¿nos aporta algo la serología a la biopsia · pdf file• vasculitis lesiones...

NOS APORTA ALGO LA SEROLOGA A LA BIOPSIA RENAL?
Curso de Nefropatologa Sociedad Norte de Nefrologa
Viana, Navarra 16 de Mayo 2015

Caso Clinico
Mujer de 21 aos. Artralgias, artritis en los ltimos 9 meses. Eritema malar
Se detectan ANA y AntiDNA +. Diagnosticada de LES, comienza con Hidroxicloroquina.
Dos meses ms tarde, orinas oscuras y edema. TA 140/90 mmHg
Proteinuria 4 g/24h, sedimento con incontables hemates, Crs de 2 mg/dl

Caso Clnico
Biopsia Renal ? Esteroides+MMF o Ciclofosfamida? Y seguimiento-ajuste de tratamiento segn evolucin de Crs, proteinuria, sedimento. Escaso valor de los ANA-Anti-DNA salvo para el diagnstico

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
May be a few isolated subepithelial or subendothelial deposits visible by immunofluorescence or electron microscopy, but not by light microscopy
Class III Focal lupus nephritisa
Active or inactive focal, segmental or global endo- or extracapillary glomerulonephritis involving

Lupus Eritematoso Sistmico Lesiones de Actividad Necrosis Fibrinoide Asas de Alambre Semilunas celulares Cariorrexis Cuerpos hematoxinfilos Infiltrados intersticiales Vasculitis
Lesiones de Cronicidad Glomrulos esclerosados Fibrosis intersticial Semilunas fibrosas Esclerosis intersticial Angiosclerosis

Biopsias Renales de Protocolo en el LES?

Mujer de 37 aos, nacida en Marruecos. Habla muy poco espaol. Desde hace 4 semanas, edema en miembros inferiores, con fvea,
hasta rodillas. Tambin nota edema palpebral. No refiere otros sntomas. Exploracin Fsica. TA 110/70 mmHg. T 36.8 C. Edema hasta rodillas. Resto normal

ANALTICA Crs 0.6 mg/dl. Ccr 77 ml/min Hgb 14.8 g/dl, leucocitos y plaquetas normales Protenas totales: 5 g/dl. Albmina 1.8 g/dl. Colesterol 289 mg/dl; Triglicridos 183 mg/dl Estudio de coagulacin normal. Proteinograma normal Proteinuria 6.5 g/da. Sedimento: 3-5 hxc. Resto normal.

Inmunoelectroforesis en sangre y orina normales Inmunoglobulinas: IgG 598; IgA 315 ; IgM 212 mg/dl. C3 101 mg/dl; C4 45 mg/dl; ANA 1/40; Anti-DNA (-) VHC, VHB, VIH negativos
Ecografa abdominal normal, a excepcin de pequea cantidad de lquido libre. Riones normales.

-El Patlogo nos informa de que no hay material adecuado para Inmunofluorescencia.
-Con el diagnstico de lesiones mnimas, se inicia
tratamiento con prednisona, 1 mg/Kg/da y furosemida 1-2 comp/da.
- Al cabo de 1 mes de tratamiento la enferma contina
edematosa y empieza a notar facies cushingoide. La Crs es 0.7 mg/dl, Proteinuria 8 g/24 h, PT 5.1 g/dl,
albmina srica 2 g/dl.

Kidney International, 24 (1983), pp. 377385 Pathologic differentiation between lupus and nonlupus membranous glomerulopathy JENNETTE JC et al.

Se reinterroga a la paciente con traductor: artralgias e incluso artritis de articulaciones de manos en los ltimos meses. Rash malar en el ltimo verano.
Se repite serologa : C3 86 mg/dl; C4 21 mg/dl ANA 1/360; Anti DNA negativo. Resto de Ac negativos
incluidos Ac anticardiolipina
Se establece el diagnstico de Membranosa Lpica

LN was confirmed in 1092 patients (31%). Most had LN proliferative forms (70%), and there were only 16 cases of thrombotic microangiopathy (TMA). Complete response to treatment was achieved in 68.3% of patients, whereas 17.9% remained with renal activity. A higher risk for persistence of renal activity was found with higher levels of baseline serum creatinine (1 vs 0.91, p=0.004) and proteinuria (2.76 vs 2.4, p=0.006). ESRD was clinically associated with positive a-dsDNA, low complement, pleuropericarditis, seizures (all p

Characterization of Patients with Lupus Nephritis Included in a Large Cohort from the Spanish Society of Rheumatology Registry of Patients with Systemic Lupus Erythematosus
RELESSER is a multicentre cross-sectional study, with information retrospectively collected from the charts of patients with SLE followed up at participant rheumatology units. Globally, 359 variables including demographic and clinical data, activity, severity, comorbidities, treatments and mortality were recorded. The following renal data were included: WHO LN histological type, proteinuria, haematuria, leukocyturia, cellular casts and creatinine clearance, treatment response, recurrence, development of ESRD and/or the need for dialysis or renal transplantation.

