Tawhari Mohammed, Al Oudah Nourah, Al Zahrani Yousof, Radwi Mansoor
Internal Medicine: Nephrology, King Saud Bin Abdulaziz University for Health Sciences, College of Medicine, Riyadh, SAU.
Research, King Abdullah International Medical Research Center, Riyadh, SAU.
Cureus. 2022 Mar 4;14(3):e22837. doi: 10.7759/cureus.22837. eCollection 2022 Mar.
IgG4 related disease (IgG4-RD) is a systemic autoimmune disease characterized by tissue invasion with IgG4-producing plasma cells, resulting in tissue dysfunction. IgG4-RD can affect the kidney in various forms, including renal mass, tubulointerstitial disease, and glomerulonephritis. IgG4-RD can mimic other autoimmune diseases and neoplasms, and as such, maintaining a high index of suspicion is the key to timely diagnosis and treatment. In this paper, we present a case of IgG4-RD that presented with pseudotumor and severe renal dysfunction that progressed to end-stage kidney disease (ESKD), associated with a rare finding of renal vein thrombosis (RVT).
IgG4相关疾病（IgG4-RD）是一种全身性自身免疫性疾病，其特征是产生IgG4的浆细胞浸润组织，导致组织功能障碍。IgG4-RD可通过多种形式累及肾脏，包括肾肿物、肾小管间质性疾病和肾小球肾炎。IgG4-RD可模仿其他自身免疫性疾病和肿瘤，因此，保持高度怀疑指数是及时诊断和治疗的关键。在本文中，我们报告一例IgG4-RD病例，该病例表现为假瘤和严重肾功能不全，并进展至终末期肾病（ESKD），同时伴有罕见的肾静脉血栓形成（RVT）。
