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Wegener's Granulomatosis

Definition


Disease: Wegener's Granulomatosis Wegener's Granulomatosis
Category: Tumors

Disease Definition:

Wegener’s granulomatosis, which is a rare disorder that causes inflammation of the blood vessels that restricts blood flow to various organs, is not an infection or a type of cancer, but its exact cause is still not known. The kidneys, lungs and upper respiratory tract are the most commonly affected organs by Wegener’s granulomatosis. This restricted blood flow can damage these organs. Wegener’s granulomatosis also produces a type of inflammatory tissue called granuloma, which is found around the blood vessels and can destroy normal tissue. Even though Wegener’s granulomatosis can be fatal when left untreated, most commonly from kidney failure, but in the case of early diagnosis and treatment, the patient can make a full recovery.
 

Work Group:


Symptoms, Causes

Symptoms:

The signs and symptoms of this disorder can either develop gradually or suddenly. Even though the first signs and symptoms of Wegener’s granulomatosis involve the respiratory tract, but the condition worsens rapidly, and may end up affecting the blood vessels and the organs they supply. Some of the signs and symptoms of Wegener’s granulomatosis include:

  • Cough
  • Constant runny nose
  • Appetite loss
  • Sinus pain and inflammation (sinusitis)
  • Shortness of breath
  • Skin sores
  • Coughing up blood
  • Weakness, which is associated with low red blood cell count (anemia)
  • Fever
  • Swelling and pain of the joints
  • Malaise (general ill feeling)
  • Ear infections
  • Double or decreased vision
  • Nosebleeds
  • Eye redness, burning or pain

 

In some cases, the disease is limited to the respiratory tract without involving the kidneys. However, when the kidneys are involved, no signs or symptoms will appear in the early course of the disease, unless with blood or urine tests. Nevertheless, kidney failure with its associated anemia will occur in time.

 

Because of the quick progress of this disease, early diagnosis is critical to receiving effective treatment. So, in case a person is suffering from a persistently runny nose that doesn’t respond to over-the-counter cold medicines, he/she should contact a doctor, especially if it’s accompanied by coughing up blood, nosebleeds and pus-like material or some of the other possible signs of this disease.
 

Causes:

Even though the exact cause of Wegener’s granulomatosis is not known, but it appears to develop as a result of an initial inflammation-causing event which triggers an abnormal reaction from the immune system. The combination of these events can lead to inflamed and constricted blood vessels as well as inflammatory tissue masses (granulomas). Some think that the initial event might be an infection, which appears to be the case in nearly half the people with Wegener’s granulomatosis, but these infections aren’t overwhelming. Some of the other causes could be a genetic predisposition to the condition, an environmental toxin, or a combination of all these.
 

Complications

Complications:

Wegener’s granulomatosis could affect the skin, eyes, ears, heart, spinal cord and kidneys, in addition to the upper and lower respiratory tracts. Some of the complications involving this disease could include:

 

Skin scarring:

Sores could develop, which may lead to severe scarring.

 

Heart attack:

Chest pain or heart attack could be the result of Wegener’s granulomatosis’ affect on the arteries of the heart.

 

Hearing loss:

This can be caused by the inflammation of granulated tissue in the middle ear.

 

Kidney (renal) damage:

The kidneys are likely to be affected as the disease progresses, which can lead to glomerulonephritis, a kidney disease that hampers the kidney’s ability to remove waste and excess fluid from the body, which in turn leads to a buildup of waste products in the bloodstream (uremia). One of the leading causes of death from Wegener’s granulomatosis is kidney failure.
 

Treatments:

Even though in some cases a long treatment could be necessary, but with early diagnosis and appropriate treatment, a person may recover from this disease within a few months. However, the doctor will continue to monitor the patient’s condition after treatment because of the chance of recurrence of this disease.

 

MEDICATIONS:

In order to treat the early signs and symptoms of Wegener’s granulomatosis, the patient may be prescribed corticosteroids, such as prednisone. Although corticosteroids may sometimes be enough, but in most cases another immunosuppressive drug is required to counteract the body’s immune reaction including azathioprine, methotrexate or cyclophosphamede.

 

Some experienced doctors in treating Wegener’s are using experimental drugs in case of the failure of standard treatments, such as rituximab; this medication reduces the number of B cell in the body, which is involved with inflammation. However, to consider this drug a standard treatment, further studies should be done. Although rituximab was developed to treat non-Hodgkin’s lymphoma, a type of cancer, but it has also been approved for the treatment of rheumatoid arthritis.

 

The patient’s condition will be monitored while taking these drugs, because of the possible side effects of the drugs used in the treatment of Wegener’s granulomatosis, such as hampering the body’s ability to fight off infection. In order to prevent drug-related side effects, the patient will be prescribed:

  • Biphosphonates, in order to prevent osteoporosis, which is the bone loss associated with the use of prednisone.
  • Folic acid, in order to prevent sores and other signs and symptoms associated with the depletion of folate in the body from methotrexate use. Folic acid is a synthetic form of the B vitamin folate.
  • Trimethoprim-sulfamethoxazole, in order to prevent lung infection.

 

SURGERY:

A person should consider a kidney transplant to restore normal kidney function in case he/she has had kidney failure as a result of Wegener’s granulomatosis. Finding an organ that minimizes the chances of the body’s rejection of it and following a treatment regimen that will allow the body to accept this new organ is what a successful transplant depends on. How the patient’s other organs have been affected by the disease is what his/her eligibility to receive a donated kidney will depend on. To determine whether a kidney transplant is the right option for a person, he/she should talk to a doctor.
 

Prognosis:

Not available

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