Introdução: Os critérios diagnósticos da arterite de Takayasu (AT) se baseiam em alterações do lúmen vascular, detectadas pela angiografia convencional (AC). Além das lesões do lúmen, as alterações na parede da aorta, caracterizadas pela ressonância magnética (RM), estão sempre presentes na AT, não sendo demonstradas pela AC. Objetivos: Descrever as lesões do lúmen da aorta e seus ramos e as alterações na parede da aorta por RM, em pacientes com AT, estabelecendo a associação entre os achados de imagem da aorta e atividade da doença. Métodos: A amostra compreendeu 45 pacientes (43 brancos, 40 mulheres, idade média de 32,5 anos + 15,5 anos), com tempo de história da doença entre três meses e 34 anos, idade de manifestação do primeiro sintoma em torno de 23 anos. Cerca de 40% dos pacientes apresentavam atividade clínica da doença, 57% em uso de corticoterapia e 30% de imunossupressor. Valores de velocidade de hemossedimentação (VHS) e de proteína C-reativa (PCR) estavam elevados em 63,2% e 52,2% dos pacientes, respectivamente. Realizadas seqüências Spin-Eco (SE), dupla inversão e recuperação (DIR) ponderadas em T1 e densidade de prótons (DP) para avaliação do espessamento na parede da aorta. Realizada seqüência DIR ponderada em T2 para pesquisa de edema na parede da aorta. Adquiriu-se seqüência angiográfica Gradiente-Eco (GE) 3D com gadolínio (Gd) para avaliação do lúmen da aorta e ramos. Através de seqüência GE 2D verificou-se a presença de realce tardio pelo Gd na parede da aorta. Resultados: Estenose foi o evento angiográfico da aorta mais encontrado (60%), preferencialmente na aorta abdominal. Dilatação foi mais freqüente na aorta torácica (12%), preferencialmente na ascendente. Irregularidade do lúmen e estenose foram os achados mais freqüentes dos ramos da aorta (69%). Espessamento na parede da aorta foi detectado por RM em todos os pacientes com AT. Edema na parede da aorta demonstrou-se ausente em 56% dos casos e realce tardio esteve presente na maioria (75%) dos casos. Não houve associação dos achados de imagem da aorta por RM com dados clínicos e laboratoriais nos pacientes com AT. Conclusões: Através da análise do lúmen e da parede arterial a RM, de forma não-invasiva, contribuiu ao diagnóstico por imagem da AT. Espessamento na parede da aorta pode ser considerado um marcador diagnóstico da doença. / Introduction: Takayasu arteritis (TA) diagnosis criteria are based on lumen abnormalities detected by conventional angiography (CA). In addition to these lesions, aortic wall abnormalities present in TA, but not clearly demonstrated by CA, can also be characterized by magnetic resonance imaging (MRI). Objectives: Describe lumen lesions in the aorta and its branches and aortic wall abnormalities using MRI, correlating these findings with disease activity in TA patients. Methods: The study comprised 45 patients (43 Caucasians, 40 women, mean age 32.5 years + 15,5 years) with a time course of TA ranging from 3 months to 34 years and first manifestation of the disease with a mean of 23 years of age. Forty percent of the patients presented clinical activity of the disease. Corticosteroid and imunossupressor therapies were used in 57% and 30%, respectively. Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were found in 63% and 52% of the patients, respectively. Aortic wall thickening was evaluated using T1 and proton density Spin-Echo (SE) and double inversion recovery (DIR) sequences. Aortic wall edema was evaluated using T2 DIR sequence. Contrast enhanced (CE) gradient-echo (GE) 3D angiography was used for the evaluation of the aortic lumen and its branches. Delayed-enhancement images of the aortic wall were investigated with a GE 2D inversion-recovery sequence after CE angiography. Results: Stenosis was the most frequent angiography finding in the aorta (60%), preferentially in the abdominal segment. Enlargement was more frequent in the thoracic aorta (12%), preferentially in the ascending segment. Minor luminal narrowing and stenosis were the most frequent findings in the aortic branches (69%). MRI detected aortic wall thickening in all patients. Aortic wall edema was absent in 56% of the patients and delayed-enhancement images of the aortic wall were present in the majority (75%) of the patients. No correlation was found between clinical and inflammatory markers and the presence of edema or delayed-enhancement in the aortic wall. Conclusions: Non-invasive MRI can accurately establish the diagnosis of TA with the analysis of the lumen and arterial wall. Aortic wall thickening could be considered a marker of the disease.

