Poor Prognosis of Patients with Primary Sjögren Syndrome Who Fulfilled at Diagnosis the Classification Criteria for Concomitant Cryoglobulinemic Vasculitis

Soledad Retamozo^1,2, Hoda Gheitasi³, Luca Quartuccio⁴, Belchin Kostov⁵, Laura Corazza⁴, Albert Bové³, Antoni Sisó-Almirall⁵, Myriam Gandía³, Manuel Ramos-Casals¹, Salvatore De Vita⁶ and Pilar Brito-Zerón¹, ¹Department of Autoimmune Diseases, CELLEX-IDIBAPS, Hospital Clínic, Barcelona, Barcelona, Spain, ²Rheumatology Unit, Hospital Privado Centro Médico de Córdoba, Córdoba, Argentina, ³Department of Autoimmune Diseases, IDIBAPS, Hospital Clínic, Barcelona, Spain, ⁴S. Maria della Misericordia, University of Udine, Italy, Udine, Italy, ⁵Primary Care Research Group, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Primary Care Centre Les Corts, CAPSBE, Barcelona, Spain, ⁶Santa Maria della Misericordia Hospital, University of Udine, Udine, Italy

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Cryoglobulinemia, death and vasculitis, Sjogren's syndrome

Session Information

Date: Sunday, November 8, 2015

Title: Sjögren's Syndrome Poster I: Clinical Insights into Sjögren's Syndrome

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose:

To evaluate the fulfilment of classification criteria for cryoglobulinemic vasculitis (CV) at diagnosis in a large cohort of patients with primary Sjögren syndrome (SS) and their correlation with poor outcomes.

Methods:

We included 515 consecutive patients tested for serum cryoglobulins who fulfilled the 2002 classification criteria for primary SS. CV classification criteria and serum cryoglobulins at diagnosis were assessed as predictors of death and lymphoma using Cox proportional-hazards regression analysis adjusted for age and gender.

Results:

Positive serum cryoglobulins were detected in 65 (12%) patients, of whom 21 (32%) fulfilled CV classification criteria. Patients with CV had a higher frequency of type II cryoglobulinemia (86% vs. 43%, p=0.04), a higher mean cryocrit level (6.58% vs. 1.25%, p<0.001), and a higher cumulated mean ESSDAI score (35.3 vs. 16.2, p<0.001) in comparison with patients carrying serum cryoglobulins who did not fulfill CV criteria. After a mean follow-up of 110 months, 45 (9%) patients developed B-cell lymphoma and 33 (6%) died. In comparison with patients without cryoglobulinemia, patients with cryoglobulins showed a high risk of development of B-cell lymphoma; the risk was higher for those who fulfilled the CV criteria (HR 7.47, CI95% 3.38-16.53) than for those who did not fulfill the CV criteria (HR 2.56, CI95% 1.03-6.35); statistical differences were found in the univariate analysis, but were not confirmed by the multivariate models. With respect to mortality, patients with CV had a higher risk of death in both the univariate (HR 11.68, CI95% 4.44-30.74) and multivariate (HR 4.36, CI95% 1.32-14.47) adjusted models in comparison with those without cryoglobulins.

Conclusion:

Patients with primary SS who fulfilled the classification criteria for cryoglobulinemic vasculitis at diagnosis are at higher risk of death.

Disclosure: S. Retamozo, None; H. Gheitasi, None; L. Quartuccio, None; B. Kostov, None; L. Corazza, None; A. Bové, None; A. Sisó-Almirall, None; M. Gandía, None; M. Ramos-Casals, Bristol-Myers Squibb, 2; S. De Vita, None; P. Brito-Zerón, None.

To cite this abstract in AMA style:

Retamozo S, Gheitasi H, Quartuccio L, Kostov B, Corazza L, Bové A, Sisó-Almirall A, Gandía M, Ramos-Casals M, De Vita S, Brito-Zerón P. Poor Prognosis of Patients with Primary Sjögren Syndrome Who Fulfilled at Diagnosis the Classification Criteria for Concomitant Cryoglobulinemic Vasculitis [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/poor-prognosis-of-patients-with-primary-sjogren-syndrome-who-fulfilled-at-diagnosis-the-classification-criteria-for-concomitant-cryoglobulinemic-vasculitis/. Accessed .

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