ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2637

Validation Study Of The International Classification Criteria For The Cryoglobulinemic Vasculitis

Luca Quartuccio1, Miriam Isola2, Laura Corazza3, Soledad Retamozo4, Manal Abdel-Moneim El-Menyawi5, Elisa Gremese6, Marco Sebastiani7, Nicolo Pipitone8, Teresa Urraro9, Vincenza Conteduca10, Christos Koutsianas11, Benjamin Terrier12, Mostafa Naguib Zoheir13, Alessandra Ghinoi14, Davide Filippini15, Francesco Saccardo16, Mohamed Nabil Salem17, Salvatore Scarpato18, Paolo Fraticelli19, Antonio Tavoni20, Eleonora Catarsi21, Cesare Mazzaro22, Pietro Pioltelli23, Mervat Matar5, Patrizia Scaini24, Matija Tomsic25, Norihiro Nishimoto26,27, Dimitrios Vassilopoulos28, Michael Voulgarelis29, Gaafar M. Ragab30, Carlo Salvarani31, Armando Gabrielli32, Patrice Cacoub33, Loic Guillevin34, Domenico Sansonno35, Anna Linda Zignego36, Gianfranco Ferraccioli6, Athanasios G. Tzioufas37, Manuel Ramos-Casals38, Clodoveo Ferri39, Maurizio Pietrogrande40, Giuseppe Monti16, Massimo Galli41, Stefano Bombardieri42 and Salvatore De Vita43, 1Rheumatology Clinic, DSMB, University of Udine, Udine, Italy, 2Institute of Statistics, DSMB, University of Udine, Udine, Italy, 3Rheumatology Clinic, University of Udine, Udine, Italy, 4Laboratorio de Enfermedades Autoinmunes Josep Font, IDIBAPS, Hospital Clínic, Barcelona, Spain, 5Faculty of Medicine, Cairo, Egypt, 6Division of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, Rome, Italy, 7Internal Medicine, University of Modena and Reggio Emilia, Modena, Italy, 8Rheumatology Service, Arcispedale S Maria Nuova, IRCCS, Reggio Emilia, Italy, 9Center for Systemic Manifestations of Hepatitis Viruses (MASVE), Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, 10Department of Internal Medicine and Clinical Oncology, University of Bari, Bari, Italy, 11Department of Pathophysiology, Medical School of Athens, Athens, Greece, 12Internal Medicine, Cochin University Hospital, Paris, France, 13Faculty of Medicine, Cairo University, Cairo, Egypt, 14Rheumatology, Arcispedale S Maria Nuova, Reggio Emilia, Italy, 15Rheumatology Unit, Ospedale Niguarda,, Milan, Italy, 16Internal Medicine Unit, Saronno Hospital, Azienda Ospedaliera di Busto Arsizio, Saronno (VA), Italy, 17Faculty of Medicine, Beni Swafe University, Beni Swafe, Egypt, 18Rheumatology Unit, M. Scarlato Hospital, Scafati, Salerno, Italy, 19Istituto di Clinica Medica, Università Politecnica delle Marche, Ancona, Italy, 20University of Pisa, Immunoallergology Unit, Pisa, Italy, 21Department of Internal Medicine, Section of Immunoallergology, University of Pisa, Pisa, Italy, 22Department of Internal Medicine, Pordenone General Hospital, Pordenone, Italy, 23Hematology, S.Gerardo Hospital, Monza, Italy, 24Nephrology, Spedali Civili di Brescia, Brescia, Italy, 25Department of Rheumatology, University Medical Centre Ljubjana, Ljubljana, Slovenia, 26Laboratory of Immune Regulation, Wakayama Medical University, Ibaraki, Japan, 27Osaka Rheumatology Clinic, Osaka, Japan, 282nd Department of Medicine, Athens University School of Medicine, Athens, Greece, 29School of Medicine, University, Department of Pathophysiology, Athens, Greece, 30Int Medicine Hosp/Rheum&Immun, Faculty of Medicine, Cairo University, Giza, Egypt, 31Rheumatology, Arcispedale S Maria Nuova-IRCCS, Reggio Emilia, Italy, 32Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Ancona, Italy, 33Médecine Interne 2, Hopital Pitié-Salpétrière, Paris, France, 34Internal Medicine, Division of Internal Medicine, Hôpital Cochin, University Paris Descartes, Paris, France, 35Section of Internal Medicine and Clinical Oncology, Department of Biomedical Sciences and Human Oncology, University of Bari, Medical School, Bari, Italy, 36University of Florence, Center for Systemic Manifestations of Hepatitis Viruses (MASVE), Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, 37Pathophysiology, School of Medicine, National University of Athens, Athens, Greece, 38Laboratorio de Enfermedades Autoinmunes Josep Font, Hospital Clínic, Barcelona, Spain, 39Department of Internal Medicine, University of Modena and Reggio Emilia, Modena, Italy, 40Internal Medicine Unit, Policlinico San Marco, Bergamo, Italy, 41Istituto di Malattie Infettive e Tropicali, Università di Milano c/o Ospedale L. Sacco, Milano, Italy, 42Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy, 43Rheumatology, DSMB, University Hospital Santa Maria della Misericordia, Udine, Italy

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: , , ,

Session Information

Title: Vasculitis III

Session Type: Abstract Submissions (ACR)

Background/Purpose: preliminary Classification Criteria for cryoglobulinemic vasculitis (CV) have been developed in 2011 by an European cooperative study, with an adequate methodology in a large number of real cases and controls (1). The aim of this study is to validate these classification criteria for CV.

