ateneo central.ppt BIS 21 Reumatologia.pdf · 2016-06-29 · warfarin with a target INR of...
Transcript of ateneo central.ppt BIS 21 Reumatologia.pdf · 2016-06-29 · warfarin with a target INR of...
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ATENEO CENTRAL
Sección Reumatología Pediátrica21 de Junio 2016
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PIQUETE VASCULAR
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Objetivos
•Presentación de un paciente con debut poco habitual de LES
•Actualización sobre el tema
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Caso Clínico
Paciente de 14 añosSexo MasculinoDerivado de Clínica privada
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Internación Clínica PrivadaMotivo de internación: Cefalea intensaVómitos incoerciblesExamen físico: Rash malar
Exámenes complementarios:•Laboratorio 24/2/16: Hto 37 GB 7120 (78%‐15%)
Plaq 107.000 PCR 0.13 BT 0.33 BD 0.14 col 189•OC: prot +++•Proteinuria 24 Hs: 2.99 gr/24 hs•Ecografía abdominal: normal•HIV‐ VDRL‐ HBsAg (‐)
Tratamiento: PHP
cediendo los vómitos ALTA MEDICA con pedido de interconsulta a REUMATOLOGÍA por rash, plaquetopenia, proteinuria
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1ra consulta al servicio29 de Febrero de 2016
• Enantema• Eritema palmar• Eucardico• Eupneico• No visceromegalias• No sinovitis
●Regular estado general, afebril
● Somnoliento,
●Cefalea ++
● Fotofobia ++
●Rash malar
● Lesiones vasculíticas en pabellón auricular bilateral
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• Antecedentes:
‐Diciembre 2015: presentó cuadro febril con faringitis? medicado con cefalexina? por 20 días?
Ecografía parotídea: PAROTIDITIS
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Conducta
INTERNACIÓN
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INTERNACIÓN‐ CEM2•TAC Cerebro con y sin contraste:
Imagen sugestiva Trombosis Seno Venoso transversoSugieren complementar con AngioResonancia
•PL: prot 0,18 glu 52, no elementos
•Laboratorio: –Hto 40, Hb 13.2, GB 8560 (73%‐26%), –Plaq 230.000, VSG 81, hepatograma y coagulograma: normal
–OC: Prot (+++), Hb (–) Leuco 0‐2
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29 febrero 2016
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Signo del Delta vacío
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INTERNACION‐ CEM2• 2 de Marzo: (2 DIT)pico febril conducta expectantesomnoliento, cefalea, fotofobia.Orina 24 hs: 3,2 gr/24 hsPPD(‐), Ecoabdominal: normalOftalmología: edema de papila bilateralInició con acetazolamida
• 3 de Marzo: (3 DIT)Cardiología: ECG y Ecocardiograma: Normal PL en quirófano: para drenaje por HTE (Presión 14 mmHg)C3: 52; C4: 3.3; FAN +1/1280 (H); Anti DNA +1/160; Ro(+); La(‐); Sm(++); RNP (++),Anticardiolipinas y B2glicoproteinas (‐)
Indice Selena/SLEDAI 26
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Revaloración:
Una vez descartado cuadro infectológico se asume al paciente con diagnóstico de:
LUPUS ERITEMATOSO SISTEMICO CON COMPROMISO NEUROPSQUIATRICO
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Tratamiento Inicial
• 3 pulsos de Metilprednisolona Dosis: 1gr/dosis a pasar en días alternos
• luego continuar con meprednisona 80 mg (0.9 mg/kg/día)
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AngioRMN 4/3 (4 DIT)
Falta de relleno a nivel del seno venoso transverso con aumento del flujo colateral
homolateral en relación con TROMBOSIS VENOSA CEREBRAL
•Se inició anticoagulación con Enoxaparina (sc)luego switch a Acenocumarol
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Evolución
• Importante mejoría clínicaSin cefaleasSin fotofobiaMejoría de lesiones cutáneas
BIOPSIA RENAL (16 de Marzo)
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INTERNACION
DÍA 21: EPISODIO CONVULSIVO
Se sospecha SangradoVasculitis
•TAC cerebral: no evidencia signos de sangrado•Oftalmología: vasos tortuosos en FO
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RMN con angioresonancia
Correcta señal y configuración de ambas carótidas y sus ramas.Tronco basilar normalHipoplasia de arteria comunicante posterior derecha
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Conducta•Pulso de Metilprednisolona •Inicia Ciclofosfamida 1er pulso
No repite episodio convulsivo, en buen estado generalse indica alta hospitalaria.‐Meprednisona‐Hidroxicloroquina‐Acenocumarol ‐Enalapril‐Aportes (Ca, Vit D)
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Resultado de BIOPSIA RENAL
NEFROPATÍA LÚPICA MEMBRANOSA CLASE V
Incremento global y difuso de la matriz y celularidad mesangial. Engrosamiento irregular difuso leve de paredes capilares. Túbulos contorneados proximales con dilatación luminal focal.
