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As vasculites sistêmicas primárias caracterizam-se, principalmente, pela inflamação da parede de grandes, médias e pequenas artérias, frequentemente associadas a manifestações graves que ameaçam o funcionamento de órgãos e a própria vida. Além disso, determinam a necessidade do uso de fármacos com considerável perfil de toxicidade. O principal desafio prático no manejo de tais pacientes é a determinação da presença de atividade da doença e de suas características, que fundamentalmente norteiam o plano terapêutico, além da definição do que é considerado eficácia do tratamento e remissão. Outra preocupação relaciona-se com o dano acumulado após períodos de recidiva e remissão. Uma vez que a qualidade da sobrevida é fundamental nas doenças crônicas recidivantes como as vasculites sistêmicas, são necessárias ferramentas que promovam o máximo de objetividade na avaliação de pacientes com vasculite primária. Neste artigo são descritas as principais ferramentas validadas de avaliação da atividade de doença e dano relacionado a doença nas vasculites sistêmicas de pequenos e grandes vasos. Unitermos: vasculites ANCA associadas; vasculites de grandes vasos; Takayasu; avaliação; dano; vasculites sistêmicas.

Introduction Behcet's disease (BD) is an immune-mediated chronic systemic vasculitis, characterized by clinical manifestations that include: mucocutaneous ulcers, ocular involvement, immunological alterations, vascular and neurological implications. The available treatments present limitations such as high cost and side effects, and the search for a low-cost biological treatment with immunomodulatory potential becomes of great value. Platelet rich plasma (PRP) has some characteristics that indicate a possible use as an immunomodulator due to the wide range of secreted cytokines, especially through the participation of TGF-β1 in the differentiation of T regulatory cells (Treg). This study aimed to characterize the PRP poor in leukocytes (P-PRP) of patients with BD and active ulcers and to evaluate its effects as an immunomodulator through a subcutaneous application. Methods We selected patients with a diagnosis of BD, with a low dose of prednisone and with no central nervous system or ocular involvement. Platelet and leukocyte count and quantification of 17 cytokines were evaluated in P-PRP. The effects of P-PRP were evaluated by cell frequency of TCD4 +, TCD8 +, Treg, natural killer (NK), and activated NK, as well as by the cytokine profile in patient's plasma, and the clinical manifestations through score and questionnaire. Also, it was evaluated the number and timing of oral ulcer closure. PRP was used as an adjuvant, with 9 applications of 3 mL, over 6 months, with a follow-up of one year. Results The results using PRP showed adequate values and no significant inter-and intra-individual variations. The systemic evaluations during the use of PRP showed significant alterations, characterized by the increase in Treg cell frequency (p = 0.0416) and a decrease in activated NK cells (p = 0.0010). However, no clinical correlation was observed through score analysis. The most relevant clinical data was the decrease in the closing time of ulcers throughout the application period. Conclusion In a pilot study with BD patients, P-PRP promoted an anti-inflammatory profile characterized by increased Treg cells and decreased activated NK cells and alterations in cytokines. A clinical improvement was observed with a decrease in the number and time of closure of oral ulcers.

In patients with ANCA-associated vasculitis, interactions between neutrophils and endothelial cells cause endothelial damage and imbalance. Endothelial colony-forming cells (ECFCs) represent a cellular population of the endothelial lineage with proliferative capacity and vasoreparative properties. This study aimed to evaluate the angiogenic capacity of ECFCs of patients with granulomatosis with polyangiitis (GPA). The ECFCs of 13 patients with PR3-positive GPA and 14 healthy controls were isolated and characterized using fluorescence-activated cell sorting, capillary tube formation measurement, scratching assays and migration assays with and without plasma stimulation. Furthermore, three patients with active disease underwent post-treatment recollection of ECFCs for longitudinal evaluation. The ECFCs from the patients and controls showed similar capillary structure formation. However, the ECFCs from the patients with inactive GPA exhibited early losses of angiogenic capacity. Impairments in the migration capacities of the ECFCs were also observed in patients with GPA and controls (12th h, p = 0.05). Incubation of ECFCs from patients with GPA in remission with plasma from healthy controls significantly decreased migration capacity (p = 0.0001). Longitudinal analysis revealed that treatment significantly lowered ECFC migration rates. This study revealed that ECFCs from the patients with PR3-positive GPA in remission demonstrated early losses of tube formation and reduced migration capacity compared to those of the healthy controls, suggesting impairment of endothelial function.

A 36-year-old man was admitted to the emergency department due to a 30-day history of abdominal distention and epigastralgia. He had described a non-intentional 10kg weight loss, dry cough, and fever 6 months before his admission. He had a history of tobacco and cocaine abuse and reported recurrent oral and genital ulcers. Physical examination showed an extensive area of venous collateral circulation on the abdominal wall, hepatomegaly, signs of a moderate ascites, and lower limb edema. Liver and renal function tests were normal. The ascitic fluid analysis did not show an inflammatory or infectious pattern. Upper flexible endoscopy revealed esophageal fine-caliber varices and colonoscopy showed an isolated terminal ileal ulcer. Abdominal imaging revealed hepatomegaly, voluminous ascites, and thrombosis of hepatic veins, inferior and superior vena cava (Figure 1). Infections and coagulation or lymphoproliferative disorders were excluded. Thereafter, the diagnosis of Budd-Chiari Syndrome in Behçet disease was established and immunosuppression treatment was started with good initial clinical evolution.

Objective Few studies have taken advantage of 18F-fluorodeoxyglucose positron emission tomography associated with computed tomography (18F-FDG PET/CT) to personalize patient evaluation and identify sites of more active disease in Takayasu arteritis (TA)–treated patients. This study aimed to evaluate the utility of 18F-FDG PET/CT in late acquisition in identifying sites of active disease in patients under full treatment for TA. Methods In this cross-sectional study, patients under full treatment underwent whole-body 18F-FDG PET/CT. Sites of increased 18F-FDG uptake were classified by a score of 3 on the visual scale using the liver uptake as reference. A quantitative analysis was also performed by measuring the maximum standardized uptake value (SUV) of the vascular wall of affected arteries. Disease activity using the National Institutes of Health criteria was also evaluated. Results Of the 20 patients, there were 18 female and 2 male patients, with a mean age of 43.6 (±11.58) years and a disease duration of 8.3 (±6.25) years. Thirteen participants (65%) were in inflammatory activity according to the criteria proposed by the National Institutes of Health. All patients received immunosuppressive agents, and one of them received immunobiological treatment. The highest SUV value was 6.2 in the aortic arch, and the lowest was 1.0 in the subclavian artery. The mean maximum SUV did not differ between clinically active and inactive patients. In the visual analysis, all participants had at least 1 vascular site with inflammatory activity, with an uptake ≥2 in relation to the liver. The aortic arch was the most frequently involved site. Conclusions This study showed that 18F-FDG PET/CT in late acquisition is an effective imaging method to assess TA activity even in fully treated patients.