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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. Many biopsies can be nonspecific, though, and 50% provide too little information for the diagnosis of GPA. [12]
Granuloma annulare is a skin disease of unknown cause in which granulomas are found in the dermis of the skin, but it is not a true granuloma. Typically, a central zone of necrobiotic generation of collagen is seen, with surrounding inflammation and mucin deposition on pathology.
The World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) is an organisation of physicians involved in the diagnosis and treatment of sarcoidosis and related conditions. [171] WASOG publishes the journal Sarcoidosis, Vasculitis and Diffuse Lung Diseases. [172]
Giant cell arteritis, [6] also known as temporal arteritis or cranial arteritis, is the most common MGC-linked disease. This type of arteritis causes the arteries in the head, neck, and arm area to swell to abnormal sizes. Although the cause of this disease is not currently known, it appears to be related to polymyalgia rheumatica. [7]
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]
Granulomatous–lymphocytic interstitial lung disease (GLILD) is a lung complication of common variable immunodeficiency disorders (CVID). It is seen in approximately 15% of patients with CVID. [ 1 ] It has been defined histologically as the presence of (non-caseating) granuloma and lymphoproliferation in the lung. [ 1 ]
Granulomas try to wall off these organisms and prevent their further growth and spread. Historically widespread and destructive diseases such as tuberculosis, leprosy and syphilis are granulomatous conditions. Granuloma formation is also the feature of many more contemporary conditions, like fungal infections, sarcoidosis and Crohn's disease. [4]