Characteristics and outcome of 49 patients with symptomatic cryoglobulinaemia.

Objective: To describe a population of patients with symptomatic cryoglobulinaemia, comparing manifestations and outcome as a function of hepatitis C virus (HCV) status.
Patients and Methods: A retrospective study on 179 patients who tested positive for cryoglobulins, seen between 1978 and 1998 in an internal medicine department.
Results: Among 179 cryoglobulin-positive patients, only 49 (18 men, 31 women; mean age 59.96+/-12 yr) had clinical manifestations attributable to cryoglobulinaemia. Thirty-three had HCV infection, 20 had systemic autoimmune diseases, two had haematological diseases, one had human immunodeficiency virus and HCV co-infection, one had HCV and HBV co-infection and six had essential mixed cryoglobulinaemia. The clinical manifestations and cryoglobulin levels in HCV(+) and HCV(-) patients did not differ significantly. Only arthralgias and elevated transaminases were significantly more frequent in HCV(+) patients (P<0.02 and <0.05, respectively). Five-year survival rates were comparable for HCV(+) and HCV(-) patients. Eight patients died (six HCV(+), two HCV(-)), with a median time between diagnosis and death of 38.7 months.
Conclusion: Clinical manifestations of cryoglobulinaemia, except arthralgias, were comparable for HCV(+) and HCV(-) patients. When systemic manifestations are present, the prognosis is poor despite intensive or prolonged therapy.