Session Information
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: Several neoplasia have been associated with Sjögren Syndrome (SS), being non-Hodgkin lymphoma the most frequent one. Our objective was to evaluate the incidence of all type of cancer in a cohort of patients with primary SS.
Methods: A retrospective descriptive study was performed in a university hospital with its own health insurance and captive population. Electronic medical records of SS patients were reviewed between 01/01/2000 and 12/31/2015. We included patients fulfilling ACR 2012 or American-European Consensus Group (AECG) 2002 SS criteria, or patients diagnosed as Primary SS by the treating rheumatologist. We registered and analyzed demographic, clinical and histopathologic information available on their medical records.
Incidence rate of cancer with their 95% CI was calculated. Patients were followed up until the end of study, death, loss of follow up, or when a diagnosis of cancer was performed. Incidence was compared with statistics on general population from United Kingdom (UK) as there is no full data available in the country where the study was performed.
Results: One hundred fifty-seven patients with Primary SS were included, and followed for 1158 patient/years. Table 1 shows patients’ characteristics. 15 patients developed a neoplasia during follow up: 3 lymphomas (two MALT lymphomas of the parotid and one disseminated non-Hodgkin lymphoma), 1 Multiple Myeloma, 4 Skin (non – melanoma) neoplasia and 7 solid organ cancer (4 Breast, 1 Lung, 1 Uterus, 1 Tongue). Table 2 shows the cohort cancer incidence rates and comparison with statistics from general population. Non- Hodgkin Lymphomas showed an increased incidence in SS patients. All 3 patients with lymphoma were females, with anemia, leukopenia, thrombocytopenia and high erythrocyte sedimentation rate. In multivariate logistic regression analysis only thrombocytopenia was associated with an increased risk of cancer in general (OR 7, CI 95% 1.9-25.7).
Conclusion: We only found an increased rate of non- Hodgkin lymphomas in this cohort of SS patients. Other types of cancer incidence rates were in ranges expected for general population
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Table 1. SS patients’ characteristics
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SS patients (n=157) |
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Females, n (%) |
150 (95.5) |
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Mean age at diagnosis, years (SD) |
49.4 (19) |
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Dry eyes, n (%) |
152 (96.8) |
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Dry mouth, n (%) |
139 (89.1) |
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Salivary gland enlargement, n (%) |
26 (16.6) |
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Arthralgia, n (%) |
106 (67.5) |
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Arthritis, n (%) |
14 (9) |
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ANA +, n (%) |
153 (96.8) |
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Anti Ro +, n (%) |
149 (94.9) |
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Anti La +, n (%) |
94 (60.3) |
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Rheumatoid Factor +, n (%) |
66 (44.9) |
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Anti CCP +, n/n performed (%) |
3/45 (6.7) |
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Low C3/C4, n/n performed (%) |
30/111 (27) |
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Elevated erythrocyte sedimentation rate, n (%) |
130 (83.3) |
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Hypergammaglobulinemia, n (%) |
95 (63) |
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Cryoglobulinemia +, n/n performed (%) |
5/50 (10) |
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Anemia, n (%) |
47 (30.1) |
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Leukopenia, n (%) |
51 (32.7) |
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Thrombocytopenia, n (%) |
23 (14.7) |
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Compatible Salivary Glands biopsy, n/n performed (%) |
24/32 (75) |
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Confirmatory dry eye test, n/n performed (%) |
49/68 (72) |
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Follow up in years, median (IQR) |
7.7 (8) |
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Deaths during follow up, n (%) |
2 (1.2) |
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Table 2. Neoplasia incidence rate in this SS cohort compared to general statistics |
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Neoplasia |
Incidence rate in this SS cohort per 100,000 person/years (95% CI) |
Incidence in general Population per 100,000 person/years (95% CI) |
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Non Hodgkin Lymphomas |
260 (50 – 750) |
22.9 (22.5-23.3)* |
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Multiple Myeloma |
86 (2- 480) |
9.3 (9.1-9.6) * |
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Skin (non – melanoma) |
340 (94- 880) |
223.6 (222.4-224.8) * |
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Breast |
340 (94- 880) |
172 (170-173)** |
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Lung |
86 (2- 480) |
79.3 (78.6-80)* |
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Uterus |
86 (2- 480) |
29.8 (29.2-30.4)* |
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Tongue |
86 (2- 480) |
19.2 (18.8-19.5)*** |
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* UK incidence data **UK incidence in women *** UK Head and neck cancer incidence rate |
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To cite this abstract in AMA style:
Brom M, Moyano S, Scolnik M, Soriano ER. Incidence of Cancer in a Cohort of Patients with Primary Sjögren Syndrome [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/incidence-of-cancer-in-a-cohort-of-patients-with-primary-sjogren-syndrome/. Accessed .« Back to 2017 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/incidence-of-cancer-in-a-cohort-of-patients-with-primary-sjogren-syndrome/