TAKAYASUOV ARTERITIS I MOGUĆE KARDIOLOŠKE REPERKUSIJE U DJEČJOJ DOBI

Ivan Malčić, Petra Detoni, Hrvoje Kniewald, Dorotea Bartoniček, Daniel Dilber, Dubravka Dietrich, Marija Jelušić

Vasculitides are rare rheumatic diseases of unknown etiology whose main characteristic is a necrotizing inflammation of blood vessels. We are presenting two patients with Takayasu arteritis (TA) as entity forms of rare rheumatic diseases. One patient had TA type IIa and the other type IV. In the first patient we found severe symptoms of obstructive lesions of aortic branches, particularly severe coronary artery stenosis and complete occlusion of the left subclavian artery, and thoracic artery stenosis below the isthmus. Th e disease was diagnosed in the acute phase, treated extensively with medicaments (glucocorticoids, cytostatics, methotrexate) and a complex cardiac surgical procedure, and due to relapse the biological (Rituximab) therapy was used. Th e second patient was detected following symptomatic arterial hypertension, with absent pulses of lower limbs, whose cause was found in severe narrowing of the aorta from diaphragm to femoral arteries bifurcation (mid-aortic syndrome). Th e disease was
not active when diagnosis was made. The patient was treated with a particular cardiac surgical procedure and with multiple medicaments due to a relapse. Both patients have reached adolescent age and are successfully treated with a satisfying quality of life. Type IIa with an additional occlusion
of coronary arteries is not described in the available literature. Forementioned vasculitides emphasize the importance of pediatric cardiologists and rheumatologists teamwork.

Vaskulitisi su rijetke reumatske bolesti nepoznate etiologije kojima je osnovno obilježje nekrotizirajuća upala krvnih žila. Posebnu skupinu čine granulomatozni vaskulitisi, izrazito rijetko opisivani u dječjoj dobi. Prikazujemo dvije bolesnice s Takayasuovim arteritisom (TA) kao entitetske oblike rijetkih reumatskih bolesti. Jedna je bolesnica imala TA tip II. a, a druga tip IV. U prve bolesnice nalazimo teške simptome opstrukcijskih lezija aortnih ogranaka, osobito tešku stenozu koronarnih arterija i okluziju lijeve potključne arterije te stenozu torakalne aorte ispod istmusa. Bolest je dijagnosticirana u akutnoj fazi, liječena je opsežno medikamentozno (glukokortikoidi, citostatici, metotreksat) i složenim kardiokirurškim pristupom, a zbog recidiva korištena je i biološka (rituksimab) terapija. Druga je bolesnica otkrivena zbog simptomatske arterijske hipertenzije, s izostankom pulseva na donjim udovima, a razlog tome nađen je u teškom suženju aorte od dijafragme do bifurkacije femoralnih arterija (mid aortic sindrom). U trenutku dijagnoze sama bolest nije bila u aktivnoj fazi. Liječena je osobitim kardiokirurškim pristupom i polimedikamentno zbog recidiva. Obje su bolesnice u adolescentnoj dobi i uspješno se liječe uz zadovoljavajuću kvalitetu života. Tip II. a s dodatnom okluzijom koronarnih krvnih žila nije prikazan u dostupnoj literaturi. Opisani vaskulitisi još uvijek snažno povezuju pedijatrijsku kardiologiju i reumatologiju, a prikaz svjedoči o važnosti timskog rada pedijatrijskih kardiologa i reumatologa.