Keck School of Medicine, Los Angeles, California 90033, USA.
Curr Opin Pulm Med. 2012 Sep;18(5):493-8. doi: 10.1097/MCP.0b013e3283560824.
Necrotizing sarcoid granulomatosis (NSG) is rare. Its various clinical and histological presentations are ambiguous and confusing. Although sometimes limited only to the lungs, other organ systems can be involved. The disease affects primarily women in their late forties but has been documented in children.
There are two schools of thought regarding the taxonomical position of NSG. Some believe that it is an independent entity, whereas others argue that it is just another manifestation of sarcoidosis. This article will review the available, current and evidence-based information about clinical and basic science aspects of NSG.
Diagnosis of NSG is perplexing; its pathogenesis is a dilemma. The disease is frequently confused with other processes including malignancy, Wegener's granulomatosis, hypersensitivity pneumonitis, sarcoidosis and lymphoid granulomatosis. Its symptoms are nonspecific and a histology finding of necrotizing granuloma has many causes. Although no specific management tool or strategies are advocated, steroids, surgical resection of localized disease or sometimes watchful waiting are recommended.
坏死性类肉瘤肉芽肿(NSG)较为罕见。其各种临床表现和组织学表现都存在模糊性和混淆性。尽管有时仅局限于肺部,但也可能涉及其他器官系统。该病主要影响四十多岁的女性,但也有儿童发病的记录。
关于 NSG 的分类学地位存在两种观点。一些人认为它是一种独立的实体,而另一些人则认为它只是结节病的另一种表现形式。本文将综述 NSG 的临床和基础科学方面的现有、当前和基于证据的信息。
NSG 的诊断令人困惑;其发病机制是一个难题。该病常与其他疾病相混淆,包括恶性肿瘤、韦格纳肉芽肿、过敏性肺炎、结节病和淋巴肉芽肿病。其症状是非特异性的,而坏死性肉芽肿的组织学发现有很多原因。虽然没有推荐特定的管理工具或策略,但推荐使用类固醇、局部疾病的手术切除或有时的观察等待。