The natural history of cryoglobulinemia: symptoms at onset and during follow-up. A report by the Italian Group for the Study of Cryoglobulinemias (GISC)
OBJECTIVE
The cryoglobulinemic syndrome (CS) may be associated with other diseases; when it is not, it is termed essential. Recently the natural history of this disease has been re-evaluated on the basis of its close association with HCV markers. In this context, the GISC has studied a large series of patients from several Italian centres.


METHODS
In a multicentric retrospective study of 913 cases, we evaluated the clinical and laboratory signs at onset in patients with essential mixed cryoglobulinemias (EMC) (654 cases) or secondary cryoglobulinemias (SC) (259 cases) and sought to define possible diagnostic criteria typical of the different CS. We also carried out a retrospective 5-year cohort study on 192 patients selected randomly from the 913 cases described above. In particular, we examined the correlation between the presence/absence of concomitant diseases and the presence of HCV markers on the clinical evolution and survival of the patients.


RESULTS
Purpura, rheumatoid factors (RF), significant C4 consumption, and Brouet's classification type II were more frequent in EMC. Purpura, Meltzer's triad, and Raynaud's phenomenon improved, while renal involvement tended to worsen over time. During the follow-up we did not note a significant change in clinical staging in the patients with Brouet's type II cryoglobulins, in the patients with HCV-related markers, or in the overall series. The most frequent causes of death in 34 patients were liver, cardiovascular, renal, and lymphoproliferative diseases. Lymphomas were diagnosed in 11 patients during follow-up, with particular frequency in the HCV-marker positive patients.


CONCLUSIONS
Specific clinical and laboratory features typical of the different CS subgroups could not be identified on the basis of these data. Our follow-up data seem, however, to confirm a role for HCV in the mixed cryoglobulinemias.
Monti
G
G
I. Divisione di Medicina, Saronno Hospital.
Saccardo
F
F
Pioltelli
P
P
Rinaldi
G
G
D000368:Aged; D015331:Cohort Studies; D003449:Cryoglobulinemia / Q000503:physiopathology* / Q000821:virology; D005260:Female; D005500:Follow-Up Studies; D016174:Hepacivirus; D018937:Hepatitis C Antibodies / Q000032:analysis; D006801:Humans; D007558:Italy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies
D018937:Hepatitis C Antibodies