IgG4-Related Infiltrative Kidney Disease without Extrarenal Organ Involvement: A Case Report
Phatadon Sirivongrangson¹, Kittisak Weerapolchai², Theerapong Lieworakul³, Nattachai Srisawat⁴,⁵,⁶,⁷
Affiliation : ¹ Division of Nephrology, Department of Medicine, Somdech Phra Pinklao Hospital, Bangkok, Thailand; ² Division of Urology, Department of Surgery, Somdech Phra Pinklao Hospital, Bangkok, Thailand; ³ Division of Anatomical Pathology, Department of Pathology, Somdech Phra Pinklao Hospital, Bangkok, Thailand; ⁴ Division of Nephrology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand; ⁵ Excellence Center for Critical Care Nephrology, King Chulalongkorn Memorial Hospital, Bangkok, Thailand; ⁶ Center of Excellence in Critical Care Nephrology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand; ⁷ Academy of Science, Royal Society of Thailand, Bangkok, Thailand
Background: IgG4-related disease (RD) is a systemic condition that can affect nearly all organs. Renal involvement encompasses interstitial nephritis, membranous nephropathy, and renal infiltrative lesions. Symptomatic IgG4-related kidney disease (RKD) without extrarenal organ manifestations is an exceptionally rare occurrence.
Case Report: The authors present a case of 48-year-old male with isolated renal IgG4-RD presented with abdominal pain. Laboratory investigations revealed acute kidney injury, proteinuria, and hypoalbuminemia. Whole abdominal computed tomography (CT) scan disclosed infiltrative lesions in both kidneys and ureters, accompanied by hydronephrosis. Kidney biopsy demonstrated lymphocytic and plasma cell infiltration, with 82 plasma cells per high power field (HPF), storiform fibrosis, and the absence of obliterative phlebitis. After receiving corticosteroid treatment, the patient’s renal function improved, and the infiltrative lesions went into remission.
Conclusion: Isolated renal IgG4-RD is a rare and challenging diagnosis. The disease can manifest as acute interstitial nephritis, glomerular disease, retroperitoneal fibrosis, or, rarely, as mass or infiltrative lesions. Corticosteroid therapy is likely to yield favorable responses, hence, prompt diagnosis is crucial. Delayed diagnosis may result in organ fibrosis and dysfunction.
Received 16 October 2023 | Revised 12 January 2024 | Accepted 12 January 2024
DOI: 10.35755/jmedassocthai.2024.2.13940
Keywords : IgG4-related disease; IgG4-related kidney disease; Infiltrative renal disease
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