El término esclerosis sistémica o esclerodermia, proviene del griego: skleros (duro) y dermis (piel), por lo que etimológicamente significa piel dura. Es una infrecuente enfermedad autoinmune caracterizada por la disregulación inmune, vasculitis y fibrosis progresiva, afectando primordialmente a la piel, aparato digestivo, pulmones, corazón y riñones.
Tiene una prevalencia en el sur de Europa es de 276-443 por millón de habitantes y una incidencia de 14-21 por millón y año.
Existen dos formas clínicas de esclerodermia sistémica, la limitada y la difusa, siendo la LIMITADA mucho más frecuente que la segunda.
Tabla de contenidos
¿Qué síntomas produce la esclerosis sistémica?
Las manifestaciones clínicas de la ES suelen ser muy variadas, y a diferencia de otras enfermedades autoinmunes no suele cursas a brotes. Aquí se reflejan algunos de los principales síntomas de esta enfermedad:
- Un síntoma muy frecuente es el fenómeno de Raynaud, que se produce por la vasoconstricción de los vasos periféricos (en los dedos de las manos o pies o incluso nariz y/o orejas). Puede cambiar de color a blanco, rojo y/o azul.
- La esclerodactilia (endurecimiento cutáneo) se encuentra presente en el 100% ES limitada y en el 70% de las difusas. La lesión cutánea de la ES evoluciona en tres fases, siendo la primera edematosa, la segunda indurativa o esclerótica, y la tercera atrófica.
- Úlceras digitales: se pueden producir por el própio fenómeno de Raynaud (vasoconstricción prolongada en el tiempo), o por la propia tirantez cutánea de la esclerodactilia.
- Calcinosis cutánea: depósitos de sales de calcio a nivel subcutáneo, más de la esclerosis sistémica limitada.
- La afectación gastrointestinal es la tercera más frecuentemente en verse afectada en ES tanto limitada como difusa pudiendo afectar a cualquier tramo del tracto digestivo. La afectación esofágica es prácticamente universal en todos los pacientes. Los síntomas cardinales son disfagia y reflujo gastroesofágico (RGE). Seguidamente la disfunción intestinal suele estar presente entre el 50 y el 70% y en menor medida la afectación hepática o del estómago
- La afectación pulmonar es la segunda afectación visceral más frecuente después de la digestiva y la primera causa de muerte en estos pacientes. Dicha afectación suele estar presente hasta el 50% de las ES difusas predominantemente con la enfermedad pulmonar intersticial (EPI) y en el 25% de las limitadas con hipertensión pulmonar.
¿Qué esperaríamos encontrar en la analítica de sangre de un paciente con esclerosis sistémica?
Los anticuerpos característicos de la esclerosis sistémica, que entran dentro de los criterios de clasificación son Anticentromero, Anti-toipoisomerasa 1 (Scl-70) y Anti-RNA polimerasa III.
NO SON DIAGNÓSTICOS POR SI SOLOS DE LA ENFERMEDAD. De igual modo, que puede diagnósticarse una esclerosis sistémica sin presentar estos anticuerpos.
¿Cómo se diagnóstica la esclerosis sistémica?
Para el diagnóstico de esclerosis sistémica no basta con presentar en la analítica de sangre anticuerpos positivos, es necesario cumplir unos determinados criterios diagnósticos. Los que están vigentes en el momento actual son los ACR/EULAR 2013.
| CRITERIO | SUBCRITERIO | PUNTUACIÓN |
| Esclerosis cutánea de los dedos de ambas manos que se extiende sobre pasando las articulaciones metacarpofalángicas.
(CRITERIO SUFICIENTE) |
(-) | 9 |
| Esclerosis de dedos
(solo se cuenta la puntuación más alta) |
“Puffy fingers”
|
2
|
| Esclerodactilia | 4
|
|
| Lesiones en las puntas de los dedos.
(solo se cuenta la puntuación más alta) |
Úlceras digitales (distal a IFP)
|
2
|
| “Pitting” (mordedura de rata) | 3 | |
| Telangiectasias (maculas redondas, no arañas vasculares) | (-) | 2 |
| Alteraciones capilaroscopicas (dilatación y/o pérdida capilar) | (-) | 2 |
| Hipertensión arterial pulmonar y/o enfermermedad pulmonar intersticial. (Máximo 2 puntos). | Hipertensión pulmonar arterial (CCD)
|
2
|
| Enfermedad pulmonar intersticial (TAC, Rx o crepitantes en velcro) | 2 | |
| Fenómeno de Raynaud | (-) | 3 |
| Autoanticuerpos relacionados con SSc (máximo 3 puntos) | Anticentromero | 3 |
| Anti-toipoisomerasa 1 | 3 | |
| Anti-RNA polimerasa III | 3 |
¿Cómo se trata?
El tratamiento de esta enfermedad se individualizará a cada paciente en función de los síntomas que presente y del grado de severidad de los mismos. Es decir, no se tratará igual un paciente que presente fenómeno de Raynaud que afectación pulmonar.
E incluso dentro de un mismo síntoma, pongamos por ejemplo fenómeno de Raynaud, no se tratará igual un paciente que presente exclusivamente dolor, a otro que presente úlceras digitales.
Bibliografía
- Van den Hoogen F, Khanna D, Fransen J et al. 2013 Classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum 2013; 65: 2737-47
- Allanore Y, Simms R, Distler O et al. Nature review Primer: Systemic sclerosis. Nat Rev 2015; 1:1-21
- Pauling JD, Hughes M, Pope JE. Raynaud’s phenomenon-an update on diagnosis, classification and management. Clin Rheumatol. 2019;38:3317-3330.
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