{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,14]],"date-time":"2026-07-14T07:07:01Z","timestamp":1784012821682,"version":"3.55.0"},"reference-count":31,"publisher":"Termedia Sp. z.o.o.","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Reumatologia"],"abstract":"<jats:p>Granulomatosis with polyangiitis (GPA) is a\u00a0vasculitis affecting small and medium-sized blood vessels. It typically presents with upper respiratory tract, lung, and kidney involvement. However, the\u00a0disease may involve multiple other organs, and not all cases present with these classic mani\u00adfestations. Periocular lesions are described in the GPA and, especially in the case of isolated, single-site disease, require extensive differentiation from other diseases, such as IgG4-related disease or myofibroblastic tumor. However, periocular lesions and coexisting symptoms suggesting changes in the central nervous system without other organ changes are undoubtedly a diagnostic challenge. Such situations are described in the literature mainly as case reports, not as a rule in connection with GPA. The impetus for writing this article and analyzing the literature was a case of GPA manifesting as infiltration of the skull base presenting as multiple cranial nerve palsies. A\u00a0review of\u00a0atypical cases of\u00a0GPA in the\u00a0literature suggests that skull-based involvement is an important yet easily overlooked presentation. Through this case-based review, we aim to raise awareness that GPA may present with isolated cranial nerve palsies.<\/jats:p>","DOI":"10.5114\/reum\/219888","type":"journal-article","created":{"date-parts":[[2026,7,14]],"date-time":"2026-07-14T06:39:22Z","timestamp":1784011162000},"page":"242-251","source":"Crossref","is-referenced-by-count":0,"title":["Orbital pseudotumor and skull base infiltration as the\u00a0sole manifestation of\u00a0granulomatosis with polyangiitis"],"prefix":"10.5114","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-6239-3041","authenticated-orcid":true,"given":"Nicole","family":"Kivel\u00e4","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-3325-6333","authenticated-orcid":true,"given":"William","family":"Karlsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1984-2959","authenticated-orcid":false,"given":"Karolina","family":"Markiet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6687-2408","authenticated-orcid":false,"given":"Anna","family":"Masiak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"3061","published-online":{"date-parts":[[2026,7,14]]},"reference":[{"key":"ref-1930515","doi-asserted-by":"publisher","DOI":"10.1002\/art.37715"},{"key":"ref-1930516","doi-asserted-by":"publisher","DOI":"10.1177\/0194599820912025"},{"key":"ref-1930517","doi-asserted-by":"publisher","DOI":"10.1016\/j.survophthal.2009.12.003"},{"key":"ref-1930518","doi-asserted-by":"publisher","DOI":"10.5114\/reum.2017.71638"},{"key":"ref-1930519","doi-asserted-by":"publisher","DOI":"10.3390\/ijms21197319"},{"key":"ref-1930520","doi-asserted-by":"publisher","DOI":"10.1016\/S0168-8278(00)00060-5"},{"key":"ref-1930521","doi-asserted-by":"publisher","DOI":"10.1053\/gast"},{"key":"ref-1930522","doi-asserted-by":"crossref","unstructured":"Muller K, Lin JH. 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Central nervous system involvement of\u00a0granulomatosis with polyangiitis: clinical\u2011 radiological presentation distinguishes different outcomes. Rheumatology (Oxford) 2015; 54: 424\u2013432. DOI: 10.1093\/ rheumatology\/keu336.","DOI":"10.1093\/rheumatology\/keu336"},{"key":"ref-1930536","doi-asserted-by":"publisher","DOI":"10.1001\/archneur"},{"key":"ref-1930537","doi-asserted-by":"publisher","DOI":"10.14639\/0392-100X-1776"},{"key":"ref-1930538","doi-asserted-by":"crossref","unstructured":"Tsuzuki K, Fukazawa K, Takebayashi H, et al. Difficulty of\u00a0dia\u00adgnosing Wegener\u2019s granulomatosis in the\u00a0head and neck region. Auris Nasus Larynx 2009; 36: 64\u201370. 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