National Reference Center for Hypereosinophilic Syndromes, CEREO, Suresnes, France.
Department of Internal Medicine, Bichat Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
Front Immunol. 2024 Apr 23;15:1379611. doi: 10.3389/fimmu.2024.1379611. eCollection 2024.
Eosinophils have widespread procoagulant effects. In daily practice, eosinophil-related cardiovascular toxicity consists of endomyocardial damage, eosinophilic vasculitis and arterial or venous thrombosis. Here we aim to report on the clinical features and treatment outcomes of patients with unexplained ophthalmic vascular manifestations and eosinophilia.
We conducted a retrospective, multicenter, observational study and a literature review of patients with eosinophilia (≥0.5 x10/L) and concomitant ophthalmic vascular manifestations independent of the underlying eosinophilic disease but with no alternative cause for ophthalmic manifestations.
Fifty-seven patients were included (20 from the observational study and 37 from the literature review). Ophthalmic vascular features were the initial manifestation of eosinophil-related disease in 34 (59%) patients and consisted of 29 central retinal artery occlusions, six branch retinal artery occlusions, five central retinal vein occlusions, two branch retinal vein occlusions, seven retinal vasculitides, two retinal vasospasms, 12 Purtscher's retinopathies, 13 anterior ischemic optic neuropathies and two posterior ischemic optic neuropathies. The median [IQR] absolute eosinophil count at onset of ophthalmic vascular manifestations was 3.5 [1.7-7.8] x10/L. Underlying eosinophil-related diseases included eosinophilic granulomatosis with polyangiitis (n=32), clonal hypereosinophilic syndrome (HES) (n=1), idiopathic HES (n=13), lymphocytic HES (n=2), adverse drug reactions (n=3), parasitosis (n=2), polyarteritis nodosa (n=1), IgG4-related disease (n=1), eosinophilic fasciitis (n=1) and primary sclerosing cholangitis (n=1). Other extra-ophthalmologic arterial or venous thromboses related to eosinophilia were reported in four (7%) and nine (16%) patients, respectively. Visual prognosis was poor: only eight (10%) patients achieved full recovery of ophthalmologic symptoms. After a median follow-up of 10.5 [1-18] months, one patient (3%) had a recurrence of an ophthalmic vascular manifestation, and three patients (10%) had a recurrence of other vascular symptoms (deep vein thrombosis in two and pulmonary embolism in one patient). At the time of recurrence, absolute eosinophil counts were above 0.5 x10/L in all cases (n=4).
This study broadens the spectrum of vascular manifestations associated with hypereosinophilia by adding ophthalmic vascular manifestations. In patients with ophthalmological vascular manifestations and hypereosinophilia, aggressive treatment of the underlying pathology (and normalization of blood count) should be implemented.
嗜酸性粒细胞具有广泛的促凝作用。在日常实践中，嗜酸性粒细胞相关的心血管毒性包括心肌损伤、嗜酸性粒细胞血管炎以及动脉或静脉血栓形成。本文旨在报告原因不明的眼部血管表现和嗜酸性粒细胞增多症患者的临床特征和治疗结局。
我们进行了一项回顾性、多中心、观察性研究，并对伴有嗜酸性粒细胞增多症（≥0.5 x10/L）和伴发性眼部血管表现的患者进行了文献复习，这些表现独立于潜在的嗜酸性粒细胞疾病，但没有眼部表现的其他病因。
共纳入 57 例患者（20 例来自观察性研究，37 例来自文献复习）。34 例（59%）患者以眼部血管表现为首发症状，包括 29 例视网膜中央动脉阻塞、6 例视网膜分支动脉阻塞、5 例视网膜中央静脉阻塞、2 例视网膜分支静脉阻塞、7 例视网膜血管炎、2 例视网膜血管痉挛、12 例 Purtscher 视网膜病变、13 例前部缺血性视神经病变和 2 例后部缺血性视神经病变。眼部血管表现发病时的嗜酸性粒细胞绝对计数中位数[四分位距]为 3.5[1.7-7.8]x10/L。潜在的嗜酸性粒细胞相关疾病包括嗜酸性肉芽肿伴多血管炎（n=32）、克隆性嗜酸性粒细胞增多症（HES）（n=1）、特发性 HES（n=13）、淋巴细胞性 HES（n=2）、药物不良反应（n=3）、寄生虫病（n=2）、结节性多动脉炎（n=1）、IgG4 相关疾病（n=1）、嗜酸性筋膜炎（n=1）和原发性硬化性胆管炎（n=1）。另外，4 例（7%）和 9 例（16%）患者分别报告了其他与嗜酸性粒细胞增多相关的眼部以外动脉或静脉血栓形成。视觉预后较差：仅有 8 例（10%）患者眼部症状完全恢复。中位随访 10.5[1-18]个月后，1 例患者（3%）复发眼部血管表现，3 例患者（10%）复发其他血管症状（2 例深静脉血栓形成和 1 例肺栓塞）。复发时，所有病例的嗜酸性粒细胞绝对计数均高于 0.5 x10/L（n=4）。
本研究通过添加眼部血管表现，拓宽了与高嗜酸性粒细胞血症相关的血管表现谱。对于眼部血管表现和高嗜酸性粒细胞血症的患者，应积极治疗潜在的病理（并使血细胞计数正常化）。
