{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T07:21:31Z","timestamp":1780471291232,"version":"3.54.1"},"reference-count":42,"publisher":"Wiley","issue":"s127","license":[{"start":{"date-parts":[[2009,6,1]],"date-time":"2009-06-01T00:00:00Z","timestamp":1243814400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["APMIS"],"published-print":{"date-parts":[[2009,6]]},"abstract":"<jats:p>Granuloma formation is a key pathologic finding in two of the anti\u2010neutrophil cytoplasmic autoantibody (ANCA)\u2010associated vasculitides: Wegener's granulomatosis (WG) and Churg\u2013Strauss syndrome (CSS). So far, no animal models have been established convincingly reproducing both vasculitic and granulomatous features typical of WG and CSS. In biopsies, granulomatous lesions are found both at distant extravascular sites and in the vicinity of inflamed vessels, e.g. in the lung. Intriguingly, WG\u2010granulomata appear to display features of tertiary lymphoid tissue. Cartilaginous and osseous destruction is caused by granulomatous inflammation invading adjacent tissues. Rhinosinusitis is regularly encountered in WG and CSS. Septal perforation, saddle nose deformity, middle and inner ear symptoms, and granulomatous invasion of the palate, orbita, meninges, or the pituitary gland may complicate WG. Both common (e.g. <jats:italic>FCGR3B<\/jats:italic> copy number) and distinct (e.g. <jats:italic>HLA\u2010DP, IL\u201010.2<\/jats:italic>) genetic factors have been identified in AAV potentially favouring inflammation and autoimmunity. The <jats:italic>HLA\u2010DPB1<\/jats:italic>\/<jats:italic>RING1<\/jats:italic>\/<jats:italic>RXRB<\/jats:italic> region constitutes a quantitative trait locus for ANCA\u2010positive WG with the strongest association to be reported up to now. A profound alteration of the T\u2010cell response including Th1 and Th17 responses, anomalously NK\u2010receptor\u2010expressing \u2018NK\u2010like\u2019 T cells, and dysfunctional regulatory T cells could facilitate and sustain granuloma formation and autoimmunity.<\/jats:p>","DOI":"10.1111\/j.1600-0463.2009.02474.x","type":"journal-article","created":{"date-parts":[[2009,6,1]],"date-time":"2009-06-01T07:22:14Z","timestamp":1243840934000},"page":"32-36","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":32,"title":["Granuloma formation in ANCA\u2010associated vasculitides"],"prefix":"10.1111","volume":"117","author":[{"given":"PETER","family":"LAMPRECHT","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"STEFAN","family":"WIECZOREK","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00d6RG T.","family":"EPPLEN","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"PETRA","family":"AMBROSCH","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"CEES G. 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