Song Brian H, Baiyee Daniel, Liang Jonathan
Department of Head and Neck Surgery, Kaiser Permanente Oakland Medical Center, Oakland, California, USA.
Allergy Rhinol (Providence). 2015 Jan;6(3):151-7. doi: 10.2500/ar.2015.6.0136.
Immunoglobulin G4 (IgG4) related sclerosing disease (rSD) is a new disease entity, first described in 2001, that involves autoimmune pancreatitis. Considered a systemic disease with lesions described in multiple organ systems, IgG4-rSD that affects the sinonasal region is rare. Our goal was to highlight the sinonasal presentation of this unique disease and to review previously reported adult cases from 2003 to 2014.
Case report (a 72-year-old man who presented with left exophthalmos, periorbital pain, and epiphora) and review of the literature.
Radiographic workup with computed tomography and magnetic resonance imaging demonstrated a left sinonasal mass that involved the left maxillary and ethmoid sinuses, with surrounding bony destruction and orbital invasion. Nasal endoscopy demonstrated a fibrous lesion emanating in the middle meatus, with surrounding mucosal inflammation. The patient underwent an endoscopic biopsy, medial maxillectomy, and ethmoidectomy with tumor debulking. Pathology demonstrated inflamed respiratory mucosa with dense lymphoplasmacytic infiltrate and fibrosis; flow cytometry demonstrated no malignant cell populations; immunophenotyping demonstrated multiple foci of IgG4 cells. Plasma IgG4 was elevated in the setting of normal total IgG. The patient was treated with postoperative systemic and topical corticosteroids. Surveillance imaging studies and nasal endoscopy demonstrated disease resolution without recurrence.
Sinonasal IgG4-rSD is a rare disease that can present with bony and soft-tissue invasion. This was an exceptional case, with osseous involvement and orbital invasion. Immunohistologic workup is essential for diagnosis. It is important to differentiate this disease from sinonasal tumors. Treatment includes corticosteroids and surgical debulking. Sinonasal IgG4-rSD represents an emerging disease that may present challenges for future rhinologists.
免疫球蛋白G4（IgG4）相关硬化性疾病（rSD）是一种新的疾病实体，于2001年首次被描述，涉及自身免疫性胰腺炎。IgG4-rSD被认为是一种全身性疾病，其病变见于多个器官系统，累及鼻窦区域的IgG4-rSD较为罕见。我们的目的是强调这种独特疾病的鼻窦表现，并回顾2003年至2014年之前报道的成人病例。
病例报告（一名72岁男性，表现为左眼突出、眶周疼痛和溢泪）及文献复习。
计算机断层扫描和磁共振成像的影像学检查显示左侧鼻窦肿物，累及左上颌窦和筛窦，伴有周围骨质破坏和眼眶侵犯。鼻内镜检查显示中鼻道有一纤维性病变，周围黏膜有炎症。患者接受了内镜活检、上颌骨内侧切除术和筛窦切除术及肿瘤减容手术。病理显示炎症性呼吸黏膜，有密集的淋巴浆细胞浸润和纤维化；流式细胞术显示无恶性细胞群；免疫表型分析显示多个IgG4细胞灶。在总IgG正常的情况下，血浆IgG4升高。患者术后接受全身和局部皮质类固醇治疗。监测影像学检查和鼻内镜检查显示疾病消退，无复发。
鼻窦IgG4-rSD是一种罕见疾病，可表现为骨质和软组织侵犯。这是一例特殊病例，伴有骨质受累和眼眶侵犯。免疫组织学检查对诊断至关重要。将这种疾病与鼻窦肿瘤相鉴别很重要。治疗包括皮质类固醇和手术减容。鼻窦IgG4-rSD是一种新出现的疾病，可能给未来的鼻科医生带来挑战。
