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Granulomatosis with Polyangiitis (Wegener Granulomatosis)

Last updated May 4, 2018

DoveMed.com

Microscopic image of a Lung biopsy showing large area of "geographic" necrosis, and granulomatous inflammation in Wegener's granulomatosis.


What are the other Names for this Condition? (Also known as/Synonyms)

  • Granulomatosis with Polyangitis 
  • Midline Granulomatosis
  • Wegener's Granulomatosis

What is Granulomatosis with Polyangiitis? (Definition/Background Information)

  • Granulomatosis with polyangiitis is a rare autoimmune disorder affecting small blood vessels, kidneys and airways of the lungs caused by abnormal inflammation of tissues. The exact cause of such inflammation is unknown.
  • It is a syndrome (combination of signs and syndrome) caused by formation of granulomas in the lungs causing a lung mass (which can sometimes be mistaken for lung cancer on radiological studies), and inflammation in blood vessels of lungs and kidneys.
  • It cannot be transmitted from person to person. It cannot be inherited from parents or transmitted to children. Different individuals can have different set of signs and symptoms.
  • The abnormal inflammation causes lung related symptoms and kidney related symptoms
  • It can be confused with a disorder called polyarteritis nodosa with which it shares some similarities. Granulomatosis with polyangiitis disease is different from Polyarteritis Nodosa.

Who gets Granulomatosis with Polyangiitis? (Age and Sex Distribution)

  • Granulomatosis with polyangiitis is a very rare condition, affecting predominantly Caucasian individuals
  • The incidence of the disorder is 1 in 33,000 in Caucasian population
  • It occurs in both males and females usually between 40 to 65 years.
  • No specific ethnic or geographical predisposition or restriction is seen
  • All races can be affected but the incidence is higher in Caucasians compared to African Americans

What are the Risk Factors for Granulomatosis with Polyangiitis? (Predisposing Factors)

  • It occurs at all ages but is more common in middle age
  • All races can be affected but the incidence is higher in Caucasians compared to African Americans
  • Some studies have proposed a genetic predisposition in some individuals but the studies are not conclusive.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Granulomatosis with Polyangiitis? (Etiology)

  • According to current medical knowledge the exact cause of Granulomatosis with polyangiitis disorder is not known.
  • Currently active research is being performed to determine the cause. Some scientists believe that it is caused by abnormal immune system after a bacterial or a viral infection.

What are the Signs and Symptoms of Granulomatosis with Polyangiitis?

Signs and symptoms may include:

  • Symptoms due Granulomatosis with polyangiitis can greatly vary between individuals. Some may have mild symptoms and other may have severe symptoms.
  • Upper respiratory tract symptoms such as sinusitis and middle ear infections can occur. Such frequent middle ear infections can cause hearing loss
  • Chronic sinus inflammation, causing congestion and pain around the sinuses of the face
  • Constantly running nose with cold like symptoms. Drying of nasal mucous membranes can cause frequent nose bleeds
  • Shortness of breath, cough, chest congestion can occur frequently
  • Fever, fatigue, body aches, joint swelling with joint pains, muscle weakness
  • Eye symptoms including itching of eyes, conjunctivitis, eyes pain, watering of eyes, vision problems
  • Skin symptoms with formation of skin ulcers, skin rash and skin nodules which are often painful
  • Presence of blood and protein in urine on urine analysis
  • Nerve symptoms causing tingling and weakness affecting either a single nerve (mono neuropathy) or multiple nerves (poly neuropathy). Mono neuropathy means that a single nerve is damaged whereas poly neuropathy means that more than one nerve is damaged

How is Granulomatosis with Polyangiitis Diagnosed?

