Different epidemiologic profiles of systemic vasculitis between Brazil and Peru-preliminary results in two referral centers from both countries.

Little is known about the epidemiology of systemic vasculitis in South American countries. The aim of this study is to compare the prevalence of systemic vasculitides in two vasculitis referral centers from Brazil and Peru. A cross-sectional study was performed and all patients above 18 years of age, with at least 6 months of follow-up and who met classification or diagnosis criteria for the most common forms of vasculitis, were included. A total of 562 patients with systemic vasculitis were analyzed, 345 (61.4%) from Brazil and 217 (38.6%) from Peru. The frequency of Behçet's disease (37.9% vs. 1.8%; p < 0.0001), Takayasu arteritis (TAK) (25.2% vs. 6.9%; p < 0.0001), and giant cell arteritis (9.8% vs. 0.9%; p < 0.0001) was higher in the Brazilian center than the Peruvian one. On the other hand, the frequency of microscopic polyangiitis (MPA) (67.3% vs. 2.8%; p < 0.0001) and renal-limited vasculitis (2.8% vs. 0.0%; p = 0.009) was higher in the Peruvian center. No differences were found concerning other forms of vasculitis. At diagnosis, Brazilian patients with TAK, granulomatosis with polyangiitis, and MPA were younger than Peruvian patients. Epidemiologic differences in the frequency of systemic vasculitis are observed between a vasculitis referral center from Brazil and another from Peru. Key Points • Significant differences are observed regarding the epidemiologic profile of systemic vasculitis between Brazil and Peru. • MPA is the predominant form of vasculitis in Peru while BD and TAK are the most frequent forms of vasculitis in Brazil. • The age at diagnosis of TAK, MPA, and GPA was lower in Brazilian patients than in Peruvian patients.

Vasculitides in HIV Infection.

PURPOSE OF REVIEW: To review the spectrum of vasculitides in HIV-infected patients and to identify the clinical features that characterize vasculitis in sero-positive HIV. RECENT FINDINGS: Epidemiological studies conducted in the post-HAART era described the rarity of vasculitis in the setting of HIV-infected patients. A study identified histopathological features such as leukocytoclastic vasculitis of the vasa vasorum and adventitial inflammation in the large artery pathology of HIV-positive patients compared with HIV-negative patients with critical lower limb ischemia. A recent retrospective cohort study reported that HIV-positive patients with LVV developed more vascular complications, responded less to antiretroviral therapy, and had worse outcome than HIV-negative patients with LVV. Vasculitides continue to be a rare disease in patients with HIV. The spectrum of vasculitis ranges from life-threatening conditions to relatively mild skin conditions. Recognizing vasculitis in the setting of HIV-positive patients is important because sometimes it require immunosuppressive treatment.

Translation and validation of the Indian Takayasu clinical activity score (ITAS2010) for the Brazilian Portuguese language.

BACKGROUND: The Indian Takayasu Clinical Activity Score (ITAS2010) was developed in 2010 as an assessment tool for disease activity in patients with Takayasu arteritis (TA). It has since been widely used in different studies and in clinical practice for the management of patients with TA. The present study aims to translate the ITAS2010 into Brazilian Portuguese language and to validate it for use in clinical practice in Brazil. METHODS: For this cross-sectional study, the ITAS2010 was translated in accordance with the guidelines described by Beaton et al. and then applied with 27 patients with TA on three assessments by two rheumatologists working independently. To measure interrater agreement, the assessments were performed on the same day within approximately 1 hour. One of the rheumatologists performed a second evaluation of patients with TA within 7 to 14 days to measure intrarater agreement. RESULTS: The correlation coefficient for the ITAS2010 score between the two raters was high (r = 0.916; p < 0.0001), as well as the intraclass correlation coefficient (ICC) [0.918 with a 95% confidence interval (95CI): 0.828-0.962]. The correlation coefficient and the ICC for intrarater agreement were moderate for ITAS2010 (r = 0.633; p < 0.0001 and ICC = 0.594; 95CI: 0.292-0.790). The ITAS2010 at baseline was compared with the physician's global assessment (PGA) and with Kerr's criteria for detecting disease activity in TA. Higher ITAS2010 scores were observed in patients with active and grumbling/persistent disease than in those presenting inactive disease according to the PGA [1.5 (0.0-3.0) vs. 0.0 (0.0-0.0); p = 0.0025]. Patients with active disease according to the Kerr's criteria had also higher ITAS2010 scores than those considered in remission [3.0 (3.0-7.0) vs. 0.0 (0.0-0.0); p = 0.0068]. CONCLUSIONS: The Brazilian Portuguese version of the ITAS2010 is a valid and reproducible tool for the assessment of disease activity in TA and it is an additional tool for the routine evaluation of Brazilian patients with TA.

Translation and validation of the Indian Takayasu clinical activity score (ITAS2010) for the Brazilian Portuguese language

