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Gomerulonefrite membranoproliferativa

Table of Contents


Definizione

 

Con il termine glomerulonefrite membranoproliferativa si definisce una nefropatia caratterizzata da una proliferazione mesangiale associata ad un aumento di spessore della parete delle anse capillari con conseguente aumento di volume dei glomeruli e accentuazione della lobatura del tubulo convoluto.

Eziologia

La glomerulonefrite membranoproliferativa può essere idiopatica o secondaria.

La forma secondaria è solita esserlo a:

Malattie autoimmuni:

  • Crioglobulinemia mista essenziale;
  • Artrite reumatoide.

Infezioni:

  • Epatopatia cronica attiva: HCV e HBV.
  • Endocardite.
  • Nefrite dello shunt.
  • Parassiti: schistosomiasi,  malaria.
  • Ascessi.

Tumori ematologici:

E’ l’unica GNM senza predominanza nei soggetti di sesso maschile (non presenza differenza tra i due sessi).

Si distinguono tre tipi:

  • Tipo I con depositi subendoteliali.
  • Tipo II con depositi “densi” della membrana basale.
  • Tipo III con depositi subendoteliali e subepiteliali​.

 

 

Epidemiologia

 

Questa forma è particolarmente frequente nelle prime tre decadi della vita e ha un lieve predominio nel sesso femminile. Più frequente negli anni 60 e 70 (15- 20%),  si è notevolmente ridotta nel corso di quest’ultimo trentennio. Nel corso di questi ultimi vent’anni la forma di tipo 1 si è notevolmente ridotta mentre la frequenza di quella di tipo II è rimasta invariata in Asia, Sudamerica, Africa. La glomerulonefrite membranoproliferative è una delle cause più frequenti di sindrome nefrosica perché è causata dalle infezioni croniche.


Sintomatologia

 

L’età di esordio della forma di tipo I è nella seconda e terza decade di vita; la forma di tipo II è, invece, più frequente nell’età pediatrica. La sintomatologia d’esordio non differisce nelle due forme. Il quadro clinico è abbastanza variabile.

  • Nel 50% dei casi si presenta con una sindrome nefrosica.
  • Nel 30%  con una proteinuria asintomatica accompagnata spesso da macro-o microematuria.
  • Nel restante 20% con una sindrome nefritica acuta: quest’ultimo aspetto è più frequente nella glomerulonefrite membranoproliferativa di tipo II.

In gran parte dei casi l’esordio è caratterizzato da episodi infettivi delle alte vie respiratorie. All’esordio della malattia possono essere presenti:

  • Ipertensione arteriosa
  • Riduzione del filtrato glomerulare
  • Anemia moderata (HB 8-10) di tipo normocromico normocitico. L”ipertensione arteriosa aumenta durante il decorso della malattia prima della comparsa della riduzione del filtrato glomerulare, per cui non sembra essere attribuibile all’insufficienza renale.
  • Una riduzione del filtrato glomerulare si osserva nel 50% dei casi; se è presente all’inizio della malattia esprime una prognosi renale infausta.

Diagnosi

 

La presenza di una proteinuria e/o una sindrome nefrosica che compare in un soggetto giovane e presenta una progressione verso l’insufficienza renale deve orientare verso la diagnosi di glomerulonefrite membranoproliferativa, a maggior ragione s e a questa sintomatologia si associa un’ ipertensione arteriosa. Comunque, per un’accurata diagnosi è necessario il referto bioptico che permette di distinguere le forme in cui si manifesta la malattia a livello renale. L’immunofluorescenza permette una migliore diagnosi differenziale tra le forme di tipo 1 e II mentre la microscopia elettronica apporta ulteriori ragguagli sulle tre forme. Nella diagnosi di glomerulonefrite membranoproliferativa è necessario differenziare le forme primitive da quelle secondarie in cui dati clinici e di laboratorio rivestono un ruolo importante​.


Decorso clinico e prognosi

Il decorso clinico della glomerulonefrite membranoproliferativa è lento e progressivo; esso è caratterizzato da una riduzione del FGR e dall’ aggravamento dello stato ipertensivo. La sopravvivenza renale a 10 anni è del 40% sia negli adulti sia nei bambini. Dopo Un follow-up medio di 5-7 anni dall’insorgenza della malattia:

  • Il 30-40% dei pazienti ha una funzione renale normale
  • Il 25-30% ha un’ insufficienza renale
  • Il 25% in trattamento dialitico 
  • Una percentuale variabile tra il 6 e il 25% è deceduta.

In una piccola percentuale, pari al 5-8% è stato osservata una remissione clinica completa della malattia, prevalentemente in bambini che presentavano una forma di tipo I. Il decorso clinico non differisce nelle forme di tipo I e II. La malattia ha una prognosi sfavorevole, specialmente quando insorge in età giovanile e all’atto della diagnosi si osservano i seguenti fattori clinici umorali:

  • Ridotto FGR
  • Ipertensione arteriosa
  • Persistenza di una sindrome nefrosica e fattori ismomorfologici costituiti prevalentemente da semilune e  lesioni tubulo-interstiziali.
  • La prognosi della glomerulonefrite membranoproliferativa di tipo II è  generalmente più infausta, 

 

Terapia

L’efficacia della terapia nella glomerulonefrite membranoproliferativa è molto controversa. Una recente indagine retrospettiva non ha evidenziato significative differenze di sopravvivenza a 10 anni tra pazienti trattati e non trattati. I tentativi terapeutici finora eseguiti sono stati effettuati con i seguenti farmaci: corticosteroidi, immunosoppressori, anticoagulanti, antiaggreganti piastrinici, antinfiammatori non steroidi.

 

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(Malattie del rene e delle vie urinarie, F.P. Schiena, F.P. Selvaggi, L. Gesualdo, M. Battaglia)​

 

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