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Currently filtering by tag: Plasma cells

Twitter Poll (December 7, 2018)

ANSWER: D MGRS was introduced to acknowledge a clonal plasma cell or B lymphocyte proliferation causing a renal lesion in the absence of hematologic malignancy or other myeloma-defining events; the renal lesion is a consequence of the MIg, which carries major implications for management and prognosis, including the potential for progressive renal injury and ESRD. MGRS caused by PC is defined as <10% bone marrow plasma cells (BMPCs), <3 g/dl of M protein, and the presence of renal lesions without any other myeloma-defining events (CRAB features; clonal PC ≥60%; serum FLC ratio of ≥100; or >1 focal lesion on magnetic...

Diagnose This (September 10, 2018)

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

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...

Sjogrens

Sjögren’s syndrome is an autoimmune disease involving primarily the lacrimal and salivary glands. However, up to 27% of patients will also have renal involvement. When clinical evidence of renal involvement is present, approximately 71% show evidence of interstitial nephritis on biopsy including 46% with chronic interstitial nephritis and 25% with acute interstitial nephritis. On renal biopsy, the inflammatory infiltrates in Sjögren’s-related interstitial nephritis (as shown here) is typically mononuclear and rich in plasma cells. Unlike the interstitial nephritis was seen with IgG4-related disease or lupus, there are no tubular basement membrane deposits present by immunofluorescence.

Chronic Interstitial Nephritis

Chronic interstitial nephritis is a diagnosis made on renal biopsy when interstitial inflammation is present in a background of fibrosis (as depicted here). The differential diagnosis is broad but consists primarily of autoimmune-related and drug-induced etiologies. As opposed to acute interstitial nephritis, which is of relatively recent onset, chronic interstitial nephritis results from a long-standing inflammatory process. The distinction between these two morphologic patterns is made based on the appearance of the background interstitium. If the inflammation is located in areas of fibrosis, as is seen in this case of CIN due to Sjögren’s syndrome, the process is designated CIN...

IgG4

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.