Methods: Centres from Europe, United States, Japan and Egypt, were involved. A dedicated chart included: l) a validated questionnaire for CV (1); 2) the pattern of organ involvement (4 items: constitutional, articular, vascular and neurologic involvement); 3) laboratory tests (3 items: rheumatoid factor, complement C4 and serum monoclonal component), according to the preliminary criteria (1). New patients with CV (Group A) and controls (Group B), i.e., subjects with cryoglobulins but lacking CV based on the golden standard clinical judgment, were studied. A sample size of at least 140 patients for each group was estimated in order to obtain a sensitivity and a specificity of at  least 90±5%, based on the previous results (1). Sensitivity and specificity were calculated by comparing Group A versus Group B. Finally, not for classification purposes, but to disclose whether the Criteria may be also clinically helpful in patients lacking serum cryoglobulins, but where CV is suspected (1), Group A was also compared with Group C, including patients with diseases mimicking CV, but without serum cryoglobulins.

Results: Six hundred forty-three patients were enrolled in 22 Centres (from Italy, Spain, France, Greece, Slovenia, Japan and Egypt). Major organizative/local issues did not allow American experts to participate. Group A comprised 268 patients with CV, Group B 182 controls with serum cryoglobulins without CV, and Group C 193 controls without serum cryoglobulins. Notably, 20 patients showed type I cryoglobulinemia, 13 in Group A, and 7 in Group B. Group C included 108/193 (55.9%) systemic vasculitides, 100/108  (92.6%) were small vessel vasculitides. The classification criteria [positivity of at least 2/3 items among questionnaire (≥2/3 positive questions), clinical item (≥3/4 clinical manifestations), laboratory (≥2/3 tests)] showed 89.9% (95% CI 86.1-93.6) of sensitivity and 93.5% (95% CI 89.7-97.2) of specificity, replicating previous results (1). Sensitivity of 91.7% and specificity of 100% were observed in the subgroup of  type I cryoglobulinemia. By the comparison of Group A vs. Group C, the Criteria showed a specificity 92.6% (88.8-96.5) and a sensitivity of 77.8% (72.6-83.0) when the laboratory item was positive (questionnaire + laboratory  item; or clinical + laboratory item).

Conclusion: the International Classification Criteria for the CV have been validated in a new real cohort. High specificity and sensitivity were confirmed. Notably, in patients where CV is suspected on clinical grounds, but where cryoglobulins are negative by initial testing, or not yet available (patients who cannot be classified as CV, as positive serum cryoglobulinemia is a conditio sine qua non for classification) (1), the Criteria appear relevant  to strengthen the suspicion for CV, and to optimize the follow-up.

1. De Vita S, et al. Ann Rheum Dis 2011;70(7):1183-90.

Disclosure:

L. Quartuccio,
None;

M. Isola,
None;

L. Corazza,
None;

S. Retamozo,
None;

M. A. M. El-Menyawi,
None;

E. Gremese,
None;

M. Sebastiani,
None;

N. Pipitone,
None;

T. Urraro,
None;

V. Conteduca,
None;

C. Koutsianas,
None;

B. Terrier,
None;

M. N. Zoheir,
None;

A. Ghinoi,
None;

D. Filippini,
None;

F. Saccardo,
None;

M. N. Salem,
None;

S. Scarpato,
None;

P. Fraticelli,
None;

A. Tavoni,
None;

E. Catarsi,
None;

C. Mazzaro,
None;

P. Pioltelli,
None;

M. Matar,
None;

P. Scaini,
None;

M. Tomsic,
None;

N. Nishimoto,

Bristol-Myers Squibb Japan,

2;

D. Vassilopoulos,
None;

M. Voulgarelis,
None;

G. M. Ragab,
None;

C. Salvarani,
None;

A. Gabrielli,
None;

P. Cacoub,
None;

L. Guillevin,
None;

D. Sansonno,
None;

A. L. Zignego,
None;

G. Ferraccioli,
None;

A. G. Tzioufas,
None;

M. Ramos-Casals,
None;

C. Ferri,
None;

M. Pietrogrande,
None;

G. Monti,
None;

M. Galli,
None;

S. Bombardieri,
None;

S. De Vita,
None.

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