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Year : 2022  |  Volume : 32  |  Issue : 5  |  Page : 512-515

Diagnostic dilemma: Cardiorenal syndrome as an unusual presentation of IgG4-related disease

1 Department of Nephrology, Manipal Hospitals, Dwarka, Delhi, India
2 Department of Histopathology and Cytopathology, BLK Super Specialty Hospital, Delhi, India

Correspondence Address:
Shailendra Singh
Department of Nephrology, Associate Consultant, Manipal Hospitals, Dwarka, Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijn.ijn_143_21

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IgG4-related kidney disease (IgG4 RKD) is a rare clinical entity characterized by lymphoplasmacytic infiltration rich in IgG4-positive plasma cells along with fibrosis affecting several organs. Tubulointerstitial nephritis is commonly the predominant finding on kidney biopsy. Our patient was admitted with a provisional diagnosis of cardiorenal syndrome of unknown etiology. The patient was dialysis dependent for around 45 days following which kidney biopsy revealed features of acute tubulointerstitial nephritis (ATIN) with IgG4-positive plasma cells and no glomerular involvement. Positron emission tomography–computed tomography was supportive of findings of sialadenitis along with myocarditis. Our patient responded to treatment with steroids with definitive improvement in both renal and cardiac functions. This case highlights the importance of IgG4 RKD as an important differential in patients with ATIN presenting as a clinical syndrome.


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Indian Journal of Nephrology
Published by Wolters Kluwer - Medknow
Online since 20th Sept '07