Case 8 - esp-congress.org · S-ANCA (Anti neutrofil cytoplasm antibodies) anti-mieloperossidase...

Case 8

Enterprise | Interest

None

Clinical data

Renal biopsy

27 year old moroccan man

case history: negative before recovery due to edema of legs connected with inflammatory respiratory symptoms

and nephrotic syndrome; no family history of renal or autoimmune diseases

Laboratory data:

serum creatinine 1,07 mg/dL (0,67-1,17)

s-urea 54 mg/dL (18-45)

proteine urine 24h 19,73 g/24h (0,00-0,15)

HBV, HCV, quantiferon test: negative

LM EM

S-ANA (IFA su Hep-2) negative;

S-ANTI ENA screening test for SS-A(Ro52 e Ro60), SS-B, Sm, RNP, Jo-1, Scl-70 negative

S-ANCA (IFA) absent;

S-ANCA (Anti neutrofil cytoplasm antibodies) anti-mieloperossidase (MPO) (method CLIA) negative

S-ANCA. anti -Proteinasi 3 (PR-3) ( method CLIA) negative

SLE panel autoantibodies:

LM

suggesting a membranoproliferative glomerunephritis

lobulated glomerular tuft

thickened GBMs

endocapillary hypercellularity

mesangial proliferation

no tubular, vascular or interstitial injuries

H&E

PAS PAS

MAS TRI

IF

IgG IgA

C1qC3c C4c

Glomerular intense stain

IgG, IgM, C3c, C4c, C1q, light chains kappa and lambda, fibrinogen

type I membranoproliferative glomerunephritis

TEM

tolouidine blue thick section subepithelial, subendothelial, intramembranous and mesangial electrondense deposits

featuring a probable Lupus Nephritis

Parallel linear deposits?

GNMP

with atypical deposits

To date the follow up is negative

•late onset and seropositivity of SLE

20032003

2010

potential subset of

“Lupus like” Nephritis

• LN by EM

• no clinical or serological signs of SLE

• no evidence of TRI

Lupus Nephritis

EM substructure deposits:

• fingerprint

• parallel linear arrays

• tubulofibrillar

2017

EM role

- to prove the localization of immune deposits

- to point out the specific substructure of immune deposits

- to compare and discern between similar morphologic deposit pattern

- to improve the challenging of differential diagnosis

Thank you for your attention!