in essential mixed cryoglobulinaemia, autoimmune disease as SLE, PAN,
HSP, infection as CMV, malaria and HBV.
While patients with cryoglobulinaemia usually present with the
manifestation of the original disease, 20-30% of patients with mixed
cryoglobulinaemia present with disease (vasculitis) caused by
cryoglobulin itself. This is termed essential mixed cryoglobulinaemia.
Clinical features:
Clinical manifestations of EMC include the following:
1- Renal, including nephrotic syndrome, nephritic syndrome or RPGN.
2- Extrarenal, including purpura, arthritis and hepatic dysfunction.
Treatment:
Steroid and cyclophosphamide are usually given in combination to
treat EMC. Plasma exchange is indicated with severe disease to lower the
level of circulating cryoglobulin.