Increased modifiable cardiovascular risk factors in patients with Takayasu arteritis: a multicenter cross-sectional study

Abstract Background: Modifiable cardiovascular risk factors (MCRFs), such as those related to aerobic capacity, muscle strength, physical activity, and body composition, have been poorly studied in Takayasu arteritis (TAK). Therefore, the aim of the study was to investigate MCRFs and their relationships with disease status and comorbidities among patients with TAK. Methods: A multicenter cross-sectional study was conducted between 2019 and 2020, in which 20 adult women with TAK were compared with 16 healthy controls matched by gender, age, and body mass index. The following parameters were analyzed: aerobic capacity by cardiopulmonary test; muscle function by timed-stands test, timed up-and-go test, and handgrip test; muscle strength by one-repetition maximum test and handgrip test; body composition by densitometry; physical activity and metabolic equivalent by IPAQ, quality of life by HAQ and SF-36; disease activity by ITAS2010 and NIH score; and presence of comorbidities. Results: Patients with TAK had a mean age of 41.5 (38.0-46.3) years, disease duration of 16.0 (9.5-20.0) years, and a mean BMI of 27.7±4.5 kg/m2. Three out of the 20 patients with TAK had active disease. Regarding comorbidities, 16 patients had systemic arterial hypertension, 11 had dyslipidemia, and two had type 2 diabetes mellitus, while the control group had no comorbidities. TAK had a significant reduction in aerobic capacity (absolute and relative VO2 peak), muscle strength in the lower limbs, increased visceral adipose tissue, waist-to-hip ratio, reduced walking capacity, decreased weekly metabolic equivalent, and quality of life (P< 0.05) as compared to controls. However, there were no correlations between these MCRFs parameters and disease activity. Conclusions: TAK show impairment in MCRFs; therefore, strategies able to improve MCRF should be considered in this disease.(AU)

Poor obstetric outcomes in Indian women with Takayasu arteritis

Abstract Introduction: Takayasu's arteritis (TA) affects young women in the childbearing age group. We studied obstetric outcomes in these patients before and after disease onset. Methods: Women aged more than 18 years with Takayasu's arteritis (ACR 1990 criteria) were included. Demographic data, clinical features, disease activity using Indian Takayasu Arteritis clinical score (ITAS), Disease Extent Index for TA (DEI. TaK) and damage assessment using TA Damage score (TA), history of conception and maternal and fetal outcomes were recorded from hospital records and telephonic interview. Results are in median and IQR. Results: Of the 64 women interviewed, aged 29 (24-38) years and disease duration 5 (4-10) years, 74 and 38 pregnancies had occurred before and after disease diagnosis in 29 and 20 women respectively. In eight, the diagnosis was made during pregnancy. Age at disease onset was 22 (18-30) years. Type 5 disease was the most common ( n = 32, 59.3%), and an equal number of patients had Ishikawa's class I and II disease ( n = 26, 40.6%). Median ITAS ( n = 44) was 13 (7-16), DEI. Tak 12.5 (9-16.75) and TADS 8 (6.5-10). Twenty-five patients wanted to get pregnant, of which 8 (32%) did not do so because of their disease. Fifteen were unmarried of whom 6 did not marry due to disease. Obstetric outcomes were poorer in pregnancies that occurred after the onset of disease as compared with those before it (RR = 1.5, p = 0.01). Pregnancies after the onset of TA carried a very high risk of maternal [RR3.9 (1.8-8.5), P < 0.001] as well as fetal complications [RR = 2.0 (1.2-3.4), p = 0.001]. Hypertension was the most common maternal complication and occurred most often in the last trimester. The baby weight at birth was lower in pregnancies after disease (2.3 vs. 3.0, p = 0.01). Wong's score greater than or equal to 4 predicted lower birth weight ( p = 0.04). ITAS, ITAS-A, DEI. Tak and TADS could not predict obstetric outcomes, and ITAS score exhibited moderate correlation with DEI. Tak ( r = 0.78) and TADS ( r = 0.58). Conclusion: Women with TA suffer from extremely high risk of poor maternal and foetal outcomes. Wong's scoring can be useful to predict birth weight.(AU)

Cerebral Aneurysm in Takayasu's Arteritis: A Case Report and Review of the Literature

Cerebral aneurysms are rarely observed in patients with Takaysu's arteritis. To our knowledge, this report presents the first case described in Brazil. Hemodynamic stress caused by obstructions of the cervical vessels developes cerebral aneurysms in these patients and they have higher rates of multiple and posterior circulation aneurysms than general population. In the majority of cases, microsurgical treatment is indicated mainly due to changes in the cervical vessels that preclude an endovascular approach.

