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Rare disease
CASE REPORT
Pulmonary vasculitis mimicking chronic thromboembolic disease
  1. Noor Ali1,
  2. Lynsey Clarke2,
  3. Robert Valentine MacKenzie Ross1 and
  4. Graham Robinson1
  1. 1 Radiology, Royal United Hospitals Bath NHS Foundation Trust, Bath, UK
  2. 2 North Bristol NHS Trust, Rheumatology, Bristol, UK
  1. Correspondence to Dr Noor Ali, noorali{at}doctors.org.uk

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.

  • radiology
  • pulmonary hypertension
  • vasculitis

https://doi.org/10.1136/bcr-2018-228409

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    Footnotes

    • Contributors All authors contributed to the write up of this case report. RVMR and LC provided clinical information and reviewed the final text. RVMR was also responsible for obtaining patient consent. GR and NA provided information relevant to the imaging. GR wrote the discussion. NA compiled and edited the final text.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Patient consent for publication Obtained.