Buehring I, Friedrich B, Schaaf J, Schmidt H, Ahrens P, Zielen S
Department of Pediatrics, Johann Wolfgang Goethe Universität, Frankfurt, Germany.
Clin Exp Immunol. 1997 Sep;109(3):468-72. doi: 10.1046/j.1365-2249.1997.4831379.x.
Chronic refractory sinusitis is a common feature in patients with primary immunodeficiencies. The efficacy of standard therapeutic strategies is questionable. In an open trial we evaluated the efficacy of azithromycin, N-acetylcysteine and topical intranasal beclomethasone (100 microg twice daily for 6 weeks) in 16 patients with primary immunodeficiencies (median age 13.5 years, range 5-32 years). All patients suffered from chronic sinusitis despite regular immunoglobulin replacement therapy every 3 weeks. Magnetic resonance imaging (MRI) scans were performed before and after 6 weeks of treatment to evaluate morphological changes in the paranasal sinuses. Nasal swabs and washings were taken for microbial analysis and measurement of inflammatory mediators (IL-8, tumour necrosis factor-alpha (TNF-alpha), eosinophilic cationic protein (ECP)) before and post therapy. Inflammatory mediators in nasal secretions were significantly elevated in patients: IL-8 median 2436 pg/ml (range 441-5435 pg/ml), TNF-alpha 37.3 pg/ml (3.75-524 pg/ml) and ECP 33 ng/ml (1.5-250 ng/ml) versus age-matched healthy controls: IL-8 median 212 pg/ml (99-825 pg/ml), TNF-alpha 3.77 pg/ml (2.8-10.2 pg/ml) and ECP 1.5 ng/ml (1.5-14.8 ng/ml) (P < 0.0001). Inflammation of the maxillary sinuses was confirmed by MRI scans in all patients, additionally infection of the ethmoidal and frontal sinuses was recorded in five patients. Bacterial growth appeared in 11 out of 16 cultures. In spite of therapy, no improvement in sinal inflammation visualized by MRI was achieved. Moreover, no significant decrease in pathogens and levels of inflammatory mediators could be detected (IL-8 1141 pg/ml, 426-4556 pg/ml; TNF-alpha 13.9 pg/ml, 4.1-291.6 pg/ml; ECP 32.3 ng/ml, 3.7-58.4 ng/ml). Our results demonstrate that conventional management of sinusitis is of little benefit in patients with chronic refractory sinusitis with an underlying immunodeficiency. More studies are needed to test antibiotic regimens, probably combined with surgical drainage and anti-inflammatory agents.
慢性难治性鼻窦炎是原发性免疫缺陷患者的常见特征。标准治疗策略的疗效值得怀疑。在一项开放性试验中，我们评估了阿奇霉素、N-乙酰半胱氨酸和鼻内局部用倍氯米松（每日两次，每次100微克，共6周）对16例原发性免疫缺陷患者（中位年龄13.5岁，范围5 - 32岁）的疗效。尽管每3周进行一次规律的免疫球蛋白替代治疗，但所有患者均患有慢性鼻窦炎。在治疗6周前后进行磁共振成像（MRI）扫描，以评估鼻窦的形态变化。在治疗前后采集鼻拭子和冲洗液进行微生物分析及炎症介质（白细胞介素-8（IL-8）、肿瘤坏死因子-α（TNF-α）、嗜酸性阳离子蛋白（ECP））的检测。患者鼻分泌物中的炎症介质显著升高：IL-8中位值为2436皮克/毫升（范围441 - 5435皮克/毫升），TNF-α为37.3皮克/毫升（3.75 - 524皮克/毫升），ECP为33纳克/毫升（1.5 - 250纳克/毫升）；而年龄匹配的健康对照者：IL-8中位值为212皮克/毫升（99 - 825皮克/毫升），TNF-α为3.77皮克/毫升（2.8 - 10.2皮克/毫升），ECP为1.5纳克/毫升（1.
