IgG4-Related Disease of the Right Proximal Ureter in a 55-Year-Old Male Patient: A Case Report

IgG4-Related Disease of the Right Proximal Ureter

Abstract

IgG4-related disease is a fibroinflammatory condition that has increasingly reported in the past decade. Due to the  systemic involvement of this disease, the presentation can  mimic various processes including inflammatory, infection  and neoplastic conditions. A 55-year-old male patient had a  history of chronic abdominal pain. The multidetector  computed tomography (MDCT) of the kidney, ureter, and bladder system with contrast showed an enhancing mass at right upper ureter, ureteric cancer was suspicious. Right  nephroureterectomy was performed. The gross  xamination showed a firm ill-defined homogeneous grey white  infiltrative mass at right proximal ureter. The histologic  sections showed two major histological features of IgG4- related disease (dense lymphoplasmacytic infiltrate and storiform fibrotic pattern). Additional immunohistochemical study showed increased IgG4 and IgG-positive plasma cells  with IgG4/IgG positive plasma cell ratio more than 40%  (49%). Further laboratory investigation showed increased  serum IgG level (644 mg/dL: > 135 mg/dL). According to the findings, this case was compatible with IgG4-related disease. Thus, the definite diagnosis of IgG4-related disease needs correlation with clinical presentation, imaging study,  serological investigation, histological features and  immunohistochemical study.