Neurorretinitis bilateral y nefritis lúpica membranosa: dos manifestaciones infrecuentes en el lupus juvenil / Bilateral neuroretinitis and membranous lupus nephritis: 2 infrequent manifestations in juvenile lupus

Joven de 15 años, previamente sana, se presentó con dolor abdominal, vómitos, diarrea, eritema malar, edema palpebral y en miembros inferiores, artralgias, rigidez matinal y visión borrosa bilateral. Estudios de laboratorio y por imágenes junto con la clínica permitieron realizar el diagnóstico de síndrome nefrótico secundario a lupus eritematoso sistémico. Al examen oftalmológico se constató 8/10 de visión en ambos ojos y edema de papila bilateral con estrella macular parcial, hallazgos compatibles con una neurorretinitis bilateral. La biopsia renal estableció el diagnóstico de nefritis lúpica membranosa. Se inició tratamiento inmunosupresor, con mejoría clínica gradual. Si bien el lupus eritematoso sistémico con nefritis lúpica membranosa y neurorretinitis es una asociación muy infrecuente, frente a un paciente con neurorretinitis bilateral debemos considerar el lupus eritematoso sistémico dentro de los diagnósticos diferenciales (AU)

Fifteen-year-old female patient, previously healthy, referred to our center for presenting abdominal pain, vomiting, diarrhea, malar erythema, palpebral and lower limb edema, arthralgia, morning stiffness and bilateral blurred vision. Laboratory and imaging studies together with the clinic allowed the diagnosis of nephrotic syndrome secondary to systemic lupus erythematosus. Ophthalmology examination revealed a visual acuity of 8/10 in both eyes and bilateral disc edema with partial macular star, findings compatible with bilateral neuroretinitis. Renal biopsy established the diagnosis of membranous lupus nephritis. Immunosuppressive treatment was started, obtaining gradual clinical improvement. Although systemic lupus erythematosus with membranous lupus nephritis and neuroretinitis is a very infrequent association, when faced with a patient with bilateral neuroretinitis, we must consider systemic lupus erythematosus within the differential diagnoses (AU)

Effects of everolimus plus minimized tacrolimus on kidney function in liver transplantation: REDUCE, a prospective, randomized controlled study

Background and aim: reduction in calcineurin inhibitor levels is considered crucial to decrease the incidence of kidney dysfunction in liver transplant (LT) recipients. The aim of this study was to evaluate the safety and impact of everolimus plus reduced tacrolimus (EVR + rTAC) vs. mycophenolate mofetil plus tacrolimus (MMF + TAC) on kidney function in LT recipients from Spain.Methods: the REDUCE study was a 52-week, multicenter, randomized, controlled, open-label, phase 3b study in de novo LT recipients. Eligible patients were randomized (1:1) 28 days post-transplantation to receive EVR + rTAC (TAC levels ≤ 5 ng/mL) or to continue with MMF + TAC (TAC levels = 6-10 ng/mL). Mean estimated glomerular filtration rate (eGFR), clinical benefit in renal function, and safety were evaluated.Results: in the EVR + rTAC group (n = 105), eGFR increased from randomization to week 52 (82.2 [28.5] mL/min/1.73 m2 to 86.1 [27.9] mL/min/1.73 m2) whereas it decreased in the MMF + TAC (n = 106) group (88.4 [34.3] mL/min/1.73 m2 to 83.2 [25.2] mL/min/1.73 m2), with significant (p < 0.05) differences in eGFR throughout the study. However, both groups had a similar clinical benefit regarding renal function (improvement in 18.6 % vs. 19.1 %, and stabilization in 81.4 % vs. 80.9 % of patients in the EVR + rTAC vs. MMF + TAC groups, respectively). There were no significant differences in the incidence of acute rejection (5.7 % vs. 3.8 %), deaths (5.7 % vs. 2.8 %), and serious adverse events (51.9 % vs. 44.0 %) between the 2 groups.Conclusion: EVR + rTAC allows a safe reduction in tacrolimus exposure in de novo liver transplant recipients, with a significant improvement in eGFR but without significant differences in renal clinical benefit 1 year after liver transplantation. (AU)

