Qiao Xi, Wang Lihua, Wang Chen, Gao Lifang, Yao Shulei, Wu Liran, Zhang Xiaoqin
Department of Nephrology, Second Hospital of Shanxi Medical UniversityWu Yi Road 382, Taiyuan 030001, Shanxi, China; Shanxi Kidney Disease InstituteWu Yi Road 382, Taiyuan 030001, Shanxi, China.
Department of Pathology, Second Hospital of Shanxi Medical University Wu Yi Road 382, Taiyuan 030001, Shanxi, China.
Int J Clin Exp Med. 2015 Oct 15;8(10):19548-55. eCollection 2015.
IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common renal manifestation of IgG4-related kidney disease (IgG4-RKD) and may cause acute or chronic renal dysfunction. Imaging often shows heterogeneous densities in the kidneys, such as a mass or multiple nodules. Serology usually demonstrates high levels of serum IgG4 and total IgG. Most patients have other organs involvement by IgG4 related disease. Although lymphadenopathy is frequently observed in patients with IgG4-TIN, it is rarely presented as the only extrarenal lesion. Herein, we present a rare case of IgG4-TIN associated with only lymphadenopathy and without elevated serum IgG4 or renal imaging abnormalities. A 61-year-old Chinese man was admitted to our hospital with seven months history of generalized lymphadenopathy and five months history of renal dysfunction. His renal imaging was normal. He had no current or previous clinical, radiographic, and/or histologic evidence of other organ involvement except for the lymphadenopathy. Renal biopsy indicated plasma cell-rich TIN with an increased number of IgG4-positive plasma cells and storiform fibrosis. Repeated lymph nodes biopsy revealed IgG4-related lymphadenopathy. However, he did not have elevated serum IgG4 or total IgG levels. Oral prednisone therapy improved his renal function and lymphadenopathy. These findings supported our final diagnosis of IgG4-TIN. Clinicians should be aware of this condition and steroid therapy should be considered for such patients. An early diagnosis and appropriate therapy can induce remission and preserve renal function.
IgG4相关性肾小管间质性肾炎（IgG4-TIN）是IgG4相关性肾病（IgG4-RKD）最常见的肾脏表现，可导致急性或慢性肾功能不全。影像学检查常显示肾脏密度不均，如肿块或多个结节。血清学检查通常显示血清IgG4和总IgG水平升高。大多数患者有其他器官受IgG4相关疾病累及。虽然IgG4-TIN患者常观察到淋巴结病，但很少仅表现为肾外病变。在此，我们报告1例罕见的IgG4-TIN病例，仅伴有淋巴结病，血清IgG4未升高且肾脏影像学无异常。一名61岁中国男性因全身淋巴结病7个月和肾功能不全5个月入住我院。其肾脏影像学检查正常。除淋巴结病外，他目前及既往均无其他器官受累的临床、影像学和/或组织学证据。肾活检显示富含浆细胞的肾小管间质性肾炎，IgG4阳性浆细胞数量增加及席纹状纤维化。重复淋巴结活检显示IgG4相关性淋巴结病。然而，他的血清IgG4和总IgG水平未升高。口服泼尼松治疗改善了他的肾功能和淋巴结病。这些发现支持我们对IgG4-TIN的最终诊断。临床医生应了解这种情况，对此类患者应考虑使用类固醇治疗。早期诊断和适当治疗可诱导缓解并保留肾功能。
