Massimo Galli1, Letizia Oreni2, Francesco Saccardo3, Laura Castelnovo3, Davide Filippini4, Piero Marson5, Maria Teresa Mascia6, Cesare Mazzaro7, Laura Origgi8, Elena Ossi9, Maurizio Pietrogrande10, Piero Pioltelli11, Luca Quartuccio12, Salvatore Scarpato13, Salvatore Sollima2, Agostino Riva2, Paolo Fraticelli14, Roberta Zani15, Dilia Giuggioli16, Marco Sebastiani16, Piercarlo Sarzi Puttini17, Armando Gabrielli14, Anna Linda Zignego18, Patrizia Scaini15, Clodoveo Ferri16, Salvatore De Vita12, Giuseppe Monti3. 1. Clinica delle Malattie Infettive, L. Sacco Department of Biomedical and Clinical Sciences, University of Milan, Italy. massimo.galli@unimi.it. 2. Clinica delle Malattie Infettive, L. Sacco Department of Biomedical and Clinical Sciences, University of Milan, Italy. 3. Rheumatology Unit, Internal Medicine Unit, Presidio Ospedaliero di Saronno, ASST della Valle Olona, Italy. 4. Rheumatology Unit, ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy. 5. Apheresis Unit, Blood Transfusion Service, University Hospital of Padua, Italy. 6. Immune-Rheumatology Unit, Department of Diagnostic and Clinical Medicine and Public Health, University of Modena and Reggio Emilia, Modena, Italy. 7. Onco-Haematology Unit, CRO Aviano, National Cancer Institute, Aviano, Italy. 8. Allergology and Clinical Immunology Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy. 9. Department of Medicine, University of Padua, Italy. 10. Department of Health Sciences, University of Milan, Italy. 11. Bicocca San Gerardo Haematology Unit, S. Gerardo Hospital, ASST Monza, Italy. 12. Rheumatology Clinic, DSMB, AOU Santa Maria della Misericordia, University of Udine, Italy. 13. Rheumatology Unit, M. Scarlato Hospital, Scafati, Italy. 14. Department of Clinical and Molecular Sciences, Clinical Medicine, Università Politecnica delle Marche, Ancona, Italy. 15. Unit of Nephrology, ASST degli Spedali Civili di Brescia, Italy. 16. Rheumatology Unit, Department of Medical and Surgical Sciences, University of Modena and Reggio Emilia, Modena, Italy. 17. Sacco Reumatologia UOC Reumatologia, ASST Fatebenefratelli-Sacco, Milan, Italy. 18. Centro Manifestazioni Sistemiche da virus epatitici, University of Florence, Italy.
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.
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.
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