Abstract Background: The Indian Takayasu Clinical Activity Score (ITAS2010) was developed in 2010 as an assessment tool for disease activity in patients with Takayasu arteritis (TA). It has since been widely used in different studies and in clinical practice for the management of patients with TA. The present study aims to translate the ITAS2010 into Brazilian Portuguese language and to validate it for use in clinical practice in Brazil. Methods: For this cross-sectional study, the ITAS2010 was translated in accordance with the guidelines described by Beaton et al. and then applied with 27 patients with TA on three assessments by two rheumatologists working independently. To measure interrater agreement, the assessments were performed on the same day within approximately 1 hour. One of the rheumatologists performed a second evaluation of patients with TA within 7 to 14 days to measure intrarater agreement. Results: The correlation coefficient for the ITAS2010 score between the two raters was high (r =0.916; p < 0.0001), as well as the intraclass correlation coefficient (ICC) [0.918 with a 95% confidence interval (95CI): 0.828-0.962]. The correlation coefficient and the ICC for intrarater agreement were moderate for ITAS2010 (r =0.633; p < 0.0001 and ICC = 0.594; 95CI: 0.292-0.790). The ITAS2010 at baseline was compared with the physician's global assessment (PGA) and with Kerr's criteria for detecting disease activity in TA. Higher ITAS2010 scores were observed in patients with active and grumbling/persistent disease than in those presenting inactive disease according to the PGA [1.5 (0.0-3.0) vs. 0.0 (0.0-0.0); p = 0.0025]. Patients with active disease according to the Kerr's criteria had also higher ITAS2010 scores than those considered in remission [3.0 (3.0-7.0) vs. 0.0 (0.0-0.0); p = 0.0068]. Conclusions: The Brazilian Portuguese version of the ITAS2010 is a valid and reproducible tool for the assessment of disease activity in TA and it is an additional tool for the routine evaluation of Brazilian patients with TA.

Cardiogenic shock as the first manifestation of large vessel vasculitis in a young patient: case report.

BACKGROUND: Cardiogenic shock secondary to coronary involvement in large vessel vasculitis (LVV) is an unsuspected finding, even more, when no other vascular territories are compromised and when it constitutes the initial clinical manifestation. This case report illustrates a case in which a complete diagnostic study uncovered this aetiology. CASE SUMMARY: A 33-year-old woman with cough and chest pain who was diagnosed with acute bronchitis returned with worsening dyspnoea, chest pain, and developed cardiogenic shock. The initial differential diagnoses included myocarditis and takotsubo cardiomyopathy (TCM) owing to a positive troponin I, and echocardiogram with left ventricular dilation, dyskinesia in mid-ventricular and apical segments, systolic dysfunction, and functional mitral regurgitation. A cardiac magnetic resonance showed contractility abnormalities resembling the pattern of TCM but lacked the characteristic myocardial oedema. Subsequently, a coronary angiography expected to result without obstructions showed a critical narrowing of the left main coronary artery. Surgical management consisted of a pericardium patch grafted in the stenotic ostium to restore adequate perfusion. The surgical specimens were sent to the pathology laboratory that reported findings compatible with LVV. Four days after the surgical intervention the patient was discharged alive with a complete recovery of left ventricular systolic function. DISCUSSION: Chest pain symptoms in a young woman, could be caused by multiple entities, and an ischaemic aetiology from a non-atherosclerotic origin should be kept in mind. A complete study with coronary angiography is crucial to rule out an ischaemic cause even in low-risk groups for atherosclerotic coronary heart disease.

Arteritis de células gigantes relacionada a grandes vasos: a propósito de un caso / Large vessel giant cell arteritis: a case report

La arteritis de células gigantes corresponde a una vasculitis granulomatosa que afecta arterias de mediano y gran tamaño. El fenotipo clínico más conocido es la arteritis de la temporal, caracterizado por síntomas craneales clásicos como cefalea temporal, claudicación mandibular y síntomas visuales. Sin embargo, esta enfermedad puede comprometer otras grandes arterias como la aorta y sus ramas principales, denomi-nándose así, como arteritis de células gigantes de grandes vasos, la cual puede o no estar asociada a síntomas craneales. Presentamos el caso de una mujer de 74 años, con un cuadro de un mes de evolución, caracterizado por claudicación intermitente de extremidades inferiores, asociado a baja de peso de 3 kilos, sudoración nocturna. Al examen físico, fiebre y pulsos dismi-nuidos en extremidades inferiores.

Giant cell arteritis is a granulomatous vasculitis that affects arteries of medi-um and large size. The most well-known clinical phenotype is temporal arteri-tis, characterized by classic cranial symptoms such as temporal headache, man-dibular claudication and visual symptoms. However, this disease can involve other large arteries such as the aorta and its main branches, known as large ves-sel giant cell arteritis, which may or may not be associated with cranial symptoms.A 74-year-old woman is presented with claudication of lower extremities, associated with weight loss of 3 kilos, night sweats and fever over the past month. Physical ex-amination reveals decreased pulses in the lower extremities.

Arteritis de células gigantes con compromiso de aorta y grandes vasos / Aortitis and large vessel involvement in giant cell arteritis

La arteritis de células gigantes (ACG) es una vasculitis sistémica de grandes vasos que no solo puede afectar a vasos craneales, si no que cada vez más frecuentemente se ha asociado a compromiso de vasos extracraneales. Este compromiso puede ser asintomático por lo que puede pasar inadvertido al estudio clínico básico y llevar a importantes complicaciones. Se presentan dos casos clínicos de ACG, ambos con aortitis y uno, además, con compromiso de grandes vasos del cayado aórtico y del cuello. Se discuten aspectos fisiopatológicos, clínicos y terapéuticos, con el objetivo de vislumbrar estrategias de abordaje de futuros pacientes con presentaciones similares.

Giant cell arteritis (GCA) is a age related, large vessels systemic vasculitis that not only affect cranial vessels, but is increasingly associated with extracranial vessel involvement. This extracranial involvement can be asymptomatic, so it can go unnoticed after basic clinical and laboratory study and lead to serious complications. We report two cases of GCA, both presenting aortitis and one of them presenting also involvement of aortic arch and of neck large vessels. We discuss physiopathologic, clinic and therapeutic aspects, with the aims of developing management strategies for the future patients with similar manifestations.