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Granulomatosis with Polyangiitis

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Definition and Facts About Granulomatosis with Polyangiitis (Wegener's)

  • Granulomatosis with polyangiitis is a rare disease in which blood vessels become inflamed (a condition called vasculitis) and localized, nodular collections of abnormal inflammatory cells, known as granulomas, are found in affected tissues.
  • Granulomatosis is the term that refers to the presence of granulomas, which are small areas of inflammation filled with immune cells.
  • The inflammation can affect various parts of the body, such as the respiratory tract (nose, sinuses, windpipe, and lungs) and kidneys.
  • When blood vessels become inflamed, blood flow to the organs is disrupted, which can damage the organs.
  • Granulomatosis with polyangiitis is rare, and its exact frequency is difficult to determine.
  • The disease can affect anyone at any age. However, it affects mostly white people, and is most common in middle-aged adults, with an average age of onset between 40 and 65 years.
  • With proper diagnosis and treatment, ganulomatosis with polyangiitis can be slowed or stopped.

What Causes Granulomatosis with Polyangiitis?

No one knows the cause of granulomatosis with polyangiitis is thought to be an autoimmune disorder in which the body's defense system attacks itself and destroys normal body tissue. Normally, the body's immune system acts as a defense against outside invaders. With an autoimmune disorder, something triggers an immune response causing the body to react against its own tissue. The result is that tissue can be damaged. With granulomatosis with polyangiitis, the organ systems that are attacked include the respiratory tract and kidneys.

What Are the Symptoms of Granulomatosis with Polyangiitis?

Upper respiratory tract (most common):

  • Sinusitis, sinus pain, bacterial infection in sinuses
  • Constant runny nose (worse than that caused by a common cold)
  • Bloody nose
  • Sores around the opening of the nose
  • Hole develops in cartilage in nose
  • Ear infections, ear canals blocked, hearing loss

Lungs (usually present):

Other symptoms (less common):

  • Muscle and joint pain, joint swelling
  • Redness and burning or pain in the eyes (conjunctivitis) and swelling in the eye
  • Double vision or decrease in vision (requires immediate medical care)
  • Sores on the skin that look like small red or purple raised areas (they may or may not cause pain)
  • Hoarseness of the voice
  • Fever, night sweats
  • Loss of appetite, weight loss

The kidneys are affected in three-fourths of people with granulomatosis with polyangiitis, but the person may have no obvious symptoms related to kidney damage. Urine, however, may be bloody or red in color.

How Is Granulomatosis with Polyangiitis Diagnosed?

Lab tests, imaging studies, and biopsies may be performed.

  • There are no blood tests that can establish the diagnosis of granulomatosis with polyangiitis with 100% certainty. Instead, blood tests are used to rule out other causes of the symptoms and to confirm the presence of inflammation in the body. Blood tests are also used to check the blood for antibodies the body is making to attack itself.
  • One specific antibody that is often found in people with granulomatosis with polyangiitis is called antineutrophil cytoplasmic antibody (ANCA). Antibodies are proteins that fight disease. If the ANCA test is positive, it would be a strong indicator the person has granulomatosis with polyangiitis.
  • The red blood cell count may be low, and the white blood cell count may be high (indicating infection).
  • A urine test will look for certain red blood cell markers in the urine caused by problems with the kidneys.
  • X-rays are taken of the chest and sinus cavities.
  • CT scans of the chest and sinuses may give a clearer view of the process.
  • The definitive way to establish the diagnosis is examination of affected tissues under a microscope (biopsy). Small samples of tissue (biopsies) may be taken from areas of inflammation, such as the nose or throat. These samples are taken with a bronchoscope, an instrument that is guided down the throat to view the airway and snip out a tissue sample. Tissue samples from the lung are taken through a small cut in the chest wall. Tissue samples from the kidney are also taken through a small cut.

If a person has two or more of these indicators, under guidelines established by the American College of Rheumatology, a diagnosis of granulomatosis with polyangiitis can be made:

  • Inflammation of the mouth or nose, bloody nose, and sores in the mouth (that may or may not be painful) are present.
  • Abnormalities are seen on a chest X-rays
  • A high number of red blood cells are present in the urine (can be seen under a microscope).
  • There is a certain type of inflammation in tissue taken as a sample during a surgical biopsy.
    • Tissue from the sinuses, lungs, or kidneys is examined under a microscope.
    • Vasculitis (inflammation) and the specific type of inflammation called granulomas may be found, indicating a diagnosis of granulomatosis with polyangiitis.

