Manzo Ciro, Martinez-Suarez Eva, Kechida Melek, Isetta Marco, Serra-Mestres Jordi
Rheumatologic Outpatient Clinic and Gerontorheumatology Service, "Mariano Lauro" Hospital, 80065 Sant'Agnello, Italy.
Center for Cognitive Diseases and Dementias, Azienda Sanitaria Locale Napoli 3 sud, 80038 Pomigliano d'Arco, Italy.
Brain Sci. 2019 Apr 15;9(4):85. doi: 10.3390/brainsci9040085.
Cognitive disorders are reported to be common in patients with primary Sjogren's syndrome (pSS). In some cases, they are the first clinical manifestation, preceding the diagnosis of pSS by two years on average.
A systematic review was conducted to explore cognitive impairment in pSS, with reference to diagnostic methods and their relationship with laboratory data and clinical manifestations.
According to the PRISMA 2009 checklist, we carried out a comprehensive literature search in the three main bibliographic databases: MEDLINE, EMBASE, and PsycINFO (NICE HDAS interface). The following main search terms were used: primary Sjogren syndrome, neurological manifestations, fatigue, cognitive functions, psychiatric manifestations, mild cognitive impairment, dementia, and neurocognitive disorder. The search was made on 14 September, 2018. References from all selected studies were also examined. Inclusion criteria were: all studies and case-reports published in any language from 2002 that assessed the association of pSS (according to classification criteria proposed by the 2002 American/European collaborative group (AECG)) with all types of cognitive impairment (including dementia). Exclusion criteria were: reviews, abstracts, secondary Sjögren's syndrome (SS), and all articles in which other classification criteria were used.
The initial search yielded 352 articles, of which 253 were excluded based on the title and abstract review. A total of 54 articles underwent a full-length review, and 32 articles were excluded. Data were extracted from 18 studies and three case-reports involving a total of 6196 participants. In most cases, cognitive dysfunction was a brain fog or a mild cognitive impairment (MCI). Occasionally, an autoimmune dementia was present. The relationship between pSS and degenerative dementias, such as Alzheimer's disease (AD), was a controversial issue, even if some investigators hypothesized that pSS could be a risk factor. Several unmet needs were highlighted. First, some of the included studies had not reported the severity of pSS; hence, few correlations between disease severity and cognitive function were possible. Secondly, the evaluation of the pathogenetic role of comorbid diseases was often absent. The lack of information on the type of dementia represented a third critical point in the majority of the included studies.
This systematic review confirmed that adequate studies on cognitive function in pSS are scarce, mostly performed on small-sized samples, and often conflicting. The routine assessment of cognitive function in patients with pSS seems advisable and it will help to elucidate some of the unmet needs highlighted by this review in future appropriately designed studies.
据报道，认知障碍在原发性干燥综合征（pSS）患者中很常见。在某些情况下，它们是首发临床表现，平均比pSS诊断提前两年出现。
进行一项系统评价，以探讨pSS中的认知障碍，参考诊断方法及其与实验室数据和临床表现的关系。
根据PRISMA 2009清单，我们在三个主要文献数据库中进行了全面的文献检索：MEDLINE、EMBASE和PsycINFO（NICE HDAS界面）。使用了以下主要检索词：原发性干燥综合征、神经学表现、疲劳、认知功能、精神表现、轻度认知障碍、痴呆和神经认知障碍。检索于2018年9月14日进行。还检查了所有选定研究的参考文献。纳入标准为：2002年以来以任何语言发表的所有研究和病例报告，评估pSS（根据2002年美国/欧洲协作组（AECG）提出的分类标准）与所有类型认知障碍（包括痴呆）的关联。排除标准为：综述、摘要、继发性干燥综合征（SS）以及所有使用其他分类标准的文章。
初步检索得到352篇文章，其中253篇基于标题和摘要审查被排除。共54篇文章进行了全文审查，32篇文章被排除。从18项研究和3篇病例报告中提取数据，共涉及6196名参与者。在大多数情况下，认知功能障碍表现为脑雾或轻度认知障碍（MCI）。偶尔会出现自身免疫性痴呆。pSS与退行性痴呆（如阿尔茨海默病（AD））之间的关系是一个有争议的问题，尽管一些研究人员推测pSS可能是一个风险因素。突出了几个未满足的需求。首先，一些纳入研究未报告pSS的严重程度；因此，疾病严重程度与认知功能之间几乎没有相关性。其次，通常缺乏对合并症致病作用的评估。在大多数纳入研究中，缺乏关于痴呆类型的信息是第三个关键点。
这项系统评价证实，关于pSS认知功能的充分研究很少，大多在小样本上进行，且结果常常相互矛盾。对pSS患者进行认知功能的常规评估似乎是可取的，这将有助于在未来适当设计的研究中阐明本评价所强调的一些未满足的需求。
