Gourt > Health > Conditions and Diseases > Rare Disorders > Wegener's Granulomatosis
In medicine (rheumatology), Wegener's granulomatosis is a form of vasculitis that affects the lungs, kidneys and other organs. Due to its end-organ damage, it can be a serious disease that requires long-term immune suppressionSeo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med 2004;117:39-50. PMID 15210387..
It is part of a larger group of vasculitic syndromes that all feature the presence for an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) and affect small and medium-sized blood vessels. Apart from Wegener's, it includes Churg-Strauss syndrome and microscopic polyangiitis.
Signs and symptoms
Initial signs are protean, and diagnosis can be severely delayed due to the non-specific nature of the symptoms. The rhinitis is generally the first sign in most patients.
- Upper airway, eye and ear disease:
- Nose: pain, stuffiness, nosebleeds, rhinitis, crusting, saddle-nose deformity
- Ears: conductive hearing loss due to Eustachian tube dysfunction, sensorineural hearing loss (unclear mechanism)
- Eyes: pseudotumours, scleritis, conjunctivitis, uveitis, episcleritis
- Airways:
- Trachea: subglottal stenosis
- Lungs: pulmonary nodules, infiltrates (often interpreted as pneumonia), cavitary lesions, pulmonary hemorrhage causing hemoptysis), and rarely bronchial stenosis.
- Kidney: rapidly progressive segmental necrotising glomerulonephritis (75%), leading to chronic renal failure
- 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.
Vascular Disorders
Genitourinary Disorders
Respiratory Disorders
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Parents of child lost to Wegener's Granulomatosis raise money for research http://t.co/ZIW59RNb
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