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Actualmente
• Paciente en seguimiento interdisciplinario con Nefrología, Hematología y Reumatología
• Recibió 4 pulsos de Ciclofosfamida, en descenso de corticoides.
• Laboratorio 16/5: C3 136; C4 21.8; FAN (+) 1/1280; DNA (‐); Ro (‐); La (‐); Sm (‐); RNP(‐) B2glicoproteínas y Anticardiolipinas (‐)
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LUPUS ERITEMATOSO SISTEMICO
JUVENIL
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LES Juvenil
Enfermedad autoinmune multisistémica por excelencia que se caracteriza por la presencia de autoanticuerpos.
El curso clínico se caracteriza por recaídas y remisiones, siendo el pronóstico ampliamente variable.
Rose E, et al Texbook of Pediatrics Rheumatology 7th edition; (2016)285-317
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LES juvenil‐ Epidemiología
• Incidencia: 0.36‐ 2.5 cada 100.000 personas
• Edad de presentación: promedio 12 años
• Género: 4.5‐5: 1 (femenino: masculino) aumentando en la adolescencia
• Raza: negra y oriental
Rose E, et al Texbook of Pediatrics Rheumatology 7th edition (2016)285-317
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LES juvenil: Criterios Clasificación
tabla
Rose E, et al Texbook of Pediatrics Rheumatology 7th edition (2016)285-317
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Rose E, et al Texbook of Pediatrics Rheumatology 7th edition (2016) 285-317
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LUPUS NEUROPSIQUIATRICO
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Lupus neuropsiquiátrico
•Prevalencia variable de acuerdo a los reportes de casos (20‐95%)
•Más frecuente en la población pediátrica
Rose E, et al Texbook of Pediatrics Rheumatology 7th edition (2016)285-317
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Neuropsychiatric syndromes in systemic lupus erythematosus (ACR 1999)
Central nervous systemAseptic meningitisCerebrovascular diseaseDemyelinating syndromeHeadache (including migraine and benign
Intracranial hypertension)Movement disorder (chorea)MyelopathySeizure disordersAcute confusional stateAnxiety disorderCognitive dysfunctionMood disorder/depressionPsychosis
Peripheral nervous systemAcute inflammatory demyelinating
polyradiculoneuropathy(Guillain Barre Syndrome)Autonomic disorderMononeuropathy, single/multiplexMyasthenia gravisNeuropathy, cranialPlexopathyPolyneuropathy
ARTHRITIS & RHEUMATISM Vol. 42, No. 4, April 1999, pp 599–608
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Frecuencia de las manifestaciones clínicas en NP
Rose E, et al Texbook of Pediatrics Rheumatology 7th edition (2016)285-317
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Trombosis del seno venoso
Desorden poco frecuente en LES que es una manifestación de NPLE
Etiología: Vasculitis, sumado a la presencia de anticuerpos antifosfolipídicos y la tendencia protrombótica.
Li Wang, et al. Noviembre 2014. Clinical Characteristics of Cerebral Venous Sinus in SLE. Journal of Inmunology
Potencialmente fatal
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Trombosis del Seno Venoso• Prevalencia del sexo femenino• Forma de inicio: agudo (70.6%)• Poco frecuente como forma de presentación inicial de
LES (5.9%)• Clínica: cefalea persistente (88.2%),
nauseas/vómitos (47.1%) alt sensorio (41.2%) visión borrosa (35.3%), edema de papila (35.3%), convulsiones (35.3%)
• Sitio de Oclusión: seno transverso (82.4%)
Li Wang, et al. Noviembre 2014. Clinical Characteristics of Cerebral Venous Sinus in SLE. Journal of Inmunology
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Clinical criteria1. Vascular thrombosis
One or more clinical episodes of arterial, venous,or small-vessel thrombosis, in any tissue or organ. Thrombosis must be confirmed by objective validated criteria (i.e., unequivocal findings of appropriate imaging studies or histopathology). For histopathological confirmation, thrombosis should be present without significant evidence of inflammation in the vessel wall.
2. Pregnancy morbidity (a) One or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation, with normal fetal morphology documented by ultrasound or by direct examination of the fetus.
(b) One or more premature births of a morphologically normal neonate before the 34th week of gestation because of (i) eclampsia or severe preeclampsia defined according to standard definitions, or (ii) recognized features of placental insufficiency
(c) Three or more unexplained consecutive spontaneous abortions before the 10th week of gestation, with maternal anatomic or hormonal abnormalities, and paternal and maternal chromosomal causes excluded
Laboratory criteria
1. Lupus anticoagulant (LA) present in plasma, on two or more occasions at least 12 weeks apart, detected according to the guidelines of the International Society on Thrombosis and Hemostasis.
