Pulmonary vasculitis mimicking chronic thromboembolic disease

BMJ Case Rep. 2019 Apr 8;12(4):e228409. doi: 10.1136/bcr-2018-228409.

Abstract

A 29-year-old female patient presented with chest pain, breathlessness and syncope on the background of constitutional symptoms, oral ulceration and a rash. Multiple investigations were performed, including a CT pulmonary angiogram (CTPA) that was initially felt to show imaging features consistent with a diagnosis of chronic thromboembolic disease (CTED). The patient was referred to a tertiary pulmonary hypertension centre and the possibility of pulmonary vasculitis was raised. Subsequent positron emission tomography (PET)-CT revealed imaging features supporting this diagnosis. The patient was treated with intravenous cyclophosphamide infusions, following which her symptoms improved. A repeat PET-CT 6 months after treatment showed resolution in pulmonary artery and mediastinal uptake, but persistence of pulmonary artery occlusions on a repeat CTPA. A final diagnosis of pulmonary vasculitis secondary to Behçet's disease was made. This case report aims to raise awareness of the imaging features of CTED and its mimics.

Keywords: pulmonary hypertension; radiology; vasculitis.

Publication types

  • Case Reports

MeSH terms

  • Administration, Intravenous
  • Adult
  • Antirheumatic Agents / administration & dosage
  • Behcet Syndrome / complications
  • Cyclophosphamide / administration & dosage
  • Diagnosis, Differential
  • Echocardiography
  • Female
  • Humans
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / drug therapy
  • Positron Emission Tomography Computed Tomography
  • Pulmonary Artery / diagnostic imaging*
  • Pulmonary Artery / physiopathology
  • Thromboembolism / complications
  • Thromboembolism / diagnosis
  • Vasculitis / complications
  • Vasculitis / diagnosis*
  • Vasculitis / drug therapy

Substances

  • Antirheumatic Agents
  • Cyclophosphamide