Avance en el tratamiento de la mastitis granulomatosa idiopática: utilidad de micofenolato de mofetilo / Breakthrough in treatment of the idiopathic granulomatous mastitis: Use of mycophenolate mofetil

Objetivos: La mastitis granulomatosa idiopática (MGI) es una patología inflamatoria inusual de la mama, con un curso insidioso, incluso recurrente que afecta a mujeres premenopáusicas. Es muy importante su reconocimiento y diagnóstico precoz con el fin de evitar tratamientos ineficaces, demostrando en nuestra casuística la eficacia del tratamiento micofenolato mofetilo (MFM).Materiales y método: Realizamos un estudio con los ocho casos de MGI en nuestro centro desde el año 2012 que fueron refractarios a los tratamientos usuales (antibióticos, limpieza quirúrgica, esteroides, metotrexato), valorando la evolución con el tratamiento a base de MFM.Resultados: La respuesta fue buena tanto radiológica como clínicamente al uso de MFM, con desaparición de las lesiones tras el uso de MFM sin que fueran necesarios tratamientos más invasivos ni hubiera tasas de recidiva significativa.Conclusiones:El uso de MFM como tratamiento parece exitoso tanto en la recurrencia como la desaparición de la enfermedad. Dado su diagnóstico diferencial y su curso benigno, el papel de las técnicas de imagen para reconocer esta enfermedad lo más pronto posible puede llevar a un mejor resultado clínico y evitar sobretratamiento innecesario. (AU)

Objective: Idiopathic granulomatous mastitis (IGM) is a rare benign inflammatory disease of the breast with persistent course, even recurrent, that frequently affects parous premenopausal women. It can mimic the appearance of breast cancer, so its diagnosis is of great help to avoid irreversible surgical endeavours. Although multiple treatments have been tried for its management, none have been truly successful and no consensus exists as which the best therapeutic option is.Methods: We conducted a study with the eight cases of IGM in our institution since 2012 that were refractory to the used treatments for IGM (antibiotics, steroids, methotrexate or observation) seeing their evolution after the use of mycophenolate mofetil.Results: They responded well radiologically as well as clinically to the use of mycophenolate mofetil, not needing more invasive course of treatment.Conclusión: IGM is a rare inflammatory condition of the breast. The use of MMF as treatment is successful in the recurrence and disappearance of this disease. Given its differential diagnosis and its benign course, the role of multimodal imaging in recognizing this entity as soon as possible can lead to an improved patient outcome and help preventing any unnecessary overtreatment. (AU)

Vogt-Koyanagi-Harada: tratamiento de la recurrencia tras la administración de 3 bolos intravenosos de 1g de corticoides y micofenolato de mofetilo / Vogt-Koyanagi-Harada: treatment of recurrence after administration of 3 intravenous bolus of 1g of corticosteroids and mycophenolate mofetil

Presentamos el caso de una mujer hondureña de 27 años sin antecedentes médicos de interés que presentó múltiples áreas de desprendimientos de retina (DR) exudativos y una mejor agudeza visual corregida de 1,3logMAR en ambos ojos. Fue diagnosticada de síndrome de Vogt-Koyanagi-Harada incompleto y tratada con prednisolona intravenosa (1g/24h) durante 3 días, tal y como recomienda la evidencia publicada, junto con micofenolato de mofetilo (2g/24h). Durante el descenso paulatino de corticoesteroides, los DR recidivaron, por lo que se reintrodujeron las megadosis de 1g/24h de corticoesteroides intravenosos durante 6 días hasta la resolución completa de los DR y se añadieron ciclosporina (100mg/24h), triamcinolona subtenoniana (40mg/mL) e inyecciones intravítreas mensuales de ranibizumab en ambos ojos, con un adecuado control de la inflamación coroidea que se tradujo en la remisión de los síntomas y signos (AU)

