Al-Saedi Zainulabdeen S, Alatta Lina, Miqdad Mohammed A, Hulwi Hasan, Rodriguez Oscar, Yang Yihe, Kuo Sheng
Medicine/Nephrology, NewYork-Presbyterian Queens, New York, USA.
Nephrology, NewYork-Presbyterian Queens, New York, USA.
Cureus. 2025 Jul 21;17(7):e88430. doi: 10.7759/cureus.88430. eCollection 2025 Jul.
This case report describes a rare presentation of Type 1 Cryoglobulinemic Glomerulonephritis due to IgG2 kappa monoclonal gammopathy in a 74-year-old man with a history of Monoclonal Gammopathy of Undetermined Significance (MGUS), classifiable under Monoclonal Gammopathy of Renal Significance (MGRS). The patient presented with acute kidney injury, hypertensive urgency, and a migratory rash. Kidney biopsy revealed glomerulitis with IgG2-kappa deposition. Pseudothrombi of IgG2-kappa paraprotein were also present within glomerular capillary and arteriolar lumens, accompanied by vasculitis. Although initial outpatient management focused on MGUS, rapid disease progression necessitated aggressive therapy, including high-dose corticosteroids, plasmapheresis, and hemodialysis. The patient's renal function improved following treatment, emphasizing the need for early recognition and timely intervention in cryoglobulinemia-related kidney disease. This case highlights the importance of kidney biopsy in diagnosing MGRS and underscores the role of targeted therapies in complex cases, particularly clone-directed treatment following initial clinical stabilization.
本病例报告描述了一名74岁男性,有意义未明的单克隆丙种球蛋白病(MGUS)病史,可归类为肾意义单克隆丙种球蛋白病(MGRS),因IgG2 κ单克隆丙种球蛋白病导致1型冷球蛋白血症性肾小球肾炎的罕见表现。患者出现急性肾损伤、高血压急症和游走性皮疹。肾活检显示肾小球炎伴IgG2-κ沉积。肾小球毛细血管和小动脉管腔内也存在IgG2-κ副蛋白假血栓,并伴有血管炎。尽管最初的门诊治疗重点是MGUS,但疾病的快速进展需要积极治疗,包括大剂量皮质类固醇、血浆置换和血液透析。治疗后患者肾功能改善,强调了对冷球蛋白血症相关肾病进行早期识别和及时干预的必要性。本病例突出了肾活检在诊断MGRS中的重要性,并强调了靶向治疗在复杂病例中的作用,特别是在初始临床稳定后进行克隆定向治疗。