What is granulomatosis with polyangiitis? Granulomatosis with polyangiitis (GPA) is a rare type of vasculitis—or inflammation of the blood vessels. The condition was formerly known as Wegener's granulomatosis. This granulomatosis disease affects about 3 in 100,000 Americans. The term polyangiitis refers to multiple types of blood vessels, including small and medium arteries and veins. Blood vessel inflammation prevents blood from flowing as it should to organs and tissues. The tissue damage this causes is granulomatosis. A granuloma is a small area of inflammation within an organ or tissue. It is a collection of immune cells that can grow and invade other tissues. GPA symptoms can develop over days or months. The specific symptoms depend on which tissues or organs it affects. Most commonly, it causes problems with the lungs, sinuses and kidneys. Typically, the first symptoms begin in the respiratory system. People often complain of symptoms that are similar to a cold or flu. However, there can also be more serious symptoms, such as coughing up bloody phlegm and collapse of the nasal septum. Doctors do not know what causes GPA. They describe it as an autoimmune disease. This means the immune system attacks the body’s own tissues. GPA occurs most commonly in middle-aged Caucasian adults. There does not seem to be a higher occurrence in one sex compared to the other. Treatment mainly consists of corticosteroids and other immune suppressants. These medications are usually very effective in resolving the inflammation. However, recurrences of the disease are common. This makes regular medical care a vital part of granulomatosis with polyangiitis treatment. Left untreated, GPA can progress to permanent organ damage and death, usually within two years. What are the symptoms of granulomatosis with polyangiitis? Symptoms of GPA depend on the organs and tissues it affects. The most common sites include the lungs, sinuses and kidneys. The eyes, ears, skin, nerves, joints, heart and brain may also be involved, along with various other tissues throughout the body. Symptoms can also vary from person to person. Some people develop symptoms very quickly, while others notice them over many months. The disease can be mild for some people and life threatening for others. In most cases, symptoms begin in the respiratory tract. About 90% of people describe symptoms that resemble those of a cold or flu. These common symptoms may include: Cough, which may produce blood-tinged phlegm Fever, body aches, fatigue, and lack of energy Runny nose, nasal congestion, or nasal crusting Shortness of breath or wheezing Other symptoms may include nosebleeds, joint pain, skin rashes, night sweats, unintended weight loss, appetite loss, numbness in the extremities, and vision or hearing problems. Ulcers can develop on the nasal septum and lead to perforation and collapse of the septum. This causes saddle nose, when the bridge of the nose looks sunken. About 75% of people with GPA will develop kidney disease. In most cases, there are no symptoms of this problem. Doctors may find it when high blood pressure develops or small amounts of blood or protein show up on a urine test. Sometimes, there is noticeable blood in the urine. It’s important to seek medical care if you have these symptoms. Early diagnosis of GPA usually leads to effective treatment. What causes granulomatosis with polyangiitis? Scientists do not know what causes GPA, but most experts consider it an autoimmune disorder. Autoimmune disorders happen when the immune system mistakes the body’s own tissues for a threat. The immune system starts attacking normal, healthy tissue in an effort to rid the body of this perceived threat. In GPA, the autoimmune reaction causes inflammation of blood vessels and formation of granulomas. Why this happens is not known. It is likely a combination of environmental and genetic factors. Research on the genetics of GPA has identified a few candidate genes that can affect a person susceptible to GPA. Some of these genes are involved in regulating the immune system and helping it recognize (and protect) the body’s own tissues from foreign tissues and proteins. What are the risk factors for granulomatosis with polyangiitis? GPA most often affects middle-aged adults between the ages of 40 and 65 years. It is rare in children. It affects men and women equally, but Caucasians tend to get the disease more often than other races. There is no known link to inheritance through a family history. One of the strongest risk factors for GPA is a specific variation of the HLA-DPB1 gene, which plays an important role in the immune system. The HLA-DPB1 gene variation occurs by chance in people with no known family history of GPA. How is granulomatosis with polyangiitis treated? Tremendous treatment advances have been made in the management of GPA. Treatment of GPA depends on the severity of the disease and which organs and tissues it affects. In general, treatment consists of two phases. The first phase is induction of remission. The goal is to quickly reduce inflammation and tamp down the immune response. The second phase is maintenance to prevent a recurrence. Induction treatment can last weeks or months and may include the following medications: Azathioprine (Azasan, Imuran) Corticosteroids such as prednisone (Deltasone) Cyclophosphamide (Cytoxan) Methotrexate (Rheumatrex, Trexall) Rituximab (Rituxan) Once the disease is under control, maintenance treatment can begin. This phase can last for several years and may include the following medications: Azathioprine (Azasan, Imuran) Methotrexate (Rheumatrex, Trexall) Mycophenolate (CellCept) Rituximab (Rituxan) Seeing your doctor on a regular basis is a vital part of GPA treatment. GPA treatment is highly effective. However, recurrences are common without medications to keep the disease under control. If you and your doctor decide to try stopping medications, it is imperative to maintain regular medical care and consult your doctor immediately if symptoms develop. What are the potential complications of granulomatosis with polyangiitis? Without proper treatment, complications will develop. GPA can lead to serious and permanent organ damage. GPA complications include: Deep vein thrombosis Heart attack, disease of the heart muscle (cardiomyopathy), inflammation of the lining of the heart (endocarditis), and inflammation of the membrane around the heart (pericarditis) Kidney failure Permanent skin scarring Vision or hearing loss GPA can also be fatal. Without medical treatment, death usually occurs within two years.