Adachi Sei, Oshikata Chiyako, Kaneko Takeshi, Tsurikisawa Naomi
Department of Allergy and Respirology, Hiratsuka City Hospital, 1-19-1 Minamihara, Hiratsuka, Kanagawa, 254-0065, Japan.
Department of Pulmonology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, Kanagawa, 236-0004, Japan.
Allergy Asthma Clin Immunol. 2022 Feb 26;18(1):18. doi: 10.1186/s13223-021-00639-x.
Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by a necrotizing vasculitis with tissue and peripheral blood eosinophilia affecting small and medium-sized arteries, capillaries, and veins. Venous thromboembolic events are uncommon in EGPA. Moreover, there are only a few reported cases of EGPA complicated by pulmonary embolism or infarction.
We report the case of a 43-year-old woman with eosinophilic granulomatosis with polyangiitis and acute respiratory and heart failure due to bilateral pulmonary artery thrombosis and left femoral vein thrombosis 12 years after disease onset. She also had cardiac involvement (myocarditis, pericardial effusion, and diastolic dysfunction), gastrointestinal symptoms, and peripheral neuropathy. The condition was refractory to treatment with systemic corticosteroids, intravenous cyclophosphamide, and mepolizumab, but the thrombosis and associated acute cardiac failure, as well as the cardiac and gastrointestinal symptoms and multiple polyneuropathy, improved after a switch to rituximab. However, the heart failure did not improve sufficiently and the patient continued to need inhaled oxygen at 1 L/min and asthma exacerbations occurred. We then swapped the patient's mepolizumab treatment for dupilumab. Not only did she have no further asthma attacks after switching to dupilumab, but also her vasculitis symptoms improved. Oxygen therapy was discontinued as the heart failure improved 5 months after starting the dupilumab.
This may be the first case report of the successful treatment by rituximab of pulmonary thromboembolism associated with EGPA. In addition, in this patient, treatment with dupilumab was effective not only for the asthma symptoms but also for the symptoms of vasculitis and heart failure.
嗜酸性肉芽肿性多血管炎（EGPA）的特征是坏死性血管炎，伴有组织和外周血嗜酸性粒细胞增多，累及中小动脉、毛细血管和静脉。静脉血栓栓塞事件在EGPA中并不常见。此外，仅有少数EGPA合并肺栓塞或梗死的病例报道。
我们报告了一例43岁女性，患有嗜酸性肉芽肿性多血管炎，在疾病发作12年后因双侧肺动脉血栓形成和左股静脉血栓形成出现急性呼吸和心力衰竭。她还存在心脏受累（心肌炎、心包积液和舒张功能障碍）、胃肠道症状和周围神经病变。该患者对全身用糖皮质激素、静脉注射环磷酰胺和美泊利单抗治疗无效，但在换用利妥昔单抗后，血栓形成及相关的急性心力衰竭、心脏和胃肠道症状以及多发性多发性神经病均有所改善。然而，心力衰竭改善不充分，患者仍需1L/min的吸氧，且哮喘发作。然后我们将患者的美泊利单抗治疗换为度普利尤单抗。换用度普利尤单抗后，她不仅没有再发生哮喘发作，而且血管炎症状也有所改善。开始使用度普利尤单抗5个月后，随着心力衰竭改善，停止了氧疗。
这可能是首例关于利妥昔单抗成功治疗与EGPA相关的肺血栓栓塞的病例报告。此外，在该患者中，度普利尤单抗治疗不仅对哮喘症状有效，而且对血管炎和心力衰竭症状也有效。
