Granulomatosis with Polyangiitis

What is Granulomatosis with Polyangiitis?

Granulomatosis withg Polyangiitis is a rare disease characterized by inflammation in a variety of tissues, including blood vessels (vasculitis). Inflammation damages vital organs of the body. Granulomatosis with Polyangiitis mainly affects the upper respiratory tract, lungs, and kidneys. Other organ systems that can be affected by the disease include the nervous system, ears, eyes, heart, and skin.

Granulomatosis with Polyangiitis can affect people of all ages from childhood to adulthood. It affects men and women equally.

Symptoms of Granulomatosis with Polyangiitis

The symptoms of Granulomatosis with Polyangiitis and their severity vary among patients. General signs of the disease may include:

• Loss of appetite
• Weight loss
• Fever
• Fatigue

Most patients first notice symptoms in the respiratory tract. Symptoms may include:

• Persistent runny nose or the formation of nasal crusts and sores
• Nasal or facial pain
• Nose bleeds or unusual nasal discharge, caused by inflammation of the nose or sinuses
• Cough which may include bloody phlegm caused by upper airway or lower airway (lung) inflammation
• Chest discomfort
• Middle ear inflammation (also called otitis media), pain, or hearing loss
• Voice change, wheezing, or shortness of breath caused by inflammation of the trachea

Causes of Granulomatosis with Polyangiitis

The cause of Granulomatosis with Polyangiitis is unknown.

Diagosing Granulomatosis with Polyangiitis

Granulomatosis with Polyangiitis has symptoms similar to a number of other disorders, which may make it difficult to diagnose. However, for the most effective and successful treatment, early diagnosis is critical.
It is the combination of symptoms, results of physical examinations, laboratory tests, x-rays and sometimes a biopsy (sample) of affected tissue (skin, nose, sinus, lung, or kidney) that together prove the diagnosis of Granulomatosis with Polyangiitis. Following treatment, these factors are also critical in judging whether the disease is active or in remission.

A positive blood test for antineutrophil cytoplasmic antibodies (ANCA) can support a suspected diagnosis of the disease. However, this blood test does not by itself prove the diagnosis of Granulomatosis with Polyangiitis or determine disease activity.

Other tests that influence a physician's judgement of disease activity include:

• Measures of anemia (red blood cell count)
• Sedimentation rate (the speed in which blood cells settle in a vertical glass tube)
• Urinalysis
• Chest or sinus x-rays

Treating Granulomatosis with Polyangiitis

Because Granulomatosis with Polyangiitis is often a life-threatening disease, it is treated with a variety of powerful medications that have been shown to be life-saving. Treatment usually includes corticosteroid medications such as Prednisone and chemotherapy drugs such as cyclophosphamide or methotrexate.
These drugs suppress the immune system and usually induce remission (the complete absence of all signs of the disease). Improvement usually occurs within days to weeks.

When the disease is in remission, patients will reduce the dosage of these medications, but will continue treatment until the disease has been in continuous remission for one year.