Nosní a krční manifestaceWegenerovy granulomatózypři multiorgánovém postižení

Nasal and CervicalManifestations of Wegener’s Granulomatosis in an Multiorgan Affection

S u m m a r y:
The authors present the case-history of a 34-year-old female patient with Wegener’sgranulomatosis with an unusually high number of extrarenal complications. The disease wasmanifested in addition to affections of the upper and lower airways by arthralgias, a febrilecondition and perforation of the cornea. Subsequently the patient suffered an acute myocardialinfarction due to coronary arteritis and valvulitis of the aortal valve. Affection of the skin withmaculopapulous efflorescences. Another unusual complication was massive enterorrhagia. Theclinical picture of the disease comprised also renal affection, i.e. necrotizing glomerulonephritiswith crescents and evidence of cANCA antibodies. When clinical and laboratory remission of thedisease was achieved the course of the disease was complicated by the development of subglotticstenosis of the trachea.

Key words:
Wegener’s granulomatosis, perforation of the cornea, myocardial infarction, aortalvasculitis, subglottic stenosis of the trachea, cANCA antibodies.