Aneurismas cerebrais são raramente observados em pacientes com arterite de Takayasu. Este é o primeiro caso desta associação descrito no Brasil. O estresse hemodinâmico causado pelas obstruções vasculares cervicais desenvolve os aneurismas cerebrais nestes pacientes e eles apresentam maiores taxas de aneurismas múltiplos e de acometimento da circulação cerebral posterior. Na maioria dos casos, o tratamento microcirúrgico está indicado pois as alterações anatômicas dos vasos cervicais dificultam a abordagem endovascular.

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review / Atualização das manifestações neurológicas das vasculitides e das doenças do tecido conjuntivo: revisão de literatura

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

As vasculites são um grupo heterogêneo de doenças autoimunes primárias ou secundárias a uma grande variedade de doenças infecciosas sistêmicas, malignas ou do tecido conjuntivo. Estas últimas são as que ocorrem com mais frequência, porém os mecanismos patogênicos ainda não foram plenamente determinados. Diversas e frequentes complicações do sistema nervoso central e periférico ocorrem nas vasculites e doenças do tecido conjuntivo. Em muitos casos, os distúrbios neurológicos têm evolução clínica atípica ou mesmo início precoce, ao que todos os profissionais de saúde devem estar cientes. O objetivo desta breve revisão foi atualizar os principais distúrbios neurológicos da vasculite comum e das doenças do tecido conjuntivo, visando ao diagnóstico e ao tratamento corretos, com ênfase nos mecanismos fisiopatológicos.

Enfermedad de Takayasu: avances en fisiopatología, diagnóstico y tratamiento / Takayasu disease: advances in pathophysiology, diagnosis and treatment

Vasculitis are a group of heterogeneous diseases characterized by inflammation of vascular territories and a systemic compromise. Currently we organize the vasculitis syndromes by the size of the affected-vessel (large, medium and small vessel vasculitis). Takayasu disease (TAK) is a type of large-vessel vasculitis. In the pathogenesis of the disease is very important the participation of the dendritic cell in the vascular adventitia and the secondary activation of lymphocytes, specially with responses TH1 and TH17. The aorta and its branches are the most frequently vessels affected. The diagnose of TAK is based in clinical characteristics (vascular claudication, pulse assimetry, arterial pressure assimetry), basic laboratory (ESR and CRP) and images. There is not a good score to evaluate activity of the disease. The images studies are very important in the follow up of these patients. New image studies as PET CT could help in the evaluation of activity. Corticosteroids continue to be the mainstay in the treatment of the patients and there is few evidence of the utility of sparing-corticosteroids drugs. Biological drugs may be in the future a great alternative with very good results in preliminary studies. Vascular interventions are often needed in patients with TAK. Vascular surgery have a good outcomes compared to endovascular procedures. The moment of vascular intervention must be choosed with care because the outcomes are better when the disease is inactive.

Las vasculitis son un grupo heterogéneo de enfermedades caracterizados por inflamación vascular y compromiso sistémico. Actualmente los síndromes vasculíticos se organizan de acuerdo al tamaño de los vasos afectados (vasculitis de vaso grande, medianos y pequeños). La enfermedad de Takayasu es un tipo de vasculitis de vaso grande. En la patogénesis de la enfermedad es muy importante la participación de las células dendríticas en la adventicia vascular y en la activación secundaria de poblaciones linfocitarias con respuestas fundamentalmente de tipo TH1 y TH2. El diagnóstico de Takayasu está basado en características clínicas (claudicación vascular, asimetría de pulsos, asimetría de presión arterial), laboratorio básico (VHS y proteína C reactiva) y estudios de imágenes. En la actualidad no existe un buen sistema de score para evaluar la actividad de la enfermedad. Los estudios de imágenes son muy importantes en el seguimiento de la enfermedad. Nuevos estudios radiológicos como el PET-CT podrían ayudar en la evaluación de actividad. Los corticoides continúan siendo el tratamiento más importante. Las drogas ahorradoras de esteroides pueden ser utilizadas pero con poca evidencia de efectividad. Las drogas biológicas han probado buena respuesta en estudios preliminares. Las intervenciones vasculares son frecuentemente requeridas en pacientes con Takayasu. Los procedimientos de cirugía vascular han demostrado mejores resultados que las intervenciones endovasculares. Los resultados de las intervenciones vasculares son mejores cuando el procedimiento se realiza con enfermedad inactiva.

