Author : Emily Stuchfield-Denby
Publisher : Unknown
Page : 0 pages
File Size : 49,8 Mb
Release : 2022
Category : Electronic
ISBN : OCLC:1417601568
Immunohistochemical Expression of DNAJB9 in IgG4-related Disease by Emily Stuchfield-Denby Pdf
Introduction. Immunoglobulin G4-related disease (IgG4-RD) is a rare immune-mediated condition with expansion of plasmablasts/plasma cells and possible upregulation of DNAJB9. DNAJB9 (a co-chaperone protein that governs the integrity and functions of cells) was described as a specific marker for fibrillary glomerulonephritis, a rare proliferative form of glomerulonephritis displaying histological similarities with IgG4-related kidney disease. DNAJB9 may be a putative autoantigen. We aimed to study the immunohistochemical expression of DNAJB9 in IgG4-RD to explore its role in disease pathophysiology. Methods. We selected patients with definite IgG4-RD (RCD, ACR/EULAR or ICDC classification criteria), non-auto-immune (NAI) controls and Sjögren's syndrome controls, and produced tissue slides for anti-DNAJB9 immunohistochemistry on which we performed double reading and applied our intensity scoring system ranging from 0 to 4. Our positive control was a renal sample suggestive of fibrillary glomerulonephritis. Results. We studied the tissue slides of 9 IgG4-RD patients and 15 NAI controls (including salivary gland, pancreatic, pulmonary, pleural, and retroperitoneal fibrotic tissue). Median intensity for endothelial cells was 0 in IgG4-RD and 2 in NAI controls (p