Aguilar González Marina, Marín Payá Emma, García Gil Romana, Feliciano Sánchez Anselmo, Gómez-Lechón Quirós Luis, España Gregori Enrique
Department of Ophthalmology, Hospital Universitari i Politecnic La Fe, Valencia, Spain.
Department of Medical Retina, Hospital Universitari i Politecnic La Fe, Valencia, Spain.
Eur J Ophthalmol. 2023 Jul;33(4):NP105-NP110. doi: 10.1177/11206721221123779. Epub 2022 Sep 1.
A case of a 48-year-old male with a nonparaneoplasic autoinmune retinopathy (nPAIR) due to chronic graft versus host disease (GVHD) after an allogenic stem cell transplantation (ASCT) is described.
The patient developed a bilateral rapidly progressive loss of visual acuity with bilateral optic disc edema and bilateral cystoid macular edema (CME) in the funduscopy, a ring scotoma in the visual field (VF) and photoreceptors dysfunction in the electroretinogram (ERG) 210 days after the ASCT. After ruling out other causes, the suspicion of autoimmune retinopathy (AIR) led to the study of antirecoverin antibodies which resulted positive. The exclusion of neoplasia discarded diagnosis of paraneoplasic autoinmune retinopathy (PAIR) and the temporal relationship with BMT led to the diagnosis of nonparaneoplasic autoinmune retinopathy (nPAIR) due to chronic graft versus host disease (GVHD). Oral corticosteroids led to resolution of the CME.
Diagnosis of AIR requires a high index of suspicion based on the typical findings on visual field, optical coherence tomography (OCT) and ERG, which force requesting antirecoverin antibodies. However, diagnosis is often delayed because of the need to exclude other causes. Knowing typical symptoms and signs in for a quick action is important because an earlier diagnosis and treatment will improve visual prognosis since the loss of vision already established is irrecoverable. To our knowledge, this is the first reported case in the literature of nPAIR with CME and optic disc edema due to GVHS after ASCT.
本文描述了一例48岁男性患者,在接受异基因干细胞移植(ASCT)后,因慢性移植物抗宿主病(GVHD)引发非副肿瘤性自身免疫性视网膜病变(nPAIR)。
患者在ASCT后210天出现双侧视力迅速进行性丧失,眼底检查显示双侧视盘水肿和双侧黄斑囊样水肿(CME),视野检查出现环形暗点,视网膜电图(ERG)提示光感受器功能障碍。排除其他病因后,怀疑自身免疫性视网膜病变(AIR),遂检测抗 recoverin 抗体,结果呈阳性。排除肿瘤后,可排除副肿瘤性自身免疫性视网膜病变(PAIR),鉴于其与骨髓移植的时间关系,诊断为慢性移植物抗宿主病(GVHD)所致的非副肿瘤性自身免疫性视网膜病变(nPAIR)。口服糖皮质激素使CME消退。
基于视野、光学相干断层扫描(OCT)和ERG的典型表现,高度怀疑AIR时需检测抗 recoverin 抗体以明确诊断。然而,由于需要排除其他病因,诊断往往延迟。了解典型症状和体征以便迅速采取行动很重要,因为早期诊断和治疗将改善视力预后,因为已经发生的视力丧失是不可恢复的。据我们所知,这是文献中首次报道的ASCT后因GVHS导致nPAIR伴CME和视盘水肿的病例。