Necrotising sarcoid granulomatosis

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 affecting 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 being also 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 upto 25% of patients may be asymtptomatic 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 include 

  • parenchymal opacification with air bronchograms
  • solitary or multiple pulmonary nodules 
    • may have egular or slightly irregular margins 
    • typically range from 2-4 cm in diameter. 
    • cavitation may be occasionally present 
  • hilar or mediastinal adenopathy - normally not compressive 
  • pleural disease

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.

It was first described in 1973 by Liebow.

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Article information

rID: 25914
Systems: Vascular, Chest
Tag: cases
Synonyms or Alternate Spellings:
  • Necrotising sarcoid granulomatosis (NSG)
  • Necrotizing sarcoid granulomatosis
  • Necrotizing sarcoid like granulomatosis
  • Necrotising sarcoid like granulomatosis

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