The case is presented on a young Honduran female with no medical history of note, who presented with multiple areas of exudative retinal detachment (RD), and a best-corrected visual acuity of 1.3logMAR in both eyes. She was diagnosed with incomplete Vogt-Koyanagi-Harada syndrome, and treated early with a combination of intravenous therapy with 1g of prednisolone per day for 3 days, as recommended by published evidence, as well as mycophenolate mofetil (2g per day). During the corticosteroids tapering, there was a recurrence of exudative retinal detachments, and megadoses of 1g of intravenous corticosteroids per day were reintroduced for 6 days until the complete resolution of the fluid of the exudative RD, and cyclosporine (100mg per day), subtenon triamcinolone (40mg/mL), and intravitreal ranibizumab once a month in the both eyes were added to the treatment, with a great control of choroidal inflammation that resulted in the remission of symptoms and signs (AU)

Evaluación de la neutropenia secundariaa micofenolato de mofetilo asociado a valganciclovir en pacientes trasplantados hepáticos / Evaluation of neutropenia secondary to mycophenolate mofetil associated with valganciclovir in liver transplant patients

Objetivo: Los fármacos inmunosupresores son necesarios para evitar oreducir el riesgo de rechazo de órganos trasplantados. La inmunosupresión generada puede dar lugar a que estos pacientes necesiten recibirantibióticos y antivíricos con los inmunosupresores para evitar el riesgo deinfecciones. Esto ha generado un incremento de neutropenia en pacientestratados conjuntamente con micofenolato de mofetilo y valganciclovir. Elobjetivo de este estudio es estimar el riesgo de neutropenia atribuible altratamiento concomitante de micofenolato de mofetilo y valganciclovir enpacientes trasplantados hepáticos.Método: Estudio de cohorte retrospectiva. Se incluyeron pacientesreceptores de hígado entre 2012 y 2017 tratados con micofenolato demofetilo o con la combinación de micofenolato de mofetilo y valganciclovir, con al menos 100 días de seguimiento postrasplante. Se excluyeronmenores de 16 años y pacientes fallecidos durante el seguimiento. Elanálisis de regresión logística binaria se utilizó para determinar la asociación del riesgo de neutropenia con el sexo, edad, diabetes, creatininabasal y al alta, y tratamiento concomitante de micofenolato de mofetilo yvalganciclovir. El riesgo relativo y los IC 95% se calcularon mediante loscoeficientes de regresión logística. (AU)

Objective: Immunosuppressive drugs are necessary to avoid or reducethe risk of rejection of transplanted organs. The immunosuppression generated may result in these patients needing antibiotics and antivirals to beprescribed to them in conjunction with their immunosuppressants to avoidthe risk of infection. This has generated an increase in neutropenia inpatients treated with mycophenolate mofetil in combination with valganciclovir. The purpose of this study is to estimate the risk of neutropeniaattributable to combination treatment of mycophenolate mofetil with valganciclovir in patients with a transplanted liver.Method: This is a retrospective cohort study. It included patients whoreceived a liver transplant between 2012 and 2017 and who were treated with mycophenolate mofetil or with a combination of mycophenolate mofetil and valganciclovir. Minimum follow-up was 100 days posttransplantation. Children under 16 years of age and patients who diedduring follow-up were excluded. Binary logistic regression analysis wasused to determine the association of neutropenia with sex, age, diabetes,creatinine at baseline and at discharge, and concomitant treatment ofmycophenolate mofetil with valganciclovir. Relative risk and 95% CI werecalculated using logistic regression coefficients. (AU)

Úlceras cutáneas en paciente con dermatomiositis amiopática anti-MDA5 positivo / Cutaneous ulceration in a patient with amyophatic dermatomyositis anti-MDA5 antibody positive

