Rheumatological Diseases of the Nose and Paranasal Sinuses

Abstract

Rheumatological problems occurring in the ear, nose and throat can be difficult both for specialists in rheumatology and in ENT to diagnose. In the main, Churg-Strauss syndrome, polychondropathia, Wegener’s syndrome, aspirin-exacerbated respiratory disease (Samter’s syndrome) and midline granulomata will be mentioned, but reference will also be made to some other conditions. Churg-Strauss syndrome (CSS) is a type of vasculitis of unknown cause leading to necrosis and affecting the smallest diameter vasculature and one which usually presents with bronchioasthma, hypereosinophilia and granulomata located extravascularly. Wegener’s syndrome (WS), which is now known as granulomatosis with polyangiitis (GPA), often presents through ENT symptoms, in fact, in greater than 70% of cases, the nose, sinuses, ear, or trachea are involved. WS classically exhibits a trio of findings: (1) both upper and lower respiratory systems show granulomata with associated necrosis; (2) systemically, lower calibre vessels both arterial and venous are vasculitic; and (3) inflamed renal glomeruli in focal areas. Relapsing polychondritis (RP) affects cartilage, particularly the otic, nasal and laryngo tracheobronchial cartilage, but occasionally can also affect ocular, cardiovascular, dermal and central nervous systems as well as peripheral joints and middle or inner ear. Its course is intermittent, but progressive and is severe in intensity. Aspirin-exacerbated respiratory disease (AERD) (Samter’s triad) involves patients who have asthma, respiratory problems exacerbated by aspirin and nasal polyp formation. It is also called the aspirin triad, AERD or Widal triad. This chapter considers rheumatological disorders affecting the nose and paranasal sinuses.