The document discusses neurologic manifestations of Sjögren's syndrome. It notes that peripheral nervous system involvement occurs in 15-20% of patients. The most common manifestations are sensory ganglionopathy (39%), small fiber neuropathy (20%), and trigeminal neuropathy (16%). Sensory ganglionopathy presents with loss of sensory nerve action potentials and preserved motor function. Small fiber neuropathy affects alpha-delta and C fibers and causes symptoms like dysesthesia. Trigeminal neuropathy is the most common cranial neuropathy and progresses indolently. Autonomic neuropathy symptoms are present in around 50% of patients and include abnormalities like Adie's pupils.

1. NEURO SJÖGREN
COMOPROMISO PERIFERICO
ALVARO FERNANDO AGUILAR
RESIDENTE DE MEDICINA INTERNA
UNIVERSIDAD DE LA SABANA

3. GENERALIDADES
3 A 11 X 100 MIL  Prevalencia 0.01% a 0.72%
•
Xerostomía 30 – 40 %
Riesgo de linfoma de células B 15 a 20 veces >
No hay estudios para para manejo organoespecifico
Manifestaciones sistémicas 30 – 40%
Xavier Mariette, Ph.D., and Lindsey A. Criswell. Primary Sjögren’s Syndrome. Nengl j med 378;10 nejm.org March 8, 2018

4. Article number: 16047 doi:10.1038/nrdp.2016.47, Published online 7 July 2016 Sjögren syndrome

5. Article number: 16047 doi:10.1038/nrdp.2016.47, Published online 7 July 2016 Sjögren syndrome

6. CRITERIOS DE EXCLUSIÓN
Hepatitis C activa
Antecedente de radioterapia cervical
Sarcoidosis
Enfermedad del tejido vs huesped
Anticolinergicos
Enfermedad por IgG 4
Xavier Mariette, Ph.D., and Lindsey A. Criswell. Primary Sjögren’s Syndrome. Nengl j med 378;10 nejm.org March 8, 2018

7. Xavier Mariette, Ph.D., and Lindsey A. Criswell. Primary Sjögren’s Syndrome. Nengl j med 378;10 nejm.org March 8, 2018

8. SINDRO
DE
SJÖGREN PRIMARIO No enf. asociada
Secundario
Artritis R.
Lupus
Esclerodermia
Ramos-Casals M, et al. Ann Rheum Dis 2019;0:1–16. doi:10.1136/annrheumdis-2019-216114

10. Article number: 16047 doi:10.1038/nrdp.2016.47, Published online 7 July 2016 Sjögren syndrome

11. COMPROMISO PERIFERICO
Robert I. Fox and Julius Birnbaum. Sjögren’s Syndrome: Interface of Immunology and Neurology. Springer Nature Switzerland AG 2019 51, T. A. Cho et al. (eds.), Neurorheumatology, https://doi.org/10.1007/978-3-030-16928-2_6

12. GENERALIDADES
•16% simultanea
Presentación previa a dx (92%)
5 – 15 % De los pacientes
SSA-Ro 33-74%  10 – 50 %
Fibra pequeña  Ro +
ANAS 20 – 67 %
SENSIBILIDAD 50 %

13. Robert I. Fox and Julius Birnbaum. Sjögren’s Syndrome: Interface of Immunology and Neurology. Springer Nature Switzerland AG 2019 51, T. A. Cho et al. (eds.), Neurorheumatology, https://doi.org/10.1007/978-3-030-16928-2_6

15. Gangliopatia sensitiva
39%
Neuropatia dolorosa de
fibra pequeña 20%
Neuropatía trigeminal
16%
Mononeuropatia múltiple
12%
Neuropatia craneal multiple
Poliradiculopatias
Neuropatias autonómicas
3 al 5%
Julius Birnbaum. Peripheral Nervous System Manifestations of. Sjögren Syndrome. Clinical Patterns, Diagnostic Paradigms, Etiopathogenesis, and Therapeutic Strategies. The Neurologist • Volume 16, Number 5, September 2010