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Lupus eritematoso sistémico de debut atípico: a propósito de 3 casos / Systemic lupus erythematosus of atypical onset: A presentation of 3 cases

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Enfermedad linfoproliferativa postrasplante tipo plasmocitoma sobre injerto renal / Plasmacytoma-like post-transplantation lymphoproliferative disorder in renal allograft

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Mycophenolate mofetil for induction and maintenance of remission in naïve patients with granulomatosis with polyangiitis without renal involvement / Micofenolato mofetilo para la inducción y el mantenimiento de la remisión en pacientes no tratados con granulomatosis con poliangitis

Antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides include granulomatosis with polyangiitis (GPA, previously called Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss). In this report we used mycophenolate mofetil (MMF) and steroids to induce and maintain remission in two newly diagnosed cases with c-ANCA associated GPA. The two patients' maintained remission with no disease relapses during one year follow-up

Las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA) incluyen granulomatosis con poliangeitis (GPA) anteriormente llamada de Wegener, poliangeítis microscópica (MPA) y granulomatosis eosinofílica con poliangeítis (EGPA) anteriormente llamada síndrome de Churg-Strauss. En este informe utilizamos micofenolato mofetilo (MMF) y esteroides para inducir y mantener la remisión en 2 casos recientemente diagnosticados con GPA asociado a c-ANCA. La remisión mantenida de los 2 pacientes sin enfermedad recae durante un año de duración del seguimiento

Pioderma gangrenoso asociado a quemaduras: reporte de dos casos / Pyoderma gangrenosum associated with burns: two-case report

El pioderma gangrenoso es una rara dermatosis idiopática, crónica, caracterizada por pústulas y úlceras coalescentes que aparece por lo general sobre la piel previamente traumatizada, con preferencia en extremidades inferiores y tronco. Se asocia a enfermedades sistémicas y los tratamientos quirúrgicos tienden a empeorar el cuadro clínico por el fenómeno de patergia. No hay un tratamiento de elección, pero se acepta el tratamiento inmunosupresor. Presentamos 2 casos de pioderma gangrenoso asociado a quemaduras, una entidad escasamente recogida en la literatura, con abordaje y evolución diferente

Pyoderma gangrenosum is a rare, chronic, and idiopathic dermatosis, characterized by pustules and coalescent ulcers. It usually develops on previously traumatized skin, especially on the lower extremities and trunk. It is associated with systemic diseases, and surgical treatments tend to worsen clinical signs and symptoms as a result of pathergy. There is no treatment of choice, but immunosuppressive therapy is accepted. Two cases of pyoderma gangrenosum associated with burns, an entity rarely collected in the literature, are presented, with different approaches and courses

Retinopatía autoinmune con anticuerpos antirecoverina no asociado a neoplasia: a propósito de un caso / Autoimmune retinopathy with positive anti-recoverin antibodies not associated with neoplasms: Case report

El objetivo fue presentar un caso de retinopatía autoinmune (AIR) no paraneoplásica con anticuerpos antirecoverina positivos. Una mujer de 28 años consultó por pérdida de la agudeza visual bilateral de 8 meses de evolución. El fondo de ojo presentó un patrón de coloración moteado desde el centro a la periferia, sin espículas pigmentarias. La angiografía mostró un patrón de áreas puntiformes, sin fuga de contraste. Se observó una reducción de las capas externas de la retina en la tomografía de coherencia óptica, mientras que el electrorretinograma mostró una ausencia de respuesta de los conos y los bastones en el ojo derecho, y una respuesta disminuida de los conos con ausencia de respuesta de los bastones en el ojo izquierdo. Se sospechó AIR, y se empezó tratamiento empírico con corticoides a la espera de los resultados del Western-blot, que posteriormente resultó positivo para recoverina, GAPDH, anti-alfa-enolasa y aldolasa. Mientras pudo ser tratada, la agudeza visual se mantuvo estable. Al retirarse el tratamiento, esta se redujo a amaurosis en el ojo derecho y movimiento de manos en el ojo izquierdo

