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

CONTEXT: 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.

OBJECTIVE: 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.

DATA SOURCES: 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.

CONCLUSIONS: 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.