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Abstract: TH-PO729

An Atypical Case of IgG4-Related Disease (IgG4-RD) Manifesting as Extensive Abdominal Periarteritis and Membranous Nephropathy (MN): A Case Report and Literature Review

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials


  • Matsumoto, Minami, Kyoto Daigaku, Kyoto, Japan
  • Yamamoto, Shinya, Kyoto Daigaku, Kyoto, Japan
  • Yokoi, Hideki, Kyoto Daigaku, Kyoto, Japan
  • Yanagita, Motoko, Kyoto Daigaku, Kyoto, Japan

IgG4-RD is a progressive immune-mediated fibrotic disease characterized by tumor-like mass formation in many affected organs. Tubulointerstitial nephritis (TIN) with increased IgG4-positive plasma cells is the dominant feature of IgG4-related kidney disease. Here we present an atypical case of IgG4-RD, and a literature review of 18 cases on IgG4-related MN.

Case Description

A 71-year-old man with a peptic ulcer was admitted with acute-onset leg edema and nephrotic syndrome. The laboratory data revealed serum albumin 1.9 g/dL, creatinine 1.1 mg/dL, and urinary protein 9.3 g/gCr. The renal biopsy showed granular staining in the capillary walls for IgG (IgG1>4) and negative PLA2R staining, suggesting secondary MN. Computed tomography (CT) scans revealed extensive wall thickening around the iliac and splenic arteries, and superior and inferior mesenteric arteries with dilatation. The findings of periarteritis, high serum IgG4 and IgE levels, and numerous IgG4-positive cells infiltration to interstitium led to the diagnosis of IgG4-RD. Other etiologies causing vascular lesions such as lymphoma, and polyarteritis nodosa, were incompatible with the clinical findings. We chose low-dose prednisolone because this patient had a peptic ulcer and high doses of prednisolone may promote aneurysm formation in IgG4-related arteritis. After the initiation of prednisolone (10 mg) and ARB, he achieved partial remission of proteinuria. We successfully controlled IgG4-RD and avoided the recurrence of peptic ulcers.


In this case, nephrotic syndrome and the absence of typical manifestations, such as pancreatitis and sialadenitis made the diagnosis of IgG4-RD challenging. It was also unique in that extensive arteritis was confined to a major branch of the abdominal aorta, not the aorta itself. These features suggest a new phenotype of IgG4-RD. Our literature review of 18 cases on IgG4-related MN, including 1 case with aortitis, demonstrated that 10 cases achieved complete remission, and 8 achieved partial remission of proteinuria with various treatments such as corticosteroids, cyclophosphamide, and rituximab. Although the number of affected organs had no relation with the response of proteinuria, the complication of TIN had an association with a good response.