Glomerulonefrite membranosa
Table of Contents
Descrizione
La glomerulonefrite membranosa è la causa più frequente di sindrome nefrosica negli adulti. La forma più frequente, come tutte le GMN, è quella idiopatica. Attualmente tale forma viene considerata a tutti gli effetti come una malattia autoimmune organo- specifica e nel 70% di questi casi è possibile riscontrare la presenza di auto-anticorpi diretti contro il recettore della fosfolipasi A2 (anti-PLAZR). Le forme secondarie più rilevanti sono quelle paraneoplastiche, per cui in un adulto con GMN membranosa è sempre necessario escludere la presenza di neoplasia solida o di un melanoma.
Altre cause di GMN secondaria comprendono:
Infezioni croniche
- HBV, malaria.
Malattie autoimmuni
- AR, LES.
Metalli pesanti
- Oro, Mercurio.
Farmaci
- Capitopril, Penicillamina, Sali di oro.
Altre
- Dermatite erpetiforme.
Si produce una lesione delle membrane basali di tutti i glomeruli che appaiono ingrossati. Nelle fasi iniziali può essere normale.
Segni clinici e diagnosi
E’ la causa più frequente della sindrome nefrosica negli adulti ed è rara nei bambini. Può essere presente ematuria. L’evoluzione può presentare una complicanza caratteristica: è la GMN che produce con maggiore frequenza una trombosi della vena renale.
Gli anticorpi contro il recettore della fosfolipasi -A2 sono presenti fino al 70% delle GNM membranosa idiopatiche (assenti in tutti casi di GMN secondaria ad altre malattie). I livelli predicono la risposta clinica alla terapia immunosoppressiva (se vi è risposta al trattamento i livelli scendono settimane-mesi prima che si verifichi un calo della proteinuria).
Prognosi
E’ possibile affermare che un terzo dei soggetti affetti da glomerulonefrite membranosa (GMN) presenta una remissione spontanea, un terzo presenta l’insufficienza renale progressiva e un terzo richiede un trattamento sostitutivo renale. Le remissioni spontanee sono più frequenti nei bambini. Dati di prognosi infausta sono insufficienza renale, una proteinuria massiva, iperlipiduria intensa e IA. Può recidivare nel trapianto.
Trattamento
I corticosteroidi in monoterapia non sembrano essere efficaci. In alcuni casi la combinazione di corticosteroide e ciclofosfamide o dorambucile dà buoni risultati.
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