HCV-unrelated cryoglobulinaemic vasculitis: the results of a prospective observational study by the Italian Group for the Study of Cryoglobulinaemias (GISC)

• Published in: Clinical and experimental rheumatology Vol. 35 Suppl 103; no. 1; pp. 67 - 76
• Main Authors: Galli, Massimo, Oreni, Letizia, Saccardo, Francesco, Castelnovo, Laura, Filippini, Davide, Marson, Piero, Mascia, Maria Teresa, Mazzaro, Cesare, Origgi, Laura, Ossi, Elena, Pietrogrande, Maurizio, Pioltelli, Piero, Quartuccio, Luca, Scarpato, Salvatore, Sollima, Salvatore, Riva, Agostino, Fraticelli, Paolo, Zani, Roberta, Giuggioli, Dilia, Sebastiani, Marco, Sarzi Puttini, Piercarlo, Gabrielli, Armando, Zignego, Anna Linda, Scaini, Patrizia, Ferri, Clodoveo, De Vita, Salvatore, Monti, Giuseppe
• Format: Journal Article
• Language: English
• Published: Italy 01.03.2017
• Subjects: Biomarkers - blood; Complement System Proteins - metabolism; Cryoglobulinemia - blood; Cryoglobulinemia - epidemiology; Cryoglobulinemia - immunology; Cryoglobulinemia - mortality; Cryoglobulins - metabolism; Disease Progression; Female; Humans; Incidence; Inflammation Mediators - blood; Italy - epidemiology; Kaplan-Meier Estimate; Logistic Models; Male; Multivariate Analysis; Odds Ratio; Prevalence; Prognosis; Proportional Hazards Models; Prospective Studies; Risk Factors; Severity of Illness Index; Systemic Vasculitis - blood; Systemic Vasculitis - epidemiology; Systemic Vasculitis - immunology; Systemic Vasculitis - mortality; Time Factors
• ISSN: 0392-856X

To investigate the clinical and laboratory patterns of HCV-unrelated cryoglobulinaemic vasculitis (CV), and the factors influencing its outcome. Prospective study of all anti-HCV and HCV-RNA negative patients with CV who have been observed since January 2004 in 17 centres participating in the Italian Group for the Study of Cryoglobulinaemias (GISC). 175 enrolled were followed up for 677 person-years. The associated conditions were primary Sjögren's syndrome (21.1%), SLE (10.9%), other autoimmune disorders (10.9%), lymphoproliferative diseases (6.8%), solid tumours (2.3%) and HBsAg positivity (8.6%), whereas 69 patients (39.4%) had essential CV. There were significant differences in age (p<0.001), gender (p=0.002), the presence of purpura (p=0.005), arthralgia (p=0.009), liver abnormalities (p<0.001), sicca syndrome (p<0.001), lymphadenopathy (p=0.003), splenomegaly (p=0.002), and rheumatoid factor titres (p<0.001) among these groups. Type II mixed cryoglobulins were present in 96 cases (54.9%) and were independently associated with purpura and fatigue (odds ratio [OR]4.3; 95% confidence interval [CI] 1.8-10.2; p=0.001; and OR2.8; 95%CI 1.3-6.3; p=0.012). Thirty-one patients died during follow-up, a mortality rate of 46/1000 person-years. Older age (for each additional year, adjusted hazard ratio [aHR] 1.13; 95%CI 1.06-1.20; p<0.001), male gender (aHR 3.45; 95%CI 1.27-9.40; p=0.015), type II MCG (aHR 3.31; 95%CI 0.09-1.38; p=0.047) and HBsAg positivity (aHR 7.84; 95%CI 1.20-36.04; p=0.008) were independently associated with greater mortality. HCV-unrelated CV is a multifaceted and often disabling disorder. The associated conditions influence its clinical severity, giving rise to significantly different clinical and laboratory profiles and outcomes.