From the Institute for Systemic Autoimmune and Neurological Diseases; Department of Oral Medicine and Oral Pathology, Faculty of Dentistry, School of Health Sciences, National and Kapodistrian University of Athens; Department of Pathophysiology, and Department of Physiology, Medical School, National and Kapodistrian University of Athens; Academy of Athens, Athens, Greece.
E. Zampeli, MD, Institute for Systemic Autoimmune and Neurological Diseases; E.M. Kalogirou, DDS, Department of Oral Medicine and Oral Pathology, Faculty of Dentistry, School of Health Sciences, National and Kapodistrian University of Athens; E. Piperi, DDS, Department of Oral Medicine and Oral Pathology, Faculty of Dentistry, School of Health Sciences, National and Kapodistrian University of Athens; C.P. Mavragani, MD, Department of Pathophysiology, and Department of Physiology, Medical School, National and Kapodistrian University of Athens; H.M. Moutsopoulos, MD, FACP, FRCP (hc), Master ACR, Institute for Systemic Autoimmune and Neurological Diseases, and Academy of Athens.
J Rheumatol. 2018 Nov;45(11):1565-1571. doi: 10.3899/jrheum.180101. Epub 2018 Jul 15.
Primary Sjögren syndrome (pSS), an autoimmune epithelitis, bears the risk of evolving to non-Hodgkin lymphoma and most frequently to the mucosa-associated lymphoid tissue (MALT) subtype. Based on the observation that pSS patients with MALT present a more atrophic and more intensely fissured tongue, we aimed to semiquantify severity of tongue atrophy and clinically assess lingual appearance in pSS patients with and without MALT, and investigate whether tongue atrophy and fissured appearance could serve as clinical indicators/signs of MALT.
A blinded complete oral examination was performed in pSS patients with and without MALT. Tongue atrophy was scored using a semiquantified atrophy score. Clinical and laboratory variables were recorded for all patients.
After excluding pSS patients with oral candidiasis, iron deficiency, and megaloblastic anemia, 19 pSS patients with salivary MALT were matched 1:3 for age, sex, and disease duration with 57 pSS patients without MALT. The pSS-MALT patients had increased prevalence of salivary gland enlargement, lymphadenopathy, monoclonal gammopathy, rheumatoid factor positivity, higher focus and Tarpley scores in the minor salivary gland biopsy, and hyposalivation, compared to the pSS non-MALT patients. A significantly higher prevalence of tongue atrophy (68% vs 30%, p = 0.006) and fissured tongue (89% vs 33%, p < 0.001) was observed in the former group. Multivariate analysis showed that fissured tongue appearance, hyposalivation, and lymphadenopathy associate independently with salivary MALT in pSS.
These results suggest that pSS patients with lymphoid malignancy exhibit a more atrophic and more fissured tongue. This particular clinical tongue appearance can serve as an additional clinical sign for salivary MALT lymphoma in pSS patients.
原发性干燥综合征（pSS）是一种自身免疫性上皮炎，有发展为非霍奇金淋巴瘤的风险，且最常发展为黏膜相关淋巴组织（MALT）型。基于这样的观察结果，即 MALT 型 pSS 患者的舌更萎缩且更有裂，我们旨在半定量评估 pSS 患者伴发和不伴发 MALT 时的舌萎缩严重程度，并临床评估其舌外观，并探讨舌萎缩和裂是否可作为 MALT 的临床指标/征象。
对伴发和不伴发 MALT 的 pSS 患者进行盲法全口腔检查。使用半定量萎缩评分评估舌萎缩程度。记录所有患者的临床和实验室变量。
排除伴口腔念珠菌病、缺铁和巨幼细胞性贫血的 pSS 患者后，19 例伴唾液腺 MALT 的 pSS 患者按年龄、性别和疾病持续时间 1:3 与 57 例不伴 MALT 的 pSS 患者相匹配。与 pSS 非 MALT 患者相比，pSS-MALT 患者的唾液腺肿大、淋巴结病、单克隆丙种球蛋白血症、类风湿因子阳性、小唾液腺活检中的焦点和 Tarpley 评分更高以及唾液分泌减少的发生率更高。前者的舌萎缩（68%比 30%，p = 0.006）和裂纹舌（89%比 33%，p < 0.001）的发生率显著更高。多变量分析显示，裂纹舌外观、唾液分泌减少和淋巴结病与 pSS 中的唾液腺 MALT 独立相关。
这些结果表明，有淋巴恶性肿瘤的 pSS 患者表现出更萎缩和更有裂的舌。这种特殊的临床舌外观可作为 pSS 患者唾液腺 MALT 淋巴瘤的附加临床征象。
