Rootman J, McCarthy M, White V, Harris G, Kennerdell J
Department of Pathology, Vancouver General Hospital, British Columbia, Canada.
Ophthalmology. 1994 Mar;101(3):570-84. doi: 10.1016/s0161-6420(94)31298-x.
Idiopathic sclerosing inflammation of the orbit is a poorly delineated, fibrosing, immune-mediated entity resulting in significant ocular disability. To characterize this process and propose more specific and effective therapy, clinical and pathologic findings in 16 cases are reviewed.
The clinical records of 16 patients with biopsy-proven disease were retrospectively reviewed to determine demographic and clinical features, radiologic features, course, management, and outcome. These findings were correlated with pathologic features to describe this unique entity. Immunohistologic characteristics were compared with those of a clinically and histopathologically similar process, retroperitoneal fibrosis.
The study included 11 male and 5 female patients, ranging in age from 8 to 81 years. Disease onset was usually unilateral (14/16) and chronic (11/15), with two distinct anatomic presentations, lacrimal (11/16) and apical (3/16), characterized by infiltration (15/16), mass effect (12/16), and visual loss (3/16). The most common signs and symptoms were dull pain (13/16), proptosis (11/16), mild inflammation (11/16), restricted motility (9/16), swelling (9/16), and diplopia (8/16). Two features, a sparse, chronic inflammatory infiltrate, the immunopathologic characteristics of which suggested a cell-mediated process, and a desmoplastic stroma of early onset, dominated the pathologic picture. Treatment with corticosteroids (11/16), radiotherapy for steroid failures (8/11), and observation alone (3/16) was inadequate, resulting in blindness in 3/16 cases, restricted movement in 10/16, and complete resolution in only 2/16 patients.
Idiopathic sclerosing inflammation of the orbit is a unique clinicopathologic entity, similar to retroperitoneal fibrosis, that is characterized by primary, chronic, and immunologically mediated fibrosis, poor response to corticosteroid treatment or radiotherapy, and frequent visual disability. Early and aggressive immunosuppressive therapy is recommended.
眼眶特发性硬化性炎症是一种定义不清的、纤维化的、免疫介导的疾病，可导致严重的眼部功能障碍。为了明确这一疾病过程并提出更具针对性和有效性的治疗方法，我们回顾了16例患者的临床和病理表现。
回顾性分析16例经活检证实患有该病患者的临床记录，以确定其人口统计学和临床特征、放射学特征、病程、治疗和预后。将这些结果与病理特征相关联，以描述这一独特疾病。将免疫组织学特征与临床和组织病理学上相似的疾病——腹膜后纤维化进行比较。
研究包括11例男性和5例女性患者，年龄范围为8至81岁。疾病通常单侧起病（14/16）且病程呈慢性（11/15），有两种不同的解剖学表现，泪腺型（11/16）和眶尖型（3/16），其特征为浸润（15/16）、占位效应（12/16）和视力丧失（3/16）。最常见的体征和症状为钝痛（13/16）、眼球突出（11/16）、轻度炎症（11/16）、眼球运动受限（9/16）、肿胀（9/16）和复视（8/16）。病理表现主要有两个特征，一是稀疏的慢性炎症浸润，其免疫病理特征提示为细胞介导过程；二是早期出现的促纤维增生性间质。使用皮质类固醇治疗（11/16）、对类固醇治疗无效者进行放疗（8/11）以及单纯观察（3/16）均效果不佳，导致16例中有3例失明，10例眼球运动受限，仅2例完全缓解。
眼眶特发性硬化性炎症是一种独特的临床病理实体，与腹膜后纤维化相似，其特征为原发性、慢性和免疫介导的纤维化，对皮质类固醇治疗或放疗反应不佳，且常导致视力残疾。建议早期积极进行免疫抑制治疗。
