necrotizing sarcoid granulomatosis

Definition: Necrotizing sarcoid granulomatosis (NSG) is a disorder of unknown etiology, rarely described in childhood, belonging to the heterogeneous group of the pulmonary angiitis and granulomatosis.

One of the characteristics of NSG is to have typically a benign clinical course with minimal treatment with systemic steroids or even with no therapy at all.

Necrotizing sarcoid granulomatosis (NSG) is characterized by pulmonary nodular infiltrates, a typical histology, and a benign clinical course. The etiology and pathogenesis of the disease are still unknown.

Synopsis

 necrotizing granulomas
 non-caseating granulomas
 fibrinoid vasculitis

Differential diagnosis

 systemic granulomatoses

• Wegener granulomatosis
• sarcoidosis
• familial chronic granulomatosis

See also

 Blau syndrome

 pulmonary angiitis and granulomatosis

• Wegener granulomatosis
• lymphomatoid granulomatosis
• necrotizing sarcoid granulomatosis
• bronchocentric granulomatosis
• allergic angiitis and granulomatosis (Churg-Strauss syndrome)

References

 Toyohara JP, Ward KM, Rabinowitz AD, Thomashow BM, Grossman ME. Necrotizing sarcoid granulomatosis with skin and pulmonary involvement. Cutis. 2007 Oct;80(4):313-6. PMID: 18038694

 Lazzarini LC, de Fatima do Amparo Teixeira M, Souza Rodrigues R, Marcos Nunes Valiante P. Necrotizing Sarcoid Granulomatosis in a Family of Patients with Sarcoidosis Reinforces the Association between Both Entities. Respiration. 2007 Mar 22; PMID: 17377369

 Popper HH, Klemen H, Colby TV, Churg A. Necrotizing sarcoid granulomatosis—is it different from nodular sarcoidosis?Pneumologie. 2003 May;57(5):268-71. PMID: 12784179

 Heinrich D, Gordjani N, Trusen A, Marx A, Hebestreit H.Necrotizing sarcoid granulomatosis: a rarity in childhood. Pediatr Pulmonol. 2003 May;35(5):407-11. PMID: 12687600

 Harada T, Amano T, Takahashi A, Furuya J, Yamaguchi E, Kaji M, Morikawa T, Itoh T, Shimizu M, Nishimura M. Necrotizing sarcoid granulomatosis presenting with elevated serum soluble interleukin-2 receptor levels. Respiration. 2002;69(5):468-70. PMID: 12232459

 Strickland-Marmol LB, Fessler RG, Rojiani AM. Necrotizing sarcoid granulomatosis mimicking an intracranial neoplasm: clinicopathologic features and review of the literature. Mod Pathol. 2000 Aug;13(8):909-13. PMID: 10955459