Crioglobulinemia
Definizione
La crioglobulinemia è una malattia caratterizzata da porpora, artralgia, astenia e neuropatia periferica, con presenza di immunoglobuline sieriche (crioglobuline) precipitabili a freddo, a temperatura inferiore a 37°C. . E’ classificata in tre sottogruppi a seconda della composizione di immunoglobuline.
Classificazone
Crioglobulinemia di tipo I: si compone di un solo isotipo o sottoclasse di immunoglobulina. Associata di solito a malattie ematologiche quali mieloma o macroglobulinemia di Waldenstrom.
Crioglobulinemia di tipo II e III: si riferiscono a crioglobulinemie miste e sono composti da entrambi i componenti IgG e IgM. Contengono fattori reumatoidi (RF).
Crioglobulinemia di tipo II: frequentemente di associa un’ epatite virale C, lupus eritematoso sistemico, sindrome di Sjögren.
Crioglobulinemia di tipo III: malattie infettive, malattie autoimmuni.
Manifestazioni cliniche
Le manifestazioni cliniche connesse con la crioglobulinemia di tipo 1 comprendono:
- Acrocianosi
- Emorragia retinica
- Fenomeno di Raynaud grave
- Porpora
- Trombosi arteriosa
Le manifestazioni cliniche associate con la crioglobulinemia di tipo II e III includono:
- Affaticamento
- Atralgie (dolore articolare e artrite, specialmente a livello delle ginocchia e delle anche).
- Malattie renali da immunocomplessi
- Vasculite cutanea
- Neuropatia periferica
Terapia
La crioglobulinemia asintomatica non richiede alcun trattamento. Lo scopo generale della terapia è il trattamento delle cause scatenanti e nei casi più gravi la soppressione della risposta immunitaria. I farmaci antinfiammatori (FANS) sono efficaci nei casi lievi e la terapia con corticosteroidi è riservata per i casi più gravi o refrattari alla terapia. I soggetti che necessitano di una immunosoppressione o di altre terapie più aggressive richiedono il controllo dello specialista.
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