Recognizing IgG4-related tubulointerstitial nephritis

AbstractPurpose of the reviewImmunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder affecting nearly all organs, including the kidney. Tubulointerstitial nephritis (IgG4-TIN) is the most common form of IgG4-related kidney disease (IgG4-RKD) and is the focus of this concise review.ObjectiveThe study aims to describe when IgG4-TIN should be suspected and to summarize the diagnosis, treatment, and natural history of the disease.Sources of informationOvid MEDLINE, Google Scholar, and PubMed were searched for full-text English language articles up to January 2016. References included in the manuscript were chosen at the authors ’ discretion based on their relevance to the subject of the review.FindingsIgG4-TIN should be considered in patients presenting with abnormal urinalysis, abnormal kidney function, renal lesions on imaging, and elevated IgG, IgE, or hypocomplementemia. Diagnosis of IgG4-TIN requires a combination of histologic features (plasma cell-enriched TIN with>10 IgG4+ plasma cells/hpf, +/ − TBM immune complex deposits in many cases) and at least one of the following:Characteristic radiologic findings (small peripheral low-attenuation cortical nodules, round or wedge-shaped lesions, or diffuse patchy involvement)Elevated serum IgG4 levelCharacteristic findings of IgG4-RD in other organsOther conditions such as lupus, vasculitis, diabetic nephropathy, and lymphoma must be excluded, as these can also present with IgG4+ plasma cells in...
Source: Canadian Journal of Kidney Health and Disease - Category: Urology & Nephrology Source Type: research