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Chronic granulomatous disease (CGD), also known as Bridges–Good syndrome, chronic granulomatous disorder, and Quie syndrome, [1] is a diverse group of hereditary diseases in which certain cells of the immune system have difficulty forming the reactive oxygen compounds (most importantly the superoxide radical due to defective phagocyte NADPH oxidase) used to kill certain ingested pathogens. [2]
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]
Note, myeloperoxidase deficiency can cause false positives in the diagnosis of chronic granulomatous disease, a condition which includes dysfunctional NADPH oxidase. [ 3 ] [ 7 ] Both disorders interfere with neutrophils’ abilities to kill pathogens through reaction with oxidative species.
The diagnosis should be made only after excluding other possible causes of similar symptoms such as tuberculosis. [6] Sarcoidosis may resolve without any treatment within a few years. [2] [5] However, some people may have long-term or severe disease. [5] Some symptoms may be improved with the use of anti-inflammatory drugs such as ibuprofen. [8]
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]
Neutrophil oxidative burst test (or chronic granulomatous disease (CGD) test) is a measure of neutrophil oxidation and is a useful assay in the diagnosis of chronic granulomatous disease and is also a useful means to determine the overall metabolic integrity of phagocytosing neutrophils. The NADPH oxidase enzyme is missing in CGD. From total ...
The diagnosis is usually made by tissue biopsy, however this cannot reliably distinguish between the granulomas of OFG and those of Crohn disease or sarcoidosis. [8] Other causes of granulomatous inflammation are ruled out, such as sarcoidosis, Crohn disease, allergic or foreign body reactions and mycobacterial infections .
Exclusion of infection is therefore an important step in management, but confirmation of the diagnosis requires lung biopsy. In people who have lung disease prior to a diagnosis of CVID, the differential diagnosis includes sarcoidosis. Sarcoid is also characterised by granulomatous involvement of the lung and therefore patients being ...