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Histopathology later ruled out ECD, suggesting an unspecified granulomatous disease, with granulomatosis with polyangiitis (GPA) initially excluded due to negative antineutrophil cytoplasmic antibodies (ANCAs) and foamy histiocytes. In 2023, repeated lung biopsy and PR3\u2010ANCA positivity led to a revised diagnosis of mass\u2010forming GPA. Rituximab therapy resulted in remission. This case highlights the diagnostic complexity of GPA with atypical histopathological features.<\/jats:p>","DOI":"10.1002\/acr2.70038","type":"journal-article","created":{"date-parts":[[2025,4,4]],"date-time":"2025-04-04T12:23:01Z","timestamp":1743769381000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Mass\u2010Forming Variants in Antineutrophil Cytoplasmic Antibody\u2013Associated Vasculitis: Diagnostic Complexities in Granulomatous Disease. 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