摘要:
Objective To analyze the clinicopathological features and prognosis of immunoglobulin G (IgG)4-related diseases with interstitial nephritis. Methods Forty cases of IgG4-related diseases diagnosed by pathology in our hospital from 2014 to 2018 were collected and their clinicopathological features were analyzed. Four patients with IgG4-related disease with interstitial nephritis were analyzed, including clinical laboratory tests and histopathological features, and immunohistochemical analysis of the type and proportion of renal interstitial infiltrating cells. At the same time, the treatment and prognosis of the patients were analyzed. Results Among 40 cases of IgG4-related diseases, 11 cases had parotid submandibular gland involvement (accounted for 28%), labial gland involvement (7 cases, 18%) and lymph node enlargement (6 cases, 15%). Patients with kidney involvement (4 cases, 10%) all presented with IgG4-related tubulo-interstitial nephritis (IgG4-TIN). All of them were elderly males, and 2 had glomerulopathy. One of them had anti-neutrophil cytoplasmic antibodies (ANCA)-related vasculitis renal damage. The number of CD4-positive cells in renal interstitium was more than CD8-positive cells. Another case complicated with IgA nephropathy. Renal dysfunction occurred in all 4 cases, and serum IgG4 level ranged from 4.65 g/L to 23.8 g/L. All 4 patients received glucocorticosteroid and symptomatic treatment, and the prognosis was good. Conclusion IgG4-related diseases may involve mul-tiple organs, renal dysfunction may occur when the kidney is involved. Interstitial nephritis is the major clinical manifestation. Glomerular lesions may accelerate the progress of IgG4-TIN. Corticosteroid therapy is effective.%目的 分析以间质性肾炎为主要表现的免疫球蛋白G(IgG)4相关性疾病的临床病理学特征及治疗预后.方法 收集2014—2018年就诊于我院经病理确诊的40例IgG4相关性疾病,分析临床病理特点,并重点分析4例以间质性肾炎为主要表现的IgG4相关性疾病患者资料,包括临床实验室检查及病理组织学特点,免疫组织化学法分析肾间质浸润细胞的种类和数量比例,并观察其治疗及预后.结果 40例IgG4相关性疾病中累及腮腺颌下腺11例,约占28%,其次为唇腺(7例,18%)、淋巴结(6例,15%),而累及肾脏(4例,10%)患者均表现为IgG4相关性间质性肾炎(IgG4-TIN),均为老年男性,2例伴有肾小球病变,其中1例合并ANCA相关性血管炎性肾损害,间质内CD4阳性细胞远大于CD8阳性细胞数目,另1例合并IgA肾病;4例患者均出现肾功能异常,血清IgG4水平在4.65~23.8 g/L.4例患者均行糖皮质激素和对症治疗,预后较好.结论 IgG4相关性疾病可累及多个脏器,累及肾脏时可出现肾功能异常,以间质性肾炎为主要表现,合并肾小球病变可能加速IgG4-TIN的进展,激素治疗可有效缓解病情.