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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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