Fibrillary glomerulonephritis combined with chronic inflammatory demyelinating polyneuropathy

Abstract

A 58-yr-old man presented with leg edema and subacute weakness of his bilateral lower extremities. Urinary and serum immunoelectrophoresis revealed the presence of lambda-type Bence Jones proteins. He was ultimately diagnosed with monoclonal gammopathy of undetermined significance (MGUS). A renal biopsy specimen showed fibrillary glomerulonephritis (FGN), which was randomly arranged as 12–20 m nonbranching fibrils in the basement membranes. Immunofluorescence studies were negative for immunoglobulin (Ig)G, IgM, IgA, C3, and kappa light chains in the capillary walls and mesangial areas. A Congo red stain for amyloid was negative. Electromyography and nerve conduction velocity examinations results were compatible with the presence of demyelinating polyneuropathy. This case showed a rare combination of FGN, without Ig deposition, and MGUS combined with chronic inflammatory demyelinating polyneuropathy (CIDP).

KASL clinical practice guidelines: management of hepatitis C

“…The anti-HCV prevalence was 5.9-14.7% in previous studies of >200 patients with chronic kidney diseases [ 27 , 28 ], 2.2% in the 2014 report of the Korean Society of Nephrology [ 29 ] and was significantly correlated with hemodialysis duration.…”

“…HCV infection rates in dialysis patients differ among regions from 3% to 80% [ 435 ], anti-HCV positivity rates from the late 1990s to early 2000s in Korea were 5.9-14.7% [ 27 , 28 , 436 ]. In contrast, the Dialysis Registry, ESRD Registry Committee of Korean Society of Nephrology reported a rate of 2.2% in 2014 [ 29 ].…”

Fibrillary Glomerulonephritis, DNAJB9, and the Unfolded Protein Response

“…Immunoglobulin-negative FGN was described in a multi-institutional cohort of 9 cases of a fibrillar GN with DNAJB9 expression which appear to lack IgG by frozen and paraffin IF and mass spectrometry [44]; the presence of IgG heavy and light chains had previously been considered a defining feature of FGN [7,[44][45][46]. Scant IgG staining with prominent C3 deposits and crescents has also been described [47].…”

DNA J homolog subfamily B member 9 and other advances in fibrillary glomerulonephritis

“…Immunoglobulin-negative FGN lacks the staining for IgG heavy and light chains that have been part of the definition of FGN [7,32 ▪ ,33,34]. The presence of DNAJB9 and lack of IgG in rare cases suggests that DNAJB9, not IgG, may be the major player in initial fibril formation [32 ▪ ], which calls into question whether FGN is truly an immune-complex mediated disease driven by IgG, or one of primary protein misfolding with or without secondary autoimmune response, a distinction that may have therapeutic implications.…”