Background CrCryoglobulinemia is a known risk factor lymphoma in primary Sjögren's syndrome (pSS) and it is an hallmark of a more aggressive systemic disease. Whether this is related to the sole presence of serum cryoglobulins (SC) or also to an associated cryoglobulinemic vasculitis (CV) is unknown, since criteria for the classification of the latter were developed and validated only very recently (1,2).
Objectives The aim of this study is to describe the characteristics of cryoglobulinemia in the course of pSS and to evaluate the association with CV, classified according to the validated criteria (1,2), and with B-cell lymphoma.
Methods 144 consecutive patients with pSS were repeatedly evaluated for SC according to the standard methods (3). The 2011 classification criteria for CV and the presence of lymphoma were investigated in positive cases.
Results SC were repeatedly present in 28/144 patients (19.4%), with a median of three determinations (range 2-33) per patient. In 14/28 cases (50%), the presence of SC was fluctuating, while in 14/28 cases (50%) SC were persistently positive in all the tests performed. SC were type II in 11/28 (39.3%) patients (8/11 persistently positive, 3/11 fluctuating), and type III in 7/28 patients (2/7 persistently positive, 5/7 fluctuating); in 10/28 cases, SC typing was not performed for the insufficient cryocrit (4/10 persistent, 6/10 fluctuating). Patients with positive SC fulfilled the CV classification criteria in 16/28 cases (57.1%). CV was more frequent in pSS with persistent SC (11/14, 78,6%) than pSS with fluctuating SC (5/14, 35,7%) (p=0,02, Pearson). Lymphoma was observed in 4/12 (33.3%) pSS patients with SC without CV, and in 6/16 (54.5%) pSS patients with CV.
Conclusions Persistently detectable, rather than fluctuating SC, are more associated with CV and with malignant lymphoma in pSS. The repetition of SC testing is then recommended in pSS.
De Vita S, et al. Preliminary classification criteria for the cryoglobulinaemic vasculitis. Ann Rheum Dis. 2011;70(7):1183-90.
Quartuccio L, et al. Validation of the classification criteria for cryoglobulinaemic vasculitis. Rheumatology (Oxford). 2014;53(12):2209-13.
Brouet JC, et al. Biologic and clinical significance of cryoglobulins. A report of 86 cases. Am J Med 1974;57:775-88.
Disclosure of Interest None declared