Wegner's Granulomatosis

1 What is Granulomatosis with Polyangiitis?

Granulomatosis with polyangiitis is an inflammation of the blood vessels in the nose, sinuses, throat, kidneys and the lungs.

Granulomatosis with polyangiitis is also called Wegener’s granulomatosis which is a blood vessels disorders also called vasculitis.

This causes the slowing of blood flow into some of the organs. The tissue when affected leads to granulomas which influences how the organs work.

When the disease is detected earlier, it can be managed to full recovery. However, it can be very fatal when not treated.

2 Symptoms

The signs and symptoms of granulomatosis with polyangiitis can develop suddenly or even take several months.

The first signs start in the respiratory tract which leads to the effects on the sinuses, throat, and or lungs.

The condition of victims of this disease get worse rapidly leading to the effect of inflammation of the blood vessels leading to the damage of the blood supply to the kidneys.

The symptoms include:

  • running nose,
  • stuffiness,
  • sinus infection and nosebleeds,
  • some people may caught,
  • with blood phlegm,
  • shortness of breath or wheezing may also be experienced,
  • fever,
  • fatigue and general body ache,
  • and pains.

Sometimes, the numbness of the limbs, fingers or toes may occur. Some people will lose weight; blood may be seen in urine, sores in the skin, redness in the eye, burning or pain, and eye infections.

The disease may affect the lungs only in some people; kidney may also be affected without warning signs. Blood and urine test can be used for the diagnosis of the condition.

In the absence of the treatment, kidney failure and anemia may occur.

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3 Causes

The cause of granulomatosis with polyangiitis is not known.

It appears to develop after an infection or any inflammatory conditions or causing events which may trigger an abnormal reaction from the immune system.

This may cause the formation of inflamed or constricted blood vessels and harmful inflammatory tissue mass known as granulomas.

This may cause the destruction of the normal tissues and narrowed blood vessels which reduce the amount of blood and oxygen that gets to the body tissues and organs.

4 Making a Diagnosis

Making a diagnosis of granulomatosis with polyangiitis is done by performing several tests.

The doctor will get to find out about the signs and symptoms.

A physical exam may be performed and medical history. A diagnostic test may also be used and procedures followed in the detection of the conditions associated with the disease.

They may include:

  • Blood tests which look for information on signs of inflammation by screening for the levels of c – reactive proteins or high levels of erythrocytes sedimentations.
  • Test for presence of antibodies for neutrophils and other cytoplasmic antibodies test may be useful.
  • Blood test can also be used for detection of anemia which occurs commonly in people with this medical condition.
  • Moreover, urine test can be used for detection of red blood cells or more proteins which is an indication of kidney damage.
  • Imaging tests for the chest may be applied to diagnose for any signs of damage in the blood vessels located in the lungs and Kidney. X-rays, CT and MRI can be applied.
  • Biopsies can be collected from the damaged organs to confirm the presence of granulomas polyangiitis.

5 Treatment

You and your doctor together will determine the best course of treatment for granulomatosis with polyangiitis.

It is necessary that you talk with your medical doctor on the best drugs and combination of drugs that will best serve you.

It will also be best to avoid the side effects. The form of treatment will depend on how the organs are affected. The doctor will prescribe corticosteroids as a way of suppressing the immune system and reducing inflammation of the blood vessels.

Several other drugs may be used to lower the immune system and cause inflammation such a cyclophosphamide, azathioprine and methotrexate. Tituximab can also be used under severe cases and serves as an equivalent of cyclophosphamide.

When the condition is under control, drugs may also be used to prevent relapse. Drugs may also be used to reduce the effects of the treatments administered such as lung infections for sulfamethoxazole-trimethoprim, bisphosphonates for osteoporosis and manage symptoms of Folate depletion.

Other treatments include use of plasma exchange or kidney transplant.

6 Risks and Complications

There are several risks and complications associated with granulomatosis with polyangiitis.

This condition can occur at any age and most often, people within the age bracket of 40-65.

Early diagnosis of the disease will allow early treatment. One will however require the use of prescription drugs for a long duration of time to reduce reoccurance.

One is expected to also visit the doctor regularly so that one’s progress can be continually monitored. This condition affects the nose, throat and lungs.

It may also affect the ears, eyes, heart and other key body organs.

Some of the complications may include:

  • hearing,
  • skin scars,
  • heart disorders,
  • kidney damage,
  • loss of height
  • and deep vein thrombosis.
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