Wegener's granulomatosis (Granulomatosis with polyangiitis)

2021-08-08 10:14 PM

Early diagnosis and treatment of Wegener's granulomatosis can result in a complete recovery. Without treatment, Wegener's granulomatosis is potentially fatal, most commonly from kidney failure.

Define

Wegener's granulomatosis is a rare disorder that causes inflammation of the blood vessels, restricting blood flow to various organs. Most commonly, Wegener's granulomatosis affects the kidneys, lungs, and upper respiratory tract. Restricting blood flow to these organs can damage them.

In addition, vasculitis occurs when Wegener's granulomatosis produces a type of inflammatory cell called a granuloma, which is found around blood vessels. Granulomas can destroy normal tissue. The cause of Wegener's granulomatosis is not known.

Early diagnosis and treatment of Wegener's granulomatosis can result in a complete recovery. Without treatment, Wegener's granulomatosis is potentially fatal, most commonly from kidney failure.

Symptoms

The signs and symptoms of Wegener's granulomatosis can develop suddenly or gradually.

The first symptoms of Wegener's granulomatosis are usually related to the respiratory tract. However, a rapidly worsening condition often affects the blood vessel and the organs it supplies, such as the kidneys. Signs and symptoms may include:

Frequent runny nose, filled with pus.

Epistaxis.

Sinus pain and inflammation.

Hoarseness.

Gingivitis.

Ear infection.

Cough.

Chest pain.

Hemoptysis.

Shortness of breath.

Wheeze.

Uncomfortable.

Unintentional weight loss.

Joint pain and swelling.

Blood in the urine.

Skin sores.

Red, burning, or painful eyes.

Double vision or decreased vision.

Fever.

Weakness and fatigue, possibly linked to a low red blood cell count (anemia).

For some people, the disease is limited to the respiratory tract and does not involve the kidneys. Kidneys are involved, often causing no symptoms initially in the course of the disease, and can be detected by blood and urine tests alone. In renal failure, however, anemia is involved, which often occurs.

See your doctor if the persistent runny nose doesn't respond to prescription (OTC) cold medicine, especially if it's accompanied by nosebleeds and resembles pus, coughing up blood, or other possible signs and symptoms. of Wegener's granulomatosis. Because the disease can progress quickly, early detection is critical to receiving effective treatment.

Causes

The cause of Wegener's granulomatosis is unknown, but it appears to develop as a result of an initial inflammatory event, which triggers an abnormal response from the immune system. The combination of these events can lead to inflammation, narrowing of blood vessels, and masses of inflammatory harmful tissue (granulomas). Scientists speculate that the triggering event may have been an infection, but no specific infection has been identified as the cause.

Risk factors

The onset of Wegener's granulomatosis can occur at any age, but it usually occurs in middle age, with an average onset at age 40. Whites are more likely than blacks to develop it. Wegener's granulomatosis.

Complications

Besides affecting the upper and lower respiratory systems, Wegener's granulomatosis can affect other organs, including the skin, eyes, ears, kidneys, spinal cord, and heart. Complications may include:

Hearing poorly. Inflammation of the granulomatous tissue in the middle ear can lead to hearing loss.

Skin scars. Pain may develop and may lead to severe scarring.

Heart problem. Wegener's granulomatosis can affect the arteries of the heart, leading to chest pain or a heart attack.

Kidney problems. As Wegener's granulomatosis progresses, it has the potential to affect the kidneys. Can lead to glomerulonephritis, a type of kidney disease that interferes with the body's ability to remove waste and excess water, leading to a build-up of waste products in the blood (uremia). Renal failure is a leading cause of death from Wegener's granulomatosis.

Tests and diagnostics

In addition to asking about signs and symptoms, doing an exam, and asking about your medical history, your doctor may order a number of tests, including:

Blood tests. If your doctor suspects Wegener's granulomatosis, blood tests can detect certain proteins (autoantibodies) in the blood called anti-cell autoantibody neutrophils (ANCA). Autoantibodies appear in the blood in 90 to 95 percent of people with active Wegener's granulomatosis. The presence of autoantibodies supports the diagnosis of Wegener's granulomatosis, but it is not sufficient to confirm the presence of the disease.