Introdução: Os critérios diagnósticos da arterite de Takayasu (AT) se baseiam em alterações do lúmen vascular, detectadas pela angiografia convencional (AC). Além das lesões do lúmen, as alterações na parede da aorta, caracterizadas pela ressonância magnética (RM), estão sempre presentes na AT, não sendo demonstradas pela AC. Objetivos: Descrever as lesões do lúmen da aorta e seus ramos e as alterações na parede da aorta por RM, em pacientes com AT, estabelecendo a associação entre os achados de imagem da aorta e atividade da doença. Métodos: A amostra compreendeu 45 pacientes (43 brancos, 40 mulheres, idade média de 32,5 anos + 15,5 anos), com tempo de história da doença entre três meses e 34 anos, idade de manifestação do primeiro sintoma em torno de 23 anos. Cerca de 40% dos pacientes apresentavam atividade clínica da doença, 57% em uso de corticoterapia e 30% de imunossupressor. Valores de velocidade de hemossedimentação (VHS) e de proteína C-reativa (PCR) estavam elevados em 63,2% e 52,2% dos pacientes, respectivamente. Realizadas seqüências Spin-Eco (SE), dupla inversão e recuperação (DIR) ponderadas em T1 e densidade de prótons (DP) para avaliação do espessamento na parede da aorta. Realizada seqüência DIR ponderada em T2 para pesquisa de edema na parede da aorta. Adquiriu-se seqüência angiográfica Gradiente-Eco (GE) 3D com gadolínio (Gd) para avaliação do lúmen da aorta e ramos. Através de seqüência GE 2D verificou-se a presença de realce tardio pelo Gd na parede da aorta. Resultados: Estenose foi o evento angiográfico da aorta mais encontrado (60%), preferencialmente na aorta abdominal. Dilatação foi mais freqüente na aorta torácica (12%), preferencialmente na ascendente. Irregularidade do lúmen e estenose foram os achados mais freqüentes dos ramos da aorta (69%). Espessamento na parede da aorta foi detectado por RM em todos os pacientes com AT. Edema na parede da aorta demonstrou-se ausente em 56% dos casos e realce tardio esteve presente na maioria (75%) dos casos. Não houve associação dos achados de imagem da aorta por RM com dados clínicos e laboratoriais nos pacientes com AT. Conclusões: Através da análise do lúmen e da parede arterial a RM, de forma não-invasiva, contribuiu ao diagnóstico por imagem da AT. Espessamento na parede da aorta pode ser considerado um marcador diagnóstico da doença. / Introduction: Takayasu arteritis (TA) diagnosis criteria are based on lumen abnormalities detected by conventional angiography (CA). In addition to these lesions, aortic wall abnormalities present in TA, but not clearly demonstrated by CA, can also be characterized by magnetic resonance imaging (MRI). Objectives: Describe lumen lesions in the aorta and its branches and aortic wall abnormalities using MRI, correlating these findings with disease activity in TA patients. Methods: The study comprised 45 patients (43 Caucasians, 40 women, mean age 32.5 years + 15,5 years) with a time course of TA ranging from 3 months to 34 years and first manifestation of the disease with a mean of 23 years of age. Forty percent of the patients presented clinical activity of the disease. Corticosteroid and imunossupressor therapies were used in 57% and 30%, respectively. Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were found in 63% and 52% of the patients, respectively. Aortic wall thickening was evaluated using T1 and proton density Spin-Echo (SE) and double inversion recovery (DIR) sequences. Aortic wall edema was evaluated using T2 DIR sequence. Contrast enhanced (CE) gradient-echo (GE) 3D angiography was used for the evaluation of the aortic lumen and its branches. Delayed-enhancement images of the aortic wall were investigated with a GE 2D inversion-recovery sequence after CE angiography. Results: Stenosis was the most frequent angiography finding in the aorta (60%), preferentially in the abdominal segment. Enlargement was more frequent in the thoracic aorta (12%), preferentially in the ascending segment. Minor luminal narrowing and stenosis were the most frequent findings in the aortic branches (69%). MRI detected aortic wall thickening in all patients. Aortic wall edema was absent in 56% of the patients and delayed-enhancement images of the aortic wall were present in the majority (75%) of the patients. No correlation was found between clinical and inflammatory markers and the presence of edema or delayed-enhancement in the aortic wall. Conclusions: Non-invasive MRI can accurately establish the diagnosis of TA with the analysis of the lumen and arterial wall. Aortic wall thickening could be considered a marker of the disease.

