Granulomatoza s poliangiitisom (GPA) i lokaliziranim zahvaćanjem gornjega dišnog sustava – prikaz bolesnice [Granulomatosis with polyangiitis (GPA) limited to upper respiratory tract - a case report]

Marković, Ivan and Pukšić, Silva and Gudelj-Gračanin, Ana and Ožegović, Ivan and Čulo, Melanie-Ivana and Mitrović, Joško and Morović-Vergles, Jadranka (2016) Granulomatoza s poliangiitisom (GPA) i lokaliziranim zahvaćanjem gornjega dišnog sustava – prikaz bolesnice [Granulomatosis with polyangiitis (GPA) limited to upper respiratory tract - a case report]. Liječnički vjesnik, 138 (1-2). pp. 54-56. ISSN 0024-3477

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Abstract

Granulomatosis with polyangiitis (Wegener's granulomatosis) is one of the anti-neutrophil cytoplasmic anti-body-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 vasculitis 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 respiratory failure. Initially, urgent tracheotomy was performed. The patient was diagnosed with granulomatosis with polyangiitis limited to upper respiratory tract. Treatment with glucocorticoids and methotrexate was followed by clinical improvement.

Abstract in Croatian

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.

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