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GLOMERULONEFRITE LUPICA
(Luglio-Agosto-Settebre/2005) Dott.ssa Marisa Arrondini (Università degli Studi di Torino)
Notizie Cliniche
- Donna di 40 anni
- Giugno 2004 comparsa di edemi declivi
- Febbraio 2005 riscontro agli esami ematochimici di:
- Anemia, leucopenia e piastropenia
- Creatininemia 1,2 mg/dl
- Ipoprotidemia
- Ipocomplementemia
- FAN +
- Anti DNA+
- Esame urine:
proteinuria 3,6 gr./24 h
10-20 RBC p.c.m.
cilindri ialini e ialinogranulosi
Diagnosi Clinica
Lupus Eritematoso Sistemico perché la paziente possiede almeno 5 dei criteri ARA.
Criteri ARA (American Rheumatism Association):
- RASH MALARE
- LUPUS DISCOIDE
- FOTOSENSIBILITA’
- ULCERE ORALI
- ARTRITE NON EROSIVA
- SIEROSITE
- INTERESSAMENTO RENALE (proteinuria> 0,5 gr./24 h. e cilindruria)
- INTERESSAMENTO NEUROLOGICO
- INTERESSAMENTO EMATOLOGICO (anemia, leucopenia, piastrinopenia)
- DISTURBI IMMUNOLOGICI (fenomeno LE, ANA+, anti dsDNA+, anti Sm+, falsa positività per la lue da almeno 6 mesi)
Quando si fa la biopsia renale?
In tutti i pazienti con LES che hanno anomalie del sedimento urinario e della funzione renale.
Perché si fa la biopsia renale?
- Clinica non permette di capire qual è il tipo di danno renale
- Decorso clinico e pianificazione terapeutica
PAS
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PAS
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PAS
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PTAH
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PTAH
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AFOG
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Immunofluorescenza
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Diagnosi Anatomopatologica
Glomerulonefrite lupica classe IV b
sec. Who modificata
Glomerulonefrite lupica classe IV-G (A)
sec. ISN/RPS 2003
The Classification of Glomerulonephritis in Systemic Lupus
Erythematosus Revisited
J Am Soc Nephrol 15: 241–250, 2004
World Health Organization (WHO) morphologic classification of lupus nephritis (modified in 1982)
Class I Normal glomeruli
a. Nil (by all techniques)
b. Normal by light microscopy, but deposits by electron or
immunofluorescence microscopy
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Active glomerular lesions
Endocapillary hypercellularity with or without leukocyte
infiltration and with substantial luminal reduction
Karyorrhexis
Fibrinoid necrosis
Rupture of glomerular basement membrane
Crescents, cellular or fibrocellular
Subendothelial deposits identifiable by light microscopy (wireloops)
Intraluminal immune aggregates (hyaline thrombi)
Chronic glomerular lesions
Glomerular sclerosis (segmental, global)
Fibrous adhesions
Fibrous crescents
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Class II Pure mesangial alterations (mesangiopathy)
a. Mesangial widening and/or mild hypercellularity
b. Moderate hypercellularity
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Class III Focal segmental glomerulonephritis
a. With “active” necrotizing lesions
b. With “active” and sclerosing lesions
c. With sclerosing lesions
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Class IV Diffuse glomerulonephritis
a. Without segmental lesions
b. With “active” necrotizing lesions
c. With “active” and sclerosing lesions
d. With sclerosing lesions
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Class V Diffuse membranous glomerulonephritis
a. Pure membranous glomerulonephritis
b. Associated with lesions of class II
c. Associated with lesions of class III
d. Associated with lesions of class IV
Class VI Advanced sclerosing glomerulonephritis
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International Society of Nephrology/Renal Pathology Society (ISN/RPS) 2003 classification of lupus nephritis
Class I Minimal mesangial lupus nephritis
Normal glomeruli by light microscopy, but mesangial immune deposits by immunofluorescence
Class II Mesangial proliferative lupus nephritis
Purely mesangial hypercellularity of any degree or mesangial matrix expansion by light
microscopy, with mesangial immune deposits
Class III Focal lupus nephritis
glomerulonephritis involving 50% of all glomeruli, typically with focal subendothelial immune deposits,
-Class III (A) Active lesions: focal proliferative lupus nephritis
-Class III (A/C) Active and chronic lesions: focal proliferative and sclerosing lupus nephritis
-Class III (C) Chronic inactive lesions with glomerular scars: focal sclerosing lupus nephritis
Class IV Diffuse lupus nephritis
glomerulonephritis involving 50% of all glomeruli, typically with diffuse subendothelial immune deposits.
-Class IV-S (A) Active lesions: diffuse segmental proliferative lupus nephritis
-Class IV-G (A) Active lesions: diffuse global proliferative lupus nephritis
-Class IV-S/G (A/C)Active - chronic lesions: diffuse segmental/global proliferative - sclerosing lupus nephritis
-Class IV-S (C) Chronic inactive lesions with scars: diffuse segmental sclerosing lupus nephritis
-Class IV-G (C) Chronic inactive lesions with scars: diffuse global sclerosing lupus nephritis
Class V Membranous lupus nephritis
Global or segmental subepithelial immune deposits or their morphologic sequelae by light
microscopy and by immunofluorescence or electron microscopy.
Class V lupus nephritis show advanced sclerosis
Class VI Advanced sclerosis lupus nephritis
90% of glomeruli globally sclerosed without residual activity
LES
Definizione:
malattia autoimmune con vasta gamma di manifestazioni cliniche
- Coinvolgimento renale
- Coinvolgimento articolare
- Coinvolgimento cutaneo
- Coinvolgimento polmonare
- Coinvolgimento cardiaco
- Coinvolgimento sierose
Etiopatogenesi
Figura 1
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Figura 2
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Figura 3
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Clinica
Variabile
- ematuria microscopica e macroscopica
- proteinuria non nefrosica, proteinuria moderata, proteinuria nefrosica
- riduzione acuta del GFR
Prognosi
- Indici di attività (0-24) e cronicità (12)
- CI è il più predittivo
<1 = 100% sopravvivenza a 10 anni
>4 = 32% sopravvivenza a 10 anni
Terapia
- CLASSE I-II non trattate
- Classe III steroide/immunosoppressore
- Classe IV steroide/immunosoppressore plasmaferesi
- Classe V 4-6 mesi di steroide e immunosoppressore
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