Saravanan M, Alexander S, Matthai S M, Korula A, Varughese S, Tamilarasi V
Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India.
Department of Central Electron Microscopic Unit, Christian Medical College, Vellore, Tamil Nadu, India.
Indian J Nephrol. 2015 Mar-Apr;25(2):113-6. doi: 10.4103/0971-4065.136886.
Immunoglobulin G4-related disease (IgG4-RD) is an emerging clinicopathological entity. Renal involvement is dominated by tubulointerstitial nephritis (TIN) with IgG4-positive plasma cells and fibrosis. IgG4-RD commonly affects middle-aged to elderly men with accompanying extra-renal lesions such as sialadenitis, lymphadenopathy, or type 1 autoimmune pancreatitis, all of which respond favorably to corticosteroid therapy. The disease burden of IgG4-related kidney disease (IgG4-RKD) in India remains largely underestimated. We report a case of IgG4-RKD manifesting as TIN associated with interstitial pulmonary disease, illustrating typical clinico-pathologic, serologic, immuno-histochemical, and ultrastructural features of this condition. In view of potential amelioration of renal dysfunction with appropriate therapy, the need for awareness of this condition and early diagnosis is highlighted.
免疫球蛋白G4相关性疾病（IgG4-RD）是一种新出现的临床病理实体。肾脏受累以伴有IgG4阳性浆细胞和纤维化的肾小管间质性肾炎（TIN）为主。IgG4-RD通常影响中年至老年男性，伴有涎腺炎、淋巴结病或1型自身免疫性胰腺炎等肾外病变，所有这些病变对皮质类固醇治疗反应良好。在印度，IgG4相关性肾病（IgG4-RKD）的疾病负担在很大程度上仍被低估。我们报告一例表现为TIN并伴有间质性肺病的IgG4-RKD病例，阐述了该病典型的临床病理、血清学、免疫组织化学及超微结构特征。鉴于适当治疗可能改善肾功能障碍，强调了认识该病及早期诊断的必要性。
