Pulmonary vasculitis mimicking chronic thromboembolic disease

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 w...

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Bibliographic Details
Published in:BMJ case reports 2019-04, Vol.12 (4), p.e228409
Main Authors: Ali, Noor, Clarke, Lynsey, MacKenzie Ross, Robert Valentine, Robinson, Graham
Format: Article
Language:English
Subjects:
Administration, Intravenous
Adult
Anemia
Ankle
Anticoagulants
Antirheumatic Agents - administration & dosage
Arthritis
Behcet Syndrome - complications
Blood clots
Cardiology
Case reports
Congenital diseases
Cyclophosphamide - administration & dosage
Diagnosis, Differential
Dyspnea
Echocardiography
Family medical history
Female
Heart
Humans
Hypertension, Pulmonary - complications
Hypertension, Pulmonary - diagnosis
Hypertension, Pulmonary - drug therapy
Literature reviews
Medical diagnosis
Medical imaging
Pain
Patients
Positron Emission Tomography Computed Tomography
Pulmonary arteries
Pulmonary Artery - diagnostic imaging
Pulmonary Artery - physiopathology
Pulmonary hypertension
Rare Disease
Surgery
Thromboembolism
Thromboembolism - complications
Thromboembolism - diagnosis
Ulcers
Vasculitis - complications
Vasculitis - diagnosis
Vasculitis - drug therapy
Veins & arteries
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Description
Summary: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.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2018-228409