16. GANGLIOPATIA SENSITIVA
Potenciales de acción de nervio sensorial no detectables
Preservacion del componente motor
Potenciales evocados ausentes
Híperintensidad en T2 limitada (Gracilis y cuneatus)
Julius Birnbaum. Peripheral Nervous System Manifestations of. Sjögren Syndrome. Clinical Patterns, Diagnostic Paradigms, Etiopathogenesis, and Therapeutic Strategies. The Neurologist • Volume 16, Number 5, September 2010

17. GANGLIOPATIA SENSITIVA
Julius Birnbaum. Peripheral Nervous System Manifestations of. Sjögren Syndrome. Clinical Patterns, Diagnostic Paradigms, Etiopathogenesis, and Therapeutic Strategies. The Neurologist • Volume 16, Number 5, September 2010

18. • Anticuerpos anti GW/B
• mRNA no codificante
• Proteínas secuestradas en los cuerpos G
GANGLIOPATIA SENSITIVA
Julius Birnbaum. Peripheral Nervous System Manifestations of. Sjögren Syndrome. Clinical Patterns, Diagnostic Paradigms, Etiopathogenesis, and Therapeutic Strategies. The Neurologist • Volume 16, Number 5, September 2010

19. 55 pacientes Anticuerpos anti GW/B
31 % SSJ 33% neuropatias

20. GANGLIOPATIA SENSITIVA
Presentación con parestesias
Propiocepción alterada
•  Movimientos finos
•  Inestabilidad
Movimientos hiperquineticos
VIH, Encefalitis, cisplatino, piridoxina.
Julius Birnbaum. Peripheral Nervous System Manifestations of. Sjögren Syndrome. Clinical Patterns, Diagnostic Paradigms, Etiopathogenesis, and Therapeutic Strategies. The Neurologist • Volume 16, Number 5, September 2010

21. TRATAMIENTO ?
Pobre respuesta a manejo inmuno supresor
Manifestación de daño crónico
Ventana terapéutica estrecha
GANGLIOPATIA SENSITIVA

22. NEUROPATIA DE FIBRA PEQUEÑA
Afección de fibras A-alfa Y C
50 % con SSJ disestesias
5 – 10% de todos los pacientes
Alodinia, disestesias, temperatura
Estudios electrofisiológicos normales
DM, amiloidosis, sx paraneoplasicos, tratamiento VIH
Julius Birnbaum. Peripheral Nervous System Manifestations of. Sjögren Syndrome. Clinical Patterns, Diagnostic Paradigms, Etiopathogenesis, and Therapeutic Strategies. The Neurologist • Volume 16, Number 5, September 2010

23. NEUROPATIA DE FIBRA PEQUEÑA
Julius Birnbaum. Peripheral Nervous System Manifestations of. Sjögren Syndrome. Clinical Patterns, Diagnostic Paradigms, Etiopathogenesis, and Therapeutic Strategies. The Neurologist • Volume 16, Number 5, September 2010

24. NEUROPATIA DE FIBRA PEQUEÑA
Anti epilépticos
•Carbamazepina, gabapentin
Triciclicos
Opioides
Julius Birnbaum. Peripheral Nervous System Manifestations of. Sjögren Syndrome. Clinical Patterns, Diagnostic Paradigms, Etiopathogenesis, and Therapeutic Strategies. The Neurologist • Volume 16, Number 5, September 2010

25. NEUROPATÍA TRIGÉMINAL
Neuropatía craneal más común
Progresiva - Indolente
Si se presenta con fibra pequeña  gangliopatia
Compromiso motor < frecuente

26. NEUROPATÍA AUTONÓMICA
Síntomas presentes en el
50 % de los pacientes
Pupilas de Adie 50 %
40 % ortostatismo
70 % sudoración excesiva
• Frecuencia urinaria, disfunción
erectil, intolerancia frio/calor
• Alteraciones gástricas
Julius Birnbaum. Peripheral Nervous System Manifestations of. Sjögren Syndrome. Clinical Patterns, Diagnostic Paradigms, Etiopathogenesis, and Therapeutic Strategies. The Neurologist • Volume 16, Number 5, September 2010

27. Ramos-Casals M, et al. Ann Rheum Dis 2019;0:1–16. doi:10.1136/annrheumdis-2019-216114