Manzo Ciro, Kechida Melek
Internal and Geriatric Department ASL NA 3 sud, Rheumatologic Outpatient Clinic hospital "Mariano Lauro", Sant'Agnello, Italy.
Internal Medicine and Endocrinology Department, Fattouma Bourguiba University Hospital, Monastir, Tunisia.
Reumatologia. 2017;55(3):136-139. doi: 10.5114/reum.2017.68913. Epub 2017 Jul 18.
Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune disease with an elevated risk of developing lymphoproliferative malignancies (LM). Whether pSS is a risk factor or not for non-lymphoma malignancies (NLM) has been scarcely evaluated in the literature. Age is per se a risk factor for malignancies: patients over 70 years old have 4 times higher risk for cancers than adults. Even if the mean age of pSS onset usually is in the 4 and 5 decade, its onset in patients aged over 65 years (Elderly Onset pSS - EOpSS) is not uncommon.
To evaluate pSS as a risk factor for NLM we performed a systematic electronic search on PubMed in the period 2006-2016 to identify all the publications on this topic. The studies were eligible for inclusion if they reported specific Standardized Incidence Ratio (SIR) with 95% CI. Studies that did not report sufficient published and/or original data were excluded.
Only 7 articles of 494 that we found in PubMed fulfilled the inclusion criterion. In the vast majority of these, SIR values were not statistically significant for NLM. The occurrence of NLM after LM was statistically significant in some studies and a NLM represented the most frequent cause of death. The possibility that NLM may represent a paraneoplastic syndrome seems much more frequent than LM, the risk of which increases with time after the diagnosis. Data regarding the neoplastic weight of EOpSS are mainly pointed out by case reports.
Primary Sjögren's syndrome is not associated with an increased risk for NLM. However the possibility that NLM may appear after recovery from lymphoma should be carefully considered because it could be cause of the patient's death. Similarly the possibility that NLM may represent a paraneoplastic syndrome must be highlighted. The relationship between EOpSS and SIRs for NLM should be deepened with studies on ad hoc cohorts.
原发性干燥综合征（pSS）是一种慢性全身性自身免疫性疾病，发生淋巴增殖性恶性肿瘤（LM）的风险升高。pSS是否为非淋巴瘤恶性肿瘤（NLM）的危险因素在文献中鲜有评估。年龄本身就是恶性肿瘤的一个危险因素：70岁以上的患者患癌症的风险是成年人的4倍。即使pSS发病的平均年龄通常在40至50岁之间，但在65岁以上患者中发病（老年发病型pSS - EOpSS）并不罕见。
为评估pSS作为NLM的危险因素，我们于2006年至2016年期间在PubMed上进行了系统的电子检索，以确定所有关于该主题的出版物。如果研究报告了具有95%置信区间的特定标准化发病率（SIR），则符合纳入标准。未报告足够已发表和/或原始数据的研究被排除。
我们在PubMed中找到的494篇文章中，只有7篇符合纳入标准。在这些文章中的绝大多数中，NLM的SIR值无统计学意义。在一些研究中，LM后发生NLM具有统计学意义，且NLM是最常见的死亡原因。NLM可能代表副肿瘤综合征的可能性似乎比LM更常见，其风险在诊断后的时间里会增加。关于EOpSS肿瘤负担的数据主要由病例报告指出。
原发性干燥综合征与NLM风险增加无关。然而，应仔细考虑淋巴瘤康复后可能出现NLM的可能性，因为这可能是患者死亡的原因。同样，必须强调NLM可能代表副肿瘤综合征的可能性。应通过对特定队列的研究来加深EOpSS与NLM的SIR之间的关系。