BIOMARCADORES
Proteinuria/Albuminuria
Sedimento Urinario
Funcin renal (Crs, eGFR)

HEMATURIA
Hematuria no glomerular, de vas o urolgica Tumores Pruebas de Imagen Infecciones Ecografa Litiasis TAC Hipercalciuria/ Urografa I.V. Hiperuricosuria

PROTEINURIA CUANTIFICACIN POR UNIDAD DE TIEMPO Por Ejemplo: 1.5 g/24 horas Cociente Proteinas (Albmina)/Creatinina en muestra aislada de orina o en orina de 24 h Por Ejemplo: Cociente Proteinas/Creatinina: 2.5 g/g Cociente Albmina/Creatinina: 325 mg/g EVITAR: proteinuria de +++ proteinuria de 3.6 g/litro

Caso Clnico Mujer de 59 aos. Diagnosticada de LES a los 19 aos.
Remitida por Anemia grave (Hgb 7 g/dl)
Crs de 2 mg/dl MDRD 28 ml/min) , ANA y anti-DNA (-)
IRC desde al menos 5 aos. Ninguna determinacin de proteinuria por unidad de tiempo (tiras o mg/dl)
Nunca Biopsia renal, nunca valorada por Nefrlogos
Informacin al paciente

Caso Clnico
Varn de 75 aos. Astenia, decaimiento general, disnea en las ltimas semanas. Antecedentes de rinitis con epistaxis y sinusitis
Rx Torax:normal Anemia, resto de hemograma normal. Crs 5.5 mg/dl. St con
intensa hematuria, proteinuria 2.5 mg/dl ANA, Anti-DNA, Anti MBG (-), ANCA (AntiPR3) +++. Serologa
viral negativa, complemento normal
Biopsia Renal o Esteroides+Ciclofosfamida (alternativa: rituximab)+plasmaferesis?

VASCULITIS DE PEQUEO VASO
Granulomatosis de Wegener** Poliangetis microscpica** Churg-Strauss** Schonlein-Henoch Vasculitis Crioglobulinmica Vasculitis cutneas
** ANCA +

C-ANCA Anti- Proteinasa 3
P-ANCA Anti-mieloperoxidasa
Wegener 90% 10%
Poliangetis microscpica
50% 50%
Churg-Strauss 10% 90%
VASCULITIS ANCA +
ANCA: Gran importancia en el diagnstico (y en la patognesis). Pero utilidad relativa en el seguimiento

Classification flowchart.
Annelies E. Berden et al. JASN 2010;21:1628-1636
2010 by American Society of Nephrology

Typical examples of glomerular lesions in each of the four categories.

Renal survival (no development of end-stage renal failure) is depicted according to the four histologic categories.

Beck L et al. N Engl J Med 2009;361:11-21
Expression of the M-Type Phospholipase A2 Receptor (PLA2R) in Normal Kidney Tissue and Glomeruli

Beck L et al. N Engl J Med 2009;361:11-21
Antibody against the M-Type Phospholipase A2 Receptor (PLA2R) and Disease Activity in a Patient with Membranous Nephropathy

CJSAN 2011

Proteinuria and PLA2R antibody levels of all patients included in the study.
Elion Hoxha et al. JASN 2014;25:1357-1366

Proteinuria and PLA2R antibody levels of patients treated with immunosuppressive therapy.

Beck LH, Salant DJ. Membranous nephropathy: recent travels and new road ahead. Kidney Int 2010.

Time to achievement of remission of proteinuria in patients with high versus low PLA2R antibody levels at study inclusion.

KaplanMeier plot for survival in remission, grouped by PLA2R antibody status at end of therapy.
Anneke P. Bech et al. CJASN 2014;9:1386-1392

Working diagnostic tree using IgG4 anti-PLA2R1 activity at the time of renal transplantation and during follow-up, and proteinuria changes after renal transplantation.
Barbara Seitz-Polski et al. Nephrol. Dial. Transplant. 2014;29:2334-2342
The Author 2014. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

Non-Nephrotic Proteinuria ACEI/ARB
Nephrotic syndrome (NS)
Observation Period in all the patients (Except in those with renal function decline) Treatment of NS: Diet, diuretics,

¿nos aporta algo la serología a la biopsia · pdf file• vasculitis lesiones...

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