Granulomatous polyangiitis

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Granulomatous polyangiitis, formerly called Wegener's granulomatosis, is a vasculitis of medium and small arteries, including venules and arterioles. It produces granulomatous inflammation of the respiratory tracts and necrotizing, pauci-immune glomerulonephritis.

While it is considered a rare disease (3 cases per 100,000 people in the U.S.),[1] Wegener's is the most common cause of saddle nose deformity in USA (nose flattened due to destruction of nasal septum by granulomatous inflammation). It also has a high mortality rate if untreated.

Diagnosis

Almost all patients with Wegener's have the c-ANCA form of antineutrophil cytoplasmic antibodies, but not vice versa.

References

  1. Patricia J Papadopoulos and Robert John O'Brian (4 August 2009), "eMedicine Specialties > Rheumatology > Vasculitis >Wegener Granulomatosis: Overview", eMedicine