MY 76 YR OLD GRANDNMOTHER FROM HER LOVING HUSBAND  OF 61 YRS AND 4 CHILDREN ON AUGUST 21ST 2010. SHE WAS A MERCY'S EMEPOLYEE FOR 25 YRS SHE WAS STILL WORKING WHEN SHE GOT SICK. SHE WAS LOVED BY EVERYONE SHE KNOW i'M MAKING THIS RARE DIEASE KNOW SO THAT MY FAMILY CAN  RASIE  AWARNESS AND LEARN MORE ABOUT IT AND RASIE MONEY FOR MORE REASAERCH. THE FOLLOWING IS WHAT IS KNOWN ABOUT THIS DIEASE

Wegener's granulomatosis

From Wikipedia, the free encyclopedia

Wegener's granulomatosis

Classification and external resources

Wegener's granulomatosis (WG), more recently granulomatosis with polyangiitis (Wegener's) (GPA), is an incurable form of vasculitis (inflammation of blood vessels) that affects the nose, lungs, kidneys and other organs. Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression.[1] Five-year survival is up to 87%, with some of the mortality due to toxicity of treatment. It is named after Dr. Friedrich Wegener, who described the disease in 1936.[2] In 2011, three professional bodies proposed a more descriptive name.[3]

Wegener's granulomatosis is part of a larger group of vasculitic syndromes, all of which feature an autoimmune attack by an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) against small and medium-size blood vessels. Apart from Wegener's, this category includes Churg-Strauss syndrome and microscopic polyangiitis.[1] Although Wegener's granulomatosis affects small and medium-sized vessels,[4] it is formally classified as one of the small vessel vasculitides in the Chapel Hill system.[5]

Signs and symptoms

Initial signs are extremely variable, and diagnosis can be severely delayed due to the nonspecific nature of the symptoms. Rhinitis is generally the first sign in most patients.[1]

 Kidney: rapidly progressive glomerulonephritis (75%), leading to chronic renal failure

 Upper airway, eye and ear disease: Nose: pain, stuffiness, nosebleeds, rhinitis, crusting, saddle-nose deformity due to a perforated septum

 Ears: conductive hearing loss due to auditory tube dysfunction, sensorineural hearing loss (unclear mechanism)

 Oral cavity: strawberry gingivitis, underlying bone destruction with loosening of teeth, non-specific ulcerations throughout oral mucosa

 Eyes: pseudotumours, scleritis, conjunctivitis, uveitis, episcleritis

Trachea: subglottal stenosis

 Lungs: pulmonary nodules (referred to as "coin lesions"), infiltrates (often interpreted as pneumonia), cavitary lesions, pulmonary hemorrhage causing hemoptysis, and rarely bronchial stenosis.

 Arthritis: Pain or swelling (60%), often initially diagnosed as rheumatoid arthritis

 Skin: nodules on the elbow, purpura, various others (see cutaneous vasculitis)

 Nervous system: occasionally sensory neuropathy (10%) and rarely mononeuritis multiplex

 Heart, gastrointestinal tract, brain, other organs: rarely affected.

Diagnosis

Immunofluorescence pattern produced by binding of ANCA to ethanol-fixed neutrophils, from a patient with Wegener's Granulomatosis. 

Wegener's granulomatosis is usually suspected only when a patient has had unexplained symptoms for a long period of time. Determination of ANCAs can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. Cytoplasmic staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with Wegener's.[1]

If the patient has renal failure or cutaneous vasculitis, these are the most logical organs to obtain a biopsy from. Rarely, thoracoscopic lung biopsy is required. On histopathological examination, a biopsy will show leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation (clumps of typically arranged white blood cells) on microscopy. These granulomas are the main reason for the appellation of "Wegener's granulomatosis", although it is not an essential feature. Unfortunately, many biopsies can be nonspecific and 50% provide too little information for the diagnosis of Wegener's.[1]

Differential diagnosis (alternative possible diagnoses) can be extensive. ANCAs can be positive after the use of certain drugs and other forms of vasculitis can present with very similar symptoms. The saddle-nose deformity may also be seen in relapsing polychondritis, cocaine abuse and in congenital syphilis.

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