Granulomatosis with polyangiitis is diagnosed as follows:

  • A thorough physical examination and a complete medical history
  • Granulomatosis with polyangiitis can cause non specific symptoms and signs, which may make it difficult for your healthcare provider to arrive at a definitive diagnosis.
  • Chest x rays and CT scan of chest and sinuses, which may show a cavitation and mass formation
  • Blood tests such as complete blood counts, ESR, Kidney function tests, Urine analysis for presence of blood and proteins
  • Blood test for antibodies: 95% of cases of Granulomatosis with polyangiitis are associated with antineutrophil cytoplasmic antibodies (ANCA), which are expressed in a cytoplasmic (c-ANCA) pattern on microscopic examination. Having a positive blood test for autoantibodies called antineutrophil cytoplasmic antibodies (ANCA) that are expressed in this specific pattern helps the healthcare providers in arriving at the diagnosis. Having a positive blood test is very helpful. A negative blood test for these specific autoantibodies does not mean that individual does not have Granulomatosis with polyangiitis.   In such case a scenario the healthcare provider relies on other testing to arrive at a diagnosis. Since c-ANCA blood test found in 95% of patients, this test is considered to be a sensitive test. Also, this test is usually found in Granulomatosis with polyangiitis and no other disease hence C-ANCA is considered to be a highly specific test for Granulomatosis with polyangiitis. 
  • Biopsy of tissue: Biopsy involves removal of a sample of tissue (eg, sinuses, skin, lung and kidney) to check for signs of Granulomatosis with polyangiitis. The biopsy of the tissue (sinus, nose, skin, lung, or kidney) is sent to the laboratory where a pathologist examines the nodule under microscope to arrive at a definitive diagnosis. The pathologist examination of the tissue biopsy would show presence of characteristic findings of presence of granulomas. Kidney biopsy may show inflammation also.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Granulomatosis with Polyangiitis?

The possible complications of Granulomatosis with polyangiitis could include:

  • Kidney failure
  • Lung failure
  • Perforation of nasal septum
  • Hearing loss due to constant ear infections
  • Chronic conjunctivitis

How is Granulomatosis with Polyangiitis Treated?

Treatment of Granulomatosis with polyangiitis consists of medical and surgical care.

  • Medical care primarily involves suppressing immune system using steroids and immunosuppressive medications.
  • Bisphosphonate (Fosamax) is given to treat osteoporosis caused by chronic steroid treatment.
  • Treatment with methotrexate can cause folic acid deficiency and hence replacement with Folic acid or folinic acid is often given.
  • Antibiotics for lung infections, ear infections are given as needed.

Surgical treatment depends on the signs, symptoms and complications caused by Granulomatosis with polyangiitis.

  • Nasal septal surgery to correct Nasal deformity
  • Opening of the tear ducts caused by Obstruction of lacrimal ducts
  • Dilatation of the bronchial airways caused by Bronchial stenoses
  • Insertion of PE tubes to treat frequent middle ear infections causing Eustachian dysfunction and kidney transplant in cases where severe inflammation causes severe damage to kidney function.

How can Granulomatosis with Polyangiitis be Prevented?

Currently there are no methods to prevent Granulomatosis with polyangiitis. Early diagnosis and prompt treatment decreases the burden of the disease.

What is the Prognosis of Granulomatosis with Polyangiitis? (Outcomes/Resolutions)

  • The prognosis depends on severity of signs and symptoms. The prognosis is good in individuals who have mild symptoms and those who respond readily to immuno-suppressive treatment.
  • In individuals where the symptoms are severe causing severe damage to lungs and kidney's the prognosis can be poor especially if such individuals do not respond well to immuno-suppressive treatment
  • Constant monitoring of disease activity with frequent medical follow ups also improves prognosis
  • The prognosis is very poor in individuals with untreated disease

Additional and Relevant Useful Information for Granulomatosis with Polyangiitis:

  • Granulomatosis with polyangiitis (Wegener's) (GPA) is also referred to as Wegener's granulomatosis (WG). The disorder is named after Friedrich Wegener, who in 1936 described the disease. This scientist was associated with Nazi's and hence the world academic professional bodies have proposed replacing his name with a descriptive name, which is Granulomatosis with polyangiitis.

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Sept. 3, 2013
Last updated: May 4, 2018

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