於風濕性多發性肌痛症女性發現第四型免疫球蛋白G相關腎臟疾病：罕見病例報告與文獻回顧 

王大齊¹、李良明¹、林克勳¹、林雍偉¹、石宏仁¹、蕭志豪¹ 、許軒豪¹、溫玉清^1,2

1台北市立萬芳醫院 泌尿科 2臺北醫學大學 醫學院 泌尿學科 

IgG4 related kidney disease found in a woman with polymyalgia rheumatica: a rare case report and literature review 

Ta-Chyi Wang¹, Liang-Ming Lee¹, Ke-Hsun Lin¹, Yung-Wei Lin¹, Hung-Jen Shih¹, Chi-Hao Hsiao¹ , Syuan-Hao Syu¹, Yu-Ching Wen^{1, 2}

¹ Department of Urology, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan. 

² Department of Urology, School of Medicine, College of Medicine, Taipei Medical University 

 

IgG4 related disease may affect several organs as recognized since the 1st international symposium held in Boston in 2011. When it happens in kidney, patients may present with hematuria, proteinuria, hypocomplementemia or chronic renal failure. It characterized by high levels of serum immunoglobulins G4, dense infiltration of IgG4+ cells and storiform fibrosis and T-cell mediated immunity can be implicated in the pathogenesis. The affected lesions may induce fibrosis in kidney parenchyma and finally cause renal function impairment because of obstructive nephropathy. Here in, we present a case report about a 64-year-old woman with polymyalgia rheumatica presented with intermittent right flank pain for a year. Dynamic renal function study showed estimated glomerular filtration rate 17.86 ml/min in left and 57.79 ml/min in right. Bilateral ureteral wall thickening was found with hydronephrosis and a mass in right ureteropelvic junction in computed tomography and magnetic resonance imaging. Ureteroscopic examination revealed thickened right upper third ureteral mucosa with irregular surface and polypoid lesions causing ureteral obstruction. Biopsy was performed for 2 times and the pathology report showed chronic inflammation. Urine cytology showed atypical cellular change. Double J stent was inserted but there was persist right hydronephrosis. Computed tomography guide biopsy was performed and the pathology report showed chronic inflammation with fibrosis. Immunohistochemically, the lymphocytes are mixed CD20-positive B cells and CD3-positive T cells. Scant IgG4-positive cell is revealed. IgG4 related kidney disease was finally diagnosed. The literature was reviewed and discussed.