National Research Institute of Tuberculosis and Lung Disease (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran TANAFFOS (Respiration) 1735-0344 12 1 2013 01 01 Upper Respiratory System Involvement as the Only Manifestation of Granulomatosis with Polyangiitis in a Child with Marfan Phenotype 74 77 241045 EN Reza Amin Department of Allergy and Clinical Immunology, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. Soheila Alyasin Department of Allergy and Clinical Immunology, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. Tooba Momen Department of Allergy and Clinical Immunology, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. Maryam Khoshkhouy Department of Allergy and Clinical Immunology, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. Sepideh Darougar Department of Allergy and Clinical Immunology, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. Journal Article 2020 12 29 We report a 9-year old girl with an unusual presentation of granulomatosis with polyangiitis in association with Marfan phenotype. The patient presented with recurrent sinusitis, epistaxis, hearing loss and hyperplastic gingivitis, without any signs or symptoms of major organ involvement. https://www.tanaffosjournal.ir/article_241045_267a5b2ed1f08ea6c9de13635ae6ce6c.pdf