Diese Arbeit beschreibt die Farb-Duplex-Sonographie bei Vaskulitiden, insbesondere bei der Arteriitis temporalis (AT), bei der Takayasu-Arteriitis und bei Vaskulitis-Overlap-Syndromen. Dabei wird die erstmals von uns beschriebene charakteristische echoarme Wandschwellung der Temporalarterien (Halo-Phänomen) bei akuter AT erläutert. Dieser Befund wird durch ein Arterienwandödem erklärt und verschwindet unter Therapie mit Kortikosteroiden innerhalb von durchschnittlich 16 Tagen. Zusätzlich lassen sich Stenosen und akute Verschlüsse der Temporalarterien in der Akutphase der Erkrankung darstellen. An den ersten 30 konsekutiven Patienten mit akuter AT führten wir eine kontrollierte, prospektive Studie durch. Diese ergab, dass das Halo-Phänomen bei 73 %, Stenosen und/oder Verschlüsse bei 80 %, und alle drei pathologischen Befunde bei 93 % der Patienten mit der klinischen Diagnose einer akuten AT nachweisbar waren. Die Sensitivität gegenüber der Temporalarterien-Histologie betrug jeweils 76 %, 86 % und 95 %. Die Spezifität des Halo-Phänomens betrug 100 % und von Stenosen/Verschlüssen 93 % gegenüber der klinischen Diagnose. Inzwischen hat der Autor dieser Arbeit 742 Untersuchungen an 630 Patienten durchgeführt. Davon hatten 80 Patienten eine akute AT und 104 weitere Patienten eine akute Polymyalgia rheumatica (PMR). Sensitivität und Spezifität sind vergleichbar mit den Ergebnissen der zuvor genannten kontrollierten Studie. Dabei liegt die Sensitivität der Sonographie sogar über derjenigen der Histologie, da längere Gefäßabschnitte untersucht werden. Bei eindeutigem klinischen und sonographischen Befund erscheint es möglich, auf die Biopsie zu verzichten. Außerdem wird der sonographische Befund der peripheren Riesenzellarteriits an der A. brachialis dargestellt. Zusätzlich fanden wir bei 10 von 33 konsekutiven Patienten mit akuter AT entzündliche Veränderungen an vielen anderen Arterien. Diese Befunde belegen, dass die akute AT stärker generalisiert ist, als bisher angenommen. Selbst bei einer Wegener`schen Granulomatose kann es zum Befall großer Gefäße, wie der Arteria carotis interna kommen, der sich sonographisch darstellen lässt, wie ein weiterer Patient zeigt. Die PMR kann gemeinsam mit der AT vorkommen. Umgekehrt war bei 7 von 102 Patienten mit PMR ohne klinische Zeichen einer AT die Sonographie der Temporalarterien wegweisend für die Diagnose einer begleitenden AT. Bei der Takayasu-Arteriitis finden sich ebenfalls charakteristische, allerdings hellere Wandschwellungen. Drei Patientinnen werden beschrieben, bei denen die Sonographie bereits im prästenotischen Frühstadium für die Diagnose wegweisend war. Die Duplex-Sonographie ist eine faszinierende Methode, wenn sie bei Patienten mit Verdacht auf eine Vaskulitis großer Gefäße angewandt wird, weil es mit ihr schnell, nicht-invasiv und mit vorhandener Technik gelingt, eine Diagnose zu stellen. / Color Duplex sonography is an excellent tool in the diagnosis of vasculitides, particularly for giant cell arteritis (GCA), for Takayasu`s artertis, and for vasculitis overlap syndromes. We described a characteristic dark wall-swelling of the temporal arteries (halo) in acute GCA that is due to an edema. It resolves within about 16 days with corticosteroid therapy. Additionally stenoses and occlusions of the temporal arteries are found in acute disease. A prospective, controlled study was performed on 30 patients with acute GCA. Sensitivity was 73 % (halo), 80 % (stenoses and/or occlusions), and 93 % (at least one of the three findings) for the clinical diagnosis, and 76 %, 86 %, and 95 % for positive histology. Specificity for the clinical diagnosis was 100 % (halo) and 93 % (stenoses, occlusions). Until now the author has performed 742 investigations in 630 patients including 80 patients with acute GCA and 104 more patients with acute polymyalgia rheumatica (PMR). Sensitivity and specificity are comparable to the results of the study. Sensitivity of sonography is even superior to that of histology because a longer segment of the artery can be investigated. In cases with typical clinical and ultrasound findings biopsy may not be necessary any more. Additionally we described the ultrasound image of large-vessel GCA in the brachial artery. In 10 of 33 consecutive patients with acute GCA we found inflammatory changes in many arteries other than the temporal arteries. Thus a generalized vasculitis occurs more frequently in GCA than assumed up to now. Even in Wegener`s granulomatosis large vessels like the internal carotid artery may be involved. We described the ultrasound image of one patient. In 102 patients with PMR and no clinical symptoms of GCA sonography of the temporal arteries delineated typical findings of GCA. In Takayasu`s arteritis sonography also shows a characteristic wall swelling which is brighter than in GCA. We described three patients in which sonography aided in establishing the diagnosis already in the early prestenotic stage of the disease. Duplex sonography is a fascinating method that can be used in the diagnosis of patients with suspected large vessel vasculitis like GCA and Takayasu`s arteritis. It is fast, non-invasive, and the equipment is widely available.