Blood tests can also measure the erythrocyte sedimentation rate - commonly known as the sed rate. By measuring the red blood cells that fall to the bottom of a blood tube over a period of one hour, this test can indicate the level of inflammation in the body. Normally, red blood cells fall faster when inflammation is present. The sed rate is also measured during treatment, to check for disease activity.

Blood tests can check for anemia, which is common in people with this condition.

A creatinine test can evaluate.

Urine test. Tests evaluate kidney function to determine if disease is affecting the kidneys.

X-ray. This test shows cavities or masses in the lungs. However, it cannot distinguish between Wegener's granulomatosis and other lung diseases.

Biopsy of affected tissue. The only way to confirm the diagnosis of Wegener's granulomatosis is to remove a small piece of tissue from an affected organ (biopsy) and examine it under a microscope. The doctor may remove cells from the nasal passages, airways, or lungs to confirm or refute the presence of vasculitis and granulomas. Other areas for biopsy may include the skin or kidneys. Some biopsies can be done on an outpatient basis using an anesthetic (local anesthetic). Others, such as an open lung biopsy, may require hospitalization.

Treatments and drugs

With early diagnosis and proper treatment, Wegener's granulomatosis is reversible within a few months. In some cases, treatment may be needed. Because the disease can recur, your doctor will continue to monitor your condition after treatment.

Medicine

Corticosteroids. Medicines such as prednisone help suppress the immune system and treat early signs and symptoms of Wegener's granulomatosis.

Immunosuppressive drugs. Most need an immunosuppressive drug, such as cyclophosphamide (Cytoxan), azathioprine (Imuran), or methotrexate (Rheumatrex), to combat the body's immune response.

When standard treatment is not effective, some physicians experienced in drug therapy use the Wegener trial. One of the drugs is rituximab (Rituxan). It reduces the number of a type of cell in the body (B cells) that is associated with inflammation. The most recent studies suggest that rituximab may be as effective as cyclophosphamide in the treatment of severe cases of Wegener's granulomatosis, and maybe even better for treating recurrent Wegener.

Side effects of treatments

Because of the potential side effects of the drugs used to treat Wegener, such as interfering with their ability to fight infection, your doctor will monitor your condition while you're taking them. Medications that may be prescribed to help prevent related side effects include:

Trimethoprim-sulfamethoxazole (Bactrim, Sepxra) to prevent lung infections.

Bisphosphonates (Fosamax) to prevent osteoporosis associated with prednisone use.

Folic acid, the synthetic form of vitamin B folate, prevents ulcers and the signs and symptoms associated with folate depletion in the body from methotrexate use.

Surgery

If you already have kidney failure with Wegener's granulomatosis, a kidney transplant may be considered to restore normal kidney function. Successful transplantation depends on finding an organ that minimizes the risk that the body will reject it and following a treatment plan that will allow the body to accept the new organ.

Talk to your doctor to determine if a kidney transplant might be an option. Eligibility to receive a donor's kidney will depend on other organs that have been affected by the disease.

Coping and supporting

With treatment, Wegener's granulomatosis is reversible. However, it is possible to feel stressed about the possibility of relapse or about any damage the disease may cause, such as to the kidneys. Here are some suggestions for dealing with this disease:

Learn about Wegener's granulomatosis. The details are known, better preparedness is possible to deal with complications, drug side effects, or possible relapses. Besides talking to your doctor, maybe talk to a counselor or social worker. Or may find it helpful to talk to others who have Wegener's granulomatosis.

Maintain a support system. Family and friends can help as they go through the diagnosis and treatment process. It is also possible to find the care and understanding of others with information and comfort about Wegener's granulomatosis. Your doctor or healthcare worker can help you get in touch with a support team.