Validation of the classification criteria for cryoglobulinaemic vasculitis

• Published in: Rheumatology Vol. 53; no. 12; pp. 2209 - 2213
• Main Authors: QUARTUCCIO, Luca, ISOLA, Miriam, Corazza L, Ramos Casals M, Retamozo S, Ragab GM, Zoheir MN, El Menyawi MA, Salem MN, Sansonno D, Ferraccioli G, Gremese E, Tzioufas A, Voulgarelis M, Vassilopoulos D, Scarpato S, Pipitone N, Salvarani C, Guillevin L, Terrier B, Cacoub P, Filippini D, Saccardo F, Gabrielli A, Fraticelli P, Sebastiani M, Tomsic M, Tavoni A, Mazzaro C, Pioltelli P, Nishimoto N, Scaini P, Zignego AL, Ferri C, Monti G, Pietrogrande M, Bombardieri S, Galli M, DE VITA, Salvatore
• Format: Journal Article
• Language: English; Japanese
• Published: England Oxford University Press (OUP) 01.12.2014
• Subjects: Adult; Aged; Case-Control Studie; Case-Control Studies; classification; classification; cryoglobulinaemia; hepatitis C; vasculitis; Adult; Aged; Case-Control Studies; Cryoglobulinemia; Female; Hepatitis C; Humans; Male; Middle Aged; Questionnaires; Sensitivity and Specificity; Systemic Vasculitis; Medicine (all); classification; cryoglobulinaemia; hepatitis C; vasculitis; Adult; Aged; Case-Control Studies; Cryoglobulinemia; Female; Hepatitis C; Humans; Male; Middle Aged; Sensitivity and Specificity; Surveys and Questionnaires; Systemic Vasculitis; Medicine (all); cryoglobulinaemia; Cryoglobulinemia; Cryoglobulinemia - classification; Cryoglobulinemia - complications; Cryoglobulinemia - diagnosis; Female; Hepatitis C; Hepatitis C - complications; hepatitis C virus; Mixed Cryoglobulinemia; cryoglobulinemic vasculitis; classification; Human; Humans; Male; Medicine (all); Middle Aged; Questionnaires; Sensitivity and Specificity; Surveys and Questionnaire; Surveys and Questionnaires; Systemic Vasculiti; Systemic Vasculitis; Systemic Vasculitis - classification; Systemic Vasculitis - diagnosis; Systemic Vasculitis - etiology; vasculiti; vasculitis; classification; vasculitis; cryoglobulinaemia; hepatitis C
• ISSN: 1462-0324; 1462-0332; 1462-0332
• DOI: 10.1093/rheumatology/keu271

The aim of this study was to validate the classification criteria for cryoglobulinaemic vasculitis (CV). Twenty-three centres were involved. New patients with CV (group A) and controls, i.e. subjects with serum cryoglobulins but lacking CV based on the gold standard of clinical judgment (group B) and subjects without cryoglobulins but with clinical features that can be observed in the course of CV (group C), were studied. Positivity of serum cryoglobulins was necessary for CV classification. Sensitivity and specificity of the criteria were calculated by comparing group A vs group B. The group A vs group C comparison was done to demonstrate the possible diagnostic utility of the criteria. The study included 268 patients in group A, 182 controls in group B and 193 controls in group C (small vessel vasculitis, 51.8%). The questionnaire (at least 2/3 positive answers) showed 89.0% sensitivity and 93.4% specificity; the clinical item (at least 3/4 clinical involvement) showed 75.7% sensitivity and 89.0% specificity and the laboratory item (at least 2/3 laboratory data) showed 80.2% sensitivity and 62.4% specificity. The sensitivity and specificity of the classification criteria (at least 2/3 positive items) were 89.9% and 93.5%, respectively. The comparison of group A with group C demonstrated the clinical utility of the criteria in differentiating CV from CV mimickers. Classification criteria for CV were validated in a second, large, international study confirming good sensitivity and specificity in a complex systemic disease.