Pulmonary vasculitis mimicking chronic thromboembolic disease

• 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
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2018-228409

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.