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OP0274 Cryoglobulinemic Vasculitis and Primary sjögren's Syndrome are Independent Risk Factors for Lymphoma in a Large Worldwide Population of Patients with Positive Serum Cryoglobulins
  1. L. Quartuccio1,
  2. L. Corazza1,
  3. M. Ramos-Casals2,
  4. S. Retamozo2,
  5. G.M. Ragab3,
  6. G. Ferraccioli4,
  7. E. Gremese4,
  8. A. Tzioufas5,
  9. M. Voulgarelis6,
  10. D. Vassilopoulos7,
  11. C. Koutsianas7,
  12. S. Scarpato8,
  13. C. Salvarani9,
  14. L. Guillevin10,
  15. B. Terrier10,
  16. P. Cacoub11,
  17. F. Saccardo12,
  18. A. Gabrielli13,
  19. P. Fraticelli13,
  20. M. Tomsic14,
  21. A. Tavoni15,
  22. N. Nishimoto16,
  23. D. Filippini17,
  24. P. Scaini18,
  25. A.L. Zignego19,
  26. C. Ferri20,
  27. D. Sansonno21,
  28. G. Monti12,
  29. M. Pietrogrande22,
  30. M. Galli23,
  31. S. Bombardieri24,
  32. S. De Vita25
  1. 1Department of Medical and Biological Sciences, Rheumatology Clinic, University Hospital of Udine, Udine, Italy
  2. 2Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, Institutd'Investigacions Biomèdiques August Pi iSunyer (IDIBAPS), Barcelona, Spain
  3. 3Faculty of Medicine – Cairo University, Cairo, Egypt
  4. 4Rheumatology Clinic, Catholic University of Sacred Heart, Rome, Italy
  5. 5Faculty of Medicine, University of Athens
  6. 6School of Medicine National University of Athens
  7. 72nd Department of Medicine and Laboratory, Clinical Immmunology-Rheumatology Unit, Athens, Greece
  8. 8Rheumatology Unit, Salerno
  9. 9Rheumatology Unit, Arcispedale S. Maria Nuova, Reggio Emilia, Italy
  10. 10Centre de Références Maladies Auto-Immunes, Service de Médecine Interne II, Hôpital Pitié-Salpêtrière
  11. 11Service de Médecine Interne, Hôpital Cochin, Paris, France
  12. 12Internal Medicine, Saronno
  13. 13Internal Medicine, Università Politecnica delle Marche, Ancona, Italy
  14. 14Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia
  15. 15Rheumatology Unit, Department of Internal Medicine, Pisa, Italy
  16. 16Department of Molecular Regulation for Intractable Diseases, Institute of Medical Science, Tokyo Medical University, Tokyo, Japan
  17. 17Rheumatology Unit, Niguarda Ca' Granda Hospital, Milan
  18. 18Nephrology Clinic, Brescia
  19. 19Center for Systemic Manifestations of Hepatitis Viruses (MaSVE), Department of Internal Medicine, University of Florence, Florence
  20. 20Rheumatology Unit, Department of Internal Medicine, University of Modena, Modena
  21. 21Section of Internal Medicine and Clinical Oncology, Department of Biomedical Sciences and Human Oncology, University of Bari, Medical School, Bari
  22. 22Internal Medicine, Zingonia (BG)
  23. 23Institute of Infectious Diseases, University of Milan, Milan
  24. 24Rheumatology Unit, University of Pisa, Pisa
  25. 25Rheumatology Clinic, DSMB, University of Udine, Udine, Italy


Background Serum cryoglobulins (SC) may be found in many diseases (1), and the presence of serum cryoglobulins is a known risk factor for lymphoma evolution in some non malignant diseases.

Objectives The aim of this study was to distiguish the role of cryoglobulinemic vasculitis (CV), classified according to the recent validated criteria (1,2), and primary Sjögren's syndrome (pSS) as risk factors of lymphoma in patients positive serum cryoglobulins. Importantly, SC, CV and pSS may occur together.

Methods 950 charts from consecutive patients with positive SC were evaluated. Patients carrying both pSS and HCV infection, as well as incomplete charts, were excluded.

Results 657 patients with SC were selected, 374 with CV and 283 without CV, according to the published criteria (2,3). PSS, classified according to the American-European Group Criteria was present in 96 patients (44 with CV, 52 without). Lymphoma was reported in 61/657 (9.8%) patients with SC. Among them, CV was present in 44/61 (72,1%; 14 also with pSS), and pSS in 17/61 (27,9%; and 14/17 had CV). Patients with SC with CV showed an higher prevalence of lymphoma than patients with SC without CV (44/374, 11.5% vs.17/283, 6.3%; p=0.025, OR=1.93 [95%IC: 1.08-3.39]. Patients with pSS, SC and CV also showed a higher prevalence of lymphoma than patients with pSS, SC but without CV (14/44, 31.8% vs. 3/52, 7.4%; p=0.001, OR=7.62 [95%CI 2.02-28.74]. CV and pSS were confirmed as independent risk factor for lymphoma by multivariate analysis (OR 2,18 95%CI 1,18-3,83, p=0,012; OR 2,65 95%CI 1,04-6,76, p=0,042, respectively). Infection by the hepatitis C virus (HCV) was detected in 467/561 (83,2%) patients with SC without pSS, and did not statistically predispose to lymphoma when associated with CV in this subset (p=1,0).

Conclusions Cryoglobulinemic vasculitis and pSS are independent risk factors for lymphoma in patients with evidence of SC. Patients with both the conditions (CV and pSS) have the highest risk. In the follow-up of SC positive patients, a very high attention should be deserved to pSS, in particular when CV is present.


  1. De Vita S, et al. Ann Rheum Dis. 2011; 2) Quartuccio L, et al. Rheumatology (Oxford). 2014

Disclosure of Interest None declared

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