An Overlapping Case of Lupus Nephritis and IgG4-Related Kidney Disease

Mazen Zaarour, Chanudi Weerasinghe, Ahmad Eter, Suzanne El-Sayegh, Elie El-Charabaty


We report a case of a 71-year-old Filipino female who was admitted to the hospital for abdominal pain, vomiting and diarrhea of 8 days duration. The patient was found to have marked acute kidney injury (AKI), which required hemodialysis in the next 3 days. Extensive workup revealed hematuria, subnephrotic range proteinuria, elevated anti-nuclear antibody (ANA) and elevated total immunoglobulin G (IgG) levels, with normal IgG4 and anti-dsDNA levels. On kidney biopsy, mild membranous glomerulonephritis was found, along with autoimmune tubulointerstitial nephritis (TIN) with afull-house pattern of immune deposits. These findings were suggestive of lupus interstitial nephritis. However, IgG4+ plasma cells were detected in the interstitium by immunostaining, favoring a diagnosis of IgG4-related kidney disease (IgG4-RKD). Our case highlights the difficulty in differentiating lupus nephritis (LN) from IgG4-RKD in some patients, raising the suspicion that these two entities can co-exist.

J Clin Med Res. 2015;7(7):575-581


IgG4-related kidney disease; Lupus nephritis; Tubulointerstitial nephritis; Membranous nephropathy; IgG4-related tubulointerstitial nephritis

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