具有侵袭性的喉IgG4相关性疾病一例北大核心CSCD

本文报道了1例喉IgG4相关性疾病的患者。患者因"声音嘶哑"发现"喉肿物",多次病理活检均提示为良性病变,然这与患者喉部影像提示"喉肿物侵犯突破甲状软骨板"的恶性征象明显不符。经手术完整切除喉肿物后病理最终诊断"喉IgG4相关性疾病"。明确诊断后于风湿免疫科行激素和环磷酰胺规律治疗,治疗随访未见喉IgG4相关性疾病复发,患者声嘶较术前改善,对治疗效果满意,随访8个月无不适。     

IgG4相关性疾病4例报告并文献回顾

目的 探讨IgG4相关性疾病的临床表现、病理学特征及影像学表现。 方法 分析4例IgG4相关性疾病的临床特征、组织病理学及影像学改变,并查阅相关文献进行总结。 结果 本组4例的平均年龄为55岁,男女比例为3∶1。分别发生于颌下腺2例、眼眶1例、颜面部1例。临床表现无显著特异性,均呈无痛渐进性肿胀。病理学改变为淋巴组织及纤维组织增生,其内有较多浆细胞浸润(IgG4阳性浆细胞>50个/HPF,IgG4阳性浆细胞>IgG阳性浆细胞的40%)。影像学上表现为病变区炎性改变。 结论 IgG4相关性疾病是目前临床较为少见的疾病,发病机制尚不明确。需结合临床表现、血清学检测、病理学特征及影像学表现做出最终诊断,进而行及时准确的治疗。     

Warthin's tumor associated with IgG4-related disease

Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory condition associated with elevated serum IgG4 levels and tissue infiltration by IgG4-expressing plasma cells. Several inflammatory conditions associated with IgG4-RD have been reported. Warthin's tumor is a benign parotid gland tumor consisting of oncocytic epithelial cells and lymphoid stroma containing lymphoid follicles with reactive germinal centers. This is the first report describing a case of Warthin's tumor with possible involvement of IgG4-RD. The patient was a 71-year-old man presenting with swollen right parotid and bilateral submandibular glands. He had a history of a Warthin's tumor of the left parotid gland, autoimmune pancreatitis, and an inflammatory abdominal aortic aneurysm. Laboratory tests revealed a high serum IgG4 level. Histological examination of the resected parotid gland showed a Warthin's tumor and a nodule showing severe lymphocytic infiltration containing many IgG4-positive plasma cells. This case shows the possible involvement of Warthin's tumor with IgG4-RD.     

IgG4-related disease with pseudotumor formation in the larynx

Mikulicz’s disease, an immunoglobulin G4-related disease (IgG4-RD) occurs frequently in the head and neck region but rarely in the larynx. We report a case of IgG4-RD with pseudotumor formation in the larynx. A 50-year-old man presented at our facility for a complete physical examination and diagnostic testing of a left arytenoid tumor. On examination, a large supraglottic mass was noted with signs of dyspnea. Movement disorder of the vocal fold was absent. The patient underwent surgery with general anesthesia to improve respiratory symptoms and a resected specimen was submitted for diagnosis. The pathology findings revealed lymph follicle formation, interstitial follicular fibrosis, angiogenesis, and inflammatory cell infiltration with plasmacytosis. Immunohistologic staining at high magnification revealed one hundred or more IgG4-positive plasma cells and fifty percent IgG4/IgG. In addition, obliterative phlebitis was observed. Medical history was positive for retroperitoneal fibrosis with serum IgG4 levels below the diagnostic criteria of IgG4-RD, but histological characteristics of IgG4-RD were met. The diagnosis of IgG4-RD was established, oral treatment with prednisolone (0.6 mg/kg/day) was initiated, and the tumor reduced in size. IgG4-RD may cause irreversible functional impairment. Early diagnosis and treatment are important in reducing the risk of permanent impairment of vocal fold mobility.     

IgG4-related disease presenting as otogenic skull base osteomyelitis

IgG4-related disease (IgG4-RD) is an emerging clinical disease entity characterized by tumefactive lesions at multiple sites with a dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells. Although almost any organ can be affected, IgG4-RD is most likely to involve the submandibular, lacrimal, or parotid glands in the head and neck region. However, skull base involvement presenting as otogenic skull base osteomyelitis (SBO) is rare. We encountered a 70-year-old male with IgG4-RD presenting primarily with severe otalgia and otorrhea. He had uncontrolled diabetes mellitus and showed clinical manifestations of otogenic SBO. Tissue immunostaining revealed typical features of increased IgG4-positive plasma cells, and hematological examination showed elevated serum IgG4 concentrations. Treatment with corticosteroids significantly improved well-being and partially resolved the lesion based on computed tomography (CT) scan.     

A novel concept of Mikulicz's disease as IgG4-related disease

Since Morgan's report in 1953, Mikulicz's disease (MD) has been considered part of primary Sjögren's syndrome (SS). However, MD has a unique presentation, including persistent swelling of the lacrimal and salivary glands, and is characterized by good responsiveness to glucocorticoids, leading to recovery of gland function. Recently, it has been revealed that MD patients show elevated serum immunoglobulin G4 (IgG4) levels and prominent infiltration of IgG4-positive plasmacytes. The complications of MD include autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel's thyroiditis, all of which show IgG4 involvement in their pathogenesis. Thus, MD is a systemic “IgG4-related disease.” In addition, recent analyses have revealed that Küttner's tumor (KT), a chronic sclerosing sialadenitis that presents with asymmetrical firm swelling of the submandibular glands, is also associated with prominent infiltration of IgG4-positive plasmacytes. MD and KT differ from SS and are thought to be singular systemic IgG4-related plasmacytic diseases. Here we discuss the results of recent studies and provide an overview of MD as an IgG4-related disease.     

A solely ear-involved IgG4-related sclerosing disease with two-years following-up

IntroductionImmunoglobulin G4-related sclerosing disease (IgG4-RSD) is a chronic fibro-inflammatory disease involving systemic multi-organ lesions, such as the salivary and lacrimal glands, lymph nodes and pancreas. The diagnosis of this disease is reliant upon clinical manifestations, laboratory tests, histopathologic results and radiological data. Some studies have found that IgG4-RSD has otological manifestations, whereas there were few studies introducing the diagnosis, therapy and long-term follow-up results of solely otological IgG4-RSD.Case summaryHere, we report a case of IgG4-RSD involving the ear alone. A female presenting with otalgia and hearing loss underwent surgery, without hormone therapy. The pathological diagnosis was otological IgG4-RSD and no clinical or radiological signs of recurrence were observed at seven and twenty-four months follow-up.DiscussionThis case indicates that IgG4-RSD can invade the ear only, and that surgical therapy without hormone therapy is efficient for solely IgG4-RSD. Pathological results are crucial for diagnosis.