Wegener’s granulomatosis

A disease of connective tissue, where the nasal passages, lungs and kidneys are inflamed and ulcerated, with formation of granulomas. It is usually fatal.


An autoimmune disorder that causes inflammation in blood vessels and damages the walls of small and medium-sized arteries and capillaries. Wegener granulomatosis is potentially life-threatening because it can impede the blood supply to tissues supplied by the damaged arteries. The disorder occurs most often in people who are in their 40s or 50s and, without treatment, is almost always fatal.


A condition believed to be an autoimmune disorder that produces inflammation of the blood vessels that damages the walls of the small and medium-sized arteries and capillaries. Wegener granulomatosis is a potentially life-threatening form of vasculitis. The normal blood supply to tissues near the arteries is disrupted, which causes injury and destruction in the parts of the body affected. The disease process is called granulomatosis because characteristic islands of inflamed cells (called granulomas) located in the tissues involved in the disease are revealed by microscopic examination. Without treatment, Wegener granulomatosis is always fatal. It occurs most commonly in a person’s 40s or 50s and affects men and women with equal frequency.


A condition primarily observed in males, characterized by the infiltration of inflammatory cells and tissue damage in vital organs such as the lungs, kidneys, blood vessels, heart, and other tissues.


An uncommon condition marked by the formation of granulomas (nodules composed of abnormal cells) within the nasal passages, lungs, and kidneys, along with chronic tissue inflammation due to vasculitis (inflammation of blood vessels). This disorder is believed to be an autoimmune condition, where the body’s immune system mistakenly attacks its own tissues.


The primary symptoms encompass a nasal discharge containing blood, bouts of coughing that occasionally result in blood-tinged sputum, breathing challenges, chest discomfort, and the presence of blood in the urine. Additional indications may involve a diminished appetite, weight loss, feelings of weakness, fatigue, and joint pain.


The management of this condition involves the use of immunosuppressant medications like cyclophosphamide or azathioprine, in combination with corticosteroids. This approach aims to alleviate symptoms and strive for a state of remission.


With timely intervention, the majority of individuals with Wegener’s granulomatosis can achieve full recovery in approximately a year, although there is a chance of kidney failure in some cases. However, in the absence of treatment, complications may arise, such as perforation of the nasal septum leading to nasal deformity, eye inflammation, skin issues like rashes, nodules, or ulcers, and potential damage to the heart muscle, which can be life-threatening.


 


Posted

in

by

Tags: