IL-5 production is pathogenetically central for the development of the disease by promoting proliferation, transvascular migration and functional activation of eosinophils. Birmingham Vasculitis Activity Score (BVAS), prednisone intake, Sino-Nasal Outcome Test (SNOT-22), Total Endoscopic Polyp Score (TENPS), Asthma Control Test (ACT), Forced Expiratory Volume one second (FEV1)%, blood eosinophilia. BVAS score significantly decreased showing a sharp reduction in disease activity score. Clinical improvements in terms of sinonasal scores and asthma symptoms were observed, in parallel with a drastic drop in eosinophil blood count. Prednisone intake was significantly reduced. In two patients, asthma exacerbations led to discontinuation in mepolizumab therapy after 6 and 12?months despite BVAS reduction. Mepolizumab treatment was well tolerated, and no severe adverse drug effects were registered. In conclusion, our 12-month real-life study suggests that mepolizumab may be beneficial and safe in active EGPA patients by improving disease activity score, sinonasal and asthma outcomes while reducing the burden of prednisone intake. infection. Patients were treated with mepolizumab as add-on therapy to oral corticosteroids (OCS), inhaled long-acting beta2-agonists (LABA)/corticosteroids (ICS) associations, plus long-acting anti-antimuscarinic drugs (LAMA) as stated by step 5 GINA guidelines for severe asthma, and intranasal corticosteroids (mometasone 50?g spray: one spray in the morning and one spray in the evening for each nostril) for rhinosinusitis with or Sauristolactam without nasal polyposis. Sauristolactam For one out of eight patients of the study not receiving prednisone at baseline, mepolizumab was added to immunosuppressor treatment. As mepolizumab is not yet approved for EGPA in Italy, the drug was prescribed for treatment of severe eosinophilic asthma, according to the Rabbit Polyclonal to CCDC102B recommendations of the Italian Drug Agency (AIFA) at a dosage of 100?mg q4w s.c. for a 12-month course. The parameters used to verify the efficacy of mepolizumab were: BVAS, SNOT-22, TENPS, ACT, FEV1%, blood eosinophils and prednisone dosage, performed at baseline (T0) and after 6 (T6) and 12?months (T12) of mepolizumab treatment. The BVAS is designed to document clinical features that are directly due to active vasculitis. It rates 10 areas of interest (general, cutaneous, mucous membranes, eyes, ENT, chest, cardiovascular, abdominal, renal, nervous system, other) [30]. Disease manifestations are scored only when they are attributable to active vasculitis. Score superior to 0 suggests active disease. SNOT-22, the most commonly used instrument for patients reported outcomes [31, 32] Sauristolactam was administered. It rates 22 different symptoms from 0 (no problem) to 5 (problem as bad as it can be) related to rhinological, ear, facial, general, physical, and psychological domains. Rhinofibroscopy (with a straight forward telescope 0 and forward oblique telescope 30, diameter 4?mm, length 18?cm. Straight forward telescope 0, diameter 3?mm, length 14?cm) was performed in order to validate a diagnosis of CRSwNP [33]. Each nostril was scored as follows: score 0-no polyps; score 1-small polyps in the middle meatus not reaching below the inferior border of the middle concha; score 2-polyps reaching below the lower border of the middle turbinate; score 3-large polyps reaching the lower border of the inferior turbinate or polyps medial to the middle concha: score 4-large polyps causing almost complete congestion/nasal obstruction of the inferior meatus. A patient with severe bilateral polyposis would therefore have a maximum score of eight. Sinus involvement was also assessed by computer tomography scan and/or magnetic resonance imaging. ACT is a validated five-item questionnaire for assessing asthma control. Each item is scored on a five-point scale from 1 to 5 (1?=?worst; 5?=?best), with a total score of 5C19 and 20C25 points describing uncontrolled and well-controlled asthma, respectively. Spirometry (model “Quark PTF”, COSMED) before and after salbutamol 400 mcg, was performed according to the guidelines of the American Thoracic Society/European Respiratory Society (ATS/ERS) [34]. FEV1, Forced Vital Capacity (FVC), FEV1/FVC were measured, and the best of three forced maneuvers was recorded. Results were expressed both as absolute values and as Sauristolactam a percentage of the predicted values referred to European Respiratory Society (ERS) 1993 reference values. Asthma exacerbations were registered as an acute or subacute worsening in symptoms and lung function from the patients usual status [35]. Pulmonary involvement Sauristolactam was assessed by high-resolution tomographic chest examination based on the presence of ground-glass opacities, areas of consolidation with thinning of the bronchial wall, and the presence of small centrilobular nodulations or pleural effusion. Cardiac involvement, a negative prognostic factor for EGPA, was assessed by electrocardiographic and echocardiographic.
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