| Summary: | Immunoglobulin G4-related disease is a recently described systemic fibro-inflammatory disease, characterized by an infiltration of abundant IgG4+ plasma cells and lymphocytes, leading to tumour-like swellings of the involved organs, and variable degrees of fibrosis. Organ involvement can occur metachronously, and as IgG4 expressionin other tissues is less specific, renal biopsy often plays a critical role in diagnosis leading to prompt treatment, which can prevent renal damage and other organ failure. Tubulointerstitial nephritis is the most common renal feature. IgG4-renal involvement is challenging to diagnose and remains under-recognized, particularly in patients with single-organ involvement. The authors describe the only 5 cases of IgG4-RD with kidney involvement identified in the last 10 years in a tertiary referral nephrology centre that serves a population of 3 million people. These cases underline the importance of an early diagnosis of IgG4-related disease, since early treatment is usually effective. They also corroborate that clinical and histopathological features are heterogeneous and that complement may play a role in its pathogenesis
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