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Granulomatosis with Polyangiitis (Wegener's Granulomatosis)

An autoimmune disease that damages the blood vessels and causes disease in the lungs, upper respiratory tract, and kidneys.

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(Source: NIH - National Institute of Diabetes and Digestive and Kidney Diseases)

About Granulomatosis with Polyangiitis

Granulomatosis with polyangiitis (GPA) is a condition that causes inflammation that primarily affects the respiratory tract (including the lungs and airways) and the kidneys. This disorder is also commonly known as Wegener granulomatosis.

A characteristic feature of GPA is inflammation of blood vessels (vasculitis), particularly the small- and medium-sized blood vessels in the lungs, nose, sinuses, windpipe, and kidneys, although vessels in any organ can be involved.

Polyangiitis refers to the inflammation of multiple types of vessels, such as small arteries and veins. Vasculitis causes scarring and tissue death in the vessels and impedes blood flow to tissues and organs.

Another characteristic feature of GPA is the formation of granulomas, which are small areas of inflammation composed of immune cells that aid in the inflammatory reaction...Read more about Granulomatosis with Polyangiitis NIH - National Library of Medicine

What works? Research summarized

Evidence reviews

Rituximab for Granulomatosis with Polyangiitis or Microscopic Polyangiitis: A Review of the Clinical and Cost-effectiveness [Internet]

The purpose of this report is to retrieve and review existing evidence for the clinical effectiveness, safety, and cost-effectiveness of rituximab (RTX) treatments, beyond the initial course, for the maintenance of remission in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) patients.

Rituximab for Granulomatosis with Polyangiitis or Microscopic Polyangiitis: A Review of the Clinical effectiveness, Cost-effectiveness, and Guidelines [Internet]

Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) belong to a group of rare autoimmune diseases called anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by inflammatory cell infiltration leading to necrosis of the blood vessels. ,

Intravenous immunoglobulin in addition to standard treatments for Wegener's granulomatosis

Wegener's granulomatosis is a rare disorder that causes inflammation of the blood vessels. This inflammation restricts blood flow to various organs which can eventually damage the organs. Organs most affected by Wegener's include the lungs, upper respiratory tract, kidneys, joints, skin and eyes. Wegener's granulomatosis also produces a granuloma (a mass or nodule of inflammatory tissue) which is found around the blood vessels and which can also damage surrounding tissue. The cause of Wegener's granulomatosis is unknown. Treatment is with corticosteroids and cytotoxic drugs which are often used for chemotherapy. Most patients get better with these drugs. However, the disorder returns in approximately half of patients. Intravenous immunoglobulin (IVIg) is an expensive and fairly rare blood product that has been used to treat Wegener's granulomatosis but its effects on the disorder are unknown. We asked if IVIg provided an advantage as an additive to standard treatments. We found one small randomized trial in which 34 participants were randomized to receive IVIg or placebo once daily in addition to azathioprine and prednisolone for remission maintenance. This trial did not provide enough evidence to determine if IVIg has an advantage over corticosteroids and immunosuppressants for the treatment of Wegener's granulomatosis.

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Summaries for consumers

Intravenous immunoglobulin in addition to standard treatments for Wegener's granulomatosis

Wegener's granulomatosis is a rare disorder that causes inflammation of the blood vessels. This inflammation restricts blood flow to various organs which can eventually damage the organs. Organs most affected by Wegener's include the lungs, upper respiratory tract, kidneys, joints, skin and eyes. Wegener's granulomatosis also produces a granuloma (a mass or nodule of inflammatory tissue) which is found around the blood vessels and which can also damage surrounding tissue. The cause of Wegener's granulomatosis is unknown. Treatment is with corticosteroids and cytotoxic drugs which are often used for chemotherapy. Most patients get better with these drugs. However, the disorder returns in approximately half of patients. Intravenous immunoglobulin (IVIg) is an expensive and fairly rare blood product that has been used to treat Wegener's granulomatosis but its effects on the disorder are unknown. We asked if IVIg provided an advantage as an additive to standard treatments. We found one small randomized trial in which 34 participants were randomized to receive IVIg or placebo once daily in addition to azathioprine and prednisolone for remission maintenance. This trial did not provide enough evidence to determine if IVIg has an advantage over corticosteroids and immunosuppressants for the treatment of Wegener's granulomatosis.

Interventions for renal vasculitis in adults

Renal vasculitis presents as rapidly progressive glomerulonephritis which is a form of kidney disease that causes damage to the small structures (glomeruli) inside the kidneys that help filter waste and fluids from blood to form urine. The disease leads to a rapid loss of kidney function. Standard suppression of the immune system with steroids and cyclophosphamide is recommended. The aim of this review was to evaluate the benefits and harms of any intervention for the treatment of renal vasculitis. Thirty one studies (2217 patients) were identified. Plasma exchange reduces the risk of end‐stage kidney disease in patients presenting with severe acute kidney failure. The use of pulse cyclophosphamide results in good remission rates but there was an increased risk of relapse. Azathioprine is effective as maintenance therapy once remission has been achieved. Mycophenolate mofetil is equivalent for remission induction than cyclophosphamide. Mycophenolate mofetil has also been tested in maintenance treatment and was found to result in a higher rate of disease relapse. Initial data on rituximab showed equivalent effectiveness to cyclophosphamide. Methotrexate and leflunomide are useful in maintenance therapy but their relative effectiveness are not clearly defined. Treatment with co‐trimoxazole may prevent respiratory infections and relapses but are unlikely to have a major impact on systemic relapses of vasculitis.

Terms to know

Blood Vessels
Tubes that carry blood to and from all parts of the body. The three main types of blood vessels are arteries, capillaries, and veins.
Granuloma
Granuloma is an inflammation found in many diseases. It is a collection of immune cells known as macrophages. Granulomas form when the immune system attempts to wall off substances that it perceives as foreign but is unable to eliminate.
Immune System
The body's system for protecting itself from viruses and bacteria or any foreign substances.
Inflammation
Redness, swelling, pain, and/or a feeling of heat in an area of the body. This is a protective reaction to injury, disease, or irritation of the tissues.
Kidney
One of a pair of organs in the abdomen. The kidneys remove waste and extra water from the blood (as urine) and help keep chemicals (such as sodium, potassium, and calcium) balanced in the body. The kidneys also make hormones that help control blood pressure and stimulate bone marrow to make red blood cells.
Musculoskeletal System
The musculoskeletal system provides form, support, stability, and movement to the body. It is made up of the bones of the skeleton, muscles, cartilage, tendons, ligaments, joints, and other connective tissue that supports and binds tissues and organs together.
Respiratory System
The organs that are involved in breathing. These include the nose, throat, larynx, trachea, bronchi, and lungs. Also called respiratory tract.
Rheumatologist
Doctors who diagnose and treat diseases of the bones, joints, muscles, and tendons, including arthritis and collagen diseases.
Vasculitis
Vasculitis is a condition that involves inflammation in the blood vessels. The condition occurs if your immune system attacks your blood vessels by mistake. This may happen as the result of an infection, a medicine, or another disease or condition.

More about Granulomatosis with Polyangiitis

Photo of an adult woman

Also called: Necrotising respiratory granulomatosis, Wegener granulomatosis, Necrotizing respiratory granulomatosis, GPA

Other terms to know: See all 9
Blood Vessels, Granuloma, Immune System

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