Chronic active Epstein-Bar virus infection complicated by pulmonary artery hypertension

Yukiko Misaki1, Daisuke Minakata2, Tatsuro Ibe3

  • 1Division of Hematology, Jichi Medical University Saitama Medical Center, Japan.

Insights

Chronic active Epstein-Bar virus infection (CAEBV) can lead to pulmonary artery hypertension (PAH). Treating PAH with vasodilators and CAEBV with chemotherapy improved patient outcomes, suggesting a link between EBV vasculitis and PAH.

Area of Science:

  • Infectious Diseases
  • Cardiology
  • Pathology

Background:

  • Chronic active Epstein-Bar virus infection (CAEBV) presents with diverse symptoms.
  • Pulmonary artery hypertension (PAH) is a known cardiovascular complication of CAEBV, but its mechanisms and treatment efficacy remain unclear.

Observation:

  • This study details 4 adult patients with CAEBV and concurrent PAH.
  • Patients received vasodilator therapy for PAH, followed by chemotherapy (with or without stem cell transplantation) for CAEBV.

Findings:

  • Transtricuspid pressure gradient improved with vasodilators in all patients.
  • Three patients showed further improvement after CAEBV-directed treatment.
  • Autopsies revealed Epstein-Barr virus-encoded small RNA (EBER)-positive cells and pulmonary artery pathology, indicating EBV-driven vasculitis.

Implications:

  • Epstein-Barr virus-infected cells can induce vasculitis, leading to PAH.
  • PAH associated with CAEBV is potentially treatable with both PAH-specific medications and CAEBV therapies.

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