论文部分内容阅读
目的探讨IgG4相关性疾病的临床病理学特点、鉴别诊断、治疗及预后。方法对1例累及肾和淋巴结的IgG4相关性疾病病理活检标本进行光镜、电镜及免疫组化染色(采用EliVision法),并结合文献探讨该病的临床病理学特点。结果患者肾穿刺活检见肾间质中大量浆细胞浸润,主要为IgG阳性细胞,其中IgG4阳性细胞(>10个/HPF);肾小球毛细血管壁弥漫增厚,并节段双轨形成。腋下淋巴结活检显示,淋巴滤泡散在分布,部分滤泡呈萎缩状态,滤泡间区明显扩大,其内可见大量浆细胞增生,并掺杂较多小淋巴细胞及少量免疫母细胞。免疫组化示IgG阳性增生的浆细胞>50个/HPF。结论 IgG4相关性疾病是IgG4阳性细胞广泛浸润的慢性系统性疾病,累及肾时可出现小管间质性肾炎,多数会累及淋巴结。对激素治疗反映较好,停药后可复发。
Objective To investigate the clinicopathological features, differential diagnosis, treatment and prognosis of IgG4 related diseases. Methods One case of pathological biopsy specimens of IgG4 related diseases involving the kidneys and lymph nodes were stained with light microscopy, electron microscopy and immunohistochemistry (using EliVision method), and the clinical and pathological features of the disease were analyzed. Results In the renal biopsy, a large number of plasma cells infiltrated in the renal interstitium, including mainly IgG positive cells (> 10 cells / HPF). The glomerular capillaries were diffuse and thickened and segmental double track was formed. Axillary lymph node biopsy showed that lymphoid follicles were scattered, some follicles showed atrophic state, interstitial follicles significantly expanded, which can be seen a large number of plasma cell proliferation and doping more small lymphocytes and a small amount of immune cells. Immunohistochemistry showed IgG-positive hyperplastic plasma cells> 50 / HPF. Conclusion IgG4-related diseases are chronic systemic diseases with extensive infiltration of IgG4-positive cells. Tubulointerstitial nephritis may occur when the kidneys are involved, most of them involve the lymph nodes. Good response to hormone therapy, relapse after stopping.