Hibino Makoto, Kondo Tetsuri
Department of Respiratory Medicine, Shonan Fujisawa Tokushukai Hospital, Japan.
Intern Med. 2017 Oct 1;56(19):2649-2653. doi: 10.2169/internalmedicine.8761-16. Epub 2017 Sep 6.
A 77-year-old Japanese woman presented to an ophthalmologist with an erythematous swollen upper eyelid and diplopia which was initially diagnosed to be idiopathic dacryoadenitis on the basis of a histological evaluation of an orbital mass that was in remission following a 3.5-month period of systemic corticosteroid therapy. She subsequently developed respiratory symptoms, and was finally diagnosed with systemic granulomatosis with polyangiitis (GPA) based on the clinical and histological features. She was successfully treated with corticosteroids and azathioprine. Dacryoadenitis in the form of an orbital inflammatory pseudotumor may be an initial presenting feature of GPA, sometimes as the limited phenotype, and occasionally progressing to systemic disease.
一名77岁的日本女性因上睑红肿和复视就诊于眼科医生。最初，根据对眼眶肿物的组织学评估，该患者被诊断为特发性泪腺炎，在接受3.5个月的全身糖皮质激素治疗后病情缓解。随后她出现了呼吸道症状，最终根据临床和组织学特征被诊断为系统性肉芽肿性多血管炎（GPA）。她接受糖皮质激素和硫唑嘌呤治疗后病情得到成功控制。眼眶炎性假瘤形式的泪腺炎可能是GPA的首发表现，有时表现为局限性表型，偶尔会进展为全身性疾病。
