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Desenmascarar la enfermedad pulmonar venooclusiva después del tratamiento con vasodilatadores en la esclerosis

Nanditha Sidda¹, Rama Bhat², Praveen Tirlangi³

¹General Medicine, Kasturba Medical College Manipal, Manipal, Karnataka, India.

BMJ case reports

|August 27, 2025

Resumen

La enfermedad pulmonar venooclusiva (PVOD) es una causa rara de hipertensión pulmonar a menudo confundida con hipertensión arterial pulmonar (HAP). La diferenciación de la PVOD es crítica, ya que los tratamientos para la HAP pueden dañar a los pacientes con PVOD.

Área de la Ciencia:

Medicina cardiovascular
Medicina de los pulmones
Reumatología

Sus antecedentes:

La enfermedad pulmonar venooclusiva (PVOD) es una forma rara y grave de hipertensión pulmonar.
PVOD se caracteriza por la oclusión de la vena pulmonar debido a la fibrosis y la proliferación íntima.
El diagnóstico erróneo de PVOD como hipertensión arterial pulmonar (HAP) puede conducir a resultados perjudiciales en el tratamiento.

Objetivo del estudio:

Para resaltar la rara asociación entre la esclerosis sistémica (SSc) y la PVOD.
Hacer hincapié en la importancia de distinguir la PVOD de la HAP en pacientes con SSc.
Subrayar la necesidad de enfoques diagnósticos y terapéuticos adaptados en el caso de la PVOD asociada a la SSc.

Principales métodos:

Informe de caso de un paciente con SSc cutánea limitada que presentaba una dispnea progresiva.
Revisión de los desafíos de diagnóstico y las implicaciones del tratamiento para diferenciar el PVOD del HAP.
Evaluación clínica y análisis de la historia clínica del paciente.

Principales resultados:

El paciente, diagnosticado inicialmente con HAP, experimentó una exacerbación de los síntomas después de recibir vasodilatadores pulmonares.
Este resultado adverso sugiere un posible diagnóstico erróneo de PVOD.
El caso subraya el vínculo poco frecuente pero crítico entre SSc y PVOD.

Conclusiones:

Se debe considerar la PVOD en pacientes con SSc que presentan hipertensión pulmonar, especialmente si empeoran con vasodilatadores.
La diferenciación precisa entre los PVOD y los HAP es esencial para una gestión adecuada.
La evaluación diagnóstica cuidadosa y la estrategia terapéutica son cruciales para los pacientes SSc con hipertensión pulmonar.

Palabras clave:

Enfermedad del tejido conectivo Hipertensión pulmonar

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