Monoclonal immunoglobulin-associated proliferative glomerulonephritis characterized by organized deposits of striated ultra-substructures: A case report. (4th July 2017)

• Record Type: Journal Article
• Authors: Hara, Shigeo; Tsukaguchi, Hiroyasu; Oka, Tastufumi; Kusabe, Makiko; Mizui, Masayuki; Joh, Kensuke
• Is Part Of: Ultrastructural pathology. Volume 41:Number 4(2017:Aug.)
• Page Start: 301
• Page End: 307
• Publication Date: 2017-07-04
• Subjects: Collagen -- fibrin -- monoclonal -- striated structure; Pathology, Cellular -- Periodicals; Ultrastructure (Biology) -- Periodicals; Diagnosis, Electron microscopic -- Periodicals; Microscopy, Electron; Pathology; 616.07582
• DOI: 10.1080/01913123.2017.1336189
• Languages: English
• ISSNs: 0191-3123

Abstract

ABSTRACT: We herein report the case of a 64-year-old male who presented with progressive glomerulonephritis notable for organized and striated ultra-substructures. The patient was diagnosed with hypertension and proteinuria 3 years prior to admission and subsequently developed nephrotic syndrome and impairment of renal function. Laboratory tests did not reveal any evidence of infections or autoimmune diseases. Monoclonal gammopathy was not detected in serum or urine, although a small population of abnormal plasma cell clones was detected by flow cytometry. A renal biopsy showed mesangial and endocapillary proliferative glomerulonephritis with lobular accentuation, accompanied with focal and segmental double-contour formation. Additionally, moderate tubulointerstitial scarring and arteriosclerosis were noted. Immunofluorescence staining revealed positive staining for IgG, IgM, C3, C1q, and fibrinogen. IgG subclass and light chain staining showed restricted positivity for IgG1κ. Electron microscopy demonstrated massive amounts of subendothelial deposits with a fibrillary and branching profile. At higher magnification, a periodic striated pattern was observed within the microfilament-like structures. Immunohistochemical staining was negative for myoglobin, laminin, and collagens (type III and IV). Steroid and antihypertensive therapy did not show improvement in renal function. The second biopsy performed 2 years later revealed a similar lobular proliferative glomerulonephritis pattern with more extensive tubulointerstitial damage, indicating poor response to immunosuppressive therapy. The patient progressed to end-stage renal disease and required hemodialysis. We discuss the possible origins of the deposits with unusual substructures observed in this case.