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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]
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 ...
Sarcoidosis (/ ˌ s ɑːr k ɔɪ ˈ d oʊ s ɪ s /; also known as Besnier–Boeck–Schaumann disease) is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. [2] The disease usually begins in the lungs, skin, or lymph nodes. [2]
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]
Not all types of interstitial lung disease that occur in adults occur also in children, and vice versa. [4] [5] The group of disorders is heterogenous, and there are different definitions of what exactly should be classed as a ChILD disorder. [1] Childhood interstitial lung disease is a serious condition, with high morbidity and mortality. [6 ...
Granuloma; Picture of a granuloma (without necrosis) as seen through a microscope on a glass slide: The tissue on the slide is stained with two standard dyes (hematoxylin: blue, eosin: pink) to make it visible. The granuloma in this picture was found in a lymph node of a patient with a Mycobacterium avium infection. Specialty: Pathology
Sarcoidosis is a disease of unknown cause that leads to the development of granulomas in various organs. While the lungs are typically involved, other organs may equally be affected. Some subforms of sarcoidosis, such as Löfgren syndrome, may have a particular precipitant and have a specific course. It is unknown which characteristics ...