Familial clustering of hypereosinophilic diseases treated with mepolizumab: a case report from Japan.

Summary: We describe a female diagnosed with non-allergic asthma. On March 24, 2016, examination of the skin-biopsy specimen revealed dense eosinophilic infiltration, and the Fip-1-like 1-platelet-derived growth factor receptor a fusion gene in peripheral blood mononuclear cells was negative. She was diagnosed with idiopathic hypereosinophilic syndrome. She was treated with intravenous methylprednisolone (MPSL), and subsequent oral MPSL. Then, she started to receive a monthly mepolizumab in June 2016, and successfully withdrew from daily use of oral MPSL. The patient has a mother diagnosed with non-allergic asthma. In February 2005, she was diagnosed with eosinophilic granulomatosis with polyangitis because of elevated antineutrophil myeloperoxidase antibodies, and the skin-biopsy specimen findings. She started to receive a monthly mepolizumab in June 2016. Corticosteroid therapy was successfully withdrawn. To our knowledge, this is the first case report suggesting mepolizumab may be a useful treatment for familial clustering of hypereosinophilic diseases.

Prospective Open-Label Study of 48-Week Subcutaneous Administration of Mepolizumab in Japanese Patients With Severe Eosinophilic Asthma.

BACKGROUND: The long-term efficacy and safety of mepolizumab in patients with severe eosinophilic asthma has been evaluated in large-scale double-blind placebo-controlled trials. However, a prospective open-label trial of long-term subcutaneous administration of mepolizumab has not been performed in Japanese patients with severe eosinophilic asthma. METHODS: This study was a prospective, 48-week, open-label trial in 32 Japanese patients with severe eosinophilic asthma who received subcutaneous mepolizumab 100 mg every 4 weeks. Nine patients required oral corticosteroids daily despite receiving high-dose inhaled corticosteroids. Six patients had aspirin-exacerbated respiratory disease. RESULTS: All patients took mepolizumab throughout the study period. No patients experienced adverse events during the treatment. None of the patients experienced asthma exacerbations during the trial. In fact, forced expiratory volume in 1 second increased significantly at 24 weeks (P<.01) and at 48 weeks (P<.05). The peripheral blood eosinophil count in peripheral blood decreased after the first administration of mepolizumab in all patients and remained low until week 48. After starting mepolizumab, all oral corticosteroid-dependent asthmatics successfully withdrew corticosteroids without exacerbations and experienced a sustained reduction in peripheral blood eosinophil count. Blood levels of thymus and activation-regulated chemokine and IgE remained unchanged after 48 weeks of therapy with mepolizumab. CONCLUSION: This first prospective open-label pilot study in Japan demonstrated the long-term efficacy and safety of mepolizumab in patients with severe eosinophilic asthma.

Favorable clinical efficacy of mepolizumab on the upper and lower airways in severe eosinophilic asthma: a 48-week pilot study.

Summary: Objective. Assessing efficacy of mepolizumab on the upper and lower airways in severe eosinophilic asthma patients. Patients and methods. This study was a 48-week prospective open-label analysis of mepolizumab in 11 asthmatics with chronic rhinosinusitis (CRS). It was administered every 4 weeks. Six patients were aspirin-exacerbated respiratory disease (AERD). Results. Blood eosinophil count was reduced after the first administration, and was continued until 48 weeks. The Sino-Nasal Outcome Test scores, the Lund-MacKay CT scoring, and forced expiratory volume in 1 second were improved. Symptom scores of anosmia and nasal congestion were not improved in the patients with AERD. All oral corticosteroid-dependent patients successfully withdrew from corticosteroids. Conclusions. This pilot study showed mepolizumab improved nasal symptoms and lung function in severe eosinophilic asthma patients with CRS, suggesting efficacy of mepolizumab on the upper and lower airway symptoms in eosinophilic asthma.

Long-term treatment with anti-interleukin 5 antibodies in a patient with chronic eosinophilic pneumonia

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Prospective open-label study of 48-week subcutaneous administration of Mepolizumab in japanese patients with severe eosinophilic asthma

Background: The long-term efficacy and safety of mepolizumab in patients with severe eosinophilic asthma has been evaluated in large-scale double-blind placebo-controlled trials. However, a prospective open-label trial of long-term subcutaneous administration of mepolizumab has not been performed in Japanese patients with severe eosinophilic asthma. Methods: This study was a prospective, 48-week, open-label trial in 32 Japanese patients with severe eosinophilic asthma who received subcutaneous mepolizumab 100 mg every 4 weeks. Nine patients required oral corticosteroids daily despite receiving high-dose inhaled corticosteroids. Six patients had aspirin-exacerbated respiratory disease. Results: All patients took mepolizumab throughout the study period. No patients experienced adverse events during the treatment. None of the patients experienced asthma exacerbations during the trial. In fact, forced expiratory volume in 1 second increased significantly at 24 weeks (P<.01) and at 48 weeks (P<.05). The peripheral blood eosinophil count in peripheral blood decreased after the first administration of mepolizumab in all patients and remained low until week 48. After starting mepolizumab, all oral corticosteroid-dependent asthmatics successfully withdrew corticosteroids without exacerbations and experienced a sustained reduction in peripheral blood eosinophil count. Blood levels of thymus and activation-regulated chemokine and IgE remained unchanged after 48 weeks of therapy with mepolizumab. Conclusion: This first prospective open-label pilot study in Japan demonstrated the long-term efficacy and safety of mepolizumab in patients with severe eosinophilic asthma

Introducción: La eficacia y seguridad a largo plazo de mepolizumab se ha evaluado mediante grandes estudios doble-ciego controlados con placebo. Sin embargo, no hay estudios prospectivos abiertos a largo plazo que analicen la administración de mepolizumab en pacientes japoneses con asma eosinofílica grave. Métodos: Es un estudio prospectivo, abierto, de 48 semanas de duración en 32 pacientes japoneses con asma eosinofílica grave y que recibieron la administración subcutánea de 100 mg de mepolizumab cada 4 semanas. Nueve pacientes necesitaban esteroides orales a diario a pesar del uso de altas dosis de corticoides inhalados. Seis pacientes tenían intolerancia respiratoria a Aspirina. Resultados: Ningún paciente fue retirado del estudio ni tuvo efectos adversos durante el tratamiento. Ningún paciente tuvo exacerbaciones asmáticas durante el periodo del estudio. El volumen expirado máximo en el primer segundo aumentó de forma significativa en la semana 24 (p < 0,01) y en la semana 48 (p < 0,05). El número de eosinófilos en sangre periférica se redujo tras la primera administración de mepolizumab en todos los pacientes y continuó hasta la semana 48. Después del comienzo de la administración de mepolizumab a todos los pacientes con necesidad de esteroides orales se les retiraron sin que tuvieran posteriormente exacerbaciones y en paralelo a la disminución de eosinófilos en sangre periférica. Los niveles séricos de la quemoquina tímica de regulación y activación ni de IgE cambiaron en las 48 semanas del estudio. Conclusión: Es el primer estudio piloto abierto y a largo plazo realizado en Japón que muestra la eficacia y seguridad de mepolizumab en pacientes con asma eosinofílica grave