Background The most severe complication of Sjögren Syndrome is the development of lymphoproliferative processes. Several neoplasia have been associated with the disease, being non-Hodgkin lymphoma the most frequent one.
Objectives Our objective was to evaluate incidence of cancer in a cohort of patients with primary Sjögren Syndrome.
Methods A retrospective descriptive study was performed in a university hospital with its own health insurance and captive population. Using electronic medical records and laboratory database were review the entries performed between 01/01/2000 and 12/31/2015. We analyzed those patients with either diagnosis of Sjögren Syndrome, complain of dry mouth/eyes, or positive antibodies anti-Ro/SSA and anti-LA/SSB.
Among these patients, we included those fulfilling either ACR 2012 or EULAR 2002 Sjögren criteria, or those who were diagnosed as Primary Sjögren Syndrome by the treating rheumatologist even if they did not fulfill criteria.
We then proceeded to register and analyze demographic, clinical and histopathologic information available on their clinical records.
Results One hundred fifty-seven patients with Primary Sjögren Syndrome were identified. Female accounted for 95.5% of the cohort; mean age at diagnosis was 49.4 years (SD 19). Median follow-up time was 7.7 years (IQR 8). The development rate and type of neoplasia was the following:
Lymphomas: Three (Two MALT lymphomas of the parotid and one disseminated non-Hodgkin lymphoma). Density of Incidence 260/100,000 person/year (CI 95%: 50 – 750/100,000 person/year)
Multiple Myeloma: One
Skin (non-melanoma) neoplasia: Four
Solid organ Neoplasia: Seven (Four breast cancer, one lung cancer, one uterus cancer, one tongue cancer). Density of Incidence 600/100,000 person/year (CI 95% 240 – 1240/100,000 person/year)
Univariate analysis showed association between lymphoma and cryoglobulinemia (p=0.01; OR=5,8), low C4 fraction of complement (p=0.01; OR=5,1), anemia (p=0.02; OR=1,96) and leucopenia (p=0.03; OR=1,67)
Conclusions Development of cancer is a known complication of Primary Sjögren Syndrome. The association between lymphoma and cryoglobulinemia, low C4 fraction of complement, anemia and leucopenia enhances the importance of periodic screening for neoplasms among this subgroup of patients with Primary Sjögren Syndrome.
Disclosure of Interest None declared