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Pulmonary Pathology Online

Pathology of Necrotizing Sarcoid Granulomatosis

Dr Sampurna Roy MD

 

                                                                                                                      

 

 

Five distinct clinical syndromes of pulmonary angiitis and granulomatosis are currently recognized:  Wegener's Granulomatosis, lymphomatoid Granulomatosis , necrotizing sarcoid granulomatosis, bronchocentric granulomatosis, and Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

Necrotizing sarcoid granulomatosis (NSG) is a rare form of pulmonary vasculitis.

The radiographic appearances vary from diffuse infiltrates to multiple bilateral nodules, or occasionally solitary nodules, usually involving the lower lobes.  

The diagnostic feature of NSG is the presence of sarcoid-like granulomas in the walls of blood vessels, both small arteries, veins and venules.

These are discrete rounded aggregates of giant cells or typical epithelioid and giant cell granulomas. 

They bulge into the lumen and some vessels may be completely replaced by granulomas. They can still be identified by elastin stains as the internal elastic lamina tends to remain intact.

Granulomas also form nodular confluent aggregates within the lung parenchyma, where they are accompanied by the deposition of hyaline connective tissue .

Irregular areas of necrosis occur within the denser aggregates, sometimes involving vessel walls.

Pulmonary nodules are unusual in classical sarcoidosis but cases of NSG are described with granulomas in hilar lymph nodes and other manifestations of sarcoidosis , such as uveitis, and the two diseases appear to be closely related.

Note:  The angiocentric forms of granulomatosis and vasculitis include Wegener's Granulomatosis, Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). , lymphomatoid granulomatosis , and necrotizing sarcoid granulomatosis. 

- Wegener's granulomatosis is a well-defined syndrome characterized by necrotizing granulomatous vasculitis of the upper and lower respiratory tracts, segmental necrotizing glomerulonephritis, and systemic small vessel vasculitis.

- Allergic angiitis and granulomatosis is a less common multisystem vasculitis with many features similar to polyarteritis nodosa.

- Lymphomatoid granulomatosis is an angiocentric and angiodestructive lymphoreticular proliferative and granulomatous disease involving predominantly the lungs and resembling lymphoma.

- Necrotizing sarcoid granulomatosis is probably a variant of sarcoidosis in which an angiitis is a prominent feature.

Further reading:

Necrotizing sarcoid granulomatosis.

Necrotizing Sarcoid Granulomatosis Mimicking Lung Malignancy: MDCT, PET-CT and Pathologic Findings.

Necrotizing sarcoid granulomatosis: a case report of gastric involvement.

Necrotizing sarcoid granulomatosis--case report and review of the literature.

Necrotizing sarcoid granulomatosis.

Necrotizing sarcoid granulomatosis: a rarity in childhood.

Necrotizing sarcoid granulomatosis. Report of a new case.

Necrotizing sarcoid granulomatosis--is it different from nodular sarcoidosis?

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

Necrotizing sarcoid granulomatosis with extrapulmonary involvement.

Pulmonary angiitis and granulomatosis revisited.

Necrotizing sarcoid granulomatosis with suppurative features.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

 


 

 

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