Condition 101 About Granulomatosis with Polyangiitis

What is the definition of Granulomatosis with Polyangiitis?

Granulomatosis with polyangiitis (GPA) is a rare disorder in which blood vessels become inflamed. This leads to damage in major organs of the body. It was formerly known as Wegener's granulomatosis.

What are the alternative names for Granulomatosis with Polyangiitis?

Formerly: Wegener's granulomatosis

What are the causes for Granulomatosis with Polyangiitis?

GPA mainly causes inflammation of blood vessels in the lungs, kidneys, nose, sinuses, and ears. This is called vasculitis or angiitis. Other areas may also be affected in some cases. The disease can be fatal and prompt treatment is important.

In most cases, the exact cause is not known, but it is an autoimmune disorder. Rarely, vasculitis with positive antineutrophil cytoplasmic antibodies (ANCA) has been caused by several drugs including cocaine cut with levamisole, hydralazine, propylthiouracil, and minocycline.

GPA is most common in middle-aged adults of northern European descent. It is rare in children.

What are the symptoms for Granulomatosis with Polyangiitis?

Frequent sinusitis and bloody nose are the most common symptoms. Other early symptoms include a fever that has no clear cause, night sweats, fatigue, and a general ill feeling (malaise).

Other common symptoms may include:

  • Chronic ear infections
  • Pain, and sores around the opening of the nose
  • Cough with or without blood in the sputum
  • Chest pain and shortness of breath as the disease progresses
  • Loss of appetite and weight loss
  • Skin changes such as bruises and ulcers of the skin
  • Kidney problems
  • Bloody urine
  • Eye problems ranging from mild conjunctivitis to severe swelling of the eye.

Less common symptoms include:

  • Joint pain
  • Weakness
  • Abdominal pain

What are the current treatments for Granulomatosis with Polyangiitis?

Because of the potentially serious nature of GPA, you may be hospitalized. Once the diagnosis is made, you will probably be treated with high doses of glucocorticoids (such as prednisone). These are given through the vein for 3 to 5 days at the beginning of treatment. Prednisone is given along with other medicines that slow down the immune response.

For milder disease other medicines that slow down the immune response such as methotrexate or azathioprine may be used.

  • Rituximab (Rituxan)
  • Cyclophosphamide (Cytoxan)
  • Methotrexate
  • Azathioprine (Imuran)
  • Mycophenolate (Cellcept or Myfortic)

These medicines are effective in severe disease, but they may cause serious side effects. Most people with GPA are treated with ongoing medicines to prevent relapse for at least 12 to 24 months. Talk to your health care provider about your treatment plan.

Other medicines used for GPA include:

  • Medicines to prevent bone loss caused by prednisone
  • Folic acid or folinic acid, if you are taking methotrexate
  • Antibiotics to prevent lung infections

What are the support groups for Granulomatosis with Polyangiitis?

Support groups with others who suffer from similar diseases may help people with the condition and their families learn about the diseases and adjust to the changes associated with the treatment.

What is the outlook (prognosis) for Granulomatosis with Polyangiitis?

Without treatment, people with severe forms of this disease can die within a few months.

With treatment, the outlook for most patients is good. Most people who receive corticosteroids and other medicines that slow the immune response get much better. Most people with GPA are treated with ongoing medicines to prevent relapse for at least 12 to 24 months.

What are the possible complications for Granulomatosis with Polyangiitis?

Complications most often occur when the disease is not treated. People with GPA develop tissue damage in the lungs, airways, and the kidneys. Kidney involvement may result in blood in the urine and kidney failure. Kidney disease can quickly get worse. Kidney function may not improve even when the condition is controlled by medicines.

If untreated, kidney failure and possibly death occur in most cases.

Other complications may include:

  • Eye swelling
  • Lung failure
  • Coughing up blood
  • Nasal septum perforation (hole inside the nose)
  • Side effects from medicines used to treat the disease

When should I contact a medical professional for Granulomatosis with Polyangiitis?

Call your provider if:

  • You develop chest pain and shortness of breath.
  • You cough up blood.
  • You have blood in your urine.
  • You have other symptoms of this disorder.

How do I prevent Granulomatosis with Polyangiitis?

There is no known prevention.

Granulomatosis
Respiratory

REFERENCES

Grau RG. Drug-induced vasculitis: New insights and a changing lineup of suspect. Curr Rheumatol Rep. 2015;17(12):71. PMID: 26503355 pubmed.ncbi.nlm.nih.gov/26503355/.

Pagnoux C, Guillevin L; French Vasculitis Study Group; MAINRITSAN investigators. Rituximab or azathioprine maintenance in ANCA-associated vasculitis. N Engl J Med. 2015;372(4):386-387. PMID: 25607433 pubmed.ncbi.nlm.nih.gov/25607433/.

Stone JH. The systemic vasculitides. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 254.

Yang NB, Reginato AM. Granulomatosis with polyangiitis. In: Ferri FF, ed. Ferri's Clinical Advisor 2020. Philadelphia, PA: Elsevier; 2020:601.e4-601.e7.

Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. [published correction appears in Ann Rheum Dis. 2017;76(8):1480]. Ann Rheum Dis. 2016;75(9):1583-1594. PMID: 27338776 pubmed.ncbi.nlm.nih.gov/27338776/.

Latest Advances On Granulomatosis with Polyangiitis

  • Condition: Granulomatosis with Polyangiitis (GPA) in Eyelid
  • Journal: Medicine
  • Treatment Used: Radiotherapy
  • Number of Patients: 0
  • Published —
This case report describes a 29-year-old man diagnosed with granulomatosis with polyangiitis (chronic blood vessel inflammation) occurring in the eyelid treated with radiotherapy.

Clinical Trials For Granulomatosis with Polyangiitis

Clinical Trial
  • Status: Not yet recruiting
  • Intervention Type: Diagnostic Test
  • Participants: 60
  • Start Date: February 2021
Evaluation of ANCA Testing by Antigen Specific Assays in Small Vessel Vasculitis
Clinical Trial
  • Status: Recruiting
  • Phase: Phase 4
  • Intervention Type: Drug
  • Participants: 76
  • Start Date: December 17, 2020
Hydroxychloroquine in ANCA Vasculitis Evaluation - A Multicentre, Randomised, Double-blind, Placebo-controlled Trial