Porpora di Shöenlein-Henoch
Definizione
La porpora di Shöenlein-Henoch è una vasculite sistemica che interessa i piccoli vasi della cute, l’intestino, le piccole articolazioni e i reni.
Epidemiologia
La porpora di Shöenlein-Henoch è più frequente nell’età pediatrica tra i 2 e i 10 anni con un età mediana di esordio intorno ai 4 anni. E’ la vasculite più frequente nel bambino. La glomerulonefrite compare più frequentemente nella prima e seconda decade di vita. In un’ampia casistica italiana il picco di prevalenza della nefrite si è collocata nella seconda decade di età. Il sesso maschile è più frequentemente colpito dalla malattia con un rapporto M/F di 1:3 negli adulti e di 1:2,5 nei bambini.
Eziopatogenesi
La maggiore incidenza della malattia è nel periodo invernale, probabilmente per l’esposizione al freddo e per l’elevata ricorrenza dei processi infettivi delle alte vie respiratorie.Sono da tenere in considerazione gli antigeni alimentari,(cioccolata e coloranti), punture di insetti, le vaccinazioni, assunzione di alcuni farmaci quale acido acetilsalicilico, antinfiammatori non steroidi, antibiotici, sulfamidici. La malattia è considerata come l’espressione di uno stato infiammatorio indotto dalla deposizione di immunocomplessi circolanti o formati in situ.
Sintomatologia
La porpora di Shöenlein-Henoch è caratterizzata da:
-
- Manifestazioni cutanee, che sono rappresentate da un’ orticaria iniziale che evolve in macule purpuriche che non scompaiono con la digitopressione;
- Atralgie agli arti superiori e inferiori (ginocchia, caviglie). Questa sintomatologia è presente nel 60% dei casi.
- Dolori addominali, compaiono nel 50-70% dei pazienti e si manifestano sotto forma di coliche addominali con nausea,vomito e spesso melena. La sintomatologia addominale è causata dalla localizzazione della vasculite a livello intestinale.
- La sintomatologia renale varia molto e oscilla dalle lievi e transitoria anomalie urinarie (microematuria e proteinuria), alla classica nefropatia con macroematuria che spesso compare contemporaneamente alla porpora o dopo qualche settimana. Nel 20% dei pazienti si può osservare una sindrome nefrosica pura. In una piccola percentuale può evolvere verso un’ insufficienza renale cronica.
Decorso clinico e prognosi
La durata della malattia è di un anno e in genere il decorso clinico della porpora di Shöenlein-Henoch è benigno, anche se in alcuni casi persistono per anni gli episodi ricorrenti di porpora, atralgie, dolori addominali e le manifestazioni renali più o meno accentuate. Più della metà dei pazienti va incontro remissione completa e un altro 30% presenza solo anomalie urinarie lievi o moderate. L’insufficienza renale cronica si sviluppa in circa il 15% dei pazienti. La prognosi è strettamente correlata all’età, alla sintomatologia e all’entità della lesione renale. La malattia nel bambino è lieve, di breve durata con scarso interessamento intestinale e renale; pertanto la prognosi è buona nel 94% dei bambini.
Terapia
Nei pazienti con sindrome nefrosica e nefritica all’esordio l’uso di un breve ciclo di boli di metil-prednisolone, seguita prednisolone per os per 4-6 mesi, ha determinato una remissione completa. L’approccio terapeutico attuale nei pazienti che presentano insufficienza renale progressiva all’esordio della malattia e l’uso dei boli di steroidi associati alla ciclofosfamide quando la risposta iniziale è negativa. Gli steroidi migliorano notevolmente la sintomatologia extrarenale con la scomparsa dei dolori addominali, delle atralgie e della porpora.
Ti potrebbero interessare:
- Gomerulonefrite membranoproliferativa
- Glomerulonefrite con depositi mesangiali di IgA o malattia di Berger
- Glomerulonefrite membranosa
- Glomerulosclerosi focale e segmentaria
- News Vasculite, novità 2020
(Bibl. Malattie del rene e delle vie urinarie, F.P. Schiena, F.P. Selvaggi, L. Gesualdo, M. Battaglia)
Le informazioni medico-scientifiche pubblicate in questo sito si intendono per un uso esclusivamente informativo e non possono in alcun modo sostituire la visita medica.
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