NORD gratefully acknowledges Eric Matteson, MD, Department of Rheumatology, Mayo Clinic, and the Vasculitis Foundation, for assistance in the preparation of this report.
Granulomatosis with polyangiitis (GPA) is a rare disorder characterized by inflammation of small- and medium-sized blood vessels (vasculitis) that results in damage to various organ systems of the body, most often the respiratory tract and kidneys. Symptoms may include ulcerations of the mucous membranes in the nose with secondary bacterial infection, a persistent runny nose, sinus pain, and chronic middle ear infection (otitis media) potentially resulting in hearing loss. In some individuals, kidney abnormalities may progress to kidney failure, a serious complication that requires dialysis or a kidney transplant. If the lungs are affected, a cough, coughing up of blood (hemoptysis), and inflammation of the thin membrane lining the outside of the lungs and the inside of the lung may be present. Other symptoms can occur depending on which organ systems are affected in an individual.
Granulomatosis with polyangiitis is not an inherited disorder. It is classified as an autoimmune disorder. Autoimmune disorders occur when the body’s immune system mistakenly attacks healthy tissue. Environmental, infectious and some genetic factors may play a role in the development of the disorder. The disorder can occur at any age, but most often affects people between 40 and 60 years of age.
For many years, granulomatosis with polyangiitis was known as Wegener’s granulomatosis or Wegener granulomatosis. Wegener is the surname of a physician who was one of the first to describe the disorder in detail in the medical literature back in the 1930s. Other doctors before Wegener also described the disease. Surnames don’t characterize diseases and terms which better describe diseases are generally preferred.
Granulomatosis with polyangiitis is classified as a form of vasculitis, and further classified as a form of antineutrophil cytoplasmic antibodies-associated (ANCA-associated) vasculitis or ANCA-associated vasculitides (AAV).
The specific symptoms and severity of the symptoms associated with granulomatosis with polyangiitis vary greatly from one person to another. Indeed, granulomatosis with polyangiitis has been described affecting almost all organ systems of the body.
In most people, the disorder affects the respiratory tract and the kidneys. Some people may have mild disease, while others develop life-threatening complications. The disorder can develop slowly over many months or it can develop rapidly over several days (acute). Because each person with granulomatosis with polyangiitis is unique, and the symptoms described below may or may not apply to a specific individual.
Initial symptoms usually occur in the upper respiratory tract and resemble those associated with a severe common cold, including a persistent runny nose (rhinorrhea), nasal crusting and nasal obstruction or congestion. More serious symptoms include nosebleeds, ulcerations of the mucous membranes in the nose with secondary bacterial infection, sinus pain, inflammation of the sinuses (paranasal sinusitis), and hoarseness. Some affected individuals will develop a hole or tear in the wall (septum) dividing the nostrils, resulting in the collapse of the bridge of the nose, a condition called saddle nose. Affected individuals may also develop recurrent middle ear inflammation (otitis media), which, if untreated, may eventually result in hearing loss.
Additional initial symptoms can include fever, general feeling of ill health (malaise), weakness and fatigue, joint paint (arthralgia), loss of appetite, and unintended weight loss. Sometimes, the upper airways are affected for years before other symptoms develop and, sometimes, granulomatosis with polyangiitis remains isolated in the upper airways and the rest of the body remains unaffected. This may be called localized or limited granulomatosis with polyangiitis.
Many affected individuals will develop symptoms affecting the lungs (pulmonary). These symptoms include a persistent cough, episodes of coughing up of blood (hemoptysis), difficulty breathing (dyspnea), chest pain, inflammation of the thin membrane lining the outside of the lungs and the inside of the lungs (pleuritis), and the excess buildup of fluid around the lungs (pleural effusion). There may be substances such as blood, pus or protein (pulmonary infiltrates) in lung tissue that can be detected on x-ray examination. Sometimes, affected individuals experience inflammation and narrowing of the area of the windpipe below the vocal cords (subglottis), a condition known as subglottic stenosis. Inflammation and narrowing of the whole windpipe (tracheal stenosis) can also occur. These conditions can cause difficulty breathing, high-pitched noisy breathing (stridor), wheezing, or voice changes. Bleeding (hemorrhaging) in the lungs is a potential serious complication that requires hospitalization and aggressive treatment. Respiratory symptoms are often the first sign in children with this disorder.
