University of Kansas School of Medicine, Wichita, KS, USA.
Rheumatol Int. 2013 Jul;33(7):1865-6. doi: 10.1007/s00296-013-2677-4. Epub 2013 Feb 1.
IgG4-related systemic disease is an emerging disease process that manifests with a constellation of features, most commonly but not exclusive to swelling and tuberous growth in the lacrimal and salivary glands, potentially involving many other organ systems. This condition often prompts investigations into malignancy or needless radical surgical procedures. A 58-year-old male was presented to a rheumatologist after several biopsies were done that were suspicious for neoplasia, involving the lacrimal gland and lung. The diagnosis was confirmed when tissue from the lacrimal gland biopsy was reviewed with special stains for IgG4, performed at the Mayo Clinic. This patient is interesting because his disease included bilateral lacrimal glands--at different intervals, the submandibular glands, the lung, and the thyroid gland. His disease responded to immunosuppression. Literature has shown resolution of the tumors upon starting glucocorticoids or rituximab. Our patient was given a course of prednisone and methotrexate with normal follow-up CT chest and physical exam.
IgG4 相关系统性疾病是一种新兴的疾病过程，表现为一系列特征，最常见但不限于泪腺和唾液腺的肿胀和结节性生长，可能涉及许多其他器官系统。这种情况常常促使人们对恶性肿瘤或不必要的根治性手术进行调查。一名 58 岁男性因多次活检怀疑为肿瘤而被转介至风湿病医生处，这些活检涉及泪腺和肺部。当对泪腺活检组织进行特殊 IgG4 染色并在梅奥诊所进行复查时，确诊了该疾病。这名患者很有趣，因为他的疾病包括双侧泪腺——在不同的时间间隔内，还包括颌下腺、肺部和甲状腺。他的疾病对免疫抑制治疗有反应。文献表明，开始使用糖皮质激素或利妥昔单抗后肿瘤会消退。我们的患者接受了泼尼松和甲氨蝶呤治疗，随访胸部 CT 和体检均正常。
