Necrotising sarcoid granulomatosis (NSG) is a rare systemic disease, characterised by sarcoid-like granuloma formation, vasculitis and variable degrees of necrosis. It is sometimes classified under the group of pulmonary angiitis and granulomatois.
It typically affects middle-aged or elderly individuals.
While many believe that it may be a variant of sarcoidosis, its proper classification is under debate due to some of its features also being found in Churg-Strauss syndrome, granulomatosis with polyangitis and hypersensitivity pneumonitis 3. Due to this, its pathological diagnosis can be challenging.
Dyspnoea and chest pain may be present, but up to 25% of patients may be asymptomatic 6.
Many features have been described, with the most common feature being subpleural predominant alveolar infiltrative like opacification without a lobar preference.
The reported features on CT chest include:
- parenchymal opacification with air bronchograms
- solitary or multiple pulmonary nodules
- may have regular or slightly irregular margins
- typically range from 2-4 cm in diameter.
- cavitation may occasionally be present
- hilar or mediastinal adenopathy - normally not compressive
- pleural disease
- pleural thickening
- pleural involvement with granulomata
Treatment and prognosis
It is considered to have more favourable prognosis when compared with other similar granulomatous diseases. No definitive single management strategy is advocated although the use of steroids, surgical resection for localised disease or sometimes watchful waiting are recommended.
History and etymology
It was first described in 1973 by Liebow.
- 1. Quaden C, Tillie-Leblond I, Delobbe A et-al. Necrotising sarcoid granulomatosis: clinical, functional, endoscopical and radiographical evaluations. Eur. Respir. J. 2005;26 (5): 778-85. doi:10.1183/09031936.05.00024205 - Pubmed citation
- 2. Chittock DR, Joseph MG, Paterson NA et-al. Necrotizing sarcoid granulomatosis with pleural involvement. Clinical and radiographic features. Chest. 1994;106 (3): 672-6. Pubmed citation
- 3. Lazzarini LC, de Fatima do Amparo Teixeira M, Souza Rodrigues R et-al. Necrotizing sarcoid granulomatosis in a family of patients with sarcoidosis reinforces the association between both entities. Respiration. 2008;76 (3): 356-60. doi:10.1159/000101013 - Pubmed citation
- 4. Popper HH, Klemen H, Colby TV et-al. Necrotizing sarcoid granulomatosis-is it different from nodular sarcoidosis?. Pneumologie. 2003;57 (5): 268-71. doi:10.1055/s-2003-39365 - Pubmed citation
- 5. Gibbs AR, Williams WJ, Kelland D. Necrotising sarcoidal granulomatosis: a problem of identity. A study of seven cases. Sarcoidosis. 1987;4 (2): 94-100. Pubmed citation
- 6. Arfi J, Kerrou K, Traore S et-al. F-18 FDG PET/CT findings in pulmonary necrotizing sarcoid granulomatosis. Clin Nucl Med. 2010;35 (9): 697-700. doi:10.1097/RLU.0b013e3181e9fade - Pubmed citation
- 7. Churg A, Carrington CB, Gupta R. Necrotizing sarcoid granulomatosis. Chest. 1979;76 (4): 406-13. Pubmed citation
- 8. Niimi H, Hartman TE, Müller NL. Necrotizing sarcoid granulomatosis: computed tomography and pathologic findings. J Comput Assist Tomogr. 1996;19 (6): 920-3. Pubmed citation
- 9. Yeboah J, Afkhami M, Lee C et-al. Necrotizing sarcoid granulomatosis. Curr Opin Pulm Med. 2012;18 (5): 493-8. doi:10.1097/MCP.0b013e3283560824 - Pubmed citation