IgG4-related kidney disease (IgG4-RKD), a progressive renal disorder, often leads to renal dysfunction. Tubulointerstitial nephritis (TIN) is the particular pathophysiology of IgG4-RKD responsible for progression and IgG4-related TIN is a distinct entity of IgG4-RKD. Renal biopsy is the gold standard for diagnosis in IgG4-related TIN, and a characteristic pattern of histology has been identified. A large IgG4 (+) plasma cell population, characteristic fibrotic pattern (storiform or “bird’s eye” fibrosis), and immune complex deposition within the tubulointerstitial compartment are the particular lesions associated with IgG4-related TIN. However, not every case of IgG4-RKD is associated with striking fibrosis, and some are difficult to distinguish from other renal processes that may have TIN. Because full details of the pathogenesis of IgG4-related TIN remain to be elucidated, the diagnosis does not yet have a molecular basis but rather is made through a synthesis of clinical, radiological and histological findings.
CITATION STYLE
Nagata, M., & Hara, S. (2016). Characteristic tubulointerstitial nephritis in IgG4-related kidney disease. In IgG4-Related Kidney Disease (pp. 105–113). Springer Japan. https://doi.org/10.1007/978-4-431-55687-9_9
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