023 May | 156 | 31 | 187 |
| 2023 April | 113 | 18 | 131 |
| 2023 March | 211 | 40 | 251 |
| 2023 February | 261 | 59 | 320 |
| 2023 January | 221 | 37 | 258 |
| 2022 December | 231 | 46 | 277 |
| 2022 November | 193 | 65 | 258 |
| 2022 October | 226 | 38 | 264 |
| 2022 September | 227 | 55 | 282 |
| 2022 August | 168 | 56 | 224 |
| 2022 July | 155 | 38 | 193 |
| 2022 June | 106 | 33 | 139 |
| 2022 May | 187 | 54 | 241 |
| 2022 April | 211 | 68 | 279 |
| 2022 March | 196 | 69 | 265 |
| 2022 February | 256 | 66 | 322 |
| 2022 January | 220 | 53 | 273 |
| 2021 December | 172 | 70 | 242 |
| 2021 November | 130 | 39 | 169 |
| 2021 October | 136 | 59 | 195 |
| 2021 September | 107 | 41 | 148 |
| 2021 August | 94 | 35 | 129 |
| 2021 July | 71 | 33 | 104 |
| 2021 June | 110 | 41 | 151 |
| 2021 May | 94 | 51 | 145 |
| 2021 April | 244 | 134 | 378 |
| 2021 March | 97 | 29 | 126 |
| 2021 February | 103 | 23 | 126 |
| 2021 January | 70 | 29 | 99 |
| 2020 December | 82 | 15 | 97 |
| 2020 November | 62 | 28 | 90 |
| 2020 October | 42 | 24 | 66 |
| 2020 September | 186 | 33 | 219 |
| 2020 August | 31 | 25 | 56 |
| 2020 July | 33 | 19 | 52 |
| 2020 June | 71 | 24 | 95 |
| 2020 May | 34 | 17 | 51 |
| 2020 April | 20 | 13 | 33 |
| 2020 March | 25 | 6 | 31 |
| 2018 December | 4 | 2 | 6 |
| 2018 November | 0 | 1 | 1 |
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