Wegener Granulomatosis, also known as Granulomatosis with Polyangiitis (GPA), is a rare autoimmune disease characterized by inflammation of blood vessels, known as vasculitis, which affects various organs and tissues in the body. The condition was initially described by Dr. Friedrich Wegener in the 1930s and was later renamed to Granulomatosis with Polyangiitis to honor his contributions.
In Wegener Granulomatosis, the immune system mistakenly attacks and damages the small to medium-sized blood vessels, leading to the formation of granulomas, which are inflammatory nodules. This results in an impaired blood flow to affected organs and tissues, causing a wide range of symptoms depending on the affected areas. The disease predominantly affects the respiratory system but can also involve the kidneys, skin, eyes, ears, and other organs.
Common symptoms of Wegener Granulomatosis include sinusitis, persistent cough, bloody or pus-filled nasal discharge, shortness of breath, chest pain, joint pain, skin rashes or sores, ear infections, and bloody urine. If left untreated, the condition can lead to severe organ damage and complications, such as kidney failure, respiratory failure, or blindness.
The exact cause of Wegener Granulomatosis is unknown, but it is believed to involve a combination of genetic predisposition and environmental triggers. Diagnosis of the disease typically involves a thorough medical history, physical examination, blood tests, imaging studies, and sometimes a biopsy of affected tissue for microscopic analysis.
Treatment for Wegener Granulomatosis aims to suppress the immune system and reduce inflammation. This usually involves a combination of medications, such as corticosteroids and immunosuppressive drugs. In severe cases, plasma exchange therapy may be recommended to remove harmful antibodies
