Reviewed By: Pramod Kerkar, MD, FFARCSI

Wegener's Granulomatosis is a medical condition that was described first in 1897 by a Scottish otolaryngologist Peter McBride. He cited about Wegener's Granulomatosis in a medical article with the title "Photographs of a case of rapid destruction of the nose and face". The Anatomical physiology was cited by Ernst Kinger. A German pathologist named Friedrich Wegener gave the detailed report of this disease.

Wegener’s Granulomatosis

Pathegeric granulomatosis was the initial name that got coined for this medical condition. Till today, Wegener's Granulomatosis gets confused with two types of malignant lymphomas namely lymphomatoid granulomatosis and lethal midline granuloma.

Definition of Wegener's Granulomatosis

GPA (Granulomatosis with polyangiitis) also termed as Wegener's granulomatosis is the disorder of the vascular system that involves both polyangiitis and granulomatosis. This disease is named after Friedrich Wegener who described it in the year 1936. Following are some of the features of Wegeners granulomatosis.

  • Wegener's Granulomatosis is a form of inflammation of small and medium sized vessels in multiple organs
  • This disease causes damage to lungs and kidneys which can turn fatal
  • Long term immunosuppression is required for treating Wegener's Granulomatosis
  • The major area affected by Wegener's Granulomatosis is sinus along with kidneys.

Signs and Symptoms of Wegener's Granulomatosis

Wegener's Granulomatosis in majority of cases affects the upper part of the respiratory track while any other organ tends to be affected as well. Some of the major symptoms of Wegener's Granulomatosis are as follows:

Symptoms of Wegener's Granulomatosis

Maximum Wegener's Granulomatosis patients, at a very initial stage, observe some major issues with the respiratory track. The symptoms include:

  • Continuously running nose
  • Formation of nasal crusts and sores
  • Facial or nasal pain
  • Nose bleeding with unusual discharge
  • Bloody phlegm cough
  • Chest discomfort
  • Shortness of breath
  • Wheezing
  • Middle ear inflammation

Other Possible Symptoms of Wegener's Granulomatosis That Are Not So Common Include:

  • Inflammation of eyes without loss of vision
  • Arthritis or joint pains
  • Skin sores
  • Rashes
  • Kidney inflammation
  • Muscle pain

Epidemiology of Wegener's Granulomatosis

Wegener's Granulomatosis is a rare disease. As low as 10-20 cases per million of population occur in a particular year. Also, this disease is the rarest in Japanese and Africans.

Prognosis of Wegener's Granulomatosis

The survival rate for 5 years is 80% for Wegener's Granulomatosis with the help of corticosteroids and cyclophosphamide. Long term complication includes an unceasing kidney failure, loss of hearing and even deafness in around 86% of Wegener's Granulomatosis cases.

Before the treatments were devised, 90% of the people suffering from Wegener's Granulomatosis had a two year mortality rate with an average survival time of five months. In Wegener's Granulomatosis, death results due to the complete respiratory failure.

Causes of Wegener's Granulomatosis

The major cause of Wegener's Granulomatosis is unknown. It is not a form of cancer and is not spread by contact. It also doesn't occur from generations to generations. The immune system plays a major role in this disease by causing the inflammation and damage of blood vessels and tissues.

Risk Factors and Complications of Wegener's Granulomatosis

Wegener's Granulomatosis can begin at any age. In majority of cases, it is seen to start during the middle ages like 40 – 65 years. Whites are more prone to Wegener's Granulomatosis in comparison to blacks. Some of the complications associated with this disease are as listed under.

Risk Factors and Complications of Wegener's Granulomatosis

  1. Loss of Hearing: This is caused by the inflammation of the granulated tissues in the middle ear.
  2. Scarring of Skin: The development of sores leads to scarring.
  3. Heart Attack: Wegener's Granulomatosis can affect the arteries that can cause chest pain and finally heart attack.
  4. Renal or Kidney Damage: Wegener's Granulomatosis can lead to glomerulonephritis which is a type of kidney disease that hampers the process of waste removal from kidneys. The waste hence builds up in the blood and results in the kidney damage.

Diagnosis of Wegener's Granulomatosis

Physical examination and medical history of a patient is taken into consideration while diagnosing this problem. Following are the tests which are conducted as a part to diagnose Wegener's Granulomatosis:

  • Blood Test: Blood test helps in the detection of the certain proteins or auto antibodies called Neutrophil cytoplasmic auto antibodies (ANCA). These antibodies confirm the diagnosis of Wegener's Granulomatosis. Blood test for creatinine also confirms that the kidneys are not properly filtering the waste products from the body.
  • Urine test: It helps to determine whether Wegener's Granulomatosis is affecting kidneys or not.
  • X ray of chest: This is used to indicate cavities in the lungs.
  • Biopsy: This is the best way to determine this disease. It is done by taking a small piece of affected tissue from either nose or lungs.

Treatment of Wegener's Granulomatosis

The treatment for Wegener granulomatosis needs to be continued for a span of 18-24 months. Treatment part includes treatment for side effects, medication and other form of treatment.

  • Medications: The medicines prescribed by the doctor to treat Wegener's Granulomatosis are as follows.
    • Corticosteroids: These medicines like prednisone suppress the immune system to treat the early symptoms.
    • Immune Suppressant Medications: Drugs like Cytoxan, Azasan Imuran and Rheumatrex Trexall are used as immunosuppressive drugs to offset the immune system.
    • Biologic Therapy: Rituxan is used to reduce the B cells in the body which is involved in causing inflammation. Rituximab is used in treating severe cases.
  • Treatments for Side Effects: The medications given to treat Wegener's Granulomatosis lower down the body's ability to fight the infections. Thus, the drugs prescribed to help prevent the drug related side effects are:
    • Sulfamethoxazole Trimethoprim: It helps to prevent the lung infection.
    • Folic acid: It is a synthetic form of Vitamin B Folate. This helps to prevent sores.
    • Fosamax: This medicine is made of biphosphonates and helps prevent bone loss.
  • Other Treatments: The other treatments used for Wegenr's Granulomatosis are as listed under.
    • Plasmapheresis: This treatment is also known as plasma exchange as it removes the liquid part of plasma and separates it from blood cells. This can help to recover the kidneys.
    • Surgery: Kidney transplant through surgery can restore the normal function of the kidney.

Coping with Wegener's Granulomatosis

The patient needs to equip himself well with the knowledge regarding this disease. The more you know, the better it is to accept and to deal with Wegener's Granulomatosis. A strong support from family and friends can help lighten the burden and take stress away. Joining a support group also helps in coping with the condition and helps in boosting the confidence. Moreover, eating a balanced diet and taking care of your health is the only way to deal with this disease.

Pramod Kerkar

Written, Edited or Reviewed By:


Pain Assist Inc.

Last Modified On: May 11, 2017

This article does not provide medical advice. See disclaimer

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