Hajas Agota, Szodoray Peter, Barath Sandor, Sipka Sandor, Rezes Szilard, Zeher Margit, Sziklai Istvan, Szegedi Gyula, Bodolay Edit
Division of Clinical Immunology, 3rd Department of Medicine, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary.
J Rheumatol. 2009 Sep;36(9):1930-6. doi: 10.3899/jrheum.081314. Epub 2009 Aug 14.
To investigate the frequency of sensorineural hearing loss (SNHL) in patients with mixed connective tissue disease (MCTD).
The study population consisted of 71 patients with MCTD (69 female; 2 male), with a mean age of 57.1 +/- 7.9 years and a mean disease duration of 14.5 +/- 8.0 years. All patients underwent audiological evaluation that included pure tone and speech audiometry. In addition, the systemic manifestations of the disease and drug therapy were recorded. All patients were tested for presence of autoantibodies. Fifty-one age-matched healthy subjects served as controls.
SNHL was found in 33 (46.4%) of the 71 patients with MCTD. There was no correlation between SNHL and age and disease duration. An association was found between Raynaud's phenomenon (p < 0.03), secondary antiphospholipid syndrome (APS) (p < 0.05), and SNHL. MCTD patients with SNHL had higher serum levels of anti-U1RNP (p < 0.05), antiendothelial cell antibodies (p < 0.001), and IgG type anticardiolipin antibodies (p < 0.0001) than patients without SNHL. Serum levels of interferon-gamma and tumor necrosis factor-alpha were increased in MCTD patients with SNHL compared to patients without SNHL. The absolute number of natural (CD4+CD25(high)FoxP+) regulatory T cells (Treg) was lower compared to patients without SNHL.
In MCTD, SNHL is a specific organ manifestation and appears frequently. We have found that pathogenic autoantibodies, decreased levels of regulatory T cells, and overexpression of proinflammatory cytokines may play a role in the pathogenesis of immune mediated inner ear disorders in MCTD.
研究混合性结缔组织病（MCTD）患者感音神经性听力损失（SNHL）的发生率。
研究对象为71例MCTD患者（69例女性，2例男性），平均年龄57.1±7.9岁，平均病程14.5±8.0年。所有患者均接受了包括纯音和言语测听在内的听力学评估。此外，记录了疾病的全身表现和药物治疗情况。所有患者均检测了自身抗体。51名年龄匹配的健康受试者作为对照。
71例MCTD患者中有33例（46.4%）发现SNHL。SNHL与年龄和病程之间无相关性。发现雷诺现象（p<0.03）、继发性抗磷脂综合征（APS）（p<0.05）与SNHL之间存在关联。与无SNHL的MCTD患者相比，有SNHL的MCTD患者血清抗U1RNP水平（p<0.05）、抗内皮细胞抗体水平（p<0.001）和IgG型抗心磷脂抗体水平（p<0.0001）更高。与无SNHL的MCTD患者相比，有SNHL的MCTD患者血清干扰素-γ和肿瘤坏死因子-α水平升高。与无SNHL的患者相比，自然（CD4+CD25（高）FoxP+）调节性T细胞（Treg）的绝对数量更低。
在MCTD中，SNHL是一种特定的器官表现且频繁出现。我们发现致病性自身抗体、调节性T细胞水平降低以及促炎细胞因子的过度表达可能在MCTD免疫介导的内耳疾病发病机制中起作用。
