Braun Jean Jacques, Gentine André, Pauli Gabrielle
Service ORL Hôpital de Hautepierre, Strasbourg, France.
Laryngoscope. 2004 Nov;114(11):1960-3. doi: 10.1097/01.mlg.0000147928.06390.db.
Sinonasal sarcoidosis remains a poorly understood and uncommon chronic granulomatous disease of unclear origin. We have attempted to characterize the main clinical and radiologic criteria for diagnosis and to discuss the treatment.
A retrospective study of 15 cases of chronic, symptomatic, and biopsy-proven sinonasal sarcoidosis and a review of the literature are realized.
Among the 15 patients, there were 8 women and 7 men with a mean age of 44 years. The most frequent presentation was a chronic, often crusty, rarely destructive inflammatory rhinosinusitis with nodules on the septum and/or the turbinates. Pulmonary sarcoidosis was associated in 12 cases. Involvement of the nasopharynx, the pharyngolarynx, the skin, the lachrymal and salivary glands, and the liver was associated in some cases. Levels of angiotensin-converting enzyme were elevated in 10 cases and normal in 3 cases. Gallium scan performed in three cases was positive. Radiologic studies showed nodules on the septum and/or the turbinates in 14 cases, complete or subtotal opacification of the sinuses and/or the nasal cavities in 13 cases, and nasopharyngeal or pharyngolaryngeal lesions in 4 cases. Treatment with corticosteroids, methotrexate, azathioprine, and surgery appear globally disappointing in view of the side effects and the relapses during a long follow-up (3-15 yr; mean, 6 yr).
On the basis of this study, we propose the following diagnostic criteria: 1) histopathologic confirmation of noncaseating granuloma; 2) chronic rhinosinusitis poorly responsive to conventional treatment and radiologic evidence of rhinosinusitis, often with nodules on the septum and/or the turbinates; 3) elevated level of angiotensin-converting enzyme; 4) positive gallium scan (if performed); 5) frequent evidence of systemic, especially pulmonary, sarcoidosis; 6) no evidence of other granulomatous diseases, such as Wegener granulomatosis.
鼻窦结节病仍是一种起源不明、了解甚少且不常见的慢性肉芽肿性疾病。我们试图明确其主要的临床和放射学诊断标准并探讨治疗方法。
对15例经活检证实的慢性、有症状的鼻窦结节病患者进行回顾性研究,并对相关文献进行综述。
15例患者中,女性8例,男性7例,平均年龄44岁。最常见的表现为慢性、常有痂皮、极少有破坏性的炎性鼻窦炎,伴有鼻中隔和/或鼻甲结节。12例患者合并有肺部结节病。部分病例还伴有鼻咽部、咽喉部、皮肤、泪腺和唾液腺以及肝脏受累。10例患者血管紧张素转换酶水平升高,3例正常。3例患者进行的镓扫描呈阳性。放射学检查显示:14例患者鼻中隔和/或鼻甲有结节,13例患者鼻窦和/或鼻腔完全或部分混浊,4例患者有鼻咽部或咽喉部病变。鉴于长期随访(3 - 15年;平均6年)期间出现的副作用和复发情况,使用皮质类固醇、甲氨蝶呤、硫唑嘌呤及手术治疗总体效果令人失望。
基于本研究,我们提出以下诊断标准:1)非干酪样肉芽肿的组织病理学证实;2)对传统治疗反应不佳的慢性鼻窦炎及鼻窦炎的放射学证据,通常伴有鼻中隔和/或鼻甲结节;3)血管紧张素转换酶水平升高;4)镓扫描阳性(如果进行了该检查);5)常有系统性尤其是肺部结节病的证据;6)无其他肉芽肿性疾病的证据,如韦格纳肉芽肿。