The case is presented of a non-paraneoplastic autoimmune retinopathy (AIR) with positive anti-recoverin autoantibodies. A 28-year-old woman presented with a rapidly progressive bilateral visual loss of 8 months onset. Funduscopic examination revealed diffuse fine mottled atrophic changes in both eyes. Fluorescein angiographic studies showed a pattern of mottled areas of early hyperfluorescence without leakage of dye. In the ocular coherence tomography it was observed that was a loss of external layers. The electroretinogram showed absence of rod and cone responses in the right eye, and diminished cone response associated to absence of rod response in the left eye. AIR was suspected, and empirical corticosteroid treatment was started while waiting for Western-blot results, which was finally positive for recoverin, GAPDH, anti-alpha-enolase, and aldolase. The patient was able to be treated, and her visual acuity remained stable, but as soon as it was suspended, vision was completely lost in the right eye and reduced to hand movement in the left eye

Hepatitis aguda por micofenolato de sodio: ¿el segundo caso? / Mycophenolate sodium-induced hepatotoxicity: The second report?

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Atrofia vellositaria duodenal con serologías negativas inducida por micofenolato de mofetilo: no todo es celiaquía / Duodenal villous atrophy with a negative serology induced by mycophenolate mofetil: not everything is celiac disease

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Mycophenolate mofetil-induced mouth ulcers in a kidney transplant patient: Case report and literature review / Úlceras orales secundarias a micofenolato mofetilo en un paciente trasplantado renal: caso clínico y revisión de la literatura

Mouth ulcers are a cutaneous complication that can often affect kidney transplant patients, mostly due to the effect of immunosuppressive treatment. Even so, before asserting that said complication is indeed secondary to drugs, it is very important to establish a differential diagnosis with other mouth ulcer causes, such as systemic diseases or viral infections, which are also common in these patients

Las úlceras orales son una de las complicaciones cutáneas que pueden afectar con frecuencia a los pacientes trasplantados renales, debido muchas veces al efecto del tratamiento inmunosupresor. Aun así, es importante, antes de asegurar que dicha complicación es secundaria a los fármacos, establecer el diagnóstico diferencial con otras causas de úlceras orales como pueden ser enfermedades sistémicas o infecciones virales, también frecuentes en este tipo de pacientes

Isolated oral mucosa leishmaniasis: An unusual manifestation / Leishmaniosis con afectación de la cavidad oral. Una localización extremadamente rara

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Podocitopatía como inicio de un lupus eritematoso sistémico / Podocytopathy as debut of a systemic lupus erythematosus

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Increase of natural killer cells in children with liver transplantation-acquired food allergy

BACKGROUND: Transplantation-acquired food allergies (TAFA) are frequently reported and considered to be caused by immunosuppressive therapy. The aim of this study was to investigate the allergic and immunologic responses in children who had liver or kidney transplantations. METHODS: Twelve children receiving liver transplantations and 10 children receiving kidney transplantations were investigated. All children underwent the allergy work-up and in most of them, lymphocyte screening and serum cytokine measurements were also performed. RESULTS: TAFA were found in 7/12 (58%) children with liver transplantations and in none of the 10 children with kidney transplantations. The mean age at transplantation was significantly lower in children who underwent liver transplantations (p < 0.001). The immunosuppressive therapy administered to children with liver transplantation was tacrolimus in 11 patients and cyclosporine in one patient, while all 10 children with kidney transplantation received tacrolimus plus mycophenolate. The most common antigenic food was egg. The natural killer (NK) cell numbers were significantly higher in liver-transplant children than in kidney-transplant children. No significant differences were found in the serum cytokine levels. CONCLUSIONS: This study confirms that liver-transplant children treated with tacrolimus alone have a higher risk of developing TAFA than kidney-transplant children treated with tacrolimus plus mycophenolate. NK cells might be involved in this difference

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