Abstract
Systemic lupus erythematosus (SLE) and IgG4-related disease (IgG4-RD) both result from altered inflammatory and immunological responses. They also present with similar clinical and renal histological findings. In both conditions, the urinalysis can appear normal despite the presence of active nephritis. The serological examination demonstrates positive anti-nuclear antibodies and hypocomplementemia, and the serum levels of IgG and IgG4 are elevated in both SLE and IgG4-RD. Although polyclonal gammopathies are found in both conditions, the frequent finding of an IgG4 predominance is characteristic of IgG4-related kidney disease (IgG4-RKD). Increases in IgE are also typical of IgG4-RKD. Tubulointerstitial nephritis (TIN) and the infiltration of IgG4-bearing plasma cells into the interstitium observed using light microscopy are common to both. Thus, careful examination with immunofluorescence and electron microscopy are essential to establishing the correct diagnosis. The “full-house” pattern of immunoglobulin and complement deposition in the tubular basement membranes are distinguishing features of TIN in lupus nephritis. In contrast, storiform fibrosis, often observed most easily with periodic acid-methenamine silver staining, is the pathognomonic feature of IgG4-RKD.
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Nishi, S., Hara, S., Imai, N. (2016). Differential Diagnosis: Lupus Nephritis and IgG4-Related Kidney Disease. In: Saito, T., Stone, J., Nakashima, H., Saeki, T., Kawano, M. (eds) IgG4-Related Kidney Disease. Springer, Tokyo. https://doi.org/10.1007/978-4-431-55687-9_25
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