What is Wegener's granulomatosis?

What is Wegener's granulomatosis?

• Wegener's granulomatosis: A rare disease involving blood vessel inflammation which can affect the blood flow to various tissues and organs and hence cause damage. The respiratory system and the kidneys are the main systems affected.
• Wegener's granulomatosis: multisystem disease chiefly affecting males, characterized by necrotizing granulomatous vasculitis involving the upper and lower respiratory tracts, glomerulonephritis, and variable degrees of systemic small vessel vasculitis; considered an aberrant hypersensitivity reaction to an unknown antigen.
  Source - Diseases Database

Wegener's granulomatosis is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Wegener's granulomatosis, or a subtype of Wegener's granulomatosis, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Name and Aliases of Wegener's granulomatosis

Main name of condition: Wegener's granulomatosis

WG, Wegener granulomatosis, Midline granulomatosis, Wegener syndrome, arteritis-pulmonary-nephropathy syndrome, granulomas-arteritis-glomerulonephritis, granulomatosis pathergic, necrotizing respiratory granulomatosis

Klinger disease Source - Diseases Database

Necrotizing Respiratory Granulomatosis, Pathergic Granulomatosis
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Types of Wegener's granulomatosis:

Parent types of Wegener's granulomatosis: Possibly autoimmune diseases, Vasculitis, Kidney conditions, Blood vessel conditions, Nose conditions

Organs Affected by Wegener's granulomatosis:

blood vessels, respiratory tract, sinuses, nose, trachea, lungs, kidneys

How many people get Wegener's granulomatosis?

Prevalance of Wegener's granulomatosis: uncommon

Who gets Wegener's granulomatosis?

Patient Profile for Wegener's granulomatosis: Any age.
Profile for Wegener's granulomatosis: This disorder can occur at any age and strikes men and women equally. (Source: excerpt from Wegener's Granulomatosis, NIAID Fact Sheet: NIAID)
Gender Profile for Wegener's granulomatosis: Men and women equally.
Race Profile for Wegener's granulomatosis: More common in Caucasians than African Americans.
Race Profile for Wegener's granulomatosis: It is rare in African Americans compared to whites. (Source: excerpt from Wegener's Granulomatosis, NIAID Fact Sheet: NIAID)

How serious is Wegener's granulomatosis?

Prognosis of Wegener's granulomatosis: progressive fatal condition, mortality 28% at 5 years
Prognosis of Wegener's granulomatosis: With the appropriate treatment, the outlook is good for patients with Wegener’s granulomatosis. In a study of 158 patients who were treated at the National Institutes of Health (NIH), 91 percent of them markedly improved. After 6 months to 24 years of follow-up, 80 percent of the patients survived. (Source: excerpt from Wegener's Granulomatosis, NIAID Fact Sheet: NIAID)
Complications of Wegener's granulomatosis: see complications of Wegener's granulomatosis

What causes Wegener's granulomatosis?

Cause of Wegener's granulomatosis: Unknown. Possible autoimmune.
Class of Condition for Wegener's granulomatosis: autoimmune possibly
Causes of Wegener's granulomatosis: see causes of Wegener's granulomatosis

How is it treated?

Treatments for Wegener's granulomatosis: see treatments for Wegener's granulomatosis
Research for Wegener's granulomatosis: see research for Wegener's granulomatosis