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Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach. Current practice relies on recommendations and guidelines addressing the management of ANCA-associated vasculitis and not specifically developed for EGPA. Here, we present evidence-based, cross-discipline guidelines for the diagnosis and management of EGPA that reflect the substantial advances that have been made in the past few years in understanding the pathogenesis, clinical subphenotypes and differential diagnosis of the disease, as well as the availability of new treatment options. Developed by a panel of European experts on the basis of literature reviews and, where appropriate, expert opinion, the 16 statements and five overarching principles cover the diagnosis and staging, treatment, outcome and follow-up of EGPA. These recommendations are primarily intended to be used by healthcare professionals, pharmaceutical industries and drug regulatory authorities, to guide clinical practice and decision-making in EGPA. These guidelines are not intended to limit access to medications by healthcare agencies, nor to impose a fixed order on medication use.

Data are sparse regarding the prevalence of pulmonary hypertension (PH) in obstructive sleep apnoea (OSA) patients without COPD and clinically manifest cardiac diseases and the role of continuous positive airway pressure (CPAP) and Uvulopalatopharyngoplasty (UPPP) in normalizing this parameter.We studied 75 consecutive OSA patients, 55 of them men, using transthoracic echocardiography. A mild PH [pulmonary artery pressure (PAPs) 38.2 ± 6.8] was found in 25 subjects (prevalence 33%). These patients were divided into two groups: group 1A (n = 17), those treated with CPAP, and group 1B (n = 8), those who have the indication for a UPPP. We scheduled a follow-up at 3, 6 and 9 months. During follow-up, we performed echocardiography, measurement of anthropometric parameters (BMI, neck and waist-hip circumference), and of biochemical parameters (uric acid, fasting glucose, cholesterol, triglycerides) and blood pressure.Patients with PH had a higher BMI: 32 ± 6 versus 29 ± 4 (p0.001) and NC: 39.8 ± 4.76 versus 37.14 ± 3.49 (p = 0.003), were predominantly men (72%) and older: 64 ± 20 versus 55 ± 16 (p = 0.025) and had a significantly higher value of uric acid: 7.91 ± 2.35 versus 6.56 ± 1.31 (p = 0.003). We found a positive correlation between PH and BMI (r = 0.456; p0.001) and between uric acidic and PH (r = 0.636; p0.001). PAPs significantly changed, from 39.8 ± 4.1 to 27.1 ± 4, to 25.2 ± 3.1 and to 22.2 ± 3 mmHg (CI 95%; 15.09-20.11; p0.001) in group 1A and from 39.5 ± 5.1 to 23.4 ± 3.2, to 23.0 ± 3.1 and to 21.9 ± 2.9 mmHg (CI 95%; 13.15-22.05; p0.001) in group 1B (difference between the groups p = 0.12).PH was frequent in OSA patients and normalized after 6 months with both CPAP and UPPP. A similar trend was noted in diastolic blood pressure.