Granlomatosis with polyangitis

Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a type of autoimmune disease that causes inflammation of blood vessels in various organs and tissues, including the lungs, kidneys, sinuses, and skin.

The cause of GPA is not fully understood, but it is believed to be a result of an abnormal immune response in which the immune system attacks healthy tissues, leading to inflammation and damage. GPA is often associated with the presence of antineutrophil cytoplasmic antibodies (ANCA), specifically c-ANCA (cytoplasmic ANCA), which target certain white blood cells called neutrophils.

Symptoms of GPA can vary depending on which organs are affected, but may include fever, fatigue, weight loss, joint pain, sinusitis, cough, shortness of breath, and kidney problems. The disease can be progressive and potentially life-threatening if left untreated.

Treatment for GPA typically involves a combination of immunosuppressive medications, such as glucocorticoids and cyclophosphamide, to suppress the abnormal immune response and reduce inflammation. Other medications may also be used to manage specific symptoms or complications associated with the disease. With appropriate treatment, many patients with GPA are able to achieve remission and maintain good long-term health.