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Year : 2021  |  Volume : 31  |  Issue : 3  |  Page : 286-289

IGg4-related disease presenting as rapidly progressive renal failure and inguinal iymphadenopathy

1 Associate Professor Department of Nephrology, VIMS, Bellary, Karnataka, India
2 Director at Anand Diagnostic Laboratory- A Neuberg Associate, Bengaluru, Karnataka, India
3 Consultant Pathologist at Anand Diagnostic Laboratory- A Neuberg Associate, Bengaluru, Karnataka, India
4 Junior Resident Department of Medicine, VIMS, Bellary, Karnataka, India

Correspondence Address:
Dr. Maniyar Iqbal Anvar
Department of Nephrology, VIMS, Cantonment, Bellary, Karnataka - 583 104
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijn.IJN_377_19

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A 58-year-old man presented with swelling in the left inguinal region and loss of appetite, nausea and azotemia with a serum creatinine of 5.2 mg/dL and urine albumin of 1+ and normal size kidneys with no hematuria. His serum creatinine increased to 9 mg/dL over 1 month, his total proteins were 10.8 and serum albumin was 3.3 g/dL, and lymph node excision biopsy showed fragments of lymphoid tissue with interfollicular areas containing sheets of plasma cells and atretic germinal centers. In view of unexplained renal failure, he underwent renal biopsy, which showed interstitial inflammatory infiltrate composed of lymphocytes and rich in plasma cells with storiform fibrosis and a possibility of IgG4-related renal disease was reported. On further evaluation, serum electrophoresis showed no M band. Serum IgG4 levels were 18.2 g/L (0.03–2 g/L). A diagnosis of IgG4-related renal disease was confirmed.


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Indian Journal of Nephrology
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Online since 20th Sept '07