Eficacia de iptacopán en glomerulonefritis membranoproliferativa por inmunocomplejos idiopática refractaria al tratamiento convencional.

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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]
Copyright of Nefrologia is the property of Revista Nefrologia and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Label: Title
  Group: Ti
  Data: Eficacia de iptacopán en glomerulonefritis membranoproliferativa por inmunocomplejos idiopática refractaria al tratamiento convencional.
– Name: TitleAlt
  Label: Alternate Title
  Group: TiAlt
  Data: Efficacy of Iptacopan in Idiopathic Immune Complex-Mediated Membranoproliferative Glomerulonephritis Refractory to Conventional Treatment.
– Name: Author
  Label: Authors
  Group: Au
  Data: <searchLink fieldCode="AR" term="%22López+Hidalgo%2C+Raquel%22">López Hidalgo, Raquel</searchLink><relatesTo>1</relatesTo><i> rlopezh@senefro.org</i><br /><searchLink fieldCode="AR" term="%22Caba+Molina%2C+Mercedes%22">Caba Molina, Mercedes</searchLink><relatesTo>2</relatesTo><br /><searchLink fieldCode="AR" term="%22Ruiz+Escolano%2C+Francisco+Javier%22">Ruiz Escolano, Francisco Javier</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Murga%2C+Elena+Delgado+Sánchez+de%22">Murga, Elena Delgado Sánchez de</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Fernández+Quirós%2C+Ma+Teresa%22">Fernández Quirós, Ma Teresa</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Espigares+Huete%2C+Ma+José%22">Espigares Huete, Ma José</searchLink><relatesTo>1</relatesTo>
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  Data: <searchLink fieldCode="JN" term="%22Nefrologia%22">Nefrologia</searchLink>. Feb2026, Vol. 46 Issue 2, p1-6. 6p.
– Name: Subject
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  Data: <searchLink fieldCode="DE" term="%22GLOMERULONEPHRITIS%22">GLOMERULONEPHRITIS</searchLink><br /><searchLink fieldCode="DE" term="%22IMMUNE+complexes%22">IMMUNE complexes</searchLink><br /><searchLink fieldCode="DE" term="%22SYMPTOMS%22">SYMPTOMS</searchLink><br /><searchLink fieldCode="DE" term="%22THERAPEUTICS%22">THERAPEUTICS</searchLink><br /><searchLink fieldCode="DE" term="%22COMPLEMENT+%28Immunology%29%22">COMPLEMENT (Immunology)</searchLink><br /><searchLink fieldCode="DE" term="%22COMPLEMENT+inhibition%22">COMPLEMENT inhibition</searchLink><br /><searchLink fieldCode="DE" term="%22KIDNEY+failure%22">KIDNEY failure</searchLink><br /><searchLink fieldCode="DE" term="%22NEPHRITIS%22">NEPHRITIS</searchLink>
– Name: Abstract
  Label: Abstract (English)
  Group: Ab
  Data: 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]
– Name: Abstract
  Label: Abstract (Spanish)
  Group: Ab
  Data: 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]
– Name: AbstractSuppliedCopyright
  Label:
  Group: Ab
  Data: <i>Copyright of Nefrologia is the property of Revista Nefrologia and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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RecordInfo BibRecord:
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    Identifiers:
      – Type: doi
        Value: 10.1016/j.nefro.2025.501426
    Languages:
      – Code: spa
        Text: Spanish
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      Pagination:
        PageCount: 6
        StartPage: 1
    Subjects:
      – SubjectFull: GLOMERULONEPHRITIS
        Type: general
      – SubjectFull: IMMUNE complexes
        Type: general
      – SubjectFull: SYMPTOMS
        Type: general
      – SubjectFull: THERAPEUTICS
        Type: general
      – SubjectFull: COMPLEMENT (Immunology)
        Type: general
      – SubjectFull: COMPLEMENT inhibition
        Type: general
      – SubjectFull: KIDNEY failure
        Type: general
      – SubjectFull: NEPHRITIS
        Type: general
    Titles:
      – TitleFull: Eficacia de iptacopán en glomerulonefritis membranoproliferativa por inmunocomplejos idiopática refractaria al tratamiento convencional.
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            NameFull: López Hidalgo, Raquel
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            NameFull: Caba Molina, Mercedes
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            NameFull: Ruiz Escolano, Francisco Javier
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            NameFull: Murga, Elena Delgado Sánchez de
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            NameFull: Fernández Quirós, Ma Teresa
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            NameFull: Espigares Huete, Ma José
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              M: 02
              Text: Feb2026
              Type: published
              Y: 2026
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              Value: 46
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