Liječnički vjesnik, Vol. 138 No. 1-2, 2016.
Review article
GRANULOMATOSIS WITH POLYANGIITIS (GPA) LIMITED TO UPPER RESPIRATORY TRACT – A CASE REPORT
Ivan Marković
Silva Pukšić
Ana Gudelj-Gračanin
Ivan Ožegović
Melanie-Ivana Čulo
Joško Mitrović
Jadranka Morović-Vergles
Abstract
Granulomatosis with polyangiitis (Wegener’s granulomatosis) is one of the anti-neutrophil cytoplasmic antibody-associated small vessel vasculitides. Upper and lower respiratory system and kidneys are most commonly affected. The disease is characterized by granulomatous inflammation of the respiratory tract and necrotizing vascu-litis of small to medium-sized blood vessels. Most patients show involvement of more than one organ systems at the time of diagnosis, and constitutional symptoms may be present. In around a quarter of patients the disease is initially localised, with involvement of upper respiratory tract or lungs. We report a 21-year-old female patient with chronic rhinitis, saddle nose deformity and subglottic stenosis who presented with inspiratory stridor and impending respirato-ry failure. Initially, urgent tracheotomy was performed. The patient was diagnosed with granulomatosis with polyan-giitis limited to upper respiratory tract. Treatment with glucocorticoids and methotrexate was followed by clinical improvement.
Keywords
Granulomatosis with polyangiitis – diagnosis, complications, immunology, drug therapy; Laryngostenosis – etiology, complications; Respiratory sounds – etiology; Tracheotomy; Methotrexate – therapeutic use; Immunosuppressive agents – therapeutic use; Glucocorticoids – therapeutic use; Nose deformities, acquired – etiology; Nasal mucosa – pathology
Hrčak ID:
172830
URI
Publication date:
29.2.2016.
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