2. Anticardiolipin antibody of IgG and/or IgM isotype in serum or plasma, present in medium or high titer (i.e., >40 GPL or MPL, or greater than the99th percentile), on two or more occasions, at least 12 weeks apart, measured by a standardized ELISA.
3. Anti-β2 glycoprotein-I antibody of IgG and/or IgM isotype in serum or plasma (in titer greater than the 99th percentile), present on two or moreoccasions, at least 12 weeks apart, measured by a standardized ELISA.
Revised Classification Criteria for Antiphospholipid Syndrome
Note: APS is present if at least one of the clinical criteria and one of the laboratory criteria are met.
Rose E, et al Texbook of Pediatrics Rheumatology 7th editionRose E, et al Texbook of Pediatrics Rheumatology 7th edition
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Sindrome AntifosfolipídicoSummary of recommendations with regards to initiation of anticoagulation
General measures for aPL carriers 1. Address life‐style measures
2. Modify their cardiovascular risk factors
3. Thromboprophylaxis of usual doses of LMWH for high‐risk events, for example surgery or prolonged immobilisation
Anticoagulation for asymptomatic aPL carrier without SLE
1. Patients with low‐risk aPL antibody profile should be monitored
2. Patients with high‐risk aPL antibody profile with no history of thrombosis should be on a low‐dose aspirin
Anticoagulation for patients with aPL and SLE with no history of thrombotic events
1. Patients with newly‐diagnosed SLE should be regularly assessed for the presence of aPL
2. Patients with confirmed positive LA or persistent aCL at medium and/or high titres should be managed with low‐dose aspirin and hydroxychloroquine
Anticoagulation for patients with APS with first presentation of venous thrombosis
1. Patients with past history of proved APS with first non recurrent venous thrombosis should be anticoagulated with warfarin with a target INR of 2.0–3.0
Anticoagulation for patients with APS with first presentation of arterial thrombosis
1. Patients should be risk‐stratified with regard to their risk of bleeding and monitoring availability. The physician can decide on the appropriate management
2. High‐intensity anti‐coagulation, i.e. manage with warfarin at an INR > 3.0 OR
3. Combined anti‐aggregant anticoagulation therapy, i.e. INR 2.0‐3.0 with added low dose aspirin
Curr Rheumatol Rep (2013) 15:318
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ARTHRITIS & RHEUMATISM Vol. 64, No. 2, February 2012, pp 504–512
Risk factors for thrombosis in autoimmune disease, asdetermined using multivariate analysis
Risk factor Hazard ratio (95% CI)*Glucocorticoid treatment 1.979 (0.809–4.842)History of thrombosis 1.401 (0.640–3.068)Hypertension 1.621 (0.750–3.504)Hyperlipidemia 1.917 (0.927–3.966)Diabetes 0.963 (0.394–2.355)Age 1.017 (0.987–1.047)Male sex 1.002 (0.385–2.606)Systemic lupus erythematosus 1.052 (0.480–2.303)Rheumatoid arthritis 0.470 (0.101–2.181)Antiphospholipid score more than 30 3.144 (1.383–7.150)†
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SERVICIO HEMATOLOGIA
Cuidados del paciente anticoaguladoInteracción con medicamentos/alimentosActividad físicaTiempo de anticoagulación
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SERVICIO NEUROCIRUGIA
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Conclusión
• El LES es la enfermedad autoinmune MULTISISTÉMICA por excelencia.
• La forma de presentación es muy heterogénea.
• El debut con cuadro de trombosis en SNC es muy infrecuente.
• Todo hallazgo en un paciente con sospecha clínica de LES deberá jerarquizarse y sospecharse como relacionado a la misma o a una intercurrencia infecciosa dado que ambas situaciones pueden poner en riesgo de vida al paciente.
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Bibliografía
● Li Wang, et al. Noviembre 2014.Clinical Characteristics of Cerebral Venous
Sinus in SLE. Journal of Inmunology
● Rose E, et al Texbook of Pediatrics Rheumatology 7th edition; (2016)285-
317
● ARTHRITIS & RHEUMATISM Vol. 42, No. 4, April 1999, pp 599–608
Clinical Characteristics of Cerebral Venous Sinus in SLE. Journal of
Inmunology
● Curr Rheumatol Rep (2013) 15:318
● ARTHRITIS & RHEUMATISM Vol. 64, No. 2, February 2012, pp 504–512
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Agradecimientos
Equipo médico del CEM 2Servicio de NefrologíaServicio de HematologíaServicio de ImágenesServicio de NeurocirugíaEquipo de Reumatología