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Symptoms and Signs of Granulomatosis with Polyangiitis

Doctor's Notes on Granulomatosis with Polyangiitis Symptoms, Treatment, and Life Expectancy

Granulomatosis with polyangiitis (also termed Wegener’s) is a rare disease that results in inflamed blood vessels (vasculitis or polyangiitis) and nodules of inflammatory cells (granulomas) in affected tissues. The most common signs and symptoms are repeated sinusitis with pain, runny and/or bloody nose with sores and associated holes in nasal cartilage, ear infections and hearing loss. Often, people can develop shortness of breath, chest pain and coughs with some bloody sputum. Less common signs and symptoms include muscle and joint pain, joint swelling, red or purple small sores on the skin, hoarseness, fever and night sweats, weight loss. Urine may be reddish or bloody. Eye involvement can show redness, burning and eye swelling; if double vision or decreased vision occurs, it is a medical emergency.

Granulomatosis with polyangiitis is rare; its cause is unknown but thought to be an autoimmune disorder. Most signs and symptoms are due to organ damage that happens when its blood supply is compromised by the disease.

Medical Author:
Medically Reviewed on 3/11/2019

Granulomatosis with Polyangiitis Symptoms, Treatment, and Life Expectancy Symptoms

Upper respiratory tract (most common):

  • Sinusitis, sinus pain, bacterial infection in sinuses
  • Constant runny nose (worse than that caused by a common cold)
  • Bloody nose
  • Sores around the opening of the nose
  • Hole develops in cartilage in nose
  • Ear infections, ear canals blocked, hearing loss

Lungs (usually present):

Other symptoms (less common):

  • Muscle and joint pain, joint swelling
  • Redness and burning or pain in the eyes (conjunctivitis) and swelling in the eye
  • Double vision or decrease in vision (requires immediate medical care)
  • Sores on the skin that look like small red or purple raised areas (they may or may not cause pain)
  • Hoarseness of the voice
  • Fever, night sweats
  • Loss of appetite, weight loss

The kidneys are affected in three-fourths of people with granulomatosis with polyangiitis, but the person may have no obvious symptoms related to kidney damage. Urine, however, may be bloody or red in color.

Granulomatosis with Polyangiitis Symptoms, Treatment, and Life Expectancy Causes

No one knows the cause of granulomatosis with polyangiitis is thought to be an autoimmune disorder in which the body's defense system attacks itself and destroys normal body tissue. Normally, the body's immune system acts as a defense against outside invaders. With an autoimmune disorder, something triggers an immune response causing the body to react against its own tissue. The result is that tissue can be damaged. With granulomatosis with polyangiitis, the organ systems that are attacked include the respiratory tract and kidneys.

Top 15 Immune System Boosters Slideshow

Top 15 Immune System Boosters Slideshow

Chronic stress depresses the immune system and increases the risk of several types of illnesses. It raises the level of hormones called catecholamines. Being stressed out leads to increased levels of suppressor T cells, which suppress the immune system. When this branch of the immune system is impaired, you are more susceptible to viral illnesses including respiratory conditions like colds and flu. Stress leads to the release of histamine, a molecule involved in allergies. Combat stress with strategies like deep breathing, meditation, exercise, and relaxation.

REFERENCE:

Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.

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