ABSTRACT
Introducción: La corticoterapia continúa siendo la piedra angular en el tratamiento del síndrome nefrótico. El manejo de los pacientes que desarrollan dependencia a esteroides es complejo, implicando distintas pautas de fármacos inmunosupresores. El rituximab, anticuerpo monoclonal anti-CD20, parece tener efectos beneficiosos en pacientes con síndrome nefrótico córtico-dependiente de difícil manejo clínico, si bien aún no está bien definido su papel en esta entidad. Con el fin de aportar información útil sobre el papel del rituximab en esta patología, presentamos nuestra experiencia personal en pacientes pediátricos tratados con este fármaco en los últimos años. Materiales y métodos: Estudio retrospectivo en pacientes con síndrome nefrótico idiopático córtico-dependiente controlados en la Sección de Nefrología Pediátrica de un hospital terciario español, y que habían recibido, al menos, un ciclo de tratamiento con rituximab durante cualquier momento de la evolución de la enfermedad. (AU)
Background: Corticosteroids have had a central role in the treatment of nephrotic syndrome. The management of these patients who become dependent to steroids is complex, involving different immunosuppressive drugs patterns. The monoclonal antibody anti CD20, Rituximab, is likely to have beneficial effects in cases of steroid-dependent nephrotic syndrome patients with no easy resolution, even when we cannot make a statement about the specific role in the impact. We bring our personal experience in pediatric patients treated with this medication during the last years, to provide a thorough overview and useful information about the role of Rituximab in this pathology. Methods: Retrospective study in patients with steroid-dependent idiopathic nephrotic syndrome controlled in the division of Pediatric Nephrology of a spanish tertiary hospital in those patients who had received at least one treatment cycle of Rituximab, at any moment along the evolution of the disease. (AU)
Subject(s)
Humans , Infant, Newborn , Child, Preschool , Child , Rituximab , Nephrotic Syndrome , Pediatrics , TherapeuticsABSTRACT
Abstract Posttransplant Lymphoproliferative Disorders (PTLDs) occur in 3 to 10% of adults with solid organ transplant (SOT). It has been associated with Epstein Barr Virus (EBV) infection. Differential diagnostics of PTLD from rejection or viral infection is difficult when the tumor infiltrates the graft, because the clinical and histopathological findings are similar. We report a case of patient with chronic kidney disease due to Ig M glomerulonephritis with cadaveric donor kidney transplantation who presented proteinuria and decreased glomerular filtration rate, with a solid mass at renal graft and confirmatory histology of polymorphic renal transplant lymphoproliferative disorder (PTLD), VEB positive, and CD 20 positive. The patient was treated with rituximab 375 mg / m2 weekly, four doses, followed by chemotherapy with ciclophosphamide, vincristine and doxorubicin. He didn't need radiotherapy or graft nephrectomy, with complete remission at one year of follow-up and optimal graft function.
Resumen Los desórdenes linfoproliferativos postrasplante (PTLD por sus siglas en inglés: Posttransplant Lymphoproliferative disorders) se presentan en 3 a 10% de adultos con trasplante de órgano sólido (TOS). Se ha asociado a infección por Virus Epstein Barr (VEB). Es difícil diferenciar PTLD de rechazo o infección viral, porque los hallazgos clínicos e histopatológicos son muy similares. Presentamos el caso de un paciente con enfermedad renal crónica (ERC) secundaria a glomerulonefritis IgM, con trasplante renal de donante cadavérico, quien presentó proteinuria y disminución de la función renal, se le documentó una masa en el injerto renal compatible con desorden linfoproliferativo pos-trasplante renal de tipo polimórfico (PTLD), VEB positivo y CD 20 positivo. El tratamiento consistió en rituximab 375 mg/m2 semanales, cuatro dosis, se realizó control con imágenes y se adicionó el esquema CHOP (ciclofosfamida, vincristina, doxorubicina). El paciente toleró de manera adecuada la quimioterapia, no requirió radioterapia, ni trasplantectomía y después del R-CHOP la masa disminuyó de manera significativa hasta desaparecer al año de seguimiento manteniendo función óptima del injerto renal.
Subject(s)
Humans , Male , Female , Kidney Transplantation , Lymphoproliferative Disorders , Colombia , Herpesvirus 4, HumanABSTRACT
The authors review the nomenclature of vasculitides and the classification of antineutrophil cytoplasm antibody associated vasculitides and present the method of measuring disease activity (Five-factor Score, Birmingham Vasculitis Activity Score) and its role in defining therapeutical needs. They discuss the treatment algorithm of antineutrophil cytoplasm antibody associated vasculitides, present the sometimes equipotential medications used during the induction therapy followed by a maintenance regimen, and outline their usage and possible side-effects that may require medical attention. They point out the importance of plasmapheresis as an adjunctive treatment in some cases, as well as indications and possible outcome of kidney transplantation in therapy-resistant cases. Finally, they review several ongoing clinical studies, as their outcome will probably influence therapeutical opportunities of antineutrophil cytoplasm antibody associated vasculitides in the next few years.
