615 © Springer Nature Switzerland AG 2021 A. Al-Qahtani et al. (eds.), Textbook of Clinical Otolaryngology, https://doi.org/10.1007/978-3-030-54088-3_54 ENT Manifestations in Systemic and Infammatory Diseases Aya Elderee, Ali Al Ansari, Hassan Haidar, and Mazin Al Khabouri 54.1 Introduction Systemic and Infammatory Diseases can affect the head and neck regions in a variety of man- ners. These manifestations may be the initial indications of the systemic disease, or sequela of the established diagnosis. The ENT symptoms and fndings may mimic a variety of local ill- nesses, thus making this distinction an important part of the evaluative process. An awareness of the possible head and neck manifestations of underlying systemic disease is important in a proper diagnosis of the illness with consequent proper management. 54.2 Hereditary Hemorrhagic Telangiectasia HHT (Formerly Osler Weber Rendu) • It’s an autosomal dominant disease. • Present with multiple telangiectasias of the skin and mucosa. • Diagnosis: Using the Curacao criteria: The HHT diagnosis is Defnite if 3 criteria are present, Possible or suspected if 2 criteria are present, and Unlikely if fewer than 2 criteria are present. – Epistaxis which is spontaneous and recurrent. – Mucosal telangiectasia, multiple, at char- acteristic sites including lips, oral cavity, fngers, and nose. – Visceral lesions such as gastrointestinal tel- angiectasia (with or w/o bleeding), pulmo- nary AVM, hepatic AVM, cerebral AVMs, spinal AVM. – Family history with a frst degree relative with HHT [1]. • Treatment options showing in Table 54.1: 54.3 Kawasaki Disease • Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome. It is an acute febrile disease of childhood and is A. Elderee (*) · A. Al Ansari ENT Department, Hamad Medical Corporation, Doha, Qatar e-mail: AElderee@hamad.qa; aalansari@hamad.qa H. Haidar Hamad Medical Corporation, Doha, Qatar M. Al Khabouri ENT Department, Al Nahdha Hospital, Muscat, Sultanate of Oman 54