Granulomatosis with Polyangiitis

Also called: GPA, Wegener's Granulomatosis

Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a rare disease. It is a type of vasculitis, or inflammation of the blood vessels. The inflammation limits the flow of blood to important organs, causing damage. It can affect any organ, but it mainly affects the sinuses, nose, trachea (windpipe), lungs, and kidneys.

The cause of GPA is unknown. It can affect people at any age. Men and women are equally affected. It is more common in whites. Symptoms may include joint pain, weakness, tiredness, and cold symptoms such as a runny nose that doesn't get better. Doctors use blood tests, chest X-rays, and biopsies to diagnose GPA and rule out other causes of the symptoms.

Early treatment is important. Most people improve with medicines to slow or stop the inflammation.

NIH: National Institute of Allergy and Infectious Diseases

Symptoms of Granulomatosis with Polyangiitis

The following features are indicative of Granulomatosis with Polyangiitis:
  • runny nose
  • stuffiness
  • sinus infections
  • nosebleeds
  • coughing, sometimes with bloody phlegm
  • shortness of breath
  • fever
  • fatigue
  • general aches and pains
  • numbness in limbs, fingers or toes
  • weight loss
  • blood in urine
  • skin sores
  • eye redness, burning
  • ear infections
It is possible that Granulomatosis with Polyangiitis shows no physical symptoms and still is present in a patient.

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Common Causes of Granulomatosis with Polyangiitis

The following are the most common causes of Granulomatosis with Polyangiitis:
  • abnormal reaction in immune system
  • genetic mutation in the HLA-DPB1 gene

Risk Factors for Granulomatosis with Polyangiitis

The following factors may increase the likelihood of Granulomatosis with Polyangiitis:
  • age group between 40 to 65 years

Prevention of Granulomatosis with Polyangiitis

No, it is not possible to prevent Granulomatosis with Polyangiitis.
  • genetic mutation

Occurrence of Granulomatosis with Polyangiitis

Number of Cases

The following are the number of Granulomatosis with Polyangiitis cases seen each year worldwide:
  • Very common > 10 Million cases

Common Age Group

Granulomatosis with Polyangiitis most commonly occurs in the following age group:
  • Aged between 40-70 years

Common Gender

Granulomatosis with Polyangiitis can occur in any gender.

Lab Tests and Procedures for Diagnosis of Granulomatosis with Polyangiitis

The following lab tests and procedures are used to detect Granulomatosis with Polyangiitis:
  • Blood tests: To look for the signs of inflammation
  • Anti-neutrophil cytoplasmic antibodies test: To diagnose the condition
  • Creatinine test: To check if kidneys are properly filtering waste products from the blood
  • Urine tests: To reveal whether the urine contains red blood cells or has too much protein
  • Imaging tests: To determine what blood vessels and organs are affected
  • Biopsy: To confirm a diagnosis of granulomatosis with polyangiitis

Doctor for Diagnosis of Granulomatosis with Polyangiitis

Patients should visit the following specialists if they have symptoms of Granulomatosis with Polyangiitis:
  • Pulmonologist
  • Otolaryngologist
  • Nephrologist

Complications of Granulomatosis with Polyangiitis if untreated

Yes, Granulomatosis with Polyangiitis causes complications if it is not treated. Below is the list of complications and problems that may arise if Granulomatosis with Polyangiitis is left untreated:
  • hearing loss
  • skin scarring
  • heart disease
  • kidney damage
  • loss of height in the bridge of the nose caused by weakened cartilage
  • deep vein thrombosis
  • kidney failure
  • anemia
  • can be fatal

Procedures for Treatment of Granulomatosis with Polyangiitis

The following procedures are used to treat Granulomatosis with Polyangiitis:
  • Plasma exchange: To remove the liquid portion of the blood that contains disease-producing substance
  • Kidney transplant: To restore the normal kidney function

Patient Support for Treatment of Granulomatosis with Polyangiitis

The following actions may help Granulomatosis with Polyangiitis patients:
  • Understand your condition: Helpful in dealing with complications
  • Maintain a strong support system: Family and friends can help in coping with disease

Time for Treatment of Granulomatosis with Polyangiitis

While time-period of treatment for each patient may vary, below is the typical time-period for Granulomatosis with Polyangiitis to resolve if treated properly under an expert supervision:
  • More than 1 year

Last updated date

This page was last updated on 2/04/2019.
This page provides information for Granulomatosis with Polyangiitis.

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