Bibliographic Details
| Title: |
Eficacia de iptacopán en glomerulonefritis membranoproliferativa por inmunocomplejos idiopática refractaria al tratamiento convencional. |
| Alternate Title: |
Efficacy of Iptacopan in Idiopathic Immune Complex-Mediated Membranoproliferative Glomerulonephritis Refractory to Conventional Treatment. |
| Authors: |
López Hidalgo, Raquel1 rlopezh@senefro.org, Caba Molina, Mercedes2, Ruiz Escolano, Francisco Javier1, Murga, Elena Delgado Sánchez de1, Fernández Quirós, Ma Teresa1, Espigares Huete, Ma José1 |
| Source: |
Nefrologia. Feb2026, Vol. 46 Issue 2, p1-6. 6p. |
| Subjects: |
GLOMERULONEPHRITIS, IMMUNE complexes, SYMPTOMS, THERAPEUTICS, COMPLEMENT (Immunology), COMPLEMENT inhibition, KIDNEY failure, NEPHRITIS |
| Abstract (English): |
Membranoproliferative glomerulonephritis due to immune complexes (MPGN-IC) idiopathic is a diagnosis of exclusion, made after ruling out the most common etiological processes associated with this pattern of glomerular injury (infectious, autoimmune diseases, gammopathies, among others). Idiopathic MPGN-IC shares with C3 glomerulopathy the activation of the alternative complement pathway, often evidenced by decreased serum C3 levels. Currently, there is no specific treatment for this type of glomerulonephritis; however, various immunosuppressive agents (such as prednisone, mycophenolate, cyclophosphamide, rituximab) have been used depending on the severity of the case. We present a clinical case diagnosed with idiopathic MPGN-IC presenting as an aggressive nephritic-nephrotic syndrome with rapidly progressive renal deterioration unresponsive to conventional therapy, requiring hemodialysis. Complete remission was achieved after initiating treatment with iptacopan, an oral inhibitor of factor B of the alternative complement pathway, and we demonstrate the patient's evolution over 12 months of treatment. [ABSTRACT FROM AUTHOR] |
| Abstract (Spanish): |
La glomerulonefritis membranoproliferativa por inmunocomplejos (GNMP-IC) idiopática es un diagnóstico de exclusión una vez descartados los procesos etiológicos más frecuentes asociados a este patrón de daño glomerular (infecciosos, enfermedades autoinmunes, gammapatías entre los más frecuentes). La GNMP-IC idiopática podría compartir con la glomerulopatía C3 la activación de la vía alternativa del complemento mostrando en muchos casos un descenso de los valores del C3 en suero. No existe en el momento actual un tratamiento específico para esta glomerulonefritis, aunque se han utilizado diversos fármacos inmunosupresores (prednisona, micofenolato, ciclofosfamida, rituximab) dependiendo la severidad del caso. Presentamos un caso clínico con diagnóstico de GNMP-IC idiopática que se presenta como síndrome nefrítico-nefrótico de curso agresivo con deterioro rápidamente progresivo de la función renal sin respuesta al tratamiento convencional con necesidad de hemodiálisis, que logra remisión completa tras iniciar tratamiento con iptacopán, un inhibidor oral del factor B de la vía alternativa del complemento y mostramos la evolución tras 12 meses de tratamiento. [ABSTRACT FROM AUTHOR] |
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| Database: |
MedicLatina |