Arterite de Takayasu / Takayasu's arteritis

O presente caso refere-se a uma menina de 15 anos de idade que apresentava febre e anemia durante seis meses, acompanhadas de claudicação do membro superior direito e sopro abdominal em região do epigástrio.

The present case refers to a 15 years-old girl that showed symptoms of fever and anemia during six months, followe of lameness of the right shoulder and abdominal blow in region of epigastrium.

Posterior reversible encephalopathy syndrome revealing Takayasu's arteritis.

Posterior reversible encephalopathy syndrome is a rare neuroradiologic condition associated with headache, seizures, altered sensorium, visual disturbances, and characteristic lesions on neuroimaging predominantly affecting the posterior regions of the brain. We report a 10-years-8-months-old girl who presented with headache, multiple seizures, and altered sensorium. Her blood pressure was 130/100 mmHg and left brachial pulse was not palpable. CT scan brain showed typical non-enhancing hypodensities in bilateral parieto-occiptal lobes. Prompt treatment of the hypertension led to rapid reversal of neurological symptoms. CT aortogram revealed aortoarteritis with bilateral renal artery stenosis.

Takayasu arteritis: clinical and CT-angiography profile of 25 patients and a brief review of literature.

OBJECTIVE: To evaluate the clinical profile, complications, site of involvement, and spectrum of CT Angiography (CTA) abnormalities in patients of Takayasu arteritis (TA). MATERIALS AND METHODS: From August 2005 to December 2006, 25 patients of TA were studied for their presenting features, pattern of vascular involvement, and spectrum of CTA abnormalities. Vascular involvement by imaging was correlated with clinical features. RESULTS: In all, 25 patients-(18 females, 7 males) were enrolled in the study. The mean age of the patients at diagnosis was 21.5 years (12-38) with a mean delay of 4.5 years (6 months7ndash;6 years) before diagnosis. Symptoms included fever in 8 (32%), malaise in 15 (60%), hypertension in 18 (72%), and pulselessness in 20 (80%) patients.The laboratory findings included elevated ESR in 15 (60%), elevated CRP in 5 (20%), and positive mantoux test in 5 (20%) patients. Almost all patients had multiple sites of arterial involvement documented by CTA with various combinations of stenosis, luminal irregularities, and aneurysm formation. TA - Type V was the most common type found in our study. CONCLUSION: TA is a rare disease with a myriad of presentations. Delay in diagnosis is frequent but can be avoided by detailed physical examination including vascular system examination. CTA is very useful for detailed vascular evaluation of patients with TA.

Arteritis de Takayasu en México: una serie de 65 casos consecutivos / Takayasu arteritis in Mexico: sixty-five consecutive cases

Dentro de las vasculitis primarias, las que afectan grandes arterias son raras. Una, la arteritis inespecífica llamada "Takayasu", parece ser común en México. Dada su morbilidad cardiovascular se ha estudiado con predilección en nuestro hospital. Objetivo. Reseña de las condiciones habituales de diagnóstico en 65 casos consecutivos con panaortografía definitiva. Material y métodos. Serie de casos, estudio retrolectivo, descriptivo, observacional. Estadística descriptiva. Resultados. La morbilidad de la enfermedad es cardiovascular y neurooftálmica, solo un cuarto de los casos tiene datos que sugieren inicio sistémico, la enfermedad crónica provoca hipertensión arterial sistémica, isquemia en distintos territorios y obvias deficiencias de pulsos, diferencia en la tensión arterial e insuficiencia cardiorrenal. En el laboratorio son útiles la biometría hemática, la eritrosedimentación, y la determinación de fibrinógeno y PCR. No hay tratamiento definido. Conclusión. Es necesario un índice de sospecha y maniobras diagnósticas específicas para reconocer la Arteritis de Takayasu. Se requiere un estudio multicéntrico para definir la terapéutica médico-quirúrgica en esta arteritis primaria