What Is the Treatment for Life Expectancy for Granulomatosis with Polyangiitis?

The goal of treatment for granulomatosis with polyangiitis is to bring about remission, maintain remission, and treat the disease if it becomes active again (relapse).

Treatment for granulomatosis with polyangiitis with medication can bring long-term remission for most people. Remission means the disease disappears or its progression is slowed, but the disease is not cured.

Without medical treatment, a person diagnosed with granulomatosis with polyangiitis has a high risk of dying of the disease within two years, usually from lung or kidney failure.

  • People with severe granulomatosis with polyangiitis need urgent care because their condition is life-threatening.
  • A less sever form is called limited granulomatosis with polyangiitis.

Both types are treated with similar medications. Surgery may  be necessary.

Because granulomatosis with polyangiitis affects so many different parts of the body, a healthcare team of doctors who specialize in lung disease (pulmonologists), kidney disease (nephrologists), arthritis, and similar conditions (rheumatologists), and ear, nose, and throat diseases (otolaryngologists).

Medications

Remission is likely in most people when treated with medications. Most respond to the drug cyclophosphamide (Cytoxan), and a majority have complete remission.

In up to half of people who respond to a course of medications, the disease eventually becomes active again (relapse), and then therapy is begun again.

  • To bring about remission, people with severe granulomatosis with polyangiitis may be given corticosteroids, for example, prednisone, for the first few months and cyclophosphamide, which is taken by pill form for up to a year. Sometimes cyclophosphamide may be given once per month through an IV injection (called pulse therapy). Blood tests are needed every two weeks to check for a low white blood cell count, which could weaken the immune system and cause infection easily.
  • People with limited granulomatosis with polyangiitis may be treated with steroids and with drugs called immunosuppressive agents (such as methotrexate) to bring about remission.
  • A person who has a relapse may be given cyclophosphamide and prednisone and possibly an antibiotic.

These are powerful medications and may cause toxic side effects. It is important to know what to expect during treatment and discuss all side effects with the doctor. With careful monitoring, side effects can be reduced.

Side effects of these drugs:

  • Prednisone: Ideally, corticosteroids are used for only a short time in order to bring sudden flares in symptoms under control. Long-term use is associated with serious side effects, such as osteoporosis, glaucoma, cataracts, mental changes, abnormal blood glucose levels, or arrested bone growth in children before puberty. After prolonged use, the corticosteroid dose must be gradually decreased over weeks to months to avoid corticosteroid withdrawal syndrome.
  • Cyclophosphamide: Blood cell counts are monitored regularly to watch for toxic effects. The urine is examined regularly for red blood cells, which may be the first sign of hemorrhagic cystitis (severe inflammation of the bladder that causes blood in the urine). It is important to drink lots of fluids while on this drug to prevent hemorrhagic cystitis.
  • Methotrexate: To guard against problems, kidney and liver function are monitored on a regular basis, as are blood cell counts. Methotrexate may cause toxic effects on blood, kidneys, liver, lungs, and gastrointestinal and nervous systems.

Surgery

  • Inflamed tissue may show necrosis (localized tissue death) and need to be surgically removed. Such tissue would be located in the nose, throat, and lungs. In the nose, this may cause frequent nosebleeds and a perforated nasal septum.
  • Problems with middle ear infections can be remedied by surgically inserting ear tubes through the eardrum to allow the ear to drain.
  • For people with severe kidney failure, a kidney transplant may be a lifesaving option.

When to Seek Medical Care for Granulomatosis with Polyangiitis

Talk with the doctor about any of these symptoms. Eye problems are cause for immediate medical attention. Other symptoms that may cause a person to contact a doctor would be chest pain, coughing up blood, or seeing blood in the urine.

Sinus Infection Symptoms and Signs

A sinus infection or sinusitis can either be acute (short-term) or chronic (long-term). Signs and symptoms of a sinus infection include:

  • Facial swelling
  • Sore throat
  • Bad breath
  • Tooth pain
  • Sensitive teeth
  • Dizziness
  • Pain or pressure around the eyes or cheeks

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Reviewed on 10/12/2018
Sources: References

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