Close Modal


Currently filtering by tag: IgG4 related disease

Not Quite Nodular

This biopsy came from a 70-year-old gentleman with acute renal failure. He had a known history of diabetes, hypertension, and chronic kidney disease.  His baseline serum creatinine is between 1.7 mg/dl and 1.9 mg/dl and was found to be elevated up to 2.9 mg/dl. Proteinuria was quantified as 0.2 g/g on urine protein to creatinine ratio.  Urinalysis showed trace blood and protein.  While processing the clinical information, one has to admit that this clinical presentation is not an unusual one.  A fair number of patients will have a decline in renal function that reflects the reality of an already struggling...

IgG4 Membranous

First identified in the pancreas as autoimmune pancreatitis, the IgG4-related disease is now known to affect many organs and body sites. Renal involvement may present as a discrete mass noted radiologically, or patients may present with renal failure secondary to diffuse sclerosing tubulointerstitial disease. Patients often have laboratory abnormalities that can support the diagnosis such as eosinophilia, low complement and hypergammaglobulinemia with an elevation of serum IgG4 levels. A combination of imaging, clinical and laboratory features, and histology are needed to make a definitive diagnosis. Patients often show a clinical and radiographic response to steroid therapy. The Jones methenamine silver...

IgG4-Related Tubulointerstitial Nephritis

IgG4-related disease is a systemic inflammatory and sclerosing disorder which may affect a wide range of different organs including the pancreas, salivary glands, lacrimal glands, lungs, blood vessels, lymph nodes, thyroid and kidneys among others. Patients may have multisystemic disease at the time of presentation, or different organs may progressively become involved over time. While the renal involvement may have different histopathologic patterns of injury, by far the most common is in the form of acute or chronic tubulointerstitial nephritis (IgG4-related tubulointerstitial nephritis). The renal biopsy shown (Fig 1-4) is from a 35-year-old male with a history of lymphadenopathy and...

Diagnose This! (September 18, 2017)

What is your diagnosis?     ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​...


Renal parenchyma with florid interstitial fibrosis with thick bands of sclerosis expanding the interstitium and pushing the tubules apart on the Jones methenamine silver stain at (100X). Examination at 400X on the H&E stain reveals a dense mixed interstitial inflammatory infiltrate that is rich in plasma cells. These findings are characteristic of renal involvement by a systemic fibroinflammatory disease known as IgG4-related disease. IgG4 staining of the tissue confirmed the presence of numerous IgG4-positive plasma cells.