Objectives ANCA-associated vasculitis (AAV) can affect all age groups. We aimed to show that differences in disease presentation and 6 month outcome between younger- and older-onset patients are still incompletely understood. Methods We included patients enrolled in the Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS) study between October 2010 and January 2017 with a diagnosis of AAV. We divided the population according to age at diagnosis: <65 years or ≥65 years. We adjusted associations for the type of AAV and the type of ANCA (anti-MPO, anti-PR3 or negative). Results A total of 1338 patients with AAV were included: 66% had disease onset at <65 years of age [female 50%; mean age 48.4 years (s.d. 12.6)] and 34% had disease onset at ≥65 years [female 54%; mean age 73.6 years (s.d. 6)]. ANCA (MPO) positivity was more frequent in the older group (48% vs 27%; P = 0.001). Younger patients had higher rates of musculoskeletal, cutaneous and ENT manifestations compared with older patients. Systemic, neurologic,cardiovascular involvement and worsening renal function were more frequent in the older-onset group. Damage accrual, measured with the Vasculitis Damage Index (VDI), was significantly higher in older patients, 12% of whom had a 6 month VDI ≥5, compared with 7% of younger patients (P = 0.01). Older age was an independent risk factor for early death within 6 months from diagnosis [hazard ratio 2.06 (95% CI 1.07, 3.97); P = 0.03]. Conclusion Within 6 months of diagnosis of AAV, patients >65 years of age display a different pattern of organ involvement and an increased risk of significant damage and mortality compared with younger patients.

Takayasu's arteritis (TAK) is a type of large vessel vasculitis which involves the aorta and its major branches. Interleukin (IL)-9 or IL-9-producing Th9 cells were found to be involved in pathogenesis of autoimmune arteritis such as giant cell arteritis, but IL-9 or Th9 cells in TAK were not well known. Here, this study aims to analyze the levels of serum IL-9 and their major source Th9 cells in TAK. With the help of cytometric bead array (CBA), a total of 21 patients with TAK were examined for serum levels of cytokines IL-4, IL-6, IL-8, IL-17, IL-10, TNF-α, IFN-γ, and IL-9. Flow cytometry techniques were used to examine the frequencies of Th9 cells from peripheral blood mononuclear cells (PBMCs) for 11 patients with active TAK and 10 healthy controls. Higher serum levels of serum IL-6 (P < 0.05), TNF-α (P < 0.05), and IL-9 level (P < 0.05) were observed in TAK patients compared to those of healthy controls. Higher frequencies of CD4 IL-9 T cells and CD4 PU.1 T cells in PBMCs and IL-9 PU.1 T cells in CD4 T cells were observed in active TAK patients than those in healthy controls (all P < 0.01). The levels of IL-9 had a positive correlation with ESR (r = 0.975, P = 0.015) in these cases. Our data suggested that Th9 cells and IL-9 could possibly be involved in the pathogenesis of TAK.

BackgroundBehcet's disease is a kind of systemic vasculitis. Multiple organs including derma, mucosae, articulation, neurologic system, gastrointestinal tract and cardiovascular system might be involved. Vital organ complications lead to poor outcomes. Cardiovascular involvement in Behcet's disease is estimated to range from 7% to 40%, with fatality rate reaches up to 29% [1–3].The characteristics of Chinese Behcet's disease patients with cardiovascular lesions were rarely reported before.ObjectivesTo investigate the clinical characteristics of cardiovascular involvement in Behcet's disease.MethodsWe reviewed 35 medical records of patients of Behcet's disease with cardiovascular lesions in Anzhen Hospital, Capital Medical University between January 2002 and January 2014. All the patients fulfilled the international Behcet's disease study group criteria [4].Results(1) The mean (±SD) age when the cardiovascular disorders flare-up was 36.9±9.6 years. 27/35 (77.1%) of the patients were male.(2) The clinical spectrum of cardiovascular abnormalities included valvular regurgitation (20,57.1%, among these patients, two patients complicated with infectious endocarditis, and five patients had perivalvular leakage after primary valvular surgery), conduction system disturbances (10,28.6%), intracardiac thrombosis (1,2.9%), aneurysms of the arteries (13,37.1%), artery stenosis or occlusion (5,14.3%), pulmonary vascular diseases (3,8.6%), venous thrombosis (4,11.4%). 20 patients had more than one of these lesions simultaneously.(3) Clinical manifestations were varied from shortness of breath (17,48.6%), febrile (9,25.7%), chest pain (5,14.3%), dizziness (4,11.4%), cough (4,11.4%), limb ischemia (4,11.4%), palpitation (3,8.6%), hemoptysis (3,8.6%), seroperitoneum (2,5.7%), facial edema (1,2.9%), dysphagia (1,2.9%), to asymmetric blood pressure (1,2.9%).(4) Inflammatory indicators erythrocyte sedimentation rate and C-reactive protein were not significantly different between the different subgroups (P=0.26 and P=0.972, respectively).(5) Ten patients received corticosteroids and immunosuppressive drugs treatment. Intervention therapy (aortic coated stent lumen isolation) was administered on one patient. Operations (including valve replacement, valular annulus reconstruction, Bentall, aorta replacement and heart transplantation) were performed on 12 patients. One patient (2.9%) died from multiple organ dysfunction during hospital stay.ConclusionsCardiovascular involvement in Behcet's disease is a serious systemic lesion deserved attention. Corticosteroids and immunosuppressive drugs together with intervention therapy or operations are effective remedies for Behcet's disease patients with cardiovascular lesions.ReferencesKajiya T, Anan R, Kameko M, et al. Intracardiac thrombus, superior vena esva syndrome, and pulmonary embolism in a patient with Behcet's disease: a case report and literature review[J]. Heart Vessels, 2007, 22(4):278–283.Geri G, Wechsler B, Thi Huong du L, et al. Spectrum of cardiac Iesions in Behcet disease: a serie...