Wegener granulomatosis is an autoimmune disease that is believed to be caused by antineutrophil cytoplasmic antibodies (ANCA) and T-cell-mediated hypersensitivity response against an otherwise relatively harmless germ. It primarily affects (with the percentage of cases in which they are affected in brackets) the lungs (causing pneumonitis) and their associated lymph nodes (95%) and kidneys (~80%), although hearing loss, blindness and permanent nasal deformities are also common complications. It leads to the formation of small benign tumours called granulomas that are created by an aberrant immune response. Normally the purpose of a granuloma is to seal off a small area of the body (usually only a centimetre or so in diametre) so that any germs or other disease-causing particles found therein cannot spread to other parts of the body. Without treatment it is almost universally fatal, with 80% of patients dying within one year of their diagnosis if untreated; but with treatment with TNF inhibitors, rituximab, cyclophosphamide and corticosteroids the disease most frequently takes a relapsing-remitting course, similarly to other autoimmune diseases like systemic lupus erythematosus.
A 2005 cohort study (involving 158 patients) sponsored by the U.S. National Institute of Health (NIH) found that the disorder caused permanent damage (including heart disease, lung disease, kidney failure, hearing loss, blindness, etc.) in many patients (86%), as did its treatment (42%; e.g., bladder cancer due to cyclophosphamide treatments).
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- ↑ 1.0 1.1 1.2 Kumar, V; Abbas, AK; Aster, AC (July 2014). "Chapter 11. Blood Vessels". Robbins & Cotran Pathologic Basis of Disease 9e. Philadelphia, USA: Saunders. pp. 511–512. ISBN 978-0-8089-2450-0.
- ↑ Tracy, CL; Papadopoulos, PJ; Bye, MR; Connolly, H; Goldberg, E; O'Brian, RJ; Sharma, GD; Talavera, F; Toder, DS; Valentini, RP; Windle, ML; Wolf, RE (25 September 2014). Diamond, HS, ed. "Granulomatosis with Polyangiitis (Wegener Granulomatosis)". Medscape Reference. New York, USA: WebMD. Retrieved 14 November 2014.
- ↑ Seo, P; Min, YI; Holbrook, JT; Hoffman, GS; Merkel, PA; Spiera, R; Davis, JC; Ytterberg, SR; St Clair, EW; McCune, WJ; Specks, U; Allen, NB; Luqmani, RA; Stone, JH; WGET Research Group (July 2005). "Damage caused by Wegener's granulomatosis and its treatment: prospective data from the Wegener's Granulomatosis Etanercept Trial (WGET).". Arthritis and Rheumatism 52 (7): 2168–78. PMID 15986348. doi:10.1002/art.21117.