GRANULOMATOZA S POLIANGIITISOM (GPA) I LOKALIZIRANIM ZAHVAĆANJEM GORNJEGA DIŠNOG SUSTAVA – PRIKAZ BOLESNICE

Ivan Marković, Silva Pukšić, Ana Gudelj-Gračanin, Ivan Ožegović, Melanie-Ivana Čulo, Joško Mitrović, Jadranka Morović-Vergles

Granulomatoza s poliangiitisom (Wegenerova granulomatoza) ubraja se u vaskulitise malih krvnih žila povezane s antineutrofilnim citoplazmatskim protutijelima. Najčešće su zahvaćeni gornji i donji dišni sustav te bubrezi. Obilježja bolesti uključuju granulomatoznu upalu dišnog sustava i nekrotizirajući vaskulitis malih do srednje velikih krvnih žila. U većine je bolesnika pri postavljanju dijagnoze zahvaćeno više organskih sustava te mogu biti prisutni i opći simptomi, dok je u oko četvrtine bolesnika inicijalno prisutan lokalizirani oblik bolesti sa zahvaćanjem najčešće gornjega dišnog sustava ili pluća. U radu smo prikazali dvadesetjednogodišnju bolesnicu s kroničnim rinitisom, razvojem sedlaste deformacije nosa i subglotičnom stenozom traheje koja je hospitalizirana zbog inspiratornog stridora. Zbog respiratorne insuficijencije učinjena je hitna traheotomija. U bolesnice je dijagnosticirana granulomatoza s poliangiitisom ograničena na gornji dišni sustav. Liječenje glukokortikoidima i metotreksatom dovelo je do kliničkog poboljšanja.

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.