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Four decades of necrotizing sarcoid granulomatosis: what do we know now?

坏死性结节病样肉芽肿的40年:我们现在了解什么?

Abstract

In the 4 decades since Dr Averill A. Liebow introduced necrotizing sarcoid granulomatosis, there have been publications of numerous cases, but its nature and possible relationship to classical and nodular sarcoidosis have been and remain controversial. Liebow introduced necrotizing sarcoid granulomatosis as a provisional diagnostic term and stated that "the problem is whether the disease represents necrotizing angiitis with sarcoid reaction, or sarcoidosis with necrosis of the granulomas and of the vessels." There has, as yet, been no definitive answer to the questions that he raised.
To determine whether there is a relationship between necrotizing sarcoid granulomatosis and nodular sarcoidosis in order to ascertain whether the current prevailing opinion that they are related is correct.
The world's literature on necrotizing sarcoid granulomatosis from 1973 to 2013 and nodular sarcoidosis from 1952 to 2013 was critically reviewed. One hundred three cases of necrotizing sarcoid granulomatosis and 111 cases of nodular sarcoidosis were found suitable for individual case analysis.
The data showed a striking overlap in the clinical, radiologic, and pathologic features of both entities, strongly supporting the conclusion that necrotizing sarcoid granulomatosis is a previously unrecognized manifestation of sarcoidosis and is essentially the same as nodular sarcoidosis. It is proposed that use of necrotizing sarcoid granulomatosis as a diagnostic term be discontinued and replaced by sarcoidosis with necrotizing sarcoid granulomatosis pattern provided that an infectious etiology can be reasonably excluded. Our concept of sarcoidosis should now be expanded to recognize that there is a continuous spectrum of necrosis ranging from minimal to extensive.

摘要

自从Dr Averill A. Liebow提出坏死性结节病样肉芽肿40年来,有很多相关病例报道,但其性质以及与经典和结节性结节病的关系仍然有争议。Liebow提出坏死性结节病性肉芽肿为一个临时性诊断术语,并指出“问题在于疾病是代表了坏死性血管炎伴类结节样反应,还是结节病伴肉芽肿和血管的坏死。”对于他提出的这个问题还没有明确的答案。

确定坏死性结节病样肉芽肿与结节性结节病是否相关,以便明确目前流行的观点是否正确。

认真地回顾了1973-2013年间关于坏死性结节病样肉芽肿的文献以及1952-2013年间结节性结节病的文献。 对103例坏死性结节病样肉芽肿和111例结节性结节病进行了研究。

数据表明,无论从临床、影像学和病理特点上,二者都具有明显的重叠,强烈支持坏死性结节病样肉芽肿是结节病以前未认识的表现、本质上与结节性结节病相同的结论。建议不再使用坏死性结节病样肉芽肿作为诊断性术语,代之以结节病伴坏死性结节病样肉芽肿,可以合理地排除感染性病因。我们关于结节病的概念现在应该扩展到认识坏死是一个连续的谱系,可以从很微小到广泛。

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