Approximately 75% of individuals eventually develop kidney (renal) disease. However, in many people, no symptoms are apparent (asymptomatic). Affected individuals may develop high blood pressure (hypertension) and/or inflammation of the cluster of blood vessels and nerve fibers of the kidney called the glomeruli, which filter the blood. Glomeruli become swollen and misshapen and cannot perform their proper function, a condition known as glomerulonephritis. This can lead to small amounts of blood and protein in the urine. Without treatment, progressive kidney damage can occur, eventually causing life-threatening kidney (renal) failure.
Most individuals experience symptoms affecting the muscles and skeleton, including pain in various joints (polyarthralgia), inflammation and swelling of the joints (arthritis), inflammation of muscles (myositis), and muscle pain (myalgia).
More than half of individuals with granulomatosis with polyangiitis experience eye (ocular) abnormalities, including inflammation of the delicate membrane that lines the eyes (conjunctivitis), corneal ulcerations, inflammation of the white, outer-covering (sclera) of the eyeball (scleritis), and inflammation of the membrane covering the sclera (episcleritis). Affected individuals may also develop an abnormal mass or sore behind the eye (orbital mass lesion or ‘pseudotumor’). Eye abnormalities may result in eye pain, redness, bulging or protrusion of the eyeballs (proptosis), double vision (diplopia), and vision loss. Additional findings can occur in some people. Sometimes, problems with the eyes are the first symptom of granulomatosis with polyangiitis.
Approximately half of affected individuals develop skin abnormalities including small raised bumps (papules), slightly larger, deeper bumps just below the surface of the skin (subcutaneous nodules), skin ulcers, bleeding (hemorrhage) within skin layers, causing the appearance of small purplish spots on the skin (petechiae), and/or areas of purple discoloration caused by bleeding vessels near the surface of the skin (purpura). Skin lesions may or may not be painful. Some affected individuals may have painfully cold fingers and toes in response to cold (Raynaud’s phenomenon) caused by lack of blood flow to these areas. Sometimes, this is severe enough to cause tissue death (gangrene) of the tips of the fingers and toes.
In rare instances, individuals may also develop neurological abnormalities including inflammation and degeneration of nerve fibers outside of the brain and spinal cord (peripheral neuropathy), injury to a few peripheral nerves in different areas simultaneously (mononeuritis multiplex), and inflammation of cranial nerves (cranial neuritis). Peripheral neuropathy can cause a sensation of numbness, burning or tingling in the hands or feet. Mononeuritis multiplex can cause pain, weakness and abnormal sensations in the affected areas. The specific symptoms depended on which areas of the body are affected. Symptoms of cranial symptoms depend on which cranial nerves are affected. Headaches, seizures, and paralysis on one side of the body (hemiplegia) have also been reported.
Other rare symptoms include abnormalities of the heart (cardiac) including inflammation of the membranous sac that surrounds the heart (pericarditis), inflammation of the endocardium (endocarditis), which is the inner membrane lining of the heart, inflammation of the arteries that supply blood to the heart muscles (coronary arteritis), and disease of the heart muscle (cardiomyopathy). Some affected individuals may be at an increased risk of having a stroke or heart attack (myocardial infarction).
The exact cause of granulomatosis with polyangiitis is not fully understood. Because of the characteristic tissue changes seen in affected tissues, and increased immune response of the body, an immune reaction has been suggested as a possible basis for the disorder. Many researchers consider the disorder an autoimmune disorder. Autoimmune disorders are caused when the body’s natural defenses against “foreign” or invading organisms begin to attack healthy tissue for unknown reasons.
In granulomatosis with polyangiitis, white blood cells called neutrophils release abnormal immune proteins that can damage the body. Normally, neutrophils are instrumental in fighting off infection by surrounding and destroying bacteria that enter the body. About 85%-90% of have antineutrophil cytoplasmic antibodies (ANCA). Of these people, about 80% have what is called cytoplasmic-ANCA, or c-ANCA. About 20% of those people have perinuclear-ANCA, or p-ANCA. Antibodies, or immunoglobulins, are specialized proteins that bind to invading or foreign substances in the body and bring about their destruction. Autoantibodies are antibodies that mistakenly attack healthy tissue. The exact role these autoantibodies play in the development of granulomatosis with polyangiitis is not fully understood.
Many researchers believe that an infection ‘sets off’ the immune system causing it to malfunction. While many researchers believe that an infection contributes to the development of the disorder, it is unlikely that an infection alone can explain a disorder as complex as granulomatosis with polyangiitis. There are likely additional factors including environmental and genetic ones that also play a role in the development of the disorder. Research into this question is ongoing, but so far, no specific infectious, genetic, or environmental factor has been conclusively identified to be associated with the disorder.
