Improvement of peripheral neuropathy in a patient with antineutrophil cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis by additional mepolizumab

Abstract

Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a vasculitis characterized by abnormally high eosinophils and frequent peripheral neuropathy. Mepolizumab is an approved therapy for EGPA, but its efficacy against peripheral neuropathy remains unknown. Case presentation A 41-year-old woman was admitted in the hospital with dyspnea and neuropathy. Ground glass opacity and infiltrative shadow in the bilateral lungs were evident on chest computed tomography images. Eosinophils were increased in serum, in bronchoalveolar lavage fluid (BALF), and in transbronchial lung biopsy, and bacteria were not detected in BALF. EGPA resulting in severe eosinophilic asthma, sinusitis, pulmonary infiltrates, and peripheral neuropathy was diagnosed. Prednisolone (50 mg/day) caused remission of eosinophilic pneumonia and sinusitis, but not peripheral neuropathy. During prednisolone tapering (7 mg/day, 10 months after treatment), eosinophils were increased, and peripheral neuropathy relapsed. The humanized anti-IL-5 antibody mepolizumab (300 mg) was initially administered, followed by prednisolone. Mepolizumab caused sustained peripheral neuropathy remission and effective prednisolone tapering. Conclusions Introduction of mepolizumab combined with prednisolone may improve peripheral neuropathy.

Severe Mononeuritis Multiplex in Eosinophilic Granulomatous Polyangiitis: A Case Report

“…Whereas when diagnosed quickly and treated appropriately, prognosis is much improved, with a 78-month survival rate of about 70-90% [ 7 ]. Mepolizumab has been effectively used in combination with prednisolone to treat peripheral neuropathy associated with EGPA and most recently, add-on low-dose mepolizumab showed good results for long-lasting neuropathy complications from EGPA [ 8 ].…”

Mononeuritis múltiple como manifestación inicial en vasculitis de pequeños vasos: un reporte de caso en México

“…4 Finalmente, la remisión dependerá del tratamiento inmunosupresor temprano, lo cual reduce notablemente la posibilidad de discapacidad a largo plazo. 9…”

“…De igual manera, esta enfermedad se presenta con mayor frecuencia en el sexo masculino, entre la quinta y sexta década de la vida, en pacientes con un promedio de edad de 50 años. [2][3][4][5][6][7][8][9] Cuando las vasculitis se asocian con anticuerpos anticitoplasma del neutrófilo (ANCA), se caracterizan por inflamación celular infiltrativa que causa necrosis de los vasos sanguíneos. Estos ANCA se detectan en dos tercios de los pacientes, comúnmente en los de mayor afectación sistémica y en general presentan un patrón perinuclear subtipo anti-mieloperoxidasa (p-ANCA-MPO) que puede contribuir en la patogenia e implicar así mecanismos autoinmunes.…”

“…Estos ANCA se detectan en dos tercios de los pacientes, comúnmente en los de mayor afectación sistémica y en general presentan un patrón perinuclear subtipo anti-mieloperoxidasa (p-ANCA-MPO) que puede contribuir en la patogenia e implicar así mecanismos autoinmunes. 9 Los mecanismos patogénicos subyacentes de la neuropatía vasculítica aún no se han dilucidado por completo. Presuntamente, la interacción de los ANCA, los granulocitos de neutrófilos activados, el sistema del complemento y las células endoteliales podrían provocar inflamación y estrechamiento luminal de la pared vascular de las pequeñas arterias epineurales, lo que daría lugar a lesiones isquémicas del nervio periférico y la pérdida de axones sensoriales y motores.…”

Eosinophilic Granulomatosis with Polyangiitis: Latest Findings and Updated Treatment Recommendations

“…Several attempts have been made to treat organ- or life-threatening manifestations of EGPA with mepolizumab, such as cardiomyopathy and gastrointestinal involvement [ 19 , 94 ], the efficacy of which is yet to be determined. However, a growing number of case reports and case series have shown that symptoms related to peripheral neuropathy were ameliorated and nerve conduction velocity tests improved with mepolizumab [ 95 , 96 , 97 ]. This appears promising, but the supporting evidence is currently insufficient.…”