Purpose of review: 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.
Recent findings: 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.
Summary: 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.