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These granulomas are the main reason for the name granulomatosis with polyangiitis, although it is not an essential feature. Nevertheless, necrotizing granulomas are a hallmark of this disease. However, many biopsies can be nonspecific and 50% provide too little information for the diagnosis of GPA. [12]
Granuloma formation is seen in other diseases such as tuberculosis and sarcoidosis. Sarcoidosis, like berylliosis, also produces granulomas without a necrotic core, and so distinction between the two processes can be difficult. Ultimately, this process leads to restrictive lung disease (a decrease in diffusion capacity).
Necrotizing pneumonia (NP), also known as cavitary pneumonia or cavitatory necrosis, is a rare but severe complication of lung parenchymal infection. [ 1 ] [ 2 ] [ 3 ] In necrotizing pneumonia, there is a substantial liquefaction following death of the lung tissue, which may lead to gangrene formation in the lung.
"Pulmonary hyalinizing granuloma" is a lesion characterized by keloid-like fibrosis in the lung and is not granulomatous. Similarly, radiologists often use the term granuloma when they see a calcified nodule on X-ray or CT scan of the chest. They make this assumption since granulomas usually contain calcium, although the cells that form a ...
ENKTCL-NT is a rare type of lymphoma that commonly involves the nasal cavity, oral cavity, and/or pharynx [5] but less commonly can also involve the eye, larynx, lung, gastrointestinal tract, skin, and various other tissues. [6] Patients presenting with highly localized midline facial disease fit the historical definition of lethal midline ...
Eosinophilic granulomatosis with polyangiitis consists of three stages, but not all patients develop all three stages or progress from one stage to the next in the same order; [7] whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and heart disease, some patients are only mildly affected, e.g. with skin lesions and nasal polyps. [8]
Sarcoidosis is characterized by the formation of non-necrotizing ("non-caseating") granulomas in various organs and tissues. [95] Giant cells, specifically Langhans giant cells, are often seen in sarcoidosis. [96] Schaumann bodies seen in sarcoidosis are calcium and protein inclusions inside of giant cells as part of a granuloma. [97]
The commonest abnormality on lung function testing is a decrease in gas transfer. [2] Both obstructive and restrictive patterns on spirometry have been reported. [2] The differential diagnosis includes infection, other interstitial lung diseases and malignant disease including lymphoma.