Pulmonary vasculitis mimicking chronic thromboembolic disease

Area of Science:

• Cardiology
• Rheumatology
• Radiology

Background:

• Behçet's disease is a multisystemic inflammatory disorder.
• Pulmonary involvement in Behçet's disease can manifest as pulmonary vasculitis, mimicking other conditions.
• Chronic thromboembolic disease (CTED) is a significant cause of pulmonary hypertension.

Observation:

• A 29-year-old female presented with constitutional symptoms, oral ulceration, rash, chest pain, breathlessness, and syncope.
• Initial CT pulmonary angiogram (CTPA) suggested chronic thromboembolic disease (CTED).
• Positron emission tomography (PET)-CT revealed findings supportive of pulmonary vasculitis.

Findings:

• Treatment with intravenous cyclophosphamide led to symptom improvement.
• Repeat PET-CT showed resolution of pulmonary artery and mediastinal uptake.
• Pulmonary artery occlusions persisted on repeat CTPA, underscoring the complexity of imaging interpretation.

Implications:

• This case underscores the importance of considering pulmonary vasculitis in the differential diagnosis of pulmonary hypertension, especially in patients with features of Behçet's disease.
• Awareness of the distinct imaging features of pulmonary vasculitis versus CTED is crucial for accurate diagnosis and management.
• Prompt diagnosis and treatment of pulmonary vasculitis can lead to significant clinical improvement.