Department of Ophthalmology, Mie University Graduate School of Medicine, Mie, Japan.
Department of Ophthalmology, Kinan Hospital, Mie, Japan.
Medicine (Baltimore). 2022 Jul 22;101(29):e29611. doi: 10.1097/MD.0000000000029611.
IgG4-related diseases (IgG4-RDs) are known to disrupt the functioning of multiple organs and are usually associated with mass lesions. Periaortitis, an inflammation of the adventitia and tissues surrounding the aorta, is an example of an IgG4-RD. In ophthalmology, an enlargement of the lacrimal gland is a well-known IgG4-RD, and scleritis has also been reported to be an IgG4-RD although it is rare. We report our findings in a case with periaortitis and posterior scleritis that were present at the same time, and they responded well to systemic steroid therapy.
A 79-year-old man with dementia and Lewy bodies was referred to our hospital because of uveitis in both eyes that did not respond to topical steroid therapy.
We found anterior scleritis in the right eye and uveitis with shallow anterior chambers in both eyes. B-mode echography showed choroidal detachments (CDs) and a T sign in the right eye. The CDs were assumed to have progressed to the posterior scleritis which then caused the severe vision reduction. The patient was referred to the Internal Medicine Department because the systemic inflammatory disease was suspected due to the high levels of C-reactive protein (CRP) and the fast erythrocyte sedimentation rate. Systemic CT scans showed periaortitis only at the lumbar region. Because of the high levels of IgG4, the patient was diagnosed with IgG4-RD.
The patient received intravenous and oral steroid therapy. The first 125 mg of methylprednisolone (mPSL) for 3 days was intravenous, after which it was switched to oral prednisolone (PSL) therapy and the dosage was gradually reduced.
The posterior scleritis and periaortitis responded well to the systemic steroid therapy. One year and a half after the onset of the disease, the patient is still taking 5 mg of PSL.
Scleritis with multiple CDs and periaortitis were strongly suspected to be due to IgG4-RD although no definitive diagnosis was made by biopsy of the lesions. Clinicians should be aware that IgG4-RD should be considered as one of the causes of posterior scleritis.
IgG4 相关疾病（IgG4-RD）已知会影响多个器官的功能，并且通常与肿块病变有关。大动脉炎是主动脉外膜和周围组织的炎症，是 IgG4-RD 的一个例子。在眼科中，泪腺肿大是一种众所周知的 IgG4-RD，而巩膜炎也被报道为 IgG4-RD，尽管很少见。我们报告了一例同时存在大动脉炎和后部巩膜炎的病例，这些病例对全身类固醇治疗反应良好。
一名 79 岁男性，患有痴呆症和路易体，因双眼葡萄膜炎对局部类固醇治疗无反应而被转至我院。
我们发现右眼有前部巩膜炎和双眼浅前房葡萄膜炎。B 型超声显示右眼脉络膜脱离（CD）和 T 征。CD 被认为进展为后部巩膜炎，导致严重视力下降。由于 C 反应蛋白（CRP）水平高和红细胞沉降率快，怀疑患者患有全身性炎症性疾病，因此将其转至内科。全身 CT 扫描仅在腰椎区域显示大动脉炎。由于 IgG4 水平高，患者被诊断为 IgG4-RD。
患者接受静脉和口服类固醇治疗。前 3 天静脉注射 125mg 甲泼尼龙（mPSL），然后改为口服泼尼松龙（PSL）治疗，并逐渐减少剂量。
后部巩膜炎和大动脉炎对全身类固醇治疗反应良好。疾病发作 1 年半后，患者仍服用 5mg PSL。
尽管未通过病变活检做出明确诊断，但强烈怀疑多 CD 后部巩膜炎和大动脉炎是由 IgG4-RD 引起的。临床医生应意识到 IgG4-RD 应被视为后部巩膜炎的原因之一。
