Article Text

SAT0175 Results of the Classification Criteria for Cryoglobulinemic Vasculitis Validation Study
  1. S. De Vita1,
  2. L. Quartuccio1,
  3. M. Isola2,
  4. L. Corazza3,
  5. M. Ramos-Casals4,
  6. S. Retamozo4,
  7. G. Ragab5,
  8. N. Zoheir5,
  9. M. M. El Menyawi5,
  10. M. Salem6,
  11. D. Sansonno7,
  12. V. Conteduca7,
  13. G. Ferraccioli8,
  14. E. Gremese8,
  15. A. Tzioufas9,
  16. M. Voulgarelis9,
  17. D. Vassilopoulos10,
  18. C. Koutsianas10,
  19. A. L. Zignego11,
  20. T. Urraro11,
  21. N. Pipitone12,
  22. C. Salvarani12,
  23. A. Ghinoi12,
  24. L. Guillevin13,
  25. B. Terrier13,
  26. P. Cacoub14,
  27. D. Filippini15,
  28. F. Saccardo16,
  29. A. Gabrielli17,
  30. P. Fraticelli17,
  31. M. Tomsic18,
  32. C. Ferri19,
  33. M. Sebastiani19,
  34. A. Tavoni20,
  35. E. Catarsi20,
  36. C. Mazzaro21,
  37. P. Pioltelli22,
  38. N. Nishimoto23,
  39. P. Scaini24,
  40. G. Monti25,
  41. M. Pietrogrande26,
  42. M. Galli27,
  43. S. Bombardieri20
  1. 1Department of Biological and Medical Sciences, Rheumatology Clinic, University Hospital of Udine
  2. 2Department of Biological and Medical Sciences, Institute of Statistics
  3. 3Rheumatology Clinic, University Hospital of Udine, UDINE, Italy
  4. 4Laboratorio de Enfermedades Autoinmunes Josep Font, IDIBAPS, Hospital Clínic, Barcelona, Spain
  5. 5Faculty of Medicine, Cairo University, Cairo
  6. 6Faculty of Medicine, Beni Swafe University, Beni Swafe, Egypt
  7. 7Section of Internal Medicine and Clinical Oncology, University of Bari, Medical School, Bari
  8. 8Rheumatology Division, Catholic University of the Sacred Heart, Rome, Italy
  9. 9Department of Pathophysiology, Medical School of Athens
  10. 10Second Department of Medicine, Hippokration General Hospital, Athens, Greece
  11. 11Center for Systemic Manifestations of Hepatitis Viruses (MaSVE), Florence
  12. 12Division of Reumatology, Arcispedale S. M. Nuova, Reggio Emilia, Italy
  13. 13Centre de références maladies auto-immunes, service de médecine interne II, hôpital Pitié-Salpêtrière
  14. 14Service de Médecine Interne, Hôpital Cochin, Paris, France
  15. 15Rheumatology Unit, Niguarda Ca’ Granda Hospital, Milan
  16. 16Internal Medicine Unit, Saronno Hospital, Saronno (VA)
  17. 17Internal Medicine, Università Politecnica delle Marche, Ancona, Italy
  18. 18Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia
  19. 19Rheumatology Unit, Department of Internal Medicine, University of Modena, Modena
  20. 20Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa
  21. 21Oncologic Centre, Aviano (PN)
  22. 22Hematology, San Gerardo Hospital, Monza, Italy
  23. 23Osaka Rheumatology Clinic, Osaka, Japan
  24. 24Nephrology, A.O. Spedali Civili, Brescia
  25. 25Internal Medicine Unit, Saronno Hospital, Saronno (VA)
  26. 26Università of Milan, Policlinico San Marco, Zingonia, Bergamo
  27. 27Infectious Diseases Institute, University of Milan, L. Sacco Hospital, Milan, Italy


Background the preliminary Classification Criteria for cryoglobulinemic vasculitis (CV) have been developed in 2011 by an European cooperative study (1).

Objectives to validate these Classification Criteria for CV with the participation of non-European Countries.

Methods 20 Centres from Europe, Egypt, and Japan partecipated. New consecutive, unselected patients with CV (Group A) and controls (subjects with cryoglobulins without a CV based on the golden standard clinical judgment; Group B) were studied. A sample size of 140 patients for each group was estimated to obtain a sensitivity (SE) and a specificity (SP) of at least 90±5%, according to the previous results (1). A dedicated chart was distributed to the Centres. The sensitivity and specificity of the 2011 Classification Criteria were calculated in the present validation series by comparing Group A versus Group B.

Results 251 patients in Group A and 175 controls in Group B were recruited. The questionnaire (at least 2/3 positive answers) showed a SE of 89.2% (95% CI 85.4-93.1) and a SP of 93.7% (95% CI 90.1-97.3); the clinical item (at least 3/4 clinical items among constitutional, articular, vascular and neurologic involvement) showed a SE of 76.1% (95% CI 70.8-81.4) and a SP of 88.6% (95% CI 83.8-93.9), and the laboratory item (at least 2/3 tests, among positive rheumatoid factor, low C4, and the presence of serum monoclonal component) showed 75.1% (95% CI 69.5-80.7) of SE and 71.5% (95% CI 64.6-78.4) of SP. The final 2011 Classification Criteria (at least 2/3 positive items) showed a SE of 89.3% (95% CI 85.3-93.3) and a SP of 93.9 % (95% CI 90.3-97.6).

Conclusions: Conclusion : The 2011 International Classification Criteria for the cryoglobulinemic vasculitis have been validated in a new cohort of real cases and controls. Patients where CV is suspected on clinical grounds, but where cryoglobulins are negative, cannot be classified, since positive serum cryoglobulinemia is a conditio sine qua non for classification (1). However, the performance of these criteria on this subset of patients is under evaluation.


  1. De Vita S, et al. Ann Rheum Dis 2011;70:1183-90.

Disclosure of Interest None Declared

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