Lu Cheng-Wei, Liu Xiu-Fen, Luan Ying, Lu Cheng-Bo, Zhou Dan-Dan, Guo Li-Min, Sun Ya-Bin, Chen Sheng-Nan, Wu Yun-Long, Hao Ji-Long
Department of Ophthalmology, The First Hospital of Jilin University, Changchun, Jilin 130021, P.R. China.
Department of Cardiology, The First Hospital of Jiamusi University, Jiamusi, Heilongjiang 154002, P.R. China.
Exp Ther Med. 2017 Jun;13(6):3337-3340. doi: 10.3892/etm.2017.4408. Epub 2017 Apr 28.
Granulomatosis with polyangiitis (GPA) is a systemic form of vasculitis that involves small to medium sized vessels and is associated with high morbidity and mortality. GPA presents a continuous and difficult clinical diagnostic concern, due to the rarity of the disease and the diversity of the manifestations. This case report discusses the unusual symptoms presented by a particular patient, discusses these manifestations and explains how the final diagnosis was identified as GPA. A 40-year old Chinese woman was initially referred to the present institution for a progressive worsening pain, redness and gradual decrease in visual acuity in the eyes over the past 7-year period. Previous therapeutic interventions included noncompliant topical and intravenous dexamethasone for 6 years. A pre-operative examination conducted in a differing hospital to search for the presence of an orbital mass, resulted in the identification of an asymptomatic space-occupying lesion in the right middle lung, which was surgically removed in March 2015. A total of four weeks later, surgery was then applied to remove a left orbital mass, in the same hospital. A total of three months later, the patient was diagnosed with peripheral ulcerative keratitis associated with GPA, at the present institution. The corneal lesions were then treated bilaterally with cryotherapy and oral prednisone and cyclophosphamide were administered. Following surgery, the condition of the eyes appeared to be stable. A total of seven months later, the redness and pain of the right eye recurred, followed by a deep lamellar keratoplasty for the treatment of necrotizing scleritis. The condition of the two eyes was subsequently observed to be stable during the nine month follow-up. The present case study reviews various points to consider in a rare, complicated and potentially blinding case of GPA. GPA must therefore be considered in the differential diagnosis of further inflammatory conditions and tumors. Early diagnosis and an appropriate interdisciplinary approach to management, are required to decrease recurrence and morbidity in patients with GPA-mediated inflammatory ocular disease.
肉芽肿性多血管炎（GPA）是一种累及中小血管的系统性血管炎，与高发病率和死亡率相关。由于该疾病罕见且表现多样，GPA一直是临床诊断中持续面临的难题。本病例报告讨论了一名特殊患者出现的不寻常症状，阐述了这些表现，并解释了最终如何确诊为GPA。一名40岁的中国女性最初因过去7年中眼部疼痛逐渐加重、发红以及视力逐渐下降而被转诊至本院。此前的治疗干预包括6年不规范的局部及静脉注射地塞米松。在另一家医院进行术前检查以寻找眼眶肿物，结果发现右中肺有一个无症状占位性病变，于2015年3月手术切除。四周后，在同一家医院又进行手术切除左眼眶肿物。三个月后，患者在本院被诊断为与GPA相关的周边溃疡性角膜炎。随后对角膜病变进行双侧冷冻治疗，并给予口服泼尼松和环磷酰胺。手术后，眼部情况似乎稳定。七个月后，右眼再次出现发红和疼痛，随后进行了深板层角膜移植术以治疗坏死性巩膜炎。在九个月的随访期间，观察到双眼情况稳定。本病例研究回顾了在GPA这种罕见、复杂且可能致盲的病例中需要考虑的各个要点。因此，在进一步的炎症性疾病和肿瘤的鉴别诊断中必须考虑GPA。需要早期诊断和适当的多学科管理方法，以降低GPA介导的炎症性眼病患者的复发率和发病率。
