Szyfter W, Wierzbicka M
Kliniki Otolaryngologii Katedry Chorób Ucha, Nosa, Gardła i Krtani AM w Poznaniu.
Otolaryngol Pol. 1996;50(2):207-20.
Lethal midline granuloma syndrome (LMG) describes lesions of the midface and is characterized by a progressive and often fatal ulceration and destruction of the upper air way involving the nose, the paranasal sinuses, the palate and the soft tissues of the face. Under the term LMG we distinguish four entities: idiopathic midline destructive disease (IMDD), polymorphic reticulosis (PR), non Hodkin's lymphoma and Wegener's granulomatosis (WG). Review of the literature allows to present the differential diagnosis making use of latest technological achievements in clinical immunology and immunohistochemistry. First of all the LMG must be discriminated from localized WG occurring in the midface. The clinical, serological and histopathological findings in WG are described. Literature review is carried out and recent concepts of it's etiology and pathogenesis are presented. Autoantibodies directed against cytoplasmic antigens of neutrophils (ANCA) with specificity for proteinase 3 (PR 3) are valuable marker for differential diagnosis and specificity are discussed. We make a comparison between the "limited" or "non renal" WG and "classical" or "renal" form of the disease.
致死性中线肉芽肿综合征（LMG）描述的是面部中部的病变，其特征为上呼吸道的进行性且常为致命性的溃疡和破坏，累及鼻子、鼻窦、腭部及面部软组织。在LMG这一术语下，我们区分出四种病症：特发性中线破坏性疾病（IMDD）、多形性网状细胞增多症（PR）、非霍奇金淋巴瘤及韦格纳肉芽肿病（WG）。文献回顾有助于利用临床免疫学和免疫组织化学的最新技术成果进行鉴别诊断。首先，必须将LMG与发生于面部中部的局限性WG区分开来。文中描述了WG的临床、血清学及组织病理学表现。进行了文献回顾并介绍了其病因学和发病机制的最新概念。针对蛋白酶3（PR 3）具有特异性的抗中性粒细胞胞浆抗原自身抗体（ANCA）是鉴别诊断的重要标志物，并对其特异性进行了讨论。我们对疾病的“局限性”或“非肾型”WG与“典型”或“肾型”形式进行了比较。