The symptoms of granulomatosis with polyangiitis occur because of inflammation of the blood vessels (vasculitis). This inflammation leads to narrowing of the vessels and results in reduced blood flow to, and loss of tissue in, various organ systems of the body. The disorder is marked by the abnormal clumping or massing immune system cells that are produced to fight infection or inflammation. These clumps of cells are called granulomas and can be found within various organ tissues and blood vessels of the body (granulomatosis).
Granulomatosis with polyangiitis is a rare disorder that affects males and females in equal numbers. In most people, onset is after the fourth or fifth decade of life; however, the disorder can occur at any age. There are some studies that suggest that girls are affected more often than boys when the disorder occurs in childhood. There are also studies that suggest that males are more likely to have severe disease, while females are more likely to have localized disease.
Estimates of the frequency of granulomatosis with polyangiitis vary greatly depending upon the specific population being studied. Because the disorder often goes unrecognized, researchers believe that it is under-diagnosed, making it difficult to determine the true frequency in the general population. Granulomatosis with polyangiitis affects Caucasians most often, but can affect people of any race or ethnic background anywhere in the world.
A diagnosis of granulomatosis with polyangiitis is made based upon a thorough clinical evaluation including routine laboratory tests, a detailed patient history, identification of classic symptoms, and a variety of specialized tests. In many people, surgical removal (biopsy) and microscopic examination of small samples of tissue from an affected organ may reveal characteristic vasculitis or granulomas. Physicians may initially choose to obtain a biopsy sample form the upper respiratory tract. However, sometimes this does not yield enough tissue to a definitive diagnosis. A biopsy of lung tissue or kidney tissue may be done. A lung biopsy may yield the best results.
In addition to biopsy, blood tests may be performed to rule out other disorders. A blood test may also reveal the presence of a specific type of antibody known as antineutrophil cytoplasmic antibody (ANCA). Because the test is positive in many individuals with granulomatosis with polyangiitis, the ANCA blood test may help support a suspected diagnosis of the disorder. This test does not differentiate from other forms of ANCA-associated vasculitis like microscopic polyangiitis or Churg-Strauss syndrome. Some people with other conditions may also test positive for ANCA including people with bacterial endocarditis, systemic lupus erythematosus, amebiasis, tuberculosis, and people who abuse cocaine. In addition, in some affected individuals, the test is negative and, therefore, should not be used in place of a biopsy to determine whether someone has granulomatosis with polyangiitis.
X-ray and specialized imaging tests are also helpful in supporting a suspected diagnosis of granulomatosis with polyangiitis. X-rays of the lungs or sinuses may reveal characteristic findings associated with the disorder (e.g., thickening of the lining of the sinus), can help to rule out other conditions, and can help to reveal the extent of the disorder.
The treatment of granulomatosis with polyangiitis is directed toward the specific symptoms that are apparent in each individual. Modern treatment has dramatically improved the life expectancy and reduced organ damage in patients with granulomatosis with polyangiitis. Treatment may require the coordinated efforts of a team of specialists. Pediatricians or primary care physicians; specialists who diagnose and treat lung disorders (pulmonologists); specialists who diagnose and treat disorders of the ear, nose and throat (otolaryngologists); specialists who diagnose and treat disorders of the immune system (immunologists): specialists who diagnose and treat disorders of the musculoskeletal system and autoimmune diseases (rheumatologists); specialists who diagnose and treat the kidneys (nephrologists); specialists who diagnose and treat skin disorders (dermatologists); specialists who diagnose and treat disorders of the central nervous system and the brain (neurologists), specialists who diagnose and treat heart disorders (cardiologists); and other healthcare professionals may need to systematically and comprehensively plan an affected child’s treatment.
Specific therapeutic procedures and interventions may vary, depending upon numerous factors, such as the presence or absence of certain symptoms; specific organs affected, overall severity of the disorder; an individual’s age and general health; and/or other elements. Decisions concerning the use of particular drug regimens and/or other treatments should be made by physicians and other members of the health care team in careful consultation with the patient based upon the specifics of his or her case; a thorough discussion of the potential benefits and risks, including possible side effects and long-term effects; patient preference; and other appropriate factors.
The treatment of granulomatosis with polyangiitis can be broken down into two stages – inducing a remission of symptoms and then maintaining the remission (maintenance therapy).
