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

M. Galli, L. Oreni, F. Saccardo, L. Castelnovo, D. Filippini, P. Marson, M.T. Mascia, C. Mazzaro, L. Origgi, E. Ossi, M. Pietrogrande, P. Pioltelli, L. Quartuccio, S. Scarpato, S. Sollima, A. Riva, P. Fraticelli, R. Zani, D. Giuggioli, M. SebastianiP. Sarzi-Puttini, A. Gabrielli, A.L. Zignego, P. Scaini, C. Ferri, S. De Vita, G. Monti

Research output: Contribution to journalArticlepeer-review

Abstract

Objective. To investigate the clinical and laboratory patterns of HCV-unrelated CV, and the factors influencing its outcome. Methods. Prospective study of all anti- HCV and HCV-RNA negative patients with CV 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 difference 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 followup, a mortality rate of 46/1000 personyears. 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. Conclusion. 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. © Clinical and Experimental Rheumatology 2017.
Original languageEnglish
Pages (from-to)67-76
Number of pages10
JournalClinical and Experimental Rheumatology
Volume35
Publication statusPublished - 2017

Keywords

  • Cryoglobulinaemic vasculitis
  • Hepatitis C virus-unrelated cryoglobulinaemia
  • Type II mixed cryoglobulins
  • complement component C4
  • cryoglobulin
  • rheumatoid factor
  • autacoid
  • biological marker
  • complement
  • adult
  • age
  • aged
  • Article
  • cryoglobulinemia
  • essential cryoglobulinaemic vasculitis
  • fatigue
  • female
  • follow up
  • hepatitis C
  • Hepatitis C virus
  • human
  • hypertension
  • liver disease
  • lymphadenopathy
  • lymphoproliferative disease
  • major clinical study
  • male
  • mortality
  • observational study
  • outcome assessment
  • priority journal
  • prospective study
  • purpura
  • Sjoegren syndrome
  • solid malignant neoplasm
  • splenomegaly
  • systemic lupus erythematosus
  • vasculitis
  • blood
  • clinical trial
  • disease course
  • immunology
  • incidence
  • Italy
  • Kaplan Meier method
  • metabolism
  • multicenter study
  • multivariate analysis
  • odds ratio
  • prevalence
  • prognosis
  • proportional hazards model
  • risk factor
  • severity of illness index
  • statistical model
  • systemic vasculitis
  • time factor
  • Biomarkers
  • Complement System Proteins
  • Cryoglobulinemia
  • Cryoglobulins
  • Disease Progression
  • Female
  • Humans
  • Incidence
  • Inflammation Mediators
  • 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
  • Time Factors

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