Zhang Hui, Shi Ju-hong, Feng Rui-e, Tian Xin-lun, Xu Zuo-Jun, Xun Wen-bing, Liu Hong-rui, Liu Tong-hua
Department of Pathology, Chinese Academy of Medical Sciences, Beijing, China.
Zhonghua Jie He He Hu Xi Za Zhi. 2012 Oct;35(10):747-51.
To observe the immunohistochemical staining of IgG4 in nonspecific interstitial pneumonia (NSIP) and to study the clinicopathological features of IgG4-related NSIP.
Retrospective analysis was carried out for 32 patients with NSIP who had been admitted into Peking Union Medical College Hospital from November 2002 to October 2010. The diagnosis of NSIP was established by surgical lung biopsy and all specimens were fixed in neutral formalin and embedded in paraffin. Sections were cut for HE and immunohistochemical stain. According to the diagnostic criteria for IgG4-related disease, 4 cases were confirmed to be IgG4-related NSIP. The clinicopathological features including clinical history, laboratory examination, and pathologic evaluation were studied.
The 4 patients with IgG4-related NSIP included 1 man and 3 women, with a median age of 48 years (range, 44 - 56 years). The presenting symptoms were dry cough or shortness of breath. One patient (1/4, 25.0%) was found to have a positive autoantibody but no cases showed positive RF in serum. The histological finding of the 4 cases was characterized by inflammatory cell infiltration in interstitium with fibrosis, and 1 case showed obliterative arteritis. The numbers of IgG4-positive plasma cells in the 4 cases were 42/hpf, 22/hpf, 11/hpf, and 33/hpf respectively, while the percentages of IgG4-positive to IgG-positive plasma cells were 70%, 71%, 57%, 43% respectively.
IgG4-related interstitial pulmonary disease can be characterized as the NSIP pattern. The pathological features of IgG4-related NSIP include infiltration of lympho-plasmacytes and eosinophils in interstitium with fibrosis, and lymphoid follicles are frequently identified in the area of lymphocyte aggregation, but obliterative arteritis is infrequently identified in the lesion. Immunohistochemical staining of IgG and IgG4 is very helpful for a definite diagnosis of IgG4-related disease.
观察非特异性间质性肺炎（NSIP）中IgG4的免疫组化染色情况，并研究IgG4相关性NSIP的临床病理特征。
对2002年11月至2010年10月在北京协和医院住院的32例NSIP患者进行回顾性分析。NSIP诊断通过外科肺活检确立，所有标本用中性福尔马林固定并石蜡包埋。切片进行HE和免疫组化染色。根据IgG4相关性疾病的诊断标准，确诊4例为IgG4相关性NSIP。对其临床病理特征包括临床病史、实验室检查及病理评估进行研究。
4例IgG4相关性NSIP患者中，男性1例，女性3例，中位年龄48岁（范围44 - 56岁）。主要症状为干咳或气短。1例患者（1/4，25.0%）自身抗体阳性，但血清中均无RF阳性病例。4例组织学表现为间质炎症细胞浸润伴纤维化，1例显示闭塞性动脉炎。4例IgG4阳性浆细胞数分别为42/hpf、22/hpf、11/hpf和33/hpf，IgG4阳性浆细胞占IgG阳性浆细胞的百分比分别为70%、71%、57%、43%。
IgG4相关性间质性肺疾病可表现为NSIP模式。IgG4相关性NSIP的病理特征包括间质淋巴细胞、浆细胞及嗜酸性粒细胞浸润伴纤维化，淋巴细胞聚集区常可见淋巴滤泡，但病变中很少见闭塞性动脉炎。IgG和IgG4免疫组化染色对明确IgG4相关性疾病诊断非常有帮助。