In 2011, the Food and Drug Administration (FDA) approved the use of rituximab (Rituxan®) in conjunction with glucocorticoids for the treatment of individuals with granulomatosis with polyangiitis. Rituximab is classified as a monoclonal antibody or biologic therapy – medications that act like antibodies, but are artificially created in a lab. Glucocorticoids are steroid hormones that are important in the regulation of the metabolism of a form of sugar called glucose and the modulation of the body’s response to stress such as reducing inflammation.
For many years, affected individuals were treated with a combination of glucocorticoid drugs that reduce inflammation such as prednisone and cytotoxic drugs that impede the abnormal growth (proliferation) of cells. Cytotoxic drugs contain chemicals that are toxic to certain cells in the body. These drugs are used to block the growth or replication of these cells. Cyclophosphamide (Cytoxan®) is the most common cytotoxic drug used to treat granulomatosis with polyangiitis.
When using a combination of a cytotoxic drug with a glucocorticoid, the duration of therapy depends on an affected individual’s response. White blood cell (leukocyte) counts are closely monitored. Dosages are reduced gradually to prevent severe deficiency of white blood cells. Attempts should be made to discontinue cytotoxic therapy if symptoms of the disorder have been absent for three to six months. Cytotoxic therapy can be replaced with another drug such as azathioprine (Imuran®) or mycophenolate mofetil (CellCept®). The possibility of kidney disease relapse is carefully monitored when reducing (tapering) medication dosage or discontinuing the drug. Methotrexate is another drug used to treat granulomatosis with polyangiitis. Glucocorticoids with methotrexate are sometimes used to treat less severe disease. Generally, methotrexate, mycophenolate mofetil and azathioprine are less toxic than cyclophosphamide.
After remission has been achieved, maintenance therapy may be required. This usually involves using azathioprine, methotrexate, or rituximab. Cyclophosphamide is usually not used for maintenance therapy due to toxicity. The dose of glucocorticoids is usually lowered in stages (tapered) as well.
Regardless of which therapy is used to achieve remission and for maintenance therapy, affected individuals can experience a recurrence (relapse) of the disorder, which can be called a “flare”. Sometimes, a relapse may be triggered by an infection.
Some affected individuals may have progressive kidney damage, and the kidneys may no longer be able to perform their normal functions. This necessitates dialysis and eventually a kidney transplant. Dialysis is a procedure in which a machine is used to perform the kidney’s basic functions of fluid and waste removal. Kidney transplantation has been successful for kidney failure resulting from granulomatosis with polyangiitis.
Antibiotics have been used to treat secondary bacterial infections sometimes associated with granulomatosis with polyangiitis. Trimethoprim-sulfamethoxazole (Bactrim® and Septra®) is an antibiotic that has been effective in treating affected individuals, particularly those with only upper airway involvement. It is often used to reduce the possibility of a lung infection called Pneumocystis jiroveci pneumonia, especially when intensive immune suppressive therapy is needed to control the vasculitis.
Surgical intervention for subglottic stenosis or tracheal stenosis to maintain the airways may be necessary in some people. Surgery can fix a saddle nose if the underlying vasculitis is not active.
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Some individuals with granulomatosis with polyangiitis who had severe disease that was resistant to other therapies received treatment with plasmapheresis. Plasmapheresis is a method for removing unwanted substances (toxins, metabolic substances and plasma components) from the blood. During plasmapheresis, blood is removed from the affected individual and blood cells are separated from plasma. The plasma is then replaced with other human plasma and the blood is transfused back into the affected individual. This procedure was used with patients with ANCA-associated vasculitis with active kidney disease (glomerulonephritis) and/or alveolar hemorrhaging. Plasmapheresis may be used along with the drug therapies described above. Plasmapheresis as a therapy for granulomatosis with polyangiitis has not been studying in clinical trials and its long-term effectiveness and safety has not been established.
Research physicians at the National Institute of Allergy and Infectious Diseases (NIAID), a part of the National Institutes of Health (NIH), have studied granulomatosis with polyangiitis and possible treatments for many years. In 2003, the NIH awarded a grant to establish a multicenter research network known as the Vasculitis Clinical Research Consortium (VCRC). This consortium fosters and facilitates clinical investigation of Wegener’s granulomatosis and related diseases. The VCRC consists of 18 academic medical centers in the United States and Canada that conduct investigator-initiated clinical and translational research. Additional information about the VCRC can be found at: www.rarediseasesnetwork.org/vcrc
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