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

Abstract

OBJECTIVES:

To investigate the clinical and laboratory patterns of HCV-unrelated cryoglobulinaemic vasculitis (CV), and the factors influencing its outcome.

METHODS:

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).

RESULTS:

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.

CONCLUSIONS:

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.

• Authors: Massimo Galli, Letizia Oreni, Francesco Saccardo, Laura Castelnovo, Davide Filippini, Piero Marson, Maria Teresa Mascia, Cesare Mazzaro, Laura Origgi, Elena Ossi, Maurizio Pietrogrande, Piero Pioltelli, Luca Quartuccio, Salvatore Scarpato, Salvatore Sollima, Agostino Riva, Paolo Fraticelli, Roberta Zani, Dilia Giuggioli, Marco Sebastiani, Piercarlo Sarzi Puttini, Armando Gabrielli, Anna Linda Zignego, Patrizia Scaini, Clodoveo Ferri, Salvatore De Vita, Giuseppe Monti
• Journal: Clinical and experimental rheumatology (Clin Exp Rheumatol) 2017 Mar-Apr Vol. 35 Suppl 103 Issue 1 Pg. 67-76 ISSN: 0392-856X [Print] Italy
• PMID: 28466806 (Publication Type: Journal Article, Multicenter Study, Observational Study)

Chemical References

• Biomarkers
• Cryoglobulins
• Inflammation Mediators
• Complement System Proteins

Topics

• Biomarkers (blood)
• Complement System Proteins (metabolism)
• Cryoglobulinemia (blood, epidemiology, immunology, 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, epidemiology, immunology, mortality)
• Time Factors