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


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27

 

  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, Internal Medicine Unit, Presidio Ospedaliero di Saronno, ASST della Valle Olona, Italy.
  5. Rheumatology Unit, ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy.
  6. Apheresis Unit, Blood Transfusion Service, University Hospital of Padua, Italy.
  7. Immune-Rheumatology Unit, Department of Diagnostic and Clinical Medicine and Public Health, University of Modena and Reggio Emilia, Modena, Italy.
  8. Onco-Haematology Unit, CRO Aviano, National Cancer Institute, Aviano, Italy.
  9. Allergology and Clinical Immunology Unit, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy.
  10. Department of Medicine, University of Padua, Italy.
  11. Department of Health Sciences, University of Milan, Italy.
  12. Bicocca San Gerardo Haematology Unit, S. Gerardo Hospital, ASST Monza, Italy.
  13. Rheumatology Clinic, DSMB, AOU Santa Maria della Misericordia, University of Udine, Italy.
  14. Rheumatology Unit, M. Scarlato Hospital, Scafati, Italy.
  15. Clinica delle Malattie Infettive, L. Sacco Department of Biomedical and Clinical Sciences, University of Milan, Italy.
  16. Clinica delle Malattie Infettive, L. Sacco Department of Biomedical and Clinical Sciences, University of Milan, Italy.
  17. Department of Clinical and Molecular Sciences, Clinical Medicine, Università Politecnica delle Marche, Ancona, Italy.
  18. Unit of Nephrology, ASST degli Spedali Civili di Brescia, Italy.
  19. Rheumatology Unit, Department of Medical and Surgical Sciences, University of Modena and Reggio Emilia, Modena, Italy.
  20. Rheumatology Unit, Department of Medical and Surgical Sciences, University of Modena and Reggio Emilia, Modena, Italy.
  21. Sacco Reumatologia UOC Reumatologia, ASST Fatebenefratelli-Sacco, Milan, Italy.
  22. Department of Clinical and Molecular Sciences, Clinical Medicine, Università Politecnica delle Marche, Ancona, Italy.
  23. Centro Manifestazioni Sistemiche da virus epatitici, University of Florence, Italy.
  24. Unit of Nephrology, ASST degli Spedali Civili di Brescia, Italy.
  25. Rheumatology Unit, Department of Medical and Surgical Sciences, University of Modena and Reggio Emilia, Modena, Italy.
  26. Rheumatology Clinic, DSMB, AOU Santa Maria della Misericordia, University of Udine, Italy.
  27. Rheumatology Unit, Internal Medicine Unit, Presidio Ospedaliero di Saronno, ASST della Valle Olona, Italy.

CER10265
2017 Vol.35, N°1 ,Suppl.103
PI 0067, PF 0076
Clinical aspects

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PMID: 28466806 [PubMed]

Received: 19/01/2017
Accepted : 27/03/2017
In Press: 19/04/2017
Published: 19/04/